Surgical Atlas of Orbital Diseases

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Surgical Atlas of

Orbital Diseases

System requirement: • Windows XP or above • Power DVD player (Software) • Windows media player 10.0 version or above • Quick time player version 6.5 or above Accompanying DVD ROM is playable only in Computer and not in DVD player. Kindly wait for few seconds for DVD to autorun. If it does not autorun then please do the following: • Click on my computer • Click the DVD drive labelled JAYPEE and after opening the drive, kindly double click the file Jaypee

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Surgical Atlas of

Orbital Diseases

Subrahmanyam Mallajosyula MS, DO Head, Dept of Ophthalmology Bhaskar Medical College Former Superintendent and Chief Dept of Oculoplastics and Orbital Services Sarojini Devi Eye Hospital Hyderabad, Andhra Pradesh, India

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JAYPEE BROTHERS MEDICAL PUBLISHERS (P) LTD New Delhi • Ahmedabad • Bengaluru • Chennai • Hyderabad • Kochi • Kolkata • Lucknow • Mumbai • Nagpur

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Published by Jitendar P Vij Jaypee Brothers Medical Publishers (P) Ltd Corporate Office 4838/24 Ansari Road, Daryaganj, New Delhi - 110002, India, Phone: +91-11-43574357 Registered Office B-3 EMCA House, 23/23B Ansari Road, Daryaganj, New Delhi - 110 002, India Phones: +91-11-23272143, +91-11-23272703, +91-11-23282021 +91-11-23245672, Rel: +91-11-32558559, Fax: +91-11-23276490, +91-11-23245683 e-mail: [email protected], Visit our website: www.jaypeebrothers.com Branches 2/B, Akruti Society, Jodhpur Gam Road Satellite Ahmedabad 380 015, Phones: +91-79-26926233, Rel: +91-79-32988717 Fax: +91-79-26927094, e-mail: [email protected] 202 Batavia Chambers, 8 Kumara Krupa Road, Kumara Park East Bengaluru 560 001, Phones: +91-80-22285971, +91-80-22382956, 91-80-22372664 Rel: +91-80-32714073, Fax: +91-80-22281761 e-mail: [email protected] 282 IIIrd Floor, Khaleel Shirazi Estate, Fountain Plaza, Pantheon Road Chennai 600 008, Phones: +91-44-28193265, +91-44-28194897, Rel: +91-44-32972089 Fax: +91-44-28193231 e-mail: [email protected] 4-2-1067/1-3, 1st Floor, Balaji Building, Ramkote Cross Road, Hyderabad 500 095, Phones: +91-40-66610020, +91-40-24758498 Rel:+91-40-32940929, Fax:+91-40-24758499 e-mail: [email protected] No. 41/3098, B & B1, Kuruvi Building, St. Vincent Road Kochi 682 018, Kerala, Phones: +91-484-4036109, +91-484-2395739 +91-484-2395740 e-mail: [email protected] 1-A Indian Mirror Street, Wellington Square Kolkata 700 013, Phones: +91-33-22651926, +91-33-22276404 +91-33-22276415, Rel: +91-33-32901926, Fax: +91-33-22656075 e-mail: [email protected] Lekhraj Market III, B-2, Sector-4, Faizabad Road, Indira Nagar Lucknow 226 016 Phones: +91-522-3040553, +91-522-3040554 e-mail: [email protected] 106 Amit Industrial Estate, 61 Dr SS Rao Road, Near MGM Hospital, Parel Mumbai 400 012, Phones: +91-22-24124863, +91-22-24104532, Rel: +91-22-32926896, Fax: +91-22-24160828 e-mail: [email protected] “KAMALPUSHPA” 38, Reshimbag, Opp. Mohota Science College, Umred Road Nagpur 440 009 (MS), Phone: Rel: +91-712-3245220, Fax: +91-712-2704275 e-mail: [email protected] USA Office 1745, Pheasant Run Drive, Maryland Heights (Missouri), MO 63043, USA, Ph: 001-636-6279734 e-mail: [email protected], [email protected] Surgical Atlas of Orbital Diseases © 2008, Jaypee Brothers Medical Publishers All rights reserved. No part of this publication and DVD ROM should be reproduced, stored in a retrieval system, or transmitted in any form or by any means: electronic, mechanical, photocopying, recording, or otherwise, without the prior written permission of the editor and the publisher. This book has been published in good faith that the material provided by contributors is original. Every effort is made to ensure accuracy of material, but the publisher, printer and editor will not be held responsible for any inadvertent error(s). In case of any dispute, all legal matters are to be settled under Delhi jurisdiction only. First Edition: 2009 ISBN 978-81-8448-394-9 Typeset at JPBMP typesetting unit Printed at Ajanta Offset & Packagins Ltd., New Delhi

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This book is dedicated to my family members, my teachers, my team members and my patients

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Contributors B Ranganadha Reddy MS Professor and Head Dept of Otorhinolaryngiology Gandhi Medical College Hyderabad, India

Geeta K Vemuganti MD Director Ophthalmic Pathology Service LV Prasad Eye Institute Hyderabad, India

Cat N Burkat MD Assistant Professor Oculoplastic Surgery Service Department of Ophthalmology and Visual Sciences University of Wisconsin 600 Highland Avenue Madison, WI 53792, USA

Golam Haider MS, FCPS Associate Professor Dept of Oculoplastics and Orbital Services National Institute of Ophthalmology Dhaka, Bangladesh

Christopher M Knapp BSc (Hons), FRC Ophth Clinical Lecturer Dept of Ophthalmology University of Leicester Leicester Royal Infirmary Leicester LEI 5 WW, UK D Ravi Varma DM (Neuroradiology) Consultant, Interventional and Neuroradiologist Dept of Radiology Krishna Institute of Medical Sciences Hyderabad, India Debraj Shome DO, DNB, FRCS (Glasgow), MNAMS, MS Consultant, Department of Ophthalmic and Facial Plastic and Ocular Oncology Aditya Jyot Eye Hospital Pvt Ltd Mumbai and Honorary Consultant Dept of Ocular Oncology Advanced Center for Treatment Research and Education in Cancer Tata Memorial Center Mumbai, India Dinesh Selva MBBS (Hons), FRACS, FRANZCO Professor and Chairman South Australian Institute of Ophthalmology University of Adelaide, Australia

Jack Rootman MD, FRCSC Professor Dept of Ophthalmology and Pathology University of British Columbia Vancouver, Canada Kahana Alon MD, PhD Assistant Professor Oculoplastic Surgery Service Department of Ophthalmology and Visual Sciences Kellogg Eye Center, University of Michigan 1000 Wall Street, Ann Arbor, MI 48105, USA Kasturi Bhattacharjee MS, DNB, FRCS (Ed) Senior Consultant and Head Dept of Orbit Ophthalmoplastic and Reconstructive Surgery Shankaradeva Netralaya Beltola, Guwahati, Assam India Kuldeep Raizada PhD Head Dept of Ocular Prosthesis Service LV Prasad Eye Institute Hyderabad Leaurence Brown FRC (Path) Consultant Histopathologist Dept of Pathology Leicester Royal Infirmary Leicester LEI 5 WW, UK

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viii Surgical Atlas of Orbital Diseases M Chandrasekhara Reddy MS, MCh Professor, Dept of Neurosurgery Gandhi Medical College Hyderabad, India Mark J Lucarelli MD Associate Professor Oculoplastic Surgery Service Department of Ophthalmology and Visual Sciences University of Wisconsin, 600 Highland Avenue Madison, WI 53792, USA Modini Pandarpurkar MS Assistant Professor of Ophthalmology Fellow Oculoplastics and Orbital Services Sarojini Devi Eye Hospital Hyderabad, India Mohd Ather MS Assistant Professor of Ophthalmology Oculoplastics and Orbital Services Sarojini Devi Eye Hospital Hyderabad, India Mohd Javed Ali MS, FRCS, FRCGP Fellow, Dept of Oculoplastics and Orbital Services Sarojini Devi Eye Hospital Hyderabad, India Nancy Kim MD, PhD Fellow, Oculoplastics and Orbital Services University of Wisconsin Medical School Madison, Wisconsin, USA Peter J Dolman MD, FRCSC Associate Professor Dept of Oculoplastics and Orbital Services University of British Columbia, Vancouver, Canada Raghavan Sampath FRCS, FRC Ophth Consultant Lid, Lacrimal and Orbit Surgeon Dept of Ophthalmology Leicester Royal Infirmary Leicester LEI 5 WW, UK Ram Vaidhyanath FRCR Consultant Radiologist Dept of Radiology Leicester Royal Infirmary Leicester LEI 5 WW, UK Raman Mittal DNB Consultant Dept of Ophthalmic Plastic Surgery Orbital Diseases and Ocular Oncology MGM Eye Institute, Raipur, India

Ramesh Murthy MD, FRCS Senior Consultant Oculoplasty and Ocular Oncology Service and Pediatric Ophthalmology and Strabismus Service LV Prasad Eye Institute Hyderabad, India Ratnakar KS MD Head Dept of Pathology Global Hospital Hyderabad, India Ravindra Mohan E MD, FRCS (Edin) Director Dept of Oculoplastics and Orbital Services Shankara Netralaya, Chennai, India Richard K Dortzbach MD, FACS Professor Emeritus Oculoplastic Surgery Service Department of Ophthalmology and Visual Sciences University of Wisconsin 600 Highland Avenue Madison, WI 53792, USA Santosh G Honavar MD, FRCS Director Dept of Oculoplastics Orbital Services and Ocular Oncology LV Prasad Eye Institute Hyderabad, India Subrahmanyam Mallajosyula MS, DO Head, Dept of Ophthalmology Bhaskar Medical College Former Superintendent and Chief Dept of Oculoplastics and Orbital Services Sarojini Devi Eye Hospital Hyderabad, India Venkatesh C Prabhakaran MD Clinical Lecturer Oculoplastic and Orbital Division South Australian Institute of Ophthalmology and Department of Pathology University of Adelaide Australia Vijay Anand P Reddy MD Chief, Dept of Radiology and Radiotherapy Apollo Hospital Hyderabad, India

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Foreword I became acquainted with Subrahmanyam Mallajosyula (Subbu) when he spent a period of time during 1998 training with me as a fellow in orbit and oculoplastics at the University of British Columbia in Vancouver, Canada. At that time, he had been trained in India by some of the top surgeons and had furthered his practical knowledge by visits and fellowships throughout the world. On a personal level, Subbu is an energetic, kind and competent man driven by a strong desire to teach and to bring contemporary care to those in great need. He has become a considerable force in aiding his colleagues in India with regard to oculoplastics and orbit. He is supported by his wife, Kalyani and family, all of whom are wedded to a deep caring for humanity. In the last three decades, advances in imaging, pathology, genetics, immunology and endocrinology, clinical evaluations have led to a consolidation of knowledge concerning diseases of the orbit. Coupled with surgical innovations, these advances have led to a better understanding of the management of disease affecting the orbit. There is, however, a need to bring together and simplify this knowledge in order to provide practical and obtainable care in the developing order. Subbu has gathered a group of distinguished and well-known orbital specialists as well as colleagues from India with vast, practical experience to accomplish this goal. His hope is to target readers who are graduate students, residents of ophthalmology, fellows in oculoplastic and orbital services, and general ophthalmologists who encounter an oculoplastic problem. This is an important and unique endeavour grounded in Subrahmanyam's long-time practice in public service in Hyderabad, where he has encountered the full range of orbital problems and challenges. He has brought to bear his skills, nurtured first in India and then through a range of travel and fellowships at some of the best orbital centers in the world. It is from these centers and from his wide collegial network that he has been able to produce this practical compendium of orbital knowledge. I believe his contribution will not only further the orbital and oculoplastic services in India, Asia and Africa, but also it will bring an awareness of the vast experience in those areas to the rest of the world. The book is meant to provide precise and succinct information on orbital disease, its evaluation and management. The emphasis is of course on common disorders but also presents information on uncommon conditions backed up by case illustrations. As a mentor, colleague and author, I feel privileged to be a part of this enterprise.

Jack Rootman

MD, FRCSC

Professor Department of Ophthalmology and Visual Sciences Department of Pathology and Laboratory Medicine University of British Columbia Vancouver, British Columbia, Canada

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Preface When I was a student, I often heard from my teachers saying "Proptosis is a Pandora's box". Surprisingly I continue to hear it even today! Many a time I am asked to speak on the topic titled "Proptosis is a Pandora's box", and I always change it to "Is Proptosis still a Pandora's box?" It was so, in the past, when the only imaging available was orbital venography. The information of the orbital disease process obtained with it was very meager. I salute my professor Dr. Vengala Rao, who used to perform orbitotomies in those days. It is the challenges he used to encounter, that stimulated me to take up this branch. Fortunately, advances in imaging techniques have made an immense contribution in the assessment of a case of proptosis, so that today, we know what we are dealing with. With careful clinical assessment, and knowledge in reading of CT/MRI, we can even arrive at the histopathological diagnosis of majority of cases. Hence, surprises are very few and far in between. Similarly, advances in histopathology and immunohistochemistry, anesthesia, chemo and radio therapy have made immense contributions in understanding and management of proptosis. The best example is Rhabdomyosarcoma. Today, nearly half the cases of proptosis can be managed by nonsurgical methods or with very minor surgical procedures. I thankfully acknowledge the roles of Dr Vengala Rao, my first teacher, Jack Rootman, Peter J Dolman, Brad Lemke and Mark J Lucarelli in furthering my understanding of orbital diseases. The specialty of orbital diseases is very well advanced in North America and Europe, but not so in most other countries. Availability of ophthalmic literatures authored by orbital surgeons from the developing countries are very few. The idea of bringing this color atlas is to share two decades of my experience in orbital diseases. Though it is an atlas in principle, enough information is provided to understand the conditions and plan treatment strategies. It covers most of the common and some of the rare causes of proptosis. With color illustrations, and case presentations, I tried to make this book interesting to read and also to provide practical knowledge in clinical situations. I thank all my contributing authors, who are internationally reputed, for their co-operation. The chapters they contributed were those in which they have a wealth of experience, viz. Jack Rootman on mesenchymal tumors, Peter J Dolman on thyroid associated orbitopathy (We rarely see such severe TAO in India ), Mark J Lucarelli on anatomy and fractures of orbit. I hope my efforts help shatter the myth that "proptosis is a Pandora's box". If this book inspires at least some ophthalmologists to pursue this specialty with more interest and vigor and blossom into efficient orbital surgeons, the purpose of this book is served. I truly believe that "What I do, you can also do" and who knows you may do even better.

Subrahmanyam Mallajosyula 15-05-2008 Hyderabad

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Acknowledgements At the outset, I wish to acknowledge the significant roles played initially by my parents and then by my wife Rama, in my professional pursuits, which took a lot of time from my family and children Harsha and Aahlad. I greatly appreciate their cooperation. My teacher Dr. Kotagiri Vengala Rao was the first to introduce me to orbital surgery during my postgraduation. I still relish those memories. I thank Dr. Jeffrey Nerad for introducing me to Dr. Jack Rootman. I acknowledge the role of Dr. Jack Rootman, Peter J Dolman in fine tuning my skills—both clinical and surgical. They are not only great teachers, but also wonderful human beings. My fellowship with them was made possible due to the financial assistance I received from them. I also thank the Orbis Inc. for awarding me the Ziegler's International Fellowship, which has part financed my fellowship at University of British Columbia, Vancouver. Similarly I thank the Association of Asian Indians in Ophthalmology for awarding the competitive fellowship, which financed my training with Mark J Lucarelli, Brad Lemke and Richard K Dortzbach at the University of Wisconsin, Madison. It was a great learning experience. I thank all my contributing authors (and their supporting staff), who are all very eminent and highly reputed, for sparing their time to make this book wonderful. I thank my fellow Dr. Mohd Javed Ali, for all his assistance in proofreading. He is ever ready to help. I wish to acknowledge the support and encouragement I received from Shri Jitendar P Vij, Chairman and Managing Director, Mr Tarun Duneja, Director (Publishing) and Mr PS Ghuman, Sr Production Manager of M/s Jaypee Brothers Medical Publishers (P) Ltd. I also thank Mr Upinder, Mr Pankaj, Mr Ram Murti and Mrs Seema Dogra of the same family (Jaypee) for their technical support.

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Contents Part One : Basic Concepts 1.

Applied Anatomy of Orbit ................................................................................................................. 03 Mark J Lucarelli, Nancy Kim Orbital osteology 3; The periorbita 6; The orbital apex 6; The cavernous sinus 8; The globe 8; The extraocular muscles 9; Lids 10; The lacrimal system 13; The nerves of the orbit 14; Vascular anatomy of the orbit: Arterial supply 17; Vascular anatomy of the orbit: Venous outflow 19; Paranasal sinuses 20; Conclusion 20

2.

Clinical Approach to Proptosis ......................................................................................................... 23 Subrahmanyam Mallajosyula Pain 23; Progression 25; Proptosis 28; Axial proptosis 29; Measurement of proptosis 35; Pulsations 37; Pupil 41; Perception of color vision 41; Prism bar-cover test (PBCT) 42; Periorbital changes 45; Lid changes 46; Conjunctival changes 48; Palpation 51; Auscultation 51; Evaluation of a case of proptosis 53

3.

Imaging a Case of Proptosis: CT and MRI .................................................................................... 56 Subrahmanyam Mallajosyula, Ravi Varma Evaluation of a CT scan of orbit 58; Common mistakes 59; Bony orbit 60; Eyeball 65; Enlarged extraocular muscle 69; Soft-tissue lesions 72; Lacrimal gland tumors 76; Cystic lesions of the orbit 78; Metastatic lesions 81; Contrast enhancement 83; 3-D reconstruction of the orbit 84

4.

Role of Cytology in Orbital Lesions ................................................................................................ 85 Geeta K Vemuganti, Anirban Bhaduri Fine needle aspiration/sampling techniques 85; Intraoperative-operative diagnosis by squash and imprint cytology 85; Squash/imprint cytology 85; Case illustrations 86

5.

Pathology of the Orbital Diseases ................................................................................................... 97 KS Ratnakar Classification 97; Diagnosis of orbital tumors 98; Developmental lesions 98; Inflammatory lesions 100; Orbital infections 101; Cysticercosis 102; Neoplastic lesions 102; Benign tumors 103; Malignant tumors 104; Metastasis 105; Grave's disease 106; Mucocele 106

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xvi Surgical Atlas of Orbital Diseases

Part Two : Disease Patterns of Proptosis 6.

Thyroid-Associated Orbitopathy .................................................................................................... 111 Peter J Dolman Incidence and epidemiology 112; Risk factors and predictive variables 112; Pathogenesis 112; Course of disease 114; Clinical classification 114; The VISA classification 114; Vision/optic neuropathy 114; Inflammation/congestion 116; Strabismus/motility restriction 117; Appearance/exposure 117; General management guidelines 118

7.

Orbital Infections ............................................................................................................................... 120 Shome debraj, Walinjkar Jaydeep, Mukherjee Angshuman Risk factors 121; Etiological causes of orbital infections 121; Bacterial infections 121; Fungal infections 121; Parasitic infections 121; Protozoal infections 121; Diagnosis 121; Imaging studies 121; Emergency department care 122; Further inpatient care 122; Case illustrations 123

8.

Orbital Inflammatory Disease ........................................................................................................ 128 E Ravindra Mohan, Moupia Goswami, Vinathi Mutyala Orbital amyloidosis 129; Sarcoidosis 130; Nonspecific orbital inflammatory syndrome (NSOIS) 131; Kimura’s disease 132; Wegener’s granulomatosis 132; Langerhan’s histiocytosis 133; Rosai-Dorfman disease 133; Orbital xanthogranuloma 134; Case illustrations 134

9.

Orbital Lymphoma ............................................................................................................................. 146 Christopher Knapp, Ram Vaidhyanath, Laurence Brown, Raghavan Sampath REAL classification 146; WHO classification of NHL 146; Modified Rye’s classification of Hodgkin’s lymphoma 147; When to suspect lymphoma 147; When to suspect idiopathic orbital inflammatory disease 148; Case illustrations 148

10.

Vascular Lesions of Orbit ................................................................................................................ 151 Subrahmanyam Mallajosyula, Mohd Javed Ali Malformations 151; Lymphangioma 151; Orbital varices 152; Cavernous hemangioma 152; Other congenital malformations 152; Sturge-Weber syndrome 152; Wyburn-Mason syndrome 153; KlippelTrenaunay syndrome 153; Shunts 153; Carotid-Cavernous fistula 153; New growths 154; Capillary hemangioma 154; Hemangiopericytoma 155; Angiosarcoma 155; Kaposi’s sarcoma 155; Hemangioendothelioma 155; Hemangioblastoma 155; Case illustrations 157

11.

Orbital Tumors of Neurological Origin ........................................................................................ 162 Christopher M Knapp, Ram Vaidhyanath, Laurence Brown, Raghavan Sampath Optic nerve glioma 162; Optic nerve meningioma 163; Orbital schwannoma (neurilemmoma) and neurofibroma 165; Case illustrations 166

12.

Mesenchymal Tumors ....................................................................................................................... 170 E Weis, J Rootman Mesenchymal soft tissue tumors 170; Rhabdomyosarcoma 170; Rhabdomyoma 172; Leiomyoma 172; Leiomyosarcomas 172; Adipose tumors 172; Liposarcoma 174; Fibrous tissue tumors 174; Histiocytic tumors 175; Fibrous histiocytoma 175; Malignant tumors of uncertain type 175; Rhabdoid tumor 175

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Contents xvii 13.

Bone Tumors of Orbit ....................................................................................................................... 180 Venkatesh C Prabhakaran, Dinesh Selva Clinical presentation 180; Osteoma 180; Fibrous dysplasia 181; Ossifying fibroma 182; Osteoblastoma 183; Chondroma 183; Cholesterol granuloma 184; Aneurysmal bone cyst 184; Giant cell lesions 184; Osteogenic sarcoma 184; Chondrosarcoma 186; Mesenchymal chondrosarcoma 186; Ewing’s sarcoma 186; Langerhan’s cell histiocytosis (LCH) 186; Intraosseous hemangioma 186

14.

Tumors of Lacrimal Gland ............................................................................................................... 190 Raman Mittal Classification 190; Epithelial cyst (Dacryops) 190; Case illustrations 191; Pleomorphic adenoma 191; Adenoid cystic carcinoma 194

15.

Cystic lesions of Orbit ...................................................................................................................... 199 Golam Haider, Subrahmanyam Mallajosyula, Mohd Javed Ali Classification 199; Dermoid and epidermoid cysts 200; Teratomas 201; Cephalocele 201; Microphthalmos with cyst 202; Mucocele 202; Cysts of the optic nerve sheath 203; Hematic cyst 203; Simple cyst 204; Retention cyst 204; Lacrimal ductal cyst 204; Implantation cyst 205; Dacryocele 205

16.

Parasitic Cysts of Orbit ..................................................................................................................... 207 Subrahmanyam Mallajosyula, Mohd Ather, Modini Pandarpurkar Cysticercosis 207; Case illustrations 208; Hydatid cyst of orbit 217

17.

Orbital Fractures ................................................................................................................................ 220 Alon Kahana, Mark J Lucarelli, Cat N Burkat, Richard K Dortzbach Introduction 220; Anatomy 220; Imaging 226; Implant materials 227; General operative considerations 229; Pediatric patients 231; Timing of surgery 231; Decision: repair or not repair 232; Floor fractures 233; Medial wall fractures 236; Lateral wall and zygomatico maxillary fractures 238; Late and secondary fracture repair 238

18.

Secondary and Metastatic Orbital Tumors ................................................................................. 244 Kasturi Bhattacharjee, Harsha Bhattacharjee, Ganesh Kuri, Shyamanga Borooah Orbital extension of intraocular tumors 244; Orbital extension of retinoblastoma 244; Orbital extension of medulloepithelioma 246; Orbital extension of uveal melanoma 247; Orbital extension of lacrimal sac tumors 250; Orbital extension of eyelid tumors 252; Basal cell carcinoma (BCC) 252; Sebaceous carcinoma of the eyelid 253; Squamous cell carcinoma of the eyelid 255; Malignant melanoma of eyelid 256; Orbital extension of intracranial tumors 257; Orbital extension of conjunctival tumors 258; Squamous cell carcinoma of the conjunctiva 258; Malignant Melanoma of the conjunctiva 259; Orbital extension of tumors of the nasal cavity and paranasal sinus 260; Orbital extension of nasopharyngeal tumor 262; Metastatic orbital tumors 263

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xviii Surgical Atlas of Orbital Diseases

Part Three : Management Strategies: Surgical 19.

Decision Making ................................................................................................................................ 271 Subrahmanyam Mallajosyula Intraconal lesion 273; Reese-Berke’s incision 273; Steps of Reese-Berke approach 274; Steps of superior lidcrease incision 275; Apical conal lesions 279; Lesions of superior peripheral space 279; Thyroid associated orbitopathy 285

20.

Orbitotomies ........................................................................................................................................ 288 Ramesh Murthy, Anirban Bhaduri, Vikas Menon, Santosh G Honavar General principles 288; Approaches 289; Anterior orbitotomy 289; Swinging lower eyelid flap 289; Lateral orbitotomy 290; Stallard-Wright lateral orbitotomy 290; Transfrontal orbitotomy 291; Complications 291; Postoperative management 291; Case illustrations 291

21.

Multidisciplinary Approach to Proptosis .................................................................................... 299 Subrahmanyam Mallajosyula, B Ranganadha Reddy, M Chandrasekhar Reddy Surgical anatomy 299; ENT approach to proptosis 300; Various etiological factors of proptosis in ENT 300; Sinus diseases causing proptosis 301; Purulent infections 301; Extensive nasal polyposis 301; Mucormycosis 301; Allergic fungal sinusitis 301; Fronto- ethmoidal mucocele 302; Tumors of paranasal sinuses causing proptosis 302; Fibrous dysplasia 302; Hemangiopericytoma 302; Juvenile nasopharyngeal angiofibroma 303; Squamous cell carcinoma 303; Rhabdomyosarcoma 303; Non-Hodgkin’s lymphoma 303; Esthesio-neuroblastoma 304; Caldwell-Luc operation 304; Lateral rhinotomy/medial maxillectomy 305; Total maxillectomy 306; Patterson’s operation 305; FESS 309; Neurosurgical approaches of proptosis 309; Transcranial approach 311; Extracranial approach 309; Case illustrations 313

22.

Orbital Exenteration .......................................................................................................................... 318 Ramesh Murthy, Anirban Bhaduri, Sima Das, Santosh G Honavar Indications 318; Patient preparation 318; Surgical procedure 318; Types 319; Management of the exenterated socket 320; Prosthesis 320; Complications of exenteration 320; Case illustrations 321

23.

Orbital Prosthesis............................................................................................................................... 327 Kuldeep Raizada Types of prosthesis 327; Complete prosthesis 327; Factors that affect the fit of an orbital prosthesis 328; Preparation of the patient 328; Impression of the orbital defect 328; Casting 329; Sculpting 329; Moulding 330; Using the desired material 331; Fabrications of ocular prosthesis 331; Assemble of prosthesis 331; Care of your prosthesis 331; Storing the prosthesis 332; Preventing mishaps 333

Part Four : Management Strategies: Nonsurgical 24.

Medical Management of Proptosis ................................................................................................ 337 Subrahmanyam Mallajosyula, Mohd Javed Ali Nonspecific inflammations of the orbit (NSOIS) 337; Specific inflammations of the orbit 338; Orbital cellulitis 338; Rhino-orbital mucormycosis 338; Chronic granulomatous infections 338; Parasitic infestations 338; Tolosa- Hunt syndrome 339; Capillary hemangioma 339; Acute intraorbital hemorrhage and emphysema 340; lymphoprolifarative and other neoplastic lesions 340; Case illustrations 340

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Contents xix 25.

Management of Ophthalmic Tumors: Role of Chemotherapy and Radiation Therapy .. 344 Vijay Anand P Reddy, Nitin More, Ramesh Murthy, Anirban Bhaduri, Santosh G Honavar Introduction 344; Ionizing radiation 344; Radiation therapy delivery methods 344; External beam radiation (teletherapy) 344; Internal radiation therapy (brachy therapy) 345; Plaque radiotherapy 346; Principles of anti-neoplastic therapy 347; Capillary hemangioma 348; Basal cell and squamous cell carcinoma 348; Tumors of lacrimal gland 348; Malignant conjunctival tumors 349; Intraocular lymphoma 349; Retinoblastoma 349; Choroidal melanomas 349; Chemoreduction regimen 350; Oculor metastasis 352; Rhabdomyosarcoma 352; Orbital lymphoma 352; Grave’s ophthalmopathy 353; Optic nerve glioma 353; Optic nerve meningioma 353; Sequelae of radiation therapy 353

26.

Carotid-Cavernous Fistulae: Role of Interventional Radiologist .......................................... 356 D Ravi Varma, D Radhika Varma Pathophysiology 357; Clinical features 357; When to suspect CCF 359; Radiological investigations 359; Management of CCF 360; Direct CCF 360; Indirect CCF 363; Prognosis 363

27.

Ocular and Systemic Associations of Proptosis ......................................................................... 366 Subrahmanyam Mallajosyula, Mohd Javed Ali Capillary hemangiomas 366; Neurofibromatosis 366; Craniofacial dysostosis 367; Encephalocele 367; Wegener’s granulomatosis 367; Wyburn-Mason syndrome 367; Hurler’s syndrome 368; Nonspecific orbital inflammation syndrome 368; Sclerosing inflammation of the orbit 368; Osteoma 368; Orbital hamartoma (tuberous sclerosis) 368; Hemangioblastom 368

Index ..................................................................................................................................................... 369

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1

Applied Anatomy of Orbit

CHAPTER Mark J Lucarelli, Nancy Kim

Fundamental to the understanding of orbital pathology and its surgical management is a sound working knowledge of the anatomy of the normal orbit in three dimensions. The goal of this chapter is to review the location of critical ocular adnexal, orbital and related craniofacial structures and the anatomic relationships between them.

OVERVIEW The orbit is defined as the bony cavity containing the globe, extraocular muscles, fat, nerves and blood vessels. Although the orbit is often described as pyramidal in shape, the space is actually pear-shaped, with its largest horizontal and vertical diameters lying 1 cm past the orbital rim and adjacent to the equator of the globe. Average orbital volume is approximately 25-30 cc, of which the globe occupies approximately 7 cc of space. The lateral walls are oriented about 90° to one another and run 40 to 45 mm in length to the apex. The medial walls of each orbit run parallel to each other and measure 45 to 50 mm in length. The optical axes themselves are also parallel to the course of the medial walls, rather than the diverging central axes of the orbits. Therefore, each globe is tonically held in adduction by the extraocular muscles to maintain ocular alignment. These relationships and other important dimensions of the orbit are illustrated in Figure 1.1.

which make up the four orbital walls: the frontal, sphenoid (greater and lesser wings), ethmoid, lacrimal, maxillary, palatine, and zygomatic bones (Figure 1.2). The roof of the orbit is comprised of the frontal bone anteriorly, and the lesser wing of the sphenoid bone posteriorly (Figure 1.3). The overall thickness of the roof is significantly greater than that of either the medial wall or orbital floor and is therefore, relatively resistant to fracture. Within the lesser wing lies the optic foramen, through which the optic nerve exits the orbit via the optic canal. In about 30% of individuals, just above the frontosphenoid suture, lies the meningeal foramen through which the recurrent meningeal artery (a branch of the external carotid system) passes to anastomose with the lacrimal artery (a branch of the internal carotid system). This communication provides an important potential source of collateral blood flow to the orbit

Orbital Osteology The orbital rim is roughly in the shape of a spiral, with its starting and end points at the anterior and posterior lacrimal crests.1 There are seven bones

Figure 1.1: An axial view of the orbits demonstrating the dimensions and relationships between associated structures

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4 Surgical Atlas of Orbital Diseases

Figure 1.2: An anterior-posterior view into the left bony orbit

Figure 1.3: The left orbital roof

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Applied Anatomy of Orbit 5 should its primary supply via the internal carotid system become disrupted. When the meningeal foramen is absent, the middle meningeal artery courses directly via the superior orbital fissure.2 Other important bony landmarks include the lacrimal gland fossa in the temporal roof and the trochlear fossa anteromedially. Just superolateral to the trochlear fossa and at the medial one-third junction of the superior rim, lies the supraorbital notch which gives passage to supraorbital artery, vein, and nerve. In some individuals, this point of egress is completely enclosed and appears as the supraorbital foramen.3 The medial wall includes the ethmoid, maxillary, and lacrimal bones, as well as the lesser wing of the sphenoid (Figure 1.4). Within the bony suture line separating the frontal from the ethmoid bone, there are two important apertures, the anterior and posterior ethmoidal foramina. These foramina are the exit points for the anterior and posterior ethmoidal arteries and nerves, respectively. The anterior ethmoidal foramen is typically located approximately 24 mm posterior to the orbital rim and the posterior ethmoidal foramen lies approximately 36 mm posterior to the rim. The optic foramen, in turn, is located approximately 6 mm posterior to the posterior ethmoidal foramen. These foramina help the surgeon delineate the frontoethmoidal suture which is an important surgical landmark for the roof of the ethmoid sinus, or fovea ethmoidalis. The orbital roof slopes downward as it travels medially. Medial to the orbital space, just beyond the frontoethmoidal suture line, the fovea ethmoidalis continues in a downward plane and ends

Figure 1.4: Medial wall of the right orbit

sagittally just above the nasal cavity and below the anterior cranial fossa at the cribriform plate. Bony dissection of the medial wall above the suture line exposes the dura of the frontal lobe. The ethmoid portion of the medial wall, the lamina papyracea, is extremely thin and is thus prone to fracture with trauma and to easily transmit infection from the ethmoid air cells into the orbit as subperiosteal abscesses. The medial wall thickens again in the area of the inferior suture between the ethmoid and maxillary bones. This maxilloethmoid strut4 provides support to the inferomedial orbital wall and often survives trauma which fractures the more superior aspects of the wall. At the anterior aspect of the medial wall is the lacrimal sac fossa, bounded by the anterior and posterior lacrimal crests. The anterior and posterior limbs of the medial canthal tendon insert on the anterior and posterior lacrimal crests, respectively. The floor of the orbit consists of the maxillary, zygomatic and palatine bones. The maxillary bone forms the bulk of the floor while the zygomatic bone contributes anterolaterally and the palatine bone contributes to the posterior floor. A major landmark in this area is the infraorbital groove, which originates approximately 25-30 mm posterior to the orbital rim. The groove deepens and becomes an enclosed canal as it travels anteriorly within the floor to open again on the face of the maxillary bone at the infraorbital foramen on the maxillary face, 4-6 mm from the rim in adults.5 This pathway contains the infraorbital neurovascular bundle which is easily injured by floor fractures or inadvertent surgical dissection. Just medial to the infraorbital groove is the thinnest portion of the maxillary bone. Not only does this render the posteromedial part of the floor particularly susceptible to blowout fractures, but it also provides an area where bone can be removed with relative ease for inferior orbital decompression. The thicker, maxilloethmoid strut lies in the medial floor and provides support for the orbital soft tissues and the globe.4, 6 In the anteromedial floor, the bony nasolacrimal duct travels from the base of the lacrimal fossa in an inferior and usually slightly posterolateral direction through the maxillary bone in the lateral nasal wall to empty into the inferior meatus of the nose. The vector of the nasolacrimal duct shows considerable variability.

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6 Surgical Atlas of Orbital Diseases The lateral wall contains the zygomatic bone and the greater wing of the sphenoid which separates the posterolateral orbit from the middle cranial fossa. The posterior borders of the lateral wall are defined by the superior and inferior orbital fissures. The boundary between the lateral wall and roof is formed by the frontosphenoid suture, which transmits the recurrent meningeal artery. The anterior part of the lateral wall is comprised of the zygoma. Important landmarks in this region include the lateral orbital tubercle, or Whitnall’s tubercle, which is the insertion point of the posterior head of the lateral canthal tendon, the lateral horn of the levator aponeurosis, the check ligament of the lateral rectus muscle, and Lockwood’s ligament. The tubercle can be found just inside the orbital rim and approximately 11 mm below the frontozygomatic suture. 7 The superoanterior zygoma also contains the zygomaticotemporal and zygomaticofacial canals through which branches of the lacrimal artery and the lacrimal and zygomatic nerves pass (Figure 1.5).

superior orbital fissure and optic canal to become continuous with the dura. The potential space outside the periorbita is an important surgical plane. Access to the orbital walls in decompression surgery, for example, entails dissection between the bone and this overlying periosteal sheet.

The Orbital Apex

The periorbita refers to the tough, fibromembranous lining of the bony orbit which acts as a physical barrier to infection and provides a scaffold to which other intraorbital connective tissues can attach. The regions of greatest adherence between this sheath and bone are at the orbital rim, suture lines, bony fissures, trochlear fossa and the lacrimal crests. At the orbital margins, at the arcus marginalis, the periorbita thickens and gives rise to the orbital septum. Deep in the orbit, the periorbita continue through the

The orbital apex merits special attention, as the region in which many critical orbital structures converge and communicate with other important, periorbital spaces (Figure 1.6). Cranial nerves II through VI, major orbital vessels, and all of the extraocular muscles excluding the inferior oblique sit in tight proximity within the apex, and pathology in this region can produce profound deficits in vision and ocular motility.8, 9 The apex is defined by only three walls; the floor is absent in the far posterior orbit. 1 Major bony landmarks include the superior orbital fissure, inferior orbital fissure, and the optic canal. The superior orbital fissure divides the sphenoid into the greater (lateral) and lesser (medial) wings and lies inferiorly and laterally to the optic foramen. This fissure measures approximately 20-22 mm in overall length and is separated into superolateral and inferomedial sections by the tendon of the lateral rectus muscle. The superotemporal part of the fissure lies above the annulus of Zinn, the fibrous ring formed by the common origin of the rectus muscles. The lacrimal, frontal and trochlear nerves, and the superior ophthalmic vein pass through this region as they traverse the apex. The inferomedial segment of

Figure 1.5: Lateral wall of the right orbit

Figure 1.6: The left orbital apex

The Periorbita

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Applied Anatomy of Orbit 7

Figure 1.7: The superior and inferior orbital fissures and associated apex structures of the right eye

the superior orbital fissure, also called the oculomotor foramen, is located inside the annulus and transmits the superior and inferior divisions of the oculomotor nerve, the abducens nerve, sympathetic fibers, and the nasociliary nerve, a terminal sensory branch of the ophthalmic division of the trigeminal nerve10-12 (Figure 1.7). The inferior orbital fissure bounds the greater wing of the sphenoid, separating it from the maxillary bone inferomedially. This fissure communicates primarily with the pterygopalatine fossa (Figure 1.8). The maxillary branch of the trigeminal nerve passes through the pterygopalatine fossa and subdivides into the infraorbital nerve which, in turn, travels anteriorly into the orbit via the infraorbital groove. The zygomatic nerve, another branch of the maxillary branch of cranial nerve V, enters the orbit through the inferior orbital fissure to provide sensory innervation to lateral orbit and cheek after passing through the zygomaticofacial foramen. Also within the pterygopalatine fossa is located the maxillary artery which gives rise to the infraorbital artery, part of the neurovascular bundle traveling through the infraorbital groove. Parasympathetic fibers originating from the pterygo-

palatine ganglion and terminating in the lacrimal gland are transmitted by the inferior orbital fissure as well.2 The inferior ophthalmic veins also pass from the orbit into the pterygoid plexus via the inferior orbital fissure. The optic canal penetrates the superomedial orbital apex through the lesser wing of the sphenoid bone as the optic foramen. The canal is approximately 6 mm in diameter and 8-10 mm in length and houses the optic nerve and the ophthalmic artery. The canal runs along the upper, lateral wall of the anterior sphenoid sinus and in the floor of the anterior cranial fossa.

Figure 1.8: Lateral cross-section of the orbit

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8 Surgical Atlas of Orbital Diseases

The Cavernous Sinus The cavernous sinus is a large venous sinus posterior to the orbital apex contained within a dural cleft which is situated lateral to the sphenoid sinus (Figure 1.9). Its tributaries consist of the ophthalmic, cerebral middle meningeal, and pterygoid veins. The left and right cavernous sinuses communicate with one another via small channels which run superior to the roof of the sphenoid sinus. Several critical structures pass through the cavernous sinus as they travel into the orbit. The carotid siphon and the sympathetics which ride along on its sheath traverse centrally. In the lateral wall of the cavernous sinus area embedded the oculomotor nerve, trochlear nerve, the ophthalmic and maxillary divisions of the trigeminal nerve, and the abducens nerve. The optic nerves course superomedially to the cavernous sinus. The optic chiasm is formed just above the anterior aspect of the cavernous sinus. As in the orbital apex, pathological processes involving the cavernous sinus such as the formation of carotid-cavernous fistulas, inflammation or infection typically cause multiple cranial neuropathies affecting the eye.13 Further, because the right and left cavernous sinuses are interconnected, disease processes extending posteriorly from the orbit on one side can spread to the other via these spaces.

The Globe The average-size globe measures approximately 23.5 mm in the horizontal meridian and 23 mm

Figure 1.9: A cross-section of the cavernous sinus

vertically, with an anterior-posterior dimension of about 24 mm. Its overall volume is about 7cc. The globe is surrounded by a loose fascial sheath, or Tenon’s capsule, which is interconnected to the sclera by fine fibrous bands. The potential space between these layers is the episcleral space, and the areas of greatest adherence between them are approximately 1.5 mm from the limbus anteriorly, and posteriorly, at the optic nerve sheath. Tenon’s capsule is suspended inside the orbit by interconnections with fine connective tissue septae within the surrounding orbital fat. This sheath must be traversed by the nerves and blood vessels which supply the globe. Likewise, the extraocular muscles must penetrate Tenon’s layer to attach to the globe. As the muscles pass from outside to inside the episcleral space to fuse with the sclera, Tenon’s capsule makes attachments with the intermuscular septum, a fibrous network which encases and interconnects the extraocular muscles (Figure 1.10).14-18 Thus, following enucleation, orbital implants that are placed within Tenon’s capsule may demonstrate a fair amount of motility even if they are not sutured to the extraocular muscles themselves.18 The intermuscular septum and rectus muscles delineate the intraconal versus extraconal space. Interconnections between the extraocular muscle sheaths and the periorbita comprise the check ligaments. Superiorly, the check ligaments consist of the fascial complex surrounding the upper lid retractors, the superior rectus and levator palpebrae muscles. 19 Similarly, the inferior check ligament consists of the muscle sheaths surrounding the lower lid retractors, the inferior rectus and inferior oblique.

Figure 1.10: The extraocular muscles and intermuscular fascia

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Applied Anatomy of Orbit 9 Medially, the muscle sheath of the medial rectus inserts just inside the posterior lacrimal crest, to the medial orbital septum and caruncle to collectively form the medial check ligament. The analogous check ligament of the lateral rectus inserts onto the lateral orbital tubercle of Whitnall.7

The Extraocular Muscles The four rectus muscles originate at the annulus of Zinn, within the orbital apex. Specifically, the superior and medial recti originate adjacent to the lesser wing of the sphenoid, next to the optic canal. The inferior rectus originates from a portion of the annulus which extends from the body of the sphenoid bone to its great wing. The lateral rectus has a bifid origin from a tendinous segment of the annulus which extends across the superior orbital fissure from the greater to the lesser sphenoid wing and a more inferior portion which extends directly from the greater wing itself. The superior rectus lies just underneath the levator palpebrae. Immediately beneath and medial to the superior rectus run the nasociliary nerve and ophthalmic artery. The superior edge of the medial rectus travels just under these structures. From its origin at the annulus, the inferior rectus closely follows the floor of the orbit until reaching the anterior orbit, where it becomes separated from the floor by the inferior oblique muscle as the latter crosses from the medial to the lateral wall, and by fat. Medial and superior to the lateral rectus, is found the ciliary ganglion which is usually adherent to the intraorbital segment of the optic nerve. The superior oblique muscle also begins along the annulus of Zinn, superomedially and extends forward superiorly and along the junction between the orbital roof and the medial orbital wall. As it courses toward the anterior orbit, the muscle belly transitions to a tendinous segment as it reaches the trochlea, a pulley-like cartilaginous structure which lies approximately 6-10 mm posterior to the superomedial orbital rim.20, 21 From this point, the tendon passes posterolaterally, making a 54° angle, to attach to the globe. It is this course which gives rise to the main actions of the superior oblique, namely intortion and depression of the globe with contraction. During orbital surgery, caution to avoid injuring the trochlea must be taken to avoid

subsequent scarring and restriction of superior oblique muscle action, or Brown’s syndrome.22 The inferior oblique, unlike the other extraocular muscles, does not originate at the annulus, but from the periosteum of the anterior, inferomedial orbit on the maxillary bone. It courses posterolaterally, just beneath the inferior rectus to insert on the inferolateral globe, which gives rise to its main actions: extortion and elevation of the globe. The capsulopalpebral fascia and Lockwood’s ligament interdigitate with the muscular fascia of the inferior oblique. The extraocular muscles approximate a spiral in the distance between their individual insertions and the corneal limbus, the so-called spiral of Tillaux (Figure 1.11). Beginning with the medial rectus, each successive muscle inserts farther from the limbus. Although there is individual variation, the average distances are: medial rectus, 5.5 mm; inferior rectus, 6.5 mm, lateral rectus, 6.9 mm, and superior rectus, 7.7 mm.23 Innervation to the extraocular muscles is largely carried by the oculomotor nerve (third cranial nerve), which supplies the medial, inferior, and superior rectus muscles, the inferior oblique, as well as the levator palpebrae superioris. The superior oblique and lateral rectus receive innervation from the

Figure 1.11: Spiral of Tillaux

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10 Surgical Atlas of Orbital Diseases trochlear nerve (fourth cranial nerve) and the abducens nerve (sixth cranial nerve), respectively. The blood supply to the muscles is carried by muscular branches of the ophthalmic artery.

Lids Understanding the general anatomy and dimensions of the major eyelid landmarks is important in the evaluation of structural disease of the lids and in planning surgical repair. The normal interpalpebral fissure height is 10-12 mm and the average length is 28-30 mm. The distance between the upper lid crease and lid margin measures approximately 8-11 mm at the pupillary axis. The highest point of the upper lid contour rests just nasal to the center of the pupil and the upper lid margin is typically located 1-2 mm below the superior limbus in adults. The lateral canthal angle sits approximately 2 mm higher than the medial canthus. Both the upper and lower eyelids can be divided into the skin, orbicularis, orbital septum, orbital fat, lid retractors, tarsus, and conjunctiva. For descriptive purposes, the layers can be grouped into the anterior

Figure 1.12: A cross-section of the upper and lower lids

and posterior lamellae. The anterior lamella contains lid structures which lie outside the orbit per se, and is comprised of the skin and the orbicularis oculi (Figure 1.12). The skin of the lids is the thinnest of the body and unlike skin elsewhere, has no subcutaneous fat layer. The portion which lies anterior to tarsus is relatively firmly attached to deeper structures while the preseptal portions are loosely connected. Anterior to the margin, the skin contains the lash follicles with their associated sebaceous glands of Zeis and apocrine glands of Moll, as well diffusely distributed eccrine sweat glands. The orbicularis muscle is a C-shaped complex of muscle fibers which functions to close the lids (Figure 1.13). It is divided into pretarsal, preseptal and orbital sections, all of which receive innervation from the facial nerve (seventh cranial nerve). The pretarsal and preseptal parts of orbicularis are primarily involved in involuntary closure of the lids, as elicited by the blink reflex. These fibers insert at the medial canthal tendon as deep and superficial heads. A subset of pretarsal orbicularis fibers, also called Horner’s muscle, inserts deep at the posterior lacrimal crest as the deep limb of the medial canthal

Figure 1.13: The orbicularis oculi

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Applied Anatomy of Orbit 11 tendon, along the posterior aspect of the lacrimal sac and surround the canaliculi. These fibers are thought to provide a pumping action as they contract which facilitates tear drainage.24, 25 Horner’s muscle is also critical in maintaining close contact between the posterior aspect of the lid and the globe. The remaining pretarsal orbicularis inserts superficially within the anterior limb of the medial canthal tendon. Laterally, slips from the upper and lower lid pretarsal orbicularis insert onto the lateral canthal tendon which in turn, inserts on the lateral orbital tubercle. Medially, the deep head of the preseptal orbicularis inserts into the fascia surrounding the lacrimal sac while the superficial head inserts onto the anterior limb of the medial canthal tendon. The orbital orbicularis is chiefly responsible for forced lid closure, such as winking or in blepharospasm. Medially, its insertions lie along the orbital rim and anterior medial canthal tendon. As they course laterally, these fibers overlie the zygoma and the elevators of the lateral mouth, the zygomaticus major and minor. Underlying the pretarsal orbicularis and resting anterior to the tarsus is the muscle of Riolan which consists of small, horizontally oriented slips of muscle. These fibers appear grossly as the “gray line” of the lid margin which is posterior to the lash line and function to turn the lashes toward the eye during blinking. The “gray line” is a useful landmark in aligning the wound edges in marginal lid laceration repair. The septum, orbital fat and posterior lamella, which consists of the retractors, tarsus and conjunctiva, are considered to be intraorbital structures. The septum is comprised of tough fibrous connective tissue arranged in sheets which originate from the periosteum of the orbital rims at the arcus marginalis. This structure acts as an relative barrier between the orbit and lid in limiting the deep spread of superficial hemorrhage and infection. In the upper lid, the septum fuses with the aponeurosis of the levator muscle, the primary upper lid retractor muscle, approximately 2-5 mm above the superior tarsal edge. 26 In the lower lid, the septum condenses with the capsulopalpebral fascia as the two layers converge toward the inferior edge of the lower tarsal plate.27

The orbital septum lies anterior to the preaponeurotic orbital fat pads, which prolapse forward with any violation of the septum. As a natural consequence of aging, as the septum thins and stretches, these fat pads tend to gradually herniate anteriorly. In the upper lid, there are two distinct fat pads, the medial and central fat pads. The medial pad can be distinguished by its relatively white color. The medial palpebral artery typically runs within this pocket and care should be taken to avoid inadvertent laceration or cauterization during surgery. Laterally, there is usually little fat in the upper lid. Instead, this space is usually filled by orbital lobe of the lacrimal gland. The lower lids contain medial, central and lateral fat pads. The medial pad lies just medial to the inferior oblique and as in the upper lid, has a characteristically white color and contains the lower palpebral artery. The central fat pad lies between the inferior oblique muscle and a fascial band that separates it from the lateral fat pad. The latter extends to the inferior edge of the lacrimal gland.28 The upper lid retractors consist of the levator muscle which is innervated by cranial nerve III, and the sympathetically innervated Müller’s muscle. The levator, which is the primary retractor, originates from a point just above the annulus of Zinn, from the lesser wing of the sphenoid bone. The levator complex includes a muscular component which is approximately 40 mm long extending from its origin on the lesser sphenoid wing just outside the annulus of Zinn, and the fibrous levator aponeurosis which is 14-20 mm in length. As the muscular portion courses forward in the orbit, it rides just above the superior rectus muscle and the two are interconnected by interdigitated fibrous bands. As they reach the equator of the globe, the levator broadens and transitions into its aponeurotic component. Medially and centrally, the aponeurosis inserts onto the anterior tarsal surface and passes through the orbicularis to insert onto pretarsal skin. These insertions create the upper lid crease. Medially, the aponeurosis separates into a single medial horn which inserts into the posterior lacrimal crest and becomes continuous with the medial canthal tendon complex. Similarly, the aponeurosis courses into a lateral horn which inserts into the lateral orbital tubercle, also

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12 Surgical Atlas of Orbital Diseases called Whitnall’s tubercle, and becomes continuous with the lateral canthal tendon complex (Figure 1.14). The normal magnitude of upper lid elevation is approximately 14-16 mm. Elevation of less than 10-12 mm is usually abnormal. Elevation of less than 5 mm is considered severe dysfunction and has important implications in ptosis surgery. Because of the close apposition and fibrous interconnections between the levator and the superior rectus muscle, when the globe is elevated, the upper lid follows. This relationship is not passive, and the levator and superior rectus actually co-contract. Likewise, when the globe is depressed, both muscles relax together and the upper lid moves downward.29 At the transitional zone between the anterior muscular component of the levator and its aponeurosis is a fascial sleeve called Whitnall’s ligament, or the superior transverse ligament. This band runs both over and beneath the levator at this point and behaves as a fulcrum point for the levator where contraction of the muscular portion in the horizontal plane becomes directed in the vertical direction.30-32 However, Whitnall’s ligament is not a stationary fulcrum,29 rather, it acts more as a swinging suspender of the levator.31 Whitnall’s ligament also provides mechanical support for the superior orbital soft tissues. Medially, this structure inserts within the fascial tissue surrounding the superior oblique tendon and the trochlea. Laterally, the ligament inserts within the inner surface of the lateral wall into the periorbita of the lacrimal gland fossa, approximately 10 mm above the lateral orbital

Figure 1.14: The levator superioris complex, including Whitnall's ligament

tubercle, or Whitnall’s tubercle. (Note that despite the shared eponym, Whitnall’s ligament does not directly insert into Whitnall’s tubercle). Prior to its lateral insertion, the ligament courses across and divides the lacrimal gland into a superior orbital lobe and an inferior palpebral lobe.30 (Figure 1.15). Müller’s muscle is a secondary upper lid retractor, providing approximately 1-2 mm of elevation. It originates just deep to the levator aponeurosis at the level of Whitnall’s ligament and is about 12-14 mm in length. Muller’s muscle inserts at the superior edge of the tarsal plate. An important landmark for

Figure 1.15: The lacrimal gland and its relation to the levator superioris complex

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Applied Anatomy of Orbit 13 this structure is the peripheral vascular arcade which lies between the levator aponeurosis and Müller’s muscle just above the tarsus. Injury to Müller’s or loss of sympathetic innervation, as occurs in Horner’s syndrome, causes a characteristic mild (1-2 mm) ptosis.33 In the lower lid, the retractor complex is called the capsulopalpebral fascia. This structure is a condensation of fibrous attachments to terminal muscle slips from the inferior rectus which course anteriorly to surround the inferior oblique muscle and fuse with its sheath. From this point, an important component of this fascial complex forms Lockwood’s ligament, which extends across the width of the inferior orbit somewhat like a hammock, inserting laterally at the lateral orbital tubercle and medially into the medial canthal tendon and providing some suspensory support to the orbital soft tissues.34 Anterior to Lockwood’s ligament, the capsulopalpebral fascia send fibers into the inferior conjunctival fornix (thus forming the suspensory ligament of the inferior fornix), while additional fibers continue on to fuse with the septum and to finally insert into the inferior border of the tarsal plate. As in the upper lid, the lower lid retractors work in tandem with the inferior rectus to lower the lid with downgaze. The analogous lower lid structure to Müller’s muscle in the upper lid is the inferior tarsal muscle. Loss of sympathetic innervation may cause a small amount of “reverse ptosis” of the lower lid, elevating the inferior lid margin by approximately 1 mm above its usual resting position.33 The tarsal plates are comprised of dense connective tissue that act at the structural skeleton of the lids. In both lids, the tarsi are 1 mm in thickness. In the upper lid, the tarsus is approximately 10-12 mm in height at the pupillary axis, while the vertical extent of the lower tarsus is 4 mm. The tarsi contain the oil-producing meibomian glands which open on the margin, just posterior to the lash line. In the upper lid, approximately 2-3 mm from the tarsal margin, lies the marginal arterial arcade. In the lower lid this arcade typically lies within 1 mm of the lashes. Distichiasis is the abnormal growth of lashes from the meibomian gland orifices and may occur as a congenital anomaly or as an acquired state. In the

latter case, distichiasis is often a result of severe chronic inflammation of the lids35. At their medial and lateral borders, the tarsi taper. The upper and lower tarsi come together at the canthus to form the deep lateral canthal tendon, which inserts just anterior to the lateral orbital tubercle. Recall that the more superficial components of the lateral canthal tendon extend from the lateral pretarsal and preseptal orbicularis oculi muscles. Similarly, the medial aspects of the upper and lower tarsi contribute to the medial canthal tendon, with larger, more superficial components which arise from the orbicularis oculi. The conjunctiva comprises the most posterior layer of the lids. Basal tear flow is provided by the accessory lacrimal glands of Krause in the upper conjunctival fornix, and the glands of Wolfring in the lower fornix. Additional mucin-producing glands are distributed within both the orbital and palpebral conjunctivae.

The Lacrimal System The main lacrimal gland lies in the anterolateral orbital roof, within the lacrimal gland fossa of the frontal bone, and measures roughly 20 × 12 × 5 mm. The gland is separated into a palpebral and an orbital lobe by the lateral levator aponeurosis. The primary suspensory support for the main lacrimal gland comes from the Whitnall’s ligament. 1 Damage to the ligament leads to forward and downward prolapse of the gland in the orbit.36 Ducts from both lobes pass through the palpebral lobe to empty into the superolateral fornix. Therefore, ideally, lacrimal gland biopsies should not be performed on the palpebral lobe, since injury here may affect drainage from both lobes37 (Figure 1.15). Innervation and blood supply are provided by the lacrimal nerve and lacrimal artery, which enter the gland posteriorly. Venous drainage occurs via the lacrimal vein, which empties into the superior ophthalmic vein. Parasympathetic inputs originate from the lacrimal nucleus of the pons. These preganglionic fibers pass through the geniculate ganglion and then travel with the greater petrosal nerve to synapse eventually within the pterygopalatine ganglion. These fibers then directly synapse in the lacrimal gland.38, 39 Additional postganglionic

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14 Surgical Atlas of Orbital Diseases fibers traveling along branches of the maxillary division of the trigeminal nerve that converge with the lacrimal nerve to enter the orbit also innervate the lacrimal gland.40 Tears drain medially via the upper and lower lid puncta, into the canaliculi, and into the lacrimal sac (Figure 1.16). The puncta are approximately 0.3 mm in diameter. The initial segment of each canaliculus extends 2 mm perpendicular to the lid margin then turns roughly 90° medially toward the canthus. These horizontal canalicular segments are approximately 8 mm in length. The lower canaliculus is typically slightly longer than its upper lid counterpart. In 90% of individuals, the upper and lower canaliculi then fuse to form a 2 mm long common canaliculus which lies between the anterior and posterior limbs of the medial canthal tendon and enters the lacrimal sac.41 The valve of Rosenmüller is located at this junction and prevents the reflux of tears from the sac retrograde into the canaliculi. The lengths of each component of the lacrimal drainage system become important when performing probing and irrigation to evaluate the patency of the outflow system. The lacrimal sac sits within the lacrimal sac fossa. It is 12 mm long and its fundus lies 3-4 mm superior to the valve of Rosenmüller.42 The sac lies just anterior to the middle turbinate of the nose. The inferior sac is contiguous with the nasolacrimal duct which courses in the wall of the lateral nose and empties via the valve of Hasner just below the inferior turbinate. The valve of Hasner may be imperforate in young infants, and is the most common site of nasolacrimal duct obstruction in this age group.

inadvertent perforation of the optic nerve sheath during retrobulbar anesthesia.43 There are four major segments to the optic nerve, including the intracranial, intracanalicular, intraorbital and the intraocular segments. The intracanalicular segment of the optic nerve is tightly surrounded by its dural sheath and tethered within the bone. Because of this, the intracanalicular segment of the optic nerve is particularly susceptible to blunt trauma.44, 45 Once it passes through the optic foramen, the length of the intraorbital portion of the nerve is roughly 24-30 mm as it traverses the 20 mm or so distance to the globe. Thus, the nerve has a slightly serpentine course inside the orbit that allows for movement of the globe and some degree of proptosis. However, severe proptosis puts the nerve on stretch, described radiographically as “globe tenting”. 46 The intraocular length of the nerve is approximately 1 mm. Sensory innervation of the orbit: Sensory innervation of the periorbital region is carried by the ophthalmic and maxillary divisions of the trigeminal nerve (fifth cranial nerve). Both branch from the trigeminal ganglion which is located within the lateral wall of the cavernous sinus. The ophthalmic branch further subdivides into three segments: the frontal, lacrimal and nasociliary nerves. The frontal and lacrimal branches enter the

The Nerves of the Orbit The optic nerve: The optic nerve (the second cranial nerve) is actually part of the central nervous system, extending directly from the brain into the orbit. Like the rest of the central nervous system, the optic nerve is invested within a dural sheath and leptomeninges, surrounded by cerebrospinal fluid, and in part, is covered with myelin. The fact that cerebrospinal fluid surrounding the optic nerve communicates with the fluid surrounding the cerebrum and brainstem is the basis for the seizures and life-threatening cardiopulmonary depression which can occur with

Figure 1.16: The lacrimal drainage system

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Applied Anatomy of Orbit 15 orbit in the superolateral part of the superior orbital fissure, outside the annulus of Zinn. The frontal nerve courses through the extraconal fat and separates in the anterior orbit into several smaller branches including the supraorbital branch which supplies the scalp, forehead, upper lid, and conjunctiva. The supraorbital nerve exits via the supraorbital notch or foramen and should be carefully avoided during dissection of the superior orbital rim. Injury to the deep, lateral branches of the supraorbital nerve which run beneath the frontalis muscle, as can occur during forehead lift surgery leads to scalp numbness to the vertex.47 The other major division of the frontal nerve, the supratrochlear nerve, exits just above the trochlea to innervate parts of the lower forehead and medial canthal region. The lacrimal nerve travels with the lacrimal artery superolaterally in the extraconal space, along the superior border of the lateral rectus (Figure 1.17). As it travels forward, it is joined by parasympathetic motor fibers within the orbit which began within the nervus intermedius and which

supply the lacrimal gland, superolateral lid and conjunctiva.37 The nasociliary nerve enters the orbit via the superior orbital fissure within the annulus of Zinn, traversing just under the superior rectus muscle and over the optic nerve medially as it courses forward in the orbit in association with the ophthalmic artery. In the posterior orbit, it subdivides into long posterior ciliary nerves which run medially and laterally toward the globe, giving off sensory fibers which travel through the ciliary ganglion without synapsing. The long ciliary nerves enter the sclera and continue forward, innervating the iris, cornea and ciliary muscles. Additional fibers from the nasociliary nerve travel superomedially and are responsible for sensation from the nasal mucosa and the skin on the medial tip of the nose via the anterior ethmoidal nerve. It is this branch which is responsible for Hutchinson’s sign in cases of herpes zoster ophthalmicus. The final anterior branch of the nasociliary nerve is the infratrochlear nerve, which

Figure 1.17: Lateral view of the orbit and major orbital sensory nerves

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16 Surgical Atlas of Orbital Diseases traverses the orbital septum inferior to the trochlea to supply the medial eyelid skin, lacrimal sac and the caruncle. The maxillary division of the trigeminal nerve exits the middle cranial fossa via the foramen rotundum to enter the pterygopalatine fossa. From here, the zygomatic branch enters the inferior orbit via the inferior orbital fissure. It further subdivides into the infraorbital, zygomaticotemporal, and zygomaticofacial nerves. The infraorbital nerve exits the orbit via the infraorbital notch or groove to supply the skin of the lower lid, cheek and medial upper lip (Figure 1.2). Injury to this nerve by fractures involving the orbital floor result in hypesthesia over these areas. The zygomaticotemporal and zygomaticofacial nerves provide sensory innervation to the lateral brow and lateral cheek, respectively. Motor innervation of the orbit: Motor innervation to the orbit involves the oculomotor, trochlear and abducens nerves, or the third, fourth and sixth cranial nerves, respectively. The oculomotor nerve exits the brainstem medially, leaving its dural sheath to enter the superolateral aspect of the cavernous sinus. Here, it divides into superior and inferior divisions which both pass into the orbit through the superior orbital fissure, within the annulus of Zinn. The superior division sends branches to the levator muscle and superior rectus while the inferior division branches into three parts to supply the medial rectus, inferior rectus and inferior oblique. The branch which innervates the inferior oblique also carries parasympathetic fibers which synapse in the ciliary ganglion. Thus, injury due to surgery or trauma to these inferior orbital structures can lead to an efferent pupillary defect and dilation.48 The trochlear nerve, the smallest and longest of the cranial nerves, arises from the dorsal midbrain, crosses the midline to emerge adjacent to the superior cerebellar peduncle. It enters the cavernous sinus along its lateral wall, reaching the orbit via the superior orbital fissure, above the annulus (along with the frontal and lacrimal nerves). It travels anteromedially above the levator just inferior to the periorbita, and enters the superior oblique at the muscle belly’s posterior third. The trochlear nerve is unique among the cranial nerves. It is the only cranial nerve innervating an extraocular muscle which does not penetrate the intraconal surface of the muscle it

serves. It is also the smallest cranial nerve, has the longest intracranial component, and is the only cranial nerve to exit dorsally from the brainstem. For these reasons, it is also the most prone to injury with closed head trauma.49 The abducens nerve originates from the pons and enters the cavernous sinus, initially following a course within the sinus near the internal carotid artery before coursing laterally along the wall. It passes into the orbit via the intra-annular portion of the superior orbital fissure, running along the inner surface of the lateral rectus and piercing the muscle belly at its posterior one-third. The intracranial course of the abducens nerve turns sharply as it crosses the petrosphenoidal ligament, making it particularly prone to injury50, 51 with acute increases50 or decreases52 in intracranial pressure. Sympathetic innervation of the orbit: Sympathetics to the orbit which supply the iris dilator, eyelid muscle, eccrine sweat glands, and blood vessels originate from the superior cervical ganglion. These fibers travel along the internal carotid artery, through the cavernous sinus and into the orbit along the ophthalmic artery, via the superior orbital fissure. The sympathetics pass through the ciliary ganglion (located lateral to the optic nerve at the apex) without synapsing.12 Parasympathetic innervation of the orbit: Parasympathetics innervate the iris sphincter muscle, ciliary muscle, lacrimal gland and orbital blood vessels to produce miosis, lacrimation and relaxation of vascular tone. These inputs originate in the Edinger-Westphal nucleus (third cranial nerve), the salivatory nucleus via the nervus intermedius 10 (the parasympathetic nerve fibers originating from the facial nerve), and the parasympathetic ganglia supporting the orbit. Preganglionic parasympathetics course with the oculomotor nerve, along its inferior division, and enter the orbit via the inferior orbital fissure. These fibers run superficially in the oculomotor nerve as it exits the brainstem adjacent to the posterior communicating artery. Thus, aneurysms of the posterior communicating artery may produce a third nerve palsy with an associated dilated pupil. These nerves synapse in the ciliary ganglion and enter the globe as the short posterior ciliary nerves.

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Applied Anatomy of Orbit 17 The ciliary ganglion lies adjacent to the lateral aspect optic nerve at the orbital apex. The ganglion also contains sympathetics that travel into the orbit via the ophthalmic artery to reach the iris dilator and ocular blood vessels, as well as sensory fibers from the nasociliary nerve which supply intraocular structures. Neither the sympathetics nor the sensory fibers synapse within the ganglion. Preganglionic fibers from the facial nerve nucleus pass through the geniculate ganglion and then travel with the greater petrosal nerve to eventually synapse within the pterygopalatine ganglion. These fibers course via the infraorbital fissure to the orbit and directly to the lacrimal gland. 37, 38 Additional postganglionic fibers travel along branches of the maxillary division of the trigeminal nerve that converge with the lacrimal nerve to enter the orbit.

Vascular Anatomy of the Orbit: Arterial Supply The ophthalmic artery, the first intracranial branch from the internal carotid artery, provides most of the blood supply to the orbit and globe. The ophthalmic artery arises just as its parent vessel exits the cavernous sinus, just inferior to the optic nerve and posterior to the anterior clinoid process. It immediately joins the optic nerve along its inferolateral surface, traveling within a common dural sheath, and entering the apex via the optic foramen.53, 54 Once inside the orbit, the artery crosses medially and gives off its major apical branches (Figure 1.18). The major intraconal vessels include the central retinal artery, branches to the extraocular muscles, and the long and short posterior ciliary arteries. The first branch of the ophthalmic artery is the central

Figure 1.18: Lateral view of the orbit and major branches of the ophthalmic artery

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18 Surgical Atlas of Orbital Diseases retinal artery. This vessel typically pierces the nerve inferomedially, at a point approximately 10 mm from the globe, and as it reaches the globe, gives off the end arteries to the retina. Branches to the extraocular muscles show greater individual variation in distribution. Generally, these vessels run within the muscle belly or along their medial surfaces. As they continue to travel anteriorly with the muscles, the terminal branches of these arteries enter the globe at the tendinous muscle insertions, becoming the anterior ciliary arteries. These branches provide anastomoses with the long posterior ciliary arteries to supply the iris, ciliary muscle and other anterior intraocular structures. Because of this contribution of the muscular arteries to the anterior segment, disinserting more than two extraocular muscles from the globe during surgery at one time is generally avoided. Typically, there are two or three posterior ciliary arteries which branch from the ophthalmic artery near the apex and run medially and laterally within the orbit. Some of these vessels divide into 15-20 short posterior ciliary arteries which enter the posterior aspect of the sclera to supply the choroid and the optic nerve head. Others, the two long posterior ciliary arteries, continue to travel anteriorly within the sclera, entering the globe medially and laterally to supply the anterior segment and anastomosing with terminal branches of the muscular arteries. The major extraconal, apical branches of the ophthalmic artery include the lacrimal and posterior ethmoidal arteries. The lacrimal artery, along with the lacrimal nerve, runs above the superior border of the lateral rectus to reach the lacrimal gland and lateral upper lid. It anastomoses with the middle meningeal artery via the recurrent meningeal artery, and the temporal arteries. The posterior ethmoidal courses medially, along the frontoethmoidal suture to exit via the posterior ethmoid foramen where it gives off branches supplying the sinus and nasal mucosa and the frontal dura.54, 55 As the ophthalmic artery continues forward in the orbit, it then gives rise to the anterior ethmoidal artery and finally, its terminal branches. The anterior ethmoidal vessel exits via the anterior ethmoidal foramen to supply frontal dura and ethmoid and frontal sinus mucosa. Anastomoses from this circulation and branches of the external carotid

provide blood flow to the nose and septum. The frontoethmoidal suture, along which the anterior and posterior ethmoidal arteries and associated branches of the nasociliary nerve run, is an important landmark for the roof of the ethmoid, or fovea ethmoidalis which lies just beyond this line. Penetration of the medial wall above this suture would allow communication between the anterior cranial fossa and the orbit. Additionally, the posterior ethmoidal foramen characteristically lies 6 mm anterior to the optic canal and 12 mm posterior to the anterior ethmoidal foramen.2 The terminal branches of the ophthalmic artery are the supraorbital, supratrochlear, dorsal nasal and the medial palpebral arteries. The supraorbital and supratrochlear arteries provide the blood supply to the forehead and medial lids, while the dorsal nasal and medial palpebral arteries supply the medial lids and nose. The supraorbital artery travels above the levator via the supraorbital notch or foramen and should be carefully avoided during surgical dissection of the orbital roof. All of these vessels anastomose extensively with external carotid branches to the face. It is clear that there is great degree of collateral flow to the orbit and lids between the internal and external carotid circulation. Therefore, a review of relevant branches of the external carotid artery, namely branches of the maxillary artery, is also important. Superiorly, the superficial temporal artery provides blood supply to the forehead, anastomosing with the circulation of the supraorbital and suprotrochlear arteries. The angular artery provides anastomoses with the dorsal nasal and palpebral arteries medially. Within the orbit itself, the sphenopalatine artery, like the ethmoidal circulation, supplies the nasosinus mucosa and nasal septum. The superficial branches of the infraorbital artery anastomose with the inferomedial palpebral arteries, while the deeper branches anastomose with the muscular arteries. The anastomosis between the lacrimal artery and the middle meningeal artery has already been discussed. This occurs via the recurrent meningeal artery, which enters the orbit through the sphenoid, through a foramen superolateral to the superior orbital fissure or directly via the fissure itself.2

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Applied Anatomy of Orbit 19

Vascular Anatomy of the Orbit: Venous Outflow The venous drainage pathways of the orbit run independently of the arteries and are a completely valveless system. There are three major outflow systems, involving the cavernous sinus, pterygoid plexus, and an anterior venous system which drains via the facial vein (Figure 1.19). The superior ophthalmic vein provides outflow from the superifical, superior periorbital and orbital regions, via the supraorbital, nasofrontal and angular veins. It can be divided into three segments as it runs anterior-posteriorly. The first segment courses adjacent to the trochlea and along the medial edge of the superior rectus. The second passes inferior to the muscle and into the cone. This segment receives the ciliary and superior vortex veins from the globe. The third portion of the superior orbital vein travels

along the lateral edge of the superior rectus and exits the orbit via the extra-annular superior orbital fissure to drain into the cavernous sinus. The inferior ophthalmic vein drains the inferior orbit, including tributaries from the inferior rectus and oblique muscles and from the inferior vortex veins. The inferior ophthalmic vein anastomoses with a branch of the superior ophthalmic vein. A portion of the outflow is directed into the pterygoid plexus and the rest directly into the cavernous sinus. Anteromedially, venous drainage occurs mainly via the angular and facial veins. Because of the high degree of anastamoses and absence of valves, some degree of venous obstruction can be redirected within the system. However, acute thromboses, particularly of the cavernous sinus, cause marked orbital congestion and subsequently, exophthalmos.

Figure 1.19: Venous drainage system of the orbit

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20 Surgical Atlas of Orbital Diseases

Paranasal Sinuses There are four pairs of paranasal sinuses: the frontal, ethmoid, sphenoid and maxillary sinuses which directly neighbor the orbital roof, medial wall, and floor. Knowledge of their anatomy is useful since these spaces can share disease processes with the orbit such as infection and tumors, and can also provide surgical access to the orbit and lacrimal system. The frontal sinus overlies the anterior portion of the orbital roof and drains into the frontonasal duct which travels though the anterior portion of the ethmoid sinus (ethmoid infundibulum) to empty into the middle meatus within the nose. This sinus develops in childhood and is usually difficult to appreciate radiographically until age 7 or so. Pneumatization is typically completed by early adulthood.56 The frontal sinus is a common site for mucocele formation.57 The ethmoid sinuses lie between the medial orbital walls and immediately posterior to the nose. The lateral wall of this sinus is comprised of the very thin lamina papyracea, which is easily fractured in surgery or trauma, or compromised by local infection. A frequent source of orbital cellulitis is ethmoid sinusitis which spreads secondarily. The ethmoid roof, or fovea ethmoidalis, is located just beneath the anterior cranial fossa and just medial to the frontoethmoidal suture line within the orbit. The most medial portion of the ethmoid roof is the cribriform plate, which overlies the nasal cavity (Figure 1.20). The sinuses themselves are comprised of many individual, thin-walled air cells and can be

divided into three groups. The anterior and middle air cells drain into the middle meatus while the posterior air cells empty into the superior meatus of the nose. In performing dacryocystorhinostomy to create a passage between the lacrimal sac and nose, the ethmoid air cells are frequently encountered extending anterior to the posterior lacrimal crest.58,59 The sphenoid sinuses are located midsagittally and posterior to the ethmoid air cells. Like the frontal sinuses, they pneumatize relatively late in life and do not reach full size until adolescence. Drainage occurs via the sphenoethmoid recess located in the anterior sinus wall. Because the contents of the orbital apex and nearby cavernous sinus exit the orbit through the sphenoid bone, the walls of the sphenoid lie in close proximity to a number of critical structures. Anteriorly and superolaterally, the optic nerve and intracavernous portion of the internal carotid artery run along the lateral sinus walls. Severe sphenoid sinusitis can therefore cause optic nerve injury. 60 Likewise, congenital dysplasia of the sphenoid, as can occur in neurofibromatosis type 1, can produce pulsatile proptosis.61, 62 The sphenoid sinus also provides a useful surgical approach to the pituitary fossa which is located posteriorly to the sinus.63 The maxillary sinus underlies the orbital floor and is the largest of the paranasal sinuses. Drainage from this sinus occurs via the maxillary ostium into the middle meatus. The ostium is located high within the medial sinus wall, close to the level of the orbital floor. Thus, trauma to the orbital floor (i.e. orbital fracture or inferomedial decompression) can obstruct drainage from the sinuses. Inside the medial walls of the maxillary sinus, lie the bony nasolacrimal canals. The posterior most aspect of the sinus extends from the area of the infraorbital fissure, and the infraorbital nerve and artery run along the maxillary roof within the infraorbital canal. Behind the maxillary sinus is located the pterygopalatine fossa and the maxillary artery runs in its posterior wall.

Conclusion

Figure 1.20: Computed axial tomography illustrating the relationship between the anterior cranial fossa, orbit and ethmoid sinus

The orbit and its surrounds represent a complex anatomical space, incorporating critical ocular, neural, and vascular structures. The purpose of this chapter has been to provide an overview of orbital anatomy, as well as basic anatomy of the eyelids, lacrimal

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Applied Anatomy of Orbit 21 system, and paranasal sinuses. A detailed understanding of this anatomy is fundamental to oculoplastic surgery and the management of orbital disease.

REFERENCES 1. Whitnall SE. The Anatomy of the Human Orbit and Accessory Organs of Vision, 2nd ed. NewYork: Oxford University Press; 1932. 2. Rootman J, Stewart B, Goldberg RA. Orbital Surgery: A Conceptual Approach New York: Lippincott-Raven, 1995. 3. Webster RC. Supraorbital and supratrochlear notches and foramina: Anatomical variations and surgical relevance. Laryngoscope 1986;96:311. 4. Kim JW, Goldberg RA, Shorr N. The inferomedial orbital strut: An anatomic and radiographic study. Ophthal Plast Reconstr Surg 2002;18:355-64. 5. Rose JG, Lucarelli MJ, Lemke BN. Lacrimal, orbital and sinus anatomy. In: Woog JJ, (Ed): Manual of Endoscopic Lacrimal and Orbital Surgery. London: Elsevier, 2003. 6. Goldberg RA, Schorr N, Cohen M. The medial orbital strut in the prevention of post-decompression dystopia in dysthyroid ophthalmopathy. Ophthalmic Plast Reconstr Surg 1992;8:32-34. 7. Whitnall S. On a tubercle on the malar bone, and on the lateral attachments of the tarsal plates. J Anat Physiol 1911;45:426-32. 8. Yeh S, Foroozan R. Orbital apex syndrome. Curr Opin Ophthalmol 2004;15:490-98. 9. Lenzi GL, Fieschi C. Superior orbital fissure syndrome: a review of 130 cases. Eur Neurol 1977;16:23-30. 10. Morard M, Tcherekayev V, deTribolet N. The superior orbital fissure: a microanatomical study. Neurosurgery 1994;35:1087-93. 11. Jordan DR. The nervus intermedius. Arch Ophthalmol 1993;111:1691. 12. Lyon D, Lemke BN, Wallow I, Dortzbach RK. Sympathetic nerve anatomy in the cavernous sinus and retrobulbar orbit of the cynomolgous monkey. Ophthalmic Plast Reconstr Surg 1992;8:1-12. 13. Keane JR. Cavernous sinus syndrome. Analysis of 151 cases. Arch Neurol 1996;53:967-71. 14. Koornneef L. New insights in the human periorbital connective tissue: results of a new anatomic approach. Arch Ophthalmol 1977;95:1269-73. 15. Koornneef L. Orbital septa: anatomy and function. Ophthalmology 1979;86:876-85. 16. Sutton J. The fascia of the human orbit. Anat Reconstr 1920;18:141. 17. Dutton J. Atlas of Clinical and Surgical Orbital Anatomy. Philadelphia: WB Saunders Co; 1994. 18. Jones LT. A new concept of the orbital fascia and rectus muscle sheaths and its surgical implications. Trans Am Acad Ophthalmol Otolaryngol. 1968;72:755-64.

19. Fink W. An anatomical study of the check ligaments of the vertical muscles of the eyes. Am J Ophthalmol 1957;44: 800-09. 20. Helveston E, Merriam W, Ellis F, Schelhamer R, Gosling G. The trochlea: a study of the anatomy and physiology. Ophthalmology 1982;89:124-33. 21. Sacks J. The shape of the trochlea. Arch Ophthalmol 1984;102:932. 22. Neely K, Ernest J, Mottier M. Combined superior oblique palsy and Brown’s syndrome after blepharoplasty. Am J Ophthalmol 1990;109:347. 23. Apt L. An anatomic reevaluation of rectus muscle insertions. Trans Am Ophthalmol Soc 1988;78:365. 24. Ahl NC, Hill JC. Horner’s muscle and the lacrimal system. Arch Ophthalmol. 1982;100:488-93. 25. Shinohara H, Kominami R, Yasutaka S, Taniguchi Y. The anatomy of the lacrimal portion of the orbicularis oculi muscle (tensor tarsi or Horner’s muscle). Okajimas Folia Anat Jpn 2001;77:225-32. 26. Meyer DR, Linberg JV, Wobig JL, McCormick SA. Anatomy of the orbital septum and tissues. Implications for ptosis surgery. Ophthal Plastic Reconstr Surg 1991;7:104-13. 27. Hawes MJ, Dortzbach RK. The relationship of capsulopalebral fascia with orbital septum of the lower eyelid: an anatomic study under magnification. J Craniofacial Surgery 2006;17:1118-20. 28. Castanares S. Blepharoplasty for herniated intraorbital fat: anatomical basis for a new approach. Plast Reconstr Surg 1951;8:46-58. 29. Kikkawa DO, Lucarelli MJ, Shovlin JP, Cook BE, Lemke BN. Ophthalmic facial anatomy and physiology. In: Levine, MR, ed. Manual of Oculoplastic Surgery, third ed. New York: Butterworth Heinemann; 2003. 30. Whitnall S. On a ligament acting as a check to the action of the levator palpebrae superioris muscle. J Anat Physiol. 1910;14:131. 31. Goldberg RA. Eyelid anatomy revisited: dynamic highresolution images of Whitnall’s ligament and upper eyelid structures with the use of a surface coil. Arch Ophthalmol. 1992;110:1598. 32. Anderson R. Dixon R. The role of Whitnall’s ligament in ptosis surgery. Arch Opthalmol 1979;97:705-07. 33. Walton KA, Buono LM. Horner syndrome. Curr Opin Ophthalmol 2003;14:357-63. 34. Lockwood C. the anatomy of the muscles, ligaments, and fascia of the orbit, including an account of the capsule of tenon, the check ligaments of the recti, and of the suspensory ligament of the eye. J Anat Physiol 1885; 20:2-25. 35. Scheie HG, Albert DM. Distichiasis and trichiasis: origin and management. Am J Ophthalmol 1966;61:718. 36. Lemke BN, Lucarelli MJ. Anatomy of the ocular adnexa, orbit, and related facial structures. In: Nesi, FA, Lisman, RD, Levine, MR, (Eds). Smith’s Ophthalmic Plastic and Reconstructive Surgery. St. Louis: CV Mosby; 1998.

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22 Surgical Atlas of Orbital Diseases 37. Smith B, Petrelli R. Surgical repair of the prolapsed lacrimal gland. Ophthalmology 1978;96:113. 38. Ruskell G. The orbital branches of the pterygopalatine ganglion and their relationship with internal carotid nerve branches in primates. J Anat 1970;106:323. 39. Ruskell G. The distribution of autonomic postganglionic fibers to the lacrimal gland in monkeys. J Anat 1971;109: 229-42. 40. Dutton J. The lacrimal systems. In: Dutton, J. Atlas of Clinical and Surgical Orbital Anatomy. Philadelphia: WB Saunders Co; 1994. 41. Tucker N, Tucker S, Linberg J. The anatomy of the common canaliculus. Arch Ophthalmol 1996;114: 1231-34. 42. Groell R, Schaffler G, Uggowitzer M, Szolar D, Muellner K. CT: anatomy of the nasolacrimal sac and duct. Surg Radiol Anat. 1997;19:189-91. 43. Drysdale D. Experimental subdural retrobulbar injection of anesthetic. Ann Ophthalmol 1984;16:716-19. 44. Anderson RL, Panje WR, Gross CE. Optic nerve blindness following blunt forehead trauma. Ophthalmology 1982; 89: 445–55. 45. Sarkies N. Traumatic optic neuropathy. Eye 2004;18: 1122–25. 46. Dalley R, Robertson W, Rootman J. Globe tenting: a sign of increased orbital tension. Am J Neuroradiol. 1984;10: 181-86. 47. Knize D. A study of the supraorbital nerve. Plast Reconstr Surg 1995;96:564-69. 48. Hornblass A. Pupillary dilation in fractures of the floor of the orbit. Ophthalmic Surg 1979;10:44. 49. Mansour AM, Reinecke RD. Central trochlear palsy. Surv Ophthalmol 1986;30:279-97. 50. Wall M, George D. Idiopathic intracranial hypertension. A prospective study of 50 patients. Brain 1991;114:155-80.

51. Hanson RA, Ghosh S, Gonzalez-Gomez I, Levy ML, Gilles FH. Abducens length and vulnerability? Neurology 2004;62:33-36. 52. Insel TR, Kalin NH, Risch SC, Cohen RM, Murphy DL. Abducens palsy after lumbar puncture. N Engl J Med 1980;303:703. 53. Hayreh SS. The ophthalmic artery. I. Origin and intracranial and intracanalicular course. Br J Ophthalmol 1962;46:65-98. 54. Lang J, Kageyama I. The ophthalmic artery and its branches, measurements and clinical importance. Surg Radiol Anat 1990;12:83-90. 55. Ettl A, Kramer J, Daxter A, Koornneef L. High resolution magnetic resonance imaging of neurovascular orbital anatomy. Ophthalmology 1997;104:869-77. 56. Zinreich SJ. Imaging of the nasal cavity and paranasal sinuses. Curr Opin Radiol 1992;4:112-16. 57. Rootman J. Disease of the Orbit. Philadelphia: JB Lippincott; 1988. 58. Buus D, Tse D, Farris B. Ophthalmic complications of sinus surgery. Ophthalmology 1990;97:612-19. 59. Blaylock W. Moore C, Linberg J. Anterior ethmoidal anatomy facilitates dacryocystorhinostomy. Arch Ophthamol 1990;108:1774-77. 60. Fujimoto N, Adachi-Usami E, Saito E, Nagata H. Optic nerve blindness due to paranasal sinus disease. Ophthalmologica 1999;213:262-64. 61. Hunt JC, Pugh D. Skeletal lesions in neurofibromatosis. Radiology 1961;76:1–19 62. Mortada A: Pulsating exophthalmos with orbital neurofibromatosis, Am J Ophthalmol 1967;64:462. 63. Kanter AS, Dumont AS, Asthagiri AR, Oskouian RJ, Jane JA, Laws ER. The transsphenoidal approach: a historical perspective. Neurosurg Focus 2005;18:1-4.

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2

CHAPTER

Clinical Approach to Proptosis Subrahmanyam Mallajosyula

Before we discuss about proptosis let me remind you that we have to distinguish it from certain conditions which resemble proptosis (pseudo-proptosis) like unilateral high myopia, buphthalmos, unilateral lid retraction, unilateral minimal ptosis of contralateral eye. Though advances in imaging techniques have revolutionized the diagnosis of orbital diseases, proper clinical evaluation of proptosis is still very important because it gives us the insight into the disease process and helps in evaluating the CT/MRI and arriving at a correct diagnosis. I follow the

Figure 2.1: Orbital abscess

Figure 2.3: Carotid-cavernous fistula

9 “P”s, an extension of the 6 “P”s of Krohel’s. The 9 Ps are: Pain, Progression (from history), Proptosis, Pulsations, Pupil, PBCT, Perception of color vision, Periorbital changes (inspection) and Palpation. Pain: When severe pain is the presenting symptom in proptosis, we have to consider the following conditions: infection and inflammatory lesions like orbital cellulitis, orbital abscess (Figure 2.1), myocysticercosis, vascular conditions like lymphangioma (Figure 2.2), high flow carotid- cavernous fistula (Figure 2.3). Metastatic lesions (Figure 2.4) are also very painful.

Figure 2.2: Lymphangioma

Figure 2.4: Metastasis from thyroid carcinoma

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24 Surgical Atlas of Orbital Diseases Retinoblastoma (Figure 2.5), and rhabdomyosarcoma (Figure 2.6) can be very painful and mimic orbital cellulitis. Thyroid orbitopathy which is usually chronic, can rarely present acutely and can be very painful. (Figures 2.7 and 2.7A) Moderate pain is a feature of idiopathic orbital inflammatory syndrome, myocysticercosis, (Figures 2.8 and 2.8A) ruptured dermoid cyst, while dull boring pain is associated with bone-erosion usually due to neoplastic tumors (Figures 2.9 and 2.9A). Pain can be a feature of proptosis following trauma (Figures 2.10 and 2.10A)

Proptosis following trauma can be immediate (due to retrobulbar hemorrhage or surgical emphysema) or delayed due to carotid cavernous fistula. I came across a single case of pulsatile proptosis following trauma, due to herniation of brain through fractured roof of orbit.

Figure 2.5: Retinoblastoma

Figure 2.6: Rhabdomyosarcoma

Figure 2.7: Acute thyroid orbitopathy with chemosis and exposure keratopathy. Notice the lid retraction of left eye Figure 2.7A: CT showing enlarged recti with sparing of tendons

Figure 2.8: Myocysticercosis presenting as ptosis and proptosis with pain. Note the periocular inflammatory response

Figure 2.8A: CT scan of the orbit showing cystic lesion involving SR-LPS complex with hyper dense spot in the cyst (Scolex)

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Clinical Approach to Proptosis 25

Figure 2.9: Eccentric proptosis with globe pushed down. Note fullness of lacrimal gland region

Figure 2.9A: CT scan orbit showing a mass in the fossa of lacrimal gland. Note the bony erosion and heterogenisity of the mass (Adenoidcystic carcinoma of lacrimal gland)

Figure 2.10: Acute proptosis following Trauma. Note severe chemosis, exposure keratitis with hypopyon

Figure 2.10A: After anterior orbitotomy shows complete recovery. Vision improved to 20/30

Progression: The onset of proptosis can be acute (hours to week), subacute (1 to 4 weeks) or chronic (more than 1 month). Acute proptosis can be due to infections, inflammations, parasitic infestations, trauma, metastatic lesions or lymphangioma. Subacute presentation is common in inflammations, parasitic infestations or metastatic neoplasia.

Figure 2.11: Metastatic orbital lesion presenting subacutely. Note the inflammatory changes

(Figure 2.11) Chronic presentation is commonly due to thyroid associated orbitopathy (Figure 2.12), orbital varices or benign neoplasia like cavernous hemangioma, (Figures 2.13 to 2.13B), Neurofibroma (Figures 2.14 to 2.14E), Schwannoma (Figures 2.15 to 2.15B), Glioma of Optic Nerve, (Figures 2.16 to 2.16C). Chronic presentation is characteristic of most of the primary neoplasia of the orbit, both benign and malignant. However, if the presentation is less than 6 months, consider the possibility of a malignant lesion.

Figure 2.12: Thyroid associated orbitopathy with chronic presentation. Note the lid retraction and lateral flare of right upper lid. Note the absence of congestion and edema

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26 Surgical Atlas of Orbital Diseases

Figure 2.13: Axial proptosis of left eye 3 yrs. Notice how quiet was the globe

Figure 2.13A: CT scan showing well encapsulated tumor (Cavernous hemangioma)

Figure 2.14: M 52, RE proptosis since 3 yrs, Def. Vision 1yr. RAPD +, VA : 20/200

Figure 2.13B: Excised tumor

Figure 2.14A: CT shows a large well defined mass with bony expansion

Figure 2.14B: Extension of tumor into superior peripheral space pushing the globe down

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Clinical Approach to Proptosis 27

Figure 2.15: F 30, presented with proptosis of 3 yrs. Defective vision(20/800) RAPD +ve

Figure 2.14C: Well encapsulated tumor on gross exam

Figure 2.15A: CT scan showing a well encapsulated intraconal mass. Note the excavation of lateral wall

Figure 2.14D: Spindle-cells of neurofibroma

Figure 2.14E: Postoperative status recovery from proptosis. VA improved to 20/40

Figure 2.15B: Excised tumor, proved to be schwannoma

Intermittent proptosis is usually due to idiopathic orbital inflammatory syndrome, lymphangioma

(Figure 2.17), orbital varices and myocysticercosis (in endemic areas). (Figure 2.18)

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28 Surgical Atlas of Orbital Diseases

Figure 2.16: M 14 yrs, proptosis of right eye since 8 years. RAPD + Vision absent PL

Figure 2.16A: CT scan orbit showing optic nerve glioma with cystic degeneration

Figure 2.16B: Excised tumor Figure 2.16C: Postoperative status. No proptosis

Figure 2.17: Girl of 12 years presenting with recurrent episodes of proptosis (3 in 5 yrs). Note the subconjunctival hemorrhage in the proptosed LE (Lymphangioma)

Proptosis: Proptosis or protrusion of the eye ball depends on the location of the orbital lesion. A lesion in the intraconal space pushes the globe forwards to cause “axial proptosis” (Figures 2.19 to 2.19B), where as a lesion in the peripheral surgical space pushes the globe to the opposite side and causes “eccentric

Figure 2.18: CT scan showing an enlarged superior rectus muscle with a cyst showing hyper-dense spot within (Myocysticercosis)

proptosis.” However, since these surgical compartments are not strictly water-tight, a large intraconal lesion can enter peripheral surgical space and cause eccentricity to an otherwise axial proptosis (Figure 2.14 series).

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Clinical Approach to Proptosis 29 Axial proptosis: Lesion is in the intraconal space. In my experience the most common lesion of intraconal space causing axial proptosis is cavernous hemangioma followed by Schwannoma, and neuro-fibroma. Optic nerve glioma and meningioma of optic nerve sheath are important but not very common lesions. Other lesions include orbital varix, lymphangioma. The most common intraconal cystic lesion is hydatid cyst (Figures 2.20 and 2.20A). One has to remember that most of the intraconal lesions can compress optic nerve and lead to visual loss. Hence loss of vision in axial proptosis does not necessarily mean that the patient had either optic nerve glioma or meningioma of optic nerve sheath. It can be due to any other lesion of intraconal space like hemangioma, schwannoma, neurofibroma, hydatid cyst or even idiopathic orbital inflammatory syndrome. Optic nerve can also be compressed by enlarged extraocular muscles as in thyroid associated orbitopathy.

Down and out proptosis is due to lesions of superomedial space (pushing the globe down and out). Frontoethmoidal lesions are the most common cause of such eccentric proptosis. Mucocele (Figures 2.21A to C), fungal granuloma, neoplastic lesions and fibrous dysplasia (Figures 2.22A and B) are the common lesions. Lesions of supero-medial space like dermoids, hemangiomas can also present with eccentric proptosis with globe displaced down and out (Figures 2.23A and B). Osteoma of ethmoid (Figures 2.24A to E) is another rare cause.

Figure 2.19: Axial proptosis of left eye due to intraconal lesion

A

B Figures 2.19A and B: CT scans of orbit showing intraconal tumor arising from optic nerve (Optic nerve glioma)

Figure 2.20: Intraconal hydatid cyst of orbit

Figure 2.20A: Cyst excised with cryo after aspirating fluid

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30 Surgical Atlas of Orbital Diseases

A

B

C Figures 2.21A to C: Eccentric proptosis with the eyeball pushed down and out. Notice the gross outward displacement with fullness in the superomedial aspect. CT scan shows a huge fronto-ethmoid mucocele

A

Figures 2.22A and B: Eccentric proptosis with globe pushed down and out due to fibrous dysplasia of frontal bone as demonstrated by the CT scan (B)

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Clinical Approach to Proptosis 31

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Figures 2.23A and B: Coronal and axial sections of CT scan orbit showing a very large cystic lesion in the superomedial peripheral space (blue arrow), displacing the Globe (green pentagon) down and out. Compare the size of the cyst with that of opposite eyeball. Note the bony excavation of the roof and medial wall (red arrow). This is a case of Hydatid cyst of orbit

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Figures 2.24A to C: This young male of 22 yrs presented with recurrent, eccentric proptosis of right eye since 1year, and defective vision since 3 months. He underwent surgery elsewhere for similar lesion 2 years back. Note the periocular fullness, and lateral displacement of the globe. He had RAPD and the vision was 20/40. CT scan of orbits revealed a huge osteoma involving the ethmoid bone (arrow)

E D

Figures 2.24D and E: Excised ivory osteoma. The postoperative recovery was uneventful. The patient's vision improved to 20/20

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32 Surgical Atlas of Orbital Diseases Down and in displacement of the globe is mostly due to enlargement of lacrimal gland, due to infection (Dacryoadenitis), inflammation (idiopathic orbital inflammation, Mukulitz syndrome (Figures 2.25A to C), or neoplasia. Pleomorphic adenoma (Figures 2.26A to D) is the most common benign tumor while Adenoidcystic carcinoma of the lacrimal gland (Figures 2.27A to D) is the most common and

dreaded malignant tumor of the lacrimal gland. Other rare causes include lymphoma (Figures 2.28A to D) pleomorphic adenocarcinoma, adenocarcinoma, squammous cell carcinoma and reactive lymphoid hyperplasia. Other lesions of the fossa of lacrimal gland like dermoids (Figures 2.29A to D) can also cause eccentric proptosis with globe displaced down and in.

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Figures 2.25A to C: Female 55 years, presented with proptosis left eye of 18 months duration. Notice the globe was pushed down and in, with fullness of outer half of left upper lid. The tear film was normal. CT scan of orbit showed bilateral enlarged lacrimal glands, molding to the globe suggestive of lymphoma (B). The excised specimen (C) (note the concavity of medial surface of the excised specimen, which corresponds to the globe) The histopatholgical diagnosis was Sjogren's syndrome

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Figures 2.26A and B: This female 32 yrs. presented with proptosis of right eye since 2 years. Note the globe pushed down and in, and the fullness of lacrimal gland region and the mass effect. CT scan of orbits showed a well encapsulated mass lesion of lacrimal gland. The surrounding bone was normal. Clinical diagnosis was pleomorphic adenoma of lacrimal gland

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Clinical Approach to Proptosis 33

D Figures 2.26C and D: The mass was excised through superior lid crease incision. Note the well encapsulated tumor (C) and well hidden incision in the lid crease (D) Histopathology confirmed it to be pleomorphic adenoma of lacrimal gland

C

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Figures 2.27A to D: This female 28yrs. presented with eccentric proptosis of 5 months duration. Note the fullness at lacrimal gland region. The globe was pushed down and in (A) CT scan revealed a lacrimal gland tumor with irregular surface and bony erosion (B). It was a case of adenoid cystic carcinoma of lacrimal gland for which exenteration was done (C). Postoperatively the patient was doing well and there was no recurrence after 8 years (D)

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34 Surgical Atlas of Orbital Diseases

A

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Figures 2.28A and B: Note the fullness of left upper lid with globe pushed down and in (A). CT scan of orbit shows enlarged lacrimal gland, molding to the globe (B)

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Figures 2.28C and D: The tumor was excised through Sub-brow incision (C). Histopathology revealed it to be a case of lymphoma. 3 days postoperative photo showing marked improvement from proptosis (D)

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Clinical Approach to Proptosis 35

B A

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Figures 2.29A to D: Female 25 years, presented with eccentric proptosis of right eye since, 2 years, She had 2 episodes of pain and worsening of proptosis in the past 6 months. Note the fullness of the upper lid, and the globe which was pushed down and in (A) Axial and coronal sections of the CT scan show a cystic lesion with variable consistency, situated at the fossa of lacrimal gland (B and C). Excised dermoid cyst (D)

Upward displacement of globe is due to either an extension of lesion of maxillary sinus into orbit (neoplasia, fungal granuloma or dumbbell dermoid) or lesions of inferior space like cavernous hemangioma, neurofibroma, schwannoma, cystic lesions like myocysticercosis involving inferior rectus (Figures 2.30A to C). Many a time lesions of maxillary sinus extend into frontoethmoidal sinuses, or nose. Anterior extension leads to fullness of cheek (Figures 2.31A to C). It is my experience that orbital extension of lesions from paranasal sinuses is a common cause of eccentric proptosis. Measurement of proptosis is a very important part of evaluation of the patient. The displacement of the globe should be quantified in all the 3 directions, anteroposterior (axial), horizontal and vertical axes. Naphzeiger’s test is a useful bedside

clinical test to detect mild proptosis (Figures 2.32A and B). Ask the patient to look at a distant object, located at the same level as that of the patient’s eye. Stand behind the patient, and gently tilt the head backwards and look down over the patient’s forehead. The proptosed eye appears ahead of the other. Another simple bedside test to detect mild proptosis is with the help of a scale. Ask the patient to gently close the eyes, and keep a scale across the eye in contact with his forehead and cheek. Normally there will be space between the scale and the closed lid. In the presence of proptosis this space is obliterated (Figures 2.33A and B). Axial proptosis is best measured with Hertel's exophthalmometer (Figure 2.34A). Leudde's exophthalmometry is less accurate and inter-observer variation is more significant.

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36 Surgical Atlas of Orbital Diseases

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Figures 2.30A to C: This female, 42 years in age, presented with proptosis left eye, which was displaced upwards (A). Note the mass lesion in lower lid, which was transilluminant (B). This demoid cyst was removed through swinging lower lid approach. Note the minimal swelling of lower lid on 2nd postoperative day (C)

B

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Figures 2.31A to C: This child presented with proptosis of right eye since 2 years. Note the fullness of cheek, and gross upward displacement of the globe due to lesion of the Maxillary sinus (A). CT scan of the orbit (B and C) revealed it to be ossifying fibroma. Note that the mass is involving the nasal cavity (red arrow) and the oral cavity (blue arrow)

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Clinical Approach to Proptosis 37 compare results over a period of time. When once the BR is recorded, subsequent readings should be taken with the same BR so that the results are comparable.

Figures 2.32A and B: Naphzeiger’s test: Note the prominence of left eye (arrow). The right eye is not visible

Another very important factor you have to remember is that while performing exophthalmometry, you should occlude the unexamined eye with your thumb, (Figure 2.34C) so that the eye being examined comes to the primary position. Then only the readings are accurate.

Figure 2.33A: Note the space between the closed right eye and the scale in a normal eye

Figure 2.33B: Note the obliteration of space. The scale is in contact with the eyelid of proptosed left eye

Hertel’s exophthalmometry consists of 2 foot plates - one is fixed and the other sliding. Each foot plate has a viewing mechanism (Figure 2.34B) where the eye being examined is seen in profile below a scale in millimeters (Figure 2.34D). There are 2 red lines for reference to avoid parallax error. Rest the fixed footplate on the anterior part of lateral wall of right orbit and slide the other footplate on the scale till it rests on the anterior part of lateral wall of left orbit. Note the distance between the 2 foot plates from the scale. This is called the “Base Reading” (BR). It is very important to record the base reading since the exophthalmometry values change with base reading. In other words, if you take 2 readings on the same patient one after the other with 2 different base readings, the values you get will be different. In fact the one with wider BR will give larger value! If you forget to note the BR, you can not accurately

To measure the horizontal displacement of the eye, put a mark on the center of root of the nose. Measure the distance from that point to the nasal limbus of the proptosed eye, with the other eye being covered. Repeat the same for the normal eye, covering the proptosed eye. The difference between these two readings gives you the horizontal displacement (Figures 2.35A and B). Vertical displacement is measured with the help of 2 rulers. Hold the first scale in line with the lateral canthi. Measure the distance from this scale to the 6 O'clock limbus of each eye. A larger reading of proptosed eye means that the globe is pushed inferiorly, and a lesser reading is obtained when the globe is displaced up (Figures 2.36A and B). Pulsations: Pulsations of globe in proptosis can be either vascular pulsations or transmitted

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38 Surgical Atlas of Orbital Diseases

Figure 2.34A: Hertel’s exophthalmometer: It consists of 2 footplates, one fixed (blue arrow) and another sliding (red arrow) which slides on a scale (green arrow). The distance between the 2 plates is the base reading (98 mm in this picture)

Figure 2.34B: Viewing system of each footplate. It has a red reference line in front (green arrow).Another redline (blue arrow) is seen through the prism. You have to align both the redlines into a single line to eliminate parallax error. The eye ball is seen in profile and its position is read from the scale (red arrow).

C

D

Figures 2.34C and D: Hertel's exophthalmometry. Note the unexamined right eye was occluded with the thumb, so that the eye being examined (left eye) is in the primary position. The scale reading of the anterior most part of the cornea seen in profile (green arrow) is the axial position of the globe

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Clinical Approach to Proptosis 39

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Figures 2.35A and B: Measurement of horizontal displacement of the eyeball. Ask the patient to fix at a distant object in the straight gaze, covering the unexamined eye. Measure the distance from the central point of reference on the root of nose (red arrow) to the nasal limbus; Compare the same with that of other eye. Lesser reading of the proptosed eye means that the globe was pushed medially (A: Central point of reference drawn on the nose, B: Nasal limbus, C; Cover)

A

B

Figures 2.36A and B: First Scale is held joining the lateral canthi and is the point of reference. The second scale measures the vertical displacement. Note that the proptosed left eye is displaced down and gives larger reading

pulsations. Vascular pulsations are due to increased blood flow and is typically seen in CarotidCavernous Fistula (CCF). It is almost always seen in high-flow CCF (Figures 2.37A to C). The other important causes include meningioma (Figures 2.38A to C), orbital varix, aneurysms, etc. Transmitted pulsations are due to cerebral pulsations which are transmitted due to bony deficiency as in

neurofibroma (Figures 2.39A and B). I have seen a child who presented with pulsatile proptosis due to herniation of frontal lobe of brain into orbit following trauma (Figures 2.40A and B). To detect pulsations observe from a side. Subtle pulsations can be recognized while recording IOP with an applanation tonometer.

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40 Surgical Atlas of Orbital Diseases

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Figures 2.37A to C: Note the caput medusae of conjunctival congestion with limitation of abduction of left eye (A). She has a pulsatile proptosis, 3 months after head injury. The IOP was 28 mm of Hg. Bruit was heard with the stethoscope (B). CT scan of orbit revealed a very grossly enlarged superior ophthalmic vein (green arrow), typical of carotid cavernous fistula (C)

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Figures 2.38A to C: This lady of 58 years, presented with pulsatile proptosis of right eye since 1 year (A). Note the temporal fullness, (blue arrow) which was showing pulsations (B), CT scan revealed a mass from temporal lobe and involving the sphenoid wing with extension in to orbit and the temple (C). Note the hyperostosis of Sphenoid bone (red arrow) This is a case of meningioma

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Clinical Approach to Proptosis 41

A Figures 2.39A and B: This male 42 years of age, presented with pulsatile proptosis of left eye since his childhood. Note the tumor involving both the lids of left eye and temporal fullness (A). He had lisch nodules on Iris and caif-au-lait spots typical of neurofibroma. CT scan (B) shows defect in the roof of orbit (red arrow), which explains the transmitted pulsations

B

A

B Figures 2.40A and B: This child presented with pulsatile proptosis following head injury. Note the herniated frontal lobe through the defect in the roof of orbit (arrow)

Pupil: Examination of pupil and its reaction is very important. The presence of Relative Afferent Pupillary Defect (RAPD) indicates that optic nerve is being damaged. Optic nerve can be damaged either due to tumors of the optic nerve or its sheaths, or by compression due to any space occupying lesion of central space, by enlarged extraocular muscles as in thyroid associated orbitopathy, or by nonspecific inflammations of the orbit. Due to narrowing of the orbital space at the apex, a smaller lesion at the orbital apex can lead to optic nerve compression. Optic nerve damage is also seen in severe stretching of optic nerve, as seen by "tenting" of the posterior pole on CT scan.

Perception of Color Vision: This is an important, simple and very sensitive way to know the status of the optic nerve. This statement is relevant for the following reasons. In very early optic nerve compression the vision can still be 20/20. The patient may not notice defective vision in the presence of diplopia, watering and discomfort/ pain. In bilateral cases, RAPD may not be elicited. During fundus examination, very early Optic disc compression can be missed. Visual field analysis may not detect any abnormality in very early cases. Hence the importance of color vision testing can not be over emphasized. When a patient of proptosis is under observation, as in a case of TAO, I instruct the patient

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42 Surgical Atlas of Orbital Diseases to look at a bright red colored object with each eye separately on every sunday, and to report to me immediately if he notices any change in brightness of the color in one eye. If color vision is defective in the proptosed eye, evaluate very carefully the pupil for RAPD, do a very detailed fundus evaluation for the presence of optic disc edema, pallor, presence of opticociliary shunts, retinal/choroidal striae, retinal detachment. Visual field assessment is mandatory.

PBCT: Limitation of ocular motility in proptosis is mostly due to restrictive pathology as in thyroid associated orbitopathy (Figures 2.41A and B), Idiopathic orbital inflammation (myositis component) (Figures 2.42A to D), myocysticercosis (Figures 2.43A and B), and fungal granuloma (Figures 2.44A to C). Another important cause is CCF, wherein the restriction is paralytic in nature (Figures 2.45A to C). Large mass lesions can cause mechanical restriction. Limitation of ocular motility following

A Figures 2.41A and B: TAO: restricted elevation due to enlarged inferior rectus (arrow)

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Figures 2.42A to D: Female 42 yrs., presented with subacute proptosis of left eye with pain and diplopia. Note the congestion of left eye and limitation of abduction (A). CT scan showed enlarged medial rectus involving the tendon. A diagnosis of idiopathic orbital inflammatory syndrome was made. (Blue arrow) (B) She responded very well to systemic steroids. Note that the conjunctival congestion disappeared (C) and abduction restored to normal within 1 week (D)

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Clinical Approach to Proptosis 43

A Figures 2.43A and B: This young girl presented with proptosis of left eye since 4 weeks. She also complained of pain and diplopia. Note the fullness of upper lid, mild congestion of conjunctiva on the medial side and convergent squint. (A) CT scan of orbit revealed myocysticercosis involving the superior oblique muscle. Note 2 cysts, one involving the muscle belly and the other the reflected tendon of superior oblique (B)

B

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trauma can be due to soft tissue edema, entrapment of muscle or its sheath in orbital fracture and rarely due to injury to the muscle itself. I came across a rare cause wherein after trauma; the displaced bone itself caused limitation of motility (Figures 2.46A to D). I routinely quantify the ocular motility in degrees, just like Hirschberg's, so that it is easier to compare the course of the disease over a long period.

Figures 2.44A to C: This elderly male, (A) an agricultural worker by occupation, presented with gross proptosis with frozen globe (B). His vision was PL. Retropulsion was positive. FDT was positive in all directions. CT scan of the orbit revealed a heterogenous mass lesion occupying the entire orbit. Note the molding of the lesion to the globe (red arrow), the tenting of posterior pole (blue arrow) and involvement of anterior ethmoidal sinus. Histopathology showed it to be a fungal granuloma (C)

When ocular motility is limited, I routinely do FDT (Forced Duction Test) and differential tonometry to know whether it is due to restrictive pathology or paralytic. I prefer FDT to FGT (force generation test).In restrictive pathology FDT is positive and you feel resistance when you try to move the globe in the direction of limitation. Elevation of intraocular pressure by more than 5 mm

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44 Surgical Atlas of Orbital Diseases

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Figures 2.45A to C: This girl presented with diplopia and restricted motility following trauma. Note subconjunctival hemorrhage in right eye. (A) FDT was positive. Clinical diagnosis was blowout fracture of floor of orbit with muscle entrapment. But CT scan of orbit revealed fracture roof of orbit with the bony spicule mechanically restricting the ocular motility. (B) Elevation restored to normal after removing the bony spicule (C)

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Figures 2.46A and B: Male 27 yrs. presented with proptosis right eye, diplopia on dextroversion and pain of 2 weeks duration. He had a closed head injury 3 months prior to the onset of proptosis. Notice mild congestion of proptosed right eye (A), better seen in the close-up of the eye (B)

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Figures 2.46C and D: Notice the congested blood vessels and the subtle under action of the lateral rectus of right eye (as evidenced by the over action of medial rectus of left eye). (C) The diagnosis of A-V Fistula is confirmed by the engorged Superior ophthalmic vein (D)

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Clinical Approach to Proptosis 45 from the base pressure when the patient attempts to move the globe in the direction of limitation of movement is significant. This occurs in restrictive pathology as the globe is compressed between the constricting muscle and its opponent which can not relax. Quantify the motility restriction, with PBCT (Prism Bar Cover Test) in different directions. This helps to know what is happening to the extraocular muscles, especially when you have to follow a patient for a long time like thyroid associated orbitopathy, and to decide if the patient benefits with prescription of prisms. Periorbital Changes: There are numerous periorbital changes which help us in understanding the pathology of the lesion and aid in clinical diagnosis. Some of these make the diagnosis very

A

obvious (like the lid retraction of thyroid associated orbitopathy, Salmon patch of lymphoma, temporal fullness of sphenoid ridge meningioma) while others throw light into the disease process and its activity. Temporal Fullness: This is very characteristic of sphenoid wing meningioma, especially of lateral part (Figure 2.36) Sphenoid wing meningioma of lateral half typically presents as proptosis with fullness of the temple, which often exhibits pulsations. Sphenoid wing meningioma of medial half presents as proptosis with restricted ocular motility (Figures 2.47A and B). Another very rare cause of temporal fullness is a dumbbell dermoid (Figures 2.48A and B), in which eyeball can move forwards on mastication due to contraction of temporalis muscle.

B

Figures 2.47A and B: Note proptosed left eye with restricted adduction. All movements other than abduction were restricted (A). CT scan of orbit revealed a heterogenous mass lesion with calcification, involving the temporal lobe, medial half of sphenoid wing. Note the hyperostosis of sphenoid bone (arrow), suggestive of meningioma (B)

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Figures 2.48A and B: Dumble dermoid with temporal fullness. Note the fullness of temple and the medial displacement of the globe (A). CT scan shows dumble dermoid, extending into the temporal region with a big defect (blue arrow) in the lateral wall of the orbit (B)

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46 Surgical Atlas of Orbital Diseases Lid changes: The important lid changes include lid retraction, lid lag, sinuous lid margin, tumors of the lid (primary malignant tumors of the lid with secondary orbital extension, primary vascular neoplasia with components of orbit and lid, or multiple tumors involving the lids and orbit like neurofibromatosis (Von Recklinghausen)). Lid Retraction is the most common lid change seen in thyroid associated orbitopathy. It can involve both upper and lower eyelids. It is measured by recording MRD values and subtracting the normal values (4 mm and 5 mm) from it. The lid retraction of TAO is due to over action of sympathetic system (Müller's muscle) and can also be due to hypotropia and limitation of elevation, so that with the attempt to move the globe, the upper lid goes up and lid retraction worsens. The lid retraction can be unilateral or bilateral. As per the NOSPECS classification, the lid retraction is classified as follows: mild if the lid margin is at limbus, moderate if up to

4 mm of sclera is seen and severe if > 4 mm of sclera is seen (Figures 2.49A to C). Usually in TAO, the contor of lid is altered in that the highest point of the upper lid is at the lateral part (lateral flare). Lid lag: Lid lag is the second most common lid change in thyroid orbitopathy. It can be unilateral or bilateral. Lag-ophthalmos is a very important finding one has to look for. It can lead to exposure keratopathy which in the early stages can present with pain and photophobia (Figures 2.50A and B). There may be associated defective vision. If the lagophthalmos is severe and prolonged, it can lead to frank corneal ulcer and even perforation. Hence it is very important to detect lag ophthalmos as early as possible and take remedial measures. To detect early lagophthalmos, look from below (Figures 2.51A and B). Severe lagophthalmos is usually seen in very gross proptosis which can be due to a very large benign tumor, but more often due to faster growing lesions like metastatic lesions (Figures 2.52A to C).

A

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C Figures 2.49A to C: Lid retraction three grades in thyroid associated orbitopathy. Mild-upper lid is at the limbus (A) Moderate 2 mm of scleral show (B) More than 4 mm of scleral show (C)

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Clinical Approach to Proptosis 47

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B Figures 2.50A and B: Lag-lag: Unilateral (left eye) and bilateral in thyroid associated orbitopathy. Note a small corneal lesion at 6 O’ clock position due to exposure

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B Figures 2.51A and B: Mild lagophthalmos may not be detected (A) unless examined from below (B)

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Figures 2.52A to C: Mild lagophthalmos in a case of TAO (A), severe lagophthalmos in metastatic orbital lesion from carcinoma of thyroid (B). The elderly female (a case of hydatid cyst of orbit) had anterior staphyloma in her left eye due to perforated corneal ulcer (C)

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48 Surgical Atlas of Orbital Diseases Mass: Look at the details of any visible mass (Figures 2.53A and B), like its surface, look for any vasculature, transillumination (Figure 2.30B), the margins, and posterior extent of the borders. Measure the size of the mass lesion. Some times there can be more than 1 mass lesion as in Von Recklinghausen (Figures 2.55A to C). The mass can

A

have an orbital and a lid component (Figures 2.54A and B). Conjunctival changes: The common conjunctival changes in proptosis are Salmon patch, chemosis, caput medusae, and subconjunctival hemorrhage. Occasionally tumor components may be visible.

B Figures 2.53A and B: Oncocytoma of lacrimal gland presenting as proptosis with a bleeding tumor involving the left upper lid (A). Proptosis in a patient of xeroderma pigmentosum due to orbital extension of squamous cell carcinoma (B)

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B Figures 2.54A and B: Capillary hemangioma involving the eyelid (A) and with its orbital component seen through the conjunctiva (B)

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Clinical Approach to Proptosis 49

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Figures 2.55A to C: Neurofibromatosis can present either as Von Recklinghausen disease with multiple tumors on the eyelid (A), or as plexiform neurofibroma (B) involving the lid and orbit as seen in the CT scan (C)

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Figures 2.56A to C: Malignant lid tumors, when neglected, can extend into orbit as seen with the Meibomian Carcinoma lower eyelid of right eye (A). Squamous cell carcinoma of right upper lid with orbital extension demonstrated in the CT scan (B and C)

Salmon patch is a pinkish, smooth mass, typically seen in the subconjunctival plane, either at the limbus, or fornix (Figures 2.57A to C). It is very typical of lymphoma. The lesion at the fornix, which is more commonly seen, is due to the extension of the tumor, as it moulds to the adjacent globe and extends anteriorly along the sub-tenon's plane and can extend upto the limbus. The isolated limbal mass is due to proliferation of the lymphocytes.

Caput medusae: Engorged blood vessels, typically around the limbus are seen in AV malformations and fistula (Figures 2.58A and B). The engorgement is usually due to increased venous pressure due to AV communication. It can be very subtle and can be easily over looked by the novice. In high flow fistula the caput medusae can be very significant and point towards the diagnosis. Some times it can be associated with chemosis of conjunctiva.

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50 Surgical Atlas of Orbital Diseases Chemosis: Chemosis is divided into 3 grades, grade 1 is when the chemosed conjunctiva covers up to half the lid margin, grade 2 when it covers the entire lid margin and grade 3 when it overhangs the lid margin (Figures 2.59A to C). Conjunctival

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chemosis can be because of active infections and inflammations like orbital cellulitis, orbital abscess, thyroid orbitopathy, or due to high flow arteriovenous communications, lymphangioma (Figures 2.60A to C)

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Figures 2.57A to C: Salmon patches of varying degrees presenting at the fornix and some extending up to limbus. Notice the typical color and also varying degrees of vasculature above them

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B Figures 2.58A and B: Caput medusae due to CCF (A). Note the grossly engorged superior ophthalmic vein (blue arrow) in the CT scan. Compare its size with the normal (red arrow)

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Figures 2.59A to C: Chemosis grade1 (covers upto half of lid margin) (A), grade 2 (covers upto entire length of lid margin) (B) and grade3 (overhangs the lid margin) (C)

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Clinical Approach to Proptosis 51

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Figures 2.60A to C: Grade 3 chemosis in orbital cellulitis (A), carotid-cavernous fistula (B) and a case of lymphangioma (C)

Subconjunctival hemorrhage is an important feature of lymphangioma, leukemia, trauma and bleeding diathesis. In a case of trauma, apart from associated ocular and systemic injuries, look for RAPD, retrobulbar hemorrhage and orbital fractures. If retrobulbar hemorrhage is present, do canthotomy and cantholysis (Figures 2.61A and B). Val salva: Increase in proptosis after Val salva maneuver is typically due to Orbital Varix (Figures 2.62A to C) Examination of nasal cavity and oral cavity is mandatory, especially in the presence of paranasal sinus involvement (Figures 2.63A and B). It is much easier to get a biopsy done from these lesions to know the nature of the lesion. Other important periocular changes involving cornea, ocular motility, pupil, visual acuity, fundus examination, color vision were already mentioned earlier.

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Palpation: Palpate the orbital margin, look for any palpable mass lesion and assess the orbital pressure by retropulsion. While palpating the orbit, ask the patient to look in the direction in which you are palpating, so that the orbital septum is relaxed. Use the pulp of your finger to palpate the orbit for any mass. If a mass is palpable, note its consistency, tenderness, extent, surface, reducibility, posterior extent. Assess the orbital tone by gently applying pressure over the closed eyelids and pushing the globe into the orbit. Compare the resistance offered by the proptosed eye with that of the normal. From this you can know if the orbital lesion is compressible or unyielding mass. Auscultation: In pulsatile proptosis, auscultate with the bell of a stethoscope for any bruit. It is typically heard in high flow fistula. Common causes of bilateral proptosis in children are congenital skeletal deformities, followed by lymphoma, leukemia and other lymphoproliferative

B

Figures 2.61A and B: Acute proptosis with subconjunctival hemorrhage, and surgical emphysema following trauma (A). Note that the conjunctiva became flat after 2 snips were made into it to permit the escape of air. Lateral canthotomy and cantholysis was performed to drain the retrobulbar hemorrhage (B)

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52 Surgical Atlas of Orbital Diseases

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Figures 2.62A to C: Female 21 years presented with proptosis of right eye since her childhood (A) She gives the history that the proptosis gets worse when ever she bends. Notice the increase in the amount of proptosis, fullness of upper lid, (B) and increase in the volume of the vascular component in a case of orbital varix (C)

A

B

Figures 2.63A and B: Nasal examination and oral examination can show the extension of lesions, especially if they arise from the Maxillary sinus. Note the visible mass in the left nostril (A) and the swelling of hard palate (B) which is the floor of maxillary sinus

disorders. Other important secondary orbital lesions that cause bilateral involvement, though not necessarily at the same time, include squamous cell carcinoma of the conjunctiva associated with xeroderma pigmentosum, and neglected retinoblastoma. The most common cause of unilateral proptosis in children is dermoid cyst, followed by hemangiomas. Rhabdomyosarcoma is the most common malignant lesion of pediatric age group. In adults, the most common cause of proptosis, either unilateral or bilateral is thyroid associated orbitopathy. Nonspecific orbital inflammation, granuloma (especially fungal granuloma involving frontoethmoid sinuses) and lymphomas are the other common bilateral lesions of orbit. However, the frequency of these conditions can change from place to place, depending upon the disease patterns of those areas. For example in India we see a lot of cases of

orbital myocysticercosis, which many of you in the western world might not have come across. Similarly, we rarely come across Wagener's granulomatosis, which is fairly common in the west. The disease presentations also can vary in different parts of the world. When compared to the west, we in South India very rarely come across severe thyroid orbitopathy. The incidence of infection due to tuberculosis is on the rise because of HIV. So is the case with lymphoma. Thus the spectrum of orbital diseases is varied in different parts of the world (Table 2.1). With a good knowledge of the disease patterns of the region and a good clinical work-up, a reasonable clinical diagnosis can be made most of the times. Advances in investigative modalities like imaging (CT, MRI) ,histopathology (FNAC, IHC, Squash) come to our aid to make a very accurate diagnosis, which goes a long way in managing the case.

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Clinical Approach to Proptosis 53 Table 2.1: Etiology of proptosis in comparision with western literature (Incidence in percentage) Lesion

Subrahmanyam

Rootman

Hendserson

Richard Dallow

1578

3919

1376

1825

Systemic Inflammatory Trauma Congenital Primary neoplasia Secondary neoplasia Metastatic lesions Vascular lesions Others

12.2% 37.1% 4.9% 2.8% 16.6% 1.9% 1.3% 3.0% 20.2%

51.7% 8.6% 4.9% 4.9% 14.3% 2.3% 1.5% 4.6% 7.2%

3.8% 4.4% — 2.8% 48.1% 19.5% 8.1% 4.7% 8.6%

32 % 13% 2% 2% 6% 2% 3% 6% 34%

This table shows considerable difference in the etiology of proptosis, when I compared my series with 3 large series reported from "clinical practice". I am not comparing with the reported series from pathology records, which have a strong bias towards neoplastic lesions. Hence knowledge of disease pattern for the area, from which the patient has come, is very important. In India we come across compressive optic neuropathy due to thyroid associated orbitopathy very rarely, whereas at vancouver I noticed that its prevalence among the locals of Indian origin is similar to the caucasians. It may be due to the gross difference in the climatic conditions or the life style and needs to be investigated.

Evaluation of a Case of Proptosis Name:

Age:

Occupation:

Address:

Sex:

Regn. Number Presenting complaints:

Treatment history Past History:(Circle the ones relevant) Thyroid disease HIV Any Medications / anticoagulants Tuberculosis Diabetes Any Surgeries Syphilis Hypertension Any known drug reactions : List them

Trauma history:

Family history: Personal history: General systemic examination:(circle the ones relevant) Pulse: Pallor Freckles BP: Icterus Neck Swellings RR: Lymphadenopathy Tremors Temperature: Caif au lait spots

Other history: (circle the one relevant): Thyroid disease (Wt loss/gain, tremors, neck swellings, palpitations, others), sinusitis, aggravation with . respiratory infections, others

CVS: Respiratory System: Abdominal Examination: ENT Examination:

History of present illness: Proptosis : Onset and Progression Defective vision: Double vision: Pain scoring:

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54 Surgical Atlas of Orbital Diseases Visual acuity Refraction Color Vision: Visual fields (confrontation) Proptosis Proper: Inspection: Compensatory head posture: Facial symmetry: Ocular symmetry: Any apparent mass description: Eyelids • • • • • • •

Position (MRD1 and MRD2): Fullness Contour Movements/Lid Lag Lagophthalmos Mass lesion (describe if present) Palpebral fissure height

Proptosis • Pulsations • MEASUREMENTS: • Hertel's (B.R……mm) • Horizontal • Vertical • Valsalva maneuver: • Periocular changes: Conjunctiva Cornea Anterior chamber Pupil Lens Fundus Ocular motility Cover test PBCT IOP Palpation: Bony regularity: Temperature : Tenderness: Crepitus: Description of Mass: Thrill/ Reducibility: Retropulsion:

RE

LE

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Clinical Approach to Proptosis 55 RE

LE

Auscultation: Fundus Examination: Impression: (D/D): Imaging: FNAC: Special Investigations: Apropriate Plan Of Management: Communication With The Patient: Procedure Performed: HPE Diagnosis: Postop Follow:

BIBLIOGRAPHY 1. Ben Simon GJ, Yoon MK, Atul J, Nakra T, McCann JD, Goldberg RA. Clinical manifestations of orbital mass lesions at the Jules Stein Eye Institute, 1999-2003. Ophthalmic Surg Lasers Imaging. 2006;37(1):25-32. 2. Dunsky IL. Normative data for Hertel exophthalmometry in normal adult black population. Optom.Vis Sci 1992;69:562-4. 3. Fledelius HC, Stubgaard M. Changes in eye position during growth and adult life as based on exophthalmometry, interpupillary distance, and orbital distance measure-ments.Acta Ophthalmologica. 1986;64:481-6. 4. Gladstone JP. An approach to the patient with painful ophthalmoplegia, with a focus on Tolosa-Hunt syndrome. Curr Pain Headache Rep. 2007;11(4):317-25. 5. Grove AS Jr. Modern examination methods of orbital disease. Orbital radionuclide examinations. Trans Am Acad Ophthalmol Otolaryngol. 1974;78(4):OP587-98. 6. Knudtzon K On exophthalmometry; the result of 724 measurements with Hertel’s exophthalmometer on normal adult individuals. Acta Psychiatr Neurol. 1949;24(3-4):523-37. 7. Kolasa P, Kaurzel Z. Post-traumatic pulsating exophthalmus coexisting with congenital carotid-cavernous fistula. Neurol Neurochir Pol. 2001;35 Suppl 5:58-63.

8. Krayenbühl HA. Unilateral exophthalmos. Clin Neurosurg. 1966;14:45-71. 9. La Mantia L, Erbetta A, Bussone G. Painful ophthalmoplegia: an unresolved clinical problem. Neurol Sci. 2005;26 Suppl 2:s79-82. 10. Malhotra R, Wormald PJ, Selva D. Bilateral dynamic proptosis due to frontoethmoidal sinus mucocele. Ophthal Plast Reconstr Surg. 2003;19(2):156-7. 11. Meyer DR. Compressive optic Ophthal-mology. 2007;114(1):199.

neuropathy.

12. Miller NR. Neuro-Ophthalmology of orbital tumors. Clin Neurosurg. 1985;32:459-73. 13. Rootman J. An approach to diagnosis of orbital disease. Can J Ophthalmol 1983;18:102-7. 14. Shields JA, Shields CL, Scartozzi R. Survey of 1264 patients with orbital tumors and simulating lesions: The 2002 Montgomery Lecture, part 1. Ophthalmology. 2004;111(5):997-1008. 15. Sugawara Y, Harii K, Hirabayashi S, Sakurai A, Sasaki T. A spheno-orbital encephalocele with unilateral exophthalmos. Ann Plast Surg. 1996;36(4):410-2. 16. Weinstein JM, Van Gilder JC, Thompson HS. Pupil cycle time in optic nerve compression. Am J Ophthalmol. 1980;89(2):263-7.

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56 Surgical Atlas of Orbital Diseases

3

CHAPTER

Imaging a Case of Proptosis: CT and MRI Subrahmanyam Mallajosyula, Ravi Varma

“Proptosis is a Pandora's box” was the most often quoted sentence in the past, and it was rightly so, since "surprises on the operation table" were quite frequent. The surprises could be in the form of pathology, localization and extent of the lesion. These problems were too frequent for the occasional orbital surgeon. Hence, many a time in the past it was the team effort—a team of neurosurgeon and ophthalmologist—that used to perform surgery on every case of proptosis. Advances in imaging techniques have changed the scenario completely. With the advent of CT scan and later MRI we know the exact location of the lesion, the nature of the lesion (tumor or a cyst, encapsulated/ infiltrating, highly vascular /less vascular, benign/malignant/ metastatic). We can also very accurately predict the histological nature of the lesion like glioma of the optic nerve/meningioma of the optic nerve sheath/ sphenoid ridge meningioma/ thyroid orbitopathy/ cavernous hemangioma/ lymphangioma/ myocysticercosis/ myositis/lymphoma, etc. Hence the surprises in proptosis were very rare now. After a thorough clinical evaluation and imaging with CT, most often we know the nature and location of the lesion, which enables us to manage proptosis in a systematic way. We strongly believe that the advances in imaging technologies have not only made the diagnosis and management of proptosis more scientific, they have revived interest in the subspecialty of orbital services. The globe, conjunctiva and optic disc are the orbital structures amenable for direct examination. Pathology involving the rest of the orbital structures

needs imaging studies for optimal evaluation. Several imaging techniques such as plain radiography, ultrasound, color Doppler, computed tomography (CT) and magnetic resonance imaging (MRI) are available for imaging orbital pathology. Each technique has its own advantages and disadvantages, and the information obtained from these studies is often complementary. Plain radiography has been the only investigation available for orbital imaging for most of the last century. Now, CT and MRI have largely replaced radiography, and skull X-rays are now performed only in selected cases of facial fractures and to screen for intraocular metallic foreign bodies before an MR examination. Sonography is used largely in the evaluation of intraocular structures. Large mass lesions in the orbit can also be visualized and characterized on sonography. Ultrasound combined with Duplex Doppler plays an important role in the noninvasive evaluation of vascular pathology involving the orbit. It can differentiate low flow lesions from high flow arteriovenous malformations. Though sonography is convenient and non-invasive, it suffers from limitations in the form of suboptimal visualization of the posteriorly placed structures, nonvisualization of intracranial pathology and need for expertise on part of the examiner. The most widely used radiological investigation for evaluation of the orbit is CT scan. It has the advantages of being widely available, fast, inexpensive and relatively easy to interpret. The technique is based on differential attenuation of X-rays by tissues, where denser tissues attenuate

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Imaging a Case of Proptosis: CT and MRI 57 more X-rays. Tissues can be characterized by their attenuation values (also called Hounsfield units (HU) - named after the inventor of CT). The retro-orbital fat imparts a natural contrast that aids in delineation of normal anatomy as well as pathology. On the Hounsfield unit scale, air is defined as –1000 HU, pure water as 0 HU, and dense cortical bone as +1000 HU. Retro-orbital fat usually measures –120 to –50 HU, cerebrospinal fluid measures 0 to +10 HU, extraocular muscles measure +40 to +50 HU and brain measures +35 to +45 HU. CT scans of the orbit are best obtained before and after intravenous administration of iodinated contrast medium. Thin sections (2-3 mm) are required to delineate the pathology and differentiate it from the normal orbital structures. Imaging in both axial and coronal planes may be required to optimally evaluate orbital pathology. The images can be viewed in different window settings to evaluate structures such as the soft tissues on bony structures. Modern day spiral CT and multidetector CT technology permit acquisition of the imaging data as a volume, within a few seconds so as to minimize movement artifacts. It is now possible to acquire images in several phases during and after intravenous administration of contrast medium, that may be instrumental in diagnosing vascular and other similar abnormalities of the orbit. In addition, post processing of spiral CT data yields extremely high resolution multiplanar reconstructions and 3dimensional images, without the need for additional exposure to radiation. CT scan images are taken with soft tissue windows (to study the details of the globe and the soft tissue lesion) and bone windows (give the details of the bone). Though CT scan is considered as the work-horse of orbital imaging, it suffers from several disadvantages such as relatively low tissue contrast as compared to MRI, and severe degradation of image quality by dental fillings. Dose of radiation to the lens, which is most sensitive organ in the body to radiation exposure, is a concern. Unlike radiography and CT scan, MR imaging does not use ionizing radiation. Image production in MRI is based on measurement of relaxation properties of protons after excitation with radiofrequency energy. By altering the parameters of data acquisition, we can obtain several image

contrasts such as T1 weighted images, T2 weighted images, proton density images, inversion recovery images, fat saturated images and gradient recalled images. MR imaging has several advantages over CT scan such as superior soft tissue contrast, direct multiplanar capability and excellent visualization of the optic nerve and intracranial pathology. Availability of higher field strength magnets and surface coils have significantly improved the image quality in orbital imaging. However, the long acquisition times that degrade the image quality due to movement artifacts and poor visualization of bony details and fractures still remain a significant impediment in the use of MRI for orbital evaluation. Furthermore, MRI is contraindicated in patients with cardiac pacemakers and defibrillators, neurostimulators, metallic foreign bodies in the eye, and other ferromagnetic implants within the body. Though, the signal pattern is highly dependant upon the specific imaging parameters used during acquisition, in general the signal patterns of various orbital and other related structures can be described as follows: T1 weighted Bone-cortex Bone-marrow Fat Muscle Aqueous/ Vitreous Lens Sclera CSF Blood vessels Air

T2 weighted Fat suppressed T1

No signal Bright Very bright Gray Dark

No signal Bright Bright Gray Bright

No signal Dark Very dark Gray Dark

Gray Gray Dark No signal No signal

Gray Gray Bright No signal No signal

Gray Gray Dark No signal No signal

Currently, the information obtained on imaging studies is complementary. The choice of imaging study should be based on the clinical presentation and the specific pathology being suspected. Further, the imaging techniques can be modified to specifically address the pathology in question. Discussion with the radiologist performing the imaging study goes a long way in ensuring optimal imaging of the orbit. While ordering for the imaging, the decision depends upon the nature of lesion. However I (MS), very rarely, if at all ask for a plain X-ray of orbit. The

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58 Surgical Atlas of Orbital Diseases information obtained from a plain X-ray is very limited and grossly inadequate to understand the nature of the lesion and plan management (Figure 3.1). My most preferred imaging modality is CT scan of Orbit, 2 mm slices of axial, coronal sections with sagittal reconstruction, with or without contrast. Contrast studies are ordered only when vascular or tumor pathology is suspected clinically. Otherwise plain study alone is ordered. For example, in thyroid orbitopathy, plain study is asked for, contrast study is not indicated. My preference for CT is because of its easy availability, cost to the patient, easy interpretation. I ask for an MRI only when I am dealing with a lesion of optic nerve or its sheath which account for 2 to 3% of all cases of proptosis. Evaluation of a CT scan of orbit: Though the soft tissue lesion attracts our attention, it is prudent to read the CT scan image in a systematic method, so that we don't miss any subtle changes, which provide very useful information. The level of the scan: The orbital scans are normally taken in a sequential order, from below upwards for axial scans and from anterior to posteriorly in coronal scans. To know the level of

the scan in the axial sections, remember that the medial walls of the orbit are parallel in the midorbit (Figure 3.2). If they were not parallel, the level of the picture is either lower or upper. Look at anterior and posterior parts of the picture. Anteriorly a flat nasal root and posteriorly the presence of brain tissue indicate that the scan was of upper part of the orbit (Figure 3.3). A prominent nose anteriorly, and the presence of oropharynx posteriorly indicate that the picture was of lower part of orbit (Figure 3.4). The important structures of orbit at various levels are shown in Figures 3.5, 3.6 and 3.7.

Figure 3.2: Mid level: Note the medial

Figure 3.1: X-ray picture of orbit of a patient of proptosis. The information obtained is very little. It is impossible to predict the nature of the lesion, and its exact location

Figure 3.3: Note the flat nasal root anteriorly

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Imaging a Case of Proptosis: CT and MRI 59

Figure 3.5: Axial CT scan at lower level of orbit showing important structures

Figure 3.4: Note the prominent nose anteriorly (blue arrow) and the or opharynx posteriorly (yellow arrow). Normal anatomy of orbit on CT

Figure 3.7: Axial CT scan at upper level of orbit showing important structures

Figure 3.6: Axial CT scan at mid level of orbit showing important structures

Common mistakes: We wish to discuss some of the common mistakes committed by the residents while interpreting the CT scan of the orbits. They are described with Figures 3.8 to 3.10.

How to read? • Anatomical location /level • Bony orbit

• • • •

Eye ball Extra ocular muscles Optic nerve Soft tissue Lesions — Borders / consistency • Hounsfield units • Contrast enhancement

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60 Surgical Atlas of Orbital Diseases

Bony Orbit Excavation or molding (Figure 3.11) erosion, (Figures 3.12 to 3.14) absence (Figure 3.15), hyperostosis (Figures 3.16A and B, 3.17) are the common changes in bony orbit.

Figure 3.8: Discontinuity of optic nerve: Due to the sinuous course of the optic nerve, axial sections often show an apparent discontinuity of the optic nerve (Yellow arrow), which was misinterpreted in cases of trauma as avulsion by the residents

Figure 3.11: Look at the bony “Excavation” with very smooth margins (yellow arrow) of the medial wall. This is due to increased orbital pressure exerted by the lesion which is in contact with the bone. This denotes a "chronic course” of a "benign lesion"

Figure 3.9: Cystic lesion abutting optic nerve: You can see a “cystic lesion” (yellow arrow) temporal to optic nerve (green arrow). Remember that the optic nerve does not emerge from the most posterior part of the globe. The apparent "cyst" is in fact the eyeball itself! This patient had glioma of the right optic nerve (orange arrow)

Figure 3.10: Enlarged Optic canal is how most of the residents interpreted A which is in fact enlarged inferior orbital fissure. Compare its size with normal inferior orbital fissure (B) Remember that optic canal is seen at the level of anterior clinoid processes and you will be able to see superior orbital fissure lateral to optic canal (Figure 3.6).

Figure 3.12: Bony erosion and hypertrophy in a case of fungal granuloma of orbit. Look at the “irregular” borders of the bone in comparison to the smooth borders of the excavation (Figure 3.11) Bony erosion is due to infiltration of the bone. It is commonly seen in malignant lesions or fungal infections

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Imaging a Case of Proptosis: CT and MRI 61

Figure 3.13: Sino-orbital mucormycosis. Note the involvement of sinus (yellow arrow), Orbit (Blue arrow) and brain (red arrow) with erosion of roof and medial walls of orbit

Figure 3.14: Fungal granuloma of the maxillary sinus with bony erosion (yellow arrows) Leading to extension into the ethmoid sinus, orbit and cheek

A

Figure 3.15: Bony dehiscence: Dumbbell Dermoid involving maxillary sinus and orbit. Note the absence of floor (yellow arrow). Note also the smooth excavation of the maxillary sinus and its expansion

B Figures 3.16A and B: Note the hypertrophy (hyperostosis) of the Sphenoid bone (yellow arrow) In a case of sphenoid ridge meningioma with extension to temporal region (green arrow). Note the intracranial component (red arrow)

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62 Surgical Atlas of Orbital Diseases

Bony Lesions Bony lesions include Osteoma (Figures 3.18A to C), fibrous dysplasia (Figure 3.19), Ossifying fibroma (Figures 3.20A and B). Subperiosteal hemorrhage (Figure 3.21) and subperiosteal abscess (Figures 3.22A to C) are other common lesion frontoethmoidal mucoceles (Figures 3.23A and B), Angiofibroma from sinuses (Figures 3.24A and B) are also seen fairly common. Fractures (Figures 3.25A and B, 3.26A and B) are quite frequent.

Figure 3.17: Hyperostosis and erosion of the sphenoid (yellow arrow) in a case of sphenoid ridge meningioma with intracranial component (red arrow), temporal fossa (green arrow) and orbital involvement (Blue arrow)

A

B

C

Figures 3.18A to C: Osteoma of the ethmoid bone involving the entire ethmoid, and leading to optic nerve compression. See the uniformly dense tumor with Hounsfield values similar to bone in both soft tissue windows (A) and bone window (B). The tumor was excised through a modified Lynch incision (C)

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Imaging a Case of Proptosis: CT and MRI 63

Figure 3.19: Fibrous dysplasia usually involves flat bones of face and hence orbital involvement is not uncommon. Imaging shows expansion of the bone with thinning of the overlying cortex. A ‘ground-glass’ appearance is common on CT

A

B Figures 3.20A and B: Ossifying fibroma Imaging shows a well circumscribed lesion eroding the bone with a sclerotic margin (yellow arrow) and foci of internal calcification (green arrow).

Figure 3.21: Sub-periosteal hemorrhage: Subperiosteal hemorrhage is less common than subperiosteal abscess. The periorbita can be seen clearly as a thickened membrane (yellow arrow). Note that the sinuses are clear in this film, unlike in subperiosteal abscess (red arrow) where the sinuses are involved (Figure 3.22)

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64 Surgical Atlas of Orbital Diseases

B

A

C

Figures 3.22A to C: Sub-periosteal abscess, infection extending from the Fronto-ethmoidal sinuses (red arrow). Note the congestion and edema of the lids and peri-orbital region, chemosis and congestion of the conjunctiva apart from the eccentric proptosis. Periorbita (yellow arrow) could be well made out

A

B

Figures 3.23A and B: Fronto-ethmoidal mucocele is a very common cause of eccentric proptosis in which the globe is pushed down and out. You can see a very gross eccentric proptosis of left eye with fullness in the superomedial quadrant. The CT Scan shows a grossly enlarged frontal sinus with mucosal thickness (yellow arrow)

A

B

Figures 3.24A and B: Angio fibroma arising from sinuses is not very rare. Note the huge tumor mass. Occupying the entire maxillary sinus, and distorting the orbital cavity. Its extension into the nose and the fullness of left cheek could be made-out. Also note the severe eccentric proptosis and corneal leucoma due to exposure

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Imaging a Case of Proptosis: CT and MRI 65

A

B

Figures 3.25A and B: Limitation of elevation of right eye with diplopia in upgaze following trauma while at play. Note the subconjunctival hemorrhage. The FDT was positive. Clinical diagnosis of fracture floor of orbit with entrapment of inferior Rectus was made. But the CT scan (B) of orbit showed fracture roof of the orbit with the displaced fragment impinging on the globe and mechanically restricting its movement (yellow arrow)

A

B

Figures 3.26A and B: Fracture floor of orbit can either involve a large area (Red arrow- A) and may show hemorrhage in the sinus or may be very tiny with trap-door mechanism and the typical "tear-drop" sign (yellow arrow- B). The rectus muscle may or may not be entrapped. Entrapment of the rectus with positive FDT and diplopia is one of the important indications for early surgery

Trauma: Trauma leading to orbital fractures (Figures 3.25A and B, 3.26A and B) is fairly common and its incidence is on the raise in view of increase in road traffic accidents and violence in the society. Apart from traumatic optic neuropathy, diplopia is another very important symptom. Persistent diplopia due to entrapment of a rectus muscle and positive FDT is one of the indications for surgery.

Eyeball: After examining the bony orbit, look at the globe and its relation to the lesion. The lesion may be soft and molding along the eyeball like a lymphoma (Figure 3.27) The globe is pushed ahead by the lesion and also can alter its shape depending on its consistency (Figures 3.28A and B). In Severe proptosis, tenting of posterior pole (Figure 3.29) can be noticed.

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66 Surgical Atlas of Orbital Diseases

Figure 3.27: Molding: Look at the lesion which is molding along the globe, without altering its curvature (yellow arrow). This is very typical of lesions which are soft like lymphoma. Molding can also be seen in fungal granuloma, adenoid cystic carcinoma of lacrimal gland

A

B

Figures 3.28A and B: Firmer lesions indent the globe and can induce refractive errors. Compression of the globe by cystic lesion (Hydatid Cyst figure A yellow Arrow) and compression of the globe by tumor (schwannoma figure B, yellow Arrow). The intraconal lesions cause hyperopic shift in the refraction, by virtue of flattening the globe, while the lesions in the peripheral space cause astigmatism

Figure 3.29: In severe proptosis, tenting of the globe with stretching of the optic nerve can occur. This also can contribute to defective vision. Look at the tenting of the posterior pole of the globe with loss of the normal curvature (yellow arrow)

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Imaging a Case of Proptosis: CT and MRI 67 After examining the contour of the eyeball, (Figures 3.30 to 3.32) look at the intraocular contents. Retinoblastoma and uveal melanoma (Figures 3.33A to D) are the most common intraocular tumors which can spread into the orbit and can cause secondary

A

proptosis. It is a routine practice to get a CT Scan of orbit in retinoblastoma. The tumor can be seen as an intraocular mass lesion with calcification. It can be confined to the globe or can extend into the orbit, optic nerve or brain.

B Figures 3.30A and B: Retinoblastoma; The typical amaurotic cat's eye reflex in the left eye( A) The intraocular mass with calcification (yellow arrow–B) is very typical of retinoblastoma. The sclera looks normal

A

B

Figures 3.31A and B: This child had a painful proptosis of right eye. Note the gross proptosis with mild congestion, increase in the corneal diameter, and amaurotic cat's eye reflex (A). The CT scan reveals intraocular masses with calcification (yellow arrow-B). in both the globes. Note the loss of integrity of the sclera with orbital extension (red arrow)

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68 Surgical Atlas of Orbital Diseases

A

C

B

Figures 3.32A to C: This child presented with recurrent mass after enucleation elsewhere for retinoblastoma. CT scan of the orbit reveals that the entire socket is filled with the mass (B) leading to expansion of the orbital walls (yellow arrow) and a very significant intracranial extension (red arrow-C)

A

C

B

D

Figures 3.33A to D: Uveal Melanoma is the most common intraocular malignancy in adults and it can extend into the orbits. Note the tumor which could be seen very clearly in external examination(A) and its brown color and the retinal vessels over it in the slit lamp examination (B). The tumor arising from the choroid is very well visualized in the CT scan in the coronal view (C) and its antero-posterior extent in the sagittal reconstruction (D). Note the absence of calcification. The sclera appears intact

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Imaging a Case of Proptosis: CT and MRI 69 Enlarged extraocular muscle: Enlarged extraocular muscle (EOM) is the most common finding we come across on imaging in proptosis, followed by mass (tumor). This is because of high prevalence of myocysticercosis as cysticercosis is endemic in our area. The important causes of enlarged extraocular muscle include thyroid

A

orbitopathy, (Figures 3.34A and 3.35A and B) myocysticercosis, (Figures 3.36A and B) idiopathic orbital inflammation (Figure 3.34B), rhabdomyosarcoma, lymphoma (Figures 3.39A to D), carotid cavernous fistula (Figures 3.37A and B to 3.38A and B), and metastasis. (Figures 3.40A to D).

B

Figures 3.34A and B: Thyroid associated orbitopathy (TAO) is the most common cause of enlarged extraocular muscle in most studies (A). Myositis due to idiopathic orbital inflammation (IOI) is also very common (B). Hence it is very important to differentiate these two conditions on imaging. The above pictures are very classical. In TAO, the tendon is spared and in myositis it is also involved. Contrast enhancement and only lateral rectus muscle involvement can occur in IOI but not in TAO. Lacrimal gland and even fat can be involved in IOI, but not in TAO. Most often in TAO, the CT scan of the orbit reveals bilateral enlargement of extraocular muscles

A

B

Figures 3.35A and B: In TAO, enlarged extraocular muscle can compress the optic nerve and cause loss of vision. Note the grossly enlarged recti muscles surrounding the optic nerve (red arrow) in the mid coronal sections of left orbit where as the optic nerve on the right side is free from compression. The posterior coronal sections reveal a severe compression of optic nerve on the left orbit. This patient had presented with a vision of 20/200 in left eye. Note that the floor and medial wall if orbit are reaching the posterior most part of the orbit, but not the zygoma. Hence in orbital decompression aimed to relieve optic nerve compression, I invariably include the medial wall or floor or both. I also excise orbital fat. Lateral wall decompression is more for cosmetic purpose

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70 Surgical Atlas of Orbital Diseases

B

A

Figures 3.36A and B: Myocysticercosis is a very frequent cause of enlargement of extraocular muscle in endemic areas. It can involve any of the extraocular muscles. The image is very characteristic in that a cystic lesion with a hyper-dense spot within (represents the scolex) is seen in an enlarged EOM. Commonly a single cyst is common. Rarely more than one cyst is encountered. I am yet to see a case where more than one muscle is affected. Intracranial cysticercosis can be rarely associated with orbital cysticercosis. In figure A notice the presence of two cysts in the superior oblique (SO) muscle. Compare the size of this grossly enlarged SO with the normal SO of left eye. Figure B shows the typical cyst with scolex involving the inferior rectus muscle

Another common cause of enlarged EOMs is Carotid- Cavernous Fistula (CCF). The incidence of it is on the raise due to increase in the incidence of trauma. CCF may be low flow or high flow in nature.

A

In view of the arteriovenous communication, enlarged EOMs and superior ophthalmic vein are seen in the CT scan

B

Figures 3.37A and B: CCF low flow fistula. Note the dilated Superior ophthalmic vein of the right eye (yellow arrow) and the EOMs. Note how big the lateral rectus (green arrow) is. Notice also the subtle enlargement of superior and inferior recti

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Imaging a Case of Proptosis: CT and MRI 71

A

B

Figures 3.38A and B: CCF High-flow fistula: Compare the grossly enlarged superior ophthalmic vein (yellow arrow) and the huge enlargement of the inferior rectus muscle( green arrow) with the previous picture. The high flow CCF usually follows trauma, and clinically characterized by pulsatile proptosis, caput medusae, chemosis, restricted ocular motility, gross retinal venous engorgement and secondary glaucoma

A

B

C

D

Figures 3.39A to D: Lymphoma: Lymphoma is characterized by a soft tissue lesion which typically molds around the globe (yellow arrow A,B and D). Salmon's patch is the typical presentation (C…green arrow). Some times enlarged EOMS can be seen in some sections of the CT scan (D…red arrow)

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72 Surgical Atlas of Orbital Diseases

A

C

B

D

Figures 3.40A to D: Metastatic lesion from breast: This patient presented with painful proptosis of left eye (A) of 1 month duration. She underwent mastectomy for carcinoma breast (B) 1 year back. CT scan of orbit shows enlarged lateral rectus muscle (C) associated with erosion of the lateral wall of the orbit (red arrow). Enlarged lateral rectus associated with bony erosion is a very common finding of CT scan of orbit in metastatic lesions, especially from breast and GIT. FNAC has confirmed the clinical diagnosis (D)

Soft-tissue Lesions The clinical spectrum of primary soft tissue lesions of the orbit is very varied. This is because of the various types of tissues that exist in the orbit. Though lesions of the optic nerve are not too common, their importance can not be over emphasized since they can lead to loss of vision The most frequent lesions of the optic nerve are glioma (Figures 3.41A and B, 3.42) and meningioma of the optic nerve sheath (Figures 3.44A and B). Optic

nerve can also be involved in idiopathic orbital inflammation (Figures 3.43A and B). It is very important to know whether the intraconal lesion is arising from the optic nerve (meningioma or glioma of optic nerve) or separate from it [cavernous hemangioma (Figures 3.45A to D), schwannoma (Figures 3.46A and B), etc]. Schwannoma (Figures 3.46A and B) is another tumor from nerve tissues, which mimics closely cavernous hemangioma (Figures 3.45A to D).

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Imaging a Case of Proptosis: CT and MRI 73

A

B

Figures 3.41A and B Optic Nerve Glioma: The axial section of CT scan shows spindle shaped tumor of the optic nerve with very distinct margins. The coronal section shows a large intraconal tumor. Note that the optic nerve is not seen separately( red arrow). The margins are well made-out. There is no evidence of calcification and there is no contrast enhancement. These are the typical feature on imaging of a optic nerve glioma, which help in distinguishing it from meningioma of optic nerve sheath

Figure 3.42: The axial CT scan of orbit shows a very large intraconal mass with distinct borders, and is similar to the Figure 3.39. It however shows distinctly cystic space (yellow arrow). It is a case of optic nerve glioma with cystic degeneration. Note the bony expansion, flattening of the posterior pole of the globe

A

B Figures 3.43A and B: Optic nerve swelling (yellow arrow) as a part of idiopathic orbital inflammation. Note the enlarged medial rectus (green arrow). Note that the margins are indistinct

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74 Surgical Atlas of Orbital Diseases

B

A

Figures 3.44A and B Meningioma of Optic Nerve Sheath: Meningioma of the optic nerve sheath is more common than the optic nerve glioma. On imaging it is characterized by indistinct margins (blue arrow), calcification, "tram- track" appearance and contrast enhancement (yellow arrow). These features help in distinguishing this lesion from glioma of optic nerve. In the picture B, note the fluffy and indistinct margins which differ from the smooth and distinct margins of glioma. The hypodense spot in the center of the lesion is the optic nerve. The hyperdense spots along the optic nerve are due to calcification. Compare these figures with those of glioma to understand the differences between these two lesions on imaging

B

A

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D

Figures 3.45A to D: Cavernous Hemangioma is characterized by hyperdense lesion with well defined margins and very minimal contrast enhancement (green arrow). It is more commonly located in the intraconal space and lateral to optic nerve (yellow arrow). In coronal section the mass can be seen separate from the optic nerve. Since most often the duration is very long (average 30 months), bony excavation of the orbit is very common. The excised tumor with very distinct borders is seen in C. Occasionally cavernous hemangioma may show calcified spots (D… yellow arrow) which represents the phleboliths. Note the subtle bony excavation of the lateral wall

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Imaging a Case of Proptosis: CT and MRI 75

B

A

Figures 3.46A and B: Schwannoma is another common benign tumor and is characterized by very distinct margins. It is often of long duration and hence shows bony expansion. Note the gross axial proptosis of the left eye (A) and the large tumor with very clear cut margins in the intra-conal space, and excavation of the lateral wall (yellow arrow). Schwannomas rarely enhance on contrast

Neurofibroma involving the orbit can be plexiform or as a part of Von Recklinghausen disease (Figure 3.47A to D).

A

C

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Figures 3.47A to D Neurofibroma: Plexiform neurofibroma involving the orbit (AandB). Note the typical shape of the lesion and the imaging showing the large tumor with indistinct margins involving the temple and the lid. Also note heterogenisity of the lesion. In Von Recklinghausen disease (C and D), the clinical picture with numerous nodular lesions is very distinctive. On imaging apart from proptosis and multiple tumors, dehiscence of the roof of orbit is common (C…yellow arrow), which results in pulsatile proptosis

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76 Surgical Atlas of Orbital Diseases Other vascular tumors like hemangioblastoma, hemangioendothelioma, hemangiopericytoma, angiosarcoma (Figures 3.48A to C), etc show on imaging a well defined mass lesion with contrast enhancement. Usually the clinical picture is characterized by shorter duration, mild pain/ discomfort. The final diagnosis is by histopathology. Lymphangioma usually shows intralesional hemorrhage (chocalate cyst).

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Lacrimal gland tumors: The lacrimal gland lesions are common.Most common benign lesion is pleomorphic adenoma (Figures 3.49A to C) and the most common malignancy is adenoid cystic carcinoma (Figures 3.50A and B) which has a very poor prognosis. Clinically pleomorphic adenoma has a long duration, and painless. Shorter duration, often less than 6 months, and mild discomfort are frequent in adenoid cystic carcinoma. Usually pleomorphic adenoma is firm in consistency, and is non-tender.

C

Figures 3.48A to C: Angiosarcoma. This patient presented with painful proptosis of 3 months duration. Note the contrast enhancing mass lesion with subtle indistinctness of posterior margins in the axial sections of the CT scan (C). The diagosis of angiosarcoma was confirmed on histopathology and immuni-histochemistry

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C

Figures 3.49A to C: Pleomorphic Adenoma: This patient presented with painless, gradually progressing proptosis of 18 months duration (A). Note that on imaging, the tumor is not molding, and is actually pushing the globe and indenting it. The downward and medial displacement of the globe is very well made-out in the CT scan (C)

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Imaging a Case of Proptosis: CT and MRI 77

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Figures 3.50A and B: Adenoid Cystic Carcinoma: The duration of proptosis in this patient is 5 months. She presented with eccentric proptosis and pain. Imaging shows a hyperdense mass lesion of lacrimal gland, molding to the globe. This is because of the softer consistency of the tumor, and can mimic lymphoma of lacrimal gland

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Figures 3.51A and B: Lymphoma of Lacrimal Gland: The clinical presentation is characterized by the presence of Salmon's patch in the superior fornix (A). The CT scan shows a well defined enlargement of the lacrimal gland which is molding to the globe. Also note subtle bony erosion of lateral wall of the orbit

Lymphoma of lacrimal gland: (Figures 3.51 A and B) is a soft lesion which moulds along the globe.

Rhabdomyosarcoma: (Figures 3.52A to D) is the most common mesenchymal and malignant tumor of children. Superior Rectus muscle is most commonly involved.

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78 Surgical Atlas of Orbital Diseases

A

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D

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Figures 3.52A to D: Rhabdomyosarcoma: This is a very common primary mesenchymal tumor of pediatric age group. The course can be rapid and some times very acute and mimic orbital cellulitis. The child (A) shows very gross proptosis with anterior staphyloma, while the child (C) shows less dramatic clinical picture. Note the fullness of the superior sulcus and eccentric proptosis with the eyeball pushed down in both the children. This is because the tumor involved superior rectus muscle (the most common EOM to be involved). Note the enlarged superior rectus with distinct margins (B and D). Note also the subtle bony erosion in B, while bone shows expansion, not erosion. Contrast enhancement is observed in these tumors. Biopsy confirms the diagnosis. The tumor responds very well to radiotherapy/chemotherapy

Cystic lesions of the orbit: A variety of cystic lesions occur in the orbit. They could be congenital cysts like dermoid (Figures 3.53A and B), congenital cystic eyeball, arachnoid cyst (Figures 3.56A and B),

A

parasitic cysts [cysticercosis (Figures 3.55A to C), hydatid cyst] (Figures 3.54A and B), cystic degenerations (glioma), mucocele (Figure 3.57), lymphangioma.

B

Figures 3.53A and B: Dermoid Cyst is the most common cystic lesion of the orbit in most of the series, other than in areas endemic for cysticercosis. Dermoids are usually congenital, preseptal and involve the temporal region in children, while they are acquired, postseptal, and nasal in location (A) in adults. On imaging they show a cystic lesion with well defined margins. Some times, a part of the lesion can be hyperdense, depending on its contents. Dumbbell -dermoids (B) are rare. Note the cystic lesion with an orbital component, and a temporal component (yellow arrows), and the gap in the lateral wall of the orbit. Note also the smooth excavation of the lateral wall (blue arrow)

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Imaging a Case of Proptosis: CT and MRI 79

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A

Figures 3.54A and B: Hydatid Cyst: Hydatid cyst is not very common. It usually occurs in younger individuals, and is often intraconal in location. Hence visual symptoms, axial proptosis of few months duration and optic disc edema in a patient having pets, especially dogs should arouse clinical suspicion. CT scan of the orbit shows a thin walled, isodense cystic lesion (red arrow). It can grow to a very large size and dwarf the globe (yellow arrow: A and B). Note the large, thin walled cyst, pushing the eyeball inferolaterally. Compare the size of the cyst with the eyeball. Also note the expansion of orbital walls. Because of such expansion of the orbital walls, and increased orbital volume, excision of the cyst leads to enophthalmos

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Figures 3.55A to C: Cysticercosis: Orbital Myocysticercosis is common in endemic areas. Usually solitary cyst is seen, involving a single extra ocular muscle. Restricted motility, diplopia associated with mild discomfort or pain are the important clinical features. Note the restricted elevation of right eye (A). CT scan of orbit shows enlarged inferior rectus muscle (green arrow) with a cyst. Note the hyperdense spot in it (yellow arrow) which represents the scolex

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Figures 3.56A and B: Arachnoid Cyst: Arachnoid cysts are very rare. Infants and young children with these congenital cysts present with painless proptosis. CT scan of the orbit reveals a cyst associated with hydrocephalus. The Hounsfield units of the cyst are usually 5 to 10 units, similar to CCF. Note the severe flattening of the posterior pole of the eyeball (yellow arrow) by the cyst (green arrow) Also note the bony expansion of lateral and medial walls. Note the gross hydrocephalus (B white arrow)

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80 Surgical Atlas of Orbital Diseases

Figure 3.57: Mucocele from frontoethmoidal sinus is a very common cause of eccentric proptosis. On imaging, the cystic enlargement of the sinuses (yellow arrow). Note that the anterior ethmoidal sinus is also involved

Secondary involvement of the orbit due to extension of lesions from the eye, sinuses (Figures

A

3.58A to C), lids (Figures 3.62A to C), lacrimal the (Figure 3.59) `brain is not uncommon.

B

C Figures 3.58A to C: Gross eccentric proptosis of left eye with perforated cornea (A).The CT scan of the orbit (B and C) shows the mucormycosis fungal granuloma involving the maxillary, ethmoid and frontal sinuses, bony erosion of floor, medial wall and roof with orbital and intracranial extension. Note how well the bony erosion is seen in the bone-window images (C)

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Imaging a Case of Proptosis: CT and MRI 81

Figure 3.59: Mucocele of the lacrimal sac is seen as a well demarcated lesion with anterior part of nasolacrimal duct CT scan is ordered only when a tumor is clinically suspected

Metastatic lesions: Metastatic lesions of the orbit usually present with pain and proptosis of a few days to few weeks duration. Most often they give history of previous surgery for a malignancy, but rarely the proptosis may be the presenting sign of a undetected primary elsewhere. CT imaging is very helpful in

A

that a mass lesion associated with bony erosion (Figures 3.60A and B) is very common. Enlarged lateral rectus muscle is common in metastatic lesions from breast or GIT. (Figures 3.61A to D). Metastasis can occur from other organs like prostate thyroid, parotids, etc (Figure 3.63).

B Figures 3.60A and B: Metastatic neuroblastoma in a male 15 years presented with painful proptosis of 1 month of right eye associated with restricted ocular motility. Note the soft tissue lesion of orbit with bony erosion of roof and lateral walls

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82 Surgical Atlas of Orbital Diseases

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D

Figures 3.61A to D: Metastatic Carcinoma from right breast in a female of 45 years who presented with mild proptosis and periocular swelling of left eye since 1 month (A). She underwent radical mastectomy 2 years back (B). CT scan of orbits reveals enlarged lateral rectus (green arrow) with bony destruction of lateral wall and the roof (C and D orange arrow). FNAC was positive for ductal carcinoma of breast. She was referred to oncologist for further management

Secondary orbital involvement can occur due to extension of tumors of eyelids (Figures 3.62A to C), intraocular tumors or intracranial tumors.

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Figures 3.62A to C: Secondary Involvemet From Lid Tumor in this elderly male who presented with a very large Basal cell carcinoma of the upper lid of 6 years duration. Note the huge upper lid tumor (A), and its orbital extension (B and C) evident in both coronal and sagittal sections

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Imaging a Case of Proptosis: CT and MRI 83

Figure 3.63: Adenoid cystic carcinoma of parotid gland with intracranial (red arrow) and orbital extension (green arrow). Also note the bony erosion

Contrast enhancement: Whenever I suspect a vascular lesion or a tumor, I ask for contrast studies. Contrast enhancement shows that the tumor is very vascular. It differentiates a cavernous hemangioma

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from a hemangioendothelioma. When the tumors are very strongly enhance on contrast as in the Figures 3.64A to D, I will keep a unit of blood reserved at the time of surgery.

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D Figures 3.64A to D: Contrast enhancement of hemangioendothelioma (B when compared to A) and Lymphoma (D and C)

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84 Surgical Atlas of Orbital Diseases 3-D reconstruction of orbit: Orbital 3-D reconstruction is ordered in severe orbital

fractures and also in congenital bone defects (Figures 3.65A and B).

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A

Figures 3.65A and B: 3-D reconstruction of the orbit and periorbital regions is very useful in cases of trauma. Note how well the bony defect in the frontal bone and the cleft palate are seen

BIBLIOGRAPHY 1. Abou-Rayyah Y, Rose GE, Konrad H, Chawla SJ, Moseley IF. Clinical, radiological and pathological examination of periocular dermoid cysts. Eye. 2002;16(5):507-12. 2. Arger PH The radiologic evaluation of unilateral proptosis. CRC Crit Rev Clin Radiol Nucl Med. 1974;5(1):43-67. 3. Char DH, Sobel D, Kelly WM, Kjos BO, Norman D Magnetic resonance scanning in orbital tumor diagnosis. Ophthalmology. 1985;92(10):1305-10. 4. Forbes G. Radiologic evaluation of orbital tumors. Clin Neurosurg. 1985;32:474-513. 5. Forbes GS, Sheedy PF 2nd, Waller RR. Orbital tumors evaluated by computed tomography. Radiology. 1980;136(1):101-11. 6. Hilal SK, Trokel SL. Computerized tomography of the orbit using thin sections. Semin Roentgenol. 1977;12(2):137-47. 7. Klöppel R, Schulz HG, Ballin R, Lommatzsch P Value of computed tomography in orbital tumors. Radiol Diagn (Berl). 1985;26(6):745-52. 8. Kokemueller H, Zizelmann C, Tavassol F, Paling T, Gellrich NC A comprehensive approach to objective quantification of orbital dimensions. J Oral Maxillofac Surg. 2008;66(2): 401-7.

9. Trokel SL, Hilal SK Recognition and differential diagnosis of enlarged extraocular muscles in computed tomography. Am J Ophthalmol. 1979;87(4):503-12. 10. Urbanik A, Chojnacka I, Herman-Sucharska I, Jele?ska I, Brzozowska-Czarnek A. Computed tomography images of selected retrobulbar orbit tumors. Przegl Lek. 2000;57(6):327-9. 11. Vignaud J, Hasso AN, Lasjaunias P, Clay C Orbital vascular anatomy and embryology. Radiology. 1974 ;111(3):617-26. 12. Wackenheim A, van Damme W, Kosmann P, Bittighoffer B. Computed tomography in ophthalmology. Density changes with orbital lesions. Neuroradiology. 1977;13(3):135-8. 13. Wei R, Cai J, Wang H, Tao X, Zhu H, Zhou H. Analysis of MRI and CT manifestations of paranasal sinuses and orbitocranial disorders with secondary exophthalmos. Zhonghua Yan Ke Za Zhi. 1999;35(3):200-2, 12. 14. Wende S, Aulich A, Nover A, Lanksch W, Kazner E, Steinhoff H, Meese W, Lange S, Grumme T. Computed tomography or orbital lesions. A cooperative study of 210 cases. Neuroradiology. 1977;13(3):123-34. 15. Wende S, Kazner E, Grumme T The diagnostic value of computed tomography in orbital diseases. A cooperative study of 520 cases. Neurosurg Rev. 1980;3(1):43-9.

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Role of Cytology in Orbital Lesions 85

4

CHAPTER

Role of Cytology in Orbital Lesions Geeta K Vemuganti, Anirban Bhaduri

Cytologic diagnosis of lesions is one of the mainstay of diagnosis for lesions affecting various organs of the body. This speciality has made tremendous impact even in ophthalmic pathology, an upcoming subspeciality, which has been referred by Fredrick Jakobiec as “queen of subspecialities of Ophthlmology.” The techniques of obtaining specimens of ocular cytology have undergone much refinement and with increasing reports of larger series of cases, the learning curve has been crossed by many experts. In general, the techniques used for orbital lesions include: Fine needle aspiration of palpebral lesions, squash and imprint cytology of fresh tissue for rapid intraoperative diagnosis.1-6 The cytology specimens could also be subjected to recently emerging molecular tools of diagnosis,7-8 thus aiding in patient management.

Fine Needle Aspiration/Sampling Technique The procedure for FNAC at any site in the body is the same. The procedure can be done by the pathologist or the surgeon, directly under vision or under guidance of CT. Usually there is no need of any local anesthesia injection by this technique, except in children where a general anesthesia may be preferred. The technique of obtaining the material could be a “sampling technique” wherein a 23 /24 guage needle is introduced into the lesion and pushed in various directions within the lesion and gently withdrawn.9 By the capillary action, the cells are drawn into the needle. This technique has also been applied to ocular cytology.3,6 The advantages of this technique are: It is easy, simple, less hemorrhagic and causes less apprehension to the

patient. The cytologic material obtained by this method is usually adequate with minimum amount of hemorrhage. A few disadvantages of fine needle aspiration in general include inadequate material, hemorrhagic aspirate, bleeding at the site, especially for highly vascular lesions. At our center, FNAC has yielded diagnostic yield in more than 90% of cases. Tijl et al.10 reported the diagnostic yield of orbital FNAB combined with clinical and radiological features as 80%. Very rare potential complications include globe penetration, retrobulbar hemorrhage, diplopia and ptosis.11

Intraoperative-operative Diagnosis by Squash and Imprint Cytology There is often a need for a reliable intraoperative diagnosis, specifically in situations where a definitive preoperative tissue diagnosis is lacking and where the tissue diagnosis is likely to influence the immediate surgical management.12 The established methods of intraoperative diagnosis include frozen section diagnosis and intraoperative cytologic diagnosis, each of which has its own merits and demerits.

Squash or Imprint Cytology The utility of imprint cytology in eye lesions was first described by Fuchs for uveal melanoma in 1988.13 Imprint cytology of fresh unfixed tissue specimens and squash cytology of central nervous system lesions have been extensively used in the last few decades, but rarely applied to ophthalmic pathology practice. The main indications for rapid intraoperative diagnosis are: a) Infiltrative lesions, suspected

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86 Surgical Atlas of Orbital Diseases malignant lesions or deeply located lesions where a preoperative tissue diagnosis is not available; b) Where there is a discrepancy between a preoperative clinical diagnosis and the intraoperative findings and: c) Unusual clinical presentations with diagnostic dilemma. Fresh unfixed tissue obtained at the time of diagnostic or excision biopsy can be used for making squash preparation and impressions on glass slides. The procedure for making squash or imprint smears is usually based on the size, shape, consistency and crushable properties of the tissue submitted. For soft, easy to spread tissues, tiny bit of the fresh tissue is placed between two clean glass slides and gently drawn apart. For large firm specimens, the imprint smears are prepared by touching the freshly cut surface of the lesion with clean slides, avoiding smearing to retain cell morphology. If the surface is covered with blood or exudates, more number of smears is made, after gently wiping the surface clean. It is preferable to make a minimum of three slides for each case. It is advisable to preserve extra unstained smears for further tests like immunocytochemsitry or for any molecular studies in future. These smears are either alcohol fixed for rapid hematoxylin and eosin staining, or fixed by air-drying for Diff-quick staining. A provisional cytologic diagnosis can be made by the pathologist based on the cellular and architectural features seen on smears prepared from either or both techniques.

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CASE ILLUSTRATIONS Case 1 A 23-year-old female presented with swelling of left upper lid for 2 months with occasional diplopia. Examination showed non-axial proptosis with downward displacement of globe and fullness of the upper lid sulcus (Figure 4.1A). There was a firm, slightly tender nodular mass in the superolateral orbit. There was limited abduction of the left eye. CT scan shows a soft tissue mass in superolateral orbit, which could not be seen separate from the lateral rectus muscle (Figure 4.1B). Incision biopsy was done. Squash and imprint preparation shows a polymorphous population of inflammatory cells consisting of lymphocytes, neutrophils and also a few eosinophils (Figure 4.1C), suggestive of a nonspecific

C Figures 4.1A to C: (A) Photograph shows the downward displacement of the left eye and fullness of the upper lid sulcus due to the presence of a lacrimal gland mass (B) CT scan (coronal cut) shows a well-circumscribed, homogenous, extraconal, soft tissue mass in the superolateral quadrant of mid-orbit, which is not seen separate from the lateral rectus muscle. There is no orbital fat streaking. (C) The squash smear shows a polymorphic population of cells consisting of lymphocytes, neutrophils, eosinophils. (Giemsa, x 500)

orbital inflammatory disease. Permanent sections confirmed the diagnosis.

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Role of Cytology in Orbital Lesions 87

Case 2

Case 3

A 12-year-old female presented with swelling of left upper lid and cheek for 1 month. There was no history of cough, hemoptysis, fever or weight loss. There was a soft, non-tender swelling in the superior orbit (Figure 4.2A). Preauriclar and submandibular lymph nodes of the left side were enlarged, firm, nontender and mobile. CT scan showed a soft tissue mass in superior orbit with bone destruction. Clinically, a provisional diagnosis of adenoid cystic carcinoma of the lacrimal gland was made. FNAC from the orbital lesion and preauricular lymph node showed lymphocytic infiltrates, epithelioid granulomas and few giant cells (Figure 4.2B). Extensive necrosis was seen in some areas. Acid-fast bacilli staining of the smears showed a few bacilli.

A 70-year-old female presented with history of painful vision loss with drooping of the upper eyelid of the right eye for 1 month. Examination showed complete external ophthalmoplegia with ptosis, dilated and fixed pupil and optic atrophy (Figure 4.3A). She was a diabetic on irregular treatment. CT scan showed a large, elongated cystic mass in posterior, superomedial orbit straddling intraconal and extraconal spaces and lying close to the optic nerve (Figure 4.3B). Differential diagnoses were: a parasitic cyst or cystic degeneration in a solid tumor. Preoperatively, an abscess was found, the wall was excised after draining the contents. Squash and imprint preparation of the walls showed chronic inflammatory cells and fibrosis with fungal filaments (Figure 4.3C). Cultures of the specimen confirmed aspergillosis.

Case 4 An 18-month-old male child presented with right sided non-axial proptosis and temporal fossa fullness

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A

B Figures 4.2A and B: (A) Photograph shows narrowed palpebral aperture and fullness of left superior sulcus due to a soft mass in the superior orbit. (B) The squash smear show cluster of epitheloid cells with slipper shaped nuclei (hematoxylon and eosin, × 200)

B

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88 Surgical Atlas of Orbital Diseases

A C Figures 4.3A to C: (A) Photograph shows complete ptosis on right side. The patient had total external ophthalmoplegia with optic atrophy in the right eye. (B) Axial CT scan shows an elongated mass with cystic changes in the superomedial quadrant of the right orbit close to the optic nerve and soft tissue mass or mucosal thickening in adjoining ethmoidal sinuses. (C) the smears shows prominent branching and septate fungal filaments with inflammatory cells in the background (Giemsa, × 1000)

of 15 days duration (Figure 4.4A). Peripheral blood smear was normal. CT scan showed an extraconal, well circumscribed soft tissue mass temporally with destruction of the greater wing of sphenoid and extending into temporal fossa and middle cranial fossa (Figure 4.4B). Incision biopsy was done from the temporal fossa. The squash and imprint smears showed cellular infiltrates consisting of neutrophils, eosinophils, plasma cells and giant cells. In addition there were large cells with moderate amount of cytoplasm and large vesicular nucleus with prominent grooves and folds (Figure 4.4C). Frequent multinucleated giant cells are seen. Tingible body macrophages and histiocytes with phagocytic activity were noted. Based on the above features on squash and imprint smears, a provisional diagnosis of eosinophilic granuloma was given which was confirmed on histology sections. Intralesional triamcinolone was injected and the lesion resolved completely. Systemic workup included USG abdomen, skeletal survey, bone marrow biopsy and liver function tests were normal. Bone remodeling was complete at 6 months.

Case 5 A 45-year-old male presented with progressive protrusion of the left eye for 3 months (Figure 4.5A)

B

C Figures 4.4A to C: (A) Photograph shows swelling and erythema of the right upper lid and fullness of the temporal fossa. (B) CT scan (axial cut) shows a homogenous, well-circumscribed, low intensity soft tissue mass with destruction of the greater wing of sphenoid. The mass involves extraconal lateral orbit, temporal fossa and middle cranial fossa. (C) The squash smears are cellular with polymorphic population of cells with multinucleated giant cells, neutrophils, eosinophils and histiocytes with prominent nuclear grooves and cleaves (hematoxylin and eosin, × 1000).

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Role of Cytology in Orbital Lesions 89 and non-tender, smooth, firm subcutaneous nodules in left breast, upper abdomen and thigh for 2 months. CT scan showed a well defined soft tissue intraconal and extraconal mass in the left orbit (Figure 4.5B). He had a previous episode of proptosis on the same side ten years ago, which was diagnosed histopathologically as reactive lymphoid hyperplasia previously, which responded to local radiotherapy after initial poor response to systemic steroids. Incision biopsy performed on the orbital mass two months ago showed sinus histiocytosis. FNAC was performed from the breast and abdominal nodule at this presentation and smears showed high cellularity with a polymorphous population of cells consisting of lymphocytes, plasma cells, histiocytes and neutrophils. A large number of histiocytic giant cells with abundant cytoplasm extended by the presence of intracytoplasmic plasma cells, lymphocytes and occasional neutrophils were present(Figure 4.5C). This feature, described as emperipolesis, confirmed that the subcutaneous

C Figures 4.5A to C: (A) Photograph shows severe chemosis and non-axial proptosis of the left eye. (B). CT scan (axial cut) shows a well defined irregular hypodense soft tissue mass in intraconal and extraconal spaces in the left orbit. (C) The smears show large multinucleated giant cells with multiple lymphocytes, plasma cells within the cytoplasm of the histiocytes-called as emperipolesis, a pathognomonic feature of Rosai-Dorfman disease ( Giemsa, × 500)

nodules were a part of multifocal Rosai-Dorfman Disease.

Case 6

A

A 48-year-old male presented with a swelling on the surface of the right eye with prominence of that eye for 6 months. Examination showed a pink fleshy conjunctival mass with intrinsic vessels (Figure 4.6A), axial proptosis and limitation of eye movements. CT scan showed a soft tissue mass moulding around the globe and extending into the conjunctiva (Figure 4.6B). Incision biopsy was done from the conjunctiva. Squash and imprint smears were cellular with a monomorphic population of lymphoid cells. These cells showed scant rim of cytoplasm and a round nucleus with moderately coarse chromatin and small nucleoli (Figure 4.6C). The biopsy confirmed a diffuse large cell lymphoma, immuno-phenotyping suggested a B cell lymphoma. Systemic workup did not show any other focus of lymphoma. He was treated with local external beam radiotherapy and was doing well on last follow-up.

Case 7

B

A 65-year-old male presented with non-axial proptosis of right eye with inferomedial displacement for 15 days. He also complained of low back ache for 3 months. Firm, non-tender, fixed nodular mass in

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90 Surgical Atlas of Orbital Diseases superolateral orbit with erosion of superior orbital rim and temporal fullness (Figure 4.7A). CT scan showed a soft tissue mass with bone destruction (Figure 4.7B). X-ray skull shows multiple punched out lesions (Figure 4.7C), skeletal survey shows multiple osteolytic lesions in long bones and spine. Cytology of the mass showed cellular smears with a large number of plasma cells in varying stages of differentiation (Figure 4.7D). Many bi-tri and multinucleated forms were noted. The Giemsa A

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C Figures 4.6A to C: (A) A closeup photograph of the right eye shows the pink fleshy conjunctival mass in the lateral bulbar conjunctiva with intrinsic vessels which seem to appear from and disappear into the tumor. This mass represents the conjunctival component of the lymphoma. There is also axial proptosis and limitation of eye movements. (B) CT scan shows a soft tissue mass classically moulding around the globe and extending into the conjunctiva. (C) The smears are cellular with monomorphic cells, with scant rim of cytoplasm and a moderately coarse chromatin patter. Note the absence of cohesion, cytoplasm and any differentiation. (Giemsa, x 500)

C

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Role of Cytology in Orbital Lesions 91

A D Figures 4.7A to D: (A) Photograph shows fullness of upper lid sulcus and downward displacement of the right eye by an orbital mass.(B). CT scan (axial cut) shows a well-circumscribed, homogenous, lobulated soft tissue mass in the orbit with bone destruction and extension into the temporal fossa and middle cranial fossa. (C). Radiograph of skull (lateral view) shows multiple, punched out osteolytic lesions in the skull bones. (D) The cytology smear shows multiple plasma cells with bi and multinucleated forms. The amphophilic cytoplasm and the perinuclear halo is classical of plasma cell lineage ( Giemsa, × 500).

stained smears are helpful in identifying the amphophilic cytoplasm and a perinuclear halo. The Bone marrow shows 15-25% plasma cells in various stages of differentiation.

Case 8 A 17-year-old male presented with gradual inferior displacement of right eye for three months and blurred vision following a trivial sports injury. Firm, fixed, slightly tender superomedial orbital mass observed. CT scan shows a soft tissue mass with patchy enhancement in the superior orbit (Figure 4.8A.) Peripheral blood smear was normal. Incision biopsy revealed a greenish yellow (chloroma) solid tumor in the peripheral surgical space. Squash imprint preparations of tissues showed a monomorphic round cell tumor. However the characteristic feature is the pale staining nucleus, irregular nuclear membrane, and pinkish cytoplasm. The Giemsa stained smears are of great importance in confirming the blast like morphology with cytoplasmic granules and sometime Auer rods can also be identified (Figures 4.8B and C), which confirms the diagnosis of leukemic deposits. Bone marrow biopsy was normal in this patient, at the time of orbital presentation suggesting the extramedullary leukemic deposits.

B

C Figures 4.8A to C: (A) CT scan orbit (coronal cut) shows a wellcircumscribed, homogenous, hypodense soft tissue mass, with no surrounding bony changes, displacing the orbital contents inferiorly. (B) The smears show large cells with scant to moderate amount of blue cytoplasm with lack of cohesion. (C) The higher magnification shows the cytoplasmic Auer rod, confirming the diagnosis of granulocytic sarcoma (Giemsa, × 1000)

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Case 9 A 6-year-old female presented with an increasing swelling below the left eye for 1 month, On examination, there was a firm mass in inferonasal orbit with non axial proptosis and superolateral displacement of the eye. CT scan showed a, wellcircumscribed, smooth soft tissue extraconal mass displacing adjacent medial rectus muscle and globe. The tumor was completely excised. Squash and imprint smears were highly cellular with irregular cells with marked pleomorphism of nuclei and cytoplasm. The cells with pink tongue like projections, spindle cells are helpful in suggesting Rhabdomyoblastic differentiation (Figures 4.9A to D). The cells contained moderate to abundant amount of cytoplasm staining deep blue and containing occasional small glycogen vacuoles. Few cells show

ill-defined relatively dense cytoplasmic inclusion. Tumor cells are found singly, but loose clusters also seen. Based on degree of myogenic differentiation Akhtar et al. 14 divided Rhabdomyoblasts into 3 categories—Early Rhabdomyoblasts are round undifferentiated cells with high nuclear: Cytoplasmic ratio. Intermediate Rhabdomyoblasts have relatively abundant pale staining cytoplasm and one or more irregular nuclei with occasional nucleoli. Late Rhabdomyoblasts contain abundant cytoplasm staining grayish blue and opaque. These cells vary from round to markedly elongated. Some cells show localized inclusion like grayish blue area within cytoplasm. The differential diagnosis include: Retinoblastoma, Burkitt's lymphoma, metastatic Neuroblastoma, PNET/Ewing's sarcoma and myeloid leukemia can all present in orbit.

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Figures 4.9A to D: (A) Photograph shows a swelling in the inferomedial orbit visible as a swelling in the lower lid and upward displacement of the globe. (B) CT scan, axial view shows a hyperdense mass in the medial orbit, indenting the globe. The mass is homogenous. (C) The cytology smears show clumps of cells with pink cytoplasm. Note the presence of cells with tongue like projections a one pole of the cells. (Hematoxylin and eosin × 500) (D) The sections from the tumor show necrotic areas and large pink cells, characteristic of rhabdomyoblasts

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Role of Cytology in Orbital Lesions 93

Case 10 A 22-year-old female presented with pain, swelling and decreased vision in right eye for 4 months. Examination showed proptosis of right eye with neurotrophic keratopathy and total ophthalmoplegia. (Figure 4.10A) There was a firm, nodular, slightly tender, immobile mass in superolateral orbit whose posterior extent could not be palpated. MRI scans showed a soft tissue mass in superolateral orbit extending towards the orbital apex and another soft tissue mass in cavernous sinus region on the same side (Figure 4.10B). Differential diagnosis included nonspecific orbital inflammation with Tolosa-Hunt syndrome and Adenoid cystic carcinoma with intracranial extension. Incision biopsy was done from the lacrimal gland. Squash and imprint preparation showed the characteristic features of basaloid cells

in sheets, finger like processes, lacy pattern and the classical 3-dimensional cell balls (Figures 4.10C and D) with minimal nuclear pleomorphism. Permanent sections showed cribriform pattern typical of adenoid cystic carcinoma. A final diagnosis of adenoid cystic carcinoma of the lacrimal gland was made with perineural spread and intracranial extension into the cavernous sinus giving rise to orbital apex syndrome.

Case 11 A 67-year-old female noticed a swelling in the upper lid of the left eye 7 months ago, which enlarged to involve the lower lid 3 months later and appearance of multiple neck masses. On presentation she had complete ptosis, there was a firm, immobile, nontender nodular mass in the anterior orbit palpable through both lids. The mass was not fixed to

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Figures 4.10A to D: (A) Photograph shows ptosis and proptosis on right side with corneal haze due to neurotrophic keratitis. The patient also has total ophthalmoplegia and loss of sensation in distribution of Ist division of trigeminal nerve (B) T2 weighted MRI scan shows a hypointense mass in the right lacrimal fossa extending posteriorly. Note the normal lacrimal gland in the left orbit. (C) The smears show classical magenta pink rounded acellular matrix with nuclei wrapped around it. (3 D cell balls) (Giemsa, × 500). (D) The sections from the tumor shows characteristic cribriform pattern of basaloid cells (× 100 Hematoxylin and eosin)

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94 Surgical Atlas of Orbital Diseases overlying skin. The lids were immobile and the conjunctival surface could not be inspected. The mass extended beyond the lower orbital rim, onto the cheek. There was little blood on the lid margin and eyelashes were intact (Figure 4.11A). She also had an enlarged preauricular lymph node on the same side and bilateral neck glands in both anterior and posterior triangles. There were no other systemic abnormalities. CT scan showed a soft tissue mass involving the lids and anterior orbit (Figure 4.11B). FNAC was done from the orbital mass and lymph nodes which showed large pleomorphic epithelial cells, seen in tissue fragments, small clumps as well as singly. The cells show abundant vacuolated cytoplasm and vesicular nucleus (Figure 4.11C). In one of the smears, an Oil Red O staining was done which showed prominent bright orange red globules within the cytoplasm of tumor cells (Figure 4.11D),

thus confirming the diagnosis of sebaceous gland carcinoma, with orbital invasion and regional lymph node metastasis.

Case 12 A 45-year old male presented with a left upper lid droop and diplopia for 1 month. There was a firm, non-tender mass in lacrimal gland region, inferomedial displacement of the globe and limitation of elevation and abduction (Figure 4.12A). Fundus examination showed choroidal folds and globe indentation. CT scan showed a soft tissue mass in lacrimal gland region with erosion of orbital roof and intracranial extension. Orbital part of the tumor was removed through a lateral orbitotomy. The Squash and imprint smears showed clumps of large polygonal cells with abundant cytoplasm, cytoplasmic pink inclusions, a large vesicular nucleus with

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Figures 4.11A to D: (A) Photograph shows irregular swelling of both eyelids of left eye causing mechanical ptosis and extending beyond the inferior orbital rim. The lids could not be opened or everted for further examination. (B) CT scan shows a soft tissue mass involving the anterior orbit. (C) The smears show large epithelial cells with classical vacuolated cytoplasm. Note the presence of lymphocytes in the background (hematoxylin and eosin, x 500) (D) The Oil Red O staining fo the cytology smear shows the presence of bright orange fat deposits (Oil Red O staining, x 500)

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Role of Cytology in Orbital Lesions 95 prominent inclusion like nucleolus.(Figure 4.12B). Histology confirmed a metastatic carcinoma with a trabecular pattern, intracytoplasmic ropy secretions, osseous metaplasia and occasional cell with bile plugs (Figure 4.12C). Subsequently, the abdominal ultrasound scan showed a well-defined mass in the right lobe of liver. Metastatic deposits from breast, lung, thyroid, hepatocellular carcinoma may be seen in the orbit and show similar appearance as seen in the primary location. This is one of the important indication of fine needle aspiration cytology of orbital lesions which influences the surgical management of the case. In summary, it is important for the ophthalmologists to apply cytology to ocular lesions

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which can result in early diagnosis with less invasive techniques, sometimes obviating the need for a surgery.

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Figures 4.12A to C: (A) Photograph shows non-axial proptosis with downward displacement of the left globe and fullness of the upper lid sulcus. (B) The cytology smears show large plemorphic epithelial looking cells with vesicular nucleus and prominent nucleoli. Note the cytoplasmic eosinophilic inclusions, which was reported as metastatic carcinoma. (C) The permanent sections revealed the trabecular pattern osseous metaplasia and bile plugs in few cells (hematoxylin and eosin, × 500)

REFERENCES 1. Orbit FNAC Burnier Jr. MN, Correia CP, McCartney ACE. Tumors of eye and ocular adnexae. In Fletcher CDM (Ed.): Diagnostic Histopathology of Tumors, (2nd ed) Churchill Livingstone, Edinberg. 2000;2:1757. 2. Zajdela A, de Maublanc MA, Schlienger P, Haye C. Cytologic diagnosis of orbital and periorbital palpable tumors using fine needle sampling without aspiration. Diagn Cytopathol 1986;2:17-20. 3. Vemuganti GK, Naik MN, Honavar SG, Sekhar GC. Rapid intraoperative diagnosis of tumors of the eye and orbit by squash and imprint cytology. Ophthalmology. 2004;111:1009-15. 4. Wolska-Szmidt E, Jakubowska A, Krzystolik K, Chosia M. Fine needle aspiration biopsy and molecular analysis in

5. 6.

7.

8.

differential diagnosis of lymphoproliferative diseases of the orbit and eye adnexa. Pol J Pathol. 2004;55:51-7. Schyberg E. Fine needle biopsy of orbital tumors. Acta Ophthalmol 1975;125:11-2. Font RL, Laucirica R, Ramzy I. Cytologic evaluation of tumors of the orbit and ocular adenexa: an analysis of 84 cases studied by the "squash technique". Diagn Cytopathol 1994;10:135-42. Wolska-Szmidt E, Masiuk M, Krzystolik K, Chosia M. Flow cytometry in the diagnosis of lymphoproliferative lesions of the orbit and eye adnexa in fine needle aspiration biopsy. Pol J Pathol. 2003;54:253-9. Coupland SE, Heimann H, Bechrakis NE. Primary intraocular lymphoma: a review of the clinical, histopathological and molecular biological features. Graefes Arch Clin Exp Ophthalmol. 2004;242:901-13.

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96 Surgical Atlas of Orbital Diseases 9. Koss LG. Diagnostic cytology and its histopatholoigc bases, (4th ed) Philadephia: JB Lippincott,1992;1-11. 10. Tijl JW, Koornneef L. Fine needle aspiration biopsy in orbital tumours.Br J Ophthalmol. 1991;75:491-2. 11. Liu D. Complications of fine needle aspiration of the orbit. Ophthalmology 1985;92:1768-71. 12. Ackermann LV, Ramirez GA. Indications for and limitations of frozen section diagnosis: A review of 1269 consecutive frozen section diagnosis. Br J Surg 1959;46:336-50.

13. Fuchs U. Smear and imprint technique in malignant lesions of the eye. Acta Ophthalmol ( Copenh) 1988;66:445-9. 14. Akhtar M, Ali MA, Bakry M, Huq M, Sackey K. Fine Needle aspiration Biopsy diagnosis of Rhabdomyosarcoma: Cytologic, histologic and ultra-structural correlations. Diagn Cytopathol 1992;8: 465-74.

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Pathology of the Orbital Diseases 97

5

CHAPTER

Pathology of the Orbital Diseases KS Ratnakar

INTRODUCTION It is a common practice to include tumor-like conditions such as inflammatory lesions, cysts and lymphoid hyperplasias which mimic true neoplasms in their clinical manifestations under the general term ‘orbital tumors’. Approximately, 70% of all orbital tumors originate from the orbital tissues while 30% invade the orbit from adjacent structures or metastasize from distant primary foci. The relative incidence of the pathological lesions causing proptosis reflects the bias of the reporting discipline, namely, surgical, ophthalmological or radiological.

CLASSIFICATION The classification essentially follows the tradition of considering the lesions as congenital/developmental, inflammatory and neoplastic. An outline of the classification is shown in Table 1. Table 1: Classification of orbital lesions 1. Developmental a.

Sphenoid wing dysplasia

b.

c. d.

Neurofibromatosis i. Optic glioma ii. Optic meningioma iii. Optic schwannoma Elephantiasis neuromatosa Plexiform neurofibroma

e. f. g. h.

Fibrous dysplasia Dermoid/epidermoid Hamartoma Meningioencephalocoele.

2. Inflammatory a. Orbital cellulites b. Idiopathic Orbital inflammation c. Abscess d. Parasitic-cysticercus', hydatid e. Granuloma: sarcoid, wegener's, tuberculosis, fungal. 3. Traumatic a. Penetrating injury b. Foreign body c. Hematoma 4. Neoplastic Lesions may be of benign or malignant type. Benign lesions a. Conal and intraconal i. Hemangioma – Cavernous (adult) Capillary (child) ii. Lymphangioma iii. Hemangiopericytoma iv. Neurofibroma v. Meningioma Malignant lesions a. Conal and intraconal i. Lymphoma ii. Metastasis iii. Rhabdomyosarcoma iv. Neuroblastoma v. Ewing's sarcoma b. Optic nerve/sheath i. Optic glioma ii. Hemangiopericytoma iii. Leukemia 5. Vascular a. AV malformation b. Carotid–cavernous fistula c. Venous varix d. Superior ophthalmic vein thrombosis 6. Endocrine–Grave's ophthalmopathy 7. Miscellaneous–Mucoceles

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DIAGNOSIS OF ORBITAL TUMORS Space occupying lesions, involving the orbit, produce symptoms and signs by compression, infiltration and/or infarction of orbital structures. In the final analysis, the clinical presentation will result from displacement and /or dysfunction of the globe, optic nerve, oculomotor nerve and blood vessels. Proptosis: It is a common feature of all orbital tumors, though its degree may vary. Those within the muscular cone, (e.g. optic glioma, hemangioma and meningioma) usually produce axial proptosis, but those outside the muscle cone, (e.g.dermoid, lacrimal gland tumor, neuroma) tend to push the eye opposite to that of the lesion, to cause eccentric proptosis. Optic neuropathy: Optic nerve involvement may result in: (a) Progressive visual loss associated with edema of disc. In many patients, however, visual loss may be minimal and of delayed onset. In such cases, testing for color vision may reveal subtle defects even when acuity is nearly normal, (b) Unilateral transient visual loss which may occur in certain positions of gaze and clears when the direction is changed, and (c) A specific triad may develop in chronic compression of the optic nerve, namely loss of vision, swelling of disc which resolves into optic atrophy and appearance of optociliary shunt veins, (e.g. in spheno-orbital meningiomas). Oculomotor paresis: The tumors located in orbital apex may involve oculomotor nerves in early stage even before causing proptosis. Some tumors may involve one or two muscles, till late in the disease. Diplopia produced by orbital masses may be neurogenic or myogenic and rarely it may be a combination of both. The mechanical restoration of ocular mobility can be confirmed by performing certain tests. Forced duction or traction test and intraocular pressure increase on looking in the direction of gaze limitation are the tests of choice for this purpose. Pain: Most of the lesions are painless. Generally, pain is more frequent with malignant tumors. Lesions that involve cavernous sinuses and paranasal sinuses are usually painful. Pupillary abnormalities: These abnormalities can occur depending upon the involvement of the parasympathetic or sympathetic nerves, but this is

often marked by involvement of oculomotor nerve palsy.

DEVELOPMENTAL LESIONS Sphenoid Wing Dysplasia Pulsating exophthalmos results due to defective development of sphenoid wing and roof of the orbit. About one-half of the cases are associated with neurofibromatosis. The lesion is evident in the early years of life, starting with ptosis and thickening of the upper eyelid when it is associated with neurofibromatosis, followed by protrusion of the eye ball. The vision is spared till late. There is usually a bulge in the temporal fossa. X-rays of the skull reveal the underlying bony deficits such as elevation of sphenoid ridge, enlargment of the orbit, absence of temporal line of the sphenoid wing, giving the appearance of an " empty orbit".

Neurofibromatosis Neurofibromatosis involves the orbit either alone or in association with sphenoid dysphasia and produces a variety of orbital lesions in bony and soft tissue segments. The peripheral nerve sheath tumors are histologically characterized by the presence of pallisading spindle cells with micro or macrocystic changes. The collagen content may vary. In plexiform type, neurites in bundles may be seen within the stroma. Case report: A 15-year-old boy presented with proptosis and diminished vision in the left eye. Intraorbital soft tissue lesion was excised. He had in addition bilateral acoustic neurinomas and intradural neurofibroma in the lumbar region. The histological study revealed wavy spindle cells embedded in lose, fibrillary matrix. At places whirling bundles of neuritis are noticed (Figure 5.1).

Meningioencephalocoeles Sinonasal encephaloceles involve the medial orbital wall and encroach into the orbit causing hypertelorism. The tissues include either meninges alone or brain parenchyma. Case report: A 12-year-female has reported with proptosis, predominantly affecting the upper medial

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Pathology of the Orbital Diseases 99 They are invariably benign and can be excised with ease. Case report: A 6-year-male child admitted with bulging at the root of nose along with fullness in the upper medial compartment of right eye. Radiological investigations showed a mass involving the anterior base of the frontal bone with orbital extension of the lesion (Figure 5.3).

Hamartoma

Figure 5.1: Spindle cells showing wavy pattern in a case of Neurofibroma. H and E × 400

aspect of left eye. Radiological examination including CT scan and MRI revealed a cranial defect in the upper orbital wall with small cerebral parenchyma protrusion into sac covered by meninges. At operation, CSF leakage was there and the sac contents along with contents are excised (Figure 5.2).

Hamartoma is a type of congenital lesion composed of tissues normal to the location. One type of tissue may be predominantly seen such as vascular tissue.

Fibrous Dysplasia

These tumors result from embryonic ectodermal sequestration. These are rare, found inside the orbit, and most of them are located in the periorbital region. Dermoids and epidermoids have the same histological features as seen elsewhere in the body.

Fibrous dysplasia is a non-neoplastic disease of the bone that affects children and young adults. Craniofacial fibrous dysplasia is a benign condition forming about 3% of all bone tumors. Dysplasia of frontal, sphenoid, ethmoid, zygomatic and maxillary bones may involve the orbit causing visual symptoms. The symptoms may range from progressive visual loss to proptosis or orbital distortion. The lesion is usually painless. On plain Xrays the lesion appears as ground glass, milky and sclerotic and in most cases some areas of radiolucency may be observed. Histological changes are consistent with an arrest in the development of immature woven bone into more mature lamellar bone

Figure 5.2: Histological section showing Neuroglial elements in the wall of the Meningioencephalocoele. H and E × 400

Figure 5.3: Section showing cyst lined by epidermis with dermal appendages from a case of dermoid cyst H and E × 400

Dermoid and Epidermoids

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100 Surgical Atlas of Orbital Diseases

Figure 5.4: Woven bone without osteoblast rimming in a case of fibrous dysplasia H and E × 400

embedded in cellular fibrous stroma. No osteoblastic rimming is seen around osseous islands. The woven bone may appear as' Chinese figures' often described to indicate their focal irregular pattern. The stroma tends to be fibrous in older individuals (Figure 5.4).

INFLAMMATORY LESIONS Orbital Cellulitis Orbital cellulites can remain as a diffuse inflammation or progress to loculation to form an abscess. There is usually a profound disturbance of oculomotor functions, pain and constitutional symptoms. Occasionally, the abscess may become chronic and the lesion may manifest like any begin space occupying lesion. Pathology reveals diffuse suppurative inflammation composed of neutrophils.

Idiopathic Orbital Inflammation Idiopathic orbital inflammation is the most common cause of an intraorbital mass. The condition is usually diagnosed clinically with classic symptoms of painful proptosis and restriction of extraocular muscle often unilateral. It is a challenging mimicker of an intraorbital neoplasm and is often a diagnosis by exclusion of other surgically remediable entities. The exact etiology is not known. Autoimmune response to antigen has been implicated. Perhaps the condition is multifactorial. IOI is often a diagnosis of exclusion of specific

etiologies. Every attempt has to be made to find the cause prior to labeling the condition as idiopathic orbital inflammation. Lymphoid lesions of the orbit continue to pose problems to the ophthalmologists and pathologists as well. Despite increase in understanding and improved methodology the diagnosis is often difficult and intrigues the treating surgeon. The inflammatory lymphoid lesions which are also called orbital idiopathic inflammation add further confusion to the understanding of the disease process. Arnold and Becker (1972) and Hochheim (1900) first attempted to classify the lymphoid lesions of the orbit into four categories namely benign lymphoma, follicular lymphoma, lymphosarcoma and lymphatic leukemia. In these groups, the first two possibly belong to the lesions of inflammatory origin. Blodi and Gass(1968) carefully analysed the non-neoplastic lymphoid lesions and divided them into follicular, diffuse lymphocytic and benign lymphocytic hyper plastic varieties. Morgan and Harry (1978) studied 98 cases of lymphoid lesions and considered large number of cases that remained confined to the orbit are of inflammatory nature. These lesions have been termed by them as lymphocytic tumors of indeterminate nature. These classifications in the present era of molecular markers, are unscientific and redundant. Clinically, the inflammatory benign lymphoid lesions present with exophthalmos with conjunctival edema, pain/inflammatory signs and occasionally with palpable mass. There is no specific age or sex predilection but appears to affect young adults predominantly. Right eye appears to be involved more frequently than the left and small percentage is found to occur bilaterally. The duration of a symptomatology varies from less than a month to over several years. These tend to heal or improve over a period of time with steroids and/or antibiotics. Small but significant percentage of lesions may focally recur, but do not necessarily indicate malignancy in all the cases. However, careful clinical follow-up is advised and a thorough systemic examination. It is imperative to rule out any disseminating malignant lymphoid lesions. Morgan and Harry (1978) observed in over 25% of cases are associated lymphoid malignancy elsewhere in the

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Pathology of the Orbital Diseases 101 body. This finding necessitates cautious thinking of any lymphoid lesion in general. However, Knowle et al. (1979) using molecular techniques, made useful and important observation that benign reactive lymphoid hyperplasias are immunologically polyclonal whereas malignant lymphomas are monoclonal in nature. The lymphoid lesions apparently looking benign may present primarily in the orbit with its systemic involvement being observed at a later date. The pathobiology cannot be clearly defined in the early stages without application of molecular markers which are rather defined. Hence the wisdom lies in careful clinical and histological interpretation (Figure 5.5).

Orbital Infections Aspergillosis Aspergillosis is a fungal disease caused by aspergillus fumigatus, the most common causative species. Aspergillosis involving the orbit and cranial content is rare. Paranasal sinus aspergillus infections are classified as non-invasive and invasive types. The invasive or fulminant type of aspergillosis occurs primarily in immunologically compromised individuals. The clinical manifestations include a rapidly progressive gangrenous necrosis of the mucoperiosteum, with destruction of nasal bones of the paranasal sinuses and orbital wall. There may be intracranial invasion. The orbital extension

Figure 5.5: Lymphoid aggregates with prominent germinal centers from a case of pseudo-lymphoma H and E × 400

is reflected by soft tissue densities extending from the sinuses especially the ethmoids into the orbital cavity. Some degree of bone destruction is identified in combination with the fungal infiltrate. The histology in acute form shows necrotising vasculitis associated with soft tissue invasion by fungal filaments. In chronic form, there is granulomatous reaction and fibrosis (Figure 5.6) Intra and extracellular aspergillus is demonstrable by fungus stains such as PAS and silver methanamine (Figure 5.7). Case report: A 60-year diabetic reported with painless proptosis of right eye of several weeks duration. There was restriction of ocular movements in all directions. Orbital exploration revealed a tough soft tissue lesion which resisted complete dissection and excision. Histology showed many granulomas containg foreign body giant cells containing fungal filaments characteristic of aspergillous species.

Tuberculosis Orbital tuberculosis is rare. In endemic areas, tuberculous infection should be considered as an important entity in the aetiology of orbital inflammation. Orbitomorphologically the lesion shows caseating coalescing granulomas. There may be variable degree of fibrosis. Similar histoplasmosis may be encountered in brucellosis, histoplasmosis, sarcoidosis. Hence thorough clinical examination

Figure 5.6: Histological section showing foreign body type of giant cells in a granualomatous lesion. From case of Aspergilloma, orbit H and E × 600

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102 Surgical Atlas of Orbital Diseases

Figure 5.7: Fungal filaments of Aspergillus species. Silver methanamine × 400 Figure 5.8: Cross-section of cysticercus. H and E × 400

serological and/or cutaneous tests should be conducted for final diagnosis.

Cysticercosis Cysticercosis of the orbit is very rare in the western literature, but Jacobiece and Font (1986) nath et al. (1977) documented nearly 24 cases in the literature. Sarada et al. (1981) found one case in the orbit among 50 cases of cysticercosis of the CNS. But in endemic areas orbital myocysticercosis is fairly common. They are usually managed with oral Albendazole and prednisolone. Only anteriorly located cysticercosis is excised and is available for histopathology (Figure 5.8). However, Murthy et al. (1990) reported on the use of ultrasound in preoperative diagnosis. Cysticercus cellulosae, histologically, when sectioned shows fibrocellular, reaction with palisading histiocytes around the parasite. The worm may get calcified in later stages. The cellular infiltrate may be dominated by eosinophils.

Neoplastic Lesions Classification of orbital tumors is given in Table 2 Benign tumors.

Table 2: Classification of orbital tumors Primary I. Mesenchymal a. Vascular: hemangioma, lymphangioma, hemangiopericytoma, hamangioendothelioma, Angiosarcoma, Kaposi’s sarcoma, b. Lipoma, liposarcoma c. Fibrous histiocytoma, d. Fibrosarcoma e. Rhabdomyosarcoma f. Leiomyoma, sarcoma g. Chondroma, chondrosarcoma h. Giant cell tumor, osteosarcoma. II. Neural Glioma, neurilemmoma, neurofibroma, amputation neuroma. III. Hemopoietic. Lymphoma, leukemia, myeloma. IV. Lacrimal gland Plemorphic adenoma, adenoid cystic carcinoma. V. Miscellaneous Meningioma, nonchromaffin paraganglioma, alveolar soft part sarcoma, malignant melanoma. Secondary I. Direct extension a. Intraocular tumors retinoblastoma, melanoma b. Eyelid tumors basal, squamous, sebaceous carcinoma, melanoma c. Conjunctival squamous carcinoma, melanoma d. Metastatic in children – neuroblastoma In adults–Lung Ca, Breast Ca.

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BENIGN TUMORS Cavernous Hemangioma The most common benign intraorbital tumor in the young and middle aged is cavernous hemangioma that produces painless proptosis. It is common in females and may enlarge during pregnancy. Despite the mention that cavernous hemangioma is the most common benign intraorbital tumor, there have not been many reports of a good series of this condition. Harris and Jacobiec (1979) could find 66 cases recorded during a 40 year period from three centers dealing specially with ophthalmic problems. Maroon and Kenerdell (1979) found five hemangiomas among 18 intraorbital tumors subjected to microsurgical treatment. The same authors reported 17 hemangiomas among 300 cases of orbital tumors seen from 1975 to 1982. Nath et al. (1977) found 12 cases among 120 cases of primary orbital tumors. Being a benign slow growing tumor, it causes progressive often painless proptosis. As the lesion is usually situated behind the globe within the muscle cone, it produces axial proptosis. Despite the prominent protrusion of the eyeball, vision is preserved and movements of the eye are spared till late in the course. About half of the patients in the series of Harris and Jacobiec(10979) had blurred vision and only three of the 66 had diplopia. Neither bruit nor pulsations were present in any of their cases. Microscopically, the lesion is composed of dilated vascular channels. Some of the channels may contain prominent smooth muscles when the lesions are called venous hemangiomas (Figure 5.9).

Capillary Hemangioma

Figure 5.9: Histological section from a case of cavernous hemangioma, showing widely spaced vascular channels rimed by fibromuscular tissue. H and E × 400

Figure 5.10: Polypoidal tissue composed of proliferating vascular channels embedded in a inflammatory and edematous stroma from a case of capillary hemangioma, pyogenic type. H and E × 400

Capillary hemangioma is a benign tumor that manifests usually in first five years of life and tends to regress thereafter. It is generally single, bright red and smooth lesion. Histologically, capillaries of small size are closely packed with no smooth muscle in between (Figure 5.10).

children and adolescents. Spontaneous hemorrhage into the cyst leads to abrupt proptosis and formation of “chocolate cysts”. Lymphangioma occurs in extraconal location and presents with pain and proptosis. Infiltration of muscles and nerves of orbit also occurs in this condition (Figure 5.11) .

Lymphangioma

Hemangiopericytoma

There are no lymphatics in the orbit. However, lymphangiomas do occur here and account for 0.5 to 3% of intraorbital tumors. It is commonly seen in

It is a rare lesion occurs in the fourth decade of life. It may be malignant in about 12% cases. The lesion consists of increased number of thin-walled vascular

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104 Surgical Atlas of Orbital Diseases

Figure 5.11: Section showing dilated vascular channels with lymphoid aggregates in the mural compartment from a case of Lymphagioma. H and E × 400

channels with perivascular massing of pericytes separated by tumor cells in a network of extracellular material (Figure 5.12).

Meningiomas Orbital meningiomas are of three types based on their origin— 1. Intracranial meningiomas which secondarily invade the orbit are the commonest. Meningiomas of the middle cranial fossa especially those of sphenoid ridge are notorious to cause proptosis. Meningiomas of the anterior cranial fossa may invade the orbital roof. In

Figure 5.13: Section shows oval to polyhedral cells with vesicular nuclei and lightly acidophilic cytoplasm arranged in nests and whorls. There are scattered psammoma bodies, from a case of meningioma. H and E × 400

addition ectopic meningiomas arising from, for example, the frontal sinus may encroach into the orbit. 2. Primary intraorbital meningiomas are rare tumors which arise from the optic nerve. This is the most difficult one to treat. The differentiation between optic nerve glioma and meningioma is an important clinical problem. 3. Those which have no apparent connection with the optic nerve are occasionally found in the orbit. These probably arise from ectopic arachnoid cell nests in the orbit. Intraorbital meningiomas are more common in women than men. The average age of onset is about 30 years. About 40% occur below 20 years. The main clinical features are loss of vision and progressive exopthalmos. Neurofibromatosis may be associated within 16% of cases (Figure 5.13).

Malignant Tumors Rhabdomyosarcoma

Figure 5.12: Histological section showing thin vascular channels with oval to spindle cells oriented externally from a case of Hemangiopericytoma. H and E × 600

Rhabdomyosarcoma is the most common malignant mesenchymal orbital neoplasm and malignant orbital tumor in children. The average age of onset is six years and the lesion is rare after 25 years. The onset is rapid and the progression simulates cellulitis. Histologically, the tumors are of

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Pathology of the Orbital Diseases 105 three types (i) embryonal (ii) adult pleomorphic and (iii) alveolar. Of these three types embryonal rhabdomyosarcoma is the most common type of malignant tumor. Structurally, tumors are composed of round, oval or stellate rhabdomyoblasts with mitosis in loose syncitium. Cells contain dense eosinophilic cytoplasm with striations. Mortality rate is 40.6%. Prognosis is best in adult pleomorphic type and it is worst in alveolar type (Figures 5.14 and 5.15).

Adenoid Cystic Carcinoma It occurs in adults of either sex in the fourth decade of life. The lesion is relentlessly progressive, invading the adjacent tissues with characteristic tendency to spread along perineural lymphatics (Figure 5.16).

Lymphoma Lymphomas in adults are encountered in 10% biopsies of orbital tumors. They are usually found in the anterior orbit. About 25% of lymphoma presenting as NSOID may evolve into lymphomas. They are essentially B cell lymphomas. Orbital lymphomas may be the first manifestation of systemic lymphomas (Figure 5.17).

Histiocytoma Malignant fibrous histiocytomas are rare, and more lethal lesions of orbit. These are common in older age group. The origin of the tumor is from the histiocytes,and the neoplastic histiocytes are bizarre with areas of hemorrhage,necrosis and frequent mitosis. Focal or diffuse storiform pattern characteristic of histiocytomas is discernible .

Metastasis Figure 5.14: Cellular lesion showing spindle cells with acidophilic cytoplasm arranged in alveolar pattern in a case of alveolar rhabdomyosarcoma H and E × 400

It accounts for 5% of orbital tumors. In children, neuroblastoma, ewing's and leukemia and in adults, carcinoma of bronchus and breast are the common

Figure 5.15: Cellular lesion showing spindle cells with acidophilic cytoplasm arranged in alveolar pattern in a case of alveolar rhabdomyosarcoma. H and E × 600

Figure 5.16: Large cellular islands of basaloid cells with focal cystic pattern embedded in fibro vascular tissue. From a case of adenoid cystic carcinoma of the orbit. H and E × 400

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106 Surgical Atlas of Orbital Diseases bilateral, though the patient may present when it is still confined to one orbit. Microscopic examination shows edema, lymphocytic infiltration sometimes forming follicles.

MISCELLANEOUS Mucoceles Mucoceles of the paranasal sinuses may require neurosurgical attention when they involve the orbit and/or cranial contents. The most frequent site is the frontal sinus.

Figure 5.17: Cellular lesion composed of monomorphic round cells with dark nuclei and scant cytoplasm seen in nodular aggregates form a case of orbital lymphoma. H and E × 400

primary sites. In about 50% of the cases the primary remains unknown. Of all metastases 30% are orbital and 70% ocular. The main symptoms are pain, proptosis and limitation of extraocular movements. Undifferentiated carcinomas arising in a paranasal sinus account for 4-9% of unilateral exophthalmos. The carcinoma of ethmoid most commonly involves the orbit. Pain, proptosis, restricted ocular motility, with ptosis occurring rapidly is a feature of malignancy of the orbit.

Optic Glioma Optic glioma are primary tumors of the optic nerve and/or chiasma. They are usually low grade pilocytic astrocytomas which may appear fibroblastic due to invasion of the leptomeninges (desmoplastic) or gelatinous with oligodendroglial component. Those of the optic nerve may be intraorbital or intracranial (Robertson and Broson, 1980; Alvord and Lofton, 1988).

Those involving the sphenoid and/or posterior ethmoidal sinuses are rare. Chen et al. (1986) and Nugent(1970) reviewed 63 cases in whom visual impairment was noted in 71% of the cases. Optic nerve damage may be caused by intracanalicular extension of the lesion with erosion of the canal walls. Periorbital swelling, pain and displacement of globe are the frequent symptoms of frontal and ethmoidal lesions. There may be a swelling over the frontal sinus with crackling sensation on palpation due to thinning of its anterior wall. Sphenoidal sinus mucoceles cause headache, peri or retroorbital pain and ophthalmoplegias due to extension into the orbital apex and cavernous sinus (pompili et al. 1990). Sellar involvement is seen frequently in sphenoidal mucocele. Surgical intervention and excision of the lesion is indicated to relieve pressure on the orbital contents.

Grave’s Disease

The aetiology of mucoceles is multifactorial. The basic cause seems to be obstruction of the ostium of the sinus by a variety of causes such as inflammation, trauma, polyps, previous surgery, allergy and benign and malignant tumors. The exact precipitating factor may not be evident in many cases (Weber and Mikulis, 1987).

It is one of the most common causes of proptosis which mostly affects females. Lid retraction is an early sign. These orbital signs may manifest at any stage of the endocrine dysfunction. Computed tomography and ultrasonography may reveal the characteristic thickening of extraocular muscles without evidence of mass lesion. The lesion is often

Histological examination (HPE) reveals fragments of polypoid tissue lined by pseudostratified ciliated epithelium. The sub epithelial tissue is made up of loose stroma with pools of small blood vessels and diffuse infiltration of mononuclear cells and prominent collections of eosinophils embedded with calcific spicules.

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Pathology of the Orbital Diseases 107

BIBLIOGRAPHY 1. Bakhshi S, Sidhu T: Pediatric orbital and ocular lymphomas, Pediatr Blood Cancer. 2008;50(4):940-1. 2. Bernardini FP, Bazzan M: Lymphoproliferative disease of the orbit, Curr Opin Ophthalmol. 2007;18(5):398-401. 3. Biswas J, Roy Chowdhury B, Krishna Kumar S, Lily Therese K, Madhavan HN: Detection of Mycobacterium tuberculosis by polymerase chain reaction in a case of orbital tuberculosis, Orbit. 2001;20(1):69-74. 4. Butnor KJ, Cummings TJ. Pathologic quiz case: left eye proptosis, ptosis, and blindness. Hemangiopericytoma of the orbit. Arch Pathol Lab Med. 2002;126(12):1555-6. 5. Cruz AA, Constanzi M, de Castro FA, dos Santos AC: Apical involvement with fibrous dysplasia: implications for vision, Ophthal Plast Reconstr surg 2007;23(6):450-4. 6. Eddleman CS, Liu JK: Optic nerve sheath meningioma: current diagnosis and treatment, Neurosurg Focus. 2007;23(5):E4. 7. Goisis M, Biglioli F, Guareschi M, Frigerio A, Mortini P: Fibrous dysplasia of the orbital region: current clinical perspectives in ophthalmology and cranio-maxillofacial surgery, Ophthal Plast Reconstr Surg. 2006;22(5):383-7. 8. Gordon LK: Orbital inflammatory disease: a diagnostic and therapeutic challenge, Eye. 2006;20(10):1196-206. 9. Harris GJ, Jakobiec FA. Cavernous hemangioma of the orbit: A clincopathological analysis of sixty-six cases. In: Ocular and adnexal tumors. Birmingham, AL: Aesculapius, 1978;741-81. 10. Honavar SG, Sekhar. G, Orbital Cysticercosis. Orbit 1998; 17(4)271-84. 11. Kaur A, Kant S, Bhasker SK: Periorbital tuberculosis, Orbit. 2007;26(1):39-42.

12. Lee V, Ragge NK, Collin JR. Orbitotemporal neurofibromatosis. Clinical features and surgical management, Ophthalmology. 2004;111(2):382-8. 13. Lin B, Looi A: Orbital lymphoma, Ophthalmology. 2007;114(7):1423. 14. Malhotra R, Wormald PJ, Selva D. Bilateral dynamic proptosis due to frontoethmoidal sinus mucocele. Ophthal Plast Reconstr Surg. 2003;19(2):156-7. 15. Nugent RA, Rootman J, Robertson WD, et al. Acute orbital pseudotumors: AJNR 1981;2:431-6. 16. Perry SR, Rootman J, White VA. The clinical and pathological constellation of wegener's granulomatosis of the orbit. Ophthalmology 1997;104:683-94. 17. Pillai S, Malone TJ, Abad JC. Orbital tuberculosis. Ophthal Plast Reconstr Surg 1995;11:27-31. 18. Rootman J, Hay E, Graeb D, et al. Orbital adenexal lymphangiomas A spectrum of hemodynamically isolated vascular hamartomas. Ophthalmology. 1986;93:1558-70. 19. Rootman J: Diseases of the orbit; A multidisciplinary approach. Lippincott Williams and Wilkins, (2nd Ed): 455-506. 20. Selva D, White VA, O'Connell JX, Rootman J: Primary bone tumors of the orbit, Surv Ophthalmol. 2004;49(3)328-42. 21. Shields JA, Shields CL, Scartozzi R. Survey of 1264 patients with orbital tumors and simulating lesions: The 2002 Montgomery lecture, part 1. Ophthalmology. 2004;111(5): 997-1008. 22. Siraj CA, Krishnan J, Nair RR, Girija AS: Invasive aspergillosis producing painful ophthalmoplegia, J Assoc Physicians India. 2005;53:901-2. 23. Swamy BN, McCluskey P, Nemet A, Crouch R, Martin P, Benger R, Ghabriel R, Wakefield D: Idiopathic orbital inflammatory syndrom: clinical features and treatment outcomes, Br J Ophthalmol. 2007;91(12):1667-70.

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6

CHAPTER

Thyroid-Associated Orbitopathy Peter J Dolman

Introduction Thyroid-associated orbitopathy (also known as Graves’ Orbitopathy, Graves’ Ophthalmopathy, Thyroid Eye Disease) is an immune-mediated inflammatory disorder causing enlargement of the orbital muscles and fat (Figure 6.1).1,2 Its clinical spectrum ranges from eyelid retraction and proptosis with exposure complaints (Figure 6.2A) to more serious problems such as orbital soft tissue inflammation (with discomfort, eyelid and conjunctival congestion and edema), extraocular motility restriction, and loss of vision from compressive optic neuropathy (Figure 6.3A).3 Severe cases may result in lasting cosmetic disfigurement and functional visual impairment. Quality of life studies have shown that it may have

Figure 6.2A: Mild TAO in a young female with bilateral upper lid retraction (worse on the left side) and secondary ocular irritation and epiphora

Figure 6.1: Axial CT scan of patient with asymmetric TAO demonstrating right proptosis from marked enlargement of the right orbital muscles and mild enlargement of the fat compartment. Compare the affected right orbit with the normal left orbit

Figure 6.2B: Same patient at the time of surgery following a posterior graded lowering of both upper eyelids

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112 Surgical Atlas of Orbital Diseases Between 25-50% of patients with immune thyroid diseases develop orbital involvement, and of those, 5-10% may develop more severe consequences such as severe inflammation and congestion, impaired motility, or compressive optic neuropathy.7 A smaller percentage may develop Graves’ lower limb dermopathy (pretibial myxedema, with deposition of subdermal hyaluronic acid), usually 1-2 years following the onset of thyroid gland dysfunction and shortly following severe orbitopathy, and a smaller subset may develop acropachy (clubbing of the fingers).8

Figure 6.3A: Patient with severe manifestations of TAO: VISA Score: V = 1/1 (bilateral optic neuropathy with vision reduced to 20/100, 20/80); I = 9/10 (pain at rest and movement, +2 chemosis, +1 eyelid edema, +1 caruncular edema, +1 conjunctival redness, +1 eyelid redness); S = 3/3 (motility restricted to less than 15° upgaze bilaterally); A = 3/3 (corneal ulceration, Hertel 26 mm bilaterally)

Figure 6.3B: Same patient following intravenous pulsed corticosteroids, orbital decompression, and upper lid posterior lowering. VISA Score: V = 0/1 (no optic neuropathy); I = 0/10; S = 1/ 3 (eyes aligned but restricted to 30° motility); A = 1/3, mild (some residual upper eyelid deformity, Hertel 21 mm bilaterally)

more significant lifestyle consequences than chronic lung disease or diabetes mellitus.4

Incidence and Epidemiology Thyroid-associated orbitopathy (TAO) is the most common orbital disease in the Americas and Europe, with an annual incidence in females of approximately 14 per 100,000 and approximately one-fifth that for males.5 Anecdotally, it may be less prevalent (or possibly causes fewer severe complications) in Africa and South Asia. However, it does occur in all races and ages, and is most common between the second and sixth decades.6

Risk Factors, Predictive Variables for Disease Severity and Associated Immune Disorders Risk factors for developing TAO include smoking, life stressors, poorly controlled hypothyroidism following radioactive iodine, and a positive family history of orbitopathy.9,10 Predictive variables for developing more serious consequences of TAO include male gender, increasing age, smoking, and a rapid onset of orbitopathy.11 Cigarette smoking has been shown by numerous studies to be correlated strongly with the development of TAO and a progressively higher incidence of smoking is seen with more severe disease.11-13 Patients with TAO have an increased probability of developing associated immune diseases, including superior limbic keratitis (SLK), myasthenia gravis, diabetes mellitus, alopecia and vitiligo.14 Psychiatric conditions such as bipolar affective disorder and anxiety occur more frequently in patients with thyroid dysfunction and with TAO.

Pathogenesis The pathologic hallmark of TAO is a lymphocytic infiltration of orbital muscle and fat with expansion of these tissues from edema and deposition of hyaluronic acid and other glycosaminoglycans.2 Patients exhibiting fat expansion alone may present with proptosis and lid retraction. Those with muscle enlargement and more inflammatory features may develop proptosis, periocular inflammatory features, and possible restriction of motility or strabismus if fibrosis develops (Figure 6.4). In a limited number

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Thyroid-Associated Orbitopathy 113 of patients with severe myopathy, or with a narrow boney orbital apex, or with tight lids limiting anterior displacement of tissues, compression of the optic nerve may ensue (Figure 6.5A). TAO is an immune-mediated disease which is strongly associated with thyroid immune disorders such as Graves' disease or Hashimoto's thyroiditis. 90% of patients with orbitopathy have a current or past history of abnormal systemic thyroid hormone levels, while others may develop abnormal levels in the future. It is important to explain to patients that the orbitopathy is associated with, but not caused by, abnormal thyroid hormone levels, since patients often believe that the orbital disease should resolve once a euthyroid state is reached. Thyroid gland epithelial cells have surface receptors which bind thyroid stimulating hormone (TSH, thyrotropin), a hormone secreted in pulses by the pituitary to control the release of thyroid hormone. In both Graves’ disease and Hashimoto's thyroiditis, circulating thyrotropin-receptor antibodies (TSH-R Antibodies, TRAb) are present which can bind to these same receptor sites, and initiate and perpetuate the disease.2 At least three subtypes of TRAb have been identified, presumed to arise from a small population of abnormal B-lymphocytes: (1) TSI (thyroid stimulating immunoglobulin), which causes hyperthyroidism; (2) blocking TRAb, which prevents TSH from binding to thyroid cells and results in hypothyroidism;

Figure 6.5A: Coronal CT scan near apex showing bilateral optic nerve crowding. This patient had impaired central vision (20/70, 20/ 40), bilateral reduced color vision, and a right afferent pupil defect. VISA Score: V = 1/1 (optic neuropathy present)

Figure 6.5B: Axial CT scan of same patient showing adequate medial wall decompression into ethmoid sinuses and resolution of optic neuropathy (V = 0/1; central vision 20/20 both eyes)

Figure 6.4: Patient with TAO myopathy attempting to look up-wards. She had bilateral upgaze limitation, the right reaching 30° and the left reaching 10°, based on the light reflexes on her cornea (VISA strabismus score = 3/3)

(3) binding TRAb, which binds onto TSH receptors transiently, and has little effect on overall thyroid hormone levels. These circulating antibodies are thought to be mediators in orbitopathy as well, with the likely target being the orbital fibroblast.2 Orbital fibroblasts are present in extraocular muscle and in orbital fat. Orbital fibroblasts from patients with TAO have increased numbers of TSH-Receptors, which are thought to bind to circulating autoantibodies (TRAb), stimulating adipogenesis and deposition of hyaluronic acid within orbital muscle and fat, the histologic hallmark of TAO.

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114 Surgical Atlas of Orbital Diseases Orbital fibroblasts in patients with TAO have also recently been shown to have an increased number of receptors to “insulin-like growth factor-1” (IGF-1 R), and serum from patients with TAO has been found to have circulating antibodies directed against IGF-1 receptors.15 Binding of IGF-1 receptors has been shown to attract and activate Tlymphocytes and macrophages through inflammatory mediators, which may be the mechanism of initiating and propagating the inflammatory and immune cascade in TAO.16

Course of Disease As with other immune disorders such as rheumatoid arthritis or Sjogren’s disease, TAO typically has a progressive (active) inflammatory phase followed by a stable (inactive) postinflammatory phase. This pattern of the disease was first described by Rundle, and the plot of orbital disease severity against time has been called Rundle's curve.17,18 The steepness of the graph in the active phase reflects the acuity of progression, with a steeper slope often leading to more serious sequellae.11 The duration of the active phase may last from 6-18 months, during which the patient may experience inflammatory symptoms of orbital discomfort, periocular and conjunctival edema and redness, and progression in proptosis, strabismus or optic neuropathy. Management during this phase is aimed at modulating the immune response and reducing the inflammation, usually with the use of steroids, radiotherapy, or other immunosuppressive agents, and hopefully limiting the destructive consequences of the active phase. A useful analogy for patients is that the inflammatory phase is like a house on fire. While ignited, efforts are made to staunch the flames or allow them to smolder if not too severe. Reconstruction is not carried out while the fire is still active. Once the disease has become quiescent, surgery may be offered to rectify damage resulting from the active stage, including reducing proptosis, aligning muscles, narrowing eyelid apertures, and debulking fat pockets in the eyelids. This would be similar to repairs being carried out after the house fire was suppressed. Reactivation of disease is fairly uncommon, 19 occurring in less than 5% of individuals, and is

sometimes associated with a major life stressor such as a family death, divorce or loss of job.

Clinical Classification One of the challenges in TAO is how to classify and grade its various clinical manifestations so that appropriate management can be instituted. Most ophthalmologists are familiar with Dr Werner’s NO SPECS classification that graded various symptoms and signs associated with the disease and assigned a global severity score.20 While this has served as a useful mnemonic for the different features of TAO, it is weak in terms of defining management and doesn't assess whether the disease is in the active or inactive, postinflammatory phase. In 1989, Drs Mourits et al introduced a clinical activity score (CAS) to stage and grade the inflammatory phase of the disease.21

The VISA Classification We have recently introduced the VISA classification which is a clinical recording form designed for the office, and which separates the various clinical features of TAO into four parameters: V (vision, optic neuropathy); I (inflammation, congestion); S (strabismus, motility restriction); A (appearance, exposure).22 The basic visit form (Figure 6.6) includes the four sections recording historical symptoms on the left and signs on the right. After each section is a progress row (better, same, worse), recording both the patient's and clinician's impression of the course of the disease since the last visit. The layout is designed to simplify data recording and possible later research data collation. Individual measurements may be completed in as much or little detail as the clinician chooses. At the end of the form is a summary grade for the activity and severity for each of the four disease parameters and a space for investigations and management plan. On the first visit, the date and rate of onset of both the systemic and orbital symptoms should be recorded, since this may help predict the ultimate severity of the inflammatory phase. a) Vision/optic neuropathy: The focus of this section is to identify TAO optic neuropathy. The history includes visual blurring or color desaturation and the progress and duration of symptoms.

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Thyroid-Associated Orbitopathy 115

Figure 6.6: Follow-up VISA classification form

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116 Surgical Atlas of Orbital Diseases Objective measures of optic neuropathy include a loss in central visual acuity and color vision, an afferent pupil defect, and infrequently congestion or pallor of the optic nerve. Ancillary testing includes coronal CT or MR Scans to confirm crowding of the orbital apex, standardized visual fields, and rarely VEP or optic nerve head photos. As a summary grade, VISA lists optic neuropathy as present or absent since most clinicians would attempt some treatment for this condition if present. The severity of the neuropathy is reflected in the individual measurements of central and color vision. My usual treatment for TAO optic neuropathy initially is high dose corticosteroids either by intravenous route (1 gram Methyl-prednisolone over 30 minutes on alternate days for 3 treatments) or oral route (100 mg prednisone daily on a tapering dose) with an expectation that vision should improve within days of therapy. In most cases, this treatment will cause incomplete or only temporary visual improvement so that surgical decompression of the medial wall is required (through a Lynch, transcaruncular or endoscopic transethmoidal approach) in order to relieve pressure more permanently on the optic nerve at the crowded orbital apex (Figure 6.5A and B). I often arrange adjunctive radiotherapy subsequent to the decompression if the disease is active to prevent further enlargement of the muscles and recurrence of neuropathy. Radiotherapy is administered using a lateral port focused behind the globe to minimize the risk of retinal or lens exposure; it is divided into 10 fractions of 200 rads over two weeks23 and is contraindicated in diabetics because of the risk of inciting or aggravating retinopathy.24 Although radiotherapy remains controversial, it is still widely used and many clinicians believe it is beneficial for certain aspects of TAO, including optic neuropathy and significant inflammation.25 Success of therapy for TAO optic neuropathy from both a clinical or research standpoint is based on specific improved measurements for central vision, color vision and visual fields. b) Inflammation/congestion: Symptoms of ocular and periocular soft tissue inflammation include orbital aching at rest or with movement, and eyelid or conjunctival swelling and redness.

The Clinical Activity Score (CAS) described and validated by Mourits and the Amsterdam Orbitopathy group assigns one point for each of the following: orbital pain at rest, orbital pain with movement, chemosis, caruncular edema, eyelid edema, conjunctival injection and eyelid injection.21 The VISA Inflammatory Score modifies the CAS slightly by widening the grade for chemosis and lid edema from 0-2. Chemosis is graded as 1 if the conjunctiva lies behind the grey line of the lid and as 2 if it extends anterior to the grey line (Figures 6.3A and 6.7A). Lid edema is graded as 1 if it is present but not causing overhanging of the tissues, and as 2 if it causes a roll in the lid skin including festoons in the lower lid (Figures 6.3A and 6.7A). The worst scores from any of the four eyelids are recorded in the inflammatory score table on the far right section of the table. The pain score is based on the patient's report of deep orbit discomfort rather than ocular surface irritation (0 = no pain, 1 = pain with movement, 2 = pain at rest). The additional grading scores for chemosis and lid edema allow for documentation of more subtle changes in inflammatory features between visits. An additional point is assigned for diurnal variation of symptoms, to reflect the variability in congestion typically seen during the active phase. Treatment of active inflammation in TAO depends on its inflammatory score and evidence of progression. If the score is less than 4 out of 10, and there is no deterioration based on history or sequential clinical examination, conservative management is offered with reassurance, cool compresses, nocturnal head elevation, and nonsteroidal anti-inflammatories. In general, if the inflammatory grade is 5 or more, or if there is subjective or objective evidence of progression in the inflammation, more aggressive therapy should be considered, including oral or intravenous corticosteroids, radiotherapy, and in refractory cases, immunosuppressive agents (Figures 6.3A and B, 6.7A and B). Combination therapy is receiving increasing attention in severe, progressive cases 26, 27 and interest is also turning to new immunomodulatory agents, such as anti-tumor necrosis factor agents (etanercept, infliximab) or B-lymphocyte directed therapy (rituximab).28 The hope from greater understanding of the immunogenic mechanisms in this disease is to

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Thyroid-Associated Orbitopathy 117

Figure 6.7A: Patient with severe active orbitopathy: V = 1/1 (optic neuropathy present); I = 10/10 (2/2 pain, 2/2 chemosis with conjunctiva overlying eyelid margins, 2/2 eyelid edema of left lower eyelid, 1/1 caruncular edema, 1/1 redness of conjunctiva, 1/1 redness of eyelid, 1/1 progression in symptoms over past month)

Figure 6.7B: Same patient 2 days following 3 doses of intravenous methyl-prednisolone with marked reduction in inflammatory signs. VISA Score: V = 1/1 optic neuropathy (improved vision, but still showing impaired central and color vision); I = 4/10 (0/2 pain, 1/2 chemosis with conjunctiva behind grey line of lower lid, 1/2 eyelid edema, with resolution of eyelid overhang, 0/1 caruncular edema, 1/1 conjunctival redness, 0/1 eyelid redness, 1/1 diurnal variation)

identify markers of progressive, more severe disease so that earlier treatment and more specific immunotherapy may be developed. c) Strabismus / motility restriction: The symptoms for strabismus include a progression from no diplopia, diplopia with horizontal or vertical gaze, intermittent diplopia in straight gaze, and constant diplopia in straight gaze.

Ocular ductions can be graded from 0° to 45° in four directions using the Hirschberg principle: the patient is asked to look as far as possible up, down, right and left while the observer points a bright light at the eyes and studies the light reflex on the surface of the eye. If the light reflex hits the edge of the pupil, the eye has moved 15°, between the pupil edge and the limbus, 30° and at the limbus, 45° (Figure 6.4). Strabismus can be measured objectively by prism cover testing in different gaze directions. Ancillary testing includes using the Goldmann perimeter to quantify ocular ductions in four directions.29, 30 The patient keeps both eyes open and follows the V4e light target, tapping a coin when the image becomes double. Management of strabismus depends on whether the orbitopathy is inflamed (measured in the previous section) or if there is evidence of progression in symptoms and signs. If inflammation is present, this is managed first, either with conservative treatment or with anti-inflammatories or radiotherapy (Figure 6.8A). During this stage, the strabismus can be managed with patching one eye or with Fresnel prisms. Once the inflammatory score has dropped to zero and there is no evidence of progression, management of strabismus might include prisms or surgical alignment (Figure 6.8B). d) Appearance/exposure: Symptoms in this category include appearance concerns such as bulging of the eyes, eyelid retraction and fat pockets, as well as exposure complaints of foreign body sensation, glare, dryness or secondary tearing. Objective measures of appearance change include eyelid retraction (measured in millimeters), proptosis (measured with the Hertel exophthalmometer), and documentation of redundant skin and fat prolapse. Measures of exposure include corneal staining or ulceration. Photographs can document the appearance changes. Management of appearance and exposure changes depend on the inflammatory stage of the disease. During the inflammatory phase (documented progression in any of the parameters or an inflammatory score > 5), lubricant drops and

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118 Surgical Atlas of Orbital Diseases tempo of disease can be documented to reflect disease activity. The classification system has been validated by showing that two clinicians could use the forms to assess patients independently with different manifestations of TAO and to choose similar management plans.22 It is currently being assessed by members of the International Thyroid Eye Disease Study (ITEDS) Group and further refinements and validation will be conducted. A common method of documenting and classifying the disease parameters is critical for conducting multicenter clinical trials and to assess response to different therapies. Figure 6.8A: Patient with active TAO and strabismus (VISA Score: I = 7/10, S = 3/3, strabismus in primary gaze). Anti-inflammatory treatment was instituted and one eye was patched for comfort

Figure 6.8B: Once the disease had progressed into the postinflammatory (inactive) phase, alignment surgery was performed: (VISA Score: V = 0/1; I = 0/10; S = 0/3, A = 0/3)

ointments can relieve ocular irritation. Rarely a tarsorrhaphy or emergency orbital decompression may be required for severe exposure or corneal ulceration. Once the inflammatory phase has settled, management for proptosis might include orbital decompression and for eyelid retraction may include upper lid lowering from an anterior or posterior approach or lower lid elevation.31 These surgical measures often relieve many of the exposure complaints (Figures 6.2A and B, 6.3A and B). e) Application of the VISA classification: The VISA Classification clusters the four functions disrupted by TAO in a logical sequence for recording and management. Subjective input and reproducible objective measurements are recorded for each section and a global severity grade can be assigned for each function. The subjective and objective progress and

General Management Guidelines Patients with TAO are often misdiagnosed initially, because the majority present with mild, nonspecific complaints such as tearing, irritation and light sensitivity as a result of exposure from mild lid retraction. Occasionally mild inflammatory features such as eyelid or conjunctival swelling may be interpreted as allergies or viral infections. Many of these patients are frustrated with their medical care by the time the correct diagnosis has been reached. More serious manifestations of the disease such as myopathy, proptosis and visual impairment generally develop rapidly and are more readily recognized. Understanding what bothers the patient most about their condition helps to build rapport and to plan future management. Patients appreciate reassurance and education about the natural course of TAO. Clarify that their endocrinologist and family practitioner will work to control any thyroid dysfunction, and that while their orbitopathy may be linked to thyroid immune disorders, a euthyroid state does not necessarily lead to resolution of the orbitopathy. Emphasize that they can take positive steps to help their condition by quitting smoking and relieving stressors in their lives. I take the time to explain what is predictable about the disease, mentioning that the disease is selflimited, and that those with a mild presentation and younger age are unlikely to progress to more serious complications. For those with more serious complications, I use the house-fire analogy to clarify the two stages of orbitopathy, and the role of antiinflammatory or immunomodulatory therapy in the active phase and surgical reconstruction if necessary in the postinflammatory stage. The internet provides a number of support groups and I also identify

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Thyroid-Associated Orbitopathy 119 patients who I have treated who are willing to talk to newly diagnosed patients to allow sharing of their experiences.

REFERENCES 1. Bahn RS, Heufelder AE. Pathogenesis of Graves' Ophthalmopathy. N Engl J Med 1993;329(20):1468-75. 2. Garrity JA, Bahn RS. Pathogenesis of Graves' Ophthalmopathy: Implications for Prediction, Prevention and Treatment. Am J Ophthalmol 2006;142(1):147-53. 3. Rootman J, Dolman PJ. Thyroid Orbitopathy (Chapter 8) in: Diseases of the Orbit. A Multidisciplinary Approach. Hagerstown: Lippincott Williams & Wilings, 2003. 4. Gerding MN, Terwee CB, Dekker FW et al. Quality of life in patients with Graves' Ophthalmopathy is markedly decreased: measurement by the medical outcomes study instrument. Thyroid 1997;7(6):885-89. 5. Bartley GB. The epidemiologic characteristics and clinical course of ophthalmopathy associated with autoimmune thyroid disease in Olmsted County, Minnesota. Trans Am Ophthalmol Soc 1994;92:477-588. 6. Kendall-Taylor P, Perros P. Clinical presentation of thyroid associated orbitopathy. Thyroid 1998;8:427-28. 7. Burch HB, Wartofsky L. Graves' Ophthalmopathy: current concepts regarding pathogenesis and management. Endocr Rev 1993;14:747-93. 8. Fatourechi V, Pajouhi M, Fransway AF: Dermopathy of Graves disease (pretibial myxedema). Review of 150 cases. Medicine (Baltimore) 1994;73(1):1-7. 9. Perros P, Kendall-Taylor P. Natural history of thyroid eye disease. Thyroid 1998;8:423-25. 10. Bartalena L, Marcocci C, Bogazzi F, et al. Relation between therapy for hyperthyroidism and the course of Graves Ophthalmopathy. N Engl J Med 1998;338:73-78. 11. Dolman PJ, Rootman J. Predictors of disease severity in thyroid-related orbitopathy. (Chap18) Orbital Disease. Present status and future challenges. Taylor and Francis, 2005. 12. Prummel MF, Wiersinga WM. Smoking and risk of Graves' disease. JAMA 1993;269:479-82. 13. Pfeilschifter J, Ziegler R. Smoking and endocrine ophthalmopathy: impact of smoking and current vs lifetime cigarette consumption. Clin Endocrinol (Oxf) 1996;45: 477-81. 14. Cruz AA, Akaishi PM, Vargas MA, et al. Association Between Thyroid Autoimmune Dysfunction and NonThyroid Autoimmune Diseases. Ophthalmic Plastic & Reconstr Surg 2007;23(2):104-08. 15. Pritchard J, Horst N, Cruikshank W, et al. Igs from patients with Graves' disease induce the expression of T cell chemoattractants in their fibroblasts. J Immunol 2002; 168: 942-50.

16. Pritchard J, Han R, Horst N, et al. Immunoglobulin activation of T cell chemoattractant expression in fibroblasts from patients with Graves' disease is mediated through the insulin-like growth factor 1 receptor pathway. J Immunol 2003;170:6348-54. 17. Rundle FF. Development and course of exophthalmos and ophthalmoplegia in Graves' disease with special reference to the effect of thyroidectomy. Clin Sci 1945;5:177-94. 18. Rundle FF. Ocular changes in Graves' disease. QJM 1960; 29:113-26. 19. Selva D, Chen C, King G. Late reactivation of thyroid orbitopathy. Clin & Exp Ophthalmol 2004;32(1),46-50. 20. Werner, SC. Classification of the eye changes of Graves' disease. American J Ophthalmology 1969;68:646-48. 21. Mourits MP, Prummel MF, Wiersinga WM, et al. Clinical activity score as a guide in the management of patients with Graves' Ophthalmopathy. Clin Endocrinol 1997;47: 9-22. 22. Dolman PJ, Rootman J. VISA Classification for Graves' Orbitopathy. Ophthal Plast Reconstr Surg. 2006;22(5): 319-24. 23. Beckendorf V, Maalouf T, George J-L, et al. Place of radiotherapy in the treatment of Graves' orbitopathy. Int J Radiation Oncology Biol Phys 1999;43:805-15. 24. Viebahn M, Marricks ME, Osterloh MD. Synergism between diabetic and radiation retinopathy: a case report and review. Br J Ophthalmol 1991;75:29-32. 25. Cockerham KP, Mourits MPh, McNab AA, et al. Does radiotherapy have a role in the management of thyroid orbitopathy? Debate. Br J Ophthalmol 2002;86:102-04. 26. Kahaly G, Schrezenmeir J, Krause U, et al. Ciclosporin and prednisone vs prednisone in treatment of Graves' Ophthalmopathy: a controlled, randomized and prospective study. Eur J Clin Invest 1986;16(5):415-22. 27. Bartalena L, Marcocci C, Chiovato L, et al. Orbital cobalt irradiation combined with systemic corticosteroids for Graves' Ophthalmopathy: comparison with systemic corticosteroids alone. J Clin Endocrinol Metab 1983;56(6): 1139-44. 28. Leandro MJ, Edwards JC, Cambridge G. Clinical outcome in 22 patients with rheumatoid arthritis treated with B lymphocyte depletion. Ann Rheum Dis 2002;61:883-88. 29. Gerling J, Lieb B, Kommerell G. Duction ranges in normal probands and patients with Graves' ophthalmopathy, determined using the Goldmann perimeter. Int Ophthalmol. 1998;21(4):213-21. 30. Dolman PJ, Kendler D, Rootman J. Measuring ocular excursions in Graves' Orbitopathy. (Abst) International Congress of Ophthalmology 2004. 31. Looi, A, Sharma B, Dolman PJ. A Modified Posterior Approach for Upper Eyelid Retraction. Ophthalmic Plast Reconst Surg 2006;22(6).

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7

Orbital Infections

CHAPTER Shome Debraj, Walinjkar Jaydeep, Mukherjee Angshuman

Many disease processes such as cancers, infections, or inflammations present with overlapping clinical manifestations, because of the confined nature of the orbital space. Also, with the multitude of muscular, neurovascular, sensory, and glandular structures located close to each other in this space, precise anatomic localization of various biologic processes can be difficult. Orbital infections and inflammations present to the clinician with similar findings: periorbital edema, erythema, proptosis, and pain. History and clinical examination determine the work-up required to better define the disease process. Orbital infections continue to be associated primarily with diseases of the paranasal sinuses.1 Hemophilus influenza type B is no longer a significant pathogen, because of an effective vaccine.2 Fungal infections extending to the orbit are becoming more frequent, due to the increased prevalence of immunocompromised patients.3 Infections of the orbit are uncommon, but they are potentially devastating infections that can quickly result in blindness, meningitis, or death. The emergency physician must make a rapid and accurate diagnosis and then quickly initiate therapy because visual loss is associated directly with the length of time to definitive treatment. Smith and Spencer4 classified orbital infections into 5 tiers which were later modified by Chandler et al.5 • Group I — Preseptal cellulitis • Group II — Orbital cellulitis • Group III — Subperiosteal abscess

• Group IV — Orbital abscess • Group V — Cavernous sinus thrombosis. This classification system does not necessarily imply an order of disease progression; however, it helps explain the physical signs and symptoms of the various infections and helps organize treatment plans.6 Orbital infections may be divided into preseptal cellulitis, in which infection is located anterior to the orbital septum (a thin sheet of fibrous tissue arising from the periosteum of the orbital margin and inserting into the tarsal plates), and orbital cellulitis, in which there is infection of orbital tissues posterior to the orbital septum.7 Preseptal cellulitis generally responds to oral antibiotics and rarely has important sequelae. However, orbital cellulitis is a serious infection which may be complicated by abscess formation (subperiosteal, orbital, or brain), meningitis, septicemia, cavernous sinus thrombosis, and death. Although orbital cellulitis is related to ethmoid sinusitis in 70-80% of cases8, it may also develop after orbital or sinus trauma.9, 10 Prompt and appropriate management of patients with orbital cellulitis or at risk of developing this minimises the risk of complications. 10 The possible causes of Mortality/Morbidity following orbital cellulitis are: • Cavernous sinus thrombosis • Brain abscess or meningitis • Permanent vision loss.

Demographic Profile Sex • Males are affected slightly more often than females.

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Orbital Infections 121

Age • Orbital infections are more common in persons younger than 19 years • Orbital infections are more severe in adults.

Risk Factors Past medical history significant for HIV, diabetes, immunosuppression, steroid use, renal disease, and travel is important. Chronologic relation with an insect sting, allergic reaction, or trauma may suggest etiologies that mimic an orbital infection.

Etiological Causes of Orbital Infections

not uncommon. The causative organisms include E granulosus, T solium,Trichinella spiralis and the Onchocerca.15

Protozol infections Although relatively uncommon, protozoal infections (most commonly with toxoplasma gondii) are seen in immunocompromised individuals.16 They have an increased likelihood for more severe and atypical presentations; this highlights the need for increased index of suspicion for HIV infection as ocular or orbital disease may be the first manifestation of life-threatening systemic toxoplasmosis.

Bacterial infections

Diagnosis

Bacteria, most commonly Streptococcus species, as well as Staphylococcus aureus, Hemophilus influenzae, and anaerobes, cause the vast majority of orbital infections.11 The incidence of Hemophilus influenzae type B has decreased since the early 1990s.2 In communities where CAMRSA is prevalent, ophthalmologists should maintain high index of suspicion and obtain microbial cultures and sensitivity studies to help guide antibiotic therapy for severe ophthalmic infections.12

The diagnosis of orbital infections includes the following: • A detailed history • Vision • Slit-lamp examination • Extraocular motility • Examination to document optic nerve function; including pupillary function • Fundus examination • Resistance to retropulsion • Exophthalmometry: Hertel's exophthalmometry is the gold standard.

Fungal infections Fungal infections are less common than bacterial infections and occur more commonly in patients who are immunocompromised (e.g., those with HIV or diabetes). (Rhizopus, Mucor, Aspergillus)13 Phycomycosis, also known as mucormycosis, is the most common and most virulent fungal disease involving the orbit. The specific fungal genus involved is usually Mucor or Rhizopus. These fungi can involve the blood vessel wall and produce thrombosing vasculitis.Therapeutic measures include controlling the underlying metabolic abnormality, local surgical excision of infected tissues, and administration of amphotericin B.14, 3

Parasitic infections Parasitic diseases may be prevalent in endemic areas (e.g. Mediterranean, Eastern Africa, Australia, Middle East Asia, South America, Eastern Europe) and in travelers to these areas, such infections are

Imaging Studies17 • Computed Tomography (CT) scan — A CT scan of the orbit, sinuses, and frontal lobe is essential for every patient showing signs of orbital involvement. 2 mm cuts are ideal for the orbit. Both Axial and coronal scans shoud be carefully looked at, to obtain a 3-dimensional perspective. • Magnetic Resonance Imaging (MRI) — While a CT scan provides enough information in most cases, a MRI scan may improve visualization of cavernous sinus thrombosis. • Ultrasonography (USG) B-scan — An USG Bscan is mostly supportive in role, due to difficulty in interpretation of posterior orbital lesions. • X-rays — Waters, Caldwell, submental vertex, and lateral view are mainly of historical interest.

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122 Surgical Atlas of Orbital Diseases

Other Tests • Fiberoptic nasopharyngeal endoscopy:18 If any suspicion of mucormycosis (i.e. elevated blood glucose, leukemia, renal disease) exists, fiberoptic nasopharyngeal endoscopy should be performed (usually, by an otolaryngologist) to seek evidence of black eschar formation. Endoscopy assisted transnasal drainage is also useful in cases with evidence of associated sinus infections. • Rapid plasma reagin (RPR), particularly in cases of insidious onset or with a history of syphilis. • Cerebrospinal fluid (CSF) analysis for gram stain, cell count, cultures, and antigens may be considered in patients with associated central nervous system signs.

Emergency Department Care • Adults with preseptal cellulitis and no signs of orbital involvement can be discharged on oral antibiotics with close follow-up care.19 — Adults with orbital signs need admission and IV antibiotics or antifungals are quickly initiated, under close supervision. — Surgical intervention should be performed immediately, in cases with CT evidence of subperiosteal or orbital abscess — for drainage.19 — Surgical drainage is not always necessary for cellulitis; however, any patient with compromised vision (20/60 or worse) should receive immediate surgery for drainage and debridement.19 — Surgical drainage of abscesses (orbital or subperiosteal) is considered, even without visual loss. Drainage of sinuses should be considered in patients with associated sinusitis. — Some patients can be monitored for 48 hours on IV antibiotics, with surgery performed for increasing proptosis, worsening visual acuity, or isolated muscle weakness. Surgery is performed after 48 hours if fever continues or antibiotics fail.20 — Orbital drainage decreases intraorbital pressure, decreases associated pressure on the nerve and retinal circulation, creates a

potential path for further drainage (the wound is closed with spaced sutures) and provides material for appropriately guided antibiotic therapy. We recommend orbital drainage by a trained orbital specialist as tissue in these cases are extremely vascular, friable and susceptible to damage. — All children are admitted prior to initiating therapy21 even if they lack orbital signsbecause children are deficient in IgG2 and are predisposed to bacteremia. For orbital cellulitis, oxacillin or nafcillin can be used with the addition of ampicillin and sulbactam in children to cover H influenzae.21 Patients who are allergic to penicillin can use vancomycin, clindamycin, or chloramphenicol. More and more organisms in countries like India are becoming resistant to conventional antibiotics. Methicillicin resistant Staphylococcus Aureus (MRSA) strains are not only prevalent in the nosocomial environment but are also found in the community. MRSA orbital cellulitis can quickly progress to irreversible blindness, despite antibiotic treatment. — Alternatively, a cephalosporin (e.g. cefuroxime, cefoxitin, cefotetan) can be used alone. — Nasal decongestants can be used to help drain the sinuses. — Concomitant steroid therapy, once clinical improvement is documented on antibiotics, is started to decrease inflammation associated collateral damage to tissue and edema, thereby further decreasing intraorbital pressure.

Further Inpatient Care Underlying disorders, if present (e.g. hyperglycemia, acidosis, infection, immunosuppression) are corrected. Consultations: Consultation with an ENT surgeon or a neurosurgeon must be considered in cases without improvement, or in cases with involvement of sinuses or the nervous system. Necrotizing fascitis, Orbital Tuberculosis and Community Acquired MRSA (CAMRSA) induced orbital cellulitis are rare entities in the spectrum of orbital infections. Although these infections are not so common, ophthalmologists should be well aware of these conditions, especially in endemic areas as

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Orbital Infections 123 the morbidity and mortality of selected cases are reduced with prompt and appropriate antimicrobial therapy. Keeping in mind this fact we find it appropriate to present the following case reports:

CASE ILLUSTRATIONS Case 1 A 25-year-old systemically healthy male patient presented with complaints of severe photophobia, redness, discharge, pain and severe swelling of the lids in the left eye, since 2 days. Past history was significant for a boil on the lower eyelid, 2 days ago. On examination, the best-corrected visual acuity was 6/6 and 6/9, in the right and left eyes respectively. Right eye examination was unremarkable. The left eye showed severe lid edema with scales on the skin and was diagnosed as having left sided periocular necrotising fascitis with associated keratoconjunctivitis (Figure 7.1A). The cornea showed multiple marginal infiltrates. Photographic documentation of the anterior segment condition was impossible because of the severe photophobia. Extraocular movements were full. A conjunctival swab and a periorbital skin swab were sent for culture and sensitivity. The corneal infiltrates were also cultured on Blood agar and Sabouraud's dextrose agar. The patient was seen by our infectious diseases expert and started on intravenous coamoxiclav 1 gram twice daily, intravenous ceftriaxone 1 gram twice daily and oral metronidazole 500 mg three times daily, pending sensitivity reports. Topical Lotepred eye drops every 3 hourly and ciprofloxacin (0.3%) eye drops 6 times a day were started, in the left eye. On follow-up 2 days later, the patient was symptomatically much better. The skin scabs had fallen off, revealing violaceous, sub-epidermal necrosis. The conjunctival inflammation had reduced and the corneal marginal infiltrates had almost disappeared (Figure 7.1B). Culture and sensitivity results showed Staphylococcus aureus, sensitive to the administered medications. The culture plates for corneal infiltrates showed negative growth and were discarded after 3 weeks. The patient was seronegative for HIV. Five days later, the skin lesions had healed and the conjunctivitis had resolved. Intravenous

antibiotics were stopped and the patient was started on oral antibiotics for a week. On final follow-up a month later, periocular skin discoloration was the only sequalae noted (Figure 7.2).

Discussion Necrotising fascitis (NF) is a serious life threatening condition, with reported mortality of more than 20%. The limbs, perineum and abdomen are frequently involved with facial involvement being rarely involved. The organisms most closely linked to NF are Group A beta-hemolytic streptococci, though these bacteria are isolated in only a minority of the cases.20

Figure 7.1A: Periocular necrotising fasciitis: External photograph of patient at presentation, showing left sided severe lid edema, erythema and necrotic tissue with overlying skin scabs

Figure 7.1B: Periocular necrotising fasciitis: Slit lamp photograph of the left eye, in diffuse illumination (with upper eyelid retracted), showing periocular skin erythema and kerato-conjunctivitis

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124 Surgical Atlas of Orbital Diseases ocular NF and the hitherto unreported anterior segment involvement.

Case 2

Figure 7.2: Periocular necrotising fasciitis: External photograph of patient, a month post presentation, showing healed skin lesions, with symmetrical palpebral apertures

Periocular NF is reported to have a better prognosis.22 Though reports of resolution of periocular NF postsurgical debridement are common, a detailed Medline and Embase search revealed only one case report which was reported to resolve with conservative management.23 Infections in the periocular region occur postsurgical procedures, post trauma, postfurunculosis or even without any antecedent cause.23 Ideally, a combination of intensive parenteral antimicrobial therapy and prompt surgical debridement of necrotic tissue should be performed. Intravenous pooled immunoglobulin and heparinisation may also have beneficial roles by neutralising super-antigen activity and aiding antibiotic perfusion.24 Mild cases especially those restricted to the eyelids alone may respond to medical therapy.23 We report the case of a 25-year-old male patient who presented with periocular NF associated with keratoconjunctivitis. We report this case to highlight the successful conservative management of peri-

A

A 60-year-old systemically healthy female was referred with a 2-month history of irritation in the left eye associated with a mass in the lower lateral orbit. Her visual acuity was 20/20 in both eyes. The right eye was unremarkable. The left eye was 2 mm enophthalmic by Hertels exophthalmometry but showed no displacement (Figure 7.3A). Ocular motility was full in range. There was a nontender, hard, lobulated mass measuring about 30 mm × 10 mm in size in the inferior orbit, felt separate from the inferior orbital rim. The mass appeared to be contiguous with the globe. General physical examination including chest roentgenogram was unremarkable. Computed tomography scan showed an illdefined extraconal anterior orbital mass located inferotemporal in the left orbit, with minimal contrast enhancement (Figure 7.3B). Differential diagnoses of scirrhous breast cancer metastasis or sclerosing orbital pseudotumor were considered and a biopsy was performed by the inferior conjunctival fornicial approach. Histopathology revealed features of noncaseating granulomatous inflammation and fibrosis (Figure 7.4A). AFB was negative both by smear and culture. PCR for Mycobacterium tuberculosis DNA was positive (Figure 7.4B). Orbital tuberculosis was diagnosed and the patient received four-drug combination antitubercular therapy for 6 months. The orbital mass completely regressed and there was no local recurrence at two years.

B

Figures 7.3A and B: Orbital tuberculosis manifesting with enophthalmos: External photograph of the face showing mild enophthalmos of the left eye (left) and a coronal computed tomography scan showing an ill-defined anterior orbital mass located inferotemporally in the left orbit, with minimal contrast enhancement (right)

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Orbital Infections 125

A

B

Figures 7.4A and B: Orbital tuberculosis manifesting with enophthalmos: Histopathology of the orbital mass showing features of non-caseating granulomatous inflammation and fibrosis (hematoxylin and eosin, x 500) (left) and polymerase chain reaction of the specimen positive for mycobacterium tuberculosis deoxyribonucleic acid (right)

Discussion Orbital tuberculosis is rare, even in endemic countries.25 Erosion of a parenchymal pulmonary tuberculous focus in a blood or a lymph vessel may lead to dissemination of the organism; with systemic involvement. 26 Orbital tuberculosis may involve orbital soft tissues, lacrimal gland, periosteum, and bones, and may extend to a contiguous paranasal sinus or intracranial cavity.25, 27 The disease course is generally slow and indolent. 25,27 Clinical manifestations include orbital tuberculoma and cold abscess presenting with proptosis, and orbital osteomyelitis manifesting with discharging sinus and inflammation.25, 27 Orbital tuberculosis presents with proptosis and is not known to present with enophthalmos.28 Common manifestations of orbital tuberculosis are proptosis or discharging sinus. Our patient, however, presented with an orbital mass and enophthalmos. Orbital tuberculosis presents with proptosis and is not known to present with enophthalmos.25,27 Herein we report a case of orbital tuberculosis presenting with an orbital mass and paradoxical enophthalmos.

On examination, the visual acuity was perception of light with inaccurate projection and 6/9 in the right and left eyes respectively. The right eye was severely proptotic, with greatly increased resistance to retropulsion. There was an upper lid wound, with purulent drainage. The bulbar conjunctiva was severely chemosed, making the remaining slitlamp examination difficult (Figure 7.5A). Extraocular movements were severely limited in all directions of gaze. Left eye examination was unremarkable. The patient was mildly febrile. Remaining systemic examination was unremarkable. An orbital Computed Tomography (CT) scan, routine blood investigations and culture and urine examination and culture were ordered. The blood and urine examination reports were unremarkable, except for mild leucocytosis. The CT scan showed a superior orbital mass suggestive of an abscess. The paranasal sinuses were clear of any obvious infection. Tenting of the ocular contents was seen on axial CT, suggestive of increased orbital pressure. A superior orbitotomy for drainage of the abscess was performed, under general anesthesia. 2 millilitres of pus was drained and sent for culture and antibiotic sensitivity testing and Pulsed field Gel Electrophoresis (PFGE), which revealed CAMRSA, resistant to all antibiotics, except vancomycin, cotrimoxazole (trimethoprin/sulfamethoxazole combination) and amikacin. The patient was admitted and started on intravenous vancomycin 1 gram every 6 hours and intravenous amikacin 1 gram daily, after consultation with our internist. The patient started improving three days post therapy. Intravenous dexamethasone 4 mg every 8 hours was started to decrease associated inflammation. This was subsequently increased to

Case 3 A 55-year-old systemically normal female patient presented to us with right-sided sudden onset severe proptosis, pain and dimunition of vision, of 2 days duration. Past history was significant for a boil on the right upper eyelid, 2 days prior. The patient was on oral amoxicillin 750 mg three times daily, at presentation.

Figure 7.5A: CAMRSA orbital cellulitis: External photograph of patient, showing severe right sided proptosis, with conjunctival chemosis and draining wound of the right upper eyelid

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126 Surgical Atlas of Orbital Diseases 8 mg every 8 hours, 2 days later. Serum creatinine and urea were monitored every 72 hours. Seven days posttherapy, the vision had improved to 6/24 and the ocular movements had normalized almost completely. However, lagophthalmos due to the earlier lid wound persisted. The patient was discharged on oral cotrimoxazole 960 mg every 12 hours and oral steroids 60 mg every day. The antibiotic was stopped a week later and steroids gradually tapered. At 3 months follow-up, the vision in the right eye was 6/9. The lid wound had completely healed (Figure 7.5B).

Discussion Methicillin-resistant Staphylococcus aureus (MRSA) has been recognized as a cause of nosocomial infections since the 1960s. Recently, MRSA infections have been reported among patients with no history of hospitalization. These infections have affected prison inmates, athletic teams, military recruits, children attending day care, and patients within no identifiable risk group. 29,30 Community acquired MRSA (CAMRSA) is no longer a pathogen unique to certain high-risk populations such as prison inmates. Most patients presenting in an outpatient setting with an MRSA soft-tissue infection are not linked to any distinct high-risk group.30 These CAMRSA strains have different genetic background and antibiotic susceptibility profiles than hospital strains. Despite their broader antibiotic susceptibility, in comparison to hospital-acquired strains, CAMRSA strains can cause severe infections, such as necrotizing pneumonias, large soft-tissue abscesses, and necrotizing fasciitis, in otherwise healthy patients.

Figure 7.5B: CAMRSA orbital cellulitis: External photograph, 3 months postorbitotomy, showing complete resolution of the condition and complete healing of the lid wound

CAMRSA causing orbital cellulitis is rare, but with an increasing incidence.31 CAMRSA is geographically widespread, as it has been reported in many regions of the US as well as Europe, Japan, and Australia.30, 31 Within the orbit; this infection is extremely virulent and being resistant to most antibiotics, causes severe damage. We report a rapidly evolving orbital cellulitis caused by CAMRSA. A rapidly evolving orbital cellulitis with an abscess in an adult from any cause should undergo prompt surgical drainage and treatment with susceptible antibiotics. So in this respect a MRSA orbital cellulitis is no different. The real teaching point in this case is to include MRSA as a significant possible cause of the cellulitis and to start empiric therapy that includes coverage for MRSA, until antibiotic susceptibilities come back. It is further recommended to maintain suspicion for MRSA in community patients with possible staphylococcal infection, failing β-lactam class antibiotic therapy, to obtain culture and sensitivity studies in cases of severe ophthalmic infections, and to be informed about the rates of MRSA in the local community.

REFERENCES 1. Henning Bier, and Uwe Ganzer. Involvement of the orbit in diseases of the paranasal sinuses. Neurosurgical Review 1990;13: 109-12. 2. Ambati BK, Ambati J, Azar N. Periorbital and orbital cellulitis before and after the advent of Haemophilus influenzae type B vaccination. Ophthalmology 2000; 107: 1450-53. 3. Kronish JW, Jhonson TE, Gilberg SM, et al. Orbital infections in patients with human immunodeficiency virus infection. Ophthalmology 1996;103:1483-92. 4. Smith AF, Spencer JF. Orbital complications resulting from lesions of the sinuses. Ann Otol Rhinol Laryngol 1948; 57: 5-27. 5. Chandler JR, Langenbrunner DJ, Stevens ER: The pathogenesis of orbital complications in acute sinusitis. Laryngoscope 1970; 80: 1414-28. 6. Uzcategui N, Warman R, Smith A, Howard CW. Clinical practice guidelines for the management of orbital cellulitis. J Pediatr Ophthalmol Strabismus 1998;35:73-79. 7. Givner, Laurence B. Periorbital versus orbital cellulitis. Concise Reviews Of Pediatric Infectious Diseases. Pediatric Infectious Disease Journal 2002.21:1157-58. 8. Shovlin JP. Orbital infections and inflammations. Curr Opin Ophthalmol. 1998; 9: 41-48.

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Orbital Infections 127 9. Mannor G. Unusual orbital infections: Oculoplastic and orbital surgery. Current Opinion in Ophthalmology 2000;11: 357-60. 10. Shuttleworth GN. (Clinical review)Orbital trauma.do not blow your nose. BMJ 1999;318:1054-55. 11. Wald ER. Periorbital and Orbital Infections. Pediatrics in Review 2004; 25: 312-20. 12. Jeyaratnam D, Reid C, Kearns A, Klein J. Community associated MRSA: an alert to paediatricians. Arch Dis Child 2006; 91: 511-12. 13. Hendrickson RG, Olshaker J, Duckett O. Rhinocerebral mucormycosis: a case of a rare, but deadly disease. J Emerg Med 1999;17:641-45. 14. Ferry AP, Abedi S. Diagnosis and management of rhinoorbitalcerebral mucormycosis. A report of 16 personally observed cases. Ophthalmology 1983;90: 1096-104. 15. Mortada A. Orbital pseudo-tumors and parasitic infections. Bull Ophthalmol Soc Egypt 1968; 6: 393-9. 16. Mun-Wai L, Kee-Siew F, Li-Yang H, Wee-Kiak L. Optic nerve toxoplasmosis and orbital inflammation as initial presentation of AIDS. Graefe's Archive for Clinical and Experimental Ophthalmology 2006;244:1542-44. 17. Luis Gorospe, Aránzazu Royo, Teresa Berrocal, GarciaRaya P, Moreno P, Abelairas J. Imaging of orbital disorders in pediatric patients. European Radiology 2003;13:2012- 26. 18. Wendy L. Wright MS. Viral or Acute Bacterial Rhinosinusitis? Determining the Difference. The Nurse Practitioner: The American Journal of Primary Health Care 2005;30:30-41. 19. D B Jones and P G Steinkuller. Strategies for the initial management of acute preseptal and orbital cellulitis. Trans Am Ophthalmol Soc. 1988;86:94-112. 20. Urschel JD. Necrotizing soft tissue infections. Postgrad Med J 1999;75:645-49.

21. Donahue SP, Schwartz G. Preseptal and orbital cellulitis in childhood. A changing microbiologic spectrum. Ophthalmology 1998; 105:1905-06. 22. Kronish JW, McLeish WM. Eyelid necrosis and periorbital necrotizing fasciitis. Report of a case and review of the literature. Ophthalmology 1991; 98; 92-8. 23. Luksich JA, Holds JB, Hartstein ME. Conservative management of necrotizing fasciitis of the eyelids. Ophthalmology 2002; 109; 2118-22. 24. Seal DV. Necrotizing fasciitis. Curr Opin Infect Dis 2001; 14: 127-132. 25. Aggarwal D, Suri A, Mahapatra AK. Orbital tuberculosis with abscess. J Neuro-Ophthalmol 2002; 22: 208-10. 26. Biswas J, Shome D. Choroidal tubercles in disseminated tuberculosis diagnosed by the polymerase chain reaction of aqueous humor: a case report and review of the literature. Ocul Immunol Inflamm 2002;10: 293-98. 27. Sen DK. Tuberculosis of the orbit and lacrimal gland: a clinical study of 14 cases. J Pediatr Ophthalmol Strabismus 1980;17:232-38. 28. Shome D, Honavar SG, Vemuganti GK, Joseph J. Orbital tuberculosis manifesting with enophthalmos and causing a diagnostic dilemma. Ophthal Plast Reconstr Surg 2006; 22: 219-21. 29. Weber JT. Community-associated methicillin-resistant Staphylococcus aureus. Clin Infect Dis 2005; 41: S269 - 72. 30. Rutar T, Chambers HF, Crawford JB, Perdreau-Remington F, Zwick OM, Karr M, Diehn JJ, Cockerham KP. Ophthalmic manifestations of infections caused by the USA 300 clone of community-associated methicillin-resistant Staphylococcus aureus. Ophthalmology 2006;113:1455-62. 31. Rutar T, Zwick OM, Cockerham KP, Horton JC. Bilateral blindness from orbital cellulitis caused by communityacquired methicillin-resistant Staphylococcus aureus. Am J Ophthalmol 2005;140:740-2.

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128 Surgical Atlas of Orbital Diseases

8

Orbital Inflammatory Disease

CHAPTER E Ravindra Mohan, Moupia Goswami, Vinathi Mutyala

The orbits represent a microcosm of the body in terms of tissues present-muscle, adipose tissue, blood vessels, nerves, skin and bone as also the eyeball with its unique architecture, histology and spectrum of diseases affecting it. Inflammatory conditions of the orbit represent the commonest afflictions of the orbits and thyroid associated orbitopathy and infections constitute the bulk of these. Excluding the above causes of orbital inflammation, the causative entities range from vasculitis, like Wegener’s granulomatosis to granulomatous conditions like sarcoidosis and the entity of idiopathic orbital inflammation, earlier popularly labelled as pseudotumor. As evident from the diversitry of underlying causes, the clinical picture, natural history, treatment and outcome of these conditions vary greatly. The age profile of patients with orbital inflammation also varies greatly, ranging from the paediatric age group for juvenile xanthogranuloma to adulthood, mostly the 3rd-5th decades of life for the vast majority of inflammatory conditions. Orbital inflammatory disorders are less common in the elderly, and must be diagnosed only after ruling out metastatic disease and infections, by tissue diagnosis if needed. Orbital inflammation affects both sexes and all races across continents. Broadly speaking, all patients with orbital inflammation present with one or more of the inflammatory symptoms of pain, swelling around the eye, proptosis, double vision or reduced vision, redness or watering. Pain is a common symptom and

is variably described as dull, aching or throbbing and is poorly localized, with headache being a common complaint. Periocular swelling and puffiness, more pronounced in the mornings after rising from sleep is not uncommon. While double vision is reported by some of these patients, it is a primary complaint only in a small proportion of the patients and at times elicited only on examination. Orbital disease, particularly resulting from inflammatory conditions is one of the few remaining areas in the practice of ophthalmology, where detailed and meticulous history taking still has an important role and bearing on arriving at a diagnosis. Details of symptoms, associated systemic features, response to medications and side effects of treatment are important areas which need to be probed carefully. For example, merely knowing that a patient’s orbital symptoms improved on oral steroids may be inadequate to arrive at a diagnosis of idiopathic orbital inflammation. If the details are sought, and suggest rapid relief starting within hours, and near total resolution, one would be more definite in making the diagnosis. Examination of a patient with orbital inflammatory disease needs to be done with meticulous attention to detail. Careful documentation including photographic documentation is invaluable in studying the natural history of an individual patient’s condition as also in prognostication and in titrating medical therapy. Close co-ordination with other specialists treating a patient is clearly essential in the management of patients with orbital inflammatory

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Orbital Inflammatory Disease 129 conditions. In addition to primary care givers, like rheumatologists in managing a patient with a vasculitic condition, other specialists often need to be involved in care as the disease progresses or complications develop as a result of medical therapy, as in the need for orthopaedic care for aseptic necrosis of neck of femur developing from prolonged systemic steroid therapy. Overall, the disease entities covered in this chapter are diverse and may have little in common except the propensity to produce orbital inflammation. Achieving a precise diagnosis, by obtaining orbital tissue for diagnosis if needed, is vital. With present day techniques and instrumentation, obtaining an adequate specimen for histopathological and immunohistochemical diagnosis is safe. Except for cases where the disease is localized to a relatively inaccessible region of the orbit like the orbital apex, or resolves fully on medical therapy based on presumptive diagnosis, an incisional biopsy is essential to achieve diagnosis and rule out other causes for orbital inflammation like masquerade syndrome related to malignant tumor. A fine needle aspiration biopsy is often inadequate for the purpose. The treatment of orbital inflammations remains centred on the control of inflammation and prevention of the sequelae of persistent and prolonged inflammatory reaction. A host of immunosuppressive drugs like cyclosporine, methotrexate and cyclophosphamide are used in addition to intravenous methylprednisolone and the widely used oral steroid medications. Surgery in patients with orbital inflammatory disease is primarily to obtain tissue diagnosis. Surgical debulking of the involved orbital tissue is rarely needed, and the use of destructive operations like orbital exenteration is only in extremely disfigured orbits with no visual potential and severely troubling symptoms. Radiation therapy has limited role. Treatment of orbital inflammatory disease needs to be tailored and titrated to the individual disease entity and patient. Since inflammation often tends to relapse and recur, long term follow-up is needed as also monitoring to assess for complications of orbital disease like ocular motility restriction or complications of therapy, like steroid induced cataract.

The prognosis for patients with orbital inflammatory disease is variable and depends on the underlying disease and its severity.

ORBITAL AMYLOIDOSIS Amyloidosis refers to a heterogenous group of disorders of protein metabolism characterized by the extracellular deposition of abnormal insoluble protein fibrils. Deposition of amyloid in the eye and its adnexal structures may occur as part of systemic amyloidosis or as a localised form. Local orbital amyloidosis is a rare condition, comprising only 4% of cases of local amyloidosis seen in the head and neck regions.1 Ocular findings in primary generalised amyloidosis include purpura of the eyelids, which can frequently be the presenting sign; bilateral, symmetrical, small amyloid papules of the skin of the eyelids, nodules in the lids, ptosis; proptosis, globe displacement with or without visual impairment, ophthalmoplegias or amyloid neuropathy affecting pupillary function or both and subconjunctival hemorrhages. Histologically the specimen shows fibrous connective tissue and massive amyloid deposit infiltrated with lymphocytes, plasma cells, and foreign body giant cells. Amyloid deposits are identified histologically by congo red staining (Figure 8.1) and viewing under polarized light where amyloid deposits produce a distinctive ‘apple green birefringence’. The pathogenic mechanisms leading to local tissue deposition of amyloid are not clear. The universal constituent of amyloid is the amyloid P component (AP). It is derived from normal

Figure 8.1: Congo red staining showing amyloid (Congo red x 200)

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130 Surgical Atlas of Orbital Diseases circulating plasma protein, serum amyloid P component (SAP). The isolated pure human SAP radiolabelled with I 123 is a highly specific tracer for all types of amyloidosis.2 The best method of orbital imaging is CT scan, for its ability to detect calcification which differentiates it from other lesions. Radionuclide SAP scans help in anatomical localisation of amyloid deposits. Management of orbital amyloidosis is difficult. Standard treatment aims to reduce reproduction of the monoclonal immunoglobulin precursor via chemotherapy or radiotherapy or surgery of the localized lesion. Total excision is usually difficult and surgery is aimed at debulking the mass with preservation of palpebral gland of the lacrimal gland, levator and rectii muscles.

It is mainly a diagnosis of exclusion based on clinical picture, laboratory tests, biopsy and radiologic evidence. Up to 90 percent of patients with ocular sarcoid have abnormal chest radiographs. Hilar lymphadenopathy is seen (Figures 8.2A and B). Lung biopsy by tracheobronchial fiber optic techniques is 90 percent accurate. Biopsy of an enlarged, potentially infiltrated lacrimal gland or conjunctival granuloma is an acceptable alternative.

REFERENCES 1. Gean-Marton AD, Kirsh CFE, Vezina LG, et al. Focal amyloidosis of the head and neck: evaluation with CT and MR imaging. Radiology 1991;181:521-5. 2. Murdoch IE,Sullivan TJ, Moseley I, Hawkins PN, Pepys MB, Tan SY, Gamer A, Wright JE. Primary localised amyloidosis of the orbit. Br J Ophthalmol 1996;80:1083-6.

SARCOIDOSIS Sarcoid (from the Greek ‘sark’ and ‘oid’ meaning “flesh-like”) or Besnier-Boeck disease or Schaumann’s syndrome. Sarcoidosis is an idiopathic chronic non necrotizing granulomatous multi-systemic disease that affects skin, brain, eyes, lungs, spleen, thyroid, and liver. It commonly affects young adults, who frequently present with hilar lymphadeno pathy, pulmonary infiltration, ocular and cutaneous lesions. Ocular involvement manifests in 25-60% of patients with systemic sarcoidosis. 1 The most common ocular manifestation in sarcoidosis is uveitis and the most common orbital manifestation is dacryoadenitis, which is frequently bilateral.2 Other manifestations include eyelid swelling and palpable eyelid masses, conjunctival nodules, retrobulbar masses, proptosis, optic nerve, chiasma or sheath involvement, optic radiation infiltration, bone destruction and rarely extraocular muscle involvement.

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B Figures 8.2A and B: CT chest showing hilar lymphadenopathy in sarcoidosis

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Orbital Inflammatory Disease 131 Typical histologic findings from biopsy include accumulation of T lymphocytes and mononuclear phagocytes, diffuse non-caseating epitheloid granulomas and derangements of the normal tissue architecture. Infectious etiologies need to be excluded by culture and/or staining. Corticosteroids are the mainstay of treatment in symptomatic cases. Severe symptoms are generally treated with steroids. In cases of steroid intolerance or resistance, steroid sparing agents such as azathioprine and methotrexate are often used. Cyclophosphamide has also been used. As the granulomas are caused by collections of immune system cells, particularly T cells, there has been some early indication of success using immunosuppressants, interleukin 2 inhibitors or anti tumor necrosis factor-alpha treatment (such as infliximab). Lofgren’s syndrome represents an acute presentation with erythema nodosum, bilateral hilar lymphadenopathy and polyarthralgia. This entity has a relatively good prognosis. The combination of anterior uveitis, parotitis and fever is called uveoparotid fever and in association with cranial nerve palsies is referred to as HeerfordtWaldenstrom syndrome.

REFERENCES 1. Hunter DG, Foster CS. Ocular manifestations of sarcoidosis. In: Albert DM, Jakobiec FA, eds. Principles and practice of ophthalmology. Philadelphia: WB Saunders, 1994; 443-50. 2. Jakobiec F, Bilyk JR, Font RL Non infectious orbital inflammations. In: Spencer WH, editor. Ophthalmic pathology - An Atlas and Textbook. WB Saunders, Philadelphia, 1996; 2810-58.

NONSPECIFIC ORBITAL INFLAMMATORY SYNDROME (NSOIS) Nonspecific orbital inflammatory syndrome (NSOIS), commonly referred to as Idiopathic Orbital Inflammation, Orbital Pseudotumor is defined as a benign, non infective clinical syndrome characterized by features of nonspecific inflammatory conditions of the orbit without identifiable local or systemic causes. Idiopathic orbital inflammation is the third most common non infectious orbital disease, following Graves’s orbitopathy and lymphoproliferative diseases. It accounts for 4.7 to 6.3% of orbital disorders.1

Idiopathic orbital inflammation has highly variable clinical features, from a diffuse to very focal process targeting specific orbital tissues, such as the lacrimal gland, extraocular muscles, optic nerve and orbital fat. Presentations vary according to the specific location and the degree of inflammation, fibrosis, and mass effect. Ptosis, chemosis, motility dysfunction, and optic neuropathy may also be found. Entrapment, compression, and destruction of orbital tissues may occur in patients with extensive sclerosis. Unilateral presentation is typical, but bilateral presentations are not uncommon. Radiological imaging studies allow tissue characterization and localization without surgical intervention and thereby have become invaluable diagnostic tools. Computed tomography is the preferred mode of imaging. Idiopathic orbital inflammation is typically seen on CT scans as a focal or diffuse mass, usually poorly demarcated and enhancing with contrast. Common CT findings include enhancement with contrast medium, infiltration of retrobulbar fat, proptosis, extraocular muscle enlargement, muscle tendon or sheath enlargement, apical fat edema, optic nerve thickening, uveal-scleral thickening, edema of the Tenon capsule, and lacrimal gland infiltration. Tendons of the extraocular muscles may be involved or spared. The histopathological spectrum of idiopathic orbital inflammation is typically non diagnostic, wide, and diverse, ranging from the typical diffuse polymorphous infiltrate to the atypical granulomatous inflammation, tissue eosinophilia, and infiltrative sclerosis. In the absence of systemic fibro inflammatory, granulomatous, and vasculitic disease, these atypical presentations are considered to be subclasses of idiopathic orbital inflammation.2 NSOIS respond rapidly to high dose steroid therapy in tapering doses but recurrences are common. In such cases, chemotherapy (e.g. methotrexate, cyclosporine) and low-dose radiation (e.g. 1500-2500 cGy EBRT) may be needed to control the inflammation.3

REFERENCES 1. Henderson JW: Orbital tumors, Newyork, Ravin press, 1994, (3rd ed), pp 13-14; 47;317-411. 2. Root man J: The classification and management of acute orbital pseudotumors: Ophthalmology 1982, 89;1040-48. 3. Leone C: Treatment protocol for orbital inflammatory diseases; Ophthalmology 1985, 92; 1325-31.

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132 Surgical Atlas of Orbital Diseases

KIMURA’S DISEASE Kimura’s disease is a chronic inflammatory disorder of uncertain etiology which typically presents as multiple cutaneous nodules in the head and neck region particularly the preauricular regions. It was first described in China in 1937 by Kim and Szeto as eosinophilic lymphogranuloma.1 Kimura’s disease is most commonly seen in patients between 20-40 years of age with a striking male predominance and is endemic among the oriental population. It is characterized by a triad of insidious onset of painless subcutaneous nodules in the head and neck region, blood and tissue eosinophilia and markedly elevated serum immunoglobulin levels.2 In the orbit, presentation is in the form of proptosis, upper lid swelling and orbital masses usually in the lacrimal gland. The histomorphology of Kimura’s nodule is characterized by intense infiltration of lymphocytes, vascular proliferation and plasma cells with a variable number of lymphoid follicles with germinal centers. Typically, there is a moderate to intense eosinophilic infiltration mainly in a perivascular pattern. Immuohistochemical stains would typically show IgE reticular network in the germinal centers.3 Following initial presentation, surgical excision and biopsy with debulking is the preferred mode of treatment, but recurrence is common. Other treatment options include radiation, systemic corticosteroids, cyclosporine and pentoxyfylline.

REFERENCES 1. Kim HT, Szeto C. Eosinophilic hyperplastic lymphogranuloma, comparison with Mikulicz’s disease. Chin med J. 1937 23:699-700. 2. Hui PK, Chan JK, Ng CS, Kung IT, Gwi E. Lymphadenopathy of Kimura’s disease. Am J Surg Pathol. 1989; 13:177-86. 3. Motoi M, Wahid S, Horie Y, Akagi T. Kimura’s disease: clinical, histological, and immunohistochemical studies. Acta Med Okayama.1992;46:449-55.

WEGENER’S GRANULOMATOSIS Wegener’s granulomatosis (WG) is a fulminant systemic disease of unknown aetiology consisting of necrotizing granulomatous vasculitis of the upper and

lower respiratory tracts, focal necrotizing glomerulonephritis, and systemic small vessel vasculitis involving multiple organ systems. Incidence of ocular involvement in WG varies from 29 to 79%.1, 2 Ocular involvement can be either due to an extension from the adjacent paranasal sinuses (contiguous) or as a result of focal vasculitis (noncontiguous).1,2 Presentation can be in the form of proptosis, dacryocystitis, scleritis with peripheral keratopathy, kerato-conjunctivitis sicca, uveitis, retinitis, retinal vascular occlusions, exudative retinal detachments, and optic neuritis. Laboratory findings support or confirm the diagnosis of WG. In the active stage of the disease raised ESR and leucocytosis are seen. Chest X-ray and computerised tomography detect pulmonary involvement. Routine urine analysis detects renal involvement. Serum antibodies against the cytoplasmic component of neutrophils and monocytes (cANCA) form a useful adjunct in the diagnosis of WG. Indirect immunofluorescence is currently the standard test for ANCA screening.3 Between 80% and 95% of all ANCA found in WG is cANCA. Diagnosis is established by biopsy in orbital and paranasal sinus involvement. The characteristic histopathologic picture is that of necrotising vasculitis of the blood vessels, usually with granuloma formation in the surrounding infiltrates. Management of WG requires a multisystem approach. Oral corticosteroids along with a cytotoxic agent, of which cyclophosphamide is the most efficacious, is the treatment of choice. Early treatment with cyclophosphamide and corticosteroids reduces both ocular and systemic morbidity. Exacerbations are common in the first two years after diagnosis. In cases of remission, azathioprine, cyclosporine A and methotrexate may be used.

REFERENCES 1. Straatsma BR. Ocular manifestations of Wegener’s granulomatosis. Am J Ophthalmol 1957; 144:789-99. 2. Bullen CL, Liesegang TJ, McDonald TJ, DeRemee RA. Ocular complications of Wegener’s granulomatosis. Ophthalmology 1983;90:279-90 3. Harman LE, Margo CE. Wegener’s granulomatosis. Survey of Ophthalmology 1998; 42:458-80.

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Orbital Inflammatory Disease 133

LANGERHANS’ HISTIOCYTOSIS Langerhans’ cell histiocytosis (LCH) is a group of idiopathic disorders characterized by the proliferation of specialized bone marrow–derived Langerhans’ cells (LCs) and mature eosinophils. These can be subdivided into three clinico-pathological entities: acute disseminated LCH, unifocal and multifocal unisystem LCH, and multisystem LCH. There are three forms of presentation. Eosinophilic Granuloma: single organ involvement. Hand-Schuller-Christian Syndrome: lytic bone lesions, diabetes insipidus and exophthalmos. Letterer-Siwe disease: severest form of the disease found in infants, involving lesions in the liver, bone marrow, spleen and skin. Langerhans’ cell histiocytosis (LCH) accounts for less than 1% of all orbital tumours. Orbital involvement in LCH is characterised by osteolytic lesions with sclerotic margins along with soft tissue involvement. The typical cytopathological picture consists of Langerhans’ cells along with eosinophils and a varying number of neutrophils, lymphocytes, macrophages and multinucleated giant cells with pale ill-defined eosinophilic cytoplasm and lobulated nuclei with longitudinal grooves, best visualized in Papanicolaou-stained smears.1 A definitive diagnosis is made by presence of Birbeck granules on electron microscopy (rod-like structures with a striated core having dilated ends giving a tennis racket appearance) or positivity for CDI antigen determinants on cryostat sections. In an appropriate clinicoradiological setting, a typical pathology alone can be used for effective diagnosis and definite proof of LCH.2 Management modalities vary from observation, curettage, intralesional steroids, low-dose radiation, high-dose systemic corticosteroids and chemotherapy, bone marrow transplantation and antibody therapy for recalcitrant cases. The most effective treatment is chemotherapy with Vincristine, Vinblastine, Etoposide and steroids. Low dose radiation in 4-6 fractions may be used when the disease is extensive, inaccessible or if it threatens an important organ.3

REFERENCES 1. Ayala AG, RO-JY, Famming CV, Flores JP, Yaskee AW. Core needle biopsy and fine needle aspiration in diagnosis of bone and soft tissue lesions. Hematol Oncol Clin North Am Jun 1995; 9:633-51. 2. Pohar-Marinsek Z, Us-Krasovec M. Cytology of Langrehans cell histiocytosis. Acta Cytol 1996; 40:1257-64. 3. Sessa S, Sommelet D, Lascombes P, Prevol J. Treatment of Langerhans cell histiocytosis in children - Experience at the children’s Hospital of Nancy. J Bone Joint Surgery-Am 1994; 76:1513-25.

ROSAI-DORFMAN DISEASE Synonyms: Sinus Histiocytosis with Massive Lymphadenopathy, Destombes Rosai-Dorfman disease. Sinus Histiocytosis with Massive Lymphadenopathy (SHML) otherwise known as Rosai Dorfman Disease, (RDD), is a rare, benign systemic, idiopathic reactive proliferation of distinctive histiocytes, characterised by massive lymphadenopathy, particularly in the head and neck region, and often associated with extra nodal involvement. The orbit is a common extranodal site of RDD.1 Widespread dissemination with liver, kidney, respiratory organs, orbit, and eyeball involvement has been reported rarely. Lymphoproliferation in the soft tissues of the orbit and in the lids has been reported in 12% of cases but intraocular involvement is rare. The histologic hallmark of sinus histiocytosis with massive lymphadenopathy (SHML) are large intrasinusoidal cells exhibiting cytophagocytosis. Microscopic examination of the lymph nodes shows a polymorphous infiltrate composed of plasma cells, neutrophils, lymphocytes, and histiocytes. The histiocytes often contain phagocytised lymphocytes, a histological finding termed emperipolesis. An immunohistochemical staining panel that includes CD31 and S100 facilitates the diagnosis of SHML.2 Most effective regimen is a vinca alkaloid combined with an alkylating agent and a corticosteroid. The most commonly used regimen is a combination of cyclophosphamide, vincristine, mercaptopurine, and prednisolone. Treatment causes regression of the tumor and resolution of

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134 Surgical Atlas of Orbital Diseases lymphadenopathy with minimal recurrence. Surgery is indicated rarely, in life or function threatening situations.

REFERENCES 1. Friendly DS, Font RL, Rao NA. Orbital involvement in ‘sinus’ histiocytosis. Arch Ophthalmol 1977;95:2006–11. 2. Slone SP, Fleming DR, Buchino JJ. Arch Pathol Lab Med 2003 Mar;127(3):341-4.

ORBITAL XANTHOGRANULOMA Adult xanthogranulomatous diseases are nonLangerhans histiocytic disorders (type II) involving the ocular or orbital tissues and constitute a group of entities with varying manifestations. They are adult onset xanthogranuloma (AOX), which is isolated with no systemic associations, adult onset asthma with periocular xanthogranuloma (AAPOX), necrobiotic xanthogranuloma (NBX) and ErdheimChester disease (ECD). Juvenile xanthogranulomas usually present with skin or intraocular lesions and orbital involvement which is rare, occurs almost exclusively in children.1 Ocular involvement maybe in the form of eyelid or orbital mass, proptosis, orbital bone mass and extraocular muscle involvement,epibulbar mass, uveal mass, uveitis and rarely retinal and choroidal involvement. While the orbit or adnexal xanthogranuloma tends to be anterior in AOX, AAPOX, and NBX, it is often diffuse in ECD and leads to visual loss. Diagnosis is confirmed by biopsy. The characteristic appearance of xanthogranulomas on histopathology is proliferation of histiocytes, plasma cells and lymphocytes with Touton giant cells that stain positive for lipid. Touton giant cells are multinucleate cells with the nuclei arranged in a wreath around a nidus of eosinophilic cytoplasm and separated from the cell membrane by a rim of translucent foamy cytoplasm.2 Necrosis (necrobiosis) with pallisading epitheliod histiocytes is mostly seen in NBX whereas large lymphoid aggregates with germinal centers are often found in cases of AAPOX. ECD exhibits florid fibrosis with fewer follicles and more dispersed lymphocytes and lipid laden histiocytes.

The clinical course is chronic and often progressive. Various treatment modalities include local excision, periocular and systemic steroids, chemotherapy with low dose chlorambucil, nitrogen mustard, cyclophosphamide, melphalan, local radiation and plasma exchange.

REFERENCES 1. Zelger B, Cerio R, Orchard G, et al. Juvenile and adult xanthogranuloma. A histological and immunohistochemical comparison. Am J Surg Pathol 1994;18:126–35. 2. Murthy R, Honavar SG, Vemuganti GK, Naik M, Burman S. Isolated giant xanthogranuloma of the orbit. Indian J Ophthalmol [serial online] 2007 [cited 2007 Jul 22]; 55: 156-58.

CASE ILLUSTRATIONS Case 1 An 18-year-old female, presented with recurrent swelling of the right upper lid of 4 months duration.There was no history of pain, redness, diplopia or defective vision. A cystic mass was excised elsewhere 8 months back, histopathology reports were not available. On examination, best corrected visual acuity in both eyes was 6/6,N6. Ocular movements were full and painless.There was fullness of the upper temporal part of the right orbit. A firm to hard cord like mass was felt preseptally. The mass was adherent to the bone at the lateral canthus. (Figure 8.3) There was no lymphadenopathy. CT scan (Figure 8.4) showed enlarged mass, not separate from lacrimal gland, surrounding the globe. It was heterogenous in density.

Figure 8.3: Showing fullness of upper temporal part of the right orbit in a patient with sarcoidosis

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Orbital Inflammatory Disease 135 Blood tests for serum calcium, phosphorus, proteins, angiotensin converting enzyme and serum lysozyme were within normal limits. No systemic medications were started in view of normal blood investigations. The patient was asymptomatic and there was no recurrence during the postoperative follow up of two years.

Case 2

Figure 8.4: CT scan showing anteriorly located heterogenous mass in the upper temporal quadrant in patient with sarcoidosis

Total excision of the mass was performed through anterior orbitotomy.Intraoperatively the mass was found to be arising from the palpebral part of the lacrimal gland. Histopathological examination revealed chronic granulomatous inflammation with no evidence of caseation. Large amounts of granuloma with epitheloid cells and giant cells were observed, suggestive of sarcoidosis (Figure 8.5). Special stain for fungus and acid fast bacilli were negative.

Figure 8.5: Photomicrograph showing non-caeseating granulomas (Hematoxylin Eosin x 200)

A male farmer, 45 years of age, presented with complaints of protrusion of right eyeball for the past 2 months. His general health was good. Previous thyroid profile was normal. Examination revealed best corrected visual acuity of 6/6p, N6 and 6/5, N6 in the right and left eyes respectively. There was periocular fullness and inferior scleral show of 2 mm.The globe was pushed forwards by 9 mm,outwards by 3 mm and upwards by 2 mm. Ocular motility was normal. There was no relative afferent papillary defect.There was increased resistance to retropulsion (Figure 8.6). A firm mass was palpable in the inferomedial orbit, posterior extent of which could not be felt. Slit lamp examination of the anterior segment and intraocular pressure were within normal limits.Fundus examination revealed disc edema with no choroidal folds. CT scan revealed an illdefined heterogenous mass lesion in the right inferomedial orbit extending upto the apex.Medial rectus and inferior rectus were included in the mass lesion (Figures 8.7 and 8.8). We did a right orbital biopsy through a subciliary incision, with debulking of the tumor under general anesthesia. Intraoperatively an infiltrating grey white firm mass was seen, which

Figure 8.6: Photograph showing periocular fullness, proptosis and scleral show in a patient with nonspecific inflammation of right orbit

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136 Surgical Atlas of Orbital Diseases followed by 400 mg BD for one month. There was significant clinical improvement postoperatively with vision improving to 6/6, and complete resolution of proptosis.

Case 3

Figure 8.7: Axial CT scan showing illdefined heterogenous mass lesion extending upto apex in a patient with nonspecific inflammation

A young female aged 15 years, presented with small swelling in the upper outer quadrant of the left eye since 2 years. The swelling was progressively increasing in size. There was no pain, visual disturbance or diplopia. On examination, fullness of superotemporal region with displacement of the globe downwards and medially was seen (Figure 8.9). A soft to firm swelling with illdefined margins was palpable. It was compressible, non reducible, non pulsatile and non tender. There was increased resistance to retropulsion. There was no lymphadenopathy. CT scan revealed well defined, heterogeneously dense, non enhancing enlargement of lacrimal gland. (Figures 8.10A and B). We excised the mass in toto through lateral orbitotomy. Histopathology examination revealed focal collection of chronic inflammatory cells with abundant fibrocollagenous tissue suggestive of nonspecific inflammation of lacrimal gland (Figure 8.11). Postoperatively, patient was symptom free on follow up of 5 years.

Case 4

Figure 8.8: Coronal CT scan - Medial rectus and Inferior rectus muscles involvement in the nonspecific inflammation of orbit

A 32-year-old female presented with complaints of protrusion, pain and redness in the right eye associated with progressive dimunition in vision since 1 year. On examination, the best corrected visual acuity in right eye was 2/60, N36. Marked lid edema and

was difficult to cut and hence was removed piecemeal. Histopathological examination of the specimen was consistent with a diagnosis of non specific inflammation with extensive fibrocollagenous tissue. In view of this finding,ultrasound abdomen was done to rule out retroperitoneal fibrosis. ANCA test was advised to rule out Wegener’s granulomatosis. Both test reports were found to be within normal limits. Patient was started on tapering dose of oral steroids, starting with prednisolone 50 mg/day, reducing it by 10 mg every 3 days and tab pentoxyfylline 400 mg TDS for one month

Figure 8.9: Photograph showing fullness of superotemporal region with downward displacement of the right globe in a patient with nonspecific inflammation of lacrimal gland

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Orbital Inflammatory Disease 137

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B

Figures 8.10A and B: Axial (A) and Coronal (B) sections of CT scan showing well defined, heterogeneously dense nonenhancing enlargement of lacrimal gland in a patient with nonspecific inflammation

Figure 8.11: Photomicrograph showing specimen of lacrimal gland with chronic inflammatory cells surrounding it, suggestive of inflammation of lacrimal gland (Hematoxylin Eosin x 100)

conjunctival congestion was seen, globe was displaced forwards and downwards. Elevation and abduction of the globe was restricted. A palpable mass was felt in the superotemporal quadrant. Preauricular and submandibular lymphadenopathy was present on the same side. Ultrasound abdomen was normal, FNAC showed reactive changes with mild eosinophilia. CT scan showed large extraconal orbital mass surrounding the globe all round, infiltrating the periocular structures (Figure 8.12). We

Figure 8.12: CT scan showing large extraconal orbital mass surrounding the globe and infiltrating the periocular structures in a patient with Kimura's disease

performed a lateral orbitotomy. Intraoperatively, the mass was firm in consistency and was extensively infiltrating the periorbital, lateral orbital and the lacrimal gland region extending upto the apex. Histopathological report confirmed the diagnosis of angiolymphoid hyperplasia with eosinophilia

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138 Surgical Atlas of Orbital Diseases (Kimura’s disease) (Figure 8.13). A course of systemic steroids was given. Visual acuity improved to 6/6, N6 in the affected eye. She came back 4 months later with severe pain and headache, with massive proptosis and keratinization of conjunctiva and cornea. Right eye had no perception of light. Patient was referred to oncologist for chemotherapy and radiotherapy. The response was not satisfactory. In view of the above, exenteration of the right orbit was done. (Figure 8.14) Patient was fitted with a spectacle mounted prosthesis. Recurrence was seen the form of tiny nodular subcutaneous lesions. CT scan evidence of orbital recurrence was seen in the form of soft tissue filling orbit (Figure 8.15). As the lesion was non malignant, it was decided to watch the lesion for 6 months. Patient thereafter was lost

Figure 8.15: Photomicrograph showing blood vessels with scattered eosinophils and lymphocytes in a patient with Kimura's disease (Hematoxylin Eosin x 200)

to follow up. This case shows that some of the benign orbital inflammations may be very aggressive and severe disease may even necessitate orbital exenteration.

Case 5

Figure 8.13: CT scan post exenteration of the patient with Kimura's disease

A male aged 41 years, presented with recurrent left cheek swelling with protrusion of left eye since 5 months. He had undergone partial maxillectomy surgery of the left side 1 year back. On examination, his best corrected vision was 6/9, N6 in both the eyes.Left proptosis with upward displacement of the globe was seen. A firm to hard swelling was palpable in the left inferior orbit and cheek area. Elevation and depression of the left eye was restricted. (Figure 8.16 ).No afferent papillary defect was present. CT scan revealed soft tissue

Figure 8.14: Appearance following orbital exenteration for severe Kimura's disease

Figure 8.16: Photograph showing left proptosis, upward displacement of globe and fullness in periocular area in a patient with Wegener's Granulomatosis

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Orbital Inflammatory Disease 139 lesion in the left maxillary sinus with extraconal extension into the orbit (Figures 8.17A and B). The CT scans showed evidence of partial resolution of findings following treatment with oral steroids (Figures 8.18A and B).

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Histopathological examination showed deposits of fibrocollagenous tissue with areas of necrosis with vessel obstruction and dense collection of chronic inflammatory cells (Figure 8.19). A diagnosis of Wegener’s granulomatosis was kept in mind. Chest

B Figures 8.17A and B: CT scan pre-treatment - showing soft tissue lesion due to Wegener's Granulomatosis in the left maxillary sinus extending to left orbit

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B Figures 8.18A and B: CT scan, post-treatment with systemic steroids in a patient with Wegener's Granulomatosis, showing partial resolution of the lesion

Figure 8.19: Photomicrograph showing diffuse inflammation with granulomatous reaction (giant cell formation). Arrow shows healed vasculitis. This is suggestive of Wegener's Granulomatosis (Hematoxylin Eosin x 100)

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140 Surgical Atlas of Orbital Diseases X-ray was normal. p ANCA and c ANCA were borderline.Patient was evaluated by rheumatologist and based on clinical diagnosis of Wegener’s granulomatosis, was started on oral steroids. Patient had resolution of symptoms and decrease in swelling following treatment.

Case 6 A male child aged 4 years was referred with CT scan of orbit as a case of malignant lacrimal gland tumor. He had painful swelling of right upper lid of 2 months duration and raised ESR. On examination, his best corrected vision was 6/18 in the right eye. Tender

swelling of the right upper lid, more on lateral aspect was noticed (Figure 8.20). There was no proptosis. Ocular motility was normal.Rest of anterior segment and fundus examination was normal. CT scan (Figures 8.21A and B) revealed an irregular heterodense mass in the lacrimal gland region, associated with lysis of lateral wall and temporal part of roof of orbit. With the presumptive diagnosis of Langerhan cell histiocytosis, he underwent a complete evaluation including bone marrow which was normal. Chest X-ray revealed interstitial pneumonia. An anterior orbitotomy revealed yellowish black material. Impression cytology and permanent sections confirmed the diagnosis of Langerhans cell histiocytosis. On pediatrician’s advise, patient was started on oral prednisolone and 6-Mercaptopurine. Patient has been free of symptoms on a followup of 3 years.

Case 7

Figure 8.20: Photograph showing swelling of the lateral part of right upper eyelid in a patient with Langerhans’ cell histiocytosis

A

A male child aged 2 years presented with gradual onset of swelling in the right upper lid since 3 months.There was sudden increase in swelling in the last 2 days. On examination, a well circumscribed, non tender, firm lesion was palpable in the superior orbit.Posterior limit could not be felt (Figure 8.22). CT scan showed heterogenous soft tissue in the

B Figures 8.21A and B: CT scan shows an irregular heterodense mass in the lacrimal gland area associated with lysis of lateral wall and temporal part of roof of orbit in a patient with Langerhans’ cell histiocytosis

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Orbital Inflammatory Disease 141

Figure 8.22: Photograph showing swelling of the right upper eyelid in another patient with Langerhans’ cell histiocytosis

B

A

Figures 8.23A and B: CT scan showing heterogenous soft tissue in the anterior superior aspect of the right orbit (predominantly preseptal) with lytic lesion in the temporal bone in a patient with Langerhans’ cell histiocytosis

anterior superior aspect of the right orbit (predominantly preseptal) with lytic lesions in the temporal bone (Figures 8.23A and B). Anterior orbitotomy with excision of mass was done. It was cream to black in colour and soft in consistency. Histopathological examination showed large collection of histiocytes, in addition to lymphocytes, eosinophils, plasma cells and few multinucleated giant cells (Figure 8.24). He was treated with intravenous steriods followed by oral steroids for one month. There was complete resolution of mass following this. He was asymptomatic during two years of follow-up. Figure 8.24: Photomicrograph showing numerous eosinophils and histiocytes suggestive of Langerhans’ cell histiocytosis (x100,Hematoxylin Eosin)

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142 Surgical Atlas of Orbital Diseases

Case 8 A 31-year-old female, presented with recurrent episodes of swelling and prominence of left eye since 8 months.She had been on steroids off and on. On examination, fullness of left side of face was seen. There was 4mm left axial proptosis. Firm irregular mass was palpable in the left inferior orbit.There was no lymphadenopathy (Figure 8.25). MRI scan showed a huge fusiform retrobulbar mass (Figures 8.26A to D). Histopathological and immunochemistry study of the biopsy specimen revealed features consistent

Figure 8.25: Photographs showing left proptosis, upward globe displacement and fullness of the left side of the face in a patient with Rosai-Dorfman disease

A

B

C

D

Figures 8.26A to D: MRI scans of the patient with Rosai-Dorfman disease shows illdefined extra and intraconal heterogenous mass lesion, isointense in T1 and hypointense in T2 weighted images with homogenous contrast enhancement. Inferior and lateral rectus muscles are thickened and displaced

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Orbital Inflammatory Disease 143

Figure 8.27: Photomicrograph showing histiocytes with lymphocytes and plasma cells in Rosai-Dorfman disease ( x 40, Hematoxylin Eosin)

Figure 8.28: Photograph showing downward displacement of right eyeball, with proptosis in a patient with Juvenile Xanthogranuloma

with Rosai Dorfman syndrome (Figure 8.27). Patient was seen by oncologist who advised radiation to the orbit. Patient refused treatment and was lost to follow-up. She came back one and a half years later with double vision and massive proptosis. There was increased retrobulbar resistance. Disc edema was seen on fundus evaluation. In view of the huge size of the lesion and the high dose of radiation that would be required with its potential complications, it was decided to perform a surgical debulking. Intraoperatively, a firm grey tumor was seen infiltrating the inferior orbit. Inferior rectus was densely adherent to it. Patient was advised tapering dose of oral steroids. Patient was free of symptoms on 2 years of follow-up.

Case 9 A male child, age 1 year, presented with prominence of right eye since last 20 days. There was no pain, redness, fever or weight loss. On examination, there was downward displacement of right eyeball with 4 mm proptosis (Figure 8.28). Resistance to retropulsion was felt. Restriction of elevation and abduction was seen. Fundus examination revealed mild disc edema with dilated tortuous retinal veins. CT scan revealed a large slightly hyperdense retrobulbar mass with excavation of medial wall of orbit (Figure 8.29). The mass was removed piecemeal by anterior orbitotomy. Histopathological examination showed features of juvenile xanthogranuloma (Figure 8.30 ). He was started on tapering dose of

Figure 8.29: CT scan showing a large slightly hyperdense retrobulbar mass with excavation of medial wall in the patient with Juvenile Xanthogranuloma

oral steroids. On 6 months followup, complete resolution of proptosis was seen. He was asymptomatic at follow-up of 3 years.

Case 10 A young female of age 35 years, presented with painless progressive protrusion of both eyes since five years. She had occasional double vision. She had been treated with oral steroids off and on with dramatic improvement in symptoms, and recurrence on stopping the same. She had received anti

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144 Surgical Atlas of Orbital Diseases tuberculosis therapy earlier. She also reported dryness of the mouth. On examination, her best corrected visual acuity was 6/36, N36 and 6/24, N6 in the right and left eye respectively.Axial proptosis was noted with firm palpable orbital masses in both orbits, most prominent in the superonasal orbit.Ocular movements were restricted in all gazes.There was no lagophthalmos (Figure 8.31). Slit lamp examination revealed reduced tear meniscus, superficial punctuate keratopathy and posterior subcapsular cataract in both the eyes. Fundus examination revealed striae in the right eye and choroidal folds in the left eye. Bilateral parotid enlargement was noted on both sides. Thus the

possibility of Sjogren’s syndrome with underlying autoimmune disease was considered. CT scan of the orbit revealed bilateral diffuse illdefined extra and intraconal soft tissue with clumps of calcification within (Figures 8.32A and B). Transeptal orbital biopsy was done under general anesthesia. Histopathological examination suggested xanthogranulomatous inflammation. Medical oncology and dermatology opinion was sought. In view of side effect of long term steroids, patient was started on oral anti-inflammatory drugs and steroid sparing immunosuppressive therapy (Cyclophosphamide, Endoxan 50 mg daily), with biweekly monitoring of WBC and platelet counts. Surgical debulking was not

Figure 8.30: Photomicrograph shows numerous Toutan giant cells, (Hematoxylin Eosin × 40 ) suggestive of Xanthogranuloma

Figure 8.31: Photograph showing bilateral proptosis in a patient with Xanthogranuloma orbit

A

B Figures 8.32A and B: CT scan in the patient with Xanthogranuloma showing bilateral diffuse illdefined periocular, extra and intraconal soft tissue lesion. Extraocular muscles cannot be identified separately.Clumps of calcification noted

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Orbital Inflammatory Disease 145 considered in view of potential risks. Oral antiinflammatory drugs had to be stopped after a month due to increase in dry eye symptoms. On last followup, patient was symptomatically better.The was considerable decrease in proptosis and improvement of extraocular motility. Patient was symptomatic due to dry eyes, but had much reduced proptosis and orbital symptoms.

ACKNOWLEDGEMENTS We are grateful to Dr J Biswas and Dr S Krishna Kumar, Ocular Pathology Service, Sankara Nethralaya for their help with the photomicrographs and Dr Veena Noronha, Radiology Service, Sankara Nethralaya for help in the interpretation of radiologic images. We are also grateful to Dr Nirmala Subramaniam, Emeritus Professor of Oculoplasty, Sankara Nethralaya for providing some of the photographs.

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146 Surgical Atlas of Orbital Diseases

9

Orbital Lymphoma

CHAPTER Christopher s, Laurence Brown, Raghavan Sampath

The Lymphoproliferative diseases, including malignant lymphoma, are not a single disease entity but a collection of disorders ranging from benign reactive hyperplasia, atypical lymphocyte infiltrate to malignant lymphoma.1 Some consider idiopathic orbital inflammation (pseudotumor) as part of the lymphoproliferative disease spectrum. 1 Approximately 75% of patients with purely orbital lymphoma will develop systemic disease, whilst 1-5% of those with systemic disease will have orbital involvement.1 Overall orbital lymphoma accounts for less than 1% of all lymphoma.2 Interestingly those patients with atypical orbital lymphocytic infiltrate are at an increased risk of systemic lymphoma.1 Various classifications of Lymphomas are in vogue. They include the following:

Revised European American Lymphoma Classification (REAL Classification)3 1. Leukemias and Lymphomas of B-cell Origin (Pan B CD 19,20+) A. Indolent B-cell malignancies: (i) Small lymphocytic lymphoma (ii) Hairy cell leukemia (iii) Follicular lymphomas (iv) Lymphoplasmacytoid lymphoma (v) Marginal zone lymphoma. B. Aggressive B-cell malignancies: (i) Diffuse large cell lymphoma (ii) Follicular large cell lymphoma (iii) Mantle cell lymphoma (iv) Burkitt's lymphoma (v) Plasmacytoma / Myeloma

2. Leukemias and Lymphomas of T-cell Origin (CD 2, 7+) A. Indolent T-cell malignancies: (i) T-CLL (ii) Cutaneous T-cell lymphoma (Sezary syndrome) B. Aggressive T-cell malignancies: (i) Peripheral T-cell NHL (ii) Angioimmunoblastic T-cell lymphoma (iii) Intestinal T-cell lymphoma (iv) Adult T-ALL

WHO Classification of NHL 1. B-cell Neoplasms A. Precursor B-cell ALL B. Mature B-cell malignancies: (i) B-cell CLL (ii) Plasmacytoma (iii) Extranodal marginal B-cell lymphoma (iv) Mantle cell lymphoma (v) Follicular lymphoma (vi) Diffuse large B-cell lymphoma (vii) Burkitt's lymphoma (viii) B-cell promyelocytic leukemia (ix) Hairy cell leukemia (x) Lymphoplasmocytic lymphoma (xi) Monocytoid B-cell lymphoma. 2. T-cell Neoplasms A. Precursor T-cell ALL B. Mature T-cell malignancies: (i) Mycosis fungicides

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Orbital Lymphoma 147 (ii) Adult T-cell lymphoma (iii) Anaplastic or Null cell lymphoma (iv) Peripheral T-cell lymphoma, not specified (v) T-cell prolymphocytic leukemia (vi) Aggresive NK cell leukemia (vii) T-cell gruanular lymphocytic leukemia.

Modified Rye's Classification of Hodgkin's Lymphoma4 1. CLASSIC HD: A. Lymphocyte-predominance B. Nodular sclerosis C. Mixed cellularity D. Lymphocyte depletion. 2. Nodular Lymphocyte-predominant HD Clinically it is difficult to differentiate the malignant and non-malignant tumors since age, sex, presenting symptoms and radiographic findings are similar. Even utilising immunohistochemical and molecular biology techniques it is difficult to separate the conditions. 1 Polymerase chain reaction is a method of amplifying target genetic material in tissue samples to identify a gene re-arrangement diagnostic of lymphoma. This is particularly useful when the microscopic morphology resembles chronic inflammation, but the clinical presentation is suggestive of lymphoma.5 Orbital lymphoma is typically a non-Hodgkins B-cell lymphoma arising from mucosa-associated lymphoid tissue (MALTOMA)1,6 and accounts for approximately 10-15%1,7 of all orbital masses. When only malignant masses are taken in to account, they account for 55% of the lesions. 1 Other types of lymphoma identified in the orbit include extranodal marginal zone lymphoma (MZL), follicular (FL), diffuse large B-cell (DLBCL), mantle cell (MCL), Bcell chronic lymphocytic leukaemia (CLL)/small lymphocytic lymphoma, peripheral T-cell lymphoma (PTCL) and natural killer cell lymphoma (NKCL).8 Orbital lymphoma tends to be a bilateral disease and can affect the conjunctiva, lacrimal gland, be found in the nasolacrimal duct, as well as intraconal and extraconal space.1,9 The orbital lesions tend to present insidiously with painless proptosis in the sixth

decade,1,7,10 whilst visual impairment is relatively rare occurring in only 13% of patients. 7 It can rarely present like acute orbital inflammation or cellulitis. MZL has the lowest risk of accompanying extraorbital disease and consequently, the lowest risk of lymphoma-associated death.3 Radiographically orbital lymphomas tend to be homogenious in nature and mould themselves around orbital structures such as the globe and optic nerve. 1,9 Bone erosion is rare although bone destruction can occur with aggressive tumors.7 Radiotherapy is an effective treatment for orbital MALT lymphoma using doses in the order of 30Gy.11,12 The 5 and 10 years survival rates for MALT lymphoma is 100% and 88%.12 Orbital inflammatory disease include a wide spectrum of conditions ranging from idiopathic orbital inflammation to orbital involvement of specific systemic inflammatory disorders such as Wegener’s granulomatosis, sarcoidosis, systemic lupus erythematosus (SLE) or Tolosa Hunt syndrome.13 In the case of Wegener’s granulomatosis and sarcoidosis ocular involvement occurs in approximately 50% of affected subjects. The inflammation may affect multiple or localized orbital tissues and can involve the sclera. 13 Investigations are therefore guided towards identifying these specific diagnoses. These include a battery of blood tests including full blood count (FBC), urea and electrolytes (U&E), C reactive protein (CRP) and/or erythrocyte sedimentation rate (ESR), autoantibody screen, anti-nuclear antibody screen (ANA), ANCA, rheumatoid factor (RhF), thyroid function tests (TFT), thyroid peroxidise antibody screen and serum ACE. Radiological tests include a chest X-ray, and orbital imaging (CT and/ or MRI). An orbital ultrasound scan can be beneficial. Where no underlying cause can be identified the diagnosis of idiopathic orbital inflammation can be made.

When to Suspect Lymphoma • Age of onset: more common in the elderly • Insidious onset • Bilateral disease with no evidence of indentation of the globe (choroidal folds) • Lacrimal gland involvement, lesions are typically firm/rubbery on palpation

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148 Surgical Atlas of Orbital Diseases • Conjunctival involvement, lesions are typically salmon colored • Orbital imaging will reveal the lesion to be moulded around the globe.

When to Suspect Inflammatory Disease

Idiopathic

Orbital

• Onset is usually acute • Symptoms include; pain, erythema, proptosis, diplopia and blurred vision • Involvement of the extraocular muscles and the sclera (scleritis) • Differential diagnosis includes; orbital cellulitis, a systemic inflammatory disease such as Wegeners granulomatosis, sarcoidosis, polyarteritis nodosa and neoplasms such as lymphoma • Investigations include; FBC, U & E, CRP, antibody screen, serum ACE, ANA, ANCA, RhF, thyroid function and thyroid peroxidase antibodies • Imaging; orbital CT and/or MRI scan, ultrasound scan to rule out scleritis and a chest X-ray where sarcoidosis is suspected.

CASE ILLUSTRATIONS Case 1 A 52 years old diabetic female presented to the orbit clinic with enlargement of the left lacrimal gland. Examination revealed proptosis of 5 mm on the left side, whilst oculomotility was full, there was no RAPD and funduscopy was unremarkable. Routine bloods were taken to rule out autoimmune conditions and inflammatory conditions such as Wegner's granulomatosis, systemic lupus erythematosus (SLE), Sjorgren's syndrome, and sarcoidosis. These were all negative. An urgent CT scan revealed a discrete soft tissue mass arising from the left lacrimal gland (Figure 9.1) extending in to the orbit and displacing the lateral rectus muscle. The right orbit was normal. The differential diagnosis included plemorphic adenoma and lymphoma. Excision biopsy via a lateral orbitotomy approach was performed since pleomorphic adenoma was suspected. Initial examination of the biopsy specimen identified a dense exudate of lymphoid cells forming prominent germinal centers. The appearance was

Figure 9.1: Left lacrimal gland enlargement

suggestive of reactive lymphoid change. Subsequent immunohistochemistry identified T and B cell proliferation and in one specimen kappa light chains could be identified. A low grade lymphoma was suspected. Molecular genetics confirmed the diagnosis of low grade B cell lymphoma. The wounds settled well postoperatively and vision was maintained at 6/9 in the left eye and 6/5 in the right. A referral was made to the lymphoma service for a course of radiotherapy. There was no evidence of systemic lymphoma. 40 Gy was given to the right orbit in 20 fractions. Postradiotherapy she suffered from a dry eye and was prescribed lubricants. A combination of radiation and diabetic retinopathy subsequently developed. However there was no evidence of recurrence at 6 years.

Case 2 A 31 years old male attended the eye casualty with a 3 weeks history of bilateral orbital inflammation (Figure 9.2) and right sided proptosis (Figure 9.3). Visual acuity was 6/5 in both eyes and IOPs were normal. Examination revealed bilateral conjunctival chemosis, associated with reduced upgaze and abduction. Fundoscopy was unremarkable. Routine bloods were taken to rule out inflammatory disorders. C-reactive protein and plasma viscosity were elevated as was the white cell count. An urgent MRI scan was arranged which revealed bilateral enlargement of the lacrimal glands (Figure 9.4) suggestive of lymphoma. Urgent lacrimal gland biopsy was performed. Histology confirmed the diagnosis of Hodgkin’s lymphoma and referral to the lymphoma service was made.

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Orbital Lymphoma 149

Figure 9.2: Bilateral orbital inflammation

revealing only chronic inflammation. Lymphoma was still suspected, and after a discussion with the patient, a more extensive orbital biopsy was performed. Histology was once again inconclusive, revealing only scant lymphoid exudates. Subsequent immunohistochemistry identified B and T cells. No evidence of lymphoma was found. Referral was made to the lymphoma team to rule out systemic lymphoma. None was identified. The patient continues to be followed up.

Case 4

Figure 9.3: Right sided proptosis

Figure 9.4: Bilateral lacrimal gland enlargement

Case 3 A 63 years old male was referred to the eye casualty with a 3 month history of diplopia and right sided proptosis. Onset had been gradual and visual acuity was 6/9 in both eyes. Examination revealed 3 mm of proptosis in the right eye which was associated with reduced ocularmotility in all directions of gaze. Intraocular pressure was normal and there was no evidence of an RAPD. Fundoscopy revealed evidence of choroidal folds on the right although both discs were healthy. There was no associated ocular pain or headache and there was no history of weight loss, fever or cough. He was an ex-smoker having stopped 6 years previously. There was no significant past medical history. Routine bloods were taken to rule out systemic causes. Urgent CT and MRI scans were performed, revealing orbital inflammation suggestive of lymphoma. An urgent orbital biopsy was performed within 3 days and the patient started on a reducing dose of steroids postoperatively. Histology was inconclusive

A 26 years old female presented to eye casualty with a three day history of right orbital swelling. Visual acuity was 6/9 in the right eye and 6/6 in the left. There was marked chemosis (Figure 9.5) and proptosis of the right eye associated with and reduced upgaze and adduction. Fundoscopy was normal and there was no evidence of papilloedema. No RAPD was noted. The patient was apyrexial. A diagnosis of orbital cellulitis was made and appropriate treatment commenced. CT scan of the orbits revealed preseptal and orbital inflammation with evidence of mild proptosis. The extraocular muscles and optic nerve were normal and sinuses clear. There was no evidence of any abscess formation. An orbital ultrasound scan was normal. Despite intravenous antibiotics no improvement in the symptoms was seen. Referral was made to the orbital service and idiopathic orbital inflammation diagnosed. Routine bloods were taken to rule out sarcoidosis, Wegeners granulomatosis, and syphilis, along with an auto-antibody screen, full blood count, thyroid function tests and C-reactive protein screen (CRP). All these tests were normal other than an elevated CRP. Systemic steroids were commenced (Prednisolone 1mg/kg) along with a histamine H2 receptor antagonist. Over the next few days the symptoms and eye movements started to resolve.

Figure 9.5: Right sided chemosis and lid edema

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150 Surgical Atlas of Orbital Diseases The patient was discharged home on a reducing dose of steroids. Urgent immunology and rheumatology out patient appointments were arranged. No underlying immunological or rheumatological abnormalities were detected. Three months following the initial episode, the patient returned to eye casualty with a flare up in the left eye, and then a further six months and two years later in the right eye. All episodes settled with a short course of systemic steroids. Biopsy is normally recommended for all suspected idiopathic orbital inflammation but in this particular patient the features were typical with no localising lesion so a biopsy wasn't performed.

Surgical Approach Surgery is generally indicated to obtain tissue for histology and to aid diagnosis of suspicious lesions. Incisional biopsy is the treatment of choice.

Incisional Biopsy The lateral 1/2 of the upper lid skin crease is marked with pen and extended down parallel to the lid margin, level with the lateral canthus, where it is extended in a horizontal plane just past the orbital rim. Local anesthetic is injected subcutaneously. A skin incision is made along line with a cutting diathermy. Both the palpebral and orbital parts of the lacrimal gland are identified and an incisional biopsy is made. Any other suspicious lesions are also biopsied and sent for histology. The deep tissues are closed with 5.0 vicryl and skin closed with 6.0 prolene. Suspicious visible conjunctival (bulbar and palpebral) and sub-conjunctival lesions should also be biopsied. A reducing dose of steroids is given for 18 days along with a histamine H2 receptor antagonist such as ranitidine.

A head-light can be worn throughout the procedure to ensure adequate illumination of the operating field.

REFERENCES 1. Akansel G, Hendrix L, Erickson BA, et al. MRI patterns in orbital malignant lymphoma and atypical lymphocytic infiltrates. Eur J Radiol 2005;53(2):175-81. 2. Norton AJ. Monoclonal antibodies in the diagnosis of lymphoproliferative diseases of the orbit and orbital adnexae. Eye 2006;20(10):1186-8. 3. Jenkins C, Rose GE, Bunce C, et al. Histological features of ocular adnexal lymphoma (REAL classification) and their association with patient morbidity and survival. Br J Ophthalmol 2000;84(8):907-13. 4. Schnitzer B. Classification of lymphomas. CRC Crit Rev Clin Lab Sci. 1978;9(2):123-78. Review. 5. Coupland SE, Krause L, Delecluse HJ, et al. Lymphoproliferative lesions of the ocular adnexa. Analysis of 112 cases. Ophthalmology 1998;105(8):1430-41. 6. White WL, Ferry JA, Harris NL, Grove AS, Jr Ocular adnexal lymphoma. A clinicopathologic study with identification of lymphomas of mucosa-associated lymphoid tissue type. Ophthalmology 1995;102(12):1994-2006. 7. Selva D, Rootman J, Crompton J Orbital lymphoma mimicking optic nerve meningioma. Orbit 2004;23(2):11520. 8. McKelvie PA, McNab A, Francis IC, Fox R, O'Day J Ocular adnexal lymphoproliferative disease: a series of 73 cases. Clin Experiment Ophthalmol 2001;29(6):387-93. 9. Sullivan TJ, Valenzuela AA Imaging features of ocular adnexal lymphoproliferative disease. Eye 2006;20(10): 1189-95. 10. Demirci H, Shields CL, Shields JA, Honavar SG, Mercado GJ, Tovilla JC Orbital tumors in the older adult population. Ophthalmology 2002;109(2):243-8. 11. Bhatia S, Paulino AC, Buatti JM, Mayr NA, Wen BC. Curative radiotherapy for primary orbital lymphoma. Int J Radiat Oncol Biol Phys 2002;54(3):818-23. 12. Hasegawa M, Kojima M, Shioya M, et al. Treatment results of radiotherapy for malignant lymphoma of the orbit and histopathologic review according to the WHO classification. Int J Radiat Oncol Biol Phys 2003;57(1):172-6. 13. Gordon LK Orbital inflammatory disease: a diagnostic and therapeutic challenge. Eye 2006;20(10):1196-206.

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Vascular Lesions of Orbit 151

10

CHAPTER

Vascular Lesions of Orbit Subrahmanyam Mallajosyula, Mohd Javed Ali

To comprehend the vascular lesions of the orbit, it is very important to have embryological, pathological and clinical concepts very clear in mind. There have been numerous ways of classifying these lesions, the most common being to divide them into malformations, shunts and new growths.1 Many entities have been placed under these three headings which we will discuss in detail.

MALFORMATIONS Malformations are present since the time of birth, though they may not manifest at that time. Flat endothelium lines their wall, and in contrast to neoplastic lesions, do not show any growth in-vitro. The orbital society has classified malformations as:2 a. No flow or hemodynamically isolated malformations. For example: Lymphangioma. b. Venous flow malformations. For example: Varices. c. Arterial flow malformations. For example: Cavernous hemangioma. d. Other congenital malformations. For example: Phakomatosis.

Lymphangioma These are benign vascular lesions seen usually in the early childhood and commonly confused with orbital venous anomalies and hemangiomas. Though they are hemodynamically isolated, they arborize the orbit and bleeding into their lumen causing chocolate cyst is not very uncommon. Lymphangiomas often

enlarge during the upper respiratory infections probably due to the inflammatory response of the lymphoid tissue within the lesion.3 Superficial lymphangiomas are lesions of the lid or conjunctiva, readily visible on inspection as multiple serous or blood filled cysts. These are usually purely lymphatic in character. Indication for management is due to cosmetic reasons and can be removed easily.4 Deep Lymphangiomas have in addition venous connections and may cause slowly progressive proptosis. They may present with increase in size during upper respiratory infection. It can also present with sudden proptosis due to bleeding into its lumen causing a chocolate cyst. A significant number of these patients may present with signs of optic nerve compression like decreased visual acuity, dyschormatopsia, diminished contrast and light brightness sensitivity and visual field defects. Imaging modalities used include Ultrasonography, CT or MRI. Of these MRI is the diagnostic modality of choice.1 USG demonstrates cystic masses in the retrobulbar space. CT shows low density masses in intra and extraconal compartments with minimal ring enhancement on contrast. No vascular component is noted on angiography. MRI is useful to delineate the lesions well and is also helpful in timing the chocolate cyst as being acute, subacute or chronic which may have clinical implications.5 Management is usually conservative since spontaneous regression of the cysts is common. Surgery though ungratifying because of incomplete

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152 Surgical Atlas of Orbital Diseases removal and recurrences, should still be carried on in the presence of signs of optic nerve compression. Carbon dioxide and contact Nd:YAG lasers are useful surgical adjuncts.6 Other recent modalities that are gaining upperhand in the initial management includes the use of sclerosing agents. Many sclerosing agents in use include Picibanil (OK-432)7,8 percutaneous ethanol9 and bleomycin.10 Certain other agents like 5% sodium morrhuate 11 and sodium tetradecyl sulfate, 12 considered to be more effective by some for the management of low flow vascular lesions have been recommended as the first line therapy for lymphangiomas.11

Orbital Varices Orbital varices are weakened, dilated segments of orbital venous system. Age at presentation varies from childhood to middle ages .Most of the cases are unilateral and upper nasal quadrant is the favoured site. Clinical signs include visible lesions in the eyelid or conjunctiva, or the patient may present with a non-pulsatile proptosis which is accentuated with increasing venous pressure like while straining, assuming a dependant posture like sitting with a head down position or by a valsalva maneuver. Since the orbital venous channels are devoid of valves, a reversible proptosis occurs.13 Rarely varices may threaten the vision by optic nerve compression due to acute hemorrhage or thrombosis. Chronic lesions may present as enophthalmos.14 Imaging modalities used include CT scan, Doppler ultrasonography and angiography. Doppler demonstrates the flow of blood. Rapid spiral CT during valsalva maneuver shows characteristic enlargement of the engorged varix. Uniform contrast enhancement is seen. Sometimes phleboliths may also be seen. Angiography shows connection of the lesion to the venous system and completely fills up following injection.1 Management is usually conservative. But in the presence of signs of optic nerve compression, surgical removal is attempted .Complete removal is usually not possible since the lesions are friable, unencapsulated and bleed easily. Embolization using coils through a distal vein is another method to diminish symptoms.13

Cavernous Hemangioma Cavernous hemangioma is the most common benign orbital tumor in adults predominantly affecting middle aged females. Most frequently it develops in the intraconal space though it may also develop elsewhere in the orbit.15 The patients present with slowly progressive unilateral axial proptosis which may be associated with decreased visual acuity, hyperopia, optic nerve compression, optic disc edema, choroidal folds and gaze-evoked amarousis, raised intraocular pressure and strabismus. Bilateral cavernous hemangiomas have also been reported.16,17 Imaging modality used commonly is a CT scan which shows a well defined intraconal mass with smooth margins that enhances either homogenously or inhomogenously with intravenous contrast. Sometimes small areas of calcification are seen.18 On MRI the lesion is isointense and hyper intense to the muscle on T1 and T2 weighted images respectively. With Gadolinium contrast, the lesion fills up homogenously.19 Management is surgical excision Lateral orbitotomy is the most common approach if the lesion is intraconal. Anterior orbital approaches are useful for extraconal lesions. Surgical removal is much simpler, since cavernous hemangiomas are well encapsulated. Cryo is a very useful adjunct. In very large lesions, passing a suture through the lesion helps in two ways, for exsanguination of the tumor reducing its size and to hold the tumor.

Other Congenital Malformations Many other congenital malformations commonly termed as phakomatosis and include 'Sturge-Weber Syndrome', 'Wyburn-Mason Syndrome', 'KlippelTrenaunay Syndrome', 'Osler-Weber-Rendu Syndrome' and many more rare malformations.1 Sturge-Weber Syndrome: It is also called as encephalofacial angiomatosis and is a sporadic phakomatosis that involves the leptomeninges, brain and eyes. Struge-Weber is characterized by a naevus flammeus or port wine stain over the area of trigeminal nerve distribution, ipsilateral leptomeningeal hemangiomas and contralateral seizures and hemiparesis. Ophthalmological features

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Vascular Lesions of Orbit 153 include ipsilateral episcleral hemangiomas, glaucomas and diffuse choroidal hemangioma and homonymous hemianopia. Diffuse choroidal hemangioma gives a characteristic 'tomato-catsup' fundus.20 Imaging modalities like CT and MRI are used. CT scan may show 'tramline markings' of cerebral calcification. Management includes the use of Erbium laser for naevus flammeus, external beam radiotherapy for diffuse choroidal hemangiomas and medical control of intraocular pressure followed by a combined trabeculotomy-trabeculectomy for glaucoma.21 Wyburn-Mason Syndrome: This is a rare A-V malformation of retina, optic nerve head and posterior fossa involving a direct communication between arteries and veins without the intervening capillary bed. Orbits are occasionally involved with ipsilateral portwine pigmented naevi over the trigeminal course.1 CT scans may show enlarged optic canal and bony orbit with a poorly defined enhancing mass.22 Klippel-Trenaunay Syndrome: This rare syndrome encompasses cutaneous hemangiomas, venous varicosities and bony and soft tissue hypertrophy of usually a single limb. Rarely is the orbit involved with vascular anomalies.23

SHUNTS Carotid-Cavernous Fistula As obvious from the name, it is an abnormal communication between the carotid artery and the cavernous sinus. The blood in the cavernous sinus becomes arterialized thereby raising the venous pressure and at the same time the arterial perfusion suffers. The fistula can be classified as 'direct or indirect', 'high flow or low flow ' and 'spontaneous or traumatic'. Barrow standardized the classification in 1985.24 Trauma is the most common cause of direct or type A fistulas usually seen in basal skull fractures. Indirect or type C, D, E, are due to congenital anomalies or spontaneous rupture of the artery

Barrow's types of carotid-cavernous fistulas24 Barrow Type

Origin

Vessels Involved

Type A

Trauma

Internal carotid

Type B

Spontaneous

Meningeal branches of internal carotid

Type C

Spontaneous

Meningeal branches of external carotid

Type D

Spontaneous

Meningeal branches of internal and external carotids.

secondary to aneurysm, atherosclerosis and severe hypertension. Clinical features include classical triad of conjunctival chemosis, pulsatile proptosis and bruit. Bruit is best heard as a flushing noise with the bell of the stethoscope, reduced by ipsilateral carotid compression in the neck. Other features include ptosis; increased intraocular pressure due to elevated episcleral pressure; 25 anterior segment ischemia hallmarked by corneal edema, ischemic pseudoiritis, rubeosis iridis and cataract; ophthalmoplegia, most frequently affecting the 6th cranial nerve due to its intracavernous location; diplopia Fundus examination reveals dilated veins, optic disc edema and intraretinal hemorrhages. Imaging modalities used are CT scan, MRI and angiography. CT scan shows enlarged superior ophthalmic vein, enlarged extraocular muscles and enlargement of the cavernous sinus.26 The definite test in selective internal or external carotid angiography which will demonstrate the fistula and its hemodynamics. Carotid cavernous fistulas are managed by interventional radiologists.Usually these patients are first seen by an ophthalmologist/orbital surgeon who makes the diagnosis and refers to interventional radiologist for management. Management indications include secondary glaucomas, ophthalmoplegia, severe proptosis and intolerable bruit.27 Balloon/ coils or surgical occlusion of the fistula is recommended for the Type A. Balloon/coil can be introduced either by an arterial or a venous route.

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154 Surgical Atlas of Orbital Diseases Type B and C can be treated with selective embolization of a single feeder vessel whereas Type D requires embolization of all the multiple feeder channels. Various complications of interventional radiology like vascular perforations, hemorrhage and permanent neurological deficit have been reported.28

New Growths New growths can be further subdivided into 'Hamartomas' and 'Neoplasms'. The hamartomas are exemplified by cavernous hemangiomas whereas the neoplasms include 'hemangiopericytoma', 'Angiosarcoma', 'Kaposi Sarcoma', 'angiomyomas', etc.1

Capillary Hemangioma Capillary hemangiomas are hamartomas characterized by growth of blood vessels along with proliferation of endothelium. These are common benign primary tumors of the orbit in children. It usually presents in the first or second week after birth and enlarges during the first year of life, after which they begin to involute. About 70% regress by 7 years of age. Clinical presentation are in the form of strawberry naevus when the hemangioma involves the lid. Involvement of the conjunctiva is important from diagnostic point of view. Within the orbit anterior and superior quadrant of the orbit is a favoured site. Orbital lesions may present with a progressive non-pulsatile proptosis, which may increase following straining and crying.29 Capillary hemangiomas have important systemic implications like high output failure, 'Kasabach-Meritt Syndrome' (Anemia + thrombocytopenia + low coagulant factors)30 and 'Maffuci Syndrome' (Hemangiomas + enchondromatosis). Imaging modalities used include CT scan, MRI and angiography. CT scans demonstrates moderately well defined lesion with finger like projections that may be present in any orbital space. There is a moderate to intense enhancement on contrast. Gadolinium enhanced T1 wieghted images with fat suppression shows diffuse homogenous or heterogenous enhancement. Multiple feeder vessels are seen on angiography.

Treatment is indicated when vision is threatened by amblyopia as a result of anisometropia, ptosis or strabismus. Intralesional injection of steroids is the most frequently used method. Usually 40-80 mg of triamcinolone with 25 mg of methylprednisolone is directly injected into the lesion. 1 Alternatively Triamcinolone 40 mg along with betamethasone 4 mg can also be used. The tumor usually begins to regress in two weeks but if necessary injection may be repeated after about two months. Early recognition and prompt treatment with intralesional steroid prevents early occlusion amblyopia, but follow-up and management of refractive amblyopia with glasses and patching is necessary in the longer term. Potential complications include skin depigmentation, fat atrophy, eyelid necrosis and rarely central retinal artery occlusion. Systemic steroids are indicated for extensive lesions especially if associated with visceral involvement. Recommended dosage used is 1.5 to 2.5 mg/kg prednisolone daily over a few weeks with titration downward depending on response.1 Though steroids are effective in large majority of patients, a recurrence is not infrequent. Recurrent or resistant cases are being treated with recombinant interferon alpha-2a and 2b with variable results.31 Recent studies have demonstrated good efficacy of interferons when given subcutaneously in a dose of 3 million units/m 2 . During clinical follow-up diagnostic ultrasound evaluation ( the depth dimension) proved helpful. One report suggested high efficacy of treatment when a combination of interferon alpha-2a with a low dose of cyclophosphamide. In the presence of very large platelet-consuming lesions as seen with Kasabach-Meririt syndrome, systemic antifibrinolytics like aminocaproic acid or tranexemic acid are used.30 Surgical resection is carried out in cases where vision is threatened or there is a failure of medical management. Surgery should be carried out under hypotensive anesthesia with constant hemostasis during removal.32

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Hemangiopericytoma These are uncommon vascular tumors of the orbit occurring in the middle ages. They are divided into benign and malignant based upon the histopathology. Clinical features include progressive painless proptosis of usually less than one year duration, predominantly in the superior part of orbit. Hemodynamically there is rapid circulation with significant shunting of blood. CT and MRI shows well defined lesions with homogenous contrast enhancement. 33 The microscopic features include cellular, myxoid and storiform components with spindle shaped pericytes, which stain positively with vimentin upon immunohistochemistry.34 Management includes careful and complete excision. The tumor has a pseudocapsule and is notorious for recurrences. Histologically benign tumors have been reported to metastasize. A very aggressive local behavior may warrant an exenteration.35

Angiosarcoma Angiosarcomas are malignant tumors of the endothelial origin, with an affinity for the head and neck regions. Mostly affects males in 6-8th decades of life. They are ill defined, multiple, involving the skin of the lids and the orbit.36 They may present with orbital apex syndrome and other neurological deficits37 Since these tumors are aggressive, a wide surgical excision is adviced.

Kaposi's Sarcoma Kaposi sarcoma has generated considerable research after the advent of AIDS.38 Though it is reported to be common in the western literature, we are yet to see a single case. Ocular involvement is usually of the skin, lids or conjunctiva as reddish or purple lesions and rarely lacrimal gland is involved. 39 Histologically vascular slit channels lined by endothelium are seen.

Management includes the use of chemotherapy and extended field radiotherapy.

Hemangioendothelioma These are very rare tumors of the orbit. It is known to affect all age groups with no age or sex prelidiction. Multifocality is present in 9-14% of the cases.40 It presents as a very rapidly enlarging mass with edema or erythema of overlying skin. The tumor is highly vascular and bleeds significantly on biopsy. Unlike rhabdomyosarcoma, it has a mass effect rather than being invasive. Imaging modalities used are CT and MRI, which demonstrates lytic, multiloculated, expansile lesions of the orbit. Histopathologically the tumor is composed of irregular vascular elements lined with immature endothelial cells with prominent anaplasia. All hemangioendotheliomas are positive for at least one endothelial marker. (CD31, CD34, factor VIII ). Management includes histological grading followed by treatment with radiotherapy, chemotherapy and surgical removal.41

Hemangioblastoma Hemangioblastoma is a rare tumor of the orbit. It presents with progressive proptosis which can be axial and abaxial as hemangioblatomas have been reported both from the recti muscles42 and optic nerve.43 Optic nerve hemangioblastomas are frequently familial, presents with visual loss and RAPD and are associated with infratentorial hemangioblastomas, angiomatosis retinae, and cysts of the abdominal viscera. CT and Magnetic resonance imaging reveals a wellenhanced mass, with an enlargement of optic canal in cases of optic nerve lesions. Management includes surgical removal with appropriate orbitotomy approaches.43

Age

Early childhood

childhood to middle age

Middle ages

Variable

Ist year of life

Middle ages

Name of the lesion

Lymphangioma

Orbital varices

Cavernous hemangioma

Carotid-Cavernous fistula

Capillary hemangioma

Hemangiopericytoma

Painless, progressive eccentric proptosis of less than a year duration

Strawberry nevus on lids and conjunctiva. Nonpulsatile proptosis that increase with valsalva maneuver. Additional features of associated Kasaback-Meritt and Mafucci syndromes

Conjunctival chemosis, pulsatile proptosis with a bruit, anterior segment ischemia and glaucoma

Slowly progressive unilateral axial proptosis, decreased vision, hyperopia, optic nerve compression, choroidal folds and gaze evoked amaraosis

Non-pulastile, reversible proptosis, increases with valsalva maneuver

Progressive proptosis that increases with upper respiratory infections, sudden proptosis with or without optic nerve compression due to bleeding into the lumen (chocolate cyst)

Clinical features

Orbital Vascular Lesions at a Glance

CT and MRI shows well defined lesions with homogenous contrast enhancement

CT, MRI and angiography. Finger like projections into the orbit with moderate to intense enhancement on contrast

CT, MRI and angiography. Enlarged superior ophthalmic vein is a feature. Definite test is angiography

CT and MRI. Well defined intraconal mass with smooth margins and enhancement with contrast

CT, Doppler USG and angiography. Phleboliths may be observed on CT

USG, CT and MRI. MRI is modality of choice as it delineates the leison well and also helps in timing the chocolate cyst

Imaging modalities

Careful excision. Aggressive tumors require exenteration

Topical, intralesional and systemic steroids. Interferons and cyclophosphamide. Surgical excision

Interventional radiology Balloon or coil embolization

Surgical excision with a lateral orbitotomy approach

Conservative, Embolization Surgical removal

Conservative, Sclerosing agents, Surgical with adjunctive lasers

Management

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CASE ILLUSTRATIONS Case 1 Mrs. B, female 45 years in age, has presented with proptosis of her right eye since 5 years and progressive loss of vision since 3 years. There was no history of pain, trauma, change with posture, or any systemic disease. She consulted an ophthalmologist elsewhere 2 years back, who ordered CT scan of orbit which was reported as Meningioma of optic nerve sheath by the radiologist and hence was advised conservative management by the ophthalmologist. On inspection, (Figure 10.1) she had a nonpulsatile proptosis of her right eye with displacement of globe by 8 mm axially, and outwards by 5 mm.There was no change with Valsalva maneuver. Fullness was seen with obliteration of superior sulcus. Minimal mechanical restriction of ocular motility was noticed. Pupil was dilated in size and direct light reaction was absent. There was no perception of light. Fundus exam revealed optic atrophy. Non-tender, firm mass was palpable in the superior peripheral space, extending into Orbit. Its posterior border could not be felt. Orbital rim was normal. Retropulsion was positive. General examination was within normal limits. Clinical Impression: In view axial proptosis, the lesion should be in the intraconal space. The long duration of proptosis, absence of visual symptoms for a long period after the onset of proptosis, and the severe degree of proptosis exclude lesions arising from optic nerve or its sheath. (We are yet to see a case of Meningioma of optic nerve sheath causing such a huge proptosis). In view of the long duration, sex (female) and the location (Cavernous hemangioma is the most common intraconal lesion in our experience), we made a diagnosis of Cavernous hemangioma. CT scan of orbit revealed a huge, hyper dense lesion, occupying entire intraconal space and extending into the peripheral space, more on the medial compartment, pushing the globe temporally (Figures 10.2 and 10.3). The lesion is very well encapsulated. It has caused excavation of bony orbit. It is not enhancing on contrast. All these features are

suggestive of Cavernous hemangioma. The tumor was excised through lateral orbitotomy. On gross examination it was very well encapsulated, measuring 55 mm × 45 mm (Figure 10.4). Histopathology confirmed it to be Cavernous hemangioma (Figure 10.5). Postoperative recovery was smooth and satisfactory but for mild enophthalmos which was due to increased orbital volume because of excavation of orbital walls (Figure 10.6).

Figure 10.1: Proptosis left eye with globe pushed temporally

Figure 10.2: CT Coronal view well Figure 10.3: CT Axial view: defined mass filling the entire conal Note the increase in orbital space volume

Figure 10.4: Gross specimen Figure 10.5: Histopathology shoof excised Well encapsulated wing dilated vascular channels tumor (H and E) cavernous hemangioma

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158 Surgical Atlas of Orbital Diseases

Figure 10.7: Eccentric proptosis with inferior fullness

Figure 10.6: Postoperative picture showing relief from proptosis

Case 2 Mr. K, male, 32 years of age presented to us with painless progressive proptosis of right eye since 3 years. There was no history of defective vision, or diplopia. On examination (Figure 10.7) we noticed eccentric proptosis of the right eye, with fullness inferiorly. The proptosis was nonpulsatile. Ocular motility was normal. Pupil was normal. There was no RAPD. Vision was 20/20 and color vision was normal. CT scan showed a well defined lesion in the inferior peripheral space with minimal contrast enhancement and bony excavation of the floor of the orbit (Figures 10.8 and 10.9), suggestive of Cavernous hemangioma. Anterior inferior orbitotomy was performed through subciliary approach, and the tumor was exsanguinated by passing a suture through the substance of it to shrink its size (Figure 10.10). This suture also helps in applying traction to assist the excision of the tumor through a smaller incision . The excised tumor was pinkish in color and was very well encapsulated. (Figure 10.11) The cut-section of the tumor (Figure 10.12) showed honey-comb like appearance with blood oozing out from the entire cut surface. On histological examination the encapsulated mass was made-up of dilated vascular channels, filled with blood (Figure 10.13), confirming the clinical diagnosis of Cavernous hemangioma. The patient recovered well (Figure 10.14). The proptosis disappeared completely. His vision remained 20/20. The ocular motility was full.

Figure 10.8: CT scan shows well Figure 10.9: CT scan well defdefined lesion in inferior space ined lesion with bony excavation

Figure 10.10: Suture through Figure 10.11: Well encapsulated exsanguinated and shrinks Aids tumor excision by traction

Figure 10.12: Cut section showing Figure 10.13: Dilated vascular honeycomb appearance with blood channels filled with blood oozing from it

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Vascular Lesions of Orbit 159

Figure 10.15: Axial proptosis of right eye

Figure 10.16: CT scan of brain showing calcified lesions

Figure 10.14: One week postop, recovery from proptosis

There was no recurrence of the tumor. The points to consider in this case were the location of cavernous hemangioma in the inferior peripheral space, the passing of suture through the hemangioma to bleed the tumor and shrink its size, which facilitates to remove the tumor through a smaller incision. Cryo is other wise an excellent tool to hold the tumor during its dissection and removal.

A

B

Figures 10.17A and B: Axial and Coronal sections of CT orbit showing well encapsulated Intraconal lesion with contrast enhancement

Case 3 Mrs. J, a female 28 years of age, presented with history that her friends and family members were commenting that her right eye was looking prominent since 3 months (Figure 10.15). She had no pain and did not complain of any visual disturbances. Past history was significant in that she had convulsions 4 years back and CT scan of brain showed 2 calcified lesions (Figure 10.16). She was on carbamazepine (Tegretol) since then. There was no relapse of convulsions. On clinical evaluation, she had 3 mm of axial and nonpulsatile proptosis, which did not increase with Val-salva maneuver. The ocular motility was normal. The pupils were brisk and the vision was 20/20. CT scan of orbit revealed a hyper-dense, contrast enhancing lesion of size 15 × 12 mm in the intraconal space (Figures 10.17A and B). In view of the short duration and contrast enhancement, provisional diagnosis of a vascular tumor like hemangioendothelioma/hemangiopericytoma was considered. Lateral orbitotomy was performed and the tumor was excised. Histopathology and Immunohistochemistry revealed it to be hemangioblastoma. Further evaluation of the patient did not show any

Figure 10.18: Postoperative status showing complete recovery

other lesions in the posterior segment of the globes, brain or elsewhere. The calcified lesions are probably intracranial hemangioblastoma and since they were inactive, neurosurgeon did not contemplate excision. The patient has no recurrence during the past 3 years of follow-up (Figure 10.18).

Case 4 Miss P, female child of 6 years presented with acute proptosis of 2 weeks duration associated with severe pain and defective vision. There was no history of trauma.She never had similar problem previously. On examination she had a non-pulsatile, proptosis of 7 mm, associated with severe periocular fullness, chemosis grade III associated with subconjunctival

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160 Surgical Atlas of Orbital Diseases hemorrhage, and restricted ocular motility in all gazes .Pupil was dilated. RAPD was present. Her best corrected vision was 20/200. Retropulsion was positive. Tenderness was present on palpation (Figure 10.19). In view of acute onset associated with pain, chemosis, restricted motility, subconjunctival hemorrhage and chemosis, in a child, Lymphangioma was the clinical diagnosis. CT scan showed a contrast enhancing lesion with a huge hemorrhage in the lesion, supporting the clinical diagnosis (Figure 10.20). Normally lymphangioma is managed conservatively since it is not possible to excise it completely and hence recurrence is very common. But in this child as the vision is being compromised, the parents were informed that though recurrence is very common, surgery was to be performed to save vision. Antero-lateral orbitotomy was performed. About 6 cc of blood was aspirated from the lesion to shrink it and excised as much as possible (Figure 10.21). The histopathology confirmed the diagnosis of lymphangioma (Figure 10.22). Postoperative recovery was smooth and the vision improved to 20/20 (Figure 10.23). After 3 years, she had a

Figure 10.23: Postoperative status. Proptosis disappeared vision improved to 20/20

recurrence which was surgically managed. There was no recurrence in the 2 year postoperative follow-up after second surgery.

REFERENCES 1. Rootman J: Diseases of the orbit; A multidisciplinary approach. Lippincott Williams and Wilkins, 2nd Edition: 455-506. 2. Harris GJ Orbital vascular malformations: a consensus statement on terminology and it clinical implications. Orbital society. Am J Ophthalmol. 1999; 127:453-55. 3. Rootman J, Hay E, Graeb D, et al. Orbital adenexal lymphangiomas. A spectrum of hemodynamically isolated vascular hamartomas. Ophthalmology. 1986; 93:1558-70. 4. Pang P, Jakobiec FA, Iwamoto T, Hornblass A Small lymphangiomas of the eyelids. Ophthalmology 1984; 91:1278-84.

Figure 10.19: Acute proptosis Figure 10.20: CT scan of orbit with severe chemosis and showing large cystic lesion in periocular swelling intraconal space extending to medial peripheral space, and hemorrhage within

5. Kazim M, Kennerdell JS, Rothfus W, et al. Orbital lymphangioma: correlation of magnetic resonance images and intraoperative findings. Ophthalmology. 1992; 99: 1588-94. 6. Harris GJ, Sakol PJ, Bonavolontu G, et al. An analysis of 30 cases of orbital lymphangiomas: pathophysiological considerations and management recommendations. Ophthalmology. 1990; 97:1583-91. 7. C Luzzatto, P Midrio, Z Tchaprassian, M Guglielmi: Sclerosing treatment of lymphangiomas with OK-432. Arch Dis Child 2000;82:316-318. 8. CM Giguere, NM Bauman, Y Sato, DK Burke, JH Greinwald, S Pransky, P Kelley, K Georgeson, and RJH Smith Treatment of Lymphangiomas With OK-432 (Picibanil) Sclerotherapy: A Prospective Multi-institutional Trial Arch Otolaryngol Head Neck Surg, October 1, 2002; 128(10): 1137-44.

Figure 10.21: Blood being drawn from the lesion intraoperatively

Figure 10.22: Histopathology showing channels filled with lymph

9. JP Deveikis. Percutaneous Ethanol Sclerotherapy for Vascular Malformations in the Head and Neck Arch Facial Plast Surg, September 1, 2005; 7(5): 322-5.

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Vascular Lesions of Orbit 161 10. Mathur NN, Rana I, Bothra R, Dhawan R, Kathuria G, Pradhan T Bleomycin sclerotherapy in congenital lymphatic and vascular malformations of head and neck. Int J Pediatr Otorhinolaryngol. 2005;69(1):75-80. 11. Schwarcz RM, Ben Simon GJ, Cook T, Goldberg RA Sclerosing therapy as first line treatment for low flow vascular lesions of the orbit. Am J Ophthalmol. 2006;141(2):333-9. 12. Wojno TH Sotradecol (sodium tetradecyl sulfate) injection of orbital. Lymphangioma. Ophthal Plast Reconstr Surg. 1999;15(6):432-7. 13. Wright JE, Sullivan TJ, Garner A, et al. Orbital venous anomalies. Ophthalmology. 1997; 104:905-13. 14. Cline RA, Rootman J. Enophthalmos: a clinical review. Ophthalmology. 1984; 91:229-37. 15. Harris GJ, Jakobiec FA. Cavernous hemangioma of the orbit: A clincopathological analysis of sixty-six cases. In: Ocular and adnexal tumors. Birmingham, AL: Aesculapius, 1978:741-81. 16. Fries PD, Char DH. Bilateral orbital cavernous hemangiomas. Br J Ophthalmol 1988; 72:871-3. 17. Sullivan TJ, Aylward GW, Wright JE, et al. Bilateral multiple Cavernous hemangiomas of the orbit. Br J Ophthalmol. 1992; 76:627-9. 18. Forbes GS, Sheedy PF, Waller RR. Orbital tumors evaluated by Computer tomography. Radiology 1980;136:101-11. 19. Ohtsuka K, Hashimoto M, Akiba H. Serial dynamic magnetic resonance imaging of orbital cavernous hemangioma. Am J Ophthalmol. 1997; 123:396-8. 20. Susac JO, Smith JL, Scelfo RJ The "tomato catsup" fundus in Sturge-Weber syndrome. J Pediatr Ophthalmol Strabismus. 1974; 92:69-70. 21. Phelps CD The pathogenesis of glaucoma in Struge-Weber Syndrome. Ophthalmology. 1978; 85:276-86. 22. Kim J, Kim OH, Suh JH, Lew HM Wyburn-Mason syndrome: An unusual presentation of bilateral orbital and unilateral brain A-V malformations. Pediatr Radiol. 1998; 28:161. 23. Good WV, Hoyt CS Optic nerve shadow enlargement in Klippel. Trenaunay-Weber syndrome. J Pediatr Ophthalmol Strabismus. 1989; 26:288-9. 24. Barrow DL, Spector RH, Braun IF, et al. Classification and Treatment of spontaneous carotid-cavernous sinus fistulas. J Neurosurg 1985; 62:248-56. 25. Talusan ED, Fishbein SL, Shwartz B Increased pressure of dilated episcleral veins with open angle glaucoma without exophthalmos. Ophthalmology. 1983; 90:257-65. 26. Vinuela F, Fox AJ, Debrun GM, et al. Spontaneous carotidcavernous fistulas: clinical, radiological and therapeutic considerations. J Neurosurg. 184; 60:976-84.

27. Debrun GM, Vinuela F, Fox AJ, et al. Indications for treatment and classification of 132 carotid-cavernous fistulas. Neurosurg. 1988; 22:285-9. 28. Lasjaunias P, Chiu M, Ter Brugge K, et al. Neurological manifestations of intracranial dural arterio-venous malformations. J Neurosurg 1986; 64:724-30. 29. Haik BG, Jakobiec FA, Ellsworth RM, Jones IS. Capillary hemangioma of the lids and orbit: an analysis of the clinical features and therapeutic results in 101 cases. Ophthalmology 1979; 86:760-92. 30. Neidhart JA, Roach RW. Successful treatment of skeletal hemangioma and Kasabach-Merritt syndrome with aminocaproic acid. Am J Med 1982; 73: 434-8. 31. Teske S, Ohlrich SJ, Gole G, et al. Treatment of orbital capillary hemangioma with interferon. Aust N Z J Ophthalmol 1994; 22: 13-7. 32. Walker RS, Custer PL, Nerad JA. Surgical excision of periorbital capillary hemangiomas. Ophthalmology 1994; 101:1333-40. 33. Kikuchi K, Kowada M, Sageshima M Orbital hemangiopericytoma: CT MRI and angiographic findings. Comput Med Imaging Graph. 1994;18:217-22. 34. Croxatto JO, Font RL. Hemangiopericytoma of the orbit: A clinico-pathological study of 30 cases. Hum Pathol 1982; 12:210-18. 35. Sullivan TJ, Wright JE, Wulc AE, et al. Hemangiopericytoma of the orbit. Aust N Z J Ophthalmol . 1992; 20:325-32. 36. Maddox JC, Evans HL. Angiosarcoma of skin and soft tissue: a Study of forty-four cases. Cancer 1981;48:1907-21. 37. Messmer EP, Font RL, McCary JA, Murphy D. Epithelioid angio-sarcoma of the orbit presenting as Tolosa-Hunt syndrome. A clinicopathological case report with review of literature. Ophthalmology 1983; 90:1414-21. 38. Holland GN, Gottlieb MS, Yee RD, et al. Ocular disorders associated with a new severe acquired cellular immunodeficiency syndrome. Am J Ophthalmol. 1982; 93:393-402. 39. Kalinske M, Leone CR Jr Kaposi's sarcoma involving the eyelid and conjunctiva. Ann Ophthalmol. 1982; 14:497-9. 40. Arie R, Aylon YG, Don R Hemangioendothelioma of the orbit. Intl J Pediatric Otorhinolaryngology. 2006;1:188-91. 41. Lyon DB, Tang TT, Kidder TM Hemangioendothelioma of the orbital bone. Ophthalmol 1992. 99:1773-98. 42. Cockerham KP, Sachs DM, Cockerham GC, Hidayat AA, Brown HG Orbital hemangioblastoma arising in a rectus muscle. Ophthal Plast Reconstr Surg. 2003 May;19(3): 248-50. 43. Hotta H, Uede T, Morimoto S, Tanabe S, Hashi K, Takeda M. Optic nerve hemangioblastoma. Neurol Med Chir (Tokyo). 1989;29(10):948-52.

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162 Surgical Atlas of Orbital Diseases

11

CHAPTER

Orbital Tumors of Neurological Origin Christopher M Knapp, Ram Vaidhyanath, Laurence Brown, Raghavan Sampath

Optic Nerve Glioma The first clinical report of optic nerve tumors was by Antonio Scarpa in 1816, however it wasn't until 1912 that Hudson described optic nerve gliomas and optic meningiomas as separate lesions.1 These tumors are relatively rare, accounting for less than 4% of all orbital tumors. Overall optic nerve gliomas account for 65%1,2 of optic nerve tumors and meningiomas 35%. 1 Optic nerve gliomas appear to be more frequent in females, whereas for optic pathway gliomas, there may be an equal sex distribution.2 Lesions typically become symptomatic in childhood.3 The mean age of presentation is 8.8 years with only 10% presenting after 19 years of age.2 Most lesions are sporadic although there is an association with neurofibromatosis (NF 1). Adult optic nerve gliomas may behave like those presenting in childhood,3 however some may show an aggressive behavior, being clinically distinct from those seen in childhood. These patients are typically middle aged with a slight male bias.3 Benign optic nerve gliomas are typically pilocytic astrocytomas originating from astrocytic glia and can involve the visual pathways anywhere from the optic nerve to the visual cortex.1,4 Histologically they are composed of delicate, hair-like elongated eosinophilic cells in an interwoven pattern. The nuclei may be round or oval. Mitoses are rare, but nuclear atypia may be observed. Malignant change is very rare. The proliferating astrocytes in an optic glioma may be associated with worm-like densely eosiniophilic bodies, known as Rosenthal fibers, surrounded by hyalinized connective tissue, a

distinctive (but not diagnostic) feature of pilocytic astrocytomas. 5 Orbital gliomas, particularly in patients suffering from NF 1, can extend through the pia and arachnoid matter in to the subdural space. They tend to remain intradural, however when incompletely excised can recur diffusely invading adjacent orbital structures. Where the lesion has spread to the subarachnoid space reactive proliferation of fibrovascular and meningeothelial tissue can occur resulting in arachnoid hyperplasia, which can resemble an optic nerve sheath meningioma.1 Two patterns of growth are identified, perineural (circumferential growth) and intraneural growth. Perineural growth results from proliferating astrocytes and fibrovascular tissue within the dural sheath, widening the epipial-subdural space and subsequently compressing the optic nerve. Intraneural growth results from growth of intra-axial astrocytes causing the subarachnoid space to be obliterated. Perineural growth appears to occur in patients with NF1.1 Enlargement of optic gliomas occurs by a combination of neoplastic cell proliferation, reactive arachnoid proliferation and accumulation of PAS positive mucinous substance. Rapid growth can result from cystic degeneration or intra-lesional hemorrhage. Malignant change can occur however this is extremely rare.1 Malignant optic nerve gliomas seen in adults show evidence of malignant astrocytomas and are thought to originate within the optic pathways. Extension is usually subpial along the optic pathways although can directly invade the substance of the brain.1

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Orbital Tumors of Neurological Origin 163 Approximately 30-50% of benign optic gliomas are associated with neurofibromatosis type 1 (NF 1)2, 4, the rest occur sporadically. The incidence of optic nerve glioma in patients with NF 1 is 15-20% of which only about 20% have visual symptoms.1, 4 The presenting symptoms depend very much on the location of the lesion. Those involving predominantly the intraorbital optic nerve present with proptosis, ocular motility abnormalities, vision loss and visual field loss. Intracranial tumors including those involving the chiasm, tend to have reduced vision and endocrine abnormalities including precocious puberty.1-4 Fundoscopy may reveal either optic atrophy or papilloedema depending on the location of the lesion. Optocilliary shunt vessels, central retinal vein occlusion and venous stastis retinopathy are occasionally seen.1,3 Hemorrhage in to the tumor can result in acute proptosis and sudden loss of vision.3 Investigation of optic nerve gliomas involves computerized tomography (CT) and magnetic resonance imaging (MRI). The appearance of the lesion depends on whether the patient has NF 1 or not. Fusiform swelling of the optic nerve is typically seen in patients without NF 1, whereas those with NF 1 tend to have more irregular nerves with areas of kinking or buckling. 3 Cystic spaces may occasionally be seen representing mucinous accumulations. With perineural growth patterns, there may be thickening of the dura with preservation of the compressed optic nerve, mimicking the tramtracking seen in optic nerve sheath meningiomas. Calcification is a rare finding.1 MRI shows the lesions to be hypointense on T1 weighted images, whereas on T2 images the glioma tends to be hyperintense.3 Contrast enhancement and fat suppression technique can help to differentiate optic nerve meningiomas from gliomas, since arachnoid hyperplasia associated with gliomas does not enhance with gadolinium.1 Treatment options depend very much on the location and extent of the lesion. Most optic nerve gliomas have an indolent growth pattern and can remain stable for many years, with some spontaneously regressing.1, 3 Those lesions confined to the optic nerve show a mortality rate of 5% from intracranial extension. Tumor resection is curative

whilst the lesion remains confined to the optic nerve. Lesions involving the chiasm have a mortality rate of approximately 28% from intracranial spread, whilst hypothalamic or 3rd ventricle involvement increases the mortality rate to more than 50% at 15 years.1 Patients with isolated optic nerve involvement and good vision should be reviewed on regularly with repeat serial MRI scans of the optic nerve. When there are signs of posterior progression the lesion should be excised en block.1 Those lesions confined to the optic nerve in blind, painful or cosmetically unacceptable eyes should be considered for surgical excision. 1,3,4 Optic nerve lesions extending to the chiasm or those primarily involving the chiasm or optic tracks could be considered for chemotherapy or radiotherapy. Children 5-7 years and younger should be treated with chemotherapy 2,4 since radiotherapy at this age may result in damage to the endocrine system and affect the future intellectual development of the child.1,4 Children aged 10 and above may be considered for radiotherapy using various delivery techniques. Doses typically given are in the range of 45 to 56 Gy given in 180cGy fractions. Malignant glioma seen in adults is invariably fatal.1 Neuro-imaging of an involved optic nerve is non-specific and the condition shows a rapid rate of progression. Lesions affecting the proximal part of the optic nerve can progress to affect both eyes within 5-6 weeks.3

Optic Nerve Meningioma Optic nerve meningiomas account for one third of primary optic nerve tumors and were first identified as a pathological entity in 1835 by Jean Cruveilheir. It wasn't until 1912 that Hudson clearly differentiated optic nerve gliomas from meningiomas. 6 Most meningiomas involving the orbit are extensions from intracranial sites such as the sphenoidal wing (secondary optic nerve sheath meningiomas). Primary optic nerve meningiomas originate from the cap cells of the arachnoid surrounding the intraorbital or intracanalicular portion of the optic nerve.3 The typical age at presentation is 40 years of age6,7 with bilateral cases presenting much earlier at approximately 13 years of age.6 There is a small sex bias with 61% of cases occurring in females.6

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164 Surgical Atlas of Orbital Diseases Histologically most orbital meningiomas are of the meningiothelial or syncytial variety. The cells are eosinophilic with abundant cytoplasm and indistinct cell margins. The nuclei are small and vesicular with occasional pseudoinclusions. Cells may be wrapped with or without psammoma bodies in tight whorls.5 Approximately 90% of meningiomas involving the orbit originate from intracranial sites, primarily the olfactory groove and the sphenoidal ridge.6 Ectopic lesions have been described in the orbit, seemingly independent of the optic nerve, these may have developed from orbital mesenchymal cells or may be a case of mistaken identity, since lesions such as fibroxanthomas and hemangiopericytomas closely resemble meningiomas.6 NF 1 is associated with optic nerve meningioma, although the relationship is no where near as high as for optic nerve glioma. The incidence of NF 1 in patients with optic nerve meningioma is 2%, whereas the incidence of NF 1 in the general population is 0.03-0.05%.6 The classical triad of clinical findings in optic nerve meningioma are visual loss, optic atrophy and optociliary shunt vessels. 3,6 The simultaneous occurrence of these three findings is however rare. The most common finding is vision loss which can take the form of visual obscurations. Other findings are reduced color vision and visual field defects.3,6 Proptosis seems to follow the onset of visual loss, tending to be reasonably mild.3,7 It is thought to occur because tumor growth results in a straightening of the optic nerve and may also account for any ocular motility defects with up gaze being most commonly affected.6,7 Orbital pain may occur. Examination may reveal disc swelling although the only signs may be optic atrophy. Optociliary shunts which present in less than one third of patients3,7 are shunts between the retinal and choroidal circulations and thought to be a result of a reopening of regressed vestigial embryonic retinociliary anastomoses.6 They may be seen in eyes with other causes of disc swelling. The disc swelling and central retinal vein congestion may proceed the shunt vessels by 1-2 years becoming noticeable as the swelling starts to resolve3 and then regress as optic atrophy sets in.6 Meningiomas arise from meningothelial cells along the meninges. Large collections are known as

pachionian bodies or arachnoid bodies whereas smaller ones are known as arachnoid villi. Optic nerve meningiomas are thought to arise from meningiothelial cap cells of the arachnoid villi.6 Two patterns of growth are seen, a syncytial pattern in which polygonal cells are arranged in sheets separated by vascular trabeculae and a transitional pattern where spindle cells are arranged in concentric whorls. Mitoses are uncommon. Psammoma bodies are occasionally seen in which calcium salts can be deposited. Meningiomas may spread in the subarachnoid spaces along paths of least resistance. They can extend in to the surrounding tissues3 and the haversian canal system of bone resulting in hyperosteosis. They do not seem to invade the brain and are not associated with raised intracranial pressure or pituitary dysfunction.6 Growth is slow although may accelerate with pregnancy and results in compression of the optic nerve.6 Investigation involves CT and MRI imaging. Calcification when present is useful in distinguishing optic nerve gliomas from meningiomas. Typically the lesions show 'tram-tracking' in which a thickened optic nerve sheath surrounds a central lucent optic nerve.3,6,7 On coronal views this is seen as a dense ring surrounding the central optic nerve.6 MRI with gadolinium enhancement is particularly sensitive in detecting meningiomas.7 Treatment options include surgery and radiotherapy, however observation is not unreasonable since the mortality rate is low.7 Surgery may be indicated for aggressive lesions however it is associated with a high degree of local recurrence and orbital invasion. Typically surgical excision results in blindness, since the tumor and optic nerve often share their blood supply.6,7 Decompression of the nerve has been attempted by opening the dural sheath, which in some cases can arrest the visual deterioration.3,6 Fractionated radiotherapy with a total of 40-54Gy given in divided doses seems to be effective in stabilizing and in some cases improve vision, whilst at the same time limiting the risks of optic nerve or chiasm damage to less than 5%.3,7,8 Interestingly the tumor volume appears unchanged following radiotherapy, despite an improvement in neurological function.8 The side effects of treatment including headache, nausea and hair loss can be

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Orbital Tumors of Neurological Origin 165 limited by fractionating the doses. Later side effects include pituitary dysfunction, retinopathy, iritis and temporal lobe atrophy.8 The risk of optic nerve or chiasm damage associated with high doses of radiotherapy. When doses greater than 59Gy are given in fractions of less than 1.9Gy there is an 11% risk of injury whilst doses greater than 1.9Gy have a 47% risk according to a University of Florida study.9 Low dose steroids at the time of radiotherapy may limit damage from radiation induced peritumor edema.8 The risk of a second malignancy following radiotherapy is 0-2% at 10-20 years.8 When a lesion is treated with a single dose of radiation the threshold for injury is 8-10 Gy.8

Orbital Schwannoma (Neurilemmoma) and Neurofibroma Schwannomas also known as neurilemmomas account for approximately 1-4% of orbital tumors, occurring most commonly in men between the second and fifth decades.10,11 They occasionally arise from the optic nerve, probably originating from schwann cells of sympathetic nerves tightly adherent to the optic nerve.3 More frequent sites are the other cranial nerves including the oculomotor, lacrimal, trigeminal and zygomaticotemporal nerves. 10,11 The most commonly affected cranial nerve is the vestibular nerve.12 Schwannomas are slow growing lesions that do not invade surrounding tissues.11 Clinical features include proptosis and diplopia. Globe compression can induce a hypermetropic shift, whilst optic nerve compression results in reduced visual acuity. Malignant transformation is rare.10 Gross examination reveals a well encapsulated yellowish grey lesion, with cysts containing clear fluid. Histologically schwannomas show two growth patterns: Antoni A where densely packed spindleshaped cells are arranged with palisaded nuclei sometimes forming Verocay bodies, and Antoni B where cells are separated by an abundant myxoid stroma with no alignment of nuclei. Mitoses are usually absent. The associated nerve may sometimes be seen in one side of the lesion, in contrast to a neurofibroma where the originating nerve is expanded by the lesion and cannot be seen. So-called "ancient schwannomas" may feature bizarre enlarged or multiple nuclei, but there are no malignant

connotations.10,13 The lesions may be very vascular, causing diagnostic confusion. Surgery is the treatment of choice11 although it is associated with usual risks of orbital surgery including loss of sight. Recent advances in steriotactic radiosurgery and fractionated radiotherapy in the treatment of vestibular schwannoma and nonvestibular schwannoma have achieved high levels of tumor growth control whilst preserving cranial nerve function.12,14 Future advances may mean that this treatment option could be used to treat and control orbital schwannomas.3 Neuro-imaging reveals a homogenious lesion isointense with rest of the surrounding neural tissue mimicking other lesions such as optic nerve gliomas, cystic spaces are sometimes seen.3,11 Histopathology is required to make a definitive diagnosis.10,11,15 Neurofibromas are another group of benign nerve sheath tumors which can occur in the orbit region.16 In a retrospective review by Rose et al. in 1991,17 looking at peripheral nerve tumors in the orbit, they found that 93% of these lesions were either schwannomas (neurilemmomas) or neurofibromas. In their series they found that most affected nerve was the first division of the trigeminal nerve. Neurofibromas are typically associated with neurofibromatosis, although they can occur in isolation. Approximately 25 to 45% of all lesions are found in the head and neck region.16 Other sites typical affected by neurofibromas include the eyelids, the orbit and rarely the lacrimal sac.18 Clinically they tend to present with painless proptosis and diplopia. Pain and altered sensation are rare.17 Grossly, the lesions appear as an encapsulated firm white mass. Histologically neurofibromas consist of wavy cells with basophilic nuclei.16 The treatment of choice is surgical excision. Even when incompletely excised recurrence is low.17

When to Suspect an Orbital Tumor of Neurological Origin • Insidious onset • Proptosis typically axial • Symptoms may include: Pain with and without eye movement, diplopia, blurring of vision, reduced color vision and increased hypermetropia

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166 Surgical Atlas of Orbital Diseases • Fundoscopy may include: Unilateral disc swelling and choroidal folds • Firmness of the globe to retropulsion.

Investigations • Bloods to rule out an inflammatory cause • Orbital imaging: CT and/or MRI imaging preferably with contrast enhancement • Orbital biopsy where imaging is suspicious or the diagnosis is in doubt.

CASE ILLUSTRATIONS Case 1 A 2 years old child with Neurofibromatosis type 1 was referred to the eye department by the pediatric oncology service following surgery for a cerebellar astrocytoma and hydrocephalus. At presentation she had an unrelated right convergent strabismus for which she underwent convergent squint surgery. Fundoscopy including the optic disc was unremarkable and there was no RAPD. There was no evidence of proptosis. Routine MRI scans (Figures 11.1 and 11.2) revealed sub-clinical bilateral optic nerve gliomas.

Over the following 8 years optic disc cupping developed, with the left disc being paler and more cupped. Color vision remained normal in both eyes.

Case 2 A 66 years old female presented to the orbit clinic with a 6 months history of horizontal diplopia. Visual acuity was 6/9 in the right eye and 6/12 in the left. Ocular motility appeared normal however diplopia was reported on dextroversion. There was proptosis of 4 mm on the left and a left relative afferent papillary defect. Only 2 of 13 ishihara plates were correctly identified by the left eye whereas color vision in the right eye was normal. There was no evidence of papilloedema, although the left disc was slightly pale. Fundoscopy revealed signs of age related macular degeneration in both eyes. Routine bloods were taken. Urgent MRI and CT scans were performed, revealing an intraconal soft tissue mass which involved the sphenoidal wing and extending intracranially to the temporal lobe (Figure 11.3). The findings were strongly suggestive of a meningioma. A subsequent CT scan showed evidence of hyperostosis of the sphenoidal wing, as well as bone loss in the posteriorlateral aspect of the orbit. The diagnosis of a sphenoidal wing meningioma was made. A neurosurgical referral was made and the patient was offered the option of a craniotomy and surgical debulking of the tumor. As there was no guarantee that this would improve her vision, surgery was declined. The condition is being managed conservatively with regular clinic follow up and routine repeat MRI scans.

Case 3

Figure 11.1: Bilateral optic nerve glioma (axial view)

Figure 11.2: Bilateral optic nerve glioma involving the chiasm (coronal view)

A 66 years old female was referred from a district hospital with retro-orbital pain. An MRI had revealed

Figure 11.3: Left optic nerve meningioma

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Orbital Tumors of Neurological Origin 167 an intraconal retrobulbar mass displacing the optic nerve superiorly, with no evidence of bone destruction. Visual acuity was 6/9 in the right eye and 6/6 in the left. Color vision slightly reduced in the right eye, seeing 13 out of 15 ishihara plates, the left was normal. A mild right RAPD was noted. Both optic discs were normal and there was no proptosis. A diagnosis of optic nerve meningioma was made. Following discussion with the patient and oncology/ radiotherapy department a dose of 50.4 Gy was given in 14 fractions to the orbit. Subsequent MRI scans revealed a reduction in the tumor following the radiotherapy. After treatment the patient experienced a dry right eye, which was treated with lubricants, and there was evidence of mild radiation retinopathy.

Figure 11.4: Low power meningioma H and E stain

Case 4 A 74 years old female with a known optic nerve meningioma was referred to the orbital clinic with a recurrence of orbital symptoms and enlargement of the lesion. Visual acuity in the affected eye was NPL, whereas the right was 6/6. Proptosis was noted on the left side. The left disc was atrophic and there was marked chorioretinal atrophy. An MRI confirmed enlargement of the lesion with extension towards the orbital apex. Following discussion with the patient a decision was made to debulk the meningioma via a lateral orbitotomy. Histology confirmed the diagnosis of a meningioma (Figures 11.4 and 11.5).

Figure 11.5: High power meningioma H and E stain

Case 5 A 36 years old male attended the orbit clinic with an 18 months history of worsening left sided proptosis (Figure 11.6). Visual acuity was unaffected; left eye 6/6, right 6/5, although color vision was slightly reduced, with only 11 out of 13 ishihara color vision plates being correctly identified in the left eye. Color vision was normal in the right eye. The visual field on the left showed generalized depression on the left side although no relative afferent papillary defect was identified. Oculomotor function was reduced on abduction and upgaze in the left eye. Fundoscopy of both eyes was normal with no evidence of papilloedema. The central nervous system examination was unremarkable and there was no past medical history of relevance.

Figure 11.6: Left sided proptosis

An urgent MRI scan of the orbits was arranged identifying a 2.5 cm rounded intraconal lesion which was enhanced following intravenous contrast administration (Figure 11.7). The optic nerve, lateral and inferior rectus were displaced and that the lesion was intraconal (Figure 11.8). The optic nerve was separate from the lesion. An MRI scan was also

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168 Surgical Atlas of Orbital Diseases

Surgical Approach Where orbital surgery is considered to obtain a tissue diagnosis or to debulk an orbital tumor, a lateral orbitotomy approach is chosen since this gives good access to the retro-orbital spaces and any lesions found there. If there is evidence of intracranial extension, referral to a neurosurgeon is indicated, a craniotomy may be indicated.

Lateral Orbitotomy

Figure 11.7: Left orbital schwannoma axial view

Figure 11.8: Left orbital schwannoma sagittal view

organized overall the MRI and CT findings suggested the lesion was a possible cavernous hemangioma. An urgent excision biopsy was arranged via a lateral orbitotomy approach. Macroscopically the tumor was found to be cystic and wrapped around optic nerve. The lesion was debulked. As the surgery proceeded the pupil became fixed and dilated. No further debulking was attempted and the wound closed. Postoperatively the pupil gradually recovered and vision remained 6/6. Histopathological examination revealed the lesion to be a cystic Schwannoma. Referral was made to the oncology service and the tumor treated with radiotherapy.

A lateral orbitotomy approach is employed to biopsy tumors arising in the anterior third of the orbit. The lateral 1/3 of the skin crease is marked with pen and extended down parallel to the lid margin to a height level with the lateral canthus. The line is then extended laterally in a horizontal plane for 1½ cm. Local anesthetic is injected subcutaneously in this region. A skin incision is made along this line with a cutting diathermy. The deep tissues are blunt dissected down to the periosteum. This is incised with the diathermy and the periosteum blunt dissected off the bone exposing the lateral wall from the frontozygomatic suture down to the lower border of the lateral wall just above the opening of the zygomaticofacial foramen. At the orbital rim the periobita is elevated off the internal aspect of the lateral wall of the orbit. The orbital contents are displaced nasally with a malleable retractor. Two holes ½ cm apart and ¾ cm back from the orbital margin are drilled parallel to the orbital rim at the superior border of the exposed bone and 2 at the bottom. A gap of approximately 3 cm will exist between the higher of the 2 lower holes and the lower of the upper 2 holes. The lateral orbital wall is incised with a power saw between the drilled holes both superiorly and inferiorly. The cuts extended for approximately 2 cm in a radial direction to an area where the bone thins. A malleable retractor should be used to protect the globe during this procedure. The section of bone is dissected off with forceps and stored in normal saline. Once the lesion is identified care should be taken to avoid the globe, optic nerve and rectus muscles, while incision biopsies are taken using a 15° blade and forceps. Pupil reactions are monitored throughout the procedure. Hemostasis is achieved with bipolar cautery. The lateral orbital wall is replaced by threading 4.0 prolene through the

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Orbital Tumors of Neurological Origin 169 pre-holes at the orbital rim and tied in place. The tissues are closed with 5.0 vicryl and skin closed with 5.0 prolene. A reducing dose of steroids is given for 18 days along with a histamine H2 receptor antagonist such as ranitidine. A head-light is worn throughout the procedure to ensure adequate illumination of the operating field. Where a drain is used this can be removed the following day.

REFERENCES 1. Dutton JJ. Gliomas of the anterior visual pathway. Survey of Ophthalmology 1994;38(5):427-52. 2. Jahraus CD, NJ Tarbell. Optic pathway gliomas. Pediatric Blood and Cancer 2006;46(5):586-96. 3. Miller NR. Primary tumours of the optic nerve and its sheath Eye 2004;18(11):1026-37. 4. Kaufman LM, O Doroftei. Optic glioma warranting treatment in children Eye, 2006. 20(10):1149-64. 5. Rosenblum MK, Bilbao JM Ang L Neuromucular system Chap 28 in Surgical Pathology Ed Rosai (9th ed) 2004;2:2461682. 6. Dutton JJ. Optic nerve sheath meningiomas. Survey of Ophthalmology 1992;37(3):167-83. 7. Carrasco JR, RB Penne. Optic nerve sheath meningiomas and advanced treatment options. Current Opinion in Ophthalmology 2004;15(5):406-10.

8. Melian E, WM Jay. Primary radiotherapy for optic nerve sheath meningioma. Seminars in Ophthalmology, 2004;19(3-4):130-40. 9. Parsons JT, et al. Radiation optic neuropathy after megavoltage external-beam irradiation: analysis of timedose factors. International Journal of Radiation Oncology, Biology, Physics, 1994;30(4):755-63. 10. Subramanian N, et al. Cystic schwannoma of the orbita case series Orbit 2005;24(2):125-29. 11. Tezer MS, et al. Schwannoma originating from the infraorbital nerve: a case report. Auris Nasus Larynx 2006; 33(3):343-5. 12. Pollock BE, RL Foote, SL Stafford. Stereotactic radiosurgery: the preferred management for patients with nonvestibular schwannomas? Int J Radiat Oncol Biol Phys, 2002; 52(4):1002-7. 13. Rutherford SA, AT King. Vestibular schwannoma management: What is the 'best' option? Br J Neurosurg, 2005;19(4):309-16. 14. Tsuzuki N, et al. Cystic schwannoma of the orbit: case report. Surg Neurol, 2000;54(5):385-7. 15. Rosai J Soft Tissues Chap 25 in Surgical Pathology Ed Rosai (9th ed) 2004;2:2237-371. 16. Chua CN, Alhady M, Ngo CT, Swethadri GK, Singh A, Tan S Solitary nasal neurofibroma presenting as compressive optic neuropathy. Eye 2006;20(12):1406-8. 17. Rose GE, Wright JE. Isolated peripheral nerve sheath tumours of the orbit Eye 1991;5(6)668-73. 18. Dailey RA, Sullivan SA, Wobig JL. Surgical debulking of eyelid and anterior orbital plexiform neurofibromas by means of the carbon dioxide laser. American Journal of Ophthalmology, 2000;130(1):117-19.

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170 Surgical Atlas of Orbital Diseases

12

Mesenchymal Tumors

CHAPTER E Weis, J Rootman

Mesenchymal Soft Tissue Tumors Tumors that are believed to arise from mesenchymal tissues occur in multiple sites with variable biologic behavior. In the orbit mesenchymal tissues include striated and smooth muscle, fibrous tissue, fat, cartilage, and bone. Bone tumors are discussed separately in Chapter 13. Despite orbital mesenchyme arising from neural crest, lesions do not differ from other locations in the body in which mesenchymal tissues arise from mesoderm. In our orbital center mesenchymal tumors account for 1.6% of orbital lesions and 9% of neoplasias; in children they constitute 5% of all disease and 19.4% of childhood neoplasia.1

Striated Muscle Tumors Rhabdomyosarcoma Epidemiology Rhabdomyosarcoma accounts for approximately 20% of all soft-tissue sarcomas making it the most common soft tissue sarcoma in children.2, 3 The head and neck is the principal location and the orbit the second most common site in the head and neck (the most common being the parameninges) accounting for about 10% of all rhabdomyosarcomas. 4-6 Rhabdomyosarcoma is thus the most common primary orbital malignancy of childhood. In our clinic it constitutes 1% of all orbital neoplasias, and 6% in children.1 It has occurred from birth to the seventh decade, but 70% present in the first decade with a mean age of 8. The embryonal subtype affects mainly children, the alveolar mainly adolescents (median age 16), and

the much rarer anaplastic (pleomorphic) subtype most commonly presents in older people (median age is 54).7-11 Embryonal rhabdoymyosarcoma is the most common subtype accounting for 49% of all rhabdomyosarcomas and their predominance in the orbit is even greater since this subtype has a predilection for the orbit and parameninges.12 Most cases are sporadic although familial, congenital, and multiple tumors in the same patient including retinoblastoma have been reported.13 Most evidence suggests that they arise from primitive mesenchyme and not from skeletal muscle as they can develop in areas with no skeletal muscle.14

Presentation The typical presentation is rapidly developing exophthalmos over weeks (mean of 5) with 60% presenting with signs of inflammation including conjunctival and eyelid swelling.9,10,15 Two-thirds of primary orbital rhabdomyosarcoma present with a mass in the superonasal quadrant.15,16 The differential diagnosis is that of a childhood progressive rapidly developing mass with or without inflammation: infantile hemangioma, lymphangioma, neuroblastoma, chloroma, cellulitis, and non-specific orbital inflammation.

Imaging There are no specific radiologic findings in rhabdomyosarcoma.17 Local bone invasion has been reported in 24% of cases,18 with destruction of the orbital wall without orbital expansion seen in 30%.15,19 CT imaging typically shows a homogeneous (92%) well-defined soft tissue mass without bone

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Mesenchymal Tumors 171 destruction that takes up contrast in a moderate to marked amount; rarely the mass is poorly defined as it invades surrounding structures. It is most often extraconal (87%), in the supero-nasal quadrant (66%), (Figures 12.1A and B) and may displace but does not appear to arise from the extraocular muscles.15,16,20 Invasion of the sinus is noted in 20%. 15 Focal hemorrhage or necrosis may result in heterogeneity.17 MRI typically shows isointensity to skeletal muscle and hypointensity to orbital fat on T1, hyperintensity on T2 to orbital fat and muscle, decreased signal intensity on all pulse sequences, and moderate to marked uptake of gadolinium.16,17,20

Classification The International Classification of Rhabdomyosarcoma combined previous histologic classification schemes to provide a system based on prognosis.12 The Pleomorphic subtype was excluded from this classification system because of its rarity in children (Table 1). The WHO classification has divided rhabdomyosarcoma into embryonal, alveolar, and pleomorphic subtypes. The spindle cell and botryoid are considered variants of the embryonal subtype.21 The alveolar subtype more commonly presents in the inferior orbit.

included the lung (66% of metastasis), lymph nodes, and bone marrow. 22 Interestingly, lymph node metastasis is highly related to site of origin with lower rates seen in orbital tumors.22 Since the development of combination therapy, including biopsy/ conservative surgery, radiation, and multi-agent chemotherapy, the majority of children are now surviving. 14 Survival is related to pre-treatment tumor extension past the site of origin, size, nodal involvement, and metastasis at presentation. 6 Anatomic site is related to prognosis with the orbit having the best prognosis (92% survival). 5,23,24 Histologic type, as previously mentioned, is also associated with survival.12 (Table 1). Management begins with pathologic confirmation and staging. Two contrasting clinical philoshophies have emerged from the main clinical trial groups. The American Intergroup RhabdoTable 1: The International classification of Rhabdomyosarcoma (1995)

Management Before the 1960’s rhabdomyosarcoma was an almost uniformly fatal disease. Common metastatic sites

A

Superior prognosis

Botryoid Spindle cell

Intermediate prognosis

Embryonal

Poor prognosis

Alveolar Undifferentiated

Subtypes whose prognosis

Rhabdomyosarcoma with

is not presently available

rhabdoid features

B

Figures 12.1A and B: This 11-year-old child presented with a 3-week history of swelling of the left upper lid associated with ptosis and intermittent diplopia. It was a nonpainful swelling. She had an interpalpebral fissure of 5 mm on the left compared to the right at 8 mm, with 3 mm downward and 1 mm axial displacement of the left globe. This was associated with a 2 diopter left hypotropia in primary position, which increased to 6 diopters in upgaze. There was a solid, rubbery, palpable mass just behind the superior oblique tendon adjacent to the trochlea. On CT scan, there was a well-defined, homogeneous, hyperdense extraconal mass in the superomedial orbit displacing the eye and medial rectus muscle downward. The superior muscle group appeared displaced laterally. Because of the rapid development of this mass, an incisional biopsy was performed. Histopathologically, the mass was consistent with an embryonal rhabdomyosarcoma. Repeat investigations revealed no other evidence of tumor, and the patient underwent chemotherapy and radiotherapy. She is alive and well 17 years later with pseudophakos (20/25-2) and some enophthalmos

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172 Surgical Atlas of Orbital Diseases myosarcoma Study Group (IRSG) have tended to be more aggressive by utilizing routine radiotherapy, except for tumors that are totally excised, followed by prolonged chemotherapeutic regimes.25,26 Whereas the European International Society of Pediatric Oncology (SIOP) have attempted to use short course chemotherapy and have avoided surgery or radiotherapy if possible.27-29 The best treatment option is likely somewhere between these two philosophies.30 The vast majority of recurrences occur within 3 years of presentation and can often be treated with chemotherapy and repeat excision. In cases with refractory orbital tumors, salvage surgery has been shown to be beneficial.31 Complications related to treatment include cataract (55-82%), dry eye (30-36%), radiation retinopathy (6%), and bony hypoplasia (24-59%) secondary to radiation induced damage. 15,32 Secondary malignancies are rare (1.2-3%) and most commonly occur in patients who have received alkylating agents and radiation.15,33

Rhabdomyoma Tumors of skeletal muscle differentiation are atypical in that malignant are more common than the benign. Rhabdomyoma is a rare benign tumor that has had only 6 cases described in the orbit.34-38 Extracardiac rhabdomyomas are divided into 4 categories: adult, fetal, genital, and the rhabdomyomatous mesenchymal hamartoma types. The adult and fetal type have a predilection for the head and neck and the rhabdomyomatous mesenchymal hamartoma occurs mainly in the periorbital and perioral subcutaneous tissue in children.14 We have reported the only case of rhabdomyomatous mesenchymal hamartoma reported in the orbit.38 Tumor excision or debulking with observation have been described if symptomatic, since they can regress with time. 39 Recurrences are extremely rare and are typically associated with incomplete removal. Very little has been written about the radiologic findings of extracardiac rhabdomyoma.17 CT imaging demonstrates an ill defined homogeneous lesion that does not show signs of necrosis or hemorrhage with heterogeneous enhancement.37 The adjacent bone can be remodelled secondary to pressure but destruction is absent.37 MRI demonstrates a well defined mass similar to muscle on T1 and T2 weighted images with variable enhancement patterns.37,40-42

Smooth Muscle Tumors Smooth muscle tumors of the orbit are exceedingly rare and can arise from Muller’s muscle and the smooth muscle overlying the inferior orbital fissure. Leiomyoma is a benign slow growing lesion with three distinct clinical groups: (1) leiomyoma cutis, (2) deep dermal (genital leiomyomas), and (3) leiomyoma of deep soft tissue (musculoskeletal).14 The most common location for these tumors is the female genital tract.44 About 20 cases in the orbit have been reported with a mean age of presentation in the orbit of 36 years.43 Complete excision is the preferred treatment, since they are not sensitive to radiation, and no orbital recurrences have been found with complete excision. 43 Interestingly, regression is commonly seen with leiomyomas in other body sites.14 Leiomyosarcomas are rapidly growing infiltrative malignancies that can vary in their natural behavior. They account for 5-10% of soft tissue sarcomas2,45,46, are more common in females, present at a median age of 60,47 and they can occur in younger individuals who have received radiation therapy.48,49 Most commonly they are retroperitoneal, thus lesions outside this location are poorly understood; yet certain differences have been consistently documented. Extra-peritoneal location seems to have a better prognosis with a 5 year survival of approximately 64% and equal gender incidence.45,50 Although extremely rare in the orbit exenteration is the preferred treatment.51-55 Adjuvant chemotherapy and radiotherapy may be beneficial.56 Imaging of subcutaneous leiomyoma typically show a well-defined mass on MR with T1 intensity similar to skeletal muscle and T2 displaying a heterogeneous high or mixed signal intensity.17 Deep leiomyomas often show calcification, T1 intermediate signal intensity, T2 variable intensity, and marked contrast enhancement.17 Leiomyosarcomas do not display specific features on imaging. Hemorrhage, necrosis, and cystic change are common. A hypervascular mass with arteriovenous shunting is often seen on arteriography.17

Adipose Tumors Despite fat making up the majority of the orbital volume and lipomas being the most common soft tissue tumor in the body 14, tumors arising from adipose tissue in the orbit are rare. Lipomas should

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Mesenchymal Tumors 173 be differentiated from prolapsed orbital fat since they are often well circumscribed, slightly more yellow, and displace orbital structures.57 (Figures 12.2A to D) They present commonly in mid-life, are more frequent in men, are not related to race, and after an initial growth phase typically do not change in size significantly.58,59 Lipomas are divided into deep and subcutaneous/superficial lesions which are much more common.

A

C

Lipomas are identical to subcutaneous fat on CT and MRI.17 In the orbit they can have lower density than the surrounding orbital fat simulating a cyst.57 When the tumor is encapsulated a low signal fibrous capsule may be visualized on MR and CT. 17 Nonencapuslated lipomas have been reported making it difficult to localize them when adjacent to normal fat.67

B

D

Figures 12.2A to D: (A) This 21-year-old female noted a nonpainful, mildly tender swelling of the left lower lid for 2 years. There was no history or findings of increase in size with Valsalva maneuver and no ecchymotic episodes. On physical examination, the vision was normal. There was a soft palpable mass not attached to the underlying tissue with 2 mm of vertical displacement and 1.5 mm of relative enophthalmos. Ocular movements were normal. (B, C) CT scan demonstrated a well-delineated low-density lesion anteriorly (C-large arrow). The lesion appeared to be adjacent to orbital fat, which was of a higher density (C-small arrow). The lesion also appeared to cause bone excavation anteriorly (B-arrow). It was thought to be a lipomatous tumor. At the time of excision, a well-defined lipomatous mass was removed en bloc from the inferolateral orbit. (D) Histologically, it had the typical features of a lipoma. Reprinted with permission from Shah NB, Chang WY, White VA, et al. Orbital lipoma:2 cases and review of the literature. Ophthalmic Plastic and Reconstructive Surgery. 2007;23:203

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174 Surgical Atlas of Orbital Diseases Liposarcoma is one the most common soft tissue sarcomas of adult life,14 but is rare in the orbit.1,60 Thirty five cases have been reported in the literature, but misclassification is likely since our definition and pathological evaluation has improved over time.61,62 In orbital cases the average age was 64 years of age with a female predilection. 55 The most common presentation is painless proptosis with an 8 month mean duration of symptoms.55 Three distinct entities have been described, each validated with cytogenetic evidence. In order of good to poor prognosis they are: well-differentiated (adipocytic, sclerosing, inflammatory, spindle cell, and dedifferentiated), myxoid/round cell, and pleomorphic.14,63 In the orbit the myxoid is the most common subtype.62 Unlike lipomas which are primarily subcutaneous in location liposarcomas are primarily deep tumors; if they occur superficially they are referred to as atypical and have an excellent prognosis since they are often amenable to complete surgical resection.64-66 It is generally believed that lipomas cannot transform into liposarcomas; the distinction in location between lipomas and liposarcomas is the primary argument cited as proof of this.14 Clinical and histopathologic diagnosis can be difficult. Treatment involves surgical excision which typically involves exenteration, although well circumscribed cases have been resected. Liposarcomas of the orbit have a favorable prognosis with no reports of a neoplasm, that meets today’s histopathological criteria for diagnosis, metastasizing and only one recurring locally.61,62 When a lesion with imaging characteristics similar to a lipoma with an adjacent non-fatty mass, liposarcoma should be added to the differential diagnosis. Liposarcomas vary from approximately a 25-75% fatty component depending on their level of differentiation.68 Liposarcomas also show calcification in approximately 12% of cases which is more common than in lipomas.17 Few papers have described the imaging in the orbit. Jakobiec et al. described central lucency due to fat seen on CT imaging resembling a cystic structure. 61 They also reported that these lesions commonly involved the extraocular muscles and one case was hyperintense on MRI T1 imaging.61 A single case report described unique characteristics including an extraconal mass with MRI T2 imaging showing hypointensity in comparison to cerebral cortex and light peripheral enhancement with gadolinium.54

Lesions shown to mimic fatty tumors on imaging include myxoid tumors, lesions associated with a subacute hematoma, muscle with fatty replacement, and tumors invading surrounding fat.17

Fibrous Tissue Tumors Despite the large number of subtypes of fibrous tumors, only a few have been reported in the orbit. Fibrosarcomas have been diagnosed much less commonly over time as our definition and pathological assessment has advanced.14 Recent series have found that they are slightly more common in men69 and usually present in the early 40’s. 70, 71 Recurrence is very common occurring in approximately 45% with surgical margins being the best predictor. 69 Scott et al.’s series found that recurrence occurred in 79% of tumors with inadequate margins and in 18% of those treated with wide or radical excision.69 Five year metastasis rates are around 63% and are typically to the lung, spine, and skull.69 A 5 years survival rate of approximately 40% has been reported.69 These lesions are rare as a primary tumor of the orbit but they can also invade from the nasal cavity or face. Exenteration or excision with wide local margins is recommended since they are commonly incompletely removed. CT and MR imaging typically show an aggressively infiltrating orbital mass,72 however infantile lesions have been reported to be well circumscribed.73 Solitary fibrous tumor classically occurs in the pleural lining of the lung, but has been described throughout the body including the orbit, adjacent nasal cavity and sinuses.14 Approximately 55 cases have been reported in the orbit since its first description there in 1994 with most cases being benign.74-76 It has been seen in all age groups, with a median age of 50,77 and usually presents as a wellcircumscribed yet unencapsulated lesion causing gradual proptosis.1 Pleural solitary fibrous tumor can vary greatly in its clinical characteristics and malignancy on pathological assessment. Even less is known about non-pleural solitary fibrous tumors. Approximately 10% of non-pleural lesions have been shown to recur and metastases are very rare. 78 Pathological assessment for atypical features was found to be a significant predictor of recurrence.78 Since these tumors are difficult to remove due to invasion and they may undergo malignant transformation en bloc resection is recommended.79

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Mesenchymal Tumors 175 On CT the lesions are well circumscribed and take up contrast intensely in a homogeneous or ring pattern,76 although one review suggests that contrast uptake is mild to moderate.72 With MRI these tumors also enhance intensely, commonly showing areas of hemorrhage and T2 hypointensity.72

Histiocytic Tumors Fibrous histiocytoma Fibrous histiocytomas histologically and clinically can vary from a slowly developing benign to locally aggressive fast growing malignant lesion. Four subtypes have been described in order of frequency: storiform-pleomorphic, myxoid (myxoidfibrosarcoma), giant cell (malignant giant cell tumor of soft parts), and inflammatory (xanthosarcoma, malignant xanthogranuloma). 14 Benign fibrous histiocytoma most commonly affects the skin but approximately 0.3% can occur in deeper locations such as muscle tissue. 80 Recurrence rates for benign cutaneous fibrous histiocytomas following excision range from 5-10%,14,81 with deeper tumors typically presenting as a much larger lesion more often resulting in incomplete resection and thus recurrence rates have been reported closer to 50%.82,83 Malignant fibrous histiocytomas are the most common adult malignant soft tissue tumor in the body.84 Font et al.’s orbital series found that 63% were benign, 26% were locally aggressive and 11% were malignant.82 They showed that if the tumor had infiltrative margins, hypercellular zones or both, recurrence rates were 57% compared to 31% in those that did not have these features.82 Typically they are slow-growing, firm, infiltrative, and present in the upper nasal quadrant with a mean age of 43 years.82 Common presenting symptoms include proptosis (60%), mass (46%), and decreased vision (25%) with a mean duration of symptoms of 29 months. 82 Complete surgical excision is recommended. This is typically accomplished by removing the tumor alone although in some infiltrative cases this might not be possible without exenteration. Recent large systemic series have reported recurrence rates from 19-21%, metastasis, mostly to lung and bone, in 31-35%, and 5 year survival rates from 65-70%.85-87 In contrast

ten year survival rates for orbital tumors is about 90%.1,82 It can be difficult to differentiate fibrous from other lesions.17,72 (Figures 12.3A and B) No specific ultrasonic and CT imaging characteristics have been found (Figures 12.4A to E).88 Imaging is also highly variable related to amounts of collagen, necrosis, hemorrhage, and myxoid tissue but often displays high signal intensity on T2. Benign tumors are typically well-circumscribed and may remodel bone, with malignant lesions typically having infiltrating margins and bone destruction. 72 Benign histiocytomas are usually homogeneous on CT, and MRI T1 and T2 imaging, whereas malignant lesions have a more heterogeneous pattern. 72 In some cases malignant lesions may have a homogeneous CT and T1 image with a heterogeneous T2 image. 72 Histopathology and immunohistiochemistry help in the diagnosis (Figures 12.5A and B)

Malignant Tumors of Uncertain Type Rhabdoid tumor is a highly aggressive neoplasm primarily seen in the kidney although it has been reported in extrarenal sites including the central nervous system and soft tissues including the orbit.14,89-96 Extrarenal sites have approximately a 15% survival, but there are too few reported cases in the orbit to come to a conclusion as to whether they behave differently than other extrarenal sites. Of the 7 described in the orbit 4 have died.

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Figures 12.3A and B: (A) This 34-year-old woman presented with a history of right upper lid swelling and ptosis over 1 year, which was associated with tearing, light sensitivity and occasional sharp pain. Physical examination findings were a palpable lacrimal gland on the right, bilateral reduced Schirmer's function, 3 mm of ptosis and 3 mm of proptosis. There was a soft nontender mass superolaterally, which was causing a 3 mm downward and 4 mm inward displacement as well as indentation of the globe, leading to elevation of macula and disc (B)

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176 Surgical Atlas of Orbital Diseases

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Figures 12.4A to E: (A, B) The ultrasound for the patient depicted a well-defined solid lacrimal mass indenting the globe. (C to E) CT scan demonstrated a well-defined, solid, enhancing, slightly bosselated mass that indents the globe and causes excavation of the adjacent lacrimal fossa. The mass appeared distinct from the lacrimal gland and enhanced to a greater degree

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Figures 12.5A and B: The patient underwent an excision biopsy of the lesion, which at the time of surgery appeared yellow and soft. It was excised from the adjacent lacrimal gland. (A, B) Histologically, it was an encapsulated, somewhat myxomatous, vascular spindle cell lesion, which was negative for S100, keratin and epithelial membrane antigen but positive for smooth muscle antigen, vimentin and CD34. The histologic differential diagnosis included fibrous histiocytoma, myxoid tumor or leiomyoma

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Mesenchymal Tumors 177

REFERENCES 1. Rootman, J, Diseases of the Orbit: A Multidisciplinary Approach. 2nd ed. 2003, Philadelphia: Lippincott Williams and Wilkins. 2. Russell, WO, et al. A clinical and pathological staging system for soft tissue sarcomas. Cancer, 1977;(4):1562-70. 3. Masson, JK and EH Soule, Embryonal rhabdomyo-sarcoma of the head and neck. Report on eighty-eight cases. Am J Surg, 1965;110(4):585-91. 4. Maurer, HM, et al., The Intergroup Rhabdomyosarcoma Study-I. A final report. Cancer, 1988;61(2):209-20. 5. Maurer, HM, et al., The Intergroup Rhabdomyosarcoma Study-II. Cancer, 1993;71(5):1904-22. 6. Crist W, et al., The Third Intergroup Rhabdomyosarcoma Study. J Clin Oncol, 1995;13(3):610-30. 7. Ashton, N and G Morgan, Embryonal sarcoma and embryonal rhabdomyosarcoma of the orbit. J Clin Pathol, 1965;18(6):699-714. 8. La Quaglia, MP, et al., The effect of age at diagnosis on outcome in rhabdomyosarcoma. Cancer, 1994;73(1): 109-17. 9. Jones, IS, AB Reese, and J Kraut, Orbital rhabdomyosarcoma. An analysis of 62 cases. Am J Ophthalmol, 1966;61(4):721-36. 10. Jones, IS, AB Reese, and J Krout, Orbital rhabdomyosarcoma: an analysis of sixty-two cases. Trans Am Ophthalmol Soc, 1965;63:223-55. 11. Furlong, MA, T Mentzel, and JC Fanburg-Smith, Pleomorphic rhabdomyosarcoma in adults: a clinicopathologic study of 38 cases with emphasis on morphologic variants and recent skeletal muscle-specific markers. Mod Pathol, 2001;14(6)595-603. 12. Newton, WA, Jr, et al., Classification of rhabdomyosarcomas and related sarcomas. Pathologic aspects and proposal for a new classification—an Intergroup Rhabdomyosarcoma Study. Cancer, 1995;76(6):1073-85. 13. Howard, GM and VG Casten, Rhabdomyosarcoma of The Orbit In Brothers. Arch Ophthalmol, 1963;70:319-22. 14. Weiss, ww and JR Goldblum, Enzinger and Weiss’s Soft Tissue Tumors (4th ed) 2001, ST. Louis: Mosby. 15. Shields, CL, et al., Clinical spectrum of primary ophthalmic rhabdomyosarcoma. Ophthalmology, 2001;108(12):228492. 16. Sohaib, SA, I Moseley, and JE Wright, Orbital rhabdomyosarcoma—the radiological characteristics. Clin Radiol, 1998;53(5):357-62. 17. Krandsdorf, MJ and MD Murphrey, Imaging of Soft Tissue Tumors (2nd ed) 2006, Philadelphia: Lippincott Williams and Williams. 18. Simmons, M and AK Tucker, The radiology of bone changes in rhabdomyosarcoma. Clin Radiol, 1978;29(1): 47-52. 19. Kirkpatrick, JA and MA Capitanio, Radiology of the orbit in infancy and childhood. Radiol Clin North Am, 1972;10(1): 143-66.

20. Mafee, MF, E Pai, and B Philip, Rhabdomyosarcoma of the orbit. Evaluation with MR imaging and CT. Radiol Clin North Am, 1998;36(6):1215-27, xii. 21. Parham, DM and FG Barr, WHO classification of Tumors. Pathology and Genetics: Tumors of Soft Tissue and Bone. 2002, Lyon, France: IARC Press. 146-49. 22. Raney, RB, Jr, et al., Disease patterns and survival rate in children with metastatic soft-tissue sarcoma. A report from the Intergroup Rhabdomyosarcoma Study (IRS)-I. Cancer, 1988;62(7):1257-66. 23. Crist, WM, et al., Prognosis in children with rhabdomyosarcoma: a report of the intergroup rhabdomyosarcoma studies I and II. Intergroup Rhabdomyosarcoma Committee. J Clin Oncol, 1990;8(3): 443-52. 24. Newton, WA, Jr, et al., Histopathology of childhood sarcomas, Intergroup Rhabdomyosarcoma Studies I and II: clinicopathologic correlation. J Clin Oncol, 1988;6(1): 67-75. 25. Breneman, JC and ES Wiener, Issues in the local control of rhabdomyosarcoma. Med Pediatr Oncol, 2000;35(2):104-9. 26. Baker, KS, et al., Benefit of intensified therapy for patients with local or regional embryonal rhabdomyosarcoma: results from the Intergroup Rhabdomyosarcoma Study IV. J Clin Oncol, 2000;18(12):2427-34. 27. Martelli, H, et al., Conservative treatment for girls with nonmetastatic rhabdomyosarcoma of the genital tract: A report from the Study Committee of the International Society of Pediatric Oncology. J Clin Oncol, 1999;17(7):211722. 28. Baldini, EH, et al., Long-term outcomes after functionsparing surgery without radiotherapy for soft tissue sarcoma of the extremities and trunk. J Clin Oncol, 1999;17(10):3252-9. 29. Oberlin, O, et al., Treatment of orbital rhabdomyosarcoma: survival and late effects of treatment—results of an international workshop. J Clin Oncol, 2001;19(1):197-204. 30. McDowell, HP, Update on childhood rhabdomyosarcoma. Arch Dis Child, 2003;88(4):354-7. 31. Mannor, GE, et al., Multidisciplinary management of refractory orbital rhabdomyosarcoma. Ophthalmology, 1997;104(7)1198-201. 32. Raney, RB, et al., Late effects of therapy in 94 patients with localized rhabdomyosarcoma of the orbit: Report from the Intergroup Rhabdomyosarcoma Study (IRS)-III, 19841991. Med Pediatr Oncol, 2000;34(6)413-20. 33. Heyn, R, et al., Second malignant neoplasms in children treated for rhabdomyosarcoma. Intergroup Rhabdomyosarcoma Study Committee. J Clin Oncol, 1993. 11(2):262-70. 34. Knowles, DM, 2nd and FA Jakobiec, Rhabdomyoma of the orbit. Am J Ophthalmol, 1975;80(6):1011-8. 35. Di Sant’Agnese, PA and DM Knowles, 2nd, Extracardiac rhabdomyoma: a clinicopathologic study and review of the literature. Cancer, 1980;46(4):780-9.

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178 Surgical Atlas of Orbital Diseases 36. Hatsukawa, Y, et al., Rhabdomyoma of the orbit in a child. Am J Ophthalmol, 1997;123(1):142-4. 37. Myung, J, et al, Rhabdomyoma of the orbit: a case report. Pediatr Radiol, 2002;32(8):589-92. 38. Mavrikakis, I, et al., Orbital mesenchymal hamartoma with rhabdomyomatous features. Br J Ophthalmol, 2007;91(5): 692-3. 39. DiMario, FJ, Jr, et al., Evolution of cardiac rhabdomyoma in tuberous sclerosis complex. Clin Pediatr (Phila), 1996; 35(12):615-9. 40. Fukuda, Y, et al., Rhabdomyoma of the base of the tongue. J Laryngol Otol, 2003;117(6):503-7. 41. Metheetrairut, C, et al., Pharyngeal rhabdomyoma: a clinico-pathological study. J Otolaryngol, 1992;21(4):25761. 42. Ho, VT and VM Rao, Recurrent adult-type pharyngeal rhabdomyoma: MR appearance. AJR Am J Roentgenol, 1992;159(5):1130-1. 43. Arat, YO, et al., Leiomyoma of the orbit and periocular region: a clinicopathologic study of four cases. Ophthal Plast Reconstr Surg, 2005;21(1):16-22. 44. Farman, AG, Benign smooth muscle tumours. S Afr Med J, 1975;49(33):1333-40. 45. Gustafson, P, et al., Soft tissue leiomyosarcoma. A population-based epidemiologic and prognostic study of 48 patients, including cellular DNA content. Cancer, 1992;70(1):114-9. 46. Hashimoto, H, et al., Leiomyosarcoma of the external soft tissues. A clinicopathologic, immunohistochemical, and electron microscopic study. Cancer, 1986;57(10):2077-88. 47. Shmookler, BM and DH Lauer, Retroperitoneal leiomyosarcoma. A clinicopathologic analysis of 36 cases. Am J Surg Pathol, 1983;7(3):269-80. 48. Font, RL, S Jurco, 3rd, and RJ Brechner, Postradiation leiomyosarcoma of the orbit complicating bilateral retinoblastoma. Arch Ophthalmol, 1983;101(10):1557-61. 49. Folberg, R, et al., Orbital leiomyosarcoma after radiation therapy for bilateral retinoblastoma. Arch Ophthalmol, 1983;101(10):1562-5. 50. Farshid, G, et al., Leiomyosarcoma of somatic soft tissues: a tumor of vascular origin with multivariate analysis of outcome in 42 cases. Am J Surg Pathol, 2002;26(1):14-24. 51. Jakobiec, FA, et al., Leiomyoma and leiomyosarcoma of the orbit. Am J Ophthalmol, 1975;80(6):1028-42. 52. Wojno, T, RR Tenzel, and M Nadji, Orbital leiomyosarcoma. Arch Ophthalmol, 1983;101(10):1566-8. 53. Meekins, BB, JJ Dutton, and AD Proia, Primary orbital leiomyosarcoma. A case report and review of the literature. Arch Ophthalmol, 1988;106(1):82-6. 54. Hou, LC, MA Murphy, and GA Tung, Primary orbital leiomyosarcoma: a case report with MRI findings. Am J Ophthalmol, 2003;135(3):408-10. 55. Lin, IC, et al., Primary orbital leiomyosarcoma. Ophthal Plast Reconstr Surg, 2005;21(6):451-3.

56. Sturgis, EM and BO Potter, Sarcomas of the head and neck region. Curr Opin Oncol, 2003;15(3):239-52. 57. Shah, NB, et al., Orbital lipoma: 2 cases and review of literature. Ophthal Plast Reconstr Surg, 2007;23(3):202-5. 58. Rydholm, A and NO Berg, Size, site and clinical incidence of lipoma. Factors in the differential diagnosis of lipoma and sarcoma. Acta Orthop Scand, 1983;54(6):929-34. 59. Solvonuk, PF, et al., Correlation of morphologic and biochemical observations in human lipomas. Lab Invest, 1984;51(4):469-74. 60. Shields, JA, et al., Classification and incidence of spaceoccupying lesions of the orbit. A survey of 645 biopsies. Arch Ophthalmol, 1984;102(11):1606-11. 61. Jakobiec, FA, et al., Primary liposarcoma of the orbit. Problems in the diagnosis and management of five cases. Ophthalmology, 1989;96(2):180-91. 62. Cai, YC, et al., Primary liposarcoma of the orbit: a clinicopathologic study of seven cases. Ann Diagn Pathol, 2001;5(5)255-66. 63. Fletcher, CDM, Diagnostic Histopathology of Tumors (2nd ed) CDM Fletcher. 2000, London: Churchill Livingstone. 64. Linehan, DC, et al., Influence of biologic factors and anatomic site in completely resected liposarcoma. J Clin Oncol, 2000;18(8):1637-43. 65. Evans, HL, EH Soule, and RK Winkelmann, Atypical lipoma, atypical intramuscular lipoma, and well differentiated retroperitoneal liposarcoma: a reappraisal of 30 cases formerly classified as well differentiated liposarcoma. Cancer, 1979;43(2):574-84. 66. Kindblom, LG, L Angervall, and AS Fassina, Atypical lipoma. Acta Pathol Microbiol Immunol Scand [A], 1982. 90(1):27-36. 67. Roberts, CC, PT Liu, and TV Colby, Encapsulated versus nonencapsulated superficial fatty masses: a proposed MR imaging classification. AJR Am J Roentgenol, 2003;180(5): 1419-22. 68. Kransdorf, MJ, et al., Imaging of fatty tumors: distinction of lipoma and well-differentiated liposarcoma. Radiology, 2002;224(1):99-104. 69. Scott, SM, et al., Soft tissue fibrosarcoma. A clinicopathologic study of 132 cases. Cancer, 1989;64(4):925-31. 70. Pack, GT and IM Ariel, Fibrosarcoma of the soft somatic tissues; a clinical and pathologic study. Surgery, 1952;31(3): 443-78. 71. Pritchard, DJ, et al., Fibrosarcoma of bone and soft tissues of the trunk and extremities. Orthop Clin North Am, 1977. 8(4):869-81. 72. Dalley, RW, Fibrous histiocytoma and fibrous tissue tumors of the orbit. Radiol Clin North Am, 1999;37(1):185-94. 73. Lee, MJ, et al., Congenital-infantile fibrosarcoma: magnetic resonance imaging findings. Can Assoc Radiol J, 1996;47(2): 121-5.

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Mesenchymal Tumors 179 74. Westra, WH, WL Gerald, and J Rosai, Solitary fibrous tumor. Consistent CD34 immunoreactivity and occurrence in the orbit. Am J Surg Pathol, 1994;18(10):992-8. 75. Cerda-Nicolas, M, et al., Solitary fibrous tumor of the orbit: morphological, cytogenetic and molecular features. Neuropathology, 2006;26(6):557-63. 76. Meyer, D and F Riley, Solitary fibrous tumor of the orbit: a clinicopathologic entity that warrants both a heightened awareness and an atraumatic surgical removal technique. Orbit, 2006;25(1):45-50. 77. Dictor, M, et al., Myofibromatosis-like hemangiopericytoma metastasizing as differentiated vascular smoothmuscle and myosarcoma. Myopericytes as a subset of “myofibroblasts”. Am J Surg Pathol, 1992;16(12):1239-47. 78. Vallat-Decouvelaere, AV, SM Dry, and CD Fletcher, Atypical and malignant solitary fibrous tumors in extrathoracic locations: evidence of their comparability to intra-thoracic tumors. Am J Surg Pathol, 1998;22(12):150111. 79. Bernardini, FP, et al., Solitary fibrous tumor of the orbit: is it rare? Report of a case series and review of the literature. Ophthalmology, 2003;110(7):1442-8. 80. Fletcher, CD, Benign fibrous histiocytoma of subcutaneous and deep soft tissue: a clinicopathologic analysis of 21 cases. Am J Surg Pathol, 1990;14(9):801-9. 81. Niemi, KM, The benign fibrohistiocytic tumours of the skin. Acta Derm Venereol Suppl (Stockh), 1970;50(63):Suppl 63: 1-66. 82. Font, RL and AA Hidayat, Fibrous histiocytoma of the orbit. A clinicopathologic study of 150 cases. Hum Pathol, 1982;13(3):199-209. 83. Franquemont, DW, et al., Benign fibrous histiocytoma of the skin with potential for local recurrence: a tumor to be distinguished from dermatofibroma. Mod Pathol, 1990; 3(2):158-63.

84. Enzinger, FM, Malignant fibrous histiocytoma 20 years after Stout. Am J Surg Pathol, 1986. 10 Suppl 1:43-53. 85. Salo, JC, et al., Malignant fibrous histiocytoma of the extremity. Cancer, 1999;85(8):1765-72. 86. Le Doussal, V, et al., Prognostic factors for patients with localized primary malignant fibrous histiocytoma: a multicenter study of 216 patients with multivariate analysis. Cancer, 1996;77(9):1823-30. 87. Zagars, GK, JR Mullen, and A. Pollack, Malignant fibrous histiocytoma: outcome and prognostic factors following conservation surgery and radiotherapy. Int J Radiat Oncol Biol Phys, 1996;34(5):983-94. 88. Jacomb-Hood, J and IF Moseley, Orbital fibrous histiocytoma: computed tomography in 10 cases and a review of radiological findings. Clin Radiol, 1991;43(2): 117-20. 89. Haas, JE, et al., Ultrastructure of malignant rhabdoid tumor of the kidney. A distinctive renal tumor of children. Hum Pathol, 1981;12(7):646-57. 90. Sotelo-Avila, C, et al., Renal and extrarenal rhabdoid tumors in children: a clinicopathologic study of 14 patients. Semin Diagn Pathol, 1986;3(2):151-63. 91. Stidham, DB, et al., Congenital malignant rhabdoid tumor of the orbit. J Aapos, 1999;3(5):318-20. 92. Walford, N, et al., Intraorbital rhabdoid tumour following bilateral retinoblastoma. Histopathology, 1992;20(2):170-3. 93. Gunduz, K, et al., Malignant rhabdoid tumor of the orbit. Arch Ophthalmol, 1998;116(2):243-6. 94. Rootman, J, KF Damji, and JE Dimmick, Malignant rhabdoid tumor of the orbit. Ophthalmology, 1989;96(11): 1650-4. 95. Niffenegger, JH, et al., Adult extrarenal rhabdoid tumor of the lacrimal gland. Ophthalmology, 1992;99(4):567-74. 96. Gottlieb, C, et al., Congenital orbital and disseminated extrarenal malignant rhabdoid tumor. Ophthal Plast Reconstr Surg, 2005;21(1):76-9.

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180 Surgical Atlas of Orbital Diseases

13

Bone Tumors of Orbit

CHAPTER Venkatesh C Prabhakaran, Dinesh Selva

Primary bone tumors of the orbit constitute 0.6 to 2.0% of all orbital tumors.1, 2 They can be classified into 4 main sub-groups (Table 1).3 Clinically, the commonest bone tumors encountered are osteomas, fibrous dysplasia and cholesterol granuloma.

Clinical Presentation Bony lesions of the orbit usually present in one of three ways:3 1. Gradual proptosis and globe displacement occurring over many years secondary to a slowly progressive non-infiltrative mass effect. This is commonly seen in benign fibro-osseous tumors, such as the osteoma. 2. Sub-acute proptosis or globe displacement occurring over weeks or months which may be complicated by a sudden increase in the signs and symptoms secondary to an intralesional hemorrhage. This presentation is characteristic of reactive bone lesions. 3. Malignant bone tumors can present with infiltrative signs such as pain, restricted

movements and decreased vision occurring over weeks or months. On radiological examination, either destruction of bone or hyperostosis is seen. It is important to note that lesions other than primary bone tumors can also cause these changes. Thus the differential diagnosis of a destructive bony lesion includes epithelial malignancies of the paranasal sinuses, bony metastases, Wegener’s granulomatosis, lymphomas, fibrosarcoma and lytic meningioma. Hyperostotic lesions may be seen in metastatic prostatic carcinoma, meningioma and osteomyelitis. Only the three commonest lesions (osteoma, fibrous dysplasia and cholesterol granuloma) will be discussed in detail. The other conditions are briefly reviewed.

Osteoma Osteomas are the commonest bony tumors affecting the orbit. Orbital osteomas usually arise in the frontal or ethmoidal sinus. They usually present in the fourth and fifth decades and occur equally in males and females.4

Table 1: Clinico-pathological classification of primary orbital bone disorders Benign fibro-osseus and cartilaginous lesions

Reactive bone lesions

Neoplasms

Vascular

Osteoma Fibrous dysplasia Ossifying fibroma Chondroma

Cholesterol granuloma Aneurysmal bone cyst Giant cell granuloma Brown tumor of hyperparathyroidism

Langerhans’ cell histiocytosis Myeloma Osteosarcoma Ewing’s sarcoma

Intraosseus hemangioma

Osteoblastoma

Mesenchymal chondrosarcoma Giant cell tumor

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Bone Tumors of Orbit 181 Clinically, osteomas cause gradual proptosis and globe displacement occurring over many years. There may be an associated headache and a bony mass is usually palpable in the superior or superomedial orbit. Obstruction of the sinus ostia may lead to sinusitis or mucocoele formation. Uncommon features include acquired Brown’s syndrome, gazeevoked amaurosis or pain, globe subluxation and erosion leading to orbital emphysema or CSF rhinorrhea. Osteomas of the sphenoid sinus, though rare, are important as they can cause an orbital apex syndrome and optic atrophy.3 An important systemic association is Gardner’s syndrome which is a phenotypic variant of the familial adenomatous polyposis syndrome. Gardner’s syndrome is an autosomal dominant condition whose features include colonic polyps, osteomas, and congenital hypertrophy of the retinal pigment epithelium (CHRPE). As the rate of malignant transformation of the colonic polyps is very high, screening for Gardner’s syndrome (dilated funduscopic examination and gastroenterologist referral) is recommended in patients with osteoma.5 On X-ray and CT scans, osteomas are extremely radiodense, well circumscribed lesions, usually arising in the sinus and invading the orbit. Bone windows on CT usually show a very dense periphery with a more radiolucent center. Histologically, osteomas are composed of lamellar bone with fibrovascular stroma. They are divided into three types: ivory, compact and fibrous osteomas based on the relative proportions of lamellar bone and fibrous stroma. 6 The ivory osteomas are considered to be the most mature form of the lesion. However, there does not seem to be a correlation between clinical behavior and histological subtype.3 Asymptomatic osteomas are treated conservatively, except when involving the sphenoidal sinus, as it is easier to remove a small lesion before it encroaches on the optic canal. When symptomatic, treatment is by surgical excision. Anterior lesions are removed via an anterior orbitotomy. A modified Lynch incision may be used for superomedial osteomas. Endoscopic techniques may also be used.7 Posterior lesions may require a combined orbitocranial approach. Recurrence is rare, even following partial removal.

CASE ILLUSTRATION (Figures 13.1A to D) Fibrous Dysplasia Fibrous dysplasia develops almost exclusively in children in the first two decades of life, though the disease may progress well into adulthood. It is a deforming but not destructive disease of bone and is caused by proliferation of fibrous tissue and osteoid in medullary bone. Three forms are described: monostotic fibrous dysplasia accounts for most cases of orbital involvement; in polyostotic disease, deformities of long bones occur together with skull lesions; and McCune-Albright syndrome which is a triad of polyostotic fibrous dysplasia, sexual precocity and cutaneous pigmentation (with ‘coast of Maine’ borders). Most patients present with facial asymmetry, proptosis and globe displacement progressing over many years. Associated symptoms include diplopia, anosmia, hearing defects, nasal obstruction and epiphora.8 Increased intracranial pressure and cranial nerve palsies can also occur. Extensive disease results in a deformed facies known as ‘leontiasis ossea’. Progressive disease can result in optic nerve compression. Acute compressive neuropathy may occur secondary to intralesional hemorrhage, sphenoidal sinus mucocoele or secondary aneurysmal bone cyst.3 Imaging shows expansion of the bone with thinning of the overlying cortex. A ‘ground-glass’ appearance is common on CT. The disease usually affects multiple orbital bones, extending across suture lines. The main differential diagnosis is hyperostotic meningioma, which occurs in an older age group, and is differentiated on imaging by the presence of a soft tissue component. Histopathology shows a loose, moderately cellular fibrous stroma containing spicules of woven bone often with characteristic ‘C’ or ‘Chinese character’ shapes. Osteoblastic activity is inconspicuous.6 As the natural history of the lesion is usually one of slow progression, a conservative approach to management is recommended, unless functional

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182 Surgical Atlas of Orbital Diseases

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Figures 13.1A to D: A 19-year-old male presented with a dull headache and a swelling in the region of the left upper lid. On clinical examination (A), fullness was noted above the left medial canthus with downward displacement of the globe. A bony-hard mass could be palpated in the left superomedial orbit. Computed tomographic scan (B and C) revealed a highly circumscribed radio-opaque mass with a bosselated contour occupying the frontal sinus and extending into the left superonasal orbit. The bone window settings (B and C) demonstrated the radiolucent trabecular center surrounded by a dense periphery, characteristic of an osteoma. Surgical removal was performed using a stereotactic assisted sino-orbital approach. The sinus component was removed by a nasal endoscopic approach following a modified Lothrop procedure. The orbital component was drilled out through an external skin-crease approach. (D). The patient made an uneventful recovery following surgery

deficits develop. Surgical treatment requires a multidisciplinary craniofacial approach, with removal of as much of the affected bone as possible and reconstruction of the resulting defect in a single operation.9

CASE ILLUSTRATION (Figures 13.2A to D) Ossifying Fibroma This is a lesion peculiar to craniofacial bones. The mandible is the site of predilection but the orbit (usually the roof) may rarely be involved.6 It usually

presents in the first two decades of life with a very slowly progressive displacement of the globe. Imaging shows a well circumscribed lesion eroding the bone with a sclerotic margin and foci of internal calcification. Histopathology reveals trabeculae of bone and osteoid lined by osteoblasts in a cellular stroma.6 A ‘psammomatoid’ variant is described, in which ossicles remniscent of psammoma bodies are seen.10 This variant shows more aggressive behavior and has a greater risk of recurrence following incomplete excision.

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Figures 13.2A to D: This 22-year-old female presented with progressive decrease of vision in the left eye. Clinical examination revealed facial bony asymmetry with prominent frontal ridges (A). On cutaneous examination (B), a large nevus with the so-called “coast of Maine” borders was seen. Computed tomographic scan (C) showed extensive expansion of craniofacial bones extending across suture lines, with a “groundglass” appearance. These features are consistent with a diagnosis of polyostotic fibrous dysplasia (McCune-Albright syndrome). As the disease was causing visual compromise, left optic nerve decompression was performed via a craniotomy. Histology shows the typical Cshaped trabeculae of woven bone (D, arrow) set in a cellular fibrous stroma

It is important to distinguish ossifying fibroma from fibrous dysplasia as the former lesion is more aggressive, and left alone, inexorably enlarges and may enter into the cranium. As incomplete excision frequently leads to recurrence6, complete surgical excision is the treatment of choice.

Osteoblastoma This is a benign tumor composed of osteoblasts and is extremely rare in the orbit.11 It affects patients in the second to third decades and presents with a slowly progressive proptosis and globe displacement. The reported cases have arisen from the roof and

ethmoid sinuses and imaging shows an osteolytic lesion larger than 1 cm with a sclerotic margin. Histologically, trabeculae of lamellar bone with osteoblastic rimming are seen. The histological appearance is indistinguishable from that of osteoid osteoma (a lesion seen in long bones and measuring less than 1 cm in diameter).6 Surgical excision is the treatment of choice.

Chondroma Chondromas are benign cartilaginous tumors that may rarely be encountered in the orbit, usually near the orbital rim or trochlea.12 Radiologically, they are

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184 Surgical Atlas of Orbital Diseases seen as dense, well circumscribed masses. On histology, they are composed of lobules of mature cartilage.13 Excision is curative.

Cholesterol Granuloma Cholesterol granuloma is a foreign body response to the presence of crystallized cholesterol. It commonly involves the middle ear and temporal bone, but the orbit may rarely be affected. In the orbit, it occurs almost exclusively in the superolateral frontal bone.14, 15 Males in the fourth to fifth decades of life are predominantly affected. The usual presentation is that of a slowly progressive superolateral mass resulting in inferior globe displacement, proptosis and diplopia in upgaze. There may be associated headache or pain and a history of trauma may be elicited in some patients. CT imaging demonstrates an osteolytic lesion expanding and eroding the frontal bone and extending into the orbit and intracranially.14, 15 With MRI, high signal intensities are seen on both T1 and T2 weighted images (similar to dermoid cysts). Histology is characterised by the presence of numerous cholesterol clefts and an associated foreign body giant cell reaction. Curettage of the lesion via a percutaneous approach is curative. An endoscope may be helpful in visualizing areas behind the superior orbital rim.16

CASE ILLUSTRATION (Figures 13.3A to F) Aneurysmal Bone Cyst This cystic lesion of the bone is rare in the orbit and usually presents with a painless proptosis in the second decade of life.17 Sudden progression may occur following an intralesional hemorrhage. Imaging shows a destructive, expansile bony lesion usually involving the roof of the orbit. Curettage of the lesion is curative and histological examination shows blood filled fibrous spaces that lack an endothelial lining. Hemosiderin laden macrophages and bony trabeculae are seen in the fibrous stroma surrounding the spaces.3

Giant Cell Lesions A histological picture dominated by giant cells is seen in three different lesions: giant cell tumor, giant cell granuloma and ‘brown tumor’ of hyperparathyroidism. Giant cell tumor or osteoclastoma is usually seen in epiphyses of long bones and affects patients between the ages of 25-40 years. 6 It uncommonly involves the paranasal sinuses and can secondarily impinge upon the orbit.6 Histology shows evenly scattered multinucleated giant cells containing between 10-100 nuclei. The stromal cells contain nuclei resembling those of the giant cells.6 En bloc excision is usually curative. Giant cell granuloma, also known as giant cell reparative granuloma, is a rare destructive lesion of bone that presents with proptosis and globe displacement. 18 Headache and pain may also be present. In contrast to giant cell tumor, giant cell granuloma is seen in younger patients (average age 18.6 years) and on histology, the giant cells are sparse and unevenly distributed and reactive new bone formation may be seen.6 This lesion responds well to curettage. ‘Brown tumor’ is histologically almost indistinguishable from giant cell granuloma. Clinically, however, it is associated with primary or secondary hyperparathyroidism. The increased osteoclastic activity leads to focal areas of bone resorption and hemorrhage. As treatment of hyperparathyroidism usually results in spontaneous healing of the bony lesion, it is important that a careful clinical evaluation is performed in patients with histology suspicious for ‘brown tumor’.19

Osteogenic Sarcoma Osteogenic sarcoma of the orbit is rare and is seen in the 4th and 5th decades of life in patients who have usually undergone previous radiotherapy for retinoblastoma or fibrous dyplasia.6 Primary orbital involvement is exceedingly rare.20 It develops rapidly over a period of weeks to months and can present with proptosis, pain, diplopia and visual impairment. Imaging shows a mixed lytic and sclerotic mass with indistinct margins. Histology shows sarcomatous

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Bone Tumors of Orbit 185 cells in a stroma with foci of osteoid formation.6 Treatment is by preoperative chemotherapy followed by resection and postoperative chemotherapy. The prognosis for craniofacial lesions, however, is poor.

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Figures 13.3A to F: This 50-year-old male presented with a 6-month history of right proptosis. Examination (A) showed 2 mm proptosis with 3 mm inferior displacement of the right eye. Computed tomographic scan (B) revealed an osteolytic lesion involving the right superolateral frontal bone. On magnetic resonance imaging, the mass showed high signal intensity on the T1-weighted image (C). The T2-weighted image showed a similar appearance. Based on the clinical and radiological findings, a diagnosis of cholesterol granuloma was made. Anterior orbitotomy was performed through an upper lid skin crease incision (D). A friable mass was seen protruding from beneath the superior orbital rim (arrow) and the lesion was curetted out. The portion of the lesion behind the superior orbital rim and abutting the dura was removed using endoscopic visualization. Histology (E) showed the characteristic cholesterol clefts (arrow) surrounded by a granulomatous inflammation. The patient made an excellent recovery (F)

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Chondrosarcoma

Myeloma

These are slow-growing, non-metastasizing locally aggressive tumors commonly arising in the paranasal sinuses and invading the orbit.13 Imaging reveals a well-defined osteolytic lesion with internal stippling. Multiple lobules of hypercellular cartilage with binucleate cells in lacunae and mitotic figures are seen on microscopy. Treatment is by radical resection but complete removal may not be possible in the craniofacial region and multiple recurrences may be seen.13

Multiple myeloma or solitary plasmacytoma may involve the orbit, usually in patients older than 50 years of age.23 Presentation is with subacute onset of pain and proptosis. An osteolytic area with a contiguous soft tissue mass is seen on imaging. Histology shows sheets of malignant plasma cells. The orbital lesions are treated with radiotherapy and chemotherapy is used for systemic disease.

Mesenchymal Chondrosarcoma This variant of chondrosarcoma has a predilection for the head and neck region. It may occur in the soft tissues of the orbit, almost exclusively in females in the second and third decades of life.21 The clinical course is rapid and patients present with proptosis and infiltrative effects of less than a year’s duration. Imaging shows a non-specific, irregular, mottled soft tissue mass. On histology areas of poorly differentiated mesenchymal cells intermixed with lobules of mature cartilage are seen. As metastasis, especially to the lungs, can occur, exenteration is the treatment of choice.21

Ewing’s Sarcoma This is a small round cell tumor of bone mainly affecting patients in the first two decades. Orbital involvement is usually by metastases or spread from adjacent areas (Ewing’s sarcoma is responsible for 10% of pediatric orbital metastasis). Primary Ewing’s sarcoma of the orbit is exceedingly rare.22 Clinically, a rapidly developing non-axial proptosis is noted and imaging reveals expansile mass with bone destruction. Microscopy shows a featureless small round cell proliferation. PAS positive glycogen granules may be seen in the cytoplasm. Immunohistiochemistry (for CD99) is helpful in making the diagnosis. Treatment is by chemotherapy followed by resection or radiotherapy.

Langerhans’ Cell Histiocytosis (LCH) LCH results from an abnormal proliferation of Langerhans’ cells- specialized histiocytic cells normally seen in the epidermis and characterised by ‘racquet-shaped’ cytoplasmic granules (Birbeck granules) on electron microscopy. One form of LCH, previously known as eosinophilic granuloma, preferentially affects the skull and presents as a localized lytic lesion of bone. The disease is usually seen in young males and the children classically present with proptosis secondary to a superolateral orbital lesion.24 On CT, a central radiolucent area with an enhancing rim is seen. Histologically, a granulomatous infiltrate with Langerhans’ cells and prominent eosinophils is observed. The treatment of choice is curettage, though intralesional steroid injections and low-dose radiotherapy have also been used. An endoscopic aided curettage may achieve complete removal of the lesion.

CASE ILLUSTRATION (Figures 13.4A to D) Intraosseous Hemangioma This is a rare vascular tumor which presents as a slowly developing orbital mass, often associated with pain or tenderness. 25 The frontal bone is usually affected and on CT, a well-defined, radiolucent mass that expands the inner and outer tables of the bone is seen. Histologically, the lesions are hemangiomas composed of thin-walled vascular spaces lined by endothelium. Treatment is by surgical excision of the lesion with a rim of normal bone. Preoperative angiography should be performed.

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Figures 13.4A to D: This 5-year-old boy presented with a 3-week history of pre-septal cellulitis involving the left upper lid that had failed to respond to antibiotics (A). A computed tomographic scan revealed an irregular, osteolytic lesion involving the left frontal bone and extending through the roof of the orbit into the anterior cranial fossa (B). This lesion was approached via an upper lid skin crease incision. The periosteum was reflected off the roof of the orbit and endoscopic visualization was used to effect safe curettage of the entire lesion, including from areas abutting the dura (C); long arrow: superior orbital rim; short arrow: dura). Histology (D) showed a polymorphic inflammatory infiltrate with predominance of eosinophils, characteristic of Langerhans cell histiocytosis. Staging showed this to be unifocal, unisystem disease and he had no further treatment. There was no recurrence of the disease after a 3-year follow up (Figure 13.4D courtesy of Prof T.Y. Khong, Adelaide)

CASE ILLUSTRATION (Figures 13.5A to C)

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Figures 13.5A to C: A 57-year-old female presented with a one-year history of a mass over the left forehead that was gradually increasing in size. She was otherwise asymptomatic. Examination revealed a bony mass over the left frontal-orbital region. Ophthalmic examination revealed a 2 mm inferior displacement of the left globe. A computed tomographic scan showed a mass lesion within the left frontal bone. Intense contrast enhancement of the lesion was seen (A). Magnetic resonance demonstrated intermediate T1 and high T2 signal intensity (B). Biopsy of the mass was performed by the neurosurgeon and revealed an intraosseus cavernous hemangioma (C). Postoperative angiography showed that the lesion was supplied by the ethmoidal branches of the left ophthalmic artery and by the left middle meningeal artery. The supply was too small for embolization and the patient has been under observation for the last 3 years, with no further symptoms

REFERENCES 1. Rootman J, Chang W, Jones D. Distribution and differential diagnosis of orbital disease. In: Rootman J, ed. Diseases of the orbit, 2nd ed. Philadelphia: Lippincott Williams and Wilkins, 2003;53-84. 2. Shields JA, Bakewell B, Augsburger JJ, Flanagan JC. Classification and incidence of space-occupying lesions of the orbit: a survey of 645 biopsies. Arch Ophthalmol 1984;102:1606-11. 3. Selva D, White VA, O’Connell JX. Primary bone tumors of the orbit. Surv Ophthalmol 2004;49:328-42. 4. Henderson JW. Fibro-osseus, osseus, and cartilaginous tumors of orbital bone. In: Henderson JW, ed. Orbital tumors, 3rd ed. Philadelphia: Raven Press, 1994. 5. McNab AA,. Orbital osteoma in Gardner’s syndrome. Aust NZ J Ophthalmol 1998;26:169-70. 6. Fu YS, Perzin KH. Nonepithelial tumors of the nasal cavity, paranasal sinuses and nasopharynx: a clinicopathologic study. II. Osseus and fibro-osseus lesions, including osteoma, fibrous dysplasia, ossifying fibroma, osteoblastoma, giant cell tumor, and osteosarcoma. Cancer 1974;33:1289-305. 7. Chen C, Selva D, Wormald PJ. Endoscopic modified lothrop procedure: an alternative for frontal osteoma excision. Rhinology 2004;42:239-43. 8. Katz BJ, Nerad JA. Ophthalmic manifestations of fibrous dysplasia. Ophthalmology 1998;105:2207-15.

9. Jackson IT, Hide TA, Gomuwka PK, et al. Treatment of cranio-orbital fibrous dysplasia. J Maxillofac Surg 1982;10:138-41. 10. Margo CE, Weiss A, Habal MB. Psammomatoid ossifying fibroma. Arch Ophthalmol 1986;104:1347-51. 11. Leone CR, Lawton AW, Leone RT. Benign osteoblastoma of the orbit. Ophthalmology 1988;95:1554-8. 12. Jepson CM, Wetzig PC. Pure chondroma of the trochlea. Surv Ophthalmol 1966;11:656-9. 13. Fu YS, Perzin KH. Non-epithelial tumors of the nasal cavity, paranasal sinuses and nasopharynx: a clinicopathologic study. III. Cartilaginous tumors (chondroma, chondrosarcoma). Cancer 1974;34:453-63. 14. Arat YO, Chaudhry IA, Boniuk M. Orbitofrontal cholesterol granuloma: distinct diagnostic features and management. Ophthal Plast Reconstr Surg 2003;19:382-7. 15. McNab AA, Wright JE. Orbitofrontal cholesterol granuloma. Ophthalmology 1990;97:28-32. 16. Selva D, Chen C. Endoscopic approach to orbitofrontal cholesterol granuloma. Orbit 2004;22:49-52. 17. Ronner HJ, Jones IS. Aneurysmal bone cyst of the orbit: a review. Ann Ophthalmol 1983;15:626-9. 18. Spraul CW, Wojno TH, Grossniklaus HE, Lang GK. Reparative giant cell granuloma with orbital involvement. Klin MonatsblAugenheilkd 1997;211:133-4. 19. Parrish CM, O’Day DM. Brown tumor of the orbit. Arch Ophthalmol 1986;104:1199-202.

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Bone Tumors of Orbit 189 20. Parmar DN, Luthert PJ, Cree IA, et al. Two unusual osteogenic orbital tumors: presumed parosteal osteosarcomas of the orbit. Ophthalmology 2001;108:1452-6. 21. Guccion R, Font R, Enzinger F, Zimmerman L. Extraskeletal mesenchymal chondrosarcoma. Arch Pathol 1973;95:336. 22. Guzowski M, Tumuluri K, Walker DM, Maloof A. Primary orbital Ewing sarcoma in a middle-aged man. Ophthal Plast Reconstr Surg 2005;21:449-51.

23. Rodman HI, Font RL. Orbital involvement in multiple myeloma: review of the literature and report of three cases. Arch Ophthalmol 1972;87:30-5. 24. Jordan DR, McDonald H, Noel L, Nizalik E. Eosinophilic granuloma. Arch Ophthalmol 1994;111:134-5. 25. Relf S`J, Bartley GB, Unni KK. Primary orbital intraosseus hemangioma. Ophthalmology 1991;98:541-7.

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14

CHAPTER

Tumors of Lacrimal Gland Raman Mittal

Mass lesions of the lacrimal gland fossa account for a substantial proportion of orbital space-occupying lesions. These are mainly inflammatory, structural and neoplastic lesions. These lesions can be broadly classified as:1

Inflammatory Lesions 1. Infective i. Bacterial ii. Viral 2. Non-infective a. Idiopathic b. Specific i. Sjogren's syndrome ii. Sarcoidosis iii. Wegener's granulomatosis

Structural Lesions 1. 2. 3. 4.

Epithelial Cyst (Dacryops) Dermoid Mucocele Implantation cyst

Neoplastic Lesions 1. Epithelial neoplasms a. Benign Epithelial neoplasms i. Pleomorphic adenoma ii. Oncocytoma iii. Warthin's tumor iv. Myoepithelioma

b. Malignant Epithelial neoplasms i. Adenoid cystic carcinoma ii. Carcinoma in pleomorphic adenoma iii. Mucoepidermoid carcinoma iv. Adenocarcinoma and ductal carcinoma v. Low grade carcinoma vi. Other rare neoplasms — Acinic cell — Epithelial myoepithelial carcinoma — Sebaceous adenocarcinoma The main focus of this section will be a discussion of common lesions.

Epithelial Cyst (Dacryops) The term Dacryops may be used to mean any simple cyst of the lacrimal gland, whether it is in the palpebral or orbital lobe.2

Clinical Features Dacryops characteristically occurs in young adults or middle aged patients, as a unilateral or bilateral, painless, non-tender, fluctuant mass in the forniceal conjunctiva supero-temporally. Most cysts either remain relatively stable or demonstrate slow progression. The diagnosis can usually be made clinically. It may be difficult to differentiate a dacryops clinically from a simple epithelial cyst of conjunctival origin, which are commoner in nasal portion. It can be differentiated from a dermoid cyst by the fact that the latter is usually attached to bone.

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Pathology and Pathogenesis Grossly, the classic dacryops is a round cyst that contains clear fluid (tears) and is lined by epithelium. The epithelium may consist of one or two layers of relatively flat cells similar to those found in a lacrimal gland duct, or it may be composed of nonkeratinizing stratified epithelium with goblet cells similar to those in the conjunctiva. Normal lacrimal gland tissue is usually identified in the histologic specimen adjacent to the cyst. It is believed that a dacryops results from obstruction of one of the secretory ducts of the lacrimal gland.3 The obstruction results in progressive dilatation of the duct with formation of a thin walled cyst.

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Management When a dacryops is small and asymptomatic no treatment is necessary. If it is larger and symptomatic, it can be managed by simple aspiration. Aspiration can lead to recurrence, so it is advisable to remove the lesion surgically using either a conjunctival approach or a lateral orbitotomy.

Prognosis The prognosis for vision and life is excellent. Complications associated with a dry eye may occur if an excessive amount of lacrimal gland tissue and duct is removed.

CASE ILLUSTRATIONS Case 1 Mr V, 47 years male, presented with complaints of recurring redness and itching in both eyes. The patient had been diagnosed earlier as a case of allergic conjunctivitis and was treated accordingly. He also had small, round, non-tender, cystic lesions in the lacrimal gland area on both the sides. So the patient was diagnosed to be a case of dacryops in both eyes and was advised excision. (Figures 14.1A and B).

Pleomorphic Adenoma Pleomorphic adenoma is the most common benign epithelial tumor of the lacrimal gland. Typically they occur at a younger age (2nd-5th decades) than malignant tumors.

B Figures 14.1A and B: Mr V, with clear, cystic lesions (Dacryops) in both eyes

Clinical Features The characteristic presentation is of a slowly progressive (more than a year), painless proptosis, downward globe displacement and swelling of the upper lid, unassociated with inflammatory signs or symptoms. Larger tumors may indent the globe and cause blurring of vision and may cause diplopia. The common signs consist of proptosis, usually non-tender, palpable mass in the superotemporal quadrant, downward and inward globe displacement and sometimes restricted upgaze. Fundus examination may show globe indentation in larger tumors and also choroidal folds sometimes.

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Imaging Pleomorphic adenoma is best seen on CT scan. Usually a well circumscribed, homogenous or heterogenous, moderate to markedly enhancing soft tissue mass lesion is seen in the area of lacrimal gland. The scans may show pressure indentation over globe and expansion of the lacrimal fossa, suggesting chronicity of the lesion, in most cases. The mass may have few hyperdense areas suggestive of calcification. Ultrasound may reflect the histologic pattern with a highly reflective pseudocapsule, cystic spaces, and a well demarcated mass.

Pathology and Pathogenesis The pleomorphic adenoma of the lacrimal gland is characteristically firm, grayish-white, encapsulated and bosselated mass on gross examination. Histologically, the tumor is composed of both epithelial and mesenchymal elements. The epithelial elements take the form of ducts, cords and squamous pearls. The mesenchymal elements usually include myxoid and chondroid tissue and sometimes osseous tissue. The diverse patterns of two components account for the name, pleomorphic adenoma. An important feature is the presence of microscopic nodular extensions into the pseudocapsule. This may account for the tendency of the tumor to recur when appropriate margins are not taken. The pathogenesis is not clear. It appears that both the cellular and stromal elements are derived from epithelial cells lining the acini and ducts.

Management If there is a strong clinical suspicion of the lacrimal gland tumor being pleomorphic adenoma, on the basis of slow growing lesion, and absence of pain, motility disturbance and bony expansion, then it is best to excise the tumor completely without capsular rupture and without a prior incisional biopsy. Incomplete excision or capsular rupture may lead to a recurrence, sometimes with malignant transformation. Therefore, an incisional biopsy is probably contraindicated if the diagnosis is strongly suspected clinically. The most appropriate approach is by a modified lateral orbitotomy. The important aspects are wide surgical exposure, excision of the periorbita, careful manipulation of the tumor to avoid rupture, removal

of a margin or adjacent tissue, and where possible, preservation of the uninvolved palpebral lobe (reducing the incidence of postoperative filamentary keratopathy).

Prognosis The prognosis of the patient with pleomorphic adenoma of the lacrimal gland is generally very good. It is likely that greater attention to a complete en bloc excision will decrease the chance of recurrence and malignant transformation.

Case 2 Ms P, 20 years female, presented to me with protrusion of the right eyeball for 1 year, associated with reduced visual acuity. She had gradually increasing non-axial proptosis of the right eyeball. As you see in the figure (Figure 14.2A) the eyeball was displaced down and in. I could palpate a hard, non-reducible mass in the right supero-temporal orbit. There was neither tenderness nor any sign of inflammation. The mass was not pulsatile. The extraocular motility of right eye was restricted in upgaze, dextroelevation and dextroversion. Anterior segment of both the eyes were within normal limits, but the fundus of the right eye had folds of the internal limiting membrane, suggesting indentation of the globe by tumor. Her CT scan (Figures 14.3A and B) showed a fairly well defined orbital mass in the area of lacrimal gland. The mass was indenting over the globe. No bony erosions could be seen. The moulding of the orbital wall contours suggested chronic and benign nature of the lesion. My clinical diagnosis was right lacimal gland tumor, most probably a pleomorphic. Adenoma: I did lateral orbitotomy to excise off the tumor completely. Cryoextraction of the tumor was done. It was a well encapsulated grayish white mass measuring 2.6 × 2 × 1.5 cm. (Figure 14.4A). Gross examination of the cut section showed grayish and chalky white areas and also cystic areas filled with mucin material. Histopathologic examination (Figure 14.4B) showed a dimorphic picture with epithelial and stromal cells in close proximation. There were glandular and dilated cystic spaces lined by double

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Figure 14.2A: Ms P, Non-axial proptosis right eye

Figure 14.2B: Well aligned eyes postoperatively

Figures 14.2A and B: Clinical picture of Ms P. Preoperative picture (A) showing non-axial proptosis of the right eyeball. The globe has been displaced downwards and inwards. Postoperative picture (B) showing that both the eyes are now well aligned

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Figures 14.3A and B: Axial and coronal sections of the CT scan showing fairly well defined orbital mass in the area of lacrimal gland

A Figure 14.4A: Picture of the excised lacrimal gland tumor

B Figure 14.4B: Photomicrograph of pleomorphic adenoma (H and E stain)

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194 Surgical Atlas of Orbital Diseases layered cuboidal cells with secretions in the lumen. Stroma consisted of basaloid cells with spindle appearance, indistinct cell borders with elongated vesicular nucleus with stellate pattern. There was abundant myxoid matrix in between the stromal cells. These all histopathologic features confirmed the diagnosis of Pleomorphic Adenoma of Lacrimal Gland, right orbit. Postoperatively, proptosis resolved completely (Figure 14.2B).

Case 3 Mr K, 50 years old male presented to us with severe pain, swelling and redness of right periocular area for 15 days. He gave the history of gradually increasing painful protrusion of the right eyeball for about a month for which he underwent evisceration 2 weeks back. On examination, I could palpate a moderate to hard mass filling the supero-temporal and temporal part of right orbit. The lids and adnexa were inflamed with conjunctival chemosis (Figure 14.5A). CT scan orbit was done which showed a well circumscribed, soft tissue mass in the supero-lateral quadrant of right orbit (Figures 14.6A and B). The mass was mildly enhancing on contrast. There were no bony erosions. A disfigured globe (S/P evisceration) was seen. So, now clinically, the sequence of events that I thought was; the patient had gradually progressing lacrimal gland tumor (most probably pleomorphic adenoma) which lead to severe proptosis with exposure keratopathy and/

Figure 14.5A: Preoperative picture of Mr K. Note the superior sulcus fullness

or perforated corneal ulcer. The previous surgeon had addressed the corneal complication by performing evisceration, without tackling the primary cause, i.e. lacrimal gland tumor. I did lateral orbitotomy to excise off the tumor completely. Cryoextraction of the tumor was performed (Figure 14.7). Histopathologic examination of this well encapsulated tumor showed cells arranged in dimorphic pattern. The background showed myxoid stroma in between. These features were consistant with Pleomorphic Adenoma of Lacrimal Gland, right orbit. He was provided with a prosthesis after the postoperative edema subsided and the patient was comfortable (Figure 14.5B).

Adenoid Cystic Carcinoma Adenoid Cystic Carcinoma (ACC) is the most common malignant epithelial tumor of the lacrimal gland. It has a tendency for bimodal occurrence i.e. more common in 2nd and 4th decades.

Clinical Features The most important feature is a short duration of onset, i.e. usually few months. Generally patients also complain of having persisting pain, usually associated with paresthesia. Other clinical features include those which are seen in other lacrimal gland tumors as well. To enumerate, these are; a mass lesion in superotemporal quadrant, proptosis, downward and nasal displacement of the globe, ptosis and decreased visual acuity.

Figure 14.5B: Postoperative appearance of patient with readymade ocular prosthesis

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B Figures 14.6A and B: Axial and coronal sections of CT scan orbit show a well defined mass located at the lacrimal gland area of right orbit

may show the tumor as isointense to brain and extraocular muscles.

Pathology and Pathogenesis

Figure 14.7: Gross appearance of the lacrimal gland tumor (Pleomorphic adenoma) along with a bone piece from lateral orbital wall

Imaging CT scan is the imaging modality of choice. An important feature (that differentiates many malignant lesion from a benign one) is bony erosions or osteolytic changes in the adjacent bone. Other features that help me to say that the lacrimal gland tumor is Adenoid Cystic Carcinoma are irregular margins of the lesion and its extension towards the orbital apex. But on CT scan, the lesion may actually appear to be a well defined, solid and fairly homogenous and hence can be easily confused with a benign lesion. Presence or absence of calcification doesn't help much. In MRI, the T2 weighted images

On gross examination, adenoid cystic carcinomas are usually grayish white, firm and may give a false impression of being circumscribed or pseudoencapsulated. Histopathologically, ACC has solid areas or cords of malignant epithelial cells. It can be divided into several histologic subtypes: 1. Cribriform (Glandular or Swiss cheese) pattern: Shows multiple lobules with many small clear spaces, giving it a cribriform appearance. 2. Sclerosing variant: Consists of hyalinized cylinders of connective tissue and elongated epithelial cords, surrounded by a dense hyalinized stroma. 3. Basaloid variant: Shows solid epithelial lobules with large basophilic nuclei and scanty cytoplasm that resembles the lobules seen in basal cell carcinoma. 4. Comedocarcinoma variant: Shows epithelial lobules with large foci of central necrosis. 5. Tubular (Ductal) variant: Is composed of elongated and comma shaped epithelial tubules lined by two or three layers of cells. In practical terms, cribriform is the most common pattern. Basaloid pattern is the least common but is most aggressive and therefore has the worst prognosis.

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Management

Case 4

The mainstay of managing ACC remains complete surgical excision with adjuvant radiotherapy and chemotherapy. For very well defined lesions, local excision with the adjacent bone removal should be done; for not so well defined lesions, orbital exenteration and for tumors that have extended into the bone or soft tissues of the orbit, radical en bloc orbitectomy by a multidisciplinary team should be done. As it is difficult to ensure complete tumor excision, surgery should be followed by adjuvant radical external beam radiotherapy. This EBRT may prevent or delay local recurrence. The role of local, regional or systemic chemotherapy remains unclear in what is essentially an indolent tumor. We used a combination of carboplatin or cisplatin with 5-fluorouracil or doxorubicin in our patients as postoperative adjuvant therapy. 4 Among all the treatment options, chemotherapy has the greatest potential to eradicate occult metastatic disease.

This patient Mrs B, 18 years old female, presented to me with left blepharoptosis and mild enophthalmos. She gave the history of orbitotomy done elsewhere on left side for orbital tumor (as is evident from eyebrow scar in the picture) (Figure 14.8), about 5 years back. Old CT scan report (CT scan films were not available) suggested inhomogenous soft tissue mass in supero-lateral quadrant of left orbit. There was orbital fossa formation without bony erosions or intracranial extension. She was not aware of previous diagnosis nor was any histopathologic report available. I did Tarsofrontal Sling (Silicon rod sling) for her and advised her for CT scan orbits and regular follow up to rule out any tumor recurrence in future (Figure 14.9).

Prognosis What so ever we may do, the prognosis of adenoid cystic carcinoma remains dismal. The usual clinical course of ACC is painful local and regional recurrence followed by distant metastasis, usually to the lungs. Tumor can recur even at a very late date (even 20 years later). Most patients die within 5 years of recurrence.

Figure 14.8: Clinical picture showing left eye enophthalmos with ptosis status post lateral orbitotomy (Note eyebrow scar)

But the patient was lost to follow up for about 18 months, and therefter reported with massive proptosis and exposure keratopathy which had subacute onset (Figures 14.10A and B). CT scan done at this stage revealed a large heterogenous mass in the supero-lateral aspect of left orbit (Figures 14.11A and B). The mass was large enough to end just short of orbital apex. Roof of the left orbit appeared eroded at places but no obvious bony breach or intracranial extension was seen. Clinical and radiological findings suggested that I was dealing with a recurrence of Lacrimal Gland Tumor, and that too a malignant one. I sent a

Figure 14.9: Clinical picture 4 weeks after ptosis correction

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Tumors of Lacrimal Gland 197

A

B Figures 14.10A and B: Patient presenting with massive proptosis and exposure keratopathy of left eye caused by a large lesion in lacrimal gland area

B

A

Figures 14.11A and B: Axial and Coronal sections of CT scan orbit show a large heterogenous mass located at the lacrimal gland area of left orbit, ending just short of orbital apex. Note the bony erosions caused by the lesion

small tissue for cytology which strongly favored the diagnosis of Adenoid Cystic Carcinoma. Finally, I did lid sparing orbital exenteration (Figure 14.12A) and sent the specimen for histopathologic examination. Histopathologic examination (Figure 14.12B) showed tumor cells arranged in cribriform pattern

with abundant mucoid matrix in extracellular spaces. The features were consistant with Adenoid Cystic Carcinoma of lacrimal gland. The patient was referred to an oncologist to rule out systemic metastasis and give adjuvant radiotherapy and chemotherapy.

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198 Surgical Atlas of Orbital Diseases

A

B Figures 14.12A and B: Exenterated specimen (A) showing cribriform pattern of adenoid cystic carcinoma (H and E stain) (B)

REFERENCES 1. Diseases of Orbit, Textbook, 2nd Edition, Rootman J, Lippincott Williams and Wilkins, Chapter - Neoplasia, Page 344-45. 2. Bullock JD, Fleishman JA, Rosset JS: Lacrimal ductal cysts. Ophthalmol 1986;93:1355-60.

3. Smith S, Rootman J: Lacrimal ductal cysts. Presentation and management. Surv Ophthalmol 1986;30:245-50. 4. Muthy R, et al. Adenoid Cystic Carcinoma of the Lacrimal Gland: Management and Outcome. (Unpublished data).

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Cystic Lesions of Orbit 199

15

Cystic Lesions of Orbit

CHAPTER Golam Haider, Subrahmanyam Mallajosyula, Mohd Javed Ali

Cystic lesions of the orbit are not uncommon. A true orbital cyst is any closed cavity or sac within the bony orbital confines that is lined with epithelium and contains a liquid or semisolid materials.1 The cysts may be developmental or acquired.

Developmental cysts • • • • •

Dermoid / Epidermoid Cytic teratomas Encephalocele Congenital cystic eye Perioptic hygromas.

Acquired cysts • • • • • • • •

Dacryops Mucocele Dacryocele or amniontocele Hematic cyst Simple retention cysts Epithelial implantation cysts Chocolate cysts Parasitic cysts like cysticercosis and hydatid cyst • Cystic degeneration of certain tumors like lymphangiomas, optic nerve gliomas and schwannomas. Another classification proposed by Shields JA and Shields CL2 for pediatric cystic lesions of the orbit is as follows:

1. Cysts of surface epithelium: These are further divided into: • Simple epithelial cyst (Epidermal, conjunctival and apocrine gland cysts) • Dermoid cysts (Epidermal and conjunctival cysts). 2. Neural cysts: Further divided as: • Those associated with ocular maldevelopment like congenital cystic eye and colobomatous cyst. • Those associated with brain and meningeal tissues like cephalocele and optic nerve meningocele. 3. Secondary cysts: The most important secondary cyst is mucocele occurring in children secondary to cystic fibrosis. 4. Inflammatory cysts: These include parasitic cysts like cysticercosis and hydatid cyst. 5. Noncystic lesions with cystic component: These include certain tumors like adenoid cystic carcinoma, rhabdomyosarcomas and lymphangiomas. Parasitic encystment is more often an inflammatory granuloma. Hematic cysts are not lined with epithelium but with a fibrous encapsulation of blood or blood products. Malignant epithelial neoplasm that secondarily invades the orbit may develop central necrosis. Parasitic cysts is being dealt as a separate chapter in this book. Cystic degeneration of tumors is discussed in their respective chapters.

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200 Surgical Atlas of Orbital Diseases

DERMOID AND EPIDERMOID CYSTS Dermoid cysts are among the most common periorbital tumors presenting in childhood 3,4 (Figure 15.1). Its, incidence is 33% of all orbital cysts. Dermoid and Epidermoid cysts are choriostomas that arise from subcutaneous epidermal rests or epidermal tissues trapped along bony suture lines during embryonic development. These cysts are present congenitally and enlarge progressively. The superficial cysts are symptomatic in childhood but deeper dermoid may not be clinically symptomatic till in adulthood. The epidermoid enlarges and develops into a cyst lined with stratified squamous epithelium, which usually is attached to the fronto-zygomatic suture superotemporally or to the naso-frontal suture superonasally. If the cyst wall contains skin appendages such as hair follicles, sweat glands or sebaceous glands, the cysts are termed as dermoid cyst.5 If skin appendages are absent, the cysts are termed as epidermoid cyst. 6 Preseptal orbital dermoid cyst occurs most commonly in the area of lateral brow adjacent to fronto-zygomatic suture. Duane's experience is that these cysts have occurred with equal frequency both nasally and temporally. Medial lesions in the infant should be distinguished from congenital encephaloceles or amniontoceles. The mass is painless, smooth, ovoid to round in shape and firm to rubbery in consistency (Figure 15.4). It is immobile, being relatively attached to the periostium of the underlying suture. But it is not

Figure 15.1: A 9 months old female child with cystic swelling in lower part of orbit

attached to the overlying skin. In adulthood dermoid cysts may have a more posterior location. 7 The posterior located cysts may be difficult to palpate. Proptosis and globe displacement are common. Long standing dermoid in the superior orbit may completely erode the roof and become adherent to duramater. Rarely an orbital cyst may pass through bony sutureline to extend intracranially or into the temporal fossa. Pressure placed on the extracranial portion of such a bilobed cyst may be transmitted through the bony dehiscence into the orbit and is a cause for the mastication proptosis reported by Bullock.8 Less commonly, orbital inflammation may be the presenting sign due to leakage of oil or keratin from the cysts. Expansion of the dermoid cyst and inflammatory response to leakage may results in an orbital cutaneous fistula, usually following incomplete surgical removal.

Investigations CT scan may show a heterogenous lesion with rim enhancement, calcification, fossa formation in the bone (Figure 15.2), bone erosion, bone sclerosis and intracranial extension.

MRI Particularly important to see the cystic nature of the lesion when the cyst present as an inflammatory orbitopathy with surrounding tissue reaction.

Figure 15.2: CT scan of left orbit showing cystic space in lower part of orbit

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Cystic Lesions of Orbit 201

Treatment Surgical excision is the treatment of choice. Superficial cyst in childhood can be excised through an incision directly over the lesion or preferably through upper lid crease. A posteriorly located cyst may require more careful planning for an approach through an anterior or lateral orbital route9,10 (Figure 15.3). Cysts located along the superior orbital roof with intracranial extension needs neurosurgical consultations and possible transcranial approach.11,12 It is important to keep the cyst wall intact during surgery. Intraoperative rupture with release of its contents may incite granulomatous inflammation. When an inadvertent rupture occurs operating area must be flooded with irrigating solution. Complete removal of the cyst wall is curative (Figures 15.4 and 15.5). Incomplete removal may lead to recurrence.9

Figure 15.4: Dermoid cyst after removal

TERATOMAS Like dermoids teratomas are congenital tumor and choristomas.13,14 Dermoid and epidermiod cysts are developed from one germ layer but teratomas arise from two or more germ layers, including ectoderm and endoderm or mesoderm or both.15 Tumors may present at any age, many at or shortly after birth. Elsewhere teratomas are common in the gonads, mediastinum and pineal area. More than 50 cases have been reported in the orbit.16,17 Histologically, the tissues are usually matured and consist of ectoderm represented by keratinized

Figure 15.5: Case 5 days postoperatively

squamous epithelium and adnexal glandular structures; mesoderm by fibrous tissue, cartilage, fat, muscle and or bone; endoderm by gastrointestinal mucosa and glandular tissue and neuroectoderm by mature brain.16,17 Indication for surgery is unacceptable cosmetic appearance to the patient. One should preserve the globe whenever possible.18 Incomplete removal leads to recurrence. Exenteration is sometimes preferred because of fear of malignancy.15

CEPHALOCELE

Figure 15.3: Inferior orbitotomy for complete removal of cyst

A congenital dehiscence in the bony cranium may enable the meningeal tissue to herniate into the orbit forming cystic structure filled with CSF – an orbital meningocele. If brain protrudes inside the sac the

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202 Surgical Atlas of Orbital Diseases condition is termed as meningo-encephalocele. It may be fronto-ethmoidal or sphenoidal.19,20 Presentation is usually during infancy and childhood. It may present with pulsatile proptosis. Common site is superomedial aspect of the orbit. The mass is soft in consistency. The size of mass is increased by straining, bending forward or weight lifting. The direction of proptosis is inferotemporal. It may present with enophthalmos due to compression of orbital contents.21 Following conditions may be associated with encephalocele:22 1. Neurofibromatosis 2. Hypertelorism 3. Malar or orbital bone depression 4. Optic atrophy 5. Optic nerve coloboma 6. Optic nerve glioma 7. Microphthalmos 8. Morning-glory syndrome.

Radiological Finding CT scan may show defect in the anterior cranial fossa. 3-dimensional coronal views can detect encephalocele easily. When associated with sphenoid bone dysplasia, enlargement of superior orbital fissure in plain film appear as "Bare orbit". CT scan may reveal an enlarged middle cranial fossa. The temporal lobe of brain may herniate through the whole posterior orbit. Enlargement of pituitary fossa and optic canal may be associated with these bony defects.22

Treatment Surgical treatment is indicated in cases of exposure and lagophthalmos. Excision of small encephalocele through transfrontal craniotomy may be done. For a larger encephalocele, dural patching and bone grafting can be done.22

MICROPHTHALMOS WITH CYST Microphthalmos with orbital cyst result from the failure of the choroidal fissure to close in embryo. This condition may be unilateral or bilateral. The presence of an orbital cyst may be beneficial for stimulating normal growth of the involved orbital bone and eyelid. The cyst is lined internally by gliotic

retina and externally by fibrous envelop. The cysts are usually located in inferior orbit and cause the lower lid to bulge. In almost all cases resulting eye is defective, smaller than normal and has an attached cystic mass at birth.23,24 The cyst may be smaller or larger than the eye. Cyst is bluish in color and translucent, and may displace the globe. In contrast congenital cystic eye results from failure of optic vesicle to involute. The eye is filled with both solid and cystic form of dysplastic neuroglial tissue.25 The cyst is connected to the brain by an astrocytic filled stalk, but it does not communicate to the anterior ventricle. Both microphthalmos with cyst and congenital cystic eye should be distinguished from cystic teratoma and encephalocele by imaging studies.26 Management of microphthalmos with cyst and congenital cystic eye involves excision of the cysts and or globe along with abnormal neuroepithelial tissue.27 There has been a lot a work done on the use of sclerosing agents in such cysts. Ethanolamine oleate sclerotherapy may be an effective minimal intervention treatment option for cosmetic rehabilitation of patients with orbitopalpebral cyst associated with congenital microphthalmos with no visual potential.28,29 Each lesion is self centered and often an orbital implantation be placed and a prosthesis fitted to orbit in a satisfactory cosmetic effect. Sometimes removal of bone and reconstruction of the socket may be done.

MUCOCELE Destruction of sinus ostium due to recurrent inflammation, trauma or intensive mucosal disease result in a mucous filled sinus or mucocele which can expand slowly to involve the orbital cavity. If the sinus is inflamed and the cyst contain pus or mucous, the term pyocele and mucopyocele respectively, apply. It may occur at any age, most common in between (40-70) years. Mucocele from frontalethmoidal sinus are most common. The enlargement of the mucocele is insidious, with proptosis and displacement of the globe being manifest in association with a palpable, smooth wall mass in the upper and inner quadrant of the orbit. Mucocele of the posterior orbit may present more insidiously with

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Cystic Lesions of Orbit 203 a complaint of headache and orbital discomfort. The mucocele arises in the sphenoidal sinus and postethmoidal air cells and may mimic retrobulbar neuritis, or cause ophthalmoplegia and ptosis by affecting branches of 3rd cranial nerve along with axial proptosis.30,31 The differential diagnosis of cystic medial orbital lesion must also include encephalocele with skull base deformity. Appearance of a typical mucocele on a CT scan includes bowing of the sinus wall into the orbit, with attenuation or even erosion of bone with a cystic cavity. MRI shows highly variable signal intensities. Treatment of mucocele is surgical for reestablishment of normal drainage, removal of the lining of the cyst. Frontal and ethmoidal mucocele are approached sub-periosteally with elevation of an osteoplastic flap32; eradication and extirpation of all diseased mucosa is done. Successful endoscopic sinus obliteration and or re-establishing sinus drainage protects against recurrence. 33 Sphenoidal sinus mucocele is approached intranasally. Neurosurgical consultation is indicated for those mucoceles with intracranial extension.34,35

CYSTS OF THE OPTIC NERVE SHEATH Dilatation and expansion of the optic nerve sheath has been observed in variety of neoplastic and nonneoplastic condition. Such a cystic growth in a optic nerve sheath filled with CSF has been perioptic hygroma, arachnoid cyst of the optic nerve sheath, optic hydrops and meningocele.36,37 Most perioptic hygromas present in patients between the ages of 30-60yrs with complaints of headache and visual disturbance. Optic nerve signs are defective vision, RAPD, optic disc edema, optic atrophy and visual field defects. The cyst can be well delineated in MRI with fat suppression technique and gadolinium contrast.

HEMATIC CYST Hematic cyst refers to the accumulation of hematogenous debris within a cavity lined with fibrous tissue but not epithelial or endothelial tissue.40,41 It can occur at any age. It should be distinguished from endothelial-lined blood containing cyst such as chocolate cyst associated with lymphangioma, venous varices and hemangioma. Hematic cysts are uncommon. Proptosis, globe displacement, motility disturbance may occur due to chronic hematic cyst (Figure 15.6). Spontaneous eyelid ecchymosis and edema may suggest this diagnosis. In acute cases there may be decrease in vision, RAPD and choroidal folds.42, 43 Hematic cyst may be due to trauma and an incompletely reabsorbed orbital hemorrhage, wherein the presence of old blood and blood breakdown product incite a granulomatous inflammation and fibrous encapsulation. Others believe that a cyst arises from bleeding within the bony diploes that breaks out into the peripheral space.44

Investigations Plain film may show bony erosion when cyst arises in the superior orbit and involve the orbital portion of the frontal bone. CT shows well-defined nonenlarging mass (Figure 15.7) having the same density as the brain. On MRI T1 and T2 weighted signal hyper intensity are constant with blood.45

Treatment Surgical evacuation of the cyst content, removal of the fibrous wall lining and establishment of meticulous hemostasis to prevent recurrence. Removal of one wall of the cyst often is all that is necessary and all that is attached to the orbital tissue can be left intact (Figures 15.8 and 15.9).

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204 Surgical Atlas of Orbital Diseases

Figure 15.6: A 15 year girl with cystic swelling in the superior orbital region

Figure 15.9: Postoperative appearance 3 weeks after the operation

Simple Cyst Simple cyst may include serous, retention and implantation cyst. These are rare in the orbit but are slightly more common in the eyelid and conjunctiva. This cyst is lined by simple epithelium. Figure 15.7: CT scan shows well defined mass in the orbital apex

Retention Cyst It originates in the glandular appendages of the conjunctiva and adnexal structure. Obstruction of the orifice of the lacrimal gland or accessory lacrimal gland from trauma or cicatrix may result in a thin walled epithelium lined cyst in the superior fornix that can cause mass effect.

Lacrimal Ductal Cyst

Figure 15.8: Surgical approach for removal of hematic cyst

It arises often in the supero-lateral orbit and can be easily seen by everting the upper lid. These are frequently bilateral,46 they present as round lesions originating from the palpebral portion of the lacrimal gland which protrudes into the superior fornix. Marsupialization may be preferable to complete excision, because attempt to excise the cyst may unnecessarily close the remaining lacrimal ductal cyst.47,48 Ductal cysts can rarely arise from accessory lacrimal glands.49

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Cystic Lesions of Orbit 205

Implantation Cyst It arises from misplacement of surface epithelium into the orbit as a result of trauma or surgery. It is difficult to eradicate due to variable depth. Removal of the entire cyst or electrodessication of the epithelial lining effects a cure.50

DACRYOCELE Distension of the nasolacrimal sac by entrapped mucoid material results in the formation of a dacryocele due to obstruction of the nasolacrimal duct. A congenital dacryocele manifests at birth as a firm bluish swelling in the medial canthal area at or below the medial canthal tendon. Lacrimal sac is tense and filled with mucous and dacryocystitis with or without cellulitis may be a frequent presentation.51 Surgical indications are dacryocystitis, cellulitis, recurrent dacryocystitis, difficulties in breathing in large nasal cyst, failure to respond to conservative treatment. Difficulties in diagnosis of congenital dacryocele include - encephalocele, mucocele, dermoid cyst, and capillary hemangioma. Congenital dacryocele should be conservatively managed initially and if there is no response probing should not be delayed.52,53 Adult dacryocele needs dacryocysto-rhinostomy (DCR) surgery.53, 54

REFERENCES 1. Henderson JW, Farrow GM: Orbital Tumors, 2nd ed. New York, Brian C Decker, 1980. 2. Shields JA, Shields CL. Orbital cysts of childhood classification, clinical features and management. Surv Ophthalmol. 2004;49(3):281-99. 3. Stefanyszyn MA, Handler SD, Wright JE: Pediatric orbital tumors. Otolaryngol Clin North Am 1988;21:103. 4. Pfeiffer RL, Nicholl R J: Dermoid and epidermoid tumors of the orbit. Arch Ophthalmol 1948;40:639. 5. Ahuja R, Azar NF. Orbital dermoids in children. Semin Ophthalmol. 2006;21(3):207-11. 6. Yanoff M, Fine BS: Ocular Pathology: A Text and Atlas, 3rd ed. Philadelphia, JB L ippincott, 1989. 7. Rootman J: Diseases of the Orbit: A Multidisciplinary Approach. Philadelphia, JB Lippincott, 1988. 8. Bullock JD, Bartley GB: Dynamic proptosis. Am J Ophthalmol 1986;102:104. 9. Yen KG, Yen MT. Current trends in the surgical management of orbital dermoid cysts among pediatric ophthalmologists. J Pediatr Ophthalmol Strabismus. 2006;43(6):337-40; quiz 363-4.

10. Pryor SG, Lewis JE, Weaver AL, Orvidas LJ. Pediatric dermoid cysts of the head and neck. Otolaryngol Head Neck Surg. 2005;132(6):938-42. 11. Srivastava U, Dakwale V, Jain A, Singhal M. Orbital dermoid cyst with intracranial extension. Indian J Ophthalmol. 2004;52(3):244-6. 12. Yuen HK, Chong YH, Chan SK, Tse KK, Chan N, Lam DS. Modified lateral orbitotomy for intact removal of orbital dumbbell dermoid cyst. Ophthal Plast Reconstr Surg. 2004;20(4):327-9. 13. Duke-Elder S: System of Ophthalmology, Vol XIII, Normal and Abnormal Development: Part II. Congenital Deformation. St. Louis, CV Mosby, 1963. 14. Levin ML, Leone CR Jr, Kincaid MC: Congenital orbital teratomas. Am J Ophthalmol 1986;102:476. 15. Thomas J, Gregory L, Louis B. Orbital Neoplasm. Orbit, Eyelid and Lacrimal System. American Academy of Ophthalmology, 2004-2005;Section 7.64. 16. Lee GA, Sullivan TJ, Tsikleas GP, Davis NG Congenital orbital teratoma. Aust N Z J Ophthalmol. 1997;25(1): 63-6. 17. Gnanaraj L, Skibell BC, Coret-Simon J, Halliday W, Forrest C, DeAngelis DD. Massive congenital orbital teratoma. Ophthal Plast Reconstr Surg. 2005;21(6):445-7. 18. Chang DF, Dallow RL, Walton DS: Congenital orbital teratoma: Report of a case with visual preservation. J Pediatr Ophthalmol Strabismus 1980;17:88. 19. Mahapatra AK, Agrawal D. Anterior encephaloceles: a series of 103 cases over 32 years. J Clin Neurosci. 2006;13(5):536-9. Epub 2006 May 6. 20. Mahapatra AK, Suri A. Anterior encephaloceles: a study of 92 cases. Pediatr Neurosurg. 2002;36(3):113-8. 21. Macfarlane R, Rutka JT, Armstrong D, Phillips J, Posnick J, Forte V, Humphreys RP, Drake J, Hoffman HJ. Encephaloceles of the anterior cranial fossa. Pediatr Neurosurg. 1995;23(3):148-58. 22. Raman Sharma R, Mahapatra AK, Pawar SJ, Thomas C, AlIsmaily M. Trans-sellar trans-sphenoidal encephaloceles: report of two cases. J Clin Neurosci. 2002;9(1):89-92. 23. Waring GO III, Roth AM, Rodrigues MM: Clinicopathologic correlation of microphthalmos with cyst. Am J Ophthalmol 1976;82:714. 24. Lieb W, Rochels R, Gronemeyer U: Microphthalmos with colobomatous orbital cyst: Clinical, histological, immunohistological, and electron microscopic findings. Br J Ophthalmol 1990;74:59. 25. Chaudhry IA, Shamsi FA, Elzaridi E, Arat YO, Riley FC. Congenital cystic eye with intracranial anomalies: a clinicopathologic study. Int Ophthalmol. 2007;27(4):223-33. Epub 2007 Apr 24. 26. Brodic G E. Cystic Lesion of the Orbit In:Principle and Practice of Ophthalmology 2nd ed. WB Saunders Company, 2000 pp 3072. 27. Doglietto F, Massimi L, Dickmann A, Tamburrini G, Caldarelli M, Di Rocco C. Microphthalmia and colobomatous cyst of the orbit. Acta Neurochir (Wien). 2006;148(10):1123-5. Epub 2006 Sep 8.

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206 Surgical Atlas of Orbital Diseases 28. Naik MN, Murthy RK, Raizada K, Honavar SG. Ethanolamine oleate sclerotherapy in the management of orbito-palpebral cyst associated with congenital microphthalmos. Am J Ophthalmol. 2005;139(5): 939-41. 29. Naik MN, Honavar SG, Murthy RK, Raizada K, Thomas R. Ethanolamine Oleate Sclerotherapy Versus Simple Cyst Aspiration in the Management of Orbitopalpebral Cyst Associated With Congenital Microphthalmos. Ophthal Plast Reconstr Surg. 2007;23(4):307-11. 30. Ajaiyeoba A, Kokong D, Onakoya A. Clinicopathologic, ophthalmic, visual profiles and management of mucoceles. J Natl Med Assoc. 2006;98(1):63-6. 31. Herndon M, McMains KC, Kountakis SE. Presentation and management of extensive fronto-orbital-ethmoid mucoceles. Am J Otolaryngol. 2007;28(3):145-7. 32. Ulualp SO, Carlson TK, Toohill RJ. Osteoplastic flap versus modified endoscopic Lothrop procedure in patients with frontal sinus disease. Am J Rhinol. 2000;14(1):21-6. 33. Har-El G. Endoscopic management of 108 sinus mucoceles. Laryngoscope. 2001;111(12):2131-4. 34. Peral Cagigal B, Barrientos Lezcano J, Floriano Blanco R, García Cantera JM, Sánchez Cuéllar LA, Verrier Hernández A. Frontal sinus mucocele with intracranial and intraorbital extension. Med Oral Patol Oral Cir Bucal. 2006;1;11(6):E52730. 35. Shah A, Meyer DR, Parnes S. Management of frontoethmoidal mucoceles with orbital extension: is primary orbital reconstruction necessary? Ophthal Plast Reconstr Surg. 2007;23(4):267-71. 36. Harris GJ, Sacks JG, Weinberg PE, O'Grady RB: Cyst of the intraorbital optic nerve sheaths. Am J Ophthalmol 1976;81:656. 37. Miller NR, Green WR: Arachnoid cysts involving a portion of the intraorbital optic nerve. Arch Ophthalmol 1975;93:1117. 38. Moschos MM, Lymberopoulos C, Moschos M. Arachnoid cyst of the optic nerve: a case report. Klin Monatsbl Augenheilkd. 2004;221(5):408-9. 39. Akor C, Wojno TH, Newman NJ, Grossniklaus HE. Arachnoid cyst of the optic nerve: report of two cases and review of the literature. Ophthal Plast Reconstr Surg. 2003;19(6):466-9. 40. Krohel GB, Wright JE: Orbital hemorrhage. Am J Ophthalmol 1979;88:254.

41. Pearson PA, Rakes SM, Bullock JD: Letter: Clinicopathologic study of hematic cysts of the orbit. Am J Ophthalmol 1986;102:804. 42. Milne HL 3rd, Leone CR, Kincaid MC, Brennan MW. Chronic hematic cyst of the orbit. Ophthalmology. 1987;94(3):271-7. 43. Privat C, Bellamy J, Courthaliac C, Kinn T, Ravel A, Mondie J, Bacin F, Boyer L. Chronic hematic cyst of the orbit (orbital subperiosteal hematoma). J Radiol. 2000;81(7):811-4. 44. Cameron JD, Letson RD, Summers CG: Clinical significance of hematic cyst of the orbit. Ophthalmic Plast Reconstr Surg 1988;4(2):95. 45. Kersten RC, Kersten JL, Bloom HR, Kulwin DR. Chronic hematic cyst of the orbit. Role of magnetic resonance imaging in diagnosis. Ophthalmology. 1988;95 (11): 1549-53. 46. Tsiouris AJ, Deshmukh M, Sanelli PC, Brazzo BG. Bilateral dacryops: correlation of clinical, radiologic, and histopathologic features. AJR Am J Roentgenol. 2005;184(1):321-3. 47. Bullock JD, Fleishman JA, Rosset JS. Lacrimal ductal cysts. Ophthalmology. 1986;93(10):1355-60. 48. Brownstein S, Belin MW, Krohel GB, Smith RS, Condon G, Codere F. Orbital dacryops. Ophthalmology. 1984;91(11):1424-8. 49. Khoury NJ, Haddad MC, Tawil AN, Ma'luf RN. Ductal cysts of the accessory lacrimal glands: CT findings. AJNR Am J Neuroradiol. 1999;20(6):1140-2. 50. Wuebbolt GE, Zuercher M, O'Donnell B, Collin R. Epithelial implantation cysts of the upper eyelid after lid-lowering procedures. Ophthalmology. 1993;100(9):1289-92. 51. Mansour AM, Cheng KP, Mumma JV, Stager DR, Harris GJ, Patrinely JR, Lavery MA, Wang FM, Steinkuller PG Congenital dacryocele. A collaborative review. Ophthalmology. 1991;98(11):1744-51. 52. Schnall BM, Christian CJ Conservative treatment of congenital dacryocele. J Pediatr Ophthalmol Strabismus. 1996;33(5):219-22. 53. O'Keefe M, Shaikh A, Bowell R, Lanigan B. Management of congenital dacryocele. Acta Ophthalmol (Copenh). 1994; 72(1):122-3. 54. Rootman J. Disease of the Orbit : A Multidisciplinary Approach. Philadelphia, JB Lippincott,1995;149.

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16

Parasitic Cysts of Orbit

C HAP T E R Subrahmanyam Mallajosyula, Mohd Ather, Modini Pandarpurkar

The prevalence of parasitic cysts of orbit varies greatly in different regions of the world. For example cysticercosis which is endemic in Asia, Latin America and Africa,1 is rare in North America and European Countries. Nearly 15% of proptosis we treat is due to cysticercosis! Hydatid is the next common parasitic cyst. However, in view of the wide spread international travel, it is essential to know about these conditions, as revealed by the fact that many cases of neurocysticercosis were reported in USA.2,3 Cysticercosis: Cysticercosis is the larval form of Taenia Solium. Man is the definitive host for Taenia Solium. The larval form of cysticercosis occurs in infested pig, which is the intermediate host. Cysticercosis occurs in humans when they take contaminated food like improperly cooked or uncooked vegetables or through water. Cysticercosis can involve many organs and subcutaneous tissues. Neurocysticercosis is very common. Extraocular cysticercosis is more frequent than intraocular. We see 40-50 new cases of myocysticercosis per year while my vitreo-retinal colleagues see 4-6 cases a year. Most often we see solitary cysts. Multiple cysts or association with neurocysticercosis is rare.4,5 Very few cases of optic nerve involvement are reported.6,7,8 The clinical presentation of orbital cysticercosis is varied. The onset can be acute/sub-acute or intermittent. Pain and swelling can vary from severe to minimal. Ocular motility restriction and diplopia are very common. FDT is positive. The horizontal recti and superior rectus/LPS complex are commonly

involved. Most of the patients are young with an average age of 16.5 years. Myocysticercosis is the most common cause of acquired ptosis in the age group of 10-20 years in our series. A high degree of suspicion is needed to investigate and diagnose this condition. Anticysticercosis antibodies are positive in about 60% of cases. CT scan or Ultrasound B scan of orbit9,10 reveal enlarged muscle with a cyst showing a hyper dense spot within (which represents scolex). We prefer CT scan to B scan for the diagnosis, since we want to know if there is associated neurocysticercosis. If neurocysticercosis also coexists, we refer the patient to neurologist. If there is no neurocysticercosis, we treat the patient with oral Albendazole 15 mg/ kg.body.wt/ day in 2 divided doses for 4 weeks along with prednisolone. In those with severe inflammatory signs, we prescribe Prednisolone at a dose of 2 mg/ kg.body wt/ day and in others at 1 mg /kg.body wt/ day, and will taper over a period of 4 weeks. We follow the patients with B scan, since it is less expensive and easily available. Awareness of the disease, high index of suspicion and imaging are necessary to diagnose this condition. When the disease is for several months, and is not diagnosed and treated properly, the cyst migrates anteriorly . It can be seen in subtenon’s space, usually in relation to an extraocular muscle. Such cysts are excised, which on histopathology show a highly convoluted membrane with scolex. These patients are investigated for the presence of cysticercosis elsewhere and treated acoordingly.Rarely spontaneous extrusion may11 occur.

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When to suspect Myocysticercosis? Young patient (5-20 yrs) Stay/visit to endemic region Onset: Acute/Subacute/Intermittent Pain: Severe/moderate Proptosis: Mild to moderate and eccentric Ocular motility: Restricted, FDT positive, Diplopia common Soft tissue changes: Severe/minimal.

Investigations Serum Anticysticercosis: Positive in about 60% cases only. Imaging: CT/MRI preferred; Enlarged extraocular muscle with a cyst and scolex.

in short time, symptoms not in proportion to the signs, myocysticercosis was our clinical diagnosis. Plain CT scan of Orbits revealed enlarged LPS-SR complex with a cyst and scolex (Figures 16.3 and 16.4), confirming our clinical diagnosis. She was treated with Albendazole 400 mg bid for 4 weeks along with Prednisolone 90 mg/day (Her wt. was 45 kg) Since the inflammation was fairly severe as evidenced by severe edema of lids and markedly enlarged. LPS-SR complex. We started her on Prednisolone at 2 mg/kg.body wt./day and tapered over a months time. The response was quite dramatic as you can see in the Figure 16.5 which was taken 1 week after starting the medication.

Treatment Albendazole: 15mg/kg body wt/day in 2 divided doses × 4 weeks along with Prednisolone 1-2 mg/kg body wt/ day, tapered over 4 weeks.

Figure 16.1: Miss S, F16 presenting with severe edema of lids and swelling extending all round into periocular region of right eye

CASE ILLUSTRATIONS Case 1 Miss S, an young girl of 16 years in age was referred to us with the history of pain and swelling of right eye since 2 days. She had a similar episode 2 weeks back for which the referring doctor treated her with systemic antibiotics and she responded. As you could see, (Figure 16.1) she presented with marked swelling of both the lids of her right eye, upper lid being more severe. She complains of pain of moderate intensity. There is a slight raise of local temperature, mild tenderness, minimal chemosis, mild proptosis and normal ocular motility (Figure 16.2). We felt the chances of orbital infection are remote since it is difficult to explain why an infection recurs in such a short time in an otherwise healthy young woman. In view of the age, intermittency/ recurrence

Figure 16.2: On elevating the eye lid, the eye looks normal but for mild chemosis and minimal proptosis

Figure 16.3: Axial CT image shows enlarged LPS-SR complex enclosing a cyst

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Figure 16.4: Coronal CT image showing a markedly enlarged LPSSR complex enclosing a cyst. The scolex( hyper-dense spot) within the cyst can be easily made-out

muscle. It is not necessary to remove the cyst along with the casing. We prefer to de-roof the surrounding casing. Live cysticercus cyst usually shows motility. In fact, most often, it tries to come-out after an incision is made in the casing and tries to wriggle-out! These movements can be very well appreciated with the operating microscope. Normally there is no need to use a cryo for the extraction of the cysticercus, unlike in hydatid cyst, wherein the use of cryo is mandatory. The excised cyst showed a dense spot which was the scolex (Figure 16.9). Histopathology revealed convoluted membrane (Figure 16.10)

Figure 16.5: One week post treatment, the edema has almost subsided, the chemosis disappeared and proptosis reduced. There is a very marked clinical improvement

Case 2 Master V, a boy of 8 years presented with a swelling of right lower lid of 4 months duration, associated with mild pain. He gives history of occasional episodes of dull retrobulbar pain for which he did not consult any doctor. On examination a cystic lesion, 15 mm × 12 mm in size and involving lower eyelid of right eye was noticed (Figure 16.6). It is getting more prominent when the child is looking up, and less prominent when the child looks downwards, demonstrating its relation to inferior rectus. Transillumination was positive (Figure 16.7). The eye was very quiet. Ocular motility was normal CT scan of orbit revealed cysticercosis cyst with scolex (Figure 16.8). Since the cyst is anteriorly located, and is very easily accessed, it was excised through conjunctival approach. We found that most often the cysticercosis is encased in a wall which is about 2 mm thick and is very closely related to the extraocular

Figure 16.6: Large cyst involving right lower lid

Figure 16.7: Transillumination of the cyst

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Case 3

Figure 16.8: CT Orbits showing cyst with scolex

Baby H, a girl of 8 years presented with painful eccentric proptosis of left eye of 2 weeks duration. On examination fullness in supero-medial space with the globe pushed down and out and severe tenderness was present. Abduction was restricted (Figure 16.11). In view of short duration, pain and inflammatory signs, inflammatory /infective etiology was suspected. CT scan of the orbit revealed sub-periosteal abscess with a cystic lesion within (Figure 16.12). Careful examination revealed a white spot within it representing scolex (Figure 16.13). The subperiosteal abscess was drained through superior Lynch incision. Along with pus, a cysticercosis cyst also was removed (Figures 16.14 and 16.15). It is the first and the only case of cysticercosis involving the subperiosteal space that we encountered. It is one of the rarest presentations of

Figure 16.9: Excised cyst showing hyper-dense spot

Figure 16.11: Eccentric proptosis with fullness and inflammatory signs

Figure 16.10: Microphotograph showing convoluted membrane

Figure 16.12: CT scan showing subperiosteal abscess with a cyst

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Figure 16.16: Microphotograph showing cysticercosis with suckers Figure 16.13: Cyst with scolex

Figure 16.14: Incision mark on first postoperative day

Figure 16.17: One month postoperative picture showing normal restoration of movement

cysticercosis and the only one to the best of our knowledge and pub med search. The diagnosis was confirmed by histopathology (Figure 16.16). The postoperative recovery was uneventful. The ocular motility was restored (Figure 16.17). Very faint incisional scar can be made out.

Case 4

Figure 16.15: Excised cysticercosis

Mrs V, female 32 years, presented with sudden diminution of vision of her right eye since 2 weeks, associated with mild pain. Examination revealed mild fullness of superior sulcus, RAPD, and normal ocular motility. Optic disc was normal. Vision was 20/400 (Figures 16.18, 16.19 and 16.20). The possibility of retro bulbar neuritis was thought of. But to exclude any other pathology, CT imaging of orbit was performed which surprisingly revealed

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212 Surgical Atlas of Orbital Diseases cysticercosis involving the lateral rectus, abetting optic nerve at orbital apex (Figures 16.21 and 16.22). This is an unexpected diagnosis, since cysticercosis is rare after 20 years, and presenting like retro bulbar neuritis is very unusual. She was treated with oral Albendazole(15 mg/kg. body wt. in 2 divided doses /day) for 4 weeks and Prednisolone at a dose of 2 mg/kg.body wt./day tapered over a period of 4 weeks. This high dose of prednisolone was used because of associated optic neuropathy. The patient recovered very well. The patient's periocular fullness disappeared. The pain subsided (Figure 16.23). Her vision improved to 20/30. How ever she had a inferior arcuate scotoma (Figure 16.24). CT imaging after 4 months showed the presence of a shrunken cyst and scolex could not be seen

Figure 16.18: Shows mild periocular fullness. Her vision was 20/ 400. Pupil was dilated and sluggish with a significant RAPD

(Figure 16.25), which means that the cyst died. The patient is doing well for the past 2 years. This is a unique case scenario. Cysticercosis very rarely causes a toxic optic neuropathy. It is rare after 25 years of age. In fact at the age of 32 years, this patient is the oldest patient of cysticercosis we have come across so far. We never suspected the possibility of cysticercosis in this patient, but the hunch to get a CT helped in making correct diagnosis and provide correct treatment for this lady. We could restore her vision. This case clearly illustrates that even those who regularly come across cysticercosis can also miss a case, unless they are extra careful.

Figure 16.21: Cyst with hyper dense spot representing scolex, involving lateral rectus at the orbital apex

Figure 16.19: Showing normal abduction

Figure 16.22: Cyst abetting the optic nerve

Figure 16.20: Showing normal adduction

Figure 16.23: Subsidence of periocular edema, recovery of vision to 20/30

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Figure 16.24: Visual fields showing inferior arcuate scotoma

Figure 16.25: CT after 4 months shows a cyst which is shrunken and does not show the scolex

Case 5 Master R, a boy of 8 years presented with pain, proptosis, drooping of left upper eye lid, since 4 days, associated with headache and nausea and diplopia. There was no history of convulsions, vomiting or fever (Figure 16.26). On examination, periocular fullness, more on superior quadrant with eyeball pushed down, minimal congestion of conjunctiva, and restricted ocular motility in vertical gaze leading to vertical diplopia were evident (Figure 16.27). The vision was 20/20, pupil was normal and optic disc showed mild hyperemia with filling up of the cup. There were no

hemorrhages. Clinical diagnosis of myocysticercosis involving LPS-SR complex was made because of patients age, acute onset, with inflammatory signs, acquired ptosis, mild proptosis, and restricted ocular motility. In view of headache, nausea and early disc edema, the coexistence of neurocysticercosis was suspected. On imaging, of the orbit, (Figures 16.28 and 16.29) a markedly enlarged LPS-SR complex was noted. A large cystic lesion was seen in relation to the thickened muscles. Scolex was made out in the cyst. Imaging of the brain confirmed our suspicion. It revealed the presence of a calcified cyst in the brain parenchyma (Figure 16.30) with normal architecture of the surrounding brain. Typical " Coin Lesions" with surrounding edema of brain was noted in other parts (Figure 16.31). A total of 3 such active cysts were noted in the serial sections of the brain. The surrounding cerebral edema indicated that the cyst is live and active. Anticysticercosis antibodies were positive at 1.68 od units, as against the values of > 0.5 od units taken as positive. Most of the patients of neurocysticercosis present with convulsions. The unique features of this case are (1) absence of convulsions, (2) multiple cysts in the brain, three are active and one is inactive, association of orbital and neurocysticercosis. In view of associated neurocysticercosis the child was referred to neurologist who treated him with Albendazole, Prednisolone and Carbamazepam. The child recovered well. Six weeks after initiating the treatment, the ptosis disappeared, and normal ocular motility was restored.The optic disc edema subsided completely. There was no recurrence during the last 2 years of follow up (Figure 16.32).

Figure 16.26: Periocular swelling, ptosis, mild eccentric proptosis with the globe pushed down in a child of 8 yrs. Onset was acute

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Figure 16.27: Grossly restricted elevation of left eye, leading to vertical diplopia

Figure 16.30: Coronal CT showing enlarged LPS-SR complex in the orbit and a calcified mass in the brain parenchyma. Note that there is no surrounding edema

Figure 16.28: CT showing a large cystic lesion in relation to LPS-SR complex

Figure 16.29: Scolex in the cystic lesion

Figure 16.31: Axial imaging of brain shows the presence of typical “coin lesion” with very severe edema surrounding the lesion. 2 more similar “active” lesions were seen in other sections of imaging

Figure 16.32: 6 weeks post-treatment, the child recovered well from periocular swelling ptosis and proptosis

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Case 6 We wish to share our experience with a unique clinical experience. Mr. S 18 years presented with a swelling on his left eye of 4 days duration. He is a college student, doing his graduation. He gives history of mild pain and discomfort. There is no history of defective vision or diplopia. He noticed mild drooping of his left upper lid since 1 week, but did not pay attention due to his approaching examinations. However he noticed this morning that the swelling has enlarged and the discomfort has increased in intensity. His food habits include eating road-side junk food, raw vegetables and green salads. On examination (Figure 16.33) but for very minimal fullness and 1 mm of ptosis of his left upperlid,the rest looked normal. Ocular motility was normal (Figures 16.34 and 16.35). On elevating the upper lid, a cyst measuring 12 cm × 10 cm, and in relation to superior rectus was seen. Surrounding conjunctiva was congested. This is the typical appearance of cysticercosis in the sub-

Figure 16.35: The ocular motility was normal. The elevation as you can see here is full. He is orthophoric on cover test

Figure 16.36: Spontaneously extruded cysticercosis in a container Figure 16.33: Anterior segment looks almost normal

tenon's space. The cyst is always in relation to an extraocular muscle. Most often the surrounding conjunctiva shows congestion. Since the cyst was very anteriorly placed, excision was planned and Mr. S was advised to come after 2 days to the operation room for excision of the cyst under local anesthesia. He attended the operation room after 2 days, carrying a small bottle in which he placed “something” that came from his affected eye that morning (Figure 16.36). To our surprise the bottle contained cysticercosis cyst. This is an example of spontaneous extrusion.

Case 7

Figure 16.34: Note the cyst in relation to superior rectus muscle. Surrounding conjunctiva was congested

We are to share with you a very rare clinical presentation of cysticercosis, which we came across only once so far. The patient, master D a young boy of 12 years, was a student of class 7, and was from a

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216 Surgical Atlas of Orbital Diseases village, 80 miles away. He was brought with a history of sudden loss of vision of his left eye since 2 weeks. He complained of dull retro bulbar pain of 2 months duration. There was no history of trauma, fever or any other symptoms of systemic illness. On examination, (Figure 16.37) there was mild proptosis of his left eye, which was more evident on Nafzeiger’s (Figure 16.38). The ocular motility was normal. The pupil was dilated with a very significant RAPD. The vision was reduced to PL only. Retropulsion was mildly positive. Fundus Examination revealed optic disc which shows nasal hyperemia, and gross pallor on the temporal side, with exudates, macular fan and degenerative changes involving macula suggestive of neuroretinitis (Figure 16.39). This child was referred to us in view of proptosis of his left eye. The association between proptosis and neuroretinitis is very unusual and difficult to explain. The fundus picture was more like a neuroretinitis rather than disc edema. We discussed about the possibilities of a fungal infection extending from the sinuses with a vascular involvement, which could explain proptosis and sudden acute fall of vision (which is due to retinal

vascular obstruction) and could not explain neuroretinitis. The possibility of cysticercosis was also discussed, but we were not for it as we did not come across such a clinical presentation. Imaging by CT scan of orbit showed a large cyst in relation to the optic nerve and scolex could be very clearly seen (Figures 16.40 and 16.41).

Figure 16.39: Severe macular changes including macular fan, exudates and pallor of optic disc

Figure 16.37: Presents with sudden loss of vision of his left eye

Figure 16.40: Axial imaging of orbit shows a cyst in relation to optic nerve. Scolex is seen very clearly

Figure 16.38: Proptosis of left eye is better appreciated by Nafzeiger's test

Figure 16.41: Cyst involving optic nerve. Scolex could be seen in the center of the cyst

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Parasitic Cysts of Orbit 217 Anticysticercosis antibodies were positive which confirms the diagnosis of cysticercosis of optic nerve. The associated neuroretinitis was due to the severe inflammatory response produced by the toxins released, which was common with cysticercosis. The child was treated with oral Albendazole and Prednisolone. Surgical excision of the cyst through the antero-lateral orbitotomy was not considered in view of the severe irreversible damage already occurred, and the very poor visual prognosis. With treatment, proptosis disappeared, but his vision was absent PL.

Hydatid Cyst of Orbit Hydatid cyst is another parasitic infestation of the orbit, less common than cysticercosis. It is caused by echinococcus granulosus or the dog tapeworm. Dogs are the primary hosts with sheep and cattle being intermediary hosts. Humans are accidental hosts, acquiring infection by ingesting ova along with raw vegetables, contaminated water, or from direct contact with dogs. Embryos pass across duodenal mucosa to liver through the portal veins. Liver, lungs, and brain are primarily affected. Orbital disease is seen in only 1% of hydatid disease.12 Hydatid cysts form 0.3-5% of all orbital diseases.13,14 Hydatid infestation commonly presents in children and young adults. Most of the patients are below 16 years.13 Rarely elderly people are affected by this disease. Proptosis is the most common presentation of orbital hydatidosis.14 Usually, the proptosis is of a few months duration, associated with mild pain or discomfort. Hydatid cysts are commonly located in the intraconal space. In our experience it is the most common cystic lesion of intraconal space. Since intraconal space is the most common location, it can present with defective vision associated with RAPD and optic disc edema. Other causes of defective vision in hydatid cyst we came across include refractive errors and corneal perforation due to exposure keratitis in one case. Other symptoms can be periorbital pain, chemosis and headache.12 Diplopia due to ocular motility restriction can be a rare symptom. 15,16 In neglected and unattended long standing cases, these cyst grow to a very large size, deforming the globe (Figure 16.42).17 Diagnosis is by a high index of suspicion especially in endemic areas, and investigations like CT scan orbit. Sometimes

A

B

Figures 16.42A and B: CT Scan of orbit showing a well encapsulated, intraconal Hydatid cyst (A) Severe proptosis and distorted globe with anterior staphyloma in a neglected case of Hydatid disease (B)

orbital hydatid may be a part of disseminated disease,18 with lung, liver and brain being commonly involved and hence in every case of hydatid cyst, ultrasound of abdomen and CT scan of chest and brain were advised.18,19

Investigations • CBP- usually normal • Stool examination does not show ova or cysts • Casoni's intradermal test is positive in 75% of the people13 • CIEP- Counter immunoelectrophoresis • Imaging techniques of importance are orbital ultrasonography and CT scan. On ultrasound, diagnostic double wall sign is confirmatory, spoke wheel pattern and water lily sign are seen with cyst calcification. CT scan shows well encapsulated cystic mass with cyst fluid showing attenuation values of 3-30 HU. The mass indents and deforms the globe. Calcification of the internal septa may be seen. We prefer CT scan of the orbit. Microscopic analysis of the cyst fluid shows scolices and hooklets. The cyst wall is laminated and has the characteristic "coats of an onion" appearance. Management: We prefer surgical excision of the cyst by performing orbitotomy . The cyst wall is very thin and can rupture during surgery. Hence, to prevent it, after exposing the cyst, we prefer to aspirate the contents, so that the cyst shrinks in size. Then it can be very safely pulled out with the help of a cryo. Akon O. et al from Turkey 20 have advocated percutaneous aspiration of the cyst under ultrasonic guidance, followed by injection of 15% of hypertonic saline and reaspiration (PAIR technique). They showed a decrease in cyst size by three months and a marked decrease to 0.5 ml. by 9 months. Medical management of hydatid cysts and recurrent cysts with Albendazole and Praziquantal has also been tried.21

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CASE ILLUSTRATION

Figure 16.43: Male 35 years presented with proptosis of right eye of 2 years duration with mild pain and discomfort. Note the eccentric proptosis with the globe pushed down and out. Note also the fullness of superior sulcus. His BCVA was 20/80

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C

Figures 16.44A to C: CT scan of the orbit shows a very large cyst in the superior peripheral space, pushing the globe down and out

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C

Figures 16.45A to C: The cyst was exposed through superior lid crease incision. Note the tense cyst, as it is protruding-out (A). About 10 ml of the fluid was aspirated, and the collapsed cyst was removed with a cryo(B) The diagnosis of hydatid cyst was confirmed by the laminar / onion peel appearance

Figure 16.46: First postoperative day picture, showing clinical improvement Note the position of the globe which has come back to normal. Also there is marked improvement in the fullness of superior sulcus. Patient's vision improved to 20/30 in course of time

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REFERENCES 1. Del Brutto OH. “Neurocysticercosis: Updating in diagnosis and Treatment” Neurolgia, 2005;20(8):412-8. 2. Del la Garza Y, Graviss E A, Daver NG,Gambarin KJ, Shandera WX, Schanz PM, White AC Jr : “Epidemiology of Neurocysticercosis in Houston, Texas” Am J Trop. Med. Hyg.2005;73(4):766-70. 3. Towas JM, Hoffmann CJ,: “Neurocysticercosis in Oregon: 1995-2000” Emerg. Infect Dis 2004;10(3):508-10. 4. Pushker N, Bajaj MS, Balasubramanya R: “ Disseminated Cysticercosis involving Orbit, brain and sub-cutaneous tissue”J. Infect. 2005;51(5)e245-8. 5. Chadha V, Pandey PK, Chauhan D, Das s: “ Simultaneous intraocular and bilateral extraocular Muscle involvement in a case of disseminated Cysticercosis” Int.Ophthalmol 2006;15. 6. Gulliani BP, Dadeya S, Malik KP, Jain DC : “Bilateral Cysticercosis of optic nerve” J Neurophthalmol 2001;21 (3):217-8. 7. Bajaj MS, pushker N: “Optic nerve cysticercosis” Clinical Experimental Ophthalmol; 2002;30(2);140-3. 8. Sudan R, Muralidhar R, Sharma P, “ Optic Nerve Cysticercosis: case report and review of current management” Orbit, 2005;24(2);159-62. 9. Sekhar GC, Honavar SG, “ Myocysticercosis Experience with imaging and therapy” Ophthalmol, 1999;106(12) 2336-40. 10. Honavar SG, Sekhar CG, “ Ultrasonological Characterestics of Extraocular Cysticercosis” Orbit 1998;17(4),271-84 11. Bansal RK, Gupta A,Grewal SP, Mohan K, “ Spontaneous extrusion of cysticercosis: Report of three cases”, Indian J ophthalmol,1992;40(2):59-60.

12. Turgut AT, Turgut M, Ko?ar U.: "Hydatidosis of the orbit in Turkey: results from review of the literature 1963-2001" Int Ophthalmol. 2004;25(4):193-200. 13. Xiao A, Xueyi C: "Hydatid cysts of the orbit in Xinjiang: a review of 18 cases", Orbit. 1999;18(3):151-55. 14. Gomez Morales A, Croxatto JO, Crovetto L, Ebner R; Hydatid cysts of the orbit. A review of 35 cases, Ophthalmology. 1988;95(8):1027-32. 15. Kiratli H, Bilgiç S, Oztürkmen C, Aydin O: Intramuscular hydatid cyst of the medial rectus muscle, Am J Ophthalmol. 2003;135(1):98-9. 16. Jhn lCrompton, Prema V Iyer, David J Merry John Tomich, Llance V Perrett "Hydatid cyst: an unusual cause of diplopia" Australian and New Zealand Journal of Ophthalmology 13 (2), 195-203. 17. Rastogi A, Arora R, Chaturvedi K. Orbital hydatid cyst: an unusual presentation", Orbit. 1998;17(2):107-111. 18. Betharia SM, Pushker N, Sharma V, Avinash M, Kashyap S: "Disseminated hydatid disease involving orbit, spleen, lung and liver". Ophthalmologica. 2002;216(4): 300-4. 19. Andronikou S, Welman CJ, Kader E: "Classic and unusual appearances of hydatid disease in children" Pediatr Radiol. 2002;32(11):817-28. 20. Akhan O, Bilgiç S, Akata D, Kiratli H, Ozmen MN:" Percutaneous treatment of an orbital hydatid cyst: a new therapeutic approach" Am J Ophthalmol. 1998;125(6): 877-9. 21. Sihota R, Sharma T:Albendazole therapy for a recurrent orbital hydatid cyst Indian J Ophthalmol. 2000;48(2):142-3.

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17

Orbital Fractures

CHAPTER Alon Kahana, Mark J Lucarelli, Cat N Burkat, Richard K Dortzbach

INTRODUCTION The surgeon encounters orbital fractures in the acute, subacute and chronic settings. In each situation, the treatment goals are similar-restoration of orbital integrity and volume. However, the nuances of treatment can vary widely according to the specific setting and injury. In this chapter, we will attempt to provide an overview of how we approach orbital fractures in a variety of settings, and provide enough reference materials to satisfy the need for additional study.

as part of La Forte II or III fractures. Fractures involving the buttresses typically present with larger displacements. Alternatively, when the buttresses are intact, trapdoor-type fractures are more common.4 The anatomic landmarks of most fractures correlate with the bony anatomy (Figure 17.2). Floor

ANATOMY The adult human orbit has a volume of approximately 30 ml, of which the globe accounts for approximately 7 ml, or about 25%. 1 It traditionally is said to be formed of 7 bones: maxillary, zygomatic, frontal, lacrimal, ethmoid (lamina papyracea), palatine, and the sphenoid, although the greater and lesser wings of the sphenoid develop independently during embryogenesis [the alisphenoid (greater wing) and orbitosphenoid (lesser wing) bones].2 The optic canal is part of the lesser wing of the sphenoid. Orbital bony strength is dependent on a series of dense bony buttresses that provide structural integrity and create a protective frame around the eye. Anteriorly are the frontomaxillary and frontozygomatic buttresses. Posteriorly is the pterygomaxillary buttress (Figure 17.1).3-9 Orbital fractures can be classified as blow-out fractures (no rim involvement), and fractures that involve the rim

Figure 17.1: Orbitomaxillary buttresses. Nasomaxillary (medial), zygomaticomaxillary (lateral). Based on Gruss et al., 1986. Diagrammatic representation of the maxillary buttresses showing the two anterior buttresses (medial or nasomaxillary and lateral or zygomaticomaxillary) and the posterior buttress (pterygomaxillary). The relationship of these buttresses to the cranial base above, the mandible below and the correct occlusion is seen

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Orbital Fractures 221 fractures often extend up to the infraorbital groove and/or canal, since the thin bone of the floor abuts the stronger bone of the canal. Posteriorly, floor fractures nearly always leave the most posterior portion intact since it is part of the large and strong palatine bone. Medially, floor fractures often leave intact the inferomedial strut, a part of the frontomaxillary buttress.10, 11 Medial wall fractures often end at the frontoethmoidal suture line: the lamina papyracea is very thin, whereas the frontal bone is thick and supported by the cribriform plate. Lateral wall fractures often occur as part of a complex fracture involving the zygomatic arch and the maxillary bone. Orbital nerves and vascular bundles can often be involved in orbital fractures, and serve as important landmarks (Figure 17.2). Since the infraorbital nerve often abuts the floor fracture edge, it is commonly contused by the trauma but rarely severed. Hence, hypoesthesia in the V2 distribution of the trigeminal nerve is very common following orbital trauma, but such numbness typically resolves, at least partially, several weeks to months after injury unless surgical repair causes further damage. Just posterior to where the infraorbital groove and canal meet, a perforating branch of the infraorbital artery is often encountered, which can cause significant

bleeding if not isolated and cauterized in the course of surgical repair.12 The zygomatic bone contains foramina for both the zygomaticofacial and the zygomaticotemporal nerves, branches of the V1 division of the trigeminal nerve. Overall, the area innervated by these nerves is small, and patients often tolerate hypoesthesia associated with injury to these nerves, which may occur from surgery as well as from the initial injury. Injury to the infratrochlear, posterior ethmoidal and/or anterior ethmoidal neurovascular bundles are uncommon, but can be associated with significant bleeding. Superomedial orbital injury may also be associated with damage to the trochlea, causing torsional diplopia. Orbital fractures can often cause ocular dysmotility. There are several general etiologies in the acute setting: direct muscle damage and/or edema, nerve damage, or muscle entrapment. The restriction caused by muscle entrapment often involves the orbital fibrous connective tissue complex, of which the extraocular muscle pulleys and septa are a part.13,14 Hence, herniation and entrapment of orbital connective tissue that is associated with an extraocular muscle can often cause a clinical picture of entrapment even though the muscle itself is not incarcerated in the fracture. Such findings can often

Figure 17.2: An anterior-posterior view into the right bony orbit

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222 Surgical Atlas of Orbital Diseases be subtle. So a high index of suspicion is important (Figures 17.3A and B, and 17.4A to D). Finally, the optic nerve enters the optic canal near the apex of the orbit. Blunt trauma can result in optic nerve injury through several mechanisms: collapse of the optic canal with crush injury to the nerve, injury of perforating vessels to the optic nerve, hemorrhage with compressive optic neuropathy, severing through avulsion of the nerve, and direct injury to the globe with transmission of the impact posteriorly (Figures 17.5 and 17.6).

EXAMINATION A patient with orbital trauma requires a complete history and ophthalmic examination, including a dilated fundus examination. Any loss of consciousness should be documented, and the possibility of an intraocular or intraorbital foreign body must be addressed. The possibility of an open globe should be considered in every patient with orbital trauma, and an open globe must be ruled out prior to any orbital evaluation and management. Loss of vision, dysmotility, hyphema, and 360° subconjunctival hemorrhage are often associated with a ruptured globe. When the examination occurs in an intensive care setting, as is often the case, exact history and a full examination cannot be obtained. In such a setting, early evaluation of the pupils, prior to sedation/ analgesia-related miosis, is critical to identifying optic nerve trauma and a relative afferent pupillary defect (RAPD). Intraocular pressure should be measured with a handheld device, such as a Tonopen (Medtronic Ophthalmics, Minneapolis, MN, USA),

and high intraocular pressure treated aggressively. In an alert patient with loss of vision and elevated intraocular pressure, the possibility of a retrobulbar hemorrhage must be assessed, and when appropriate, a lateral canthotomy with cantholysis performed acutely. Particular attention must be given to patients who are on blood-thinning medications, such as warfarin, which can make an orbital hemorrhage both more likely and more severe. Canthotomy incision is a simple and fairly benign technique for rapidly reducing vision-threatening orbital pressure, and the addition of a cantholysis can further improve the decompression.15 It is not rare for patients with an orbital compartment syndrome to report improvement in vision within minutes of a canthotomy and cantholysis. Evacuation of an orbital hematoma in the acute setting has been described, including a minimally invasive technique.16 While extraocular motility cannot be evaluated in the sedated patient, radiologic suggestion of entrapment should be further investigated with forced duction testing at the bedside, which the sedation facilitates (Figures 17.7A to E). It should be

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Figures 17.3A and B: Mild muscle entrapment. Patient is a 45 years old man who suffered a left blow-out fracture and experienced diplopia in upgaze. He presented several weeks after his initial trauma with a CT scan taken shortly after the injury. Examination found mild restriction of the left eye in upgaze. The CT scan showed left inferior rectus rounding, consistent with muscle entrapment. He was only minimally symptomatic in upgaze with no diplopia in primary or downgaze, and did not require surgical repair

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Orbital Fractures 223

C

D Figures 17.4A to D: Severe entrapment with muscle pinching. Patient is a 19-year-old college student involved in a nightclub alteration, who attributed his pain to swelling and bruising. One week later, when the swelling subsided, he continued to have pain and nausea with eye movement, with significant diplopia. Examination revealed right inferior rectus restriction (A and B). CT showed pinching of an entrapped muscle in a small minimally-displaced floor fracture (C). Urgent exploratory surgery found a dusky IR. After muscle release and fracture repair, he was instructed to patch his uninjured left eye and use his right eye to read and do homework. Over the next few weeks, motility recovered to better than 80% of normal, with only minimal diplopia in extreme down and up-gaze (D)

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224 Surgical Atlas of Orbital Diseases

Figures 17.5: Optic canal injury. Patient was a 12 years old involved in an unhelmeted accident while riding an all terrain vehicle (ATV). She developed a left relative afferent pupillary defect. Maxiface CT scan revealed evidence of fractures (arrowhead) through the sphenoid sinus extending into the left optic canal, with a small air bubble located at the intracranial opening of the optic canal (arrow).

Figures 17.6: Axial section of CT scan showing small air bubble (arrow) at the intracranial opening of optic canal. These subtle fracture findings are a common occurrence in pediatric patients in whom the bones are malleable

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E Figures 17.7A to E: Forced ductions—before (A to C) and after (D and E) repair. (A) Forced ductions before repair demonstrating vertical restriction, (A to C) Forced ductions after floor fracture repair with release of entrapped inferior rectus muscle, demonstrating normal ductions (D and E)

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Orbital Fractures 225 remembered that ductions may also be limited by orbital edema, so the ductions should be compared with one another. Often, the pupils cannot be dilated because of the tenuous neurological state of these patients in the immediate postinjury period. In these cases a direct ophthalmoscope may be used to view the vitreous and disc. A vitreous hemorrhage should be noted and further investigated for a possible rhegmatogenous retinal detachment using B-scan ultrasound. Likewise, the presence of a corneal abrasion or a hyphema should be assessed and treated. The evaluation of facial fractures is often performed by a multi-disciplinary team, and it is important to communicate effectively with other members of that team. The orbital evaluation should include palpation of the rim for any step-offs, and of the periorbital region for crepitus (Figures 17.8A and B). If the patient is alert, sensation in the trigeminal distribution can be assessed. Hypoesthesia in the distribution of the V2 branch of the trigeminal suggests an orbital floor fracture, but can also be associated with nerve contusion and orbital edema. Hertel exophthalmometry is a useful indicator of the

risk of enophthalmos, and in our experience, the presence of 1.5 mm or more of enophthalmos in the acute posttrauma period suggests that further enophthalmos may develop once swelling is reduced. Additional structural and functional consequences of orbital fractures should be carefully assessed. Attention should be given to integrity and symmetry of the medial and lateral commissures. Zygomatic fractures can often cause lateral canthal dystopia (Figures 17.9A to D), whereas nasoethmoid

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Figures 17.8A and B: Lateral canthal dystopia post-ZMC fracture repair. Note the right lateral canthal dystopia. Patient had suffered a motor vehicle accident with right orbital fractures. His zygoma was reduced to achieve alignment in 2 dimensions (rather than 3 dimensions), with resultant enlargement of the orbital cavity. He presented to our clinic with enophthalmos and diplopia. Subsequent surgical repair resolved his enophthalmos and diplopia

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Figures 17.9A to D: Traumatic telecanthus (A). Patient suffered significant facial trauma following a fall, with severe comminuted nasoethmoid fractures along with maxillary and zygomatic fractures (B and C). Initial repair did not fully restore the anatomy of the medial canthus and she was referred for a consultation. Intraoperatively, scar tissue was debulked and miniplate fixation was used to anchor the medial canthal tendon. Postoperatively, she has medial scleral show, and only 1mm of telecanthus (D)

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226 Surgical Atlas of Orbital Diseases fractures can often cause telecanthus (Figures 17.10A and B). Nasoethmoid fractures are also associated with damage to the nasolacrimal duct, leading to an increased risk of posttraumatic epiphora. Often, surgical repair of orbital fractures can be delayed until a better examination can be performed and an informed consent can be obtained. In this setting, useful adjuncts in the evaluation of diplopia and ocular dysmotility are ocular alignment measurements of heterotropia, and the binocular visual field (also known as diplopia field) which uses the Goldmann perimeter to delineate the area of fusion.

IMAGING Computed tomography (CT) scan without contrast continues to be the workhorse of orbital fracture imaging. A study including axial, coronal and sagittal cuts through the orbit is prefered.9, 17 Displacement should be noted using the bone-window, whereas the presence of soft-tissue herniation, including fat and muscle herniation, should be assessed using the soft-tissue window. A careful and systematic review of the orbital bones is necessary in order to avoid missing a small fracture that may be clinically relevant. Once a fracture has been identified, the imaging study is carefully reviewed for other facial fractures, especially nasal, frontal, zygomatic arch, and mandibular fractures, as well as any fractures of the orbital buttresses. The orbital rim, inferomedial strut, and the bony platforms at the edges of fracture are assessed. The repair of pan-facial fractures in most centers is coordinated with the rest of the trauma team in order to achieve the best possible outcome for the patient. Of great import is the overall size of

the orbital fractures, as well as the extent of displacement. We typically recommend surgical repair for orbital wall fractures that total more than 50% of the size of the orbital floor, since such fractures, when not repaired, can lead to significant enophthalmos.18 (Figures 17.11A to C). Next, the soft-tissue windows should be carefully examined for herniation of fat and/or extraocular muscle. However, it is important to emphasize that muscle entrapment is a clinical diagnosis, not a radiological diagnosis. The presence of retrobulbar hemorrhage should be noted, and its size approximated. The appearance of the extraocular muscles should be carefully noted for a rounding effect (which may signify entrapment) or for enlargement that can be associated with a hematoma (Figure 17.3). If muscle entrapment is noted clinically but not supported radiologically, we recommend that surgical exploration be carefully considered.

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Figures 17.10A and B: Naso-ethmoid comminuted fracture repair with miniplates, and fixation of the medial canthal ligament to the miniplates. A nasal splint with silastic bolsters can facilitate reconstruction of the medial canthal architecture

C Figures 17.11A to C: Enophthalmos. Three examples of clinicallyapparent enophthalmos resulting from orbital trauma

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Orbital Fractures 227

IMPLANT MATERIALS The choice of material for orbital fracture repair is broad, and includes both autogenous tissues and alloplastic materials. Each has advantages and disadvantages.19 Alloplastic implants are easily available, requiring no harvesting procedure with its associated morbidity. Several, such as titanium plates and porous polyethylene, have proven over time to be strong and effective. 20-24 The disadvantage of alloplasts is that they do not completely integrate with the living orbital tissues, which can lead to early and late complications, including infection, exposure and/or extrusion. The bacterial load required to infect an alloplastic implant can be lower by a factor of 10,000 than for an autogenous graft.25 Implant exposure can particularly pose a risk for infection. In addition, because the integration of even the best alloplastic implants is incomplete, migration and exposure can occur, both in the early postoperative period and as a late complication. Finally, when a complication occurs with an alloplastic implant, management will often require removal of the implant, which can be challenging. Alloplastic materials include porous polyethylene (e.g. Medpor, Porex Surgical, Newnan, GA, USA), hydroxyapatite (e.g. Biocoral, Wilmington, DE, USA), titanium mesh (such as from KLS Martin, Tuttlinger, Germany; Stryker Craniomaxillofacial, Portage, MI, USA; Synthes Inc., West Chester, PA, USA), and nylon (such as Supramid, S. Jackson, Alexandria, VA). Titanium is a strong, inert metal. It does not integrate but can be easily fixated to the surrounding bones and can provide excellent support for orbital structures. 23 If bone resorption occurs, it may infrequently require removal. Overall, titanium mesh can be an excellent choice for orbital fracture repair and has a significant track record of safety. 24 However, in our experience, reoperations following implantation of titanium mesh are more difficult as fibrous scar tissue insinuates itself into the holes of the mesh (Figures 17.12A and B). Hence, titanium mesh is not our first choice in the repair of orbital floor fractures. In cases of severe obliteration of more than one of the orbital walls, titanium mesh may be an excellent choice, owing to its ability to be shaped

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Figures 17.12A and B: Titanium mesh with scarring. Patient is a young woman who was injured in a motor vehicle accident. She suffered severe ocular injuries that eventually resulted in an enucleation. In addition, she underwent floor fracture repair with titanium mesh. Her implant motility was severely limited and she developed significant enophthalmos with superior sulcus deformity. She was referred for an orbital consultation and underwent orbital volume augmentation. Intraoperatively, her inferior rectus and surrounding orbital tissues were found to be tightly adherent to the titanium mesh, with extensive scarring through the holes in the mesh. These were dissected off to free the orbital tissues from incarceration in the titanium mesh

and maintain the desired shape. The use of titanium mini-plates in the stabilization of orbital rim and buttress fractures is a mainstay of fracture fixation techniques. Nylon sheets come in a variety of sizes and are easy to use.26 Fixation can be achieved with a fibrin sealant (e.g. Tisseel, Baxter, Deerfield, IL, USA) or a biological glue (BioGlue, CryoLife, Kennesaw, GA, USA). However, late complications with nylon sheets are not uncommon, and in particular, the capsule that forms around the nylon sheet can spontaneously hemorrhage.27 Our preferred alloplastic implant material is porous polyethylene, which is strong, biocompatible and can integrate well.20, 28-30 Medpor Barrier implants (Porex Surgical, Newnan, GA, USA), are porous polyethylene plates that are coated with a thin, nonporous, high density polyethylene barrier that is heat-bonded to the porous material on one side. This barrier is positioned toward the orbital tissues to reduce scarring and attachment of orbital tissues to the plate.31 The barrier also has the added benefit of strengthening the sheet. Porous polyethylene implants can also be easily secured to the rim with screws if necessary, either using a channel implant or directly through the porous polyethylene. They are malleable and can be cut into various sizes and shapes with a large Metzenbaum scissors. A newer version of the porous polyethylene implant is the TITAN implant (Porex surgical), which can hold curved shapes particularly well. This implant is well

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228 Surgical Atlas of Orbital Diseases suited for repair of large floor fractures and reconstructions that involve both the floor and the medial wall (such as loss of the inferomedial strut) (Figures 17.13A to D). The titanium mesh embedded in the porous polyethylene contains holes through which screws can be placed for improved fixation if necessary. Autogenous bone grafts have a proven track record of reliability, but require a harvesting procedure unless a cadaveric bone graft is used. Given recent concerns with infectious agents and prion diseases, cadaveric bone grafts may be seen as less desirable to many patients. Fresh bone contains living osteocytes and integrates well with the surrounding bones. When cranial bone is used, early integration

is achieved and minimal resorption is observed.32-34 This is most likely the result of the neural crest origin of calvarial bone, which is shared with orbital bones. The neural crest-derived craniofacial bones form through intramembranous ossification, whereas the mesodermal bones of the ribs and pelvis form through endochondral ossification.35, 36 Calvarial bone can be harvested without the need for surgical preparation of a different body site (Figures 17.14A to D). In addition, calvarial access can be hidden behind the hairline, and when a bicoronal approach is used for orbital fracture repair, the same exposure can be used for harvesting the graft. A major disadvantage of calvarial bone grafts is that despite the overall safety of the harvesting procedure, the

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Figures 17.13A to D : Medpor TITAN in complex fractures and orbital reconstructions. A: Posterior large medial wall fracture and Medpor TITAN implant prior to placement. The implant was cut to size, incorporating a notch for the inferior oblique. It was then bent and placed into position. A=anterior. P=posterior. S=superior. B: Even a large Medpor TITAN implant can be bent and will hold its shape. This patient underwent orbital reconstruction following excision of sino-orbital squamous cell carcinoma that involved the inferomedial orbital bones. Fixation was achieved with glutaraldehyde-crosslinked albumin adhesive (BioGlue, Cryolife Inc)

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Orbital Fractures 229

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D Figure 17.14A to D: Calvarial bone grafts. After exposure of the skull, a partial thickness calvarial graft is harvested. Next, it is used to repair an orbital floor fracture

inner table can be penetrated, and bleeding and brain damage can occur.37-39 Particular care must be taken to avoid harvesting within 2 cm of midline in order to prevent injury to the sagittal sinus.40 Patients will often feel a depression at the donor site, which must be explained preoperatively. At our institution, we rarely use calvarial bone grafts, and when we do, it is in the context of extensive craniofacial reconstruction performed by a team that includes craniofacial and neurological surgeons. Iliac crest and rib bone grafts are commonly by plastic surgeons. They offer a large supply of easily accessible cortical bone. Ribs are malleable, which can be both an advantage and a disadvantage. Iliac bone is hard and can be difficult to contour but can be used successfully.41 Donor site morbidity, in the form of bleeding, pain, and gait disturbance, can be significant. Both types of bone are of mesodermal origins, and ossify through an endochondral process.

Their harvest into the neural crest-derived facial skeleton can result in delayed integration and significant resorption. We rarely use rib or iliac crest bone grafts, which are more commonly considered in facial reconstruction following craniofacial tumor resection, and is beyond the scope of this chapter. The interested reader is encouraged to refer to the excellent reviews and textbooks that focus on this subject (e.g. Holck and Ng, 200642).

GENERAL OPERATIVE CONSIDERATIONS In evaluating orbital fractures, several issues should be addressed. These include any indications for fracture repair (such as fracture size and diplopia with entrapment), the timing of fracture repair, managing patient expectations, working with multiple surgical services, risk of infection and antibiotic

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230 Surgical Atlas of Orbital Diseases coverage, orbital edema and the use of steroids, concomitant management of soft tissue injuries, the use of blood thinning products, and elective versus urgent repair. Patients should be advised to avoid blowing their nose,43 and prescribed nasal saline spray 2-4 times daily. Patients are instructed to keep their head elevated, and to use ice-cold compresses for 23 days. Any blood thinning medications, especially aspirin, clopidogrel (Plavix, Bristol-Myers Squibb) or warfarin, should be discontinued in co-ordination with the prescribing internist. Other blood thinning products include non-steroidal anti-inflammatory drugs, vitamin E, garlic, ginseng, and ginkgo biloba.44,45 With patients on warfarin, the prothrombin time should be checked in the early preoperative period. Since an orbital hemorrhage can have catastrophic effects on vision, care must be taken in operating on anticoagulated patients.

Antibiotics Orbital cellulitis is a serious but uncommon complication of orbital fractures.46 Practice patterns vary widely regarding the use of antibiotics in the context of orbital fractures. Multiple published studies have shown that postoperative antibiotics do not alter the rate of infection associated with orbital fractures, although good studies have not been performed.22,47,48 A randomized trial with 181 patients who underwent open reduction and fixation of mandibular fractures also showed no advantage to postoperative antibiotics.49 Risk factors for orbital infection in the context of orbital fractures include open fractures, sinusitis, and contaminated wounds. 46 In these situations, we always give preoperative antibiotics, since preoperative administration of antibiotics has been shown to provide improved prophylaxis.50 In addition, in cases of frank wound contamination, we carefully treat the wounds with 5% Betadine solution and irrigate the wound intraoperatively with bacitracin solution. Our antibiotics of choice are Cefazolin, Ampicillin-Sulbactam (Unasyn, Pfizer, NY, USA) or Clindamycin, with the latter two providing improved coverage of anaerobic microorganisms. The risk of antibiotic overuse cannot be overemphasized. The human body is continuously colonized by bacteria that exist in steady-state equilibrium in the context of the normal flora. Treatment with antibiotics alters the equilibrium,

which can potentially lead to the proliferation of antimicrobial-resistant pathogenic organisms. Hence, overuse of antibiotics can cause an infection with resistant bacteria. We usually limit antibiotic usage to the preoperative setting, with administration prior to surgical incision in order to achieve significant tissue concentration at the surgical site intraoperatively. Postoperative antibiotics are given only if there are active signs of infection or if systemic steroids are prescribed in the context of a higher risk of infection (as discussed below).

Steroids Glucocorticoids can be extremely useful in the perioperative management of orbital fractures.51-53 However, their use can lead to complications, especially an increased risk of infection. Hence, any steroid administration is done using a very rapid taper regimen. The most useful contexts of steroid usage are in the preoperative assessment of extraocular muscle function and in the prophylaxis of postoperative emesis (which can cause implant movement and bacterial spread). In the preoperative setting, it is sometimes difficult to distinguish between extraocular muscle contusion or edema and frank muscle entrapment. This distinction is important to make since it can strongly influence the decision to recommend surgical exploration repair. Therefore, when there is significant dysmotility associated with moderate to severe orbital edema, a small orbital fracture and no obvious entrapment radiologically, we document the dysmotility with a binocular visual field (also known as a diplopia field, performed with both eyes open on a Goldmann Perimeter), and prescribe a rapid taper of Prednisone or Methylprednisolone over the course of 5-7 days. A typical Prednisone taper for an adult would be 40, 30, 20, 10, and 5 mg over the course of 5 days. We re-evaluate motility toward the end of the treatment course (in 5-7 days) and reassess the need for surgical intervention. In numerous cases, these exams will reveal significantly improved motility and near resolution of diplopia, avoiding the need for surgical exploration of a fracture that was otherwise too small to warrant surgical repair. Another context for prescribing a steroid taper is in children, who tend to swell more and scar faster. We try to operate on children earlier (within 1 week

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Orbital Fractures 231 and in cases of severe restriction, even sooner). A-5 day steroid taper can help control posttraumatic edema and facilitate early and safe surgery. Intraoperative IV steroids can be very useful in two respects: control of nausea, and expedited resolution of postoperative edema and discomfort. In an adult, 8-10 mg of Dexamethasone are often given by the anesthesiologist toward the end of surgery. Intraoperative steroid irrigation of the orbit is performed rarely, and usually in the context of late repair of a scarred musculoseptal system. Infrequently, a postoperative steroid taper is prescribed if severe edema is anticipated (e.g. late repair of severely scarred orbit), or if edema would interfere with the postoperative evaluation and patient management (e.g. with extraocular motility evaluation). However, there are no good studies to direct perioperative steroid use, and the potential side effects must be fully considered.

Pediatric patients Special attention must be given to nondisplaced fractures and floor fractures in pediatric patients since muscle entrapment with frank muscle pinching may occur.54 Steroids will not only fail to resolve the dysmotility, but will also delay needed surgical intervention, which can lead to irreversible muscle injury. Such fractures have been termed "greenstick" fractures, and are the result of the fact that the facial bones of children have a higher cancellous composition and thinner cortices, resulting in increased bone flexibility.55-58 At times, orbital floor fractures result in minimal soft tissue injury, no orbital edema and no enophthalmos but severe dysmotility with entrapment, pain, and nausea. CT scanning may only demonstrate minimal tissue herniation and fracture displacement. This presentation has been termed "white eye syndrome."54, 59 Clinical signs of muscle pinching and entrapment include restriction in upgaze, severe pain worsened by upgaze, and oculocardiac reflex induced by upgaze, which can include nausea, vomiting, bradycardia and/or syncope.58, 60, 61 Timely management of fractures with muscle pinching is essential in order to reduce the risk of irreversible muscle damage and strabismus, both in the pediatric and adult population. In patients

with White Eye Syndrome, surgical exploration and repair must be carried out urgently. The examination of a child who is in pain and distressed following orbital trauma can be quite challenging, and the surgeon must maintain a high level of suspicion, especially when the eye and orbit appear normal but there may be a motility disturbance.

Timing of surgery The ideal timing of surgical exploration and repair depends on the clinical findings, the overall medical condition of the patient, the radiographic evidence and any concomitant injuries. Delayed repair has the advantages of reduced swelling, a more thorough preoperative evaluation, and an increased opportunity to establish a meaningful patient-doctor relationship prior to any surgery. Early repair has the advantage of reduced fibrosis and scarring. In general, we prefer to repair orbital fractures within 2 weeks of injury once the majority of post-traumatic edema has resolved.18, 60 There are several exceptions to this “2 week” guideline whereby early or late intervention would be preferred. First, if the patient's overall medical condition is unstable, then medical stabilization must take precedence. Second, if entrapment with muscle pinching is encountered (often in children as part of a White Eye Syndrome), urgent repair is advisable in order to reduce the risk of irreversible muscle damage. Third, when the optic nerve may be compromised, the status of the optic nerve should be properly investigated prior to any orbital surgery. Otherwise, surgery may further compromise the already-traumatized nerve, or the patient may perceive the surgery as the cause of any optic nerve damage. The status of the globe and the possibility of a retinal detachment must also be fully addressed prior to any orbital fracture surgery. Finally, if other surgical interventions are planned, coordinating care can be an overall advantage to the patient by minimizing multiple inductions of anesthesia. Another softer exception to the 2-week rule is the pediatric population. Children swell more, but their edema resolves faster. They heal and scar faster, and are more prone to greenstick fractures and muscle pinching. Hence, expedited repair is often recommended for children.

232 Surgical Atlas of Orbital Diseases

Decision: repair or not repair? Surgical repair of orbital fractures is a very safe procedure when done appropriately. Nevertheless, surgery can cause complication.62, 63 The decision of whether to repair an orbital fracture is complex, and must rely on the individual circumstances as well as on the experience of the surgeon. However, several guidelines have been published that can be of great help in making a recommendation regarding surgical repair. These guidelines address the risk of posttraumatic enophthalmos, 64 ocular dysmotility, diplopia, and facial deformity. Fracture size correlates well with the risk of posttraumatic enophthalmos.18, 65 Hence, radiographic assessment of fracture size can be a critical part of the decision tree. There are several methods that have been advocated in the evaluation of post-traumatic enlargement of orbital volume. When the zygoma is not involved, it is simplest to assess whether the sum of orbital floor and medial wall fractures constitutes half of the floor or more. This algorithm is simple to execute but does not take into account any lateral wall/ZMC fractures. 65 Another method requires measurement of the added volume caused by the fracture. This is determined by comparing the fractured orbit with the non-traumatized orbit.66-68 For example, using one algorithm, measurements that reveal greater than 13% orbital volume enhancement would lead to a recommendation for surgical repair.67 The weakness of this algorithm is that many patients sustain injury to both orbits, which precludes useful comparison. Zygoma fractures can seriously complicate any volumetric assessment, since floor fractures are often present but get reduced once the ZMC fracture is reduced. 65 Nevertheless, the rotation and/or displacement of a fractured zygoma can lead to severe orbital volume changes, and incomplete reduction of ZMC fractures can be a common cause for postoperative enophthalmos. ZMC reduction requires special care since without three point fixation, reduction may be incomplete although the fracture may appear well aligned along the orbital rim. Rotation and orbital volume expansion can still occur and can often be hard to detect. Hence, threepoint reduction of ZMC fractures with proper fixation should be the goal of these surgical repairs.65,69,70

Ocular dysmotility and diplopia are debilitating consequences of orbital trauma. Ocular dysmotility can occur for several reasons, and it is the task of the surgeon to distinguish between the etiologies in order to make the appropriate recommendation. Posttraumatic extraocular muscle dysfunction can occur as a result of muscle contusion, hematoma, fibrosis or avulsion, muscle entrapment with or without pinching, cranial nerve injury and muscle paresis, generalized orbital swelling, or contracture of the antagonist muscle. Muscle fibrosis or contractures are later complications that must be prevented by proper management. However, at times it can be very challenging to distinguish between ocular dysmotility with or without entrapment. Limitation in up-gaze with associated pain and nausea can be telltale signs of muscle entrapment. In addition, CT scans can be particularly helpful in this task. Rounding of an extraocular muscle is often associated with entrapment. The radiologist and surgeon must be particularly cognizant of orbital tissue herniation without frank muscle herniation: the herniated tissues may cause entrapment by virtue of their numerous fibrous attachments to a muscle. Such a fracture should be repaired. Some surgeons advocate exploration and repair of orbital floor fractures for non-resolving infraorbital hypoesthesia. However, a meta-analysis of the literature found little evidence to support such a recommendation since the reported surgical outcomes were poor. However, if infraorbital pain is worsening, exploration is probably warranted.71-73 The decision to operate is complex and must be individualized. Our preference is to operate within 2 weeks of injury except in children, in which case surgical repair would preferably take place within a week. In cases with pinched muscles, urgent repair within 1-3 days of injury would be advocated. When orbital edema makes the evaluation difficult, a steroid taper can be very helpful. When the examination is inconclusive, a re-examination is warranted and can be critical to making the proper diagnosis and surgical plan. Irrespective of the type and location of the fractures, the goals of surgery are fundamentally similar: repositioning of herniated orbital tissues,

Orbital Fractures 233 reconstructing the orbital bony support, and restoring normal orbital volume and structure.

FLOOR FRACTURES Orbital floor fractures are common, and result from blunt orbital trauma in which force is delivered to the thin bones of the orbital floor, typically along the infraorbital canal. Often, an orbital floor fracture will not involve the orbital rim, which is much thicker and stronger. In such an instance, the term "blowout fracture" is often applied. The term was first used by Smith and Regan in 1956 to describe orbital fractures caused by striking the orbital rim with a hurling ball.74-76 The orbital floor is the shortest of the walls. It consists of the roof of the maxillary sinus with a small contribution posteriorly from the palatine bone, and contains the infraorbital groove and canal. The edge of the canal forms a weak spot in the floor structure. Hence, most floor fractures extend up to the canal, but the canal is often left mostly intact. Concomitant displaced zygomatic fractures should be repaired first, since reduction and fixation of the ZMC fracture will often reduce the floor fracture as well.65 Floor fractures are often associated with medial wall fractures. When making the decision to proceed with or forgo surgery, the total wall area involved by the fracture, including the floor and medial wall, must be taken into account and addressed. The evaluation of a suspected floor fracture often requires CT scanning with coronal sections, which facilitates the assessment of bone displacement and extraocular muscle findings. It must be emphasized again that muscle entrapment is a clinical diagnosis, which should be supported by the radiologic evidence but need not be. Extraocular muscle entrapment in the context of orbital floor fractures is not uncommon.(Figure 17.4) However, the finding of muscle entrapment can be subtle, and a high level of suspicion must be maintained (Figure 17.3). Tissue herniation is very common in the context of orbital floor blow-out fractures. All orbital tissues must be retrieved and repositioned into the orbit at the time of repair; otherwise, tissue incarceration can lead to necrosis and permanent muscle dysfunction.

Our favored approach to the inferior orbit is through a transconjunctival approach.77-80 We usually avoid the lateral canthotomy and inferior cantholysis, but at times, when better exposure is required for a larger fracture, a tighter orbit, or more extensive herniation, the canthotomy and cantholysis can greatly aid in obtaining adequate exposure. Care must be taken to perform the exposure correctly to avoid postoperative complications.81 The inferior fornix and lateral canthus are infiltrated with 2-3 ml of local anesthetic containing 1% Lidocaine, 0.25% Bupivicaine, and 1:100,000 dilution of epinephrine. The patient's face and any wounds are carefully prepped with 5% Betadine solution, and sterile drapes are placed. We routinely place a lubricated plastic corneal protective shield on the globe. First, forced ductions are performed to assess for muscle entrapment (Figure 17.7). Next, the assistant retracts the lower eyelid down with a Desmarres retractor. The surgeon uses a malleable retractor to sweep the orbital fat posteriorly and drape the conjunctiva and lower lid retractors over the inferior orbital rim (Figure 17.15A). The surgeon then uses a monopolar electrocautery device with a microdissection needle (such as the Colorado needle, Colorado biomedical, Evergreen, CO, USA) to cut through conjunctiva, lid retractors and orbital rim periosteum to reveal the bony rim. The incision is made inferiorly in the fornix (at least half way between the inferior edge of the tarsus and the deepest part of the fornix) to help minimize cicatricial changes (retraction or entropion) of the eyelid margin (Figure 17.15B). We then use a Freer elevator to lift the periorbita off of the orbital floor. Our dissection is guided by the CT findings: the initial sub-periosteal dissection is performed away from the fracture and then brought to the fracture site. The fracture edges are carefully defined and visualized. A malleable retractor is used to gently retract the globe superiorly while the herniated orbital contents are lifted back into the orbit using a Freer elevator. Often, the herniated tissues need to be bluntly separated from any early scars that form at the edge of the fracture and in the maxillary sinus; this must be done slowly and carefully, taking care to avoid injury to the infraorbital neurovascular bundle and the perforating artery. The maxillary sinus is typically well visualized through the fracture (Figure 17.15C).

234 Surgical Atlas of Orbital Diseases When the sinus is full of blood, we typically evacuate the blood using suction, which also improves visibility. Once the herniated tissue is released, a Teflon sizer (DuPont, Wilmington, DE, USA) is used to assess the size of the needed implant (Figures 17.15D and E). In the absence of a sizer, aluminum foil from a suture packing, sterilized X-ray film, or any other similar strong and sterile material can be used. Adequate overlap with the fracture edges must be confirmed. Particular attention must be given to avoidance of orbital tissue incarceration between the implant and the fracture's bony ledges. The implant is soaked in an antibiotic solution (e.g. bacitracin solution), and cut to size with the

appropriate scissors (Figure 17.15F). It is then molded into the proper curvature. It is slipped carefully into the orbit to cover the fracture (Figures 17.15G to I). If a porous polyethylene implant with high density "barrier" surface is used, the barrier side is oriented toward the orbital soft tissue and the porous surface turned toward the maxillary sinus. Care must be taken to avoid an implant that is too long which might cause damage to the orbital apex. The support of the implant by the bony ledges is confirmed, and any tissue incarceration is reduced. The end point should be a stable implant that can slide 1 mm with gentle force but is otherwise immobile. When implant stability is in question, the implant should be rigidly fixated to the bony rim with a titanium microscrew.

Figure 17.15A: The lower lid is retracted and the rim palpated. A malleable retractor is used to protect the globe, expose the rim, and keep the fat pads pushed posteriorly

Figure 17.15B: The monopolar unit with a micro-dissection needle is used to incise conjunctiva, eyelid retractors and orbital rim periosteum deep in the inferior fornix

Figure 17.15C: After elevation of the periorbita with a freer elevator, the orbital floor fracture is exposed. A combination of the freer elevator and thin retractors is used to retrieve any prolapsing orbital contents from the maxillary sinus

Figure 17.15D: The position, dimensions and posterior-most aspect of the floor fracture are measured with the help of a probe

Orbital Fractures 235

Figure 17.15F: Once the correct sizer is identified, the Medpor implant is cut to size Figure 17.15E: A Teflon sizer is used to assess coverage of the fracture

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I Figures 17.15G to I: The implant is placed on top of the fracture. Care must be taken not to incarcerate any orbital tissues between the implant and the orbital floor

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MEDIAL WALL FRACTURES

Figure 17.15J: Once the implant is stable, the eyelid retractors are reapproximated using buried interrupted sutures

A screw can be placed right through the Medpor Barrier implant or through the TITAN MTB implant. Some surgeons favor surgical glue for fixating the implant temporarily. The Desmarres retractor is then removed, and the eyelid retractors sutured with buried, interrupted 7-0 polyglactin (Vicryl) suture using 3-point fixation (Figure 17.15H). The conjunctiva is left unsutured. When no bony ledge is present to support the fracture, cantilevering of the TITAN implant or a Medpor Channel plate over the rim can provide rigid fixation. Some surgeons favor titanium mesh in this situation. Other methods of fixation include the use of fibrin sealant (such as Tisseel, Baxter, Deerfield, IL, USA) and biological glue. BioGlue (CryoLife, Kennesaw, GA, USA) is a two-component glue, containing engineered bovine albumin in one tube and a glutaraldehyde solution in the other. When the two substances mix, the glutaraldehyde cross-links albumin molecules to each other and to the surrounding proteins. Glutaraldehyde cross-linking resembles a peptide bond, and hence can easily form covalent bonds among proteins in contact with the chemical. When a porous implant is used, the albumin infiltrates through the pores as it gets cross-linked, resulting in strong biocompatible chemical bonding of the implant to the surrounding tissues. The use of biological glues and sealants is particularly useful when implant stability is in question, yet the surgical exposure is inadequate for complete rigid fixation (often because the globe should only be retracted gently and carefully).

Medial wall blowout fractures are common but historically were under-diagnosed because they can be asymptomatic in the acute post-traumatic phase.4, 65 The nearly ubiquitous use of CT scanning in the United States in the evaluation of facial fractures has made the diagnosis of medial fractures much easier. 82 However, when CT is unavailable, the evaluating physician must maintain a high level of suspicion. Epistaxis can be associated with medial wall fractures because of tears in the sinus mucosa. The epistaxis is typically self-limited. Visionthreatening orbital hemorrhage can occur if the anterior or posterior ethmoidal vessels are injured. It is important to remind patients to avoid blowing their nose, since this can lead to orbital emphysema and a compartment syndrome (Figures 17.16A and B).43, 83 The thin lamina papyracea constitutes most of the medial wall of the orbit, behind the posterior lacrimal crest. The medial orbital wall is completed posteriorly by the lesser wing of the sphenoid, and anteriorly by the lacrimal bone. Both the sphenoid and the lacrimal bones are thicker. Hence, medial wall fractures occur because of the weakness of the thin lamina papyracea, and are typically localized at the boundary between the lamina papyracea and one of the thicker bones. Medial wall fractures commonly occur in the context of orbital floor blowout fractures, but can also occur in isolation.4, 26 They can also be associated with nasal fractures, which can lead to

A

B

Figures 17.16A and B: Orbital/periorbital emphysema. Monocular patient was assaulted with a blow to the left orbit. He presented to the emergency department after blowing his nose, leading to severe pain and reduced vision. In the ER, his intraocular pressure was measured at 60 mm Hg. Emergent lateral canthotomy and inferior cantholysis were performed, resulting in decompression of the orbit, restoration of vision and rapid decline in intraocular pressure to the teens. A CT scan following the cantholysis revealed air in the orbit. His lateral canthus was repaired 4 days later

Orbital Fractures 237 telecanthus and possible damage to the nasolacrimal drainage apparatus.84, 85 Described approaches to the medial wall fracture include a Lynch incision in the medial canthus, a bicoronal incision, a medial upper lid crease incision, or a transconjunctival incision. However, our preferred approach is the transcaruncular incision.8688 When the medial wall fracture is large, or associated with a floor fracture, multiple incisions may be necessary, such as a secondary lower lid fornix incision.26, 89 The transcaruncular approach requires meticulous hemostasis in order to properly expose and visualize the fracture,. Pinpoint cautery with a micro-dissection needle (e.g. Colorado needle, Colorado Biomedical, Evergreen, CO, USA) is very helpful, as is the use of thrombin solution, absorbable gelatin sponge (Gelfoam, Pfizer), FloSeal (a combination of gelatin foam and thrombin, Baxter) or 3% hydrogen peroxide solution. Care must be taken with hydrogen peroxide, since it can inhibit tissue healing, and its use has been associated with air embolization.90-92 Good lighting is also important, and a head-mounted light source is nearly always used in our cases. Prior to initiating surgical repair, forced ductions are performed to assess for muscle entrapment

Figure 17.17A: Transcaruncular exposure of the left medial wall following disinsertion of the inferior oblique muscle

(Figure 17.7). The transcaruncular approach begins with infiltration of the medial fornix with local anesthetic containing 1:100,000 dilution of epinephrine. An incision is made through the caruncle anterior to the plica semilunaris using Westcott scissors.86, 87 The incision is extended superiorly and inferiorly along the conjunctival fornices in order to create sufficient exposure and prevent uncontrolled tearing of the conjunctiva intraoperatively. A sufficient distance away from the canaliculi is maintained. Curved Stevens tenotomy scissors are then used to bluntly dissect along Horner's muscle, which inserts on the posterior lacrimal crest. Care must be taken not to iatrogenically fracture the lamina papyracea just posterior to the crest, since this will make subsequent elevation of the periorbita more challenging. Once the posterior crest is exposed, the periorbita is incised with a monopolar unit and a microdissection needle. Achieving excellent hemostasis is critical at this point. Next, a Freer elevator is used to lift the periorbita anteriorly in order to fully expose the posterior lacrimal crest. The periorbita is then lifted posteriorly to create a subperiosteal dissection plane along the lamina papyracea (Figures 17.17A and B). The anterior ethmoidal neurovascular bundle is typically found approximately 24 mm posterior to the posterior lacrimal crest, along the fronto-ethmoidal suture line. This neurovascular bundle is carefully and thoroughly cauterized with a bipolar cautery unit, and then divided with the monopolar unit. The subperiosteal dissection is then completed to fully expose the fracture.

Figure 17.17B: Inferior oblique isolation. The IO muscle may be tagged with 6-0 polyglactin sutures and the origin disinserted to provide good exposure

238 Surgical Atlas of Orbital Diseases If the fracture is very posterior, the posterior ethmoidal bundle may be encountered. In such cases, the posterior ethmoidal bundle should be carefully cauterized with the bipolar cautery to avoid intraor postoperative hemorrhaging. The posterior bundle is typically found approximately 12 mm from the anterior bundle, and on average only 6 mm from the optic canal. After adequate hemostasis and exposure are achieved, herniated orbital tissues are retrieved and repositioned into the orbit. The fracture size is measured, and implant properly sized. We have found Teflon implant sizers to be very useful at this step. The implant is then placed over the fracture, ensuring that no orbital tissues are left incarcerated. When using a Medpor Barrier plate, the barrier side should be turned toward the orbit to reduce the risk of tissue scarring to the implant. If the fracture extends inferiorly to involve the orbital floor, an inferior fornix transconjunctival incision can be made to increase exposure. At times, the inferior oblique muscle must be disinserted from its origin. At the end of the case, the oblique is then reapproximated to its origin with 6-0 polyglactin suture. The floor and medial wall fractures can be repaired with a single implant. For this, the Medpor TITAN Barrier implant is ideal, since it can be molded into the required semi-cylindrical shape and will maintain this shape. Such a large implant is better placed through the inferior fornix incision, and then the positioning adjusted through the caruncular incision. Some surgeons advocate multiple implants that can overlap, using very thin implants.26 Closing the transcaruncular incision involves placing one to three buried interrupted 6-0 fastabsorbing plain gut sutures to close the caruncular conjunctiva. Medial wall fractures are particularly challenging to repair when they are part of a comminuted nasoethmoid fracture.93 In these circumstances, posttraumatic telecanthus is common, and can be quite disfiguring (Figure 17.9). Repair of the fractured posterior lacrimal crest and stabilization of the medial canthal tendon should be attempted during primary repair. Good exposure is critical, and when the nasoethmoid fractures are part of panfacial trauma, a bicoronal approach has many merits.94 Transnasal wiring has been described for the repair of post-

traumatic telecanthus,93, 95 and may be necessary in combination with miniplate fixation when repairing severely comminuted naso-ethmoid fractures. However, whenever possible, we favor miniplate fixation of the comminuted bones and the medial canthal tendon (Figures 17.10A and B).96

LATERAL WALL AND ZYGOMATICO MAXILLARY FRACTURES Fractures of the lateral wall of the orbit are common, and typically result from direct blunt trauma to the zygoma and lateral orbital rim. The zygoma articulates with the sphenoid, maxillary, frontal and temporal bones. Therefore, fractures of the zygoma can often disrupt the architecture of the entire region, and hence are often referred to as zygomaticomalar complex fractures (ZMC fractures). Importantly, the orbital floor is always fractured in the context of a displaced zygomatic fracture, but will typically reduce once the zygoma fracture is reduced.65 Numerous surgical approaches to the ZMC fracture have been described for open reduction, including transconjunctival,78, 97-99 intraoral,100 temporal (Gillies), brow incision and bicoronal flap. In addition, some surgeons advocate closed reduction or observation for non-comminuted and uncomplicated zygoma fractures.101, 102 The approach to the ZMC fracture is greatly aided by careful evaluation of the CT scan, and different classification systems have been proposed in an effort to provide guidance to proper preoperative planning. Whichever approach is chosen, a reduction of the zygoma should attempt to replace the zygoma back into its normal anatomic location. Because the zygoma can rotate around an axis formed by the inferolateral orbital rim, reduction of just the fronto-zygomatic and zygomatico-maxillary sutures is not sufficient: a third point of alignment should be confirmed in order to ensure proper reduction and avoid late enophthalmos.65 Exploration of the lateral floor of the orbit can greatly assist in the fracture reduction, as well as help avoid orbital tissue incarceration between the zygoma and the sphenoid bones. When non-comminuted fracture displacement is noted on examination and CT scans, we favor surgical exploration and repair through a subconjunctival

Orbital Fractures 239 inferior fornix and lateral canthotomy approach.99 First, forced ductions are performed to assess for possible muscle entrapment. The lateral canthus and inferior fornix are infiltrated with local anesthetic containing 1:100,000 dilution of epinephrine, and a lubricated plastic corneal protective shield is placed on the eye. A lateral canthotomy and inferior cantholysis (and sometimes superior cantholysis, too), are performed to release the lateral canthal attachments and provide good exposure of the lateral rim. The lateral inferior fornix is then incised to reveal the inferolateral rim. The periosteum is incised along the entire exposed rim, and a freer elevator is used to lift the periosteum and expose the rim from above the fronto-zygomatic suture down to below the maxillo-zygomatic suture and medially to the infraorbital canal. When a depressed floor fracture is present, the inferior fornix incision can be carried out medially to provide full exposure of the floor. Next, the zygoma must be properly reduced and aligned along three points: the fronto-zygomatic suture, the maxillo-zygomatic suture at the inferior rim, and a third point that can be the zygomaticsphenoid suture at the lateral orbital floor, the zygomatico-maxillary buttress, and/or the zygomatic arch.65, 69, 70, 99 The first two points ensure anterior alignment, whereas a third point ensures posterior alignment. Failure to achieve posterior alignment can predispose to enophthalmos by enlarging the posterior orbital volume (Figure 17.8). A useful tool in ZMC fracture alignment has been the T-bar screw, also known as the Carroll-Girard screw (Walter Lorenz Surgical, Jacksonville, FL, USA).99, 103 This cork-screw-like instrument is screwed onto the thick bone at the malar eminence and used to manipulate the zygoma into proper position (Figure 17.18A and B). This tool is particularly useful in minimally-comminuted fractures, and allows for exceptional control of the zygoma through a smallincision approach. However, when severe comminution exists without solid bone for placement of the screw, multiple incisions would be required to achieve complete reduction and rigid fixation. Following reduction of the fracture, fixation is accomplished using titanium miniplates. Forced ductions are performed again to ensure that no entrapment was caused by the fracture reduction. The lateral floor is explored to assess the zygomatico-

A

B

Figures 17.18A and B: The Carroll-Girard (T-bar) screw is a powerful tool for 3-dimensional control and manipulation of the zygomatic or maxillary bones during orbital fracture repair. After drilling a small guidance hole, the screw is positioned and attached to the T bar (Photos courtesy of Benjamin Marcus, MD, University of Wisconsin)

sphenoid suture. Next, the periosteum is sutured and the lower lid retractors are reapproximated using buried interrupted 7-0 polyglactin suture. The lateral canthal tendon is resuspended to the periosteum at Whitnall's tubercle. If lower lid laxity is present, horizontal tightening is performed to reduce the risk of postoperative lower lid retraction. Placement of a Frost suture can be done in the presence of concomitant lower lid trauma and when the risk of cicatricial ectropion is felt to be high.

LATE AND SECONDARY FRACTURE REPAIR As far back as 1957, Smith and Regan made the point that "late cases are far more difficult to correct."74 Nevertheless, thirty years ago, many surgeons advocated observation and delay of surgery for all but the most severe orbital fractures.104, 105 However, with the advent of CT scanning, an evolution in implants and fixation devices, and greatly improved surgical techniques, primary repair has become the standard of care for symptomatic or large orbital fractures. Still, there are occasions when late repair of orbital fractures is still encountered. Some reasons include lack of access to medical care following trauma, misdiagnosis, multiple medical problems preventing timely repair, or a suboptimal repair that requires reoperation. The goal of late fracture repair is the same as for early repair, namely to restore orbital anatomy and reduce or eliminate any symptoms. Late repair is nearly always complicated by significant fibrosis and abnormal anatomy that can distort the normal anatomic landmarks. When dysmotility is a significant symptom and its repair a main goal of the operation,

240 Surgical Atlas of Orbital Diseases good motility measurements are critical, as is the assistance of an expert in adult strabismus. Dysmotility that results from entrapment can be corrected when the muscle entrapment is reduced. However, if the dysmotility is the result of irreversible muscle or nerve damage, further orbital surgery will not restore normal motility, and can cause a significant increase in orbital fibrosis in addition to the risk of an unnecessary orbital surgery. Instead, strabismus surgery would be a better option. Hence, careful diagnosis of the underlying cause of dysmotility is crucial. In our practice, we will obtain a CT scan to evaluate the bony anatomy, and occasionally a dynamic MRI scan to evaluate the cause of dysmotility. In addition, we request a consultation from an expert in strabismus, and employ a teamoriented approach to caring for these patients. A common reason for late surgical repair is severe enophthalmos (Figure 17.11). In a paper by Koo et al. (2006),64 clinically apparent enophthalmos was found when enophthalmos was measured at 3-4 mm or more. In such cases, patients must weigh their symptoms against the risks of surgical complications. It is important to emphasize that counseling and managing expectations are very important for achieving a result that is satisfactory to both the surgeon and the patient. Our approach to enophthalmos repair stresses pre-operative counseling in order to clearly define what the patient desires and what we believe can be accomplished safely. Our surgical technique emphasizes good exposure, optimized illumination, and meticulous hemostasis. We employ the same incisions that we utilize for early fracture repair, namely the inferior fornix, lateral canthotomy/ cantholysis, and transcaruncular incisions. Often, the main decision is whether to attempt reduction of a healed fracture or to focus on the enophthalmos and provide orbital volume augmentation through the use of porous polyethylene implants. Given the added risk of re-fracturing and reducing healed fractures, we usually opt for orbital volume enhancement and accept the mild deformities that may be associated with poorly positioned but healed ZMC fractures. For volume enhancement, we typically utilize porous polyethylene wedge implants that are shaped to reduce orbital volume or implants with titanium that can be fixated at the orbital rim (Figure 17.19).

Figure 17.19: Medpor wedge implant. Orbital volume enhancement will often require placement of space-occupying implants. The Medpor wedge implant, which comes in both right-sided and left-sided configurations, is a useful tool. Alternatively, multiple flat implants can be stacked to achieve the appropriate amount of volume augmentation

CONCLUSION Orbital fractures are commonly encountered by the ophathlmologist, otolaryngologist, and orbitofacial plastic surgeon. Proper treatment requires a complete ophthalmic evaluation, systematic review of the radiographic evidence, and thoughtful surgical planning with careful attention to anatomic principles. The use of smaller and hidden incisions (particularly conjunctival incisions) has made surgical treatment of orbital fractures more esthetically satisfying while reducing the risk of complications. The choice of implant materials has never been greater, providing the surgeon with options that can be tailored to the needs of the patients. While surgical repair of orbital fractures has advanced considerably over the past 50 years, there is no doubt that we will continue to see innovations and further improvements in surgical techniques, to the continued benefit of our patients.

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242 Surgical Atlas of Orbital Diseases 41. Kontio RK, Laine P, Salo A, et al. Reconstruction of internal orbital wall fracture with iliac crest free bone graft: clinical, computed tomography, and magnetic resonance imaging follow-up study. Plast Reconstr Surg 2006; 118: 1365-74. 42. Holck DE and Ng JD, Eds. Evaluation and Treatment of Orbital Fractures: A Multidisciplinary Approach. (1st ed.) 2005, Saunders. 544. 43. Reeves DL, Lucarelli MJ, and Rose JG, Jr. Severe subcutaneous emphysema following orbital blowout fracture. Ophthal Plast Reconstr Surg 2005; 21: 465-7. 44. Messina BA. Herbal supplements: Facts and myths--talking to your patients about herbal supplements. J Perianesth Nurs 2006; 21: 268-78; quiz 279-81. 45. Ang-Lee MK, Moss J, and Yuan CS. Herbal medicines and perioperative care. Jama 2001; 286: 208-16. 46. Ben Simon GJ, Bush S, Selva D, and McNab AA. Orbital cellulitis: a rare complication after orbital blowout fracture. Ophthalmology 2005; 112: 2030-4. 47. Martin B and Ghosh A. Antibiotics in orbital floor fractures. Emerg Med J 2003; 20: 66. 48. Westfall CT and Shore JW. Isolated fractures of the orbital floor: risk of infection and the role of antibiotic prophylaxis. Ophthalmic Surg 1991; 22: 409-11. 49. Miles BA, Potter JK, and Ellis E, 3rd. The efficacy of postoperative antibiotic regimens in the open treatment of mandibular fractures: a prospective randomized trial. J Oral Maxillofac Surg 2006; 64: 576-82. 50. Classen DC, Evans RS, Pestotnik SL, et al. The timing of prophylactic administration of antibiotics and the risk of surgical-wound infection. N Engl J Med 1992; 326: 281-6. 51. Flood TR, McManners J, el-Attar A, and Moos KF. Randomized prospective study of the influence of steroids on postoperative eye-opening after exploration of the orbital floor. Br J Oral Maxillofac Surg 1999; 37: 312-5. 52. Millman AL, Della Rocca RC, Spector S, et al. Steroids and orbital blowout fractures–a new systematic concept in medical management and surgical decision-making. Adv Ophthalmic Plast Reconstr Surg 1987; 6: 291-300. 53. Courtney DJ, Thomas S, and Whitfield PH. Isolated orbital blowout fractures: survey and review. Br J Oral Maxillofac Surg 2000; 38: 496-504. 54. Jordan DR, Allen LH, White J, et al. Intervention within days for some orbital floor fractures: the white-eyed blowout. Ophthal Plast Reconstr Surg 1998; 14: 379-90. 55. Koltai PJ, Amjad I, Meyer D, and Feustel PJ. Orbital fractures in children. Arch Otolaryngol Head Neck Surg 1995; 121: 1375-9. 56. Chandler DB and Rubin PA. Developments in the understanding and management of pediatric orbital fractures. Int Ophthalmol Clin 2001; 41: 87-104. 57. Hatton MP, Watkins LM, and Rubin PA. Orbital fractures in children. Ophthal Plast Reconstr Surg 2001; 17: 174-9. 58. Egbert JE, May K, Kersten RC, and Kulwin DR. Pediatric orbital floor fracture: direct extraocular muscle involvement. Ophthalmology 2000; 107: 1875-9.

59. Bansagi ZC and Meyer DR. Internal orbital fractures in the pediatric age group: characterization and management. Ophthalmology 2000; 107: 829-36. 60. Burnstine MA. Clinical recommendations for repair of isolated orbital floor fractures: an evidence-based analysis. Ophthalmology 2002; 109: 1207-10; discussion 1210-1; quiz 1212-3. 61. Grant JH, 3rd, Patrinely JR, Weiss AH, et al. Trapdoor fracture of the orbit in a pediatric population. Plast Reconstr Surg 2002; 109: 482-9; discussion 490-5. 62. Mauriello JA, Jr. Complications of orbital trauma surgery. Adv Ophthalmic Plast Reconstr Surg 1987; 7: 99-115. 63. Jordan DR, St Onge P, Anderson RL, et al. Complications associated with alloplastic implants used in orbital fracture repair. Ophthalmology 1992; 99: 1600-8. 64. Koo L, Hatton MP, and Rubin PA. When is enophthalmos "significant"? Ophthal Plast Reconstr Surg 2006; 22: 274-7. 65. Pearl RM. Treatment of enophthalmos. Clin Plast Surg 1992; 19: 99-111. 66. Cooper WC. A method for volume determination of the orbit and its contents by high resolution axial tomography and quantitative digital image analysis. Trans Am Ophthalmol Soc 1985; 83: 546-609. 67. Raskin EM, Millman AL, Lubkin V, et al. Prediction of late enophthalmos by volumetric analysis of orbital fractures. Ophthal Plast Reconstr Surg 1998; 14: 19-26. 68. Fan X, Li J, Zhu J, et al. Computer-assisted orbital volume measurement in the surgical correction of late enophthalmos caused by blowout fractures. Ophthal Plast Reconstr Surg 2003; 19: 207-11. 69. Rohner D, Tay A, Meng CS, et al. The sphenozygomatic suture as a key site for osteosynthesis of the orbitozygomatic complex in panfacial fractures: a biomechanical study in human cadavers based on clinical practice. Plast Reconstr Surg 2002; 110: 1463-71; discussion 1472-5. 70. Gruss JS, Van Wyck L, Phillips JH, and Antonyshyn O. The importance of the zygomatic arch in complex midfacial fracture repair and correction of posttraumatic orbitozygomatic deformities. Plast Reconstr Surg 1990; 85: 878-90. 71. Burnstine MA. Clinical recommendations for repair of orbital facial fractures. Curr Opin Ophthalmol 2003; 14: 236-40. 72. Boush GA and Lemke BN. Progressive infraorbital nerve hypesthesia as a primary indication for blow-out fracture repair. Ophthal Plast Reconstr Surg 1994; 10: 271-5. 73. Anderson AG, Frank TW, and Loftus JM. Fractures of the medial infraorbital rim. Arch Otolaryngol Head Neck Surg 1988; 114: 1461-3. 74. Smith B and Regan WF, Jr. Blow-out fracture of the orbit; mechanism and correction of internal orbital fracture. Am J Ophthalmol 1957; 44: 733-9. 75. Converse JM, Smith B, Obear MF, and Wood-Smith D. Orbital blowout fractures: a ten-year survey. Plast Reconstr Surg 1967; 39: 20-36.

Orbital Fractures 243 76. Converse JM and Smith B. Blowout fracture of the floor of the orbit. Trans Am Acad Ophthalmol Otolaryngol 1960; 64: 676-88. 77. Tenzel RR and Miller GR. Orbital blow-out fracture repair, conjunctival approach. Am J Ophthalmol 1971; 71: 1141-2. 78. Tessier P The conjunctival approach to the orbital floor and maxilla in congenital malformation and trauma. J Maxillofac Surg 1973; 1: 3-8. 79. Kushner GM Surgical approaches to the infraorbital rim and orbital floor: the case for the transconjunctival approach. J Oral Maxillofac Surg 2006; 64: 108-10. 80. Goldberg RA, Lessner AM, Shorr N, and Baylis HI. The transconjunctival approach to the orbital floor and orbital fat. A prospective study. Ophthal Plast Reconstr Surg 1990; 6: 241-6. 81. Westfall CT, Shore JW, Nunery WR, et al. Operative complications of the transconjunctival inferior fornix approach. Ophthalmology 1991; 98: 1525-8. 82. Grove AS, Jr., Tadmor R, New PF, and momose KJ. Orbital fracture evaluation by coronal computed tomography. Am J Ophthalmol 1978; 85: 679-85. 83. Jordan DR, White GL, Jr., Anderson RL, and Thiese SM. Orbital emphysema: a potentially blinding complication following orbital fractures. Ann Emerg Med 1988;17:853-5. 84. Gruss JS, Hurwitz JJ, Nik NA, and Kassel EE. The pattern and incidence of nasolacrimal injury in naso-orbitalethmoid fractures: the role of delayed assessment and dacryocystorhinostomy. Br J Plast Surg 1985; 38:116-21. 85. Becelli R, Renzi G, Mannino G, et al. Posttraumatic obstruction of lacrimal pathways: a retrospective analysis of 58 consecutive naso-orbitoethmoid fractures. J Craniofac Surg 2004; 15: 29-33. 86. Garcia GH, Goldberg RA, and Shorr N. The transcaruncular approach in repair of orbital fractures: a retrospective study. J Craniomaxillofac Trauma 1998; 4: 7-12. 87. Shorr N, Baylis HI, Goldberg RA, and Perry JD. Transcaruncular approach to the medial orbit and orbital apex. Ophthalmology 2000; 107: 1459-63. 88. Kim S, Helen Lew M, Chung SH, et al. Repair of medial orbital wall fracture: transcaruncular approach. Orbit 2005; 24: 1-9. 89. Kim KS, Kim ES, and Hwang JH. Combined transcutaneous transethmoidal/transorbital approach for the treatment of medial orbital blowout fractures. Plast Reconstr Surg 2006; 117: 1947-55. 90. Rees JE. Where have all the bubbles gone? An ode to Hydrogen peroxide, the champagne of all wound cleaners. Accid Emerg Nurs 2003; 11: 82-4.

91. Miranda P, Cabrera A, Esparza J, and Jerez A. An oxygen embolism after hydrogen peroxide scalp infiltration. Case illustration. J Neurosurg 2006; 104: 152. 92. Detorakis ET, Drositis I, Drakonaki EE, et al. Pneumocephalus and presumed meningitis following inconspicuous penetrating periocular trauma. Acta Ophthalmol Scand 2004; 82: 603-5. 93. Sargent LA and Rogers GF. Nasoethmoid orbital fractures: diagnosis and management. J Craniomaxillofac Trauma 1999; 5: 19-27. 94. Shaw RC and Parsons RW. Exposure through a coronal incision for initial treatment of facial fractures. Plast Reconstr Surg 1975; 56: 254-9. 95. Leipziger LS and Manson PN. Nasoethmoid orbital fractures. Current concepts and management principles. Clin Plast Surg 1992; 19: 167-93. 96. Shore JW, Rubin PA, and Bilyk JR. Repair of telecanthus by anterior fixation of cantilevered miniplates. Ophthalmology 1992; 99: 1133-8. 97. Converse JM, Firmin F, Wood-Smith D, and Friedland JA. The conjunctival approach in orbital fractures. Plast Reconstr Surg 1973; 52: 656-7. 98. Nunery WR. Lateral canthal approach to repair of trimalar fractures of the zygoma. Ophthal Plast Reconstr Surg 1985; 1: 175-83. 99. Chang EL, Hatton MP, Bernardino CR, and Rubin PA. Simplified repair of zygomatic fractures through a transconjunctival approach. Ophthalmology 2005; 112: 1302-9. 100. Courtney DJ. Upper buccal sulcus approach to management of fractures of the zygomatic complex: a retrospective study of 50 cases. Br J Oral Maxillofac Surg 1999; 37: 464-6. 101. Zingg M, Laedrach K, Chen J, et al. Classification and treatment of zygomatic fractures: a review of 1,025 cases. J Oral Maxillofac Surg 1992; 50: 778-90. 102. Honig JF and Merten HA. Classification system and treatment of zygomatic arch fractures in the clinical setting. J Craniofac Surg 2004; 15: 986-9. 103. Kreutziger KL. Zygomatic fractures: reduction with the T-bar screw. South Med J 1992; 85: 1193-202. 104. Emery JM, Noorden GK, and Sclernitzauer DA. Orbital floor fractures: long-term follow-up of cases with and without surgical repair. Trans Am Acad Ophthalmol Otolaryngol 1971; 75: 802-12. 105. Putterman AM, Stevens T, and Urist MJ. Nonsurgical management of blow-out fractures of the orbital floor. Am J Ophthalmol 1974; 77: 232-9.

244 Surgical Atlas of Orbital Diseases

18

CHAPTER

Secondary and Metastatic Orbital Tumors Kasturi Bhattacharjee, Harsha Bhattacharjee, Ganesh Kuri, Shyamanga Borooah

Secondary orbital tumors are due to the extension of a primary tumor into the orbit. The orbit may be affected secondarily by tumors arising in the adjacent structures. Such tumors commonly arise from the globe, lids, conjunctiva, nasopharynx, paranasal sinus, and lacrimal sac. Intracranial tumors can also have intraorbital extension. Several factors contribute to the extension of tumors into the orbit. These include the site of the primary tumor, aggressiveness of the tumor and adequacy of initial treatment.

Orbital Extension of Intraocular Tumors This is more commonly seen in less developed countries due to a delay in presentation for treatment of a primary tumor. In more advanced countries systems of ocular screening have reduced both the morbidity and mortality from these neoplasms. Though most intraocular tumors can invade the orbit if not managed aggressively and adequately, however higher frequency is encountered amongst retinoblastoma, medulloepithelioma and uveal melanoma.1 Retinoblastoma and medulloepithelioma are usually found in pediatric population whilst melanomas are more common amongst adults.

Retinoblastomas generally remain within the globe for a considerable duration of time. However, once they penetrate Bruch's membrane they become highly aggressive. With time this leads onto extraocular spread. This may remain localized to the soft tissues surrounding the eye or the orbit (Figure 18.1) or may extend via the optic nerve into the brain (Figure 18.2) and meninges with subsequent seeding of the spinal fluid. Distant metastasis may occur through hematogenous spread involving the bones, bone marrow, liver, pancreas, kidney, spleen, lungs and gonads. Extraocular extension of retinoblastoma into the orbit most commonly occurs through the periemissarial blood vessels. Extension also occurs directly into the choroid and through erosion of the globe. Once the tumor enters the orbit, it enters a state of neoplastic proliferation and rapidly develops into an orbital mass. Spread of retinoblastoma into

Orbital Extension of Retinoblastoma Retinoblastoma (RB) is the most common childhood intraocular tumor. The incidence is approximately 1 in 15,000 to 1 in 20,000 live births. Diagnosis and treatment can salvage life. However, early diagnosis can salvage not only the eye, but also sight. In developed countries the mortality rate from extraocular retinoblastoma is as low as 5 %, but this increases to over 90% in underdeveloped countries.2

Figure 18.1: Bilateral retinoblastoma with orbital extension

Secondary and Metastatic Orbital Tumors 245 the central nervous system usually occurs by direct invasion of the optic nerve and the subarachnoid space by the tumor cells. Very rarely retinoblastoma of peripapillary choroid can egress into the central nervous system via the posterior ciliary vessels. Orbital invasion of retinoblastoma carries a poor prognosis and is a predictive factor of metastasis (Figures 18.3 and 18.4). Kapelman JE et al3 produced a multivariate analysis suggesting that orbital invasion and optic nerve invasion were the most highly predictive risk factors of death from retinoblastoma.

The main goal of treatment for extraocular retinoblastoma is to extend survival and the disease free interval. Rootman J et al4 reported that only 9.4 % of patients with orbital extension lived more than 2 years after diagnosis. It was noted that orbital extension of retinoblastoma was frequently associated with distant metastasis. Zyguiska Manchowa H et al 5 had reported 14 cases of extraocular retinoblastoma or with recurrence of which 78.6% had died. Though there is no proven effective therapy for extraocular retinoblastoma, it should be managed with aggressive chemotherapy and radiotherapy to the orbit. When there is meningeal involvement craniospinal irradiation and/or intrathecal chemotherapy is indicated. However, often palliative treatment is required if therapy is ineffective. Highly individualized aggressive therapy enhances longer survival in patients with overt extraocular retinoblastoma (Figure 18.5). Chantada G had reported use of neoadjuvant combination chemotherapy followed by surgery in the form of enucleation and/or resection of residual orbital tumor mass and adjuvant chemotherapy and radiotherapy.6 Chemotherapy includes Vincristine, Etoposide, Carboplatin, Cyclophosphamide, Doxorubicin, Idorubicin, and Cisplatin.7 They reported that this treatment regimen was highly efficacious for patients with orbital extension of retinoblastoma.

Figure 18.2: CT brain of the child in figure 18.1 revealed a pinealoma giving a trilateral presentation of RB .Classic of hereditary type

Figure 18.3: Bilateral advanced retinoblastoma with extraocular extension involving the orbit and adnexae on the left side

Figure 18.4: Bilateral retinoblastoma. The child had undergone six cycles of chemotherapy. The left eye was salvaged , however the right eye had undergone enucleation

246 Surgical Atlas of Orbital Diseases retinoblastoma cells. Medulloepithelioma forms a stroma composed of loose, delicate fibrils with abundant ground substance resembling embryonic mesenchyme or myxoid tissue.

Figure 18.5: Fundus photograph illustrating regression of retinoblastoma following SALT

Keralli H et al 8 reported that exenteration followed by chemotherapy and radiotherapy does not prolong the survival of patients with massive orbital involvement of intraocular retinoblastoma.

Orbital Extension of Medulloepithelioma Intraocular medulloepithelioma of the ciliary body is a rare tumor occurring during the first decade of life. This embryonal tumor usually arises from the neuroepithelium of the ciliary body.9 Rarely, it may arise from iris, retina and optic nerve.10-12 Though the tumor tends to locally invade the surrounding ocular structures, it can also extend into the orbit and rarely metastasize. It can be classified as teratoid or nonteratoid medulloepithelioma. Medulloepithelioma that contains heterogenous tissues like skeletal muscle, cartilage and hair are classified as teratoid.13,14 Histologically, medulloepithelioma consists of two cellular components: epithelial cords and a fibrillar matrix. Epithelial cells are arranged in convoluted patterns or in a circular pattern around a lumen or more commonly as elongated, interlacing cords. Both pigmented and non-pigmented cells may be present. More malignant tumors contain some very poorly differentiated cells which may resemble

Clinically it presents with loss of vision, pain, photophobia, ciliary body or anterior chamber mass,leucocoria and as proptosis in advanced cases (Figures 18.6 to18.9). Presence of a fleshy grey, pink, yellow or brown color mass with cystic areas in the ciliary body region is very characteristic. Very often lens coloboma may be the only presenting sign occurring due to absent zonules.The tumor may present either as a solid or polycystic mass or as sheet behind the lens, resembling a cyclitic membrane.The cysts of the tumor may be free floating cysts or may settle in anterior chamber or vitreous.The tumor may contain heterogenous tisues like cartilage which presents like chalk particles as grey-white opacities. Complications are rubeosis, glaucoma (Buphthalmos), cataract and retinal detachment. Malignant changes of medulloepithelioma are rare. These changes are characterized by increased pleomorphic and mitotic activity with areas of poorly differentiated neuroblastic cells or sarcomatous changes with invasion of surrounding ocular structures.15 Charif CM et al. reported a case of a 4 years old child with malignant medulloepithelioma involving the sclera with extension into the orbital fat. 16 Though tumors predominantly occur in children, they can rarely present in adults. J Michael Jumper had reported a case of a 45 years old man with invasive medulloepithelioma.17 Diagnosis is usually established by slit lamp examination along with radiological investigations like ultrasound biomicroscopy, CT scan and MRI. Julian G Feijoo had reported characteristic ultrasonographic features of medulloepithelioma which included echogenic heterogenicity of the tumor with presence of multiple cysts with stalk-like prolongation of the surface.18 However diagnosis is confirmed by histopathological examination and by immunohistochemistry. Treatment of choice is wide local excision. In malignant tumors enucleation is preferred. However, with extensive extraocular spread exenteration is performed followed by chemotherapy.Role of radiotherapy is palliative only.

Secondary and Metastatic Orbital Tumors 247

Figure 18.6

Figure 18.7

Figure 18.8

Figure 18.9

Figures 18.6 to 18.9: Ciliary body medulloepithelioma with orbital extension. 5 years old boy presenting with buphthalmos and underwent trabeculectomy initially elsewhere. 3 months following this presented to us with proptosis and orbital mass. On biopsy it was diagnosed as medulloepithelioma which was confirmed by immunohistochemistry. The child underwent six cycles of chemotherapy and radiotherapy followed by total exenteration

Orbital Extension of Uveal Melanoma Orbital involvement of uveal melanomas occur after extrascleral extension of massive intraocular lesions. Although extraocular spread may present with its own set of clinical problems, ultimately it is the distant metastases which ultimately decide the long-term prognosis of the patient. Uveal melanomas are a diverse group of pigmented tumors originating in the iris, ciliary body

and most commonly the choroid. They are the most common primary intraocular tumors with an annual incidence that varies worldwide. Melanomas tend to be more common in those of caucasian descent and tend to have increased incidence with decreasing latitude suggesting that increased sun exposure may be a factor in development. They predominate in the older age group peaking in the sixth decade. 19 Predisposing factors include ocular melanosis and melanocytoma.

248 Surgical Atlas of Orbital Diseases The tumors can be subclassified by histological cell type using the modified version of the prognostic cell type introduced by Callendar. Spindle A, spindle B, epithelioid and mixed types can be seen (Figures 18.10A to G) with epithelioid cells having the worst

prognosis.20 Other prognostic factors include size of the primary and age of the patient at presentation.21 Recently, genetic mutations have also been noted including monosomy 3 and duplication of chromosome 8q confer worse prognosis.

A

B

C

D

E

F

Secondary and Metastatic Orbital Tumors 249

G Figures 18.10A to G: Case of oculodermal melanocytosis with choroidal melanoma. Patient had undergone enucleation and intraoperatively pigmentation was found in the orbital fat which was later confirmed by HPE to be melanocyte with mitotic epitheloid and spindle cells

Extrascleral extension has also been found to portend a worse prognosis.21 This is linked to the fact that there is increased risk of distant metastasis.22 Orbital extension of an intraocular primary occurs via scleral emissary channels, vortex vein and posterior ciliary artery (Figures 18.11A to C) . Rare cases of extension through the optic nerve have also been reported.23 Shammas et al found that upto 0.4% of uveal melanomas showed orbital extension however, it is thought that microscopic extension has already occurred in between 10-40% of cases.24,25 Clinically, uveal melanomas may be difficult to diagnose. Patients will rarely complain of any visual disturbance unless central vision is affected. Occasionally, pain may be noted due to impingement on the posterior ciliary nerves. Nevertheless, the vast majority remain asymptomatic. The advent of indirect ophthalmoscopy has greatly assisted in peripheral viewing and perception of depth. However, simple examination may still provide some difficulty in differentiating these lesions from eccentric disciform lesions, naevi or other choroidal lesions such as hemangiomas. This is where ultrasound B-scan may be of some assistance in determining the size and depth of the lesion. Although CT may have little place in investigation, contrast enhanced MRI can assist.26 It is especially useful in determining early extra scleral spread as well as assisting in finding the extent of the tumor to assist management.

It is thought that by the time of presentation 40-45% of tumors will have metastasized. 27 Epithelioid and mixed cell types and larger tumors are more likely to involve the orbit.24 It is this distant spread which will ultimately decide the long-term survival of the patient. Consequently, signs and symptoms of spread should be sought and liver function tests and chest-radiograph can be performed at diagnosis to assist early screening. There is still little effective treatment for metastatic disease. As a result, it has been shown that aggressive surgical treatment of these tumors has had little effect on

A

250 Surgical Atlas of Orbital Diseases

B

C

Figures 18.11A to C: Case of intraocular melanoma with extension to sclera and orbit. Patient had undergone exenteration and socket was reconstructed with temporalis muscle transfer covered with split thickness skin graft

survival and consequently management has tended to be more conservative. Treatment is partly tailored to the size and type of extrascleral extension. Plaque radiotherapy has been shown to be effective against flat or small nodular extensions, whereas with larger nodular extension and recurrence external beam radiotherapy followed by exenteration can be used. Vortex vein involvement requires resection of the vortex vein followed by further enucleating or radiotherapy. Recently, brachytherapy has also been proposed for as a substitute for radiotherapy. Early findings suggest that the technique requires less time for completion of treatment whilst delivering a higher dose of radiation to the tumor. Despite improving diagnosis and management of melanoma, survival has improved little. Thus the aim is still to be able to diagnose these tumors prior to extrascleral extension.28

Orbital Extension of Lacrimal Sac Tumors Occurance of lacrimal sac tumor is very rare. In majority of patients they are epithelial in origin These epithelial tumors include squamous and transitional cell papillomas, oncocytic adenomas and carcinomas like transitional cell carcinoma, squamous cell carcinoma, adenocarcinoma, adenoid cystic carcinoma, mucoepidermoid carcinoma and poorly differentiated carcinoma. 29,30 Amongst the nonepithelial tumor the most common is fibrous

histiocytoma followed by a variety of other tumors including lymphoid tumor, malignant melanoma, hemangiopericytoma, neurofibroma, granulocytic sarcoma and lipoma. 30 It has been found that approximately 55% of lacrimal sac tumors undergo malignant transformation.30 Though these tumors are locally invasive, however at times it can invade the orbit and can also metastasize becoming life threatening. The age of presentation of lacrimal sac tumors depend on the histologic pattern of the neoplasm. Benign tumors like papillomas are found in younger age group. However, the malignant tumors are seen within 41-75 years of age.31 Ni C and co-worker had reported that genetic and environmental factors may play a role in clinical presentation of lacrimal sac tumor.32 The signs and symptoms are usually insidious with epiphora being the most common symptom and lacrimal sac mass as the most common presenting sign. Histopathologically, lacrimal sac tumors are classified as epithelial tumors and non-epithelial tumors. These tumors are either benign or malignant. The malignant tumors are found to arise either from pre-existing tumors or arise de novo. The papillomas are the most common lacrimal sac tumors .According to their growth the papillomas

Secondary and Metastatic Orbital Tumors 251 are subdivided as exophytic, inverted and mixed. However according to the histologic patterns, papillomas are classified as squamous cell papillomas characterized by acanthotic, stratified squamous epithelium transitional cell papilloma with stratified columnar epithelium and mixed papilloma with a mixture of squamous cell and transitional cell. Malignant transformation in the form of carcinomas like squamous cell, transitional cell or adeno carcinoma seem to originate from the epithelial lining of the lacrimal sac. Other epithelial tumors are oncocytic adenomas and mucoepidermoid carcinoma. Oncocytic tumors are composed of special types of epithelial cell called oncocytes due to their abundant eosinophilic cytoplasm. These tumors rarely undergo metastasis. Mucoepidermoid carcinoma is a rare epithelial tumor of the lacrimal sac. These tumors are composed of a mixture of different cells like mucus secreting cells, epidermoid cells and basal cells. Seven types of non-epithelial tumors of lacrimal sac are reported. The common amongst them are fibrous histiocytoma, lymphoma, melanoma and hemangioopericytoma. 30 In general, lacrimal sac tumors are rarely life threatening. Distant metastasis can be fatal. A thorough history directed to pertinent clinical features is very important. A comprehensive ocular and systemic examination is necessary to assess the firmness and location of the mass in relation to the medial canthal tendon. Careful examination to rule out any punctal discharge and regional lymphadenopathy and a complete nasal and sinus evaluation should be done. Lacrimal function tests the rapidity of flow from the lacrimal sac. Benign tumors demonstrate outflow stenosis and carcinomas show complete obstruction. Dacryocystogram of a lacrimal sac tumor shows a discrete mass with a filling defect or a distended sac shadow with uneven or mottled density of the contrast media and some patency. Computed tomography of orbit and sinuses is essential to outline the tumor and assess erosion or invasion into nearby structures. Located within the lacrimal sac fossa, anterior to the posterior lacrimal

crest along the medial orbital wall, the nasolacrimal sac is usually not prominently identified on radiographic imaging (CT/MRI). Expansion of lacrimal sac fossa, destruction of bone, a mass extending beyond the lacrimal sac suggest a lacrimal sac tumor. A chest X-ray, hematologic tests including full blood count with differential, ESR, renal and hepatic function tests, antineutrophil cytoplasmic antibody and leutic serology may be considered. Swabbing for microbiologic culture is essential in an ulcerated lesion. Biopsy for histopathological diagnosis is mandatory for treatment. Traditionally, suspected lacrimal sac tumor is managed by dacryocystectomy with tissue biopsy and frozen section analysis (Figures 18.12A and B). This was followed by further treatment as required including radical resection, radiotherapy, or chemotherapy. Nowadays, incisional biopsy is done in large ulcerative tumors to plan subsequent therapy. Following definitive histological examination, if no evidence of tumor is found, a DCR is performed. If the only suggestion of a lacrimal sac tumor is a filling defect on DCG, a DCR and biopsy should be performed. If the biopsy reveals a locally invasive tumor, nasal endoscopic monitoring is advised. Aggressive tumors are treated with radical surgery or radiotherapy.

Figures 18.12: A case of lacrimal sac tumor which was later diagnosed as squamous cell carcinoma of lacrimal sac

252 Surgical Atlas of Orbital Diseases Management varies as to the tumor origin. For lacrimal sac neoplasms, surgery with adjuvant radiotherapy is advised. In lymphomas, chemotherapy is the definitive treatment. For localized epithelial and mesenchymal tumors, wide surgical excision is performed. Postoperative radiotherapy is recommended for malignant epithelial tumors. Further surgery and adjuvant radiotherapy is performed for recurrent lesions. For malignant melanomas extensive surgical resection, radiotherapy or chemotherapy may delay recurrence but survival remains poor. A partially or totally irreducible lacrimal sac mucocele indicates an underlying neoplasm. This is managed by DCR followed by definitive therapy determined by histology with biopsy if bone erosion is absent on CT. If radiology suggests malignancy, treatment is as outlined above.

Orbital Extension of Eyelid Tumors Malignant eyelid tumors have the propensity of aggressive growth and for metastasis necessitating aggressive management.Though there are different types of eyelid tumors, however epithelial tumors are most common malignant eyelid tumors. These are usually slowly enlarging and destructive tumors which can invade the adnexal tissues and the orbit. The clinical features of malignant eyelid tumors are variable.Very often they present as chronic blepheritis or meibomianitis or as a lid mass.There may be associated loss of eyelashes or pitting or notching of lid lamella.Presence of superficial telengiectatic blood vessels on the surface of the mass is characteristic of malignant eyelid tumors.The important and common epithelial malignancies of the eyelid are basal cell carcinoma, squamous cell carcinoma and sebaceous gland carcinoma.

Basal Cell Carcinoma (BCC) Basal cell carcinoma is the most common malignant eye lid tumor in Caucasians (80 to 90%), while it shares equal incidence with sebaceous gland and squamous cell carcinoma in Japan and Asia (20 to 40%).33,34 Its incidence is increasing worldwide. 35 Incidence of BCC in caucasians is generally higher in males than females. It occurs rarely before 20 years of age, but age specific incidence increases thereafter peaking between 40 to 80 years with an average age of onset of 48 years.

Independent risk factors for BCC include red hair and light skin color.36 Dynamic tanning and burning reaction to the skin is also a clear predisposing factor.37 Other risk factors include ionizing radiation, arsenic exposure, smoking history and patients with AIDS.38,39 Rare syndromes associated with BCC include albinism, xeroderma pigmentosum, Gorlin's syndrome, milia, spina bifida and syndactyly. The most frequent site of involvement is the lower lid (50 to 60%) and medial canthus (25 to 30%). Upper lid (15%) and lateral canthus (5%) are less frequently involved.39 In the early stages BCC appears as a pearly, raised area, through which dilated vessels are seen. They ultimately undergo ulceration and present as destructive lesions which distort the eyelid anatomy. It has an indolent and painless course. Histologically, BCC can be classified as nodulo-ulcerative (rodent ulcer), pigmented, morphea or sclerosing, superficial, keloidal and fibroepithelial variety (pinkuus tumor). It may cause loss of eyelash and mimic chronic infection of eyelid margin. The nodular variety is the most common and it appears as a raised pearly nodule with telangectasia and central ulceration. Histology shows nests of basal cells that originate from the basal cell layer of the epithelium with peripheral palisading. Morpheoform lesions are firm and slightly elevated with ill-defined margins. Histology shows thin cords which radiate peripherally. Morpheoform is more aggressive than nodular tumor. BCC is a slow growing tumor and it is locally invasive. It rarely spreads to distal parts of the body.40 Reported incidence of metastasis is 0.0028 to 0.55%.3 Choroidal invasion occurs at advanced stages with median survival of 3 years at presentation. Orbital extension is usually found with inner canthal lesions (Figure 18.13). This is due to delayed presentation or treatment and multiple recurrence following incomplete excision. Surgical excision is the most common mode of treatment, followed by curettage and cautery, cryosurgery and radiotherapy.41 Mohs micrographic surgery as practised in many centers allow 100% freeing of excision margin. Micrographic surgery allows 99% success rate with tumor removal by smaller margin.42 Cryosurgery is suitable for superficial lid tumors and gives 92% five years cure rate.43 Curettage is usually combined with cautery and usually requires

Secondary and Metastatic Orbital Tumors 253 the characteristic intraepithelial or pagetoid spread of the tumor which involves the basal layers of skin and mucous membrane in a radial fashion. It can also present as thickening of the lid on the tarsoconjunctival border with areas of conjunctival injection.

Figure 18.13: Basal cell carcinoma of eyelids with orbital extension

multiple applications. A course of radiotherapy is advocated for large tumors in older patients. Photodynamic therapy and carbon dioxide lasers have emerged recently. Percutaneous delta aminolaevulinic acid is used with exposure to light in the 620-670 nm range. The success rate of photodynamic therapy in superficial BCC is 92 to 100%.44 Other modalities of treatment include intra-lesional and perilesional interferon α2 band 5-flurouracil.45,46 Management of basal cell carcinoma with orbital invasion should be very aggressive. Radical surgery includes wide excision under frozen section or Mohs micrographic surgery technique. It may require radical surgical procedures such as orbital exenteration, bony removal and even craniotomy.

Sebaceous Carcinoma of the Eyelid Sebaceous gland carcinoma has an incidence of 1-5% of all eyelid malignancies.2 It has a much higher prevalence amongst the Asians and especially those of Indian origin. It occurs with increasing frequency in advancing age. The upper lid is most commonly involved in approximately 2/3rd of cases, followed by lower lid involvement and rarely the caruncle. Sebaceous gland carcinoma of eyelids is aggressive with a tendency for widespread metastasis. Clinical diagnosis at an early stage has a wide differential. It may masquerade as chronic blepharitis, blepharoconjunctivitis, chalazion, keratoconjunctivitis or basal cell carcinoma. 47 The blepharoconjunctivitis associated with sebaceous gland carcinoma is due to

Histopathologic features of sebaceous gland carcinoma include the presence of cells of sebaceous origin confirmed by lipid stains (oil red O) on fresh tissue specimen. The tissue shows varying degrees of sebaceous differentiation and infiltration with loss of normal architecture of the gland which is replaced by pleomorphic cells with prominent vacuolated cytoplasm with high mitotic activity. The degree of differentiation starts from the periphery and progresses towards the center. Differentiated cells are vacuolated or foamy with slight basophilic cytoplasm. However, less differentiated tumor cells are more deeply basophilic and are anaplastic. Minimally invasive tumors are composed of lobules of varying sizes with minimal tumor extension. Invasive tumors are composed of diffuse cords of cells with minimum lobule formation that extends to the stroma. Rao and coworkers reported a histologic variant of sebaceous gland carcinoma called comedocarcinoma in which the tumor lobules undergo central areas of necrosis.48 Orbital extension of sebaceous gland carcinoma has an incidence varying from 6 to 35% and is associated with a 70 % mortality rate. There is 70 % involvement of preauricular, cervical and submaxillary lymph nodes. Lymphatic spread of the tumor may occur to lung, liver, skull and brain. The overall 5 year mortality is approximately 15%.2 The management of sebaceous gland carcinoma is surgical removal of the tumor under frozen section. Rarely, surgical extirpation enhances the long-term prognosis of the tumor (Figures 18.14A to C and 18.15A to E). Patients with widespread lid involvement or orbital extension require exenteration. These patients require very close follow up with map biopsies because of its multicentric presentation and pagetoid spread. Involvement of the adjacent lymphatic gland requires radical resection. Role of radiotherapy in management of sebaceous gland carcinoma is very limited. Though the tumor is radiosensitive and

254 Surgical Atlas of Orbital Diseases

Figure 18.15A: A case of Sebaceous gland carcinoma presenting with thickening of lower lid on the right side for 6 months

Figure 18.14A: A case of sebaceous gland carcinoma

Figure 18.14B: Lid Reconstruction with Cutler Beard surgery

Figure 18.15B: Lid reconstruction with Hughes surgery

Figure 18.14C: 2 months postoperative

Figure 18.15C: 2 months postoperative

Secondary and Metastatic Orbital Tumors 255

Figure 18.15D: 6 months later there was a mass in the orbit medially and HPE revealed a sebaceous gland carcinoma. This must have occurred due to incomplete removal of the primary tumor

papilloma virus55 exposure to ultraviolet radiation,56 immunodeficiency due to immunosuppressives following organ transplantation57 and exposure to arsenic.50 People with xeroderma pigmentosum, a condition characterized by defective DNA repair following UV radiation damage to DNA and oculocutaneous albinism are also predisposed.58 Squamous cell carcinoma of the eyelid (Figure 18.16) can arise de novo in relatively normal skin or from actinic keratosis. 59,60 The mean age at presentation is around 60 years and is found to be higher amongst the males.61 It shows a tendency towards involving the lower lid and lid margin.50 Local spread of the tumor is more aggressive than basal cell carcinoma and it may also metastasize to regional lymph nodes (Figures 18.17A and B).50 The lesions present as firm hyperkeratotic papules or plaques with erythema of the surrounding skin and induration; ulceration may be present. The tumor may spread perineurally and this is associated with a higher incidence of recurrence and metastasis.50,62 Orbital involvement from squamous cell carcinoma may occur due to neglected, longstanding tumors 63,64 and occurs in 2.5 to 5.9% of cases.60,64 Presentation may be with pain or restriction of globe movement. Poorly differentiated carcinomas, those arising from scars, tumor size > 2 cm and regional node involvement are associated with increased risk for recurrence or death.65 Metastasis occurs in 1-21% of eyelid SCC.66

Figure 18.15E: Secondary reconstruction was done with removal of the orbital mass along with excision of the medial part of the eyelid and medial canthus with reconstruction with forehead flap

responds to radiotherapy initially the recurrence rate is high. Thus radiotherapy is indicated in patients in whom surgery is not possible.

Squamous Cell Carcinoma of the Eyelid Squamous cell carcinoma is the second most common cancer among whites.49,50 The relative incidence of BCC to SCC varies from 13:1 to 40:1.51 Risk factors for developing squamous cell carcinoma include increasing age, 51 fair skin, 49,50 history of sunlight exposure during childhood with a tendency to sunburns,52 PUV-A therapy,53,54 human

Figure 18.16: Squamous cell carcinoma of the eyelid

256 Surgical Atlas of Orbital Diseases

A

B Figures 18.17A and B: Extensive squamous cell carcinoma of the eyelid with extension into the conjunctiva and the orbit

Biopsy is required for confirmation of diagnosis. Usual treatment consists of the excision of the tumor with monitoring of the margins by Moh's micrography or frozen section. Local control with Moh s' surgery is 96.9% at 5 years.67 Radiotherapy may be preferred for patients who are unable to tolerate surgery or who have inoperable tumors. The five year tumor control rate with radiotherapy was 93.3%. Irradiation was equally successful in primary cases and in those recurrent following surgery.68 Cryotherapy may be used for tumors of large size to reduce their vascularity and size so that surgical excision and repair can be carried out with better surgical and functional results. It is also useful as a primary mode of therapy for small tumors with a wide base or those situated at the lid margin. 69 Exenteration is required for tumors extending to the orbit.70 Other eyelid tumors that can extend into the orbit are malignant melanoma of eyelid and Merkels cell tumor.

survival experience. 71 Based on the clinical and histological features the cutaneous melanomas are classified as superficial spreading, acral, nodular and lentigo melanoma. Of all the melanomas involving the eye and the adnexa, the melanomas involving the eyelid margins and conjunctiva have worse prognosis.72 Orbital extension of cutaneous or eyelid melanomas constitute less than 1% of secondary orbital tumors. Treatment is tailored to the size and extension of the tumor. The management of malignant melanoma of the eyelid is surgical removal

Malignant Melanoma of Eyelid Malignant melanoma of the eyelid is very rare. It may either arise denovo or from preexisting precursor lesions like lentigo maligna or Hutchinson's melanotic freckle, dysplastic naevus and giant naevus. In the last 10 years we had come across only 4 cases of melanoma of eyelid and only one case had orbital extension (Figure 18.18). The relative risk of malignant melanomas is extremely low for AfroCarrabian. Intra-orbital melanomas have the worst

Figure 18.18: Eyelid melanoma with orbital extension

Secondary and Metastatic Orbital Tumors 257 of the tumor under frozen section control. Rarely, surgical extirpation enhance the long-term prognosis of the tumor. Patients with widespread lid involvement or orbital extension requires exenteration. Our case of malignant melanoma of the eyelid with orbital extension was managed by total exenteration and socket reconstruction with temporalis muscle transfer.

Orbital Extension of Intracranial Tumors The orbit is very rarely invaded by tumors other than meningiomas from the intracranial cavity. As a result this section will mainly focus on the management of meningiomas. Other tumors that do invade the orbit include parasellar tumors such as pituitary tumors and craniopharyngiomas.1,2,73,74 These tend to involve the superior orbital fissure and optic nerve foramen resulting in compression of structures at these sites. Gliomas are the most common intracerebral malignancy. However, they rarely involve the orbit. It is only the high grade and aggressive glioblastoma multiforme that may invade the orbital contents either directly or via the foramina. Unfortunately, prognosis is poor for these tumors. External beam radiotherapy may temporarily halt growth and reduce short-term visual deterioration, but this treatment is only palliative.3/75 Meningiomas account for approximately 18 % of all intracranial neoplasms.4,76 Annual incidence is approximately 2 per 10000 per year afflicting females twice as often as males.5,77 Meningiomas originate from the meningoepithelial cells of the arachnoid villi. Consequently, they tend to originate at sites where the arachnoid villi are present. These include parasagittal regions, cavernous sinus and foramen magnum. The sites of interest with regards to orbital extension include the sphenoid wing, lamina cribrosa and tuberculum sella.6,78 There is still some debate as to the etiology of these tumors. Various theories have been proposed including trauma or viruses. However, as yet only ionizing radiation 7,79 and deletions of chromosome 22 have been accepted.8,80 The alterations of chromosome 22 mean that there is also a link to neurofibromatosis type 2. It has also been noted that some meningiomas express progesterone receptors and that there is a preponderance for meningiomas in female breast

cancer patients.9,81 Thus there may also be a hormonal driving force towards growth. Meningiomas have been classified into 15 subtypes under the WHO classification system.10,82 94% are thought to be benign, 5% atypical and 1% malignant.11,83 As the majority are benign they mostly exert their effects locally by compression or more distantly through mass effect or raised intracranial pressure. Consequently, it is apparent that the structures affected will be dependent on the site of the tumor. Orbital invasion most commonly presents with early unilateral painless visual loss. The visual loss is usually gradual, but in less than 5 % of cases may be acute.12,84 Other symptoms may include headache, nausea and diplopia. Examination may reveal optic atrophy or papilloedema (Figure 18.19A). Meningiomas also display bone involvement showing hyperostosis and less frequently lysis. This can be visualized well with CT. MRI can be used to define the involvement of local structures (Figure 18.19B). With the increasing availability of 3 dimensional reconstruction planning of therapy is now much easier. The aim of surgery is to completely excise the tumor (Figure 18.19C). If this is not possible then aggressive debulking should be attempted with

Figure 18.19A: Intracranial meningioma with orbital extension

258 Surgical Atlas of Orbital Diseases

Figure 18.19B: CT scan showing a mass in the temporal fossa with extension into the orbit and shows hyperostosis of the temporal bone, sphenoidal bone and inferolateral orbital bones

postoperative radiotherapy. A combined surgical approach can be performed with a neurosurgical craniotomy and an ophthalmic orbitotomy. Ideally, both neurosurgical and ophthalmic teams are involved, although increasingly excision is performed solely by neurosurgeons. Orbitotomy sites vary with position of extension of the meningioma into the orbit. Whilst craniotomies can be fronto-temporal, pterional, supraorbital ridge or subfrontal. For cases in which an operative approach may not be suitable external beam radiotherapy(EBRT) or IMRT can be applied. Newer treatments such as chemotherapy with hydroxyurea seems to have achieved little success in tumor debulking.13/85 Whilst hormonal therapy with anti-progesterone receptor agents seems to have had some success with selected tumors.14/86 Final success depends on recurrence. The recurrence rate of meningiomas varies between 10 and 20%. They may recur upto 20 years after initial excision and yearly close follow up with CT scanning is advocated. 15,87 Recurrence tends to yield large bulky tumors that affect local structures and require extensive debulking thus resulting in an increased morbidity.

Figure 18.19C: 1 week postoperative following removal of the meningioma by orbito-temporal approach

Orbital Extension of Conjunctival Tumors Squamous cell carcinoma of the conjunctiva The incidence of conjunctival squamous cell carcinoma is 1-2.8 per100, 000.88 It affects the elderly with a mean age of 60 year. Over 70% of the patients are male.88,89 Sunlight exposure due to UV-B radiation90 is a risk factor.91 HPV has also been implicated especially in types 16 and 18.92,93 Ultraviolet induced mutations in TP53 have also been implicated. It occurs at a younger age and is more aggressive in patients with immunosuppression. The lesion occurs commonly in the interpalpebral area, with the majority being perilimbal. The presentation varies from a gelatinous mass or nodule to a flat patch of leukoplakia or a diffuse invasive lesion (Figure 18.20A).88,94 The commonest presenting symptoms are red eye and ocular irritation. 95 Squamous cell carcinoma mimicking sclerokeratitis has also been reported. The tumor may involve the underlying sclera in one third of the cases with intraocular involvement in 11-13% and orbital invasion in 11-15% (Figure 18.20B).87,95 Risk of orbital involvement may be higher with mucoepidermoid variants. 96 Impression

Secondary and Metastatic Orbital Tumors 259 cytology may aid in the diagnosis by displaying keratinized dysplastic cells, hyperkeratosis, syncytial-like groupings and prominent and large nucleoli.97 UBM can show intraocular extension of conjunctival squamous cell carcinoma (Figure 18.20C). Management of OSSN requires adequate excision and careful follow up to monitor any recurrence.98 Recurrence following excision depends on histopathological grade of tumor and involvement of surgical margins. 99,100 Cryosurgery, topical 5-fluorouracil or mitomycin C treatment following

surgical excision has been shown to decrease the recurrence rate.101,102 Topical interferon α2b has been used in the treatment of presumed recurrent corneal and conjunctival intraepithelial neoplasia.103 Once the orbit is involved exenteration may be required.

Malignant melanoma of the conjunctiva has an incidence of 0.2 to 0.8 per 1000 in whites104-106 and comprises 2-3% of ocular tumors 107,108 and is less commonly seen in the pigmented races.106 It has

Figure 18.20A: Conjunctival squamous cell carcinoma (SCC) with 360° involvement of limbus

Figure 18.20B: Scleral necrosis and invasion by the conjunctival SCC

Malignant Melanoma of the Conjunctiva

Figure 18.20C: UBM showing intraocular extension of conjunctival SCC in ciliary body region

260 Surgical Atlas of Orbital Diseases increasing preponderance with age109 and arises de novo or from a nevus. However, most commonly110 these develop from primary acquired melanosis (PAM) with atypia (Figure 18.21). 111,112 The most common site of origin is the limbus.104 Other sites include the fornices, palpebral conjunctiva and caruncle (Figures 18.21A and B). Folberg R and Co-workers had reported that 50% of PAM with atypia may progress to malignant melanoma. 113-115 PAM appears as flat, patchy pigmentation in the conjunctival epithelium and can remain dormant for years or show slow progression or may wax and wane. 116,117 Development of thickening within areas of PAM may indicate invasive growth. However, a junctional nevus may be indistinguishable from PAM with atypia histologically. Junctional nevi are seen in childhood while PAM occurs in middle-aged and elderly individuals. Biopsy of lesions that are widespread, large, thickened, dark, palpebral, unusually vascular or progressive has been suggested.118 Conjuctival melanoma arising from pre-existing nevus119,120 may be characterized by change in color, nodularity or bleeding. The 10 year survival rate in one series was 77.7%. Unfavorable tumor location (palpebral conjunctiva, fornix, caruncle, corneal stroma, eyelid), age greater than 55 years, higher TNM category, tumor thickness more than 10 mm, high mitotic index and more epitheloid cells were associated with higher risk of tumor related death. Tumors with unfavorable location, higher TNM grade, and excision alone as initial therapy had a

Figure 18.21A: Conjunctival malignant melanoma

higher probability of local relapse than favorably located (bulbar and limbal conjunctiva) tumors, lower TNM grade, and excision plus adjuvant therapy.121,122 Orbital extension of malignant melanoma of conjunctiva occurs far less frequently when compared to intraocular melanomas in the ratio of 4:23.123 Treatment includes extensive tumor removal, cryotherapy and topical mitomyin C with amniotic membrane allograft for diffuse conjunctival and corneal melanoma arising from primary acquired melanosis (Figure 18.20B).124 As primary and adjuvant therapy, topical mitomycin yielded an overall recurrence rate of 50%.125 Survival rates were found to be worst for those with intraorbital spread.107

Orbital Extension of Tumors of the Nasal Cavity and Paranasal Sinus Malignant tumors of this region are a diverse group of neoplasms. They are relatively rare and as a result few definitive studies have been performed into their management. The studies that have been undertaken illustrate that prognosis and management vary depending on the type and site of tumor diagnosed.

Figure 18.21B: Postoperative total excision of conjunctival malignant melanoma with application of cryotherapy to the conjunctival margins. 5 years postoperative without any recurrence

Secondary and Metastatic Orbital Tumors 261 Growth is often large prior to symptoms from these tumors, partly due to the nature of the cavities from which they arise. Since only a thin bony wall separates the nasal cavity and sinuses from important structures, invasion of nerves, brain and orbit are frequent. These tumors are more commonly found in males with 80% presenting between the ages of 45 and 85. There is an increased risk in people living in developing countries.126 Due to the non-specific nature of presentation diagnosis is often difficult. Patients may present with nasal obstructive symptoms, rhinorrhea, epistaxis and less commonly pain. If the orbit is involved then proptosis and ocular motility may be affected depending on the site of the original tumor. Vision is usually affected later unless the orbital apex is involved whilst conjunctival congestion may result from a larger mass in the orbit. Thus when nasal symptoms are persistent cases should be investigated further. Endoscopy and more detailed radiography have indeed improved earlier pick up. The maxillary sinus is the most common site of primary tumor origin (55%) (Figure 18.22), followed by the nasal cavity (35%) and then the ethmoids (9%). The sphenoidal and frontal sinuses are rarely involved but when they are they confer a poor prognosis.2,127 The majority of tumors are squamous cell carcinomas (80%) 127,128 with adenocarcinoma and adenoid cystic carcinoma making up the majority of the rest. The orbit is thus also most frequently invaded by squamous cell carcinoma. Routes include the foramina, perineural spread or direct invasion via bony erosion. Lymphatic spread is however rare. Squamous cell carcinomas most commonly arise from the turbinates. Consequently, they present more commonly with signs of congestion and nasolacrimal duct obstruction. Up to 80 % have bony erosion of the orbit at the time of diagnosis.129 Nickel and chrome workers have a higher incidence of squamous carcinomas.130 It is also increasingly thought that smoking is a risk factor for development of these tumors.131 Biopsy is often important as the various different forms of squamous carcinoma confer different modes of spread and hence affect prognosis. Adenocarcinoma is more common in woodworkers and is linked to other chemicals for instance those found in the leather tanning industry. 132

The prognosis from these tumors varies with histological grading. They commonly invade the base of skull and the orbit affecting vital structures. However, distant metastasis is rare. Undifferentiated carcinomas have a very poor prognosis, usually being picked up at a late stage. Unlike squamous carcinomas they are commoner in women than men. Small cell carcinomas are similar to their lung counterparts and may present with early proptosis, epistaxis and obstructive symptoms. X-ray was the initial tool for diagnosis and is still useful in determining location, size and presence of bony erosion. 129 However, it has now been surpassed by computed tomography which highlights bony involvement. MRI T2 weighted helps differentiate tumor from surrounding tissue and edema. Bowing of tumor-fat interface in fat saturated images gives a clue as to invasion. If this changes from smooth to irregular, invasion is far more likely.133 Treatment of nasal and sinus tumors usually falls beyond the domain of ophthalmologists. Specialists from the fields of head and neck surgery and maxillofacial surgery provide the best hope for patients. Surgeons with skills in facial reconstruction may also help the patients once treatment is completed. There is still some debate over the best modality of treatment partly due to the lack of adequate research. However, in a recent meta-analysis, surgery alone seemed to be better than combination with radiation. Worst of all appeared to be radiation alone.134 Often radiotherapy is given to help reduce the size of the tumor prior to surgery, however, as yet there is little evidence that this improves long-term survival. Thus, surgery with wide local margins still seems to be preferred for low stage cancers and surgery with some adjunctive radiotherapy used for higher stage cancers. Different approaches have been used to gain access dependent on the size and position of the tumor. Recently, endoscopic approaches have been used to reduce postoperative morbidity with complete transnasal resections, although this is still a developing field.135 Another area of debate is the role of exenteration with orbital involvement. Studies have shown that preservation of the eye has not affected survival rates. 136 Indeed if adequate reconstruction is performed a good functional outcome can be achieved in up to 90% of cases.137

262 Surgical Atlas of Orbital Diseases However, we recommend orbital exenteration with involvement of the orbital apex, extraocular muscles, bulbar conjunctiva and sclera.Radiotherapy has been used for some time in the treatment of head and neck tumors. However, high doses of radiation are required for successful treatment. Since the tumors are found close to structures that are highly radiosensitive, morbidity is often high following treatment. In a review of 48 patients treated with curative radiotherapy 33% developed unilateral blindness whilst 4 developed bilateral blindness secondary to optic nerve damage. 138 However, irradiation can be used in rarer instances when cervical lymph node spread has occurred. Chemotherapy is usually reserved for certain types of tumor. These include lymphoma, neuroendocrine, enthesioneuroblastoma and undifferentiated carcinomas. The trouble in the past has been the systemic side effects. However, increasingly a local approach has been attempted with some success. Prognosis from a recent review suggests an overall survival of around 40%. Prognosis varied with site and type of lesion. Nasal fared better than ethmoidal which in turn had better survival than maxillary. There was also a worse prognosis for frontal and sphenoidal involvement due to increased invasion of the brain and dura. Glandular carcinomas had a better prognosis than squamous carcinomas.134

Figure 18.22: Carcinoma of maxillary sinus with involvement of paranasal sinus,nasal cavity, the orbit and surrounding adnaxae leading to extreme lateral displacement of the right orbit

Other studies have shown that although orbital involvement is not detrimental per se, and deep orbital involvement leads to a poorer survival, hence the advice for exenteration when these tissues do become involved.139/140

Orbital Extension of Nasopharyngeal Tumor Although nasopharnygeal tumors are found in close proximity to those of the nasal cavity, they have an altogether different pattern of presentation partly because of the vast lymphatic network present in the region. They are almost a distinct entity affecting a generally younger age group, between 45-55. There is a higher incidence amongst males with the highest incidence amongst the southern Chinese populations. Here 15-30 males per 100,000 are diagnosed with the condition per year. However, in other parts of the world the disease is relatively rare. There is thought to be a genetic element as the risk continues to remain high even in second generation Chinese emigrants despite the change of environment factors. 141/142 Epstein-Barr virus has been implicated as the environmental agent as carcinoma cells have been found to contain viral DNA.143 Changes in the make up of these cells are thought to result in squamous cell carcinoma. Unlike nasal tumors they are far more likely to incur systemic spread. Patients tend most commonly to present with a neck mass secondary to regional lymphnode metastasis. This is often linked with dysphagia. Other symptoms include nasal symptoms such as rhinorrhea and nasal congestion, auditory problems such as hearing loss, fullness and recurrent otitis media. If the orbit is involved then symptoms may include proptosis, reduced vision, ocular motility disorders and pain. The orbit is rarely involved and is usually affected later than other structures.144 Direct invasion through bone is rare. However, common routes include pterygopalatine fossa, inferior orbital fissure and then secondarily through the paranasal sinuses. Either CT or MRI can be used to help detect the pathway of tumor entry into the orbit.145 Unlike nasal and paranasal sinus tumors surgery plays only a minor role in the management of these tumors, because local metastatic spread is already likely to have occurred. Thus radiotherapy is the primary treatment modality for orbital invasion. Chemotherapy is used as an adjunctive treatment for

Secondary and Metastatic Orbital Tumors 263 advanced regional metastasis or more distant spread. Recent reviews of cases have shown that orbital involvement confers a particularly bad prognosis. The 5 years survival of a recent case series of nasopharyngeal cancer with orbital extension was only 28%.146

Metastatic Orbital Tumors Metastasis to the orbit occurs secondary to hematological spread of a primary tumor. Of all the orbital cases 1.5 to 3.3 % are metastatic of which 7 % cases are bilateral. In the pediatric age group, the common primaries include neuroblastoma, Wilm's tumor and Ewing's sarcoma. In adults, the common primary sites for metastatic tumors to the orbit are from breast, prostate, lung, gastrointestinal tract, kidney and cutaneous melanoma. Most commonly, the patient has a known primary tumor. The most common malignancies that metastasize to the orbit are carcinoma of the breast in females and carcinoma of the lung in males.147,148 Metastases to the orbit have also been reported to occur from carcinoma of the tongue, pancreas, gallbladder, cervix, penis, urinary bladder, thyroid intestinal carcinoid, renal cell carcinoma and carcinosarcoma of the parotid gland.149-157 Orbital metastasis may also be the first manifestation of a systemic malignancy. Metastases to fat and bone are twice as common than muscle. Usually the primary tumor is known except in about one quarter of patients. Certain carcinomas like melanoma and breast carcinoma are likely to have a known primary. Average survival is approximately 9 months from the orbital presentation. The first manifestation of symptoms due to orbital metastasis usually occurs after a mean period of 31-64 months after diagnosis of primary disease, but tumors such as breast cancer and thyroid cancer have a longer delay (3-5 years). Melanoma is intermediate (2 years) and those from the lung and gastrointestinal tract are diagnosed shortly before or after orbital presentation. The mean age of the patients was 64 years in one study.147 These tumors present similarly to others involving the orbit. Symptoms include lid swelling, red eye, pain, proptosis and diplopia and signs include incomitant strabismus with diplopia, blepharoptosis, decreased vision, proptosis and a

palpable mass.158,159 However, motility disturbance out of proportion to the proptosis is characteristic of an orbital metastatic tumor. Unusually, enophthalmos has been reported with schirrous carcinomas of the breast whilst metastases from the prostate to the orbit are usually osteoblastic in nature. 160,161 Renal cell carcinoma has a tendency to induce hemorrhage and consequently it may initially be difficult to differentiate from orbital hematoma.156 Diagnosis of metastatic origin follows simple lines. Site of the original tumor may also be ascertained with symptoms and signs suggestive of other systems being involved. Investigations may include nonspecific tests such as carcinoembryonic antigen (CEA) which is elevated in metastatic disease of the bowel. Other specific tests may be refined with regards to the primary tumor suspected. Both CT and MRI have a place in management. Both hyperostotic and spiculated CT appearances have been described.147,149 MRI may assist in soft tissue differentiation and to assess spread. Ultimately, however, a fine needle aspiration biopsy may be required to confirm the diagnosis in situations where an orbitotomy is not possible.162 The survival rate of these patients is poor and consequently treatment is mainly palliative and is aimed at providing symptomatic relief 163 with radiotherapy, hormonal therapy, chemotherapy and surgery depending on the type and stage of the disease.

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264 Surgical Atlas of Orbital Diseases 7. Namaune F, Doz F, Tangley ML, et al: high dose chemotherapy with carboplatin, etoposide and cyclophosphamide followed by a haematopoetic stem cell rescue in patients with high risk retinoblastoma. A SFOP and SFGM study. European Journal of cancer. 1997;33(14); 2368-75. 8. Keralli H, Belgic S, Ozerdem U: management of massive orbital involvement of intraocular retinoblastoma. 9. Hemsmann N, Steform F H: Medullooepithelioma of the ciliary body: Acta Ophthalmol 1991: 69: 398-401. 10. Myron Y, Ben S F: Ocular pathology. (3rd ed) 1989; 504 11. Llyod WC 3rd,O'Hara M: Malignant teratoid Medulloepithelioma: clinical-echographic- histopathologic correlation: JAAPOS 2001: 5: 395-7. 12. O'Keepe M, Fulcher T, Kelly P, Luv W, Dudges J: Medulloepithelioma of the Optic nerve head: Archives of Ophthalmology .1997: 115; 1325-27. 13. Shields JA, Eagle RC Jr, Shields CL, Singh AD, Robitaille J. Pigmented Medulloepithelioma of the ciliary body. Arch Ophthalmol 2002; 120: 207-10. 14. Brougthon WL, Zimerman LE. A clinicopathologic study of 56 cases of intraocular Medulloepithelioma. Am J Ophthalmol 2000; 130:364-6. 15. Pradhan SB, Adhikari RC, Shrestha B, Sayami G, Dali S, Shrestha HG: Malignant teratoid Medulloepithelioma in eye. Kathmandu University Medical Journal. 2003:1:42-4. 16. Charif Chefchaouni M, El Bakkali M, Abdellah H, El Amarti A, Benchrifa F, Saidi O, Berraho A: Medulloepithelioma of the ciliary body- a case report. J Fr Ophthalmol. 2000; 23(2):165-9. 17. J Michael Jumper, Devron H Char, Edward L Howes Jr, Daniel G Bitner: Neglected malignant Medulloepithelioma of the eye: Orbit. 1999: 18:1 37-43. 18. Julian Garcia- Feijoo et al. Medulloepithelioma of the ciliary body-ultrasonographic biomicroscopic findings. J Ultrasound Med. 2005: 24: 247-50. 19. Collaborative ocular melanoma study group: enucleation radiation of large choroidal melanoma I: Characteristics of patients enrolled. Am J Ophthalmol 1998;125(6): 76778. 20. Mclean IW, Zimmerman LE, Evans RM: Reappraisal of Callender's spindle a type of malignant melanoma of choroids and ciliary body Am J Ophthalmol 1978;86(4): 557-64. 21. Polito E, Leccisotti A Primary and secondary melanomas: A clinical and prognostic study. Ophtal Plast Reconstr Surg 1995;11: 169-81. 22. Hungerford J. Prognosis in ocular melanoma. Br J Ophthal 1989; 73: 689-90. 23. Saadati H, Calvano C and Diba R. Orbital and optic chiasmal extension of uveal melanoma. Ophthal Plast Reconstr Surg 2004;20(5) 387-90. 24. Shammas HF, Blodi FC. Orbital extension of choroidal and ciliary body melanomas. Arch Ophthalmol 1977; 95: 2002-5.

25. Shields CL, Shields JA, Yarian DL Augsberger JJ. Intracranial extension of choroidal melanoma via th eoptic nerve. Br J Ophthal 1987; 71: 172-6. 26. Recsan Z, Karlinger K, Fodor M et al. MRI for the evaluation for scleral invasion and extrascleral extension of uveal melanomas. Clinical radiology 2002; 57: 371-6. 27. Collaborative ocaular melanoma study group. The COMS randomized trial of iodine 125 brachytherapy for choroidal melanoma III: Initial mortality findings: COMS no.18 Arch Ophthalmol 2001;119:969-82. 28. Lin KL, Tena LB, Finger PT Orbital brachytherapy for extrascleral extension of choroidal melanoma. BR J Ophthalmol. 2006;90:1438-39. 29. McNab AA, Mc Kelmme P, malignant melanoma of lacrimal sac complicating primary acquired melanosis of the conjunctiva.Ophthalmic Surg Lasers 1997; 28;501. 30. Stefanyszy MA, Hidayat AA, Peer JJ, Flanagan JC; Lacrimal sac tumours: Ophthal plastic reconstr surg: 1994. Sept; 10 (3): 169-84. 31. Ryan SJ, Font RL: Primary epithelial neoplasms of the lacrimal sac. Am J Ophthalmol 1973: 76: 73-88. 32. Ni C, D' Amico DJ, Fan CQ, Kuo PK: Tumours of the lacrimal sac. A clinicopathologic analysis of 82 cases. Int Ophthalmol clin 1982: 22: 121-40. 33. Haward J Loff; Landa N:Malignant eyelid lisions: Ophthalmic hyper guide; section: oculoplastics; 2006:1-6. 34. Takamura H; Yamashita H: Clinicopathological analysis of malignant eyelid tumor cases at Yamagata hospital: Statistical comparision of tumor incidence in Japan and other countries; Japanese journal of Ophthalmology: Vol 49, No 5, 2005;349-54. 35. Lear J T, Harvy I, de Barkar D: Basal cell carcinoma: Journal of the Royal Society of Medicine, 1998;91:585-8. 36. Kricker A, Armstrong B K, English D R : Sun exposure and non melanocytic skin cancer . Cancer Causes Control 1994;5(4):367-92. 37. Vitasa B C, Taylor H R, Strickland P T et al, Association non melanoma skin cancer and actinic keratosis with cumulative solar ultraviolate exposure in Maryland watermen:cancer 1990:65;2811-17. 38. Wojne T H: The association between cigarette smoking and basal cell carcinoma of eyelid in woman : Ophthal plast reconstr surg.1999;15(6),390-2. 39. Shield C L, basal cell carcinoma of the eyelid : Int Ophthalmol Clinic, 1993;33:124. 40. Lo J, Snow S, Reinzer G: Metastatic basal cell carcinoma: Report of 12 cases with a review of literature, J Am Acad Dematol, 1991;24,715-19. 41. Motley R J, Gouldf D J, Douglas W S, Sympson N B: Treatment of basal cell carcinoma by dermatologist in the UK, Br J Dermatol, 1995;132: 437-40. 42. Mak A S, Poom A M Leung C Y et all, Audit of basal cell carcinoma in Princess Margarat Hospital, Hong Kong : Usefulness of frozen section examination in surgical treatment: Scand J Plast Reconstr Surg Hand Surg 1995;29:149-52.

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270 Surgical Atlas of Orbital Diseases

Decision Making 271

19

Decision Making

CHAPTER Subrahmanyam Mallajosyula

The etiology of proptosis is multifactorial. As we know it can be congenital, or acquired due to various causes like infections both nonspecific and specific bacterial like tuberculosis,1-4 sarcoidosis,5-7 fungal infections, inflammations, parasitic infestations, vascular malformations, primary orbital tumors arising from various tissues like nerves and their sheaths, vascular tissues, mesenchymal, lymphoproliferative, bony lesions apart from secondary involvement from the globe, paranasal sinuses and the brain. Metastatic lesions from breast,8 thyroid, prostate, GIT and other primary lesions elsewhere9-12 were documented in the literature. Involvement of Orbit in generalized lymphoproliferative lesions like systemic lymphomas, leukemia is very well known. It is really surprising to know the wide variety of pathology that can involve such a small structure as orbit. It is not an exaggeration that in the orbit one can come across almost all the types of pathology that can involve human body. With such a wide spectrum of pathological lesions causing proptosis, it is natural that the management strategies also vary. The options include observation and reassurance, medical management, surgery, radiotherapy and chemotherapy. The surgical procedures include biopsy, debulking, excision of tumor/mass/cyst through various surgical approaches, multi-speciality approaches along with ENT/Neurosurgeon, and exenteration.The decision depends upon accurate diagnosis of the nature of the lesion (infective/cystic/tumor: benign/ malignant/Secondary/metastatic) and its location and extent (intraconal/peripheral space/extension

to paranasal sinuses/intracranial extension). A detailed clinical evaluation, and imaging with CT scan/MRI of Orbit usually give us enough information to plan proper management strategy. Other investigations like serology, FNAC13,14, Squash and IHC (Immuno-Histochemistry) are useful aids in establishing the diagnosis and planning the management. In this chapter I am outlining the different management strategies and which to choose from. The details of the management procedures are described in the following chapters. Medical management is indicated when proptosis is due to orbital cellulitis, orbital myocysticercosis, idiopathic orbital inflammation, most cases of thyroid associated orbitopathy (the drug-trial for TAO is well known), Wegener's granulomatosis, tuberculosis of the orbit, fungal granulomas, in other words in all the inflammatory and infective conditions. However, drain an orbital or sub-periosteal abscess, along with systemic antibiotics. The role of medical management is discussed in a separate chapter. Chemotherapy and radiotherapy have a great role in the management of rhabdomyosarcoma, lymphoma and other lymphoproliferative diseases, retinoblastoma. They can also be used occasionally in thyroid orbitopathy, idiopathic orbital inflammation. They are helpful in the treatment of other malignancies like adenoid cystic carcinoma of lacrimal gland, secondary and metastatic lesions. These were discussed in a separate chapter. For me, it is always very important to get at an accurate diagnosis as early as possible, so that I am not missing any serious condition. That is why, even in a case of clinically suspected orbital cellulitis, I get

272 Surgical Atlas of Orbital Diseases a CT scan as early as possible to make sure that I am not missing other conditions like rhabdomyosarcoma, retinoblastoma which can occasionally present as orbital cellulitis. The CT scan also informs me if there is an abscess which I have to drain, or it is only orbital cellulitis which I can manage medically. When the diagnosis is not definite, I prefer to do a biopsy and get a histopathological diagnosis. The tissue diagnosis can be by way of FNAC (Figures 19.1A and B and 19.2A and B), biopsy or intraoperative Squash smear examination. However I personally don't perform FNAC on a lacrimal gland tumor. Instead I wish to excise the tumor in toto and plan further treatment depending on the histopathological diagnosis. Biopsy is indicated for histopathological confirmation of clinical diagnosis (Figures 19.3A to C) as in lymphoma, meningioma of optic nerve sheath, rhabdomyosarcoma, so that the patient can be referred to an oncologist. Biopsy is also indicated when the clinical diagnosis is not definite (Figures 19.4A to E).

A

B

Figures 19.1A and B: FNAC showing malignant cells in whorl pattern, suggestive of neuroblastoma. The details are better seen in higher magnification. This patient presented with painful proptosis and restricted ocular motility. CT scan revealed a mass lesion with bony erosion. Diagnosis of metastatic neuroblastoma was made from FNAC

A

When I am planning excision of the tumor, the approach depends on its location. If it is involving surrounding structures like PNS, or brain, it will be a multispeciality approach. However, FESS (Functional endoscopic sinus surgery) is the best way to manage fronto-ethmoid mucocele. If the tumor is confined to the orbit, it will be dealt with by me alone, the only exception being a small apical lesion, for which I prefer a transcranial approach. The orbital approaches to proptosis are lateral orbitotomy approaches and anterior orbitotomy approaches. The anterior orbitotomy approaches are further divided into cutaneous approaches and conjunctival approaches. When choosing a procedure I consider the surgical exposure and also the cosmetic result. Though I wish to hide the scar and give the best cosmetic result, adequate surgical exposure of the lesion is of paramount importance, which can not be over-emphasized. I advise the beginners and the occasional orbital surgeons to choose simpler procedures first, and as they gain experience, they can perform other complicated procedures. I wish

B

A

B

Figures 19.2A and B: FNAC showing cells with large, peripheral nucleus and vacuolated cytoplasm arranged in clusters, from metastatic ductal carcinoma of breast leading to proptosis

C

Figures 19.3A to C: Typical Salmon's patch of lymphoma arising from the upper fornix (A) and lower fornix (B). Biopsy is indicated for histopathological confirmation (C)

Decision Making 273

A

Figures 19.4A and B: This patent presented with painful proptosis of 1 month duration.He had exposure Keratitis and his vision was 20/ 400. Note the severe swelling, tarsorrhaphy for exposure keratitis, and chemosed conjunctiva. The CT scan shows a heterogenous lesion surrounding the optic nerve. Radiologist reported it as meningioma. The past history was significant that he had discontinued treatment for tuberculosis.Since this type of acute/subacute presentation is unusual for meningioma, I thought of infective pathology ( tuberculosis) and wanted to confirm the diagnosis by a biopsy

B

C

D

E

Figures 19.4C to E: When lateral orbitotomy was performed (C) a pinkish mass was found around the optic nerve, which was biopsied. The histopathology showed it to be non-Hodgkins B-cell lymphoma (D) He was referred to an oncologist and he responded very well to radiotherapy (E)

to emphasize that usually there are more than one option, and the procedure chosen varies from one surgeon to other, depending upon the individual's preference. Intraconal Lesion: Lateral orbitotomy is indicated for intraconal lesions and lacrimal gland lesions. There are different approaches; the most commonly performed are Stallard-Wright's procedure, Reese-Berke's procedure and superior lidcrease incision (Figures 19.5A and B). Each has its own merits. I will outline these approaches, before I discuss my preferences. Stallard-Wright's incision starts at the lateral third to half, beneath the eyebrow, up to the lateral end of the brow, and then descends vertically along the lateral border of the orbit, and extends horizontally along the crow's feet .Thus it has two horizontal incisions, one at the level of sub-brow, and the other

A

B

Figures 19.5A and B: Lateral Orbitotomy incisions in frontal (A) and lateral (B) views. Stallard-Wright (yellow), Reese- Berke (White) and superior lid crease incision (pink)'

at crow's feet, which are well hidden. But the vertical component of the incision leaves a visible scar. The advantages of the incision include a very large and adequate area of surgical exposure to deal with huge tumors. The other advantage is that the lateral canthus is not disturbed. Reese-Berke’s incision (Figures 19.6A to 19.9B) is a horizontal incision that starts from the lateral canthus and extends 4-5 cm horizontally along the crow's feet. This incision gives a very good exposure to deal with most of the tumors. The scar is very well hidden in the crow's feet and surgical scar is never an issue. The only disadvantage is that the lateral canthus is disturbed and needs to be reconstructed at the end of the surgery. For those of you who are routinely doing oculoplastic procedures, this is neither difficult, nor time consuming. In those situations where the patient had a long standing and prominent proptosis, you may even do lateral tarsal strip at the time of reconstruction of the lateral canthus and correct horizontal laxicity of the lid. Superior lid crease incision (Figures 19.10A to 19.11C) is another excellent approach, where in the incision is along the lid crease, and then extends along the crow's feet. The entire incision is very well hidden, so that the scar is not visible. It also gives a very adequate exposure. The lateral canthus is not disturbed. However, damage to Levator Palpebrae Superioris leading to ptosis is a known complication.

274 Surgical Atlas of Orbital Diseases

Steps of Reese-Berke Approach

A

B

Figures 19.6A and B: Steps of lateral orbitotomy Reese-Berke's incision. Traction suture was applied to lateral rectus (A). Horizontal incision made from the lateral canthus (B)

A

B

Figures 19.7A and B: Zygoma was exposed (A), cuts were made above and below and the zygoma was being removed with a rounger (B)

A

B

Figures 19.8A and B: Periosteum was incised, and reflected (A). Lateral Rectus was identified. Traction suture applied earlier helps in its identification. Lateral rectus was retracted away from the tumor (B)

Decision Making 275

A

B

Figures 19.9A and B: The tumor was removed with the help of cryo (A). After securing hemostasis, the Zygoma was replaced in its place and secured. Drain is placed in a separate stab incision and not in the original incision. This gives a better scar. The wound is closed in layers

Steps of Superior Lidcrease Incision

A

B

C

Figures 19.10A to C: The incision is along the lid crease and extends horizontally along the crow's foot (A and B). Carefully dissect up to the septum, and approach the lateral wall (C)

A

B

C

Figures 19.11A to C: The lateral wall is exposed (A), which has been removed. Periosteum was incised. The tumor was exposed (B), dissected, and removed with the help of a cryo (C)

276 Surgical Atlas of Orbital Diseases I believe that a scar on the face is a cosmetic blemish. I wish to perform the orbitotomy leaving as minimal scar as possible. The scar induced by the vertical component of Stallard-Wright's incision is of concern, and I wish to avoid it. Hence it was more than 2 decades since I performed Stallard-Wrights incision. My routine is Reese-Berke's approach. It gives adequate exposure to deal with most of the tumors (Figures 19.12A to 19.13C). If the tumor is too big, then I perform superior lid crease incision. The scar induced in these two procedures is very well hidden and patient's satisfaction is very high. However it is very important to remember that intraconal tumor can be excised by lateral orbitotomy

A

approach, only if it is lateral to optic nerve. If the tumor is medial to optic nerve, it cannot be excised through lateral orbitotomy as the optic nerve can get damaged. In these situations, antero-lateral orbitotomy is preferred (Figures 19.14A to 19.21B). Hence the importance of assessing the relationship of the tumor with optic nerve in coronal sections of CT scan cannot be over-emphasized. In antero-lateral orbitotomy, lateral orbitotomy is performed to get space and better surgical exposure. 180° peritomy is performed, the medial rectus is disinserted, the globe is retracted laterally and the tumor is removed between the globe and the retracted medial rectus. Then the medial rectus is reattached.

B

D

C

Figures 19.12A to D: Note the gross proptosis of right eye in this female, due to a very large cavernous hemangioma occupying most of the orbit, as shown in the CT scan imaging. Such a large tumor could be excised through Reese-Berke's incision excised tumor (C) and postoperative recovery (D)

A

B

C

Figures 19.13A to C: Another patient with a prominent proptosis of right eye due to a large neurofibroma, excised through Reese-Berke's incision. Note that the incisional scar is very well hidden in the crow's feet

Decision Making 277

A

A

B

Figures 19.14A and B: Diagrammatic representation of anterolateral orbitotomy for a case of axial proptosis (A) due to intraconal tumor located medial to optic nerve (B)

B

Figures 19.15A and B: Lateral orbitotomy was performed with the lateral wall removed. The medial rectus muscle was disinserted, with double armed 6-0 vicryl sutures attached to the tendon

B

A

Figures 19.16A and B: The eyeball and the medial rectus were retracted to get adequate space for dissection of the tumor. The tumor was excised through this space with the help of a cryo (A). The medial rectus was reinserted, conjunctiva was sutured. The lateral orbitotomy incision was closed

A

B

C

Figures 19.17A to C: Female 45 years presented with painless, progressive proptosis of left eye of 3 years duration and progressive blurring of vision since 6 months. She had RAPD with optic disc edema (A). Her BCVA was 20/200. CT scan (B and C) revealed a homogenous, hyperdense tumor with very well defined borders in the intraconal space and medial to the optic nerve (red arrow …B). There was no enhancement on contrast. Since the tumor was intraconal and medial to optic nerve, anterolateral orbitotomy was planned

278 Surgical Atlas of Orbital Diseases

A

B

C

Figures 19.18A to C: Lateral orbitotomy was performed with Reese-Berke's incision. The Zygoma was removed, and periorbita incised to facilitate moving the globe laterally (A). 180° peritomy was performed medially from12 to 6 o'clock position. Medial Rectus muscle was identified (B), and disinserted after applying 6-O vicryl sutures (C)

A

A Figures 19.19A and B: The globe is retracted laterally, and the disinserted medial rectus medially to get adequate surgical space (S…A). By careful dissection in this space, the tumor was identified (T…B)

A

B Figures 19.20A and B: The tumor was dissected carefully from the surrounding structures and was removed carefully with the help of a cryo (A and B)

Decision Making 279

A

B

Figures 19.21A and B: The medial rectus muscle was anchored to its original incision (A). The conjunctiva was sutured into its place (B). The lateral orbitotomy wound was closed as usual

Lesions of Superior Peripheral Space: Anterior orbitotomy approaches are normally used for these lesions, the exception being lateral orbitotomy for lacrimal gland tumors. However I am excising lacrimal gland tumors, up to moderate size through anterior orbitotomy approaches. For tumors of the superior peripheral and subperiosteal spaces, both subciliary and superior lidcrease incisions give very good surgical exposure (Figures 19.22A and B). However the surgical scar is better camouflaged in the lid crease and hence is my preferred procedure (Figures 19.23A to 19.31C).

Apical conal lesions are better approached through transcranial approach with the help of a neurosurgeon. This was discussed in the concerned chapter. Imaging studies tell us if we are dealing with optic nerve glioma or meningioma of optic nerve sheath. I prefer to follow closely a case of optic nerve glioma with imaging every 6 months and perform surgery only if the eye has become blind or the tumor is nearing the orbital apex. For meningioma, I do lateral orbitotomy and take a biopsy for histopathological confirmation (which is mandatory here) before referring for radiotherapy.

A

B Figures 19.22A and B: Superior Anterior orbitotomy incisions, subciliary (white) and Lid-crease (yellow) incisions

280 Surgical Atlas of Orbital Diseases

A

B

C

Figures 19.23A to C: Female 38 years, presented with proptosis of left eye of 2 years duration. Note that the globe is pushed down and in, with fullness of superior sulcus (A). CT scan of the orbit revealed lacrimal gland tumor pushing the globe down and in (B) Excavation of the lateral wall of orbit could be seen (C)

A

B Figures 19.24A and B: Lid-crease incision was made (A), the septum was identified and reflected from the orbital rim (B)

A

B Figures 19.25A and B: The lacrimal gland mass was removed with the help of cryo (A). The excised tumor is shown in B

Decision Making 281

A

B

Figures 19.26A and B: Female 18 years, presented with progressive, painless proptosis of right eye since 8 years. Note the severe proptosis of right eye with the eyeball pushed down and out. Note also the bluish mass lesion in the right upper lid (A and B)

A

B

Figures 19.27A and B: CT scan of the orbit shows multilobulated lesion, occupying most of the intraconal space, pushing the optic nerve infero-laterally, and extending from the apex to the upper eyelid

A

B

Figures 19.28A and B: Superior lid-crease incision was chosen, since if needed, it could be converted to lateral orbitotomy also. Note the tumor (T) on either side of the superior oblique (S.O) tendon. The tendon of superior oblique was outlined yellow to facilitate recognition

282 Surgical Atlas of Orbital Diseases

A

B

Figures 19.29A and B: The tumor was excised carefully, without damaging the superior oblique, with the help of cryo (A). Note that the superior oblique tendon is intact (B), and also the cavity formed after the excision of the tumor Figure 19.30: The excised tumor (Cavernous hemangioma)

A

C

B

Figures 19.31A to C: A patient with cavernous hemangioma located in the anterior part of superior peripheral space being excised in toto through superior lid crease incision. The incision in this situation can be smaller (A) The tumor is seen in B. Look how well the incision is camouflaged in the lid crease (C) in the figure taken immediately after completion of surgery. The surgery was performed under local anesthesia

For lesions of medial peripheral space, the approaches can be percutaneous Lynch incision or transconjunctival incisions (Figures 19.32A and B).

A

Subcaruncular incision is popular for thyroid decompression of medial wall, since it gives access to ethmoid sinus. The scar is never an issue with transconjunctival approaches.

B Figures 19.32A and B: Lynch Incision (white line) and transconjunctival approaches (subcaruncular incision blue line and subconjunctival peritomy incision redline)

Decision Making 283 large, Lynch incision is my choice (Figures 19.33A to 19.34B).

However, the beginner finds it a little difficult with these approaches. Lynch incision is simpler, and a better procedure for the beginner.If the lesion is

A

B

Figures 19.33A and B: Eccentric proptosis of right eye (A) progressing for the past 3 years. He has RAPD and optic disc edema. CT scan, revealed a huge osteoma of ethmoid (B) Since the tumor is very large, the only practical approach is transcutaneous, modified Lynch incision

A

B

Figures 19.34A and B: The osteoma being removed through a modified Lynch incision (A) The excised osteoma is seen in (B)

Lesions of inferior peripheral space, can be approached either through the skin or conjunctiva (Figures 19.35A and B). Subciliary is more popular of the skin approaches. It still leaves a scar, which is mostly cosmetically

A

acceptable. However, trans-conjunctival approaches are without a visible scar (Figures 19.36A to 19.38B). Swinging lower eyelid approach causes a scar which is hidden in the crow's feet.

B

Figures 19.35A and B: The approaches for inferior peripheral space can be cutaneous like Subciliary incision (yellow line), or conjunctival approach. The swinging lower lid approach (Pink line) is a combine of both skin and conjunctival approaches and gives adequate room for surgery

284 Surgical Atlas of Orbital Diseases

A

B

C

Figures 19.36A to C: Note a large cystic lesion involving left orbit, pushing the globe up (A) Also note how brilliantly it is transilluminating (B). The cyst was excised through transconjunctival approach. The result on first postoperative day (C) is satisfactory. There is no visible scar, and the normal contour of the lid was restored

A

B

Figures 19.37A and B: This male, 24 years of age, presented with a painless swelling of 1 year duration. Examination revealed, a firm, nontender lesion in the inferior space, with orbital extension, pushing the eyeball up (A). The lid was everted, the conjunctiva and inferior retractors were separated from the lower border of the tarsus (B)

C

D

Figures 19.37C and D: Dissection was carried in this plane, and the tumor was identified (C), and carefully dissected-out and removed (D) I find Westcott Scissors and Hoskins forceps very useful in these dissections

Decision Making 285

A

B

Figures 19.38A and B: The conjunctiva and the inferior retractors are carefully reattached to the lower border of tarsus with interrupted, 6-0 Vicryl buried sutures (A) Note that the normal contour of the lid was restored on the first postoperative day. Note also that the position of the globe is back to normal, and there was no scar. This surgery can be performed comfortably under local anesthesia

Swinging lower eye lid approach is one of my favorite procedures for large lesions in peripheral surgical space, for optic nerve decompression in thyroid associated orbitopathy, and orbital floor fractures. The advantages include an excellent exposure of field of surgery, possibility of 3-wall decompression and very minimal scar (Figures 19.39A to 19.43B). Thyroid associated orbitopathy: In India, I find the clinical presentation of thyroid orbitopathy less aggressive than in western reports. This is the same observation of almost all my colleagues in India. However, I came across many Indians at Vancouver whose presentation of thyroid associated orbitopathy

A

was as severe as in the Caucasians. The weather conditions or the life style may be the reason for this difference and requires a systematic evaluation. In thyroid orbitopathy, steroids are indicated when the patient has inflammatory symptoms or diplopia. Optic nerve compression is another indication, before surgical decompression is performed. Radiotherapy with linear accelerators in non-diabetics patients is an option when the inflammatory signs and symptoms are controlled with steroids. Surgery is usually in stages, decompression followed next by muscle surgery for squint/diplopia, and then lid surgery for blepharochalasis/lid retraction.

B

Figures 19.39A and B: Female 55 years presented with bilateral proptosis of 6 months and defective vision of 3 months. She is hypothyroid and diabetic and has typical features of thyroid associated orbitopathy. Her BCVA was 20/200 20/400, and has severe optic disc edema

286 Surgical Atlas of Orbital Diseases

A

B

Figures 19.40A and B: CT scan of the orbits showed gross enlargement of the inferior, medial and superior recti, sparing the tendons. Note the bilateral apical compression, which is evident in both axial and coronal sections

A

B

Figures 19.41A and B: She underwent bilateral 3 wall decompression along with excision of fat (about 6 cc) from each orbit by swinging lower eyelid approach

A

B

Figures 19.42A and B: Note the gross difference between the preoperative (A) and postoperative (B) conditions. Note that the postoperative scar after swinging lower eyelid approach is practically not visible. The patient's vision improved to 20/20 and 20/30

Decision Making 287

A

B Figures 19.43A and B: The visual fields of right eye before(A) and after(B) orbital decompression showing marked improvement

REFERENCES 1. Kaur A, Agrawal A : "Orbital tuberculosis - an interesting case report", Int Ophthalmol. 2005;26(3):107-9. 2. Shome D, Honavar SG, Vemuganti GK, Joseph J. "Orbital tuberculosis manifesting with enophthalmos and causing a diagnostic dilemma" Ophthal Plast Reconstr Surg. 2006; 22(3):219-21. 3. Aversa do Souto A, Fonseca AL, Gadelha M, Donangelo I, Chimelli L, Domingues FS, "Optic pathways tuberculoma mimicking glioma: case report" Surg Neurol. 2003; 60(4):349-53. 4. Aggarwal D, Suri A, Mahapatra AK, "Orbital tuberculosis with abscess" J Neuroophthalmol. 2002;22(3): 208-10. 5. Mavrikakis I, Rootman J.: "Diverse clinical presentations of orbital sarcoid", Am J Ophthalmol. 2007;144(5): 769-77. 6. Biswas J, Krishnakumar S, Raghavendran R, Mahesh L: Lid swelling and diplopia as presenting features of orbital sarcoid"Indian J Ophthalmol. 2000;48(3):231-3. 7. Segal EI, Tang RA, Lee AG, Roberts DL, Campbell GA: "Orbital apex lesion as the presenting manifestation of sarcoidosis"J Neuroophthalmol.2000;20(3):156-8.

8. Ahmad SM, Esmaeli B. "Metastatic tumors of the orbit and ocular adnexal" Curr Opin Ophthalmol. 2007;18(5): 405-13. 9. Sivagnanavel V, Riordan-Eva P, Jarosz J, Portmann B, Buxton-Thomas M; "Bilateral orbital metastases from a neuroendocrine tumor" J Neuroophthalmol. 2004;24(3):240-2. 10. Bakri SJ, Krohel GB, Peters GB, Farber MG: " Spermatic cord leiomyosarcoma metastatic to the orbit" Am J Ophthalmol. 2003;136(1):213-5. 11. McCulley TJ, Yip CC, Bullock JD, Warwar RE, Hood DL: "Cervical carcinoma metastatic to the orbit", Ophthal Plast Reconstr Surg. 2002;18(5):385-7. 12. Mohadjer Y, Wilson MW, Fuller CE, Haik BG: "Primary pelvic telangiectatic osteosarcoma metastatic to both orbits", Ophthal Plast Reconstr Surg. 2004;20(1):77-9. 13. Heerema A, Sudilovsky D: "Mucinous adenocarcinoma of the ovary metastatic to the eye: report of a case with diagnosis by fine needle aspiration biopsy", Acta Cytol. 2001;45(5):789-93. 14. Saikia B, Dey P, Saikia UN, Das A : "Fine needle aspiration cytology of metastatic scalp nodules", Acta Cytol. 2001;45(4):537-41.

288 Surgical Atlas of Orbital Diseases

20

Orbitotomies

CHAPTER Ramesh Murthy, Anirban Bhaduri, Vikas Menon, Santosh G Honavar

General Principles Before undertaking any surgery in the orbit, a thorough knowledge of the normal eyelid and orbital anatomy is essential. In addition we always evaluate every case starting with a thorough history, meticulous examination, imaging and laboratory investigations. This helps us to arrive at a differential diagnosis. As a general rule, an excisional biopsy is indicated for well circumscribed lesions or those which are benign, while an incisional biopsy is performed for a lesion which is suggestive of malignancy or inflammation. Thorough understanding of CT and MRI is a must and we must emphasize that familiarity with surgical approaches is essential. One should observe and assist orbital surgeries before one embarks into this independently. One should also understand the instrumentation needed for this surgery. When using electric saws one needs to ensure safety of the patient's eyeball as well as of the surgeon and the team. Protective glasses should be worn along with face masks to prevent inadvertent spillage of blood and bone fragments. Use of retractors is required to provide adequate exposure. One also needs to be careful not to exert pressure on the globe when using the retractors. Wright's and malleable ribbon retractors are used not only to expose the tissues but also to protect the surrounding tissues. Proper illumination and adequate magnification is essential to visualize the orbital structures.1 The key to safe surgery is good surgical exposure. The surgical incision should be of adequate length. This can be supplemented by traction sutures at

appropriate locations. While cosmesis is desirable, safe and adequate surgical access is the aim. Patience is required when dissecting lesions in the orbit. Gentle blunt dissection is performed using a Freer elevator or lens spatulas to separate the orbital mass from the surrounding tissues. Repositioning of the retractors is essential as the dissection proceeds. In order to perform dissection, the blunt tipped Westcott tenotomy scissors is used. The little finger is a useful instrument to palpate the lesion and perform blunt dissection. Gentle counter traction is needed when dissecting an orbital mass. A cryoprobe can be used to hold the mass and pull it gently while dissection is going on.2 Adequate hemostasis needs to be ensured during orbital surgery. Hypotensive anesthesia is advantageous. However the anesthetist should bring back the blood pressure to normal following surgery, to ensure adequate intraoperative hemostasis. Bipolar cautery should be used in the orbit. When the source of bleed cannot be identified, simple packing with gauze should be performed. Indirect pressure over the closed lids is also useful. Bone wax or gel foam soaked in thrombin can also be used to stem bleeding during orbital surgery. For an accurate pathological diagnosis, it is necessary to obtain a sample of adequate size, representing the lesion and undamaged by cautery or surgical instrumentation. Routine samples are usually sent in formalin. If a fresh sample needs to be sent or frozen section analysis is required, it is best to inform the pathologist beforehand. The requisition form sent to the pathologist should have detailed clinical findings.

Orbitotomies 289

Approaches Many approaches can be used to gain access to the orbit.3 The various types of incisions are demonstrated in Figure 20.1. The approaches can be 1. Anterior orbitotomy a. Approach to the superior orbit — Benedict incision4 — Upper lid crease incision — Byron Smith lid split incision5 b. Approach to the inferior orbit — Mc Cord swinging lower lid incision6 — Subciliary incision c. Approach to the medial orbit — Lynch incision — Gull wing incision — Transcaruncular incision 2. Lateral orbitotomy 3. Transnasal endoscopic approach 4. Transantral or Calwell–Luc approach 5. Transfrontal orbitotomy.

Anterior Orbitotomy This approach is useful for anterior orbital lesions, for the drainage of hematomas and abscesses and incision biopsy of posteriorly placed orbital lesions.

For superior lesions a transcutaneous approach through the upper lid skin crease leaves a less visible scar. However approach thorough the lower lid skin can leave an unsightly scar. The Byron Smith lid split incision which can be used to access large superomedial lesions is not very popular. A Lynch incision can be used to approach the medial subperiosteal space. A transcaruncular approach is also useful and may be combined with a lateral orbitotomy. For inferior orbital lesions a lower eyelid transconjunctival approach is the least disfiguring.

Swinging Lower Lid Flap The first step is to make a mark on the skin horizontally at the lateral canthus. A bridle suture is placed through the inferior rectus muscle and then a mosquito artery forceps is applied to the lateral canthus along the skin mark to provide hemostasis when the incision is made. The skin is then incised with a Bard parker knife or a Colorado needle. After cutting the lateral canthus, inferior cantholysis is performed. Two 4/0 silk traction sutures are passed through the lower lid margin through the grey line of the lower lid. The traction sutures are secured to provide exposure. A conjunctival incision is then made at the inferior border of the tarsus or slightly lower starting laterally and then extending it medially. The plane between the orbicularis muscle

Figure 20.1: Various surgical approaches for orbit

290 Surgical Atlas of Orbital Diseases and the orbital septum is dissected to the inferior orbital margin. Further traction sutures can be placed through the conjunctiva and inferior retractors to improve visualization. A Desmarre's retractor can be used to give traction and expose the tissues. The periosteum or septum is then opened. With the help of retractors the lesion is exposed and blunt dissection performed to separate the lesion and perform a biopsy. Adequate hemostasis is ensured. The edges of the periosteum are sutured with 6.0 vicryl interrupted sutures. The conjunctiva is closed with interrupted 6/0 vicryl sutures and the lateral canthotomy is repaired by using 6/0 prolene sutures to secure the tarsus to the lateral orbital periosteum. Closure is performed in 2 layers with 6/0 vicryl for the soft tissues and 6/0 prolene for the skin. Antibiotic ointment is instilled and a pressure dressing is applied. The steps of this technique have been demonstrated in Figures 20.2A to Q.

Lateral Orbitotomy This is a useful technique for lesions in the intraconal space and lesions lateral to the optic nerve. In addition this is useful for large lesions anywhere as this can be combined with other approaches to allow the globe to be moved laterally for increasing surgical exposure. The Berke-Reese incision disturbs the lateral canthus and leaves a less satisfactory scar.7 The modified Stallard Wright approach is a good approach. We are presently using a lid crease approach which gives aesthetically pleasing results.

Stallard-Wright Lateral Orbitotomy8 The first step is to pass 4/0 silk sutures below the insertions of the lateral and superior rectus muscles and form a loop. This is tied to get a hold on the muscle and for identifying the muscle as surgery progresses to avoid any inadvertent damage to the muscles. An incision is made on the skin. The incision starts from just below the lateral aspect of the brow and ends in a rhytid over the anterior zygomatic arch. The skin incision is made with a no. 11 BardParker blade. Under stretch and lifting the tissues, the subcutaneous tissues are dissected down to the periosteum using a radiofrequency monopolar probe. As this dissection proceeds, one must ensure that adequate hemostasis is achieved by using a bipolar cautery. Multiple 4/0 silk traction sutures are placed

to gain exposure. The periosteum is exposed over the whole lateral orbital margin. The periosteum is cut with a monopolar probe about 4 mm behind the orbital rim starting superiorly and ending inferiorly just above the zygomatic arch. Relaxing incisions need to be given to the periosteum. The periosteum is then reflected. The periorbita is also lifted away from the orbital bone upto the anterior one third of the orbit. This has to be performed with care to avoid any breach. The zygomaticotemporal and zygomaticofacial vessels may bleed and may need to be cauterized. The temporalis muscle also needs to be separated laterally and reflected. This muscle is very vascular and adequate hemostasis needs to be ensured. Incision lines are made on the bone about 3 mm above the frontozygomatic suture superiorly and just above the zygomatic arch inferiorly. A Desmarre's retractor is placed to pull the skin and subcutaneous tissue laterally and a lid guard is placed inside the orbit to protect the contents of the orbit. Using an oscillating saw, cuts are made along the incision lines on the bone. Irrigation is performed as the saw is being used. Drill holes may be made on both sides adjacent to the bone cut. Once the cuts have been made, the bone fragment is held with a bone rongeur and moved back and forth until it fractures posteriorly. It is then removed and wrapped in wet saline gauze. The bone can be further nibbled with a bone punch or removed with a burr for further exposure. Hemostasis is essential especially in the region of the temporal fossa. Bone wax may be used to cover any bleeding points in the bone. A T shaped incision is made in the periorbita. This is done with a no 15 Bard Parker blade or a blunt tipped Westcott tenotomy scissors. The incision is then extended circumferentially. A nick is made posteriorly and the periorbita at the cut edges is grasped and gently spread apart to extend the cut posteriorly. Dissection of the orbital mass is performed by blunt dissection. Location of the orbital mass can be confirmed by gentle palpation. Wrights retractors and malleable retractors are used to gently retract the globe and keep the orbital fat away from the area of dissection. Hemostasis is achieved by bipolar forceps. The pupil is checked at regular intervals.9 A cryoprobe can be used to aid delivery of the lesion. If it is an encapsulated lesion, dissection is performed close to the capsule using a Freer elevator. Once the orbital

Orbitotomies 291 surgery has been completed, the periorbita can be closed with 6/0 interrupted vicryl sutures. The bone fragment may be replaced and either a wiring through the holes is performed or the cut edges are stuck with cyanoacrylate glue. The periosteum is then closed with 6/0 vicryl sutures. The subcutaneous tissues are apposed with 6/0 vicryl sutures and the skin is closed with 6/0 prolene continuous sutures. Intravenous steroids are preferably given at the end of the procedure. A suction drain may or may not be placed. A pressure dressing is placed after applying antibiotic ointment. The steps of this technique have been demonstrated in Figures 20.3A to S.

Transcarcuncular Approach This approach through the conjunction for medial orbital lesions is technically difficult but can give better cosmesis than a skin approach. This can be combined with a lateral orbitotomy for greater exposure.

Transnasal Endoscopic Approach and Transantral Approach This is best performed by or with the assistance of an ENT surgeon and is especially useful for biopsy of lesions near the orbital apex or arising from the sinuses. This is also a useful approach to remove any bone fragments that may be impinging on the optic nerve following an orbital fracture. This is useful for inferior lesions especially those arising predominantly from the maxillary or ethmoidal sinuses for performing a biopsy of these lesions.10

Transfrontal Orbitotomy11-13 This approach is performed to access lesions at the orbital apex. A team approach including a neurosurgeon is needed. There are potential complications in this procedure especially ptosis and extraocular muscle palsy. A bicoronal flap is created. The frontal bone flap is hinged laterally, still attached to the temporalis muscle and pericranium. This provides good exposure of the medial, superior and lateral orbital apex. If the lesion is confined to the orbit, an extradural approach is undertaken. For orbital lesions extending into the cranium, an intradural approach is needed. The orbital roof is removed, keeping the periorbita intact. The frontal nerve is an important landmark, which runs

anteroposteriorly over the levator muscle. Entry into the orbit is made medially avoiding the area of the superior orbital fissure. The orbital roof is reconstructed by using an alloplastic material or using the inner table of the frontal bone flap.

Complications This is a major surgery and there can be complications.14 1. Vascular a. Bleeding b. CRVO, BRVO c. Vitreous hemorrhage d. Short posterior ciliary artery occlusion 2. Neural/Muscular a. Corneal anesthesia b. Internal ophthalmoplegia c. Extraocular muscle paresis d. Lateral rectus adhesion e. Ptosis f. Optic neuropathy g. CSF leak h. hypoesthesia

Postoperative Management The patient is advised bed rest with the head elevated and advised not to strain to prevent increase in the venous pressure. Steroids and anti-inflammatory medication is prescribed. Systemic antibiotics may be prescribed. The vision, pupil, ocular motility and fundus is assessed.

CASE ILLUSTRATIONS Case 1 (Swinging lower lid incision of McCord) A 7-year-old girl presented with complaints of a palpable mass below right eye, gradually increasing in size over 3 months (Figure 20.2A). Clinically a firm to hard mass was palpable in the anterior inferior orbit (arrow indicates the inferomedial orbital mass) (Figure 20.2B). The mass was immobile and non-tender. The mass was causing superior displacement of the globe. Anterior segment and fundus examination were normal except for indentation effect of the mass on the globe, seen inferiorly. There was no limitation of ocular movements.

292 Surgical Atlas of Orbital Diseases CT scans showed an ill defined, hyperdense, extraconal mass in the inferomedial quadrant of the orbit with indentation of the globe and distortion of the medial wall of the orbit (Figure 20.2B). Excision biopsy of the mass was planned and was done through an anterior orbitotomy approach using a swinging eyelid incision and the mass was removed completely (Figures 20.2C to Q).

Steps of Surgery

Figure 20.2C: 4/0 silk sutures are passed through the lower lid margin, one near the lateral canthus and one centrally

Figure 20.2A: A 7-year-old girl presented with a mass below the right eye (arrow) with history of gradual increase in size over 3 months

Figure 20.2D: Mark is made on the skin horizontally at the lateral canthus and a lid speculum is placed. An artery forceps is used to crush the tissues along the mark

Figure 20.2B: CT scan (coronal section) revealed an ill defined hyperdense, inferior orbital mass in the right orbit (arrow)

Figure 20.2E: A lateral canthotomy is performed with scissors

Orbitotomies 293

Figure 20.2F: Inferior cantholysis is performed

Figure 20.2G: A conjunctival incision is made a few millimeters below the inferior tarsal margin

Figure 20.2H: The plane between the orbicularis muscle and the orbital septum is dissected to the inferior orbital margin. Desmarre's retractors are used to give traction and expose the tissues

Figure 20.2I: The periosteum is cut 4 mm from the orbital margin with a radiofrequency monopolar electrode

Figure 20.2J: Using a Freer elevator the periosteum is lifted up and access to the inferior orbit is achieved

Figure 20.2K: The orbital mass is isolated and separated from the adjoining structures

294 Surgical Atlas of Orbital Diseases

Figure 20.2L: A pink firm well defined mass was removed from the inferior orbit

Figure 20.2M: The cut edges of the periosteum are then isolated

Figure 20.2N: The edges of the periosteum are sutured with 6/0 vicryl interrupted sutures

Figure 20.2O: The conjunctiva is closed with interrupted 6/0 vicryl sutures and the lateral canthotomy is repaired by using 6/0 prolene sutures to secure the tarsus to the lateral orbital periosteum

Figure 20.2P: Closure is performed in 2 layers with 6/0 vicryl for the soft tissues and 6/0 prolene for the skin

Figure 20.2Q: One week post surgery, there is minimal lid edema and the wound is well apposed. Histopathological examination revealed it was a degenerated parasitic cyst with chronic inflammation and calcification. The child had an uneventful recovery thereafter

Orbitotomies 295

Case 2 (Modified Stallard Wright orbitotomy) A 30-year-old female presented with gradually increasing protrusion of the left eye for one year associated with diplopia followed by progressive loss of vision, watering, redness and pain in the eye. On clinical examination, she had severe non-axial proptosis measuring 12 mm with lateral displacement of the globe (Figure 20.3A). There was protrusion of orbital fat through the orbital septum. The mass was palpable and seemed to encompass the globe. It was firm in consistency, with mild tenderness. There was severe restriction of eye movements, lagophthalmos and exposure keratopathy. Fundus examination showed a pale optic disc with blurred margins and

Figure 20.3A: A 30-year-old lady presented with progressive proptosis of the left eye with downward and lateral displacement of the globe

Figure 20.3C: CT scan (axial section) showed the ill defined mass occupying most of the left orbit (arrow)

collateral vessels on the disc. CT scan showed an irregular intraconal mass; optic nerve could not be seen separately (Figures 20.3B and C). The mass showed specks of intralesional calcification and was causing distortion of the globe and medial orbital wall indicative of a large optic nerve sheath meningioma. Incision biopsy was done through a subbrow incision which confirmed the diagnosis of meningioma. As there was no optic canal or intracranial extension, it was decided that debulking of the mass would restore the eye to its natural position although the eye was blind. Debulking was subsequently done through a lateral orbitotomy approach (modified Stallard-wright incision).

Steps of Surgery

Figure 20.3B: CT scan (coronal section) showed a large, irregular, hyperdense mass behind the left globe

Figure 20.3D: A mark is made on the skin for the S shaped incision

296 Surgical Atlas of Orbital Diseases

Figure 20.3E: The skin incision is made with a no 11 Bard Parker blade. The incision starts from just below the lateral aspect of the brow and ends in a rhytid over the anterior zygomatic arch

Figure 20.3F: Under stretch and lifting the tissues, the subcutaneous tissue is dissected down to the periosteum

Figure 20.3G: The periosteum is cut with a monopolar probe about 4 mm behind the orbital rim starting superiorly and ending inferiorly just above the zygomatic arch

Figure 20.3H: The periosteum is then reflected. The periorbita is also lifted away from the orbital bone upto the anterior one third of the orbit

Figure 20.3I: The temporalis muscle is separated from the underlying bone using a bipolar cautery

Orbitotomies 297

Figure 20.3J: Incision lines are made on the bone about 3 mm above the frontozygomatic suture superiorly and just above the zygomatic arch inferiorly. A Desmarre's retractor is placed to pull the skin and subcutaneous tissue laterally and a lid guard is placed inside the orbit to protect the contents of the orbit. Using an oscillating saw, cuts are made along the incision lines on the bone. Irrigation is performed as the saw is being used

L

Figure 20.3K: Once the cut have been made, the bone fragment is held with a bone rongeur and moved back and forth until it fractures posteriorly

M Figures 20.3L and M: T shaped incision is made in the periorbita with a blunt tipped Westcott scissors

Figure 20.3N: The cut edges of the periorbita are grasped and gently spread apart to extend the cut posteriorly

Figure 20.3O: Retractors are used to keep the orbital fat away and dissection of the mass is performed by blunt dissection

298 Surgical Atlas of Orbital Diseases

Figure 20.3Q: The periorbita is closed with interrupted 6/0 vicryl sutures

Figure 20.3P: A reddish friable firm mass was removed from the orbit

Figure 20.3R: The subcutaneous tissue is apposed with 6/0 vicryl sutures

REFERENCES 1. Wright JE et al. Continuous monitoring of the visual evoked response during intraorbital surgery. Trans Ophthalmol Soc UK. 1973;93:311. 2. Putterman A, Goldberg MF Retinal cryoprobe in orbital tumour management. Am J Ophthalmol 1975;80:88. 3. Leone CR Surgical approaches to the orbit. Ophthalmology 1979;86:930. 4. Benedict WL Surgical treatment of tumours and cysts of the orbit. Eleventh de Schweinitz lecture. Am J Ophthalmol 1949;32:763-73. 5. Smith B The anterior surgical approach to orbital tumours. Trans Am Acad Ophthalmol Otolaryngol 1966;70:607-11. 6. Mc Cord CD Jr Orbital decompression for Graves' disease. Exposure through lateral canthal and inferior fornix incision. Ophthalmology 1981;88:533-41.

Figure 20.3S: The skin is closed with continuous 6/0 prolene suture. The patient was doing well at last follow-up

7. Berke RN Modified Kronlein operation. AMA Arch Ophthalmol 1954;51:609-32. 8. Wright JE Surgical exploration of the orbit. Trans Ophthalmol Soc UK 1979;99:238-40. 9. Simonton JT, Garber PF, Ahl N Margins of safety in lateral orbitotomy. Arch Ophthalmol 1977;95:1229-31. 10. Maroon JC, Kennerdell JS. Microsurgical approach to orbital tumours. Clin Neurosurg 1979;26:479-89. 11. Shucart W Transfrontal approach to the orbit. In Hornblass A(ed): Tumours of the Ocular Adnexa and Orbit St Louis, CV Mosby, 1979. 12. Love JG, Benedict WL Transcranial removal of intraorbital tumours. JAMA 1945;121:777-84. 13. Schurmann K, Oppel O. Transfrontal orbitotomy as a method of operation in retrobulbar tumours. Klin Monatsbl Augenheikld 1961;139:130-59. 14. Long JC, Ellis PP Total unilateral visual loss following orbital surgery. Am J Ophthalmol 1971;71:218-20.

21

CHAPTER

Multidisciplinary Approach to Proptosis Subrahmanyam Mallajosyula, B Ranganadha Reddy, M Chandrasekhar Reddy

Introduction The orbit is located between the facial structures, paranasal sinuses and the skull base. Some of the bony walls that separate the orbit and the paranasal sinuses are very thin. Orbit is in direct communication with brain through the optic canal. Various pathological lesions extend from these surrounding structures in to the orbit and vice versa. Rarely the lesion can involve the sinuses, orbit and the brain, like sino-orbito-cranial mucormycosis. Thus orbit is an area of interest for several other surgical specialties like ENT specialist and neurosurgeon. Ophthalmic surgeons can deal with orbit by a number of direct orbital approaches. ENT surgeons can gain access to pathological conditions arising with in the air sinuses through percutaneous approaches, oral cavity or endonasal endoscopic approaches. Neurosurgeons can access to those tumors that invade both the intracranial and orbital space. A detailed discussion of these lesions is beyond the scope of this book. However we review the various ENT and neurological lesions that may present with proptosis and outline various surgical approaches towards managing these lesions.

Surgical Anatomy The orbital cavity is a 30 ml pear shaped; four walled structure. The central axis of the orbit and the visual axis of the globe are separated by 23 degrees. The medial wall is formed by the lacrimal and ethmoidal bones along with body of the sphenoid bone.

Lateral wall of the orbit is formed by zygomatic bone and greater wing of sphenoid, floor is formed zygomatic, maxillary and palatine bones and roof is formed by horizontal portion of the frontal bone and by the lesser wing of the sphenoid bone. The optic canal: A tubular cavity lying in the deepest portion of the orbit enters the cranial cavity medial to the anterior clinoid process. The optic canal measures an average 5 to 10 mm long, 4.5 mm wide and 5 mm in height. The thickness of the medial wall of the optic canal is an important surgical consideration in the transethmoidal and transsphenoidal approaches to the canal. In about 12% of cases the medial wall of the optic canal is bordered not by the sphenoid sinus but by a posterior or superior ethmoidal air cell. The inclination angle of the optic canal relative to its surroundings is practically important concern. For the superior orbital fissure, through which the intracranial duramater joins the periorbita, medial margin is formed by the lesser wing of the sphenoid and greater wing of sphenoid forms the lateral margin. Optic nerve has a flattened longitudinal shape, measure approximately 4 × 6 mm. As it enters the cranial end of the optic canal, it is circular and 5 mm in diameter, and continues to the globe as a 6 × 4 mm vertically oval structure. The intracranial pia and arachnoid accompanies the nerve from the chiasm and both fuse at the globe. There are loose trabeculations in the subarachnoid space.

300 Surgical Atlas of Orbital Diseases The annulus of Zinn forms the tendinous insertion of the extraocular muscle cone at the apex. It spans the superior orbital fissure creating an intraconal and extraconal compartment of the fissure, dissecting the structures that run through it.

ENT APPROACH TO PROPTOSIS The paraorbital region represents the paranasal air sinuses surrounding the orbit. Tumors of the orbit and paraorbital region sometimes distort the natural architecture of the eye. Although proptosis may seem to be primarily the concern of the ophthalmologist, because of the close proximity of the orbit and para nasal sinuses and various connecting fissures and foramina between the two, many ENT lesions may present with proptosis.

Various Etiological Factors of Proptosis in ENT There are various etiological factors of interest to the otolaryngologists, which can cause proptosis. Diseases can be classified as follows: a. Diseases of the orbit caused by the inflammation in Paranasal sinuses. b. Tumors of the orbito-sinual-region. c. Diseases of the lacrimal apparatus secondary to sinonasal diseases.

a. Infection and inflammation A variety of inflammatory and infective sinonasal conditions may impinge on the orbit; the commonest of which are as follows: 1. Acute purulent sinusitis 2. Gross polyposis-particularly when it begins at an early age 3. Fungal infections 4. Mucocele-commonest is frontoethmoidal mucocele.

b. Tumors of the orbito–sinual disease The Paranasal sinuses tumors are classified into benign and malignant.

Benign Paranasal sinus tumor A. Epithelial tumors: 1. Papilloma 2. Inverting papilloma 3. Tumors of minor salivary glands

B. Angiomatous tumors: 1. Juvenile nasopharyngeal angiofibroma 2. Hemangiopericytoma 3. Lymphangioma C. Mesenchymal tumors: 1. Fibrous dysplasia 2. Osteomas D. Odontogenic tumors: Ameloblastoma E. Neurogenic tumors 1. Schwannoma 2. Neurofibroma

Malignant paranasal sinus tumors A. Epithelial tumors 1. Squamous cell carcinoma 2. Adenoid cystic carcinoma 3. Esthesioneuroblastoma 4. Malignant melanoma of sinonasal tract B. Lymphoreticular tumors: 1. Lymphoma–Non-Hodgkin's 2. Extramedullary plasmacytoma C. Mesenchymal tumors: 1. Osteogenicsarcoma 2. Rhabdomyosarcoma 3. Fibrosarcoma 4. Chondrosarcoma

c. Diseases of the lacrimal apparatus 1. Chronic dacryocystitis 2. Tumors of the lacrimal apparatus

Clinical Manifestations and Evaluation The most common problems the patients complained are a. Proptosis b. Nasal obstruction c. Epistaxis d. Reduced vision e. Facial swelling f. Nasal discharge g. Redness of eye h. Diplopia After thorough ENT clinical examination the patients presented with following manifestations.

Multidisciplinary Approach to Proptosis 301 • • • • • • • • •

anosmia, epistaxis and eccentric proptosis. Diagnosis is by CT scan of paranasal sinuses. Medical treatment includes nasal steroidal sprays. Surgical treatment includes intranasal polpectomy, intranasal ethmoidectomy, external ethmoidectomy and FESS.

Proptosis Nasal mass Restricted eye movements Septal deviations Facial swelling Reduced vision Naso-pharyngeal mass Congestion of the eye Nasal discharge

Mucormycosis

A. Sinus Diseases Causing Proptosis Purulent infections Most of the bacterial infections in the orbit are caused by the spread through infections of the paranasal sinuses (Figures 21.1A and C). Spread of infection to the orbit is through valve less veins, and direct spread through lamina papyracea. The most common organisms involved in this disease are Streptococcus pneumoniae, H. influenza, Beta-hemolytic Streptococci, Staph. Aureus. Ethmoid sinus is most commonly involved.

Treatment High dose, intravenous broad spectrum antibiotics for 2 weeks along with nasal decongestants. Surgical decompression and drainage if necessary.

Extensive nasal polyposis Ethmoidal polyps can be seen in individuals having history of allergy. This condition is most common in Indians especially males. Patient presents with bilateral nasal obstruction, nasal discharge, hypo/

A

B

This is a fulminant opportunistic infection caused by saprophytic fungi of the order mucorales, commonly seen in immunocompromised patients (Figures 21.2A and B). It occurs as rhinocerebral, pulmonary, ocular, superficial and disseminated forms.Rhinocerebral is again subdivided into rhinomaxillary and rhinoorbitocerebral form. It is characterized by bloody nasal discharge, facial swelling, proptosis, altered mental status, palatal or gingival necrosis, facial nerve palsy. Dry black crust is seen in the inferior turbinate, septum and palate. In advanced cases brain and major vascular structures in the head can be involved. This condition is diagnosed by CT scan and frozen sections. This is treated by controlling the underlying predisposing factors, immediate debridement of all devitalized tissues and IV amphotericin.

Allergic fungal sinusitis This condition occurs when an atopic individual is exposed to inhaled fungi. The fungi deposited in the sinus cavity initiate the immunological reactions, causing mucosal edema, stasis of the secretions, and inflammatory exudates blocking the sinus ostia. This process may expand to involve adjacent sinuses and may produce sinus expansion and bony erosion. Secondary bacterial infection can supervene.

C

Figures 21.1A to C: Male 42 years, presented with acute, painful proptosis of left eye of 4 days duration. Note the severe edema, of lids and periorbital swelling with purulent discharge (A) CT scan of orbit axial views (B and C) show severe inflammation of orbit associated with infected ethmoidal and sphenoidal) sinusitis

302 Surgical Atlas of Orbital Diseases The patient presents with nasal congestion, rhinorrhea, headache, epistaxis, and eccentric proptosis. This condition can be diagnosed by raised serum IgE levels, PAS stain, KOH mounting, CT scans of PNS. Treatment includes steroid therapy, surgical debridement and creating permanent drainage.

Frontoethmoidal mucoceles It is an epithelial lined, mucus containing sac filling the sinus and capable of expansion. Frontal and ethmoidal sinuses are most commonly affected. Secondary bacterial infection may change these to pyoceles. These are formed due to obstruction of the affected sinus and inflammation. Patient presents

with proptosis, headache, and facial pain. This condition is diagnosed by CT scans. This condition can be treated surgically by external ethmoidectomy/ FESS.

B. Tumors of Paranasal Sinuses Causing Proptosis • Benign tumors • Inverted papilloma Lateral wall of the nose is the most common site. There is proliferation of the covering epithelium and extensive finger like inversions into the underlying stroma of the epithelium. Patient presents with nasal obstruction, epistaxis, and proptosis. This condition is premalignant. This condition can be diagnosed by CT scan. Treatment of choice is surgery either by lateral rhinotomy and medial maxillectomy or mid facial degloving.

Fibrous dysplasia

A

B

Figures 21.2A and B: A case of mucormycosis with proptosis and severe ptosis of right eye. MRI (B) shows the involvement of maxillary, ethmoid and frontal sinuses. Note intracranial and orbital extensions

A

B

Figures 21.3A and B: A case of allergic fungal sinusitis with involvement of the anterior and posterior ethmoid sinuses and a huge orbital extension (B) which is causing a severe eccentric proptosis of the right eye (A)

Fibrous dysplasia is a skeletal developmental anomaly of the bone-forming mesenchyme that manifests as a defect in osteoblastic differentiation and maturation. The following 4 disease patterns are recognized: • Monostotic form • Polyostotic form • Craniofacial form • Cherubism Monostotic form is the most common type(70-80%). Sites of involvement most commonly include the frontal, sphenoid, maxillary, and ethmoidal bones. Hypertelorism, cranial asymmetry, facial deformity, and proptosis may occur because of involvement of orbital and periorbital bones. However visual impairment, leading to blindness is rare. Involvement of the sphenoid wing and temporal bones may result in vestibular dysfunction, tinnitus, and hearing loss. When the cribriform plate is involved, hyposmia or anosmia may result. Diagnosis is by X-rays and CT scans. Treatment is by local excision.

Hemangiopericytoma A

B

Figures 21.4A and B: Eccentric proptosis of left eye (A) with the globe pushed down and out. Note the infected ethmoid sinus (B)

It is of vascular origin arising from Zimmermann pericyte cell. Patient presents with nasal obstruction, epistaxis, and proptosis. Distant metastasis is common to lungs, liver and bone. Diagnosis is

Multidisciplinary Approach to Proptosis 303 confirmed by histopathological examination. Treatment is by radiotherapy followed by wide local excision.

Juvenile nasopharyngeal angiofibroma It is a highly vascular, benign but biologically aggressive tumor originating in the nasopharynx exclusively seen in adolescent males. This tumor has both vascular and fibrous elements. Patient presents with nasal obstruction, epistaxis, facial deformity, proptosis, and headache. Biopsy is contraindicated. Investigations required are CT scan with contrast, and MRI angiogram. Preoperative embolization is useful. Treatment includes lateral rhinotomy with medial maxillectomy, transpalatine with or without trans- antral approach, mid facial degloving.

Malignant Tumors Squamous cell carcinoma Maxillary sinus is the most commonly involved. It is seen in elderly males. Patients present with nasal obstruction, mass in the nasal cavity, epistaxis, facial swelling, altered facial sensation. If mass involves ethmoids then patient presents with proptosis,

A

B

Figures 21.5A and B: Eccentric proptosis of left eye (A) due to angiofibroma. CT scan shows the tumor involving the sinus and the nasal cavity (B)

A

B

swelling in the bridge of nose, CSF rhinorrhea, signs of raised intracranial involvement. Diagnosis is by biopsy, and imaging with CT scan and MRI. Treatment includes surgery, radiotherapy and chemoradiotherapy. Surgery includes lateral rhinotomy and medial maxillectomy, partial maxillectomy, total and extended maxillectomy and craniofacial dislocation.

Adenoid cystic carcinoma These arise from minor salivary glands of the nose. These grow very aggressively and have perivascular and perineural spread. Patients die of distant metastasis. Diagnosis is by biopsy. Treatment includes surgical resection with postoperative radiation.

Rhabdomyosarcoma Most common soft tissue tumor of childhood. Patient presents with proptosis, visual impairment, painless facial swelling, nasal obstruction, and epistaxis. Diagnosis is by biopsy, CT scan, bone marrow examination. Treatment includes radical surgery, radiotherapy and chemotherapy.

Non-Hodgkin's lymphoma These are commonly seen in elderly males. B-cell lymphomas are much more common than T-cell lymphomas. The maxillary and ethmoid sinuses are most commonly involved. Patient presents with nasal obstruction, epistaxis, proptosis, infraorbital anesthesia, facial swelling. Histopathological evaluation, including immunohistochemistry of biopsy specimen is diagnostic. If it is plasmacytoma then serum electrophoresis should be done. Treatment includes surgical resection, radiation and chemotherapy.

C

Figures 21.6A to C: Note the pinkish tumor in the right nostril. CT scan shows a hyperdense lesion filling the entire ethmoidal sinuses and extending into the right orbit (B and C)

304 Surgical Atlas of Orbital Diseases

Esthesioneuroblastoma Also known as olfactory neuroblastoma or neuroendocrine tumor, this arises in the upper part of nasal cavity from stem cells of neural crest origin. Patient presents with mass in the nasal cavity, epistaxis, proptosis. It is a slow growing tumor which may become large and destructive. Biopsy and using specific tumor markers is the diagnostic tool. Treatment includes surgical resection and radiation.

Various approaches for tumor removal • Caldwell-luc operation • Lateral rhinotomy • Trans palatal approach to remove postnasal tumors • Intranasal ethmoidectomy • External ethmoidectomy: Lynch-Howarth procedure • Patterson's operation • Maxillectomy: — Medial — Partial — Total — Extended • Craniofacial approach • Functional endoscopic sinus surgery

Caldwell-Luc operation Incision extends from canine ridge, runs laterally for 3.5-4 cm parallel to the teeth. The soft tissues are incised down to bone. Using a periosteum elevator, the bony anterior wall of the antrum is exposed. Using a 5 mm Jenkin's gouge, a sliver of anterior wall is elevated and removed with forceps. The bony opening is enlarged with Hajek's sphenoidal punch forceps.

A

The antral mucosa is incised and the contents are examined. After surgery the cavity is packed with BIPP.

Jansen-Horgan operation After performing the Caldwell-Luc operation the posterior ethmoidal cells are opened through the antrum. This is achieved by pushing a closed Tilley-Henckle forceps in an upward, medial and posterior direction at the upper and inner angle of the antrum. The opening is enlarged with suitable punch forceps to allow adequate exenteration of the posterior and middle ethmoid cells. Great care is necessary in enlarging the opening as it may damage cribriform plate or optic nerve.

Intranasal ethmoidectomy Under general anesthesia the polypus bearing area of the nose is exposed and the polypoid masses are removed with Henckel's forceps. The remaining pieces of polypoid mucosa are removed with Citelli's upturned forceps, which is also used in uncapping the ethmoidal bulla and for removing every visible trace of edematous mucosa from the air cells.

External Ethmoidectomy Lynch-Howarth's operation Under general anesthesia a slightly curved incision, medial to, and concave towards the medial canthus of the eye is made. The periosteum is elevated to

B Figures 21.7A and B: Caldwell-Luc operation

Figure 21.8: Jansen-Horgan procedure

Multidisciplinary Approach to Proptosis 305

Figure 21.9: Intranasal ethmoidectomy

reveal the nasal process of both the maxilla and frontal bone and medial orbital wall. The lacrimal sac is elevated from its groove and displaced laterally. The thin ethmoidal wall medial to the orbit is penetrated thus exposing the ethmoidal cells.The cells are progressively exenterated.

Figure 21.10: Incisions for ENT approaches. Lynch-Howarth approach (Green), Patterson's approach (Blue) and Lateral Rhinotomy (Brown) incisions

Retraction of the cheek laterally enables the anterior face of the maxilla to be removed as far as the level of the infraorbital foramen.

Patterson's operation An incision of size 1-2 cm is made in the natural crease line about a finger's breadth below the infraobital margin. The orbicularis muscle is exposed and split in the line of the fibers. The periosteum is elevated from the bone. The medial wall of the orbit defines the lateral limit of the surgery. All cells medial to this line are exenterated using the Tilley- Henkel forceps.

A

B

C

D

Lateral rhinotomy/ medial maxillectomy Under general anesthesia an incision is made from a point halfway between the medial canthus and the dorsum of the nose running medially to the nasomaxillary groove upto the nasal ala. Elevation and retraction of the skin and periosteum over the nasal bone and frontonasal process of the maxilla allow removal of these bones. Bone removal can be extended to include both lacrimal bone and the lamina papyracea of the ethmoid. By this means the ethmoidal labyrinth can be removed.

Figures 21.11A to D: Steps of surgery Lateral Rhinotomy: A; Incision exposing the lateral wall of the nasal bone. B: Periosteum was incised and reflected, and a small window was made in the nasal bone (white arrow), which was extended (C) to expose the mass lesion (yellow arrow). Note the clear space after excision of the mass (D)

306 Surgical Atlas of Orbital Diseases

Total Maxillectomy

an osteotome. The upper part of the maxilla is freed. Then hard palate is separated from the soft palate. Then the medial and lateral pterygoid plates are removed to separate the posterior wall of the maxilla. Then the free maxilla bone is separated from the mucosal fibers and muscle fibers.

A Weber-Fergusson skin incision is marked out starting at the philtrum of the lip and going upto the columella. The incision is continued round the margin of the ala of the nose and up, along the lateral wall of the nose to the medial corner of the eye, turning laterally in a rounded fashion to go 5 mm below the lid margin on the lower lid. The remainder of the incision is intraoral and follows the alveolar buccal sulcus, around the maxillary tuberosity and across the palate at the junction of the soft palate with posterior end of hard palate. The final incision is slightly lateral to the midline to join the original incision in the region of the upper first incisor tooth. Facial skin is elevated. Clearance of the orbit should be done first. If the orbital contents are left in situ they can be elevated. Then ethmoid labyrinth should be exenterated. The bony orbital floor is divided with

A

B

Figures 21.12A and B: The external markings for incision (A), and in the oral cavity for total maxillectomy

A

B

C

D

Figures 21.13A to D: External marking for the incision (A) intraoral incision made and the cheek flap reflected (B) Anterior wall of the maxilla exposed (C), which was opened and the fungal granuloma excised (D)

Multidisciplinary Approach to Proptosis 307

Craniofacial Approach Preliminary temporary tarsorraphies are made to protect the cornea. The classical lateral rhinotomy incision is continued upto forehead either in the midline or in a prominent frown crease. The soft tissues are dissected off the underlying facial bones. The periosteum of frontal bone is carefully elevated and dissected laterally. The window craniotomy is created. Dura is carefully separated. Then the dissection is extended to encompass both ethmoids and the anterior wall of the sphenoid sinus. The specimen is finally freed by dividing the perpendicular plate of the ethmoid. The lamina payracea, anterior wall of the sphenoid and medial antral wall must all be removed.

A

Trans palatal approach to remove postnasal tumors Under general anesthesia, a curved incision bowed forwards is made between the maxillary tuberosities, keeping internal to the greater palatine foramen. If the operation is performed for the removal of the tumor which extends forward into the nasal cavity, the incision should be carried more anteriorly. The incision is deepened through mucosa and periosteum. The mucosa on the upper surface of the palate is divided transversely and postnasal space is examined. Depression of the soft palate with a retractor will give adequate exposure of the postnasal space.

B

C

Figures 21.14A to C: Incision for cranio-facial approach (A), which is indicated when the lesion involves the sinuses, nasal cavity, orbit and the brain as seen in the CT scan images (B and C)

A A

B

B

Figures 21.15A and B: Incisions similar to lateral Rhinotomy, and exposure of the nasal bone

Figures 21.16A and B: After removing the lateral wall of the nose, the part of the tumor in the nasal cavity and ethmoid sinus was excised by the ENT surgeon

308 Surgical Atlas of Orbital Diseases

A

C

B

Figures 21.17A to D: The incision is extended by the neurosurgeon, onto the fore-head and the horizontal part of it is behind the hair-line. The Frontal bone is exposed (A) Bur-holes are made (B) and the frontal bone is removed as a flap(C), exposing the dura covering the brain. The frontal sinus was exposed (D)

D

A

Figures 21.18A to D: Note the frontal sinus component (yellow arrow) and the intracranial component (white arrow) of the mass (A) The entire mass could be visualized (green arrow) after removing the overlying bone (B). The mass was excised. The incision was closed in layers with interrupted sutures (C). The preoperative condition with eccentric proptosis is seen in (D)

C

B

D

Multidisciplinary Approach to Proptosis 309

Neurosurgical Approach to Proptosis The first known craniotomy for an intraorbital tumor producing proptosis was performed by Durante in Rome in 1887. The choice of various approaches to the orbit requires that the surgeon has a perfect knowledge of the orbital pathology and a thorough knowledge of the diverse surgical approaches as well as their advantages, indications, limitations and contraindications. Various surgical approaches to the orbit may be divided into two groups: Extracranial approach and transcranial approach.

Extracranial approach Lateral orbitotomy Figure 21.19: Incisions for transpalatal approach

Anterior orbitotomy

A

B

Figures 21.20A and B: Bony ostium is made in the medial wall of orbit and fat is being removed (white arrow A). Note how big a bony ostium can be made in the medial wall of the orbit for decompression of orbit in thyroid associated orbitopathy

Functional endoscopic sinus surgery( FESS) During the past decade and half, FESS has become more popular and is increasingly used to treat lesions of the sinuses with orbital extension, and for orbital decompression surgeries as in Thyroid Associated Orbitopathy. It can also be very safely used for biopsy, debulking or excising the mass lesion. The procedure is safe, gives a very good access to all the sinuses without a surgical scar. Removal of disease and relevant contact areas while conserving normal mucosa without exposing bony margins and preserving normal anatomy to the extent possible is the advantage, with restoration of ventilation and restoration of mucociliary transport.

Transethamoidal orbitotomy Frontal trans-sinusoidal Inferior orbitotomy Transcranial approaches Sub frontal Frontolateral Frontoorbital Frontotemporo-orbital Pterional

Kronlein, 1889 Rowbotham, 1949 Krayenbuhl, 1967 Stalard, 1947 Berke, 1953 Brihaye, 1968 Kennerdell & Maroon, 1976 Knapp, 1847 Benedict, 1949 Niho, 1961 Colohan, 1941 Davis, 1940 Dandy, 1941 Housepain, 1969 Naffziger, 1948 Tym, 1961 Birhaye, 1968 Jane, 1982 Kennerdell & Maroon, 1976 Hamby, 1982

Because of its outline, the orbit has five sides, each of which may be used to reach the orbital contents. 1. The anterior surgical approach, anterior orbitotomy can be performed through the eyelids or the conjunctiva. 2. The orbitonasal route approach is termed transethmoidal orbitotomy. 3. Transmaxillary orbitotomy through the inferior orbital wall. 4. Temporal orbitotomy or external lateral approach through the temporal fossa. 5. Transcranial or transcranial orbitotomy through the superior wall.

310 Surgical Atlas of Orbital Diseases The temporal and superior or transfrontal approaches are of surgical interest in neurosurgery. Transcranial approach to the orbit is the most complex and demanding route.

Indications • Cranio-orbital disease • Sphenoid wing or anterior fossa meningiomas with orbital extension • Fronto-orbital fibrous dysplasia • Chondroma • Chondrosarcoma • Epidermoid cyst • Osteoma • Aneurysmal bone cyst • Primary intraorbital tumors-those arising in the orbital apex or in the superointernal quadrant of the orbit. • Optic nerve sheath meningiomas • Optic nerve glioma • Neurinoma • Intraconal cavernous hemangioma • Inflammatory pseudotumors • Perioptic metastases • Vascular malformations • Lymphangioma In addition, two wall transcranial decompression and extended pterional orbital decompression has been tried in the management of graves ophthalmopathy.

Supraorbital Craniotomy Under general anesthesia Supine position with head slightly extended to allow separation of the frontal lobe from the orbital roof by the gravity. The sagittal axis of the skull is rotated 10° toward the contralateral side.

Incision-hemicoronal or bicoronal skin incision A cutaneous flap is raised and dissected from the pericranium and from the temporal muscle aponeurosis, including the superior branch of the facial nerve in the folded planes. The most anterior portion of the temporal muscle is dissected and the burr hole is made in the temporal

fossa behind the zygomatic process of the frontal bone, exposing the dura of the anterior fossa in its upper portion and the periorbita in its lower portion both separated by the orbital roof. A second opening is made immediately above the orbital rim in the superointernal angle outside the glabella. Standard precautions are required because of presence of frontal sinus. Both openings may be connected with the craniotome, otherwise, third frontal burr hole is made equidistant from the first two and approximately 4 to 5 cm from the orbital roof. From the lateral burr hole a Gigli saw is passed toward the lateral rim which is then sectioned. From this same opening the duramater and the periorbita are protected with small spatulas the orbital roof is sectioned with the chisel directed toward the midline. From the medial frontal opening the superior orbital rim and the orbital roof was sectioned with the chisel directed laterally and posteriorly toward the osteotomy previously performed. The frontal bone flap is then easily raised together with the superior orbital rim and the anterior and wider sector of the orbital roof, exposing the frontal duramater and periorbita. It is necessary to complete bone resection of the orbital roof and the upper margin of the optic canal in apical tumors. The surgical microscope and self retaining retractors are placed with only minimal frontal displacement. An anteroposterior incision is made medially to the levator palpebraris muscle in the periorbita. Next, the inner and superior rectus muscles are explored by microsurgical technique until the optic nerve is located. This method carries the least risk of causing irreversible ophthalmoplegia. In optic nerve gliomas, the nerve is often extremely thickened in its distended meningeal coverings, showing regular contours. In these cases the lesion is dissected laterally, the anterior portion from behind the eyeball and the posterior one at the level of the annulus of Zinn, followed by opening of the frontobasal dura to explore the optic nerve along its intracranial course. The nerve is then resected from the prechiasmatic level toward the optic canal, including the foraminal portion. In sheath meningiomas presenting as diffuse thickening the dural sheath may be opened antero posteriorly and the tumor may be dissected jointly

Multidisciplinary Approach to Proptosis 311 with the invaded sheath by following the subarachnoid space. In few cases particularly in lesions located anteriorly sight preservation is achieved provided that careful dissection is carried out and vascularization is spared in the nerve and retina. When the tumoral mass is large and not clearly restricted to the nerve course, exhibits micro nodular infiltration of the muscles at the apex, the eye is most likely amaurotic.Thus it is advisable to resect this type of mass with the nerve. In such cases it is essential to perform intracranial exploration of the optic nerve which may be surrounded by the tumor extending through the optic canal. In neurinomas and cavernous hemangiomas, once the lesion has been located and may be removed in toto because of attachment to critical structures is unusual. In rare cases of hour glass neurinomas extending toward the middle cranial fossa combined intracranial and intraorbital procedure is required.

Transcranial approach with resection of the orbital roof Tumors invading or arising from the orbital roof demand a technique different from the one used for purely intraorbital lesions. Frontal or frontotemporal craniotomy is done according to the lesion extension. When marked hyperostosis is present, the procedure begins with gradual resection of the pathological bone with burrs, drills and gouges. It is often necessary to continue bone resection toward the roof and lateral orbital wall leaving the orbital contents amply exposed. In purely osseous lesions this stage

A

ends with total removal of the lesion.The final step is to perform plastic reconstruction of the resected dura with pericranium.The roof and the lateral wall of the orbit is reconstructed with methylmethacrylate.

Summary The choice of the most suitable surgical approach for orbital tumors is made on the basis of tumor location, size, and apparant site of origin, propagation route and probable histological type. Superior orbitotomy is indicated in frontoethmoidal mucoceles, dermoid cyst and lacrimal gland tumors. Lateral orbitotomy is indicated in cavernous hemangiomas, neurinomas, inflammatory pseudo tumors and metastasis. Inferior orbitotomy is less frequently used approach in rare lesions such as lymphomas and metastasis. Transantral approach is indicated for tumors originating from ethmoid cells and maxillary sinus. Larger lesions require extended approaches for example superolateral and inferolateral according to tumor topography and used for large hydatid cyst or huge cavernous hemangiomas. Transcranial approach is indicated for cranio orbital meningiomas of the sphenoid wing, and with anterior cranial fossa disease with orbital extension. The transcranial route is the only one that allows decompression of the optic canal and intracranial exploration of the optic nerve along its prechiasmatic course.

B

Figures 21.21A and B: Axial proptosis of right eye in a male of 35 years (A) due to Schwannoma. Note that the well defined tumor is partly in the orbit and partly in the brain (arrow) as it is extending through superior orbital fissure

312 Surgical Atlas of Orbital Diseases

A

B

Figures 21.22A and B: After making the transcoronal incision, the burr-holes are made in the frontal bone (A) and the frontal bone is reflected as a flap (B)

A

C

B

D Figures 21.23A to D: The tumor is exposed (A) (yellow arrow), which was excised (B). The incision was closed in layers with interrupted sutures (C and D) shows postoperative recovery

Multidisciplinary Approach to Proptosis 313

CASE ILLUSTRATIONS Case 1 Female 48 years (Figure 21.24A) presented with eccentric proptosis of 2 years duration, and defective vision since 2 months. She gives history of recurrent episodes of sinusitis. On examination, she had eccentric proptosis of left eye with the globe pushed up (6 mm), outwards (7 mm) and forwards by 7 mm.The ocular motility was restricted mildly in all directions. Pupil showed RAPD, and her BCVA was 20/60 and color vision was 6/17. Fullness of left cheek was noted. CT scan of orbit (Figures 21.24B

A

and C) showed a large, hyperdense lesion filling entire maxillary sinus, with orbital extension. Extension in front of maxilla into cheek was found. The optic nerve was pushed by the mass. In view of recurrent sinusitis, involvement of the sinuses, fungal granuloma was the clinical diagnosis. The lesion was debulked through a modified lateral rhinotomy incision, along with my ENT colleague. The orbital component was debulked through the horizontal incision, exposing the floor of the orbit. She received amphotericin-B for the fungal granuloma. Her vision improved to 20/30, and there was no recurrence in the past 4 years.

B

C

Figure 21.24A to C: Note the eccentric proptosis of left eye with fullness of left cheek. CT scan of orbit (B and C) showed a large, hyperdense lesion filling entire maxillary sinus (yellow arrow) with orbital extension (green arrow) and extension in front of maxilla into cheek (red arrow). Note how the optic nerve (white arrow) was pushed by the mass

A

B

C

Figures 21.25A to C: The lesion in front of the maxilla was exposed (white arrow A) and excised through a modified lateral rhinotomy incision. Then the anterior wall of maxilla was opened, the mass was exposed (yellow arrow B) and removed. The orbital component was debulked through the horizontal incision, exposing the floor of the orbit. At the end of the surgery (C) note the concavity in the cheek due to excision of the mass in the cheek

314 Surgical Atlas of Orbital Diseases

Case 2 Female of 16 years presented with eccentric proptosis of left eye since 1 year and blurring of vision since 1 month. She was an agricultural worker. She had an eccentric proptosis of left eye with the eyeball pushed up and out (Figure 21.26A). Ocular motility was restricted in elevation and abduction pupil showed RAPD in the left eye. Her BCVA was 20/40. CT scan (Figures 21.26B and C) showed a mass lesion of the

A

maxillary and ethmoid sinuses, with bony destruction and orbital extension. Since most of the orbital floor was destroyed, we planned for a swinging lower eyelid approach, which gives adequate exposure to the floor of orbit (Figure 21.27A) and the mass was debulked (B). Postoperative recovery was good (C). However there was significant enophthalmos. She improved with antifungal drugs and there was no recurrence during 3 years follow-up.

B

C

Figures 21.26A to C: This female of 16 years presented with eccentric proptosis of left eye since 1 year and blurring of vision since1 month. Left eye was pushed up and out (A). CT scan showed a mass lesion of the maxillary and ethmoid sinuses, with bony destruction and orbital extension (B and C)

A

B

C

Figures 21.27A to C: Swinging lower eyelid approach, which gives adequate exposure to the floor of orbit (roof of maxilla A) and the mass was debulked (B). Postoperative recovery was good. Note the minimal scar (C)

Case 3 A 22 years male presented with complaints of left sided nasal obstruction since 5 months, bleeding from the left nasal cavity since 4 months, swelling over the left cheek since 3 months and protrusion of the left eye since 3 months. On anterior rhinoscopic examination a pinkish mass was seen in the left nasal cavity extending into the posterior choana which was observed in the posterior rhinoscopy. There was a

proptosis of the left eye (Figure 21.28A). Ocular movements and vision were normal. CT scan showed a space occupying lesion arising in the left maxillary sinus which is extending into the nasal fossa causing displacement of inferior and medial walls of the orbit s/o neoplasm (Figure 21.28B). The mass was excised through the Moure's lateral rhinotomy approach. Histopathology showed it to be nasopharyngeal angiofibroma (Figure 21.28C and D).

Multidisciplinary Approach to Proptosis 315

C A

B

Figures 21.28A and B: Eccentric proptosis of left eye with the globe pushed up. Note the fullness below the lower eyelid (A) CT scan (B) shows a hyperdense mass with contrast enhancement, involving the maxillary sinus, extending into the nasal cavity. Note the bony expansion of the sinus, leading to proptosis

Case 4 Boy of 2 years was brought with complaints of right sided nasal obstruction since 2 months, protrusion of the right eye since 1 month and watering of the right eye since 1 month (Figure 21.29A). On anterior rhinoscopic examination a grayish mass was seen in the right nasal cavity. Right eye was proptosed. Ocular movements were normal. CT scan (Figure 21.29B) revealed a well defined, non enhancing lesion in the right anterior ethmoidal air cells with central hyperdense component causing expansion of adjacent

A

B

Figures 21.29A and B: Eccentric proptosis of right eye (A) with the globe pushed laterally and down. Note the fullness of superior sulcus. CT scan (B) shows a large, heterogenous lesion involving the ethmoid sinuses, with bony expansion

Figures 21.28C and D: Histopathology showing a picture of vascular spaces with less stroma of fibrous tissue (C) suggestive of Angiofibroma (D) shows postoperative picture with recovery from proptosis

bony structures and proptosis of the right eye. The mass was excised through medial orbitotomy approach and the mass was sent for HPE which showed infected granuloma (Figures 21.30A and B).

Case 5 Male 15 years, presented with complaints of left sided nasal obstruction since 5 months and protrusion of the left eye since 3 months (Figure 21.31A). On anterior rhinoscopic examination a grayish white mass was seen in the left nasal cavity. There was eccentric proptosis of the left eye. Ocular movements and visual acuity were normal. CT scan showed a large well defined soft tissue lesion seen in the region of the ethmoidal air cells on the left side extending into left nasal cavity and sphenoid sinus. The lesion was surrounded by thick irregular sclerotic bony margins and there was expansion of the bony outlines into the left orbit causing proptosis, suggestive of fibrous dysplasia. In view of his symptoms and cosmetic considerations, the mass was excised through external ethmoidectomy approach. Histopathology confirmed the diagnosis of fibrous dysplasia (Figures 21.32A and B).

A A

B Figures 21.30A and B: Excised mass (A) and the early postoperative recovery (B)

D

B

Figures 21.31A and B: Note the eccentric proptosis with the globe pushed outwards (A) CT scan revealed an expansile lesion of the ethmoid bone (B) with sclerotic margins, heterogenisity and cystic spaces within, suggestive of fibrous dysplasia

316 Surgical Atlas of Orbital Diseases

A

B

Figures 21.32A and B: Microphotography showing well demarcated islands of cartilage with interwoven fibro-osseous tissue, suggestive of fibrous dysplasia (A) The early postoperative recovery with improvement in proptosis is evident (B)

Case 6 Female 34 years, presented with proptosis of right eye of 6 months duration, and defective vision since 1 month. She had axial proptosis of 5 mm and

downward displacement by 4 mm (Figure 21.33A). RAPD was observed. Her BCVA was 20/30 in right eye. The past history was significant in that she had convulsions 2 years back, and was using anticonvulsants since then CT scan of the orbit revealed an intraconal, heterogenous, apical mass with very distinct margins (Figures 21.33B and C). The CT scan of brain (Figures 21.34A to C) showed calcified lesions without any surrounding edema in the frontal and occipital cortex. The possibility of hemangioblastoma was considered. Fundus examination was normal. In view of the apical location, a transcranial approach (A) was performed (Figures 21.35A and B). Histopathology confirmed it to be hemangioblastoma. There was no recurrence in the 2 years follow-up.

A

B

C

Figures 21.33A to C: Female 34 years, with proptosis of right eye of 6 months duration, and defective vision since 1 month. She had axial proptosis of 5 mm and downward displacement by 4 mm (A). CT scan of the orbit revealed an intraconal, heterogenous, apical mass with very distinct margins (B and C)

Multidisciplinary Approach to Proptosis 317

A

B

C

Figures 21.34A to C: The CT scan of brain showed calcified lesions without any surrounding edema in the frontal and occipital cortex

A

B

Figures 21.35A and B: In view of the apical location, a transcranial approach (A) was performed. Histopathology confirmed it to be hemangioblastoma. Postoperative figure (B) showing complete recovery

BIBLIOGRAPHY 1. Atallah N (1981) osteomas of the paranasal sinuses, journal of laryngology and otology. 2. Balasingam V, Noguchi A, McMenomey SO, Delashaw JB Jr Modified osteoplastic orbitozygomatic craniotomy. Technical note J Neurosurg. 2005;102(5):940-4. 3. Bordley, JE nad Bosley, mucoceles of the frontal sinus, annals of otology, rhinology and laryngology. 4. Canalis RL, ethmoidal mucocele. Archives of otolaryngology. 5. Cheesman AD Lund, Cranio facial resection for tumors of nasal cavity and paranasal sinuses. 6. Doxanas MT Clinical Orbital Anatomy. 7. Eichel the intranasal ethmoidectomy procedures. 8. Fearon BEdwards orbital- facial complications of sinusitis in children. 9. Freedman HM (1979) complications of intranasal ethmoidectomy. 10. Fu JD, Zhao JW, Yin DL, Liu HC, Qiu E, Zhang JL, Zhang TM. Surgical treatment of fibrous dysplasia of the skull with neuro-navagation .Zhonghua Yi Xue Za Zhi. 2004;17;84(10):808-12. Chinese. 11. Goisis M, Biglioli F, Guareschi M, Frigerio A, Mortini P Fibrous dysplasia of the orbital region: current clinical perspectives in ophthalmology and cranio-maxillofacial surgery. Ophthal Plast Reconstr Surg. 2006;22(5):383-7.

12. Harrison DFN (1971) surgical anatomy of maxillary and ethmoid sinuses. 13. Harrison DFN (1980) The ENT surgeon looks at the orbit, journal of laryngology and otology. 14. Harrison DFN (1981) Surgical approach to the medial orbital wall. Annals of otology,rhinology and laryngology. 15. Harrison DFN (1987) juvenile angiofibroma, archives of otolaryngology-head and neck surgery. 16. Hejazi N, Hassler W, Offner F, Schuster A. Cavernous malformations of the orbit: a distinct entity? A review of own experiences. Neurosurg Rev. 2007;30(1):50-4; discussion 54-5. Epub 2006. 17. Howard and Lund, the midfacial degloving approach to sinonasal disease, journal of laryngology and otology. 18. Howard, DJ Lund reflections on the management of adenoid cystic carcinoma of nasal cavity and paranasal sinuses. Otolaryngology 93. 19. Hubert (1937) orbital infections due to nasal sinusitis, New York journal of medicine37. 20. Korinth MC, Ince A, Banghard W, Gilsbach JM. Follow-up of extended pterional orbital decompression in severe Graves’ ophthalmopathy. Acta Neurochir (Wien). 2002;144(2):113-20; discussion 120. 21. Linnet J, Hegedus L, Bjerre PK. Neurosurgical treatment of patients with severe thyroid-associated ophthalmopathy. Transcranial two-wall orbital decompression Ugeskr Laeger. 2002;6;164(19):2505-8. Danis 22. Lloyd(1988) Diagnostic imaging of nose and paranasal sinuses. 23. Lund, tomors of paranasal cavities, Oto-Rhino-Laryngology 45. 24. Natvig K and Larsen, mucoceles of paranasal sinuses, Journal of Laryngolog and Otology. 25. Reisch R, Perneczky A. Ten-year experience with the supraorbital subfrontal approach through an eyebrow skin incision. Neurosurgery. 2005;57(4 Suppl):242-55; discussion 242-55. 26. Rontal (1979) Surgical anatomy of the orbit, annals of Otology, Rhinology and Laryngology 88. 27. Schramm, Orbital complications of sinusitis, Otolaryngology, 86. 28. Zizmor(1968) cysts and benign tumors of the paranasal sinuses,, seminars in Roentgenology.3

318 Surgical Atlas of Orbital Diseases

22

Orbital Exenteration

CHAPTER Ramesh Murthy, Anirban Bhaduri, Sima Das, Santosh G Honavar

Exenteration refers to removal of the eyeball along with the orbital contents. This disfiguring and destructive procedure is reserved for the treatment of life-threatening conditions where other approaches have failed. The first mention of orbital exenteration was 400 years ago by Bartische in his treatise on eye diseases. 1 He described partial exenteration. Extensive orbital exenteration was described by Golovine, Nowikoff and Filatov in the early 1900s.2

Indications We usually perform this procedure in the following situations. 1. Malignancies a. Primary orbital lesions like extensive adenoid cystic carcinoma of the lacrimal gland b. Intraocular lesions like orbital extension of retinoblastoma, choroidal melanoma c. Orbital extension of periocular malignancies i. Carcinoma of the paranasal sinuses ii. Eyelid tumors iii. Skin malignancies 2. Infections like sino-orbital mucormycosis and other fungal infections. 3. Relative indications a. Severe orbital contracture with inability to wear a prosthesis b. Neurofibromatosis with orbital deformity

c. Orbital meningioma and lymphangioma causing disfiguring proptosis d. Recalcitrant orbital inflammations unresponsive to other treatment modalities

Patient Preparation The patient needs to understand the need for such a destructive surgery and the gross disfigurement caused by the procedure. We also counsel our patients extensively that their facial appearance can never be restored to what it was before. However, an orbital prosthesis can restore appearance to an acceptable extent, although it will never provide eyelid or ocular motility. Psychological help is sometimes sought when we feel that the patient may not be able to withstand the consequences of the surgery.

Surgical Procedure We always perform the procedure under general anesthesia. In the rare situation when this is not possible, local anesthesia can also be used with 2% lignocaine with 1:200,000 adrenaline given as a retrobulbar injection and followed by injections around the orbital rim and in addition with infraorbital, nasociliary and frontal nerve blocks. For every patient we have a pint of blood ready for transfusion if required. In addition hypotensive anesthesia is used for all the cases. We also ensure that the cauteries are working well and have sufficient gauze on table to control bleeding. The skin is marked all around with a methylene blue marker. A 4-0 silk suture is passed through the eyelid skin and orbicularis to exit from the tarsal

Orbital Exenteration 319

Types Type

Contents removed

Contents preserved

Final appearance

Complications

Anterior exenteration/ extended enucleation

Globe, posterior lamella of eyelid, conjunctival sac

Periorbita, posterior orbital contents

Shallow socket, immobile eyelids present

• Delayed healing • Immobile ill fitted prosthesis

Lid sparing exenteration/ Subtotal exenteration

Orbital contents including periosteum of orbital walls Eyelid margins

Anterior lamina of the eyelid including skin and some orbicularis muscle

• Hematoma behind the skin flaps • Necrosis of skin flaps

Total exenteration/ Eyelid sacrificing

Orbital contents, periorbita and lids

Bare orbital bones with or without a skin graft

Deep orbit. Residual skin and orbicularis edges sutured together forming a smooth lining Spectacle mounted prosthesis can be fitted after the healing is complete

Radical exenteration

Dissection involves paranasal sinuses, face, jaw, palate, skull base.

Frontal bone replaced, cavity covered with myocutaneous vascular flap with vascular anastomosis

Cavity can be filled with myocutaneous vascular flaps or a maxillofacial prosthesis can be used to close the palatal defect along with split skin graft.

• Exposure of intracranial contents and associated complications • Poor cosmesis

plate at the lid margin and then passed similarly through the tarsal plate and skin and tied to secure the lids together. This suture is left long to provide traction during the procedure. A lid sparing exenteration is less disfiguring than a total exenteration. In such a case we make the incision a short distance away from the lid margin. If however there is involvement of the lid, the incision is made just inside the orbital rim. The skin incision is made with a 11 no. Bard Parker knife, starting inferiorly and progressing laterally, superiorly and medially. The superonasal quadrant is approached last as it bleeds extensively. If the lid skin is being spared, it is dissected along with orbicularis to reach the periosteum of the orbital rim. Once the lid skin is cut, the underlying tissues can then be cut with a monopolar cautery. The periosteum is cut 6 millimetres or so from the orbital rim and then elevated using a periosteal elevator. The sharp end of the periosteal elevator is used to separate it from the orbital rim and the blunt end for the intraorbital portion. The attachments in the region of the medial and lateral canthal tendon and the trochlea are lifted off from the underlying bone. The neurovascular bundle entering through the infraorbital and supraorbital foramen is usually cauterised before cutting it. We separate the

• • • •

Sino-orbital fistula CSF leak Hematoma formation. Keratinization of the skin graft causing a malodorous socket • Infection

periorbita from the bone superiorly, laterally and inferiorly first using the blunt end of the periosteal elevator or a lens spatula. The periorbita is firmly attached to the bone near the orbital rim and loosely within the bony orbit. Medially the lacrimal sac is elevated and cut near the nasolacrimal duct. Occasionally if there is a suspicion of tumor spread through the nasolacrimal duct we remove bone in that region using the Kerrison's rongeur. In cases of tumors where there is bone involvement, we remove all the involved bone. If such a step is pre-empted, then occasional help from a neurosurgeon for tumors extending superiorly and sometimes our ENT colleague especially for sino-orbital mucormycosis is sought. Once we reach the orbital apex, we are wary of the vessels that might bleed before we finally remove all the contents of the orbit. We clamp the tissues near the apex with a curved artery forceps before cutting the apical stump. For visualization, we use a lid spatula to push the contents to one side. Bleeding can be a problem and we use a strong cautery to stem it. Bone wax is needed for the occasional recalcitrant bleeder. We wait for the blood pressure to return to normal before we close the lid skin (if performing a lid sparing exenteration). We use absorbable 6-0 vicryl for the underlying tissues and

320 Surgical Atlas of Orbital Diseases 6-0 prolene continuous suture for the skin. If the lid skin has been removed, the socket is packed with Betadine soaked gauze. We give the patient systemic antibiotics and antiinflammatory medication and give a pressure patch for 2 days. On the third day following surgery we remove the patch and perform daily aspiration of the contents ( in lid sparing) or cleaning and packing in total exenteration.

Management of the Exenterated Socket It is important to note that the primary objective of exenteration is to remove all disease. Even after exenteration, the patient needs to be monitored carefully clinically or by imaging for any recurrence.

Spontaneous Granulation This has to be performed when the surgical excision has been radical and the lid skin excised. In these cases we leave the remaining skin to line the socket and pack the socket with gauze soaked in Betadine as a pressure pack and for about 2 weeks with regular changing of dressings in the hospital, or by the patient at home. The entire socket is allowed to heal by granulation. Of course the advantage of this is the fact that any recurrent disease can be identified easily. However the healing is a slow process.

Skin Grafting Partial thickness skin grafts have been in vogue for a long time since they were popularized by Wheeler.4 Using a dermatome, skin is harvested from a non hair bearing area such as the inner thigh and abdomen. The graft is then fitted to the exenteration cavity, trimmed and sutured using 6-0 prolene sutures. Slits are made to facilitate drainage and prevent collection of fluid underneath. Healing usually takes 6 weeks, following which the patient is fitted with prosthesis.5

Skin Flaps This is my preferred technique where the lid skin is spared and used to close the cavity. Postsurgery pressure patch is placed and aspiration of the socket is performed daily for a week to remove any collection of fluid. Once the healing is complete, a smooth surface is achieved over which prosthesis can be placed easily.

Myocutaneous Flaps Other techniques like temporalis muscle transposition, forehead, cheek, pectoralis flaps have also been described.6-9

Prosthesis Exenteration prosthesis is our preferred technique of managing the cosmetic issues following exenteration, which is discussed as a separate chapter in the book.

Complications of Exenteration Bleeding can be life threatening especially during the surgery and adequate support from the anesthetic team is a must. In addition blood for transfusion should be available. One should have a good cautery machine. Socket infection can occur and needs to be managed by systemic and local antibiotics. Any gape in the sutured wound should be closed if possible or in the presence of infection should be allowed to granulate spontaneously. Recurrence of the primary disease can occur and hence monitoring at regular intervals postsurgery is essential.

CASE ILLUSTRATIONS Case 1 A 51-year-old female presented with pain and swelling of the left eye for 15 days. She had a history of gradual loss of vision in the left eye for 2 years followed by gradual protrusion of that eye for 1 year. On examination, there was proptosis of the left eye, with swelling of the lids and ptosis (Figure 22.1A). On lifting the ptotic lid, a brown, firm perilimbal nodule was seen next to an opaque cornea (Figure 22.1B). There was complete restriction of eye movements. CT scan of the orbit showed a soft tissue mass filling the orbit almost upto the apex (Figures 22.1C and D). The globe contour was distorted and no normal orbital structures were recognizable. A diagnosis of uveal melanoma with secondary orbital extension was made. There was no lymphadenopathy in the head and neck region. Systemic workup was done which did not reveal any systemic metastasis.

Orbital Exenteration 321 The patient underwent a lid-sparing exenteration. The floor and medial wall bones were deficient because of pressure effect, but the periorbita was

intact. Subsequently, the patient underwent external beam radiotherapy to the left orbit and paranasal sinuses.

Steps of Surgery (Figures 22.1E to Q)

Figure 22.1A: A 51-year-old lady presented with proptosis of the left eye with associated lid swelling and ptosis

C

Figure 22.1B: A black mass was seen to be prolapsing out of the cornea of the left eye suggestive of extraocular spread of melanoma

D Figures 22.1C and D: CT scan of the orbit revealed a diffuse soft tissue mass of variable density filling the entire orbit

322 Surgical Atlas of Orbital Diseases

Figure 22.1E: The skin was marked on both the upper and lower lids to identify the site of incision

Figure 22.1F: A rolled up wet cotton gauze was placed over the conjunctiva. A 4-0 silk suture is passed through the eyelid skin and orbicularis to exit from the tarsal plate at the lid margin and then passed similarly through the tarsal plate and skin and tied to secure the lids together

Figure 22.1G: 3 such sutures are passed and the ends kept long for traction during the procedure

Figure 22.1H: The skin incision is made with a 11 no. Bard Parker knife, starting inferiorly and progressing laterally, superiorly and medially

Figure 22.1I: In a lid sparing exenteration, the skin incision is made a short distance away from the lid margin

Figure 22.1J: The underlying tissues are retracted and cut with a radiofrequency monopolar cautery

Orbital Exenteration 323

Figure 22.1K: The skin and subcutaneous tissues are incised up to the level of the periosteum

Figure 22.1L: The periosteum is cut about 6 mm from the orbital rim and elevated using the periosteal elevator

Figure 22.1M: The periorbita is separated from the bone superiorly, laterally and inferiorly first using the blunt end of the periosteal elevator or a lens spatula. The periorbita is firmly attached to the bone near the orbital rim and loosely within the bony orbit. Medially the lacrimal sac is elevated and cut near the nasolacrimal duct

Figure 22.1N: Near the orbital apex, the tissues are clamped with a curved artery forceps before cutting the apical stump

Figure 22.1O: The exenterated contents are dark brown in color suggestive of a melanoma

Figure 22.1P: 6/0 vicryl sutures are used to suture the subcutaneous tissue once hemostasis has been achieved

324 Surgical Atlas of Orbital Diseases

Figure 22.1Q: The skin is closed with 6/0 prolene

Figure 22.2A: A 40-year-old male presented to us with progressive proptosis of the left eye of 4 months duration

Case 2 A 40-year-old male presented to us with eccentric protrusion (outward and lateral displacement) of the left eye which was gradually increasing for last 4 months (Figure 22.2A). A non-tender mass, firm to hard in consistency was palpable deep in the superior and nasal part of left orbit. There was no apparent nasal or paranasal sinus pathology. On presentation, he had best corrected visual acuity of 6/7.5 and 6/12 in right and left eyes respectively. Ocular movements in the left eye were grossly restricted; adduction more than abduction. Anterior segment examination was unremarkable in both eyes. Fundus in the right eye was normal and in the left eye showed presence of early disc edema. The patient was non-diabetic and non-hypertensive. Ultrasound B scan of the left eye revealed diffuse thickening of medial rectus with maximum diameter being 14.3 mm. CT scan showed presence of a fairly well defined, uniformly isodense, intraconal mass located between medial rectus and optic nerve. Medial rectus could not be appreciated separately from the mass posteriorly (Figures 22.2B and C). An excision biopsy by a lid split medial orbitotomy approach was done. Postoperatively his proptosis was reduced but the vision in left eye was reduced to PL with inaccurate projection of rays, and he had grade 1 relative afferent pupillary defect. Histopathology of the biopsy specimen showed presence of giant cells and elongated budding fungal filaments. Microbiological evaluation confirmed a diagnosis of aspergillosis.

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C Figures 22.2B and C: CT scan revealed the presence of a fairly well defined uniformly isodense intraconal mass between the medial rectus and optic nerve

Orbital Exenteration 325 4 months later the patient came back with exacerbation of proptosis with a firm mass palpable in the medial canthal region (Figure 22.2D). Exophthalmometry readings were 19 and 30 mm in the right and left eye respectively. He had no vision in the left eye at this visit. CT scan showed a solid mass, which was filling almost the entire orbital cavity; extraocular muscles could not be made out separately from the mass (Figures 22.2E and F). The mass could be seen extending up to the superior orbital fissure. Lateral wall of orbit showed excavation of bone. Contiguous middle cranial fossa and paranasal sinuses appeared uninvolved. Lid sparing exenteration was done in view of extensive nature of disease.

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Figure 22.2D: 4 months later the patient came back with exacerbation of proptosis and a firm palpable mass in the medial canthal region

F Figures 22.2E and F: CT scan axial (E) and coronal (F) sections revealed a hyperdense mass filling most of the orbit

Figure 22.2G: Periodic acid schiff (PAS) stain (400×) showed the presence of dark filaments confirming the diagnosis of aspergillus flavus

Figure 22.2H: Gomori’s methanamine silver (GMS) stain showed of dark filaments aspergillus flavus

326 Surgical Atlas of Orbital Diseases The patient was maintained on oral antifungals and was stable with no recurrence till last follow up 3 months postsurgery (Figure 22.2I).

REFERENCES

Figure 22.2I: 3 months postsurgery the skin wound had healed and the patient did not show any signs of recurrence

Histopathology of the exenterated specimen revealed multiple septate branching fungal filaments within the giant cells and stroma. There was no definite evidence of vascular invasion. There was no involvement of the globe. Microbiology confirmed the presence of Aspergillus flavus [Figure 22.2G periodic acid schiff (PAS) stain showing the dark filaments and Figure 22.2H Gomori's methanamine silver (GMS) stain showing the dark filaments against a green background] in the exenterated orbit.

1. Bartische G Ophthalmodouelia, das ist Augendiest. Dresden, Matthes Stockwell, 1583; 217-19. 2. Golovine SS Orbitosinus exenteration. Ann Ocul 1909;141:413-31. 3. Nowikoff V Extirpation of the orbit. Lyon Chir 1927; 26: 17-27. 4. Wheeler JM The use of epidermic graft in plastic eye surgery. Internat Clin 1922; 3:292-300. 5. Shields JA, Shields CL, Demirci H, Honavar SG, Singh AD Experience with eyelid-sparing orbital exenteration: the 2000 Tullos O. Coston Lecture. Ophthal Plast Reconstr Surg 2001;17(5):355-61. 6. Donahue PJ, Liston SL, Falconer DP, Manlove JC. Reconstruction of orbital exenteration cavities. The use of the latissimus dorsi myocutaneous free flap.Arch Ophthalmol. 1989; 107(11):1681-3. 7. Uusitalo M, Ibarra M, Fulton L, Kaplan M, Hoffman W, Lee C, Carter S, O'Brien J. Reconstruction with rectus abdominis myocutaneous free flap after orbital exenteration in children. Arch Ophthalmol. 2001; 119(11):1705-9. 8. Bonavolonta G Frontalis muscle transfer in the reconstruction of the exenterated orbit.Adv Ophthalmic Plast Reconstr Surg. 1992; 9:239-42. 9. Arlyan S, Cuono CB.Use of the pectoralis major myocutaneous flap for reconstruction of large cervical, facial or cranial defects. Am J Surg. 1980;140(4):503-6.

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23

Orbital Prosthesis

CHAPTER Kuldeep Raizada

INTRODUCTION Loss of an eye has a very traumatic effect. It leads to loss of self belief, can make the patient and family very depressed. Eye to eye contact is very important in conversation, which is usually lost if the person has a very disfiguring eye or an empty socket. They seek aesthetic improvement in their cosmetic appearance. Patient's rehabilitation with prosthesis is very challenging, especially when it is a facial prosthesis where a natural looking prosthesis is the final goal. Orbital prosthesis and ocular prosthesis deal with the fabrication of an artificial substitute for different kind of orbital deformities, which can be due to diseases, surgery, trauma or congenital malformation. Several cases of devastating facial injuries, incurred in battle, were treated in the early nineteenth century with indigenous appliances and these reconstructive procedures gave definite push to the field of facial replacement.1 Great strides in the field have been made in the past decades.

Orbital Prosthesis Orbital prosthesis is meant for the face to improve the cosmetic appearance of the individuals and most often it is required in conditions where there is an additional loss of periocular tissues like eyelids, eyelashes and eyebrows.3 While fabricating an orbital prosthesis utmost care should be taken to not only replace lost periocular tissue but also to match them in terms of color and texture to the surrounding tissues and the fellow orbit.

Types of Prosthesis • Orbital Complete – Spectacle mounted prosthesis – Adhesive retained prosthesis – Magnetic retained prosthesis Partial – Adhesive retained prosthesis Complete prosthesis: means prosthesis with not only an ocular prosthesis but also it contains eye lids, eye brow and eye lashes, to restore the normal anatomical appearance to the patient. On the basis is retention it can be further classified.1-4,8 Spectacle mounted prosthesis: is used in conditions where surface remains moist and a silicon prosthesis is not going to stay. A facial prosthesis made up of acrylic can be attached to spectacle, can be an option for such kind of cases. Adhesive retained prosthesis: in conditions where surface is dry and a silicon prosthesis is going to stay. A facial prosthesis made up of medical grade silicon can be attached externally in such cases. Magnetic retained prosthesis: this is fabricated in a very special situation where patient had radical orbital exenteration. The surface is provided with the support of magnetic anchors that helps in retaining the prosthesis. Partial prosthesis: described in literature earlier for the nasal and auricular prosthesis. Recently Raizada et al.5-6 described the method of fabricating the partial orbital prosthesis where at first a custom made ocular

328 Surgical Atlas of Orbital Diseases prosthesis is placed and later on the lower eyelids are designed using the medical grade silicon and then it can be retained with epithane.

Factors that Affect the Fit of an Orbital Prosthesis There are many factors that affect the fit of an orbital prosthesis.It is important to evaluate the orbital defect and later on choosing the modalities of retaining the orbital prosthesis. 1. If the orbit had an incidence of tumors, there should be no recurrence. 2. The surface should be well healed; there should be no edema, or infected external surface if the case is of open defects like in the cases of radical orbital exenteration. 3. If it is a case of orbital trauma and severe deformity like lid coloboma, they may benefit with partial upper or lower lids prosthesis. 4. If the patient had extensive damage to the orbital cavity, a glue based prosthesis will be interfering with patient eyelids and may not be very comfortable. Such cases may be either considered for blepharorrhaphy or else an spectacle mounted orbital prosthesis with vaulted back surface. 5. If the patients have ocular disfigurement in the lower lids due to the extensive trauma and lids cannot be constructed, a partial prosthesis with integrated lower lids can be fabricated. In this chapter I shall be discussing the technique of fabricating orbital prosthesis (a hybrid type as a complex prosthesis is made up of two different materials, so called as hybrid prosthesis). There are many steps involved but the following steps involved fabricating an orbital prosthesis are of more concern: • Preperation of the patient • Impression • Casting • Sculpting • Moulding • Coloring • Using the desired material • Fabrication of ocular prosthesis.

Preparation of the Patient This is a very important step as patient needs to understand what we are going to do. A proper counselling about the whole procedure makes the patient very calm, and make the work very comfortable. I believe that the more the patient understands, the better he co-operates. Explain to the patient the whole procedure using the other patient's pictures or illustrations. You can now ask the patient to lay down on a couch or bed in order to make patient completely relaxed as this is very tiring because the whole procedure takes about 30 to 45 minutes.

Impression of the Orbital Defect Impression is taken while the patient is lying in the supine position. Vaseline is applied on the places of eyebrows, eyelashes as well as in the raw area of the defect if present. There are many ways to take an impression. Many people believe that only defective side impression makes work much easier, but I believe that taking the impression of the whole face gives better pictures, hence forth I prefer to take the impression of both sides of the face so as to obtain accurate information about the defective as well as normal side too. We make use of hydrophilic colloid as the basic material of taking the impression of the orbital cavity. It is not only just an impression material that is needed but you also require the reinforcing materials such as metal clips, gauge piece and the final layer of casting stone. Prior to taking an impression of the defect, it is essential to tell the patient about the type of impression you are going to take as some time the defects are open as in case of radical exenteration, and slight mistakes give really a hard time. By using the base plate wax and adhesives like micropore tape, mark the boundary of defect. Use Vaseline on the area of the eye lashes and eyebrow, so that while taking out, the impression comes out easily. (Figures 23.1A to C) Figure 23.1E : This is a lateral view showing how the base plate wax and micropore adhesive makes a boundary of the defect and make easier to take an

Orbital Prosthesis 329 impression. Use the hydrophilic colloid in the ratio of 1:1 with water and after mixing thoroughly with the flat spatula, pour it first on the defect area and later in the surrounding tissues (Figure 23.1D). Use the reinforcing materials to make the impression so that while putting the second layer of die stone, it gets adhered to the surface and makes a stable impression (Figure 23.1F). As hydrophilic colloid gets set very fast it is recommended that mix the die stone in ratio of 1:1 with water and pour above the hydrophilic colloid material. Meanwhile use the reinforcing material to make the die-stone mechanically strong (Figure 23.1G). We prefer to use even gauze piece over the dye stone (Figure 23.1H), so that it get integrated with dye-stone. In about 10 minutes the die-stone gets set and exothermic reactions starts and we use mild water on the surface so that it remains cool. Once the die-stone is set, ask the patient to squeeze the face and remove the impression which is called as negative impression (Figure 23.1I).

Casting Casting is replicating a negative impression to positive impression, and this will reflect the defect of the patient. Stablize the impression material using the clay or sand box and slowly pour the mixture of dye-stone, step by step. Let it set for its optimal time of about 15 minutes (depends on the type of dye stone used), (Figures 23.1J to L shows the junction line of dye-stone and hydrophilic colloid from where you can separate the negative and positive impression). Remove dye-stone cast at the junction line slowly from the hydrophilic colloid and this will represent the patients deformity (Figures 23.1I and L). You should always compare the impression of the defected area and the patient's actual orbital defect. Once you are sure then only proceed ahead. (Figure 23.2A).

Sculpting Use the tinfoil of 0.01 mm on the defect to make the model easier to take out from the cast and to check

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Figures 23.1A to L: (A) patient with front view and left orbital exenteration (B) base plate wax boundary to hold the impression material (C) front view with base plate wax boundary in place with applied vaseline on the face (D) front view with the placement of hydrophilic colloid and reinforce material (E) lateral view shows the use of adhesive tape on the boundary of base plate wax (F) placements of uniform layer of die-stone (G) placement of tiny reinforce material so that while taking out the impression cast should not break (H) placement of a gauge piece on the same (I) after removal of cast from the cavity called negative impression (J) on the negative impression after placing layers of die-stone (K) once the die-stone is set, you can see the differentiation zone of these two (L) after separation of the two-piece

330 Surgical Atlas of Orbital Diseases it on the patient face (Figure 23.2B). There are various kinds of materials used such as clay, waxes or, direct silicon for sculpting model. We prefer to start the procedure with the base plate wax as it has property of moulding into desired shapes (Figure 23.2C). Now we need to choose an ocular prosthesis for the same anterior curvature of the patient, preferably a flatter one when you have shallow defect of the orbit (Figure 23.2D). By using the Purkinje's images of the fellow eye and measuring the inter pupillary distance (distance from the mid line of face to the center of the fellow eye) to locate the exact corneal reflex and even the use of hurtle exophthalmometer for assessing the exact amount of proptosis helps greatly in fabrication. Once satisfied with the corneal position, I use the thin strip of the base plate wax to sculpt the eyelids. It is preferable to see the patient's face from all angles so that when prosthesis is fabricated, it should meet the criteria of having equal amount of elevation from the base of the orbit (Figures 23.2E to H). I also take the pictures of the patient with the final sculpted model and download in the computer,

using the 'Adobe Photoshop 6.0' as graphical visualization makes it much easier to correct further the lids alignment, and to create a better symmetry (Figure 23.2I). Once I am done with these all steps I again go back and check the sculpted model and compare to the patient's defect, use the desire spectacle frame and cut in that fashion (Figure 23.2J).

Moulding Making a two-piece mould is not a very complicated job, but of course making a mould is an art. Once the wax model is finished, apply the vaseline on the rear side of the prosthesis in order to get a smooth moulded surface. Mix die-stone in a ratio of 1: 1 with water. Use the vibrating unit to remove all the air bubbles and pour the mixture of die-stone and water in to a metal flask, apply some of the mixture on the back side of the model in case of any undercuts and then invest this model in the metal flask. Look very carefully as some of the dye-stone may be fast setting because before it sets you need to remove the excess material from the mould surface and check if any undercuts are there, as presence of undercuts in the

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Figures 23.2A to O: (A) positive case from the impression of the face (B) use of tin foil so that model can be taken out with out distortion (C) Use of the base plate wax a base on the top of the thin tin foil (D) placement of an ocular prosthesis, front view looking at the Purkenje's images (E) eye lids after attaching the lump of wax and carved with the metal spatula (F) lateral view of the same patient on the normal side (G) lateral view of the same patient on the affected side (H) placement of final wax model on the face (I) use of computer programme Adobe Photoshop to make the grid and analyse further, in order to get a better symmetry (J) final wax model placed on the positive cast of the impression (K) invest of the wax model in the metal flask using the die-stone (L) once the die-stone is set, apply thin layer of "Cold Mould Seal" so that mould can be open (M) once the thin layer of the "Cold Mould Seal" is dried second layer of die-stone is also poured in the metal flask (N) open the mould, see that how beautifully the mould opens up (O) do remove the all wax from mould using the hot water and soap

Orbital Prosthesis 331 mould will finally affect the quality of prosthesis. Once the stone is set, apply the separating media (from DPI) and pour the other in the same fashion. Once the mould is set, open it very carefully and remove the wax model from the mould, clean it with the hot water and reapply the separating media (Figures 23.2K to 23.2O). Now your mould is ready for pouring the desired material.

Using the Desired Material Attach the prosthesis to the stone moulds in its original position. Using the cynoacrylate, fix the prosthesis into its curvature so that it remains in same position as done while sculpting (Figures 23.3A to C). Till this step everything is common, and now you have to decide which type of prosthesis you are looking for. Here I describe the making of silicon prosthesis. I mix MDX4210 with Dow corning silicon in 1:1 along with combinations of artist colors and dyes, try to match the shades of the patient skin and add more flocking so that it give much better skin appearance. I usually prefer intrinsic coloring MDX4210. As it gets set, pour into the moulds and take out all the air bubbles. Now close it from back to front, so that in case any air bubble remains, it will come on the back of the prosthesis and can be later taken care by doing the patch work (Figures 23.3D to F). Cure the silicon at room temperature under high pressure. Once the silicon is cured, open the moulds and you have the prosthesis ready in your hand. Trim the extra margins using the 3M (Factor II) trimming wheels in a tapering fashion, so that it

mingles well with the surrounding tissues. Sometimes one may need an extra touch up to the colors to make the appearance better (Figures 23.3G and H).

Fabrications of Ocular Prosthesis Make the mould of same prosthesis,7 locate the Iris position and make a fresh mould, paint an iris button of the same curvature as the fellow eye and polymerize with the white base using the pressurized curing unit. Once it is cured, open the mold, create the blood vessels using the cotton rayon threads. Paint the scleral shades using dry earth pigments and cure with the clear layer of PMMA. Trim the extra portions, polish and insert into the patient orbital prosthesis, attached the eye lashes and eyebrows (Figure 23.3I).

Assemble the Prosthesis Once you are done with fabrication of ocular prosthesis and the facial prosthesis, your ocular piece can go very easily in the cavity that has been formed in place of dummy prosthesis. Attach the eyelashes on the upper lid and lower lids. If needed some external coloring can be done in order to look better (Figure 23.3J). Once satisfied, you have to instruct the patient regarding the use of the prosthesis.

Care of Your Prosthesis Preparing Your Skin and Your Prosthesis 1. Repeatedly practice positioning your prosthesis without adhesive to ensure accurate placement.

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Figures 23.3A to J: (A) mould shows smooth surface without pit and holes ideal for using the silicon (B) place the ocular prosthesis in the eye cavity groove (C) use the cynoacarylate glue to fix this in same place (D) shows the silicon from factor II and intrinsic colors (E) shows the steering of silicon along with flock and intrinsic colors in the silicon (F) using the thin cellophane sheet and checking the final color with patient skin tone (G) shows the room temperature cured prosthesis (H) indicated the rough edge of the prosthesis (I) fabrication of an ocular prosthesis (J) final finished prosthesis

332 Surgical Atlas of Orbital Diseases

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Figures 23.4A and B: (A) patient with left orbital exenteration due to Basal cell carcinoma of the left eye (B) patient with silicon glue on prosthesis in place, further cosmetic appearance improved with simple pair of glasses

2. Wash and thoroughly dry your hands and skin where your prosthesis is to be placed. 3. Clean your prosthesis with a soft, bristled toothbrush, mild soap, (e.g. Ivory liquid) and warm water.

Applying Your Prosthesis 1. If adhesive is used, it should be applied with cotton tipped swab by evenly spreading a thin layer of the adhesive along the outer edges of the backside of your prosthesis according to the manufacturer's instructions. 2. Allow the adhesive to reach its proper reapplication state depending on the type of adhesive used (e.g. dried clear for Pros-Aide). 3. Using a mirror, carefully position and press your prosthesis onto your skin to ensure good contact.

Removing Your Prosthesis 1. Remove your prosthesis from skin on a daily basis to keep your tissues healthy and to maintain hygiene. Grasp the thickest edge of your prosthesis and gently remove it very slowly so as not to tear the edges or irritate your skin. 2. If necessary, use a moist washcloth over the surface of the prosthesis to loosen adhesive from your skin.

Cleaning Your Prosthesis 1. If adhesive was used, remove it gently rolling the adhesive off the prosthesis (starting from the center to the outer edges) with your fingertips, using gauze or textured cloth. Soaking the prosthesis in a cup of warm water helps to soften adhesive and makes it easier to remove.

2. Clean the prosthesis with a soft, bristled toothbrush, mild soap (e.g. Ivory), and warm water. 3. Remove any traces of adhesive or oil by gently wiping the tissue side with a gauze or softtextured cloth moistened with rubbing alcohol. Repeat this step using a gauze or soft-textured cloth moistened with Listerine on the backside of the prosthesis. 4. If your prosthesis has an ocular component, remove and clean it with soap and water. The ocular component should NOT be cleaned with rubbing alcohol. Place a drop of mineral oil on the eye and shine it once a week. Replace the eye carefully and adjust the location by squeezing the prosthetic eyelids together. 5. If your prosthesis is retained with magnets, clips, or plastic buttons, take care to clean around each fixture with a soft, bristled brush, soap and water.

Cleaning Your Skin 1. Wash your face with soap and water after removing the prosthesis and remove any residue of adhesive from the skin. Avoid the use of harsh solvents such as benzene or xylene. 2. Apply a moisturizing lotion on nightly basis to restore natural body oils. 3. Report any areas of inflammation or irritation to the office or clinic.

Color Changes 1. Avoid smoking, as it will stain prosthesis yellow. 2. Avoid prolonged exposure to sunlight, which can cause color dissolution and weakening of the prosthetic material. 3. Avoid the use of strong solvents, such as benzene and xylene, which can cause dissolution and weakening of the prosthetic material.

Storing the Prosthesis 1. Store the prosthesis in a dry, inconspicuous but safe place (for example, a bedside table drawer). Keep it out of the reach of children and animals. 2. If you have an orbital prosthesis, store it in an upright position.

Orbital Prosthesis 333

Preventing Mishaps 1. Avoid extreme temperature changes, which can cause adhesive to fail. 2. Carry extra-adhesive and pre-packaged alcoholsoaked cotton balls in a small plastic bag. 3. Avoid placing the prosthesis in purses or pockets close to items such as ink pens and makeup that could stain it. 4. If adhesive are prescribed, be careful not to spill the adhesive bottle. To prevent evaporation, keep the lid tightened when not in use.

CONCLUSION In majority of cases facial prosthesis restores the cosmetic appearance as well as self confidence (Figures 23.4A and B). However, the patient should be educated and motivated to use the prosthesis and maintain it properly. A cosmetic rehabilitation without the proper counselling is not effective. Overall a facial prosthesis remains an option in minority of cases.

REFERENCES 1. Prince JH, A short history of the development of artificial eyes. In: Ocular prosthesis. E and S Livingstone Ltd, Edinburgh, 1946:6-7. 2. Jackson, IT, Tolaman, DE, Desjardins, RP,Branemark, PI: A new method for fixation of external prosthesis, Plast Reconst. Surg. 1986;77:668-72. 3. Raizada K, Murthy R, Honavar SG. Ocular prosthesis with lower lid augmentation for disfigured lids following chemical burns. Journal of Ophthalmic Prosthesis J Ophth. Prosth. Volume II PP 24-6. 4. Raizada K, D Deepa Rani, Naik M, Honavar SG, Journal of Facial and Somato Prosthesis. Post Enucleation Socket Syndrome: an ocularist View, Journal of Facial and Somatoprosthesis, 2005;1-12. 5. Yeatts RP. The esthetics of orbital exenteration.Am J Ophthalmol. 2005;139(1):152-3. 6. Bulbulian AH. Prosthetic reconstruction of the exenterated orbit. In: Facial Prosthetics. Charles C Thomas, Springfield, IL, 1973: 48-63. 7. Jahrling RC. Contracted socket following enucleation after multiple surgical procedures. Problems and treatment of enucleation, evisceration, exposure. Intercontinental Medical book corporation 1974:12;27-9. 8. Bulbian AH, Facial Prosthetics, method of retention of facial prosthesis 364.

24

CHAPTER

Medical Management of Proptosis Subrahmanyam Mallajosyula, Mohd Javed Ali

Some of you may be surprised to know that nearly half the cases of proptosis can be managed without surgery. This is because of the advances in diagnostic and therapeutic interventions made in the past few decades which made medical managements more scientific, evidence based and safer. Many patients of proptosis obviously are to benefit from these advances. We are confident that most of the active orbital surgeons across the globe agree with this statement. Many etiological factors involved in proptosis can now be safely managed medically. This chapter outlines the standard protocols and recent trends invading this arena. The role of medical management in thyroid orbitopathy, and the role of chemotherapy were dealt in detail in separate chapters and hence not included in this. Let us consider the medical management in the following headings: • Nonspecific inflammations of the orbit • Specific inflammations of the orbit • Vascular lesions • Structural lesions • Lymphoproliferative and other neoplastic lesions.1

NONSPECIFIC INFLAMMATIONS OF THE ORBIT (NSOIS) Based upon the location of inflammation the nonspecific orbital inflammation or idiopathic orbital inflammation can present as five entities : Myositic,

lacrimal, anterior, diffuse and apical. Apart from clinical presentation, imaging is very helpful in diagnosis. NSOIS is usually acute or subacute in onset and painful. It is usually unilateral, and occasionally bilateral. Rarely it is recurrent. The visual symptoms include diplopia, and defective vision. Histologically NSOIS is characterized by polymorphous infiltrations.

Nonspecific Myositic Inflammation This is the most common presentation in our experience. We manage patients presenting as a single muscle disease with nonsteroidal anti-inflammatory drugs and low dose corticosteroids. Recurrence is unlikely in them. Patients presenting with multiple muscle disease, are prone to recurrences. We treat them more aggressively with oral Prednisolone 2 mg/kg body weight tapered over 4-6 weeks or with intravenous methyl prednisolone2 pulse therapy. Patients with recalcitrant disease require immunosuppressives. Such patients were found to benefit from methotrexate in a dose of 15-25 mg per week3 usually marked clinical response is evident within a week. Those cases which fail to respond to the drugs warrant a biopsy to exclude a lymphoma.

Nonspecific Lacrimal Inflammation Nonspecific dacryoadenitis should prompt a suspicion for systemic disease where a percutaneous biopsy is recommended first. Management includes moderate doses of steroids such as 1mg/kg oral prednisolone which can be tapered over 4-6 weeks. Majority of nonspecific dacryoadenitis resolves over 6-12 weeks.

338 Surgical Atlas of Orbital Diseases

Specific Inflammations of the Orbit These are the most common etiological factors in proptosis for which medical management is commonly carried out. Therapeutic options in management of inflammations are ever expanding not only because biologically targeted agents are becoming increasingly available that can act on specific segments of inflammatory cascades but also because of advances in our understanding of etiopathogenesis.

Orbital Cellulitis This is the most common cause of painful proptosis, acute in onset and most often unilateral. The principles of management of a case of orbital cellulitis are control of infection by the use of appropriate antibiotics, preventions of ocular as well as nonocular complications, surgical drainage when necessary and careful follow up. We insist on imaging on an emergency basis in every case of orbital cellulitis for two reasons : (1) To make sure that we are not missing other neoplastic lesions like Rhabdomyosarcoma, Retinoblastoma which clinically mimic orbital cellulitis (2) To plan the course of action: We prefer surgical drainage of orbital or sub-periosteal abscess. In the absence of abscess, medical management is preferred. The patient should be carefully monitored during the treatment for any threat of loss of vision, and clinical response to the drugs. From our experience and also from the literature we wish to emphasize that there is a great difference in the prognosis and hence management strategies of orbital cellulitis in children and adults. Usually in children under the age of 9 years the infection is by a single aerobic organism such as pneumococci4 and respond to medical management, the threat to affect vision is rare and surgery is rarely needed. In contrast adults harbor polymicrobial infection and the threat to vision is common and hence drainage is frequently required in addition to systemic antibiotics.5 Orbital cellulitis secondary to sinusitis is known to harbour organisms like Strep pneumoniae, H influenzae, Bacteroids and anaerobic cocci. Recommended antibiotics include third generation cephalosporins like cefotaxime, ceftriaxone and cefuroxime. Alternatively piperacillin with taxobactum or ticarcillin with clavulanate can be used. Orbital cellulitis secondary to trauma or foreign-body

are known to be harbouring organisms like S aureus, S epidermidis, Streptococci and anerobes. Recommended antibiotics for such cases include Vancomycin along with third generation cephalosporins or imipenem1 Imaging with a CT or a MRI can accurately monitor the progress and effect of treatment. Septic thrombosis of cavernous sinus either due to spread from contiguous structures or septicemia demands prompt recognition and treatment with broad spectrum antibiotics as discussed above for optimal clinical outcome.

Rhino-orbital Mucormycosis This is one of the very harmful infections and can lead to death. It is almost always associated with uncontrolled diabetes mellitus and usually with ketoacidosis.6-8 Once the diagnosis is established based on clinical findings, microscopic fungal examination and culture, a multidisciplinary approach is commonly practiced. Diabetes should be managed simultaneously. Following wide excision of devitalized tissue, the area is daily irrigated with amphotericin. Systemic treatment with amphotericin is also recommended. Some believe in the usefulness of hyperbaric oxygen for such cases.9

Chronic Granulomatous Infections Since the advent of AIDS, incidence of certain granulomatous infections of importance in proptosis like tuberculosis and syphilis is on a rise. Orbital involvement in tuberculosis is usually by direct invasion from sinuses or hematological dissemination. Periostitis, cold abscess and orbital tuberculomas are well recognized lesions.10 In doubtful cases PCR is helpful. Orbital involvement is diagnosed with a high index of suspicion and aspiration biopsy. Systemic anti-tuberculous drugs are recommended in coordination with a chest physician. Though literature says that syphilis is on a rise with immunosuppressive syndromes, we are yet to come across a case of syphilis with orbital involvement. Periostitis, acute and chronic inflammations are recognized lesions. Systemic antibiotic therapy usually with penicillins is useful in resolution of the disease.

Parasitic Infestations Cysticercosis, echinococcosis and trichinosis are common parasitic etiological factors in the causation of proptosis.

Medical Management of Proptosis 339 Cysticercosis is caused by C.cellulosae, the larval form of tapeworm, Taenia solium. Though orbit is considered to be a rare site in the west, many reports in the literature suggests that orbital involvement is most frequent among Asians.11 Once diagnosis is established with the help of imaging and serology, systemic albendazole (15 mg/kg) along with steroids (prednisolone 1-2 mg/kg) for a period of four weeks is found to be effective11 If there is evidence of associated neurocysticercosis, treatment is with steroids (prednisolone 1mg/kg) and praziquantel 50 mg/kg in three divided doses for a period of 15 days. Association between orbital myocysticercosis and neurocysticercosis is not very common. We prefer to refer these patients to a neurologist for in-patient treatment since there is a rare possibility of generalized seizures. The response can be monitored and progress can be documented with imaging. Echinococcosis or hydatid cyst as it is commonly called is an intestinal infestation of dogs. Orbital cysts are seen in 1% of echinococcosis. Systemic albendazole has been found to be effective in resolving the cyst.15 Steroids are recommended for violent inflammatory reactions following rupture of the cyst during aspiration or attempted surgical removal16 Certain studies have shown high efficacy in disease resolution when combination of praziquantel with albendazole12-14 is used. Trichinosis occurs as cysts in extraocular muscles, which may show evidence of calcification on imaging. Treatment recommended include systemic thiabendazole along with steroids to reduce inflammation. Personally we have no experience as we are yet to come across a case of trichinosis with orbital involvement.

Vasculitis Vasculitis or angiitides as some may call it is a clinical syndrome that encompasses acute or chronic inflammation of vessels with vaso-obliterative signs and symptoms17 Most common vasculitis that involve the orbit include Wegener's granulomatosis and Polyarteritis nodosa. Diagnosis is usually established by imaging, biopsy with a histopathological examination and specially for wegener's a serological examination in the form of C-ANCA 15 Dramatic improvement is noticed when systemic steroids are combined with an alkylating agent like cyclo-

phosphamide.21,22 Recent studies have suggested a role of anti-TNFs (tumor necrosis factors) like Infliximab and Etanercept. The development of novel approaches focusing on blockade of specific molecules including TNF alpha is awaited. 22 Another novel approach that is showing promise in the management of refractory Wegener's and C-ANCA related vasculitis is the use of Rituximab, a chimeric antiCD20 monoclonal antibody.23,24

Tolosa-Hunt Syndrome This nonspecific granulomatous inflammation though rare is nevertheless an important differential diagnosis of apical orbital inflammations. The clinical course is marked by remissions and recurrences. SPIR MRI (spectral presaturation with inversion recovery MRI) has been recommended for diagnosis1 Management includes a high dose of systemic steroids which often produces a dramatic clinical improvement. SPIR MRI before and after corticosteroids have been found to be useful in some studies for definite diagnosis and monitoring of the disease.1

Vascular Lesions Capillary Hemangioma Treatment is indicated when vision is threatened by amblyopia as a result of anisometropia, ptosis or strabismus. Intralesional injection of steroids is the most frequently used method. Usually 40-80 mg of triamcinolone with 25 mg of methylprednisolone is directly injected into the lesion. 1 Alternatively Triamcinolone 40 mg in combination 4 mg betamethasone can be used. The tumor usually begins to regress in two weeks but if necessary injection may be repeated after about two months. Early recognition and prompt treatment with intralesional steroid prevents amblyopia exanopsia, but followup and management of refractive amblyopia with glasses and patching is necessary in the longer term. Potential complications include skin depigmentation, fat atrophy, eyelid necrosis and rarely central retinal artery occlusion. Systemic steroids are indicated for extensive lesions specially if associated with visceral involvement. Recommended dosage used is 1.5 mg/kg to 2.5 mg/kg Prednisolone daily over a few weeks with titration downward depending on response.1

340 Surgical Atlas of Orbital Diseases Though steroids are effective in large majority of patients, a recurrence is not infrequent. Recurrent or resistant cases are being treated with recombinant interferon alpha-2a and 2b with variable results16 Recent studies have demonstrated good efficacy of interferons when given subcutaneously in a dose of 3 million units/m 2. During clinical follow-up diagnostic ultrasound evaluation ( the depth dimension) proved helpful. One report suggested high efficacy of treatment when a combination of interferon alpha-2a with a low dose of cyclophosphamide.17 In the presence of very large platelet-consuming lesions as seen with Kasabach-Meririt syndrome, systemic antifibrinolytics like aminocaproic acid or tranexemic acid are used.18

Structural Lesions Acute Intraorbital Hemorrhage and Emphysema Post-traumatic fractures, soft tissue injury, contusions and retrobulbar blocks may be associated with acute rise in intraorbital pressure as blood is trapped in confined spaces. The effects of such rapid rise in the pressure include optic nerve ischemia and retinal hypoperfusion. The optic nerve head may demonstrate arterial pulsations. Hence the importance of prompt recognition and early management cannot be overemphasized. Though severe visual threat is a surgical emergency, for moderate degrees of orbital tension, treatment includes 500 mg of acetazolamide i.v., and mannitol 1-2 ml/kg i.v over 30 minutes has been advocated.1,19 Orbital emphysema is another cause of acute orbital tension and is almost always secondary to trauma. This rarely requires decompression as the air tends to absorb rapidly. Though most of the patients are managed with antibiotics, prophylactic use is usually not required for clean wounds.20

advances. Among the lymphoproliferative lesions, reactive lymphoid hyperplasia appears to be steroid sensitive as it responds to moderate doses of Prednisolone. Failure to respond to steroids can be managed by cytotoxic agents and low dose radiotherapy.1 Another clinicopathological entity; the indeterminate lymphoproliferative lesions are steroid resistant and may require treatment with immunosupressives or radiotherapy.1 The widespread use of chemotherapy for lympho-proliferative lesions and other neoplastic conditions is being dealt in detail in a separate chapter.

CASE ILLUSTRATIONS Case 1 Mrs.K, female 54 years presented with acute, painful proptosis of left eye of 5 days duration. She had severe pain, nausea and mild fever. There was a very severe edema of the lids and periorbital edema (Figure 24.1A). The upper lid had complete ptosis. On everting the upper lid, the globe was found to be proptosed. The conjunctiva was congested and chemosed. Ocular motility was restricted (Figures 24.1B and C).CTscan showed orbital cellulitis without any abscess. She was given intravenous (Amoxicillin and clavilanic acid) and Metronidazole, with which she showed a marked improvement within 5 days. She was relieved from pain, proptosis reduced, and the ptosis improved markedly( Figure 24.1D) The conjunctival chemosis and congestion improved and the ocular motility restored to normal (Figures 24.1E and F).

Lymphoproliferative and Other Neoplastic Lesions These disorders encompasses a wide range of clinical syndromes. The advent of immunodiagnostics and molecular techniques had a profound effect on better understanding of pathogenesis and therapeutic

Figure 24.1 A: Female 54 years presented with acute, painful proptosis of left eye. Note the severe edema of the lid and periorbital edema, and the gross ptosis

Medical Management of Proptosis 341

B

C Figures 24.1B and C: On elevating the lid, note the conjunctival congestion and chemosis. Note the restricted ocular motility both in adduction (B) and abduction (C)

Figure 24.1D: After medical management, note the improvement in the edema of the lids, ptosis, chemosis and congestion of the conjunctiva

E

F Figures 24.1E and F: Note the restoration of ocular motility both in adduction (E) and abduction (F)

Case 2 Female 17 years, presented with proptosis of right eye associated with mid pain since 1 month. There was no history of trauma, defective vision or diplopia. Examination revealed mild proptosis of the right eye with fullness of right upper lid in the supero-temporal region (Figure 24.2A). CTscan of

the orbit revealed enlarged lacrimal gland molding to globe (Figure 24.2 B). The possibility of lymphoma was thought off. FNAC and immunohistochemistry were negative for lymphoma. Hence, a diagnosis of nonspecific orbital inflammation, involving the lacrimal gland was made; the girl was treated with systemic steroids to which she responded well (Figure 24.2C).

342 Surgical Atlas of Orbital Diseases CT scan revealed inflammation at the apex of the orbit (Figures 24.3E and F) with mild enlargement of superior ophthalmic fissure. In view of subacute onset, associated pain, restricted ocular motility and defective vision, a diagnosis of superior ophthalmic fissure syndrome was made, and he was treated with systemic steroids. The patient responded very well. The vision improved from 20/200 to 20/30 in a fortnights time. Figure 24.2 A: Note the fullness at the supero-temporal region of the right upper lid, with mild displacement of the globe and minimal ptosis

Figure 24.3A: Male 28 years, presented with severe ptosis of right upper eye lid and mild proptosis of right eye

Figure 24.2B: Coronal section of CT scan of orbit showing enlarged lacrimal gland molding to the globe Figure 24.3B: Note the restricted adduction in the right eye

Figure 24.3C: Note the restricted depression in the right eye Figure 24.2C: One week after oral prednisolone, note the improvement in the fullness of the supero-temporal region of the right upper lid, and in ptosis

Case 3 Male 28 years presented with proptosis of right eye, subacute in onset and associated with mild pain and defective vision( Figure 24.3A) On examination, he had mild proptosis associated with ptosis, and restricted ocular motility (Figures 24.3B to D).

Figure 24.3D: Note the restricted abduction in the right eye

Medical Management of Proptosis 343 8. Nithyanandam S, Jacob MS, Battu RR, Thomas RK, Correa MA, D'Souza O. Rhino-orbito-cerebral mucormycosis. A retrospective analysis of clinical features and treatment outcomes Indian J Ophthalmol. 2003;51(3): 231-6. 9. Ferry AP, Abedi S. Diagnosis and management of rhinoorbitocerebral mucormycosis. A report of 16 personally observed cases. Ophthalmology 1983;90:1096-104.

E

F Figures 24.3E and F: CT scan, Axial and sagital sections of the orbit show a hyperdense lesion abutting the optic nerve at the orbital apex. Its margins are indistinct. Axial section of the CT shows enlarged superior ophthalmic fissure

REFERENCES 1. Rootman J: Diseases of the orbit ; A multidisciplinary approach. Lippincott Williams and Wilkins, (2nd ed): 455506. 2. Nugent RA, Rootman J, Robertson WD, et al. Acute orbital pseudotumors: AJNR 1981;2:431-6. 3. Hemady R, Tauber J, Foster CST. Immunosuppressive drugs in immune and inflammatory ocular disease. Surv Ophthalmol 1991;35:369-85. 4. Donahue SP, Schwartz G. Preseptal and orbital cellulitis in childhood: a changing microbiologic spectrum. Ophthalmology 1998; 105:1902-6. 5. Harris GJ. Subperiosteal abcess of orbit. Age as a factor in the bacteriology and response to treatment. Ophthalmology 1994;101:585-95. 6. Ameen M, Arenas R, Martinez-Luna E, Reyes M, Zacarias R: The emergence of mucormycosis as an important opportunistic fungal infection: five cases presenting to a tertiary referral center for mycology. Int J Dermatol. 2007 Apr;46(4):380-4. 7. Bhadada S, Bhansali A, Reddy KS, Bhat RV, Khandelwal N, Gupta AK : Rhino-orbital-cerebral mucormycosis in type 1 diabetes mellitus, Indian J Pediatr. 2005;72(8):671-4.

10. Pillai S, Malone TJ, Abad JC. Orbital tuberculosis. Ophthal Plast Reconstr Surg 1995;11:27-31. 11. Honavar SG, Sekhar.G, Orbital Cysticercosis. Orbit 1998; 17(4): 271-84. 12. Srivastava VK, Srivastava A, Singhal KC. Albendazole therapy in orbital cysticercosis. Ind J Physiol Pharmacol 1996; 40:265-66. 13. Richards KS, Morris DL Effect of albendazole on human hydatid cysts: an ultrastructural study. HPB Surg 1990; 2: 105-13. 14. Gomez MA, Croxatto JO, Crovetto L, Ebner R. Hydatid cysts of the orbit. A review of 35 cases. Ophthalmology 1998; 95:1027-32. 15. Perry SR, Rootman J, White VA. The clinical and pathological constellation of wegener's granulomatosis of the orbit. Ophthalmology 1997; 104:683-94. 16. Nolle B, Coners H, Duncker G. ANCA in ocular inflammatory disorders. Adv Exp Med Biol 1993;336: 305-7. 17. Teske S, Ohlrich SJ, Gole G, et al. Treatment of orbital capillary hemangioma with interferon. Aust N Z J Ophthalmol 1994; 22: 13-7. 18. Neidhart JA, Roach RW. Successful treatment of skeletal hemangioma and Kasabach-Merritt syndrome with aminocaproic acid. Am J Med 1982; 73: 434-8. 19. Rootman J, Stewart B, Goldberg RA. Orbital Surgery: A conceptual approach. Philadelphia: Lippincott-Raven, 1995. 20. Fleishman JA, Beck RW, Hoffman RO. Orbital emphysema as an ophthalmologic emergency. Ophthalmology 1984; 91:1389-91. 21. Selamet U, Kovaliv YB, Savage CO, Harper L. ANCAassociated vasculitis: new options beyond steroids and cytotoxic drugs. Expert Opin Investig Drugs. 2007;16(5): 689-703. 22. Svozilkova P, Rihova E, Brichova M, Diblik P, Kuthan P, Poch T. Infliximab in the treatment of Wegener's granulomatosis: case report. Cesk Slov Oftalmol. 2006;62(4):280-6. 23. Tamura N, Matsudaira R, Hirashima M, Ikeda M, Tajima M, Nawata M, Morimoto S, Kaneda K, Kobayashi S, Hashimoto H, Takasaki Y. Two cases of refractory Wegener's granulomatosis successfully treated with rituximab. 24. White ES, Lynch JP Pharmacological therapy for Wegener’s granulomatosis. Drugs. 2006;66(9):1209-28.

344 Surgical Atlas of Orbital Diseases

25

CHAPTER

Management of Ophthalmic Tumors: Role of Chemotherapy and Radiation Therapy Vijay Anand P Reddy, Nitin More, Ramesh Murthy, Anirban Bhaduri, Santosh G Honavar

INTRODUCTION Ocular Oncology deals with the diagnosis, surgical and nonsurgical management of tumors involving the eyelids, external ocular surfaces, intraocular structures and the orbit. Ocular tumors invariably are managed by multidisciplinary team of ocular surgeon and an oncologist experienced in the treatment of such tumors. Radiation Oncology is the clinical and scientific discipline devoted to the management of patients with cancer and other diseases with ionizing radiation alone or combined with other modalities like surgery and chemotherapy. The aim of radiation therapy is to deliver a precisely measured dose of radiation to a defined tumor volume with minimal damage to surrounding healthy tissue. Radiation used for cancer treatment is called ionizing radiation because it forms ions in the cells of the tissues it passes through, as it dislodges electrons from atoms. Ions are atoms that have acquired an electric charge through the gain or loss of an electron. This can kill cells or change genes. Other forms of radiation, such as radio waves, microwaves, and light waves are called non-ionizing. They have lower energy and hence can not ionize cells.

Ionizing radiation is of two major types a. Non-particle Photons (X-rays and γ-rays), which are most widely used. b. Particle radiation (electrons, protons, neutrons). The common types of radiation used for cancer treatment are:

1. High-energy photons come from radioactive sources such as cobalt, cesium, or a machine called a linear accelerator. This is by far the most common type of radiation treatment in use today. 2. Electron beams produced by a linear accelerator or beta particle emitting radioactive source like Strontium and Ruthenium. They are used for tumors close to a body surface, e.g. skin, conjunctiva and sclera. 3. Protons are a newer form of treatment. Protons are parts of atoms that cause little damage to tissues they pass through but have maximum effect at the end of their path. This means that proton beams may be able to deliver more radiation to the cancer while causing fewer side effects to normal tissues nearby. Although it is used routinely for certain types of ocular and brain tumors, it still needs more study in others. 4. Neutrons are used for some cancers of the head, neck, and prostate. They can sometimes be helpful when other forms of radiation therapy do not work especially when the tumor has anoxic zones.

Radiation therapy delivery methods are as follows: 1. External Beam Radiation (Teletherapy): It is the most widely used type of radiation therapy. The radiation is focused from machine outside the body onto the area affected by the cancer. This type of radiation is most often given by either radioactive source like cobalt or cesium or with linear accelerators (Figure 25.1). The

Management of Ophthalmic Tumors: Role of Chemotherapy and Radiation Therapy 345 radiation is aimed at the tumor, but also affects the normal tissue it passes through on its way into and out of the body. External beam radiation allows large areas of the body to be treated and allows treatment of more than one area such as the primary tumor and nearby lymphnodes. External radiation is usually given in daily treatments, 5 days per week over several weeks. 2. Internal Radiation Therapy (Brachytherapy): It is also known as brachytherapy, which means short-distance therapy. With this method, radioactive sources are placed directly into the tumor or into a cavity close to the tumor. The advantage of brachytherapy is the ability to deliver a high dose of radiation to a small area. It is useful in situations that require a high dose of radiation. The main types of internal radiation are: a. Interstitial radiation: The radiation source is placed directly into or next to the tumor using small pellets, wires, tubes, or containers. For example, carcinoma tongue (Figures 25.2A and B). b. Intracavitary radiation: A container of radioactive material is placed in a cavity of the body such as the vagina, nasopharynx.

c. Surface (mould): Radiation sources are placed over the tissue to be treated, e.g. Ca hard palate. d. Plaque Brachytherapy: Concave shaped radioactive plaque is placed over the sclera for ocular melanomas, retinoblastoma, etc (Figure 25.3A). e. Intraluminal: Sources are placed in a lumen, e.g. carcinoma esophagus. f. Intravascular: A single source is placed into small or large arteries such as coronary artery for prevention of stent restenosis. Based on the duration of treatment, brachytherapy is classified as: a. Permanent (low dose rate) Brachy therapy: Dose is delivered over the lifetime of the source until complete decay. b. Temporary (high dose rate): Dose is delivered over a short period of time and the sources are removed after the prescribed dose has been reached.

A

B Figure 25.1: Teletherapy machine for external beam radiation therapy

Figure 25.2A and B: Interstitial brachytherapy

346 Surgical Atlas of Orbital Diseases Radiation therapy may be used alone or in combination with other cancer treatments, such as chemotherapy or surgery. In some cases, a patient may receive more than one type of radiation therapy.

External Radiotherapy in Ocular Tumors Linear accelerators equipped with both photon and electron facility and multileaf collimators (MLC) are mainly used for the external radiation therapy of orbital tumors where multiple radiation beams are focused on the tumor. Simulator with all the parameters similar to linear accelerator but capable of only diagnostic X-rays is used for radiation therapy planning. It simulates the beams of the external radiotherapy machines before the patient is being taken for the actual treatment. Conventionally radiation therapy planning is carried out by immobilizing the patient in treatment position and a CT scan is done in the treatment position with the immobilization in place. The images are transferred to the computerized treatment planning system. The tumor volume and the critical structures are delineated by the radiation oncologist and then the medical physicist plans the various beam angles and energies and gives various treatment options. The optimal plan is selected by the radiation oncologist and the patient is simulated according to bony landmarks visible under fluoroscopy and the data given by the treatment planning system. The computerized scan is invariably being used in all orbital tumors and the 3-dimensional conformal radiation therapy (3D-CRT) is planned.

Figure 25.3A: Radioactive plaques for intraocular tumors

All the efforts in the development of radiation therapy techniques are directed towards proper inclusion of tumor in the target volume and to spare the surrounding normal tissues. Intensity modulated radiation therapy (IMRT) is newer form of external radiation therapy that is capable of obtaining desired dose distribution in irregular and concave shapes sparing the adjoining critical organs like optic chiasm and pituitary gland.

Plaque Radiotherapy A radioactive plaque is a device that can be used to deliver a high dose of radiation precisely and selectively to a tumor and negligible dose to the surrounding structures. It is made with radioactive Cobalt, Ruthenium, Iridium, Palladium or Iodine sealed within. Plaques come in various shapes and sizes ranging from 10 to 25 mm in diameter (Figure 25.3B). The procedure is done under anesthesia. Preoperative assessment of the lesion: location, size and thickness are measured by means of ultrasound B scan and the details are given to the ocular radiation oncologist. The radiation oncologist along with the radiation medical physicist plans the treatment with the help of computerized automated dosimetry software. An appropriate plaque, the radiation dose, dose rate and treatment time is selected. The treatment time may vary from 24 to 96 hours based on type and size of tumor. This process needs expertise of a radiation therapist and a radiation physicist well versed in brachytherapy.

Figure 25.3B: Ruthenium 106 plaque being placed on the eye for a choroidal melanoma

Management of Ophthalmic Tumors: Role of Chemotherapy and Radiation Therapy 347 After obtaining a proper consent the patient is taken for the operative procedure. The concave shaped dummy plaque similar to the radioactive plaque is placed and checked and then the radioactive plaque is placed and sutured in place (Figure 25.4). Patient is placed in isolation till the entire period of radiotherapy. The plaque is removed under anesthesia after the required dose of radiation is delivered. Indications of plaque brachytherapy are as follows: 1. Retinoblastoma measuring < 16 mm in basal diameter and < 8 mm in thickness a primary treatment, as an adjuvant to chemoreduction, and for failure of focal therapy. 2. Primary treatment for most medium-sized and some large choroidal and ciliary body melanomas in an eye with salvageable vision. 3. Choroidal hemangioma. 4. Choroidal metastasis. 5. Extensive retinal capillary hemangioma.

Cell Cycle and the Principles of Anti-neoplastic Therapy It is important to understand the growth pattern of tumor cells that affect the overall biological behavior of tumor and response to anti-neoplastic therapy, either radiation or cytotoxic chemotherapy. Cell cycle

is composed of four distinct phases. The G1 phase consists of cells that have recently completed division and are committed to continued proliferation. After a variable period of time, these cells begin to synthesize DNA, marking the beginning of the S phase. After DNA synthesis is complete, the end of the S-phase is followed by the premitotic rest interval called the G2 phase. Finally, chromosome condensation occurs and the cells divide during the mitotic M phase. Resting diploid cells that are not actively dividing are described as being in the G0 phase. Ionizing radiation generally affects the neoplastic cells those are in synthetic and mitotic phase where the DNA of the cell is damaged either temporarily or permanently, causing subsequent cell death. Radiation therapy is usually delivered in multiple fractions to target the tumor cells which were in resting phase during earlier fraction of radiation. Similarly, some anticancer agents induce their cytotoxic effects during specific phases of the cell cycle. Chemotherapeutic agents are used either as single agent or in the combination of different agents. Combination chemotherapy agents are selected according to different mechanism of action to have synergistic effect and with different toxicity profile. Cumulative doses of individual drugs are typically low in combination chemotherapy regimens, potentially minimizing the long-term toxicity and improving the therapeutic ratio.

Management of Ophthalmic Tumors Ocular tumors with proptosis as the first symptom arise from eyelid and lacrimal gland, orbital soft tissues, optic nerve and orbital bones. Prominent ones among those are squamous and basal cell carcinoma of eyelid, rhabdomyosarcoma, non-hodgkin's lymphoma, optic nerve glioma, meningioma and idiopathic orbital inflammation. Occasionally, retinoblastoma in advanced stage could present with proptosis of eye.

Tumors of the Eyelid

Fig. 25.4: Cell cycle

Most eyelid masses are benign tumors such as skin squamous cell papilloma, melanocytic nevus or congenital and acquired cysts. Common malignant tumors of eyelid include basal cell and squamous cell carcinoma.

348 Surgical Atlas of Orbital Diseases

Capillary Hemangioma It is the most common pediatric eyelid tumor. It may be a component of Hippel-Lindau syndrome or Surge-Weber syndrome. The natural history of lesion is spontaneous regression over 3 to 4 years, therefore usually these lesions are observed. Indications of treatment are obstruction of the vision, amblyopia, ulceration of the eyelid due to vascular compression. Steroids are the first line of treatment. Radiation therapy is reserved until other treatment methods have failed. Low energy photons or electrons are used in dose of 500 to 750 cGy in 2-3 fractions or fractionated low dose to a total of 1600 to 2000 cGy.1

Basal Cell and Squamous Cell Carcinoma Basal cell carcinoma (BCC) represents 90% of malignant eyelid tumors. Its four morphological types are the nodular, ulcerative, pigmented and the morpheaform tumor. It mostly involves the lower eyelid. More likely to affect fair skinned persons with high solar exposure. It may be mistaken for a chalazion or chronic blepharitis. Complete excision with frozen section control of tumor margins using cryo is the standard treatment for localized tumors. Larger lesions are treated with definitive surgery and appropriate reconstruction. Squamous cell carcinoma (SCC) is the second most common malignancy of the eyelids. Sun exposure is the most important factor in developing SCC of the skin. It may occur in previously normal appearing skin or more commonly arises from a pre-existing lesions like actinic keratoses, skin damaged by ionizing radiation or xeroderma pigmentosum. Unlike BCC of the eyelid, SCC can be an aggressive tumor and has the potential to invade the orbit, metastasize to lymph nodes and distant sites. Primary excision is curative for small lesions in basal cell and squamous cell carcinoma of eyelid. Irradiation could be used for unresectable and recurrent basal cell carcinoma. It also could be used as an alternative to surgery with more than 90% of cure rate if patient prefers radiation therapy over surgery. Cryotherapy could be used for recurrent lesions; usually in medial canthus. Orbital exenteration is reserved for deep invasive lesions. Radiation dose of 50 to 60 Gy should be delivered

with low energy X-rays or electrons with appropriate shielding of lens.2

Sebaceous Carcinoma Ocular sebaceous carcinoma is a very rare but aggressive tumor, most commonly occurs in patients 60 to 80 years of age although the range is from early childhood through the nineties. It usually arises from the meibomian glands followed by glands of Zeiss and caruncle. It may be multicentric resulting in local recurrences. The incidence appears to be somewhat greater in women and in Asian population.3 General principle of treatment is wide excision (with 5 to 6 mm surgical margins) with either frozen section or permanent section control as primary management of sebaceous carcinoma.4 Map biopsies of eyelids and conjunctiva should be carried out. Radiotherapy provides acceptable cosmesis both for primary treatment and for treatment of recurrent disease. Somewhat higher irradiation dosages in the range of 60 to 65 Gy in six to seven weeks are recommended, and the control rates are in the range of 80 to 90% or better.5

Tumors of Lacrimal Gland Epithelial tumors of lacrimal gland could be benign, e.g. pleomorphic adenoma (benign mixed tumor) or malignant, e.g. adenoid cystic carcinoma or pleomorphic adenocarcinoma (malignant mixed tumor). On imaging, pleomorphic adenoma appears as round to ovoid superotemporal orbital mass which may cause bony indentation, without bone erosion. Malignant tumors have irregular margin, often with adjoining bone destruction. Complete excision of mass (excisional biopsy) is curative for pleomorphic adenoma. Incomplete excision can lead to recurrence and malignant transformation. Malignant tumors should be completely excised if possible. Routinely postoperative radiation therapy to a dose of 5000 to 6000 cGy is delivered. In locally advanced lacrimal gland carcinoma, neoadjuvant chemotherapy with cisplatin and 5-fluorouracil followed by surgery and postoperative radiation therapy is given. Results of intracarotid chemotherapy with Cisplatin and Doxorubicin followed by surgery and radiation therapy are encouraging6,7

Management of Ophthalmic Tumors: Role of Chemotherapy and Radiation Therapy 349

Malignant Conjunctival Tumors Squamous cell carcinoma is the most common primary malignant tumor of conjunctiva, manifests usually as a fleshy vascularized mass at the limbus. Conjunctival tumors are treated by complete excision biopsy with frozen section control of tumor margins and cryotherapy of the tumor bed. Reconstruction can be done then by simple closure, conjunctival grafting or amniotic membrane transplant. Primary or adjunctive use of local treatment with some chemotherapeutic agents such as MitomycinC, 5- Flourouracil and Interferon alpha 2-b have been reported. In some diffuse radiosensitive tumors such as lymphoma, fractionated external beam radiotherapy or application of a radioactive plaque may be employed.

Intraocular Tumors Intraocular tumors arise from iris, choroid, retina or optic nerve head. Iris masses could be melanocytic as nevus and melanoma, granuloma, hemangioma, leiomyoma, lymphoma, metastases, and extension from a ciliary body tumor. Choroidal melanoma is the most common intraocular tumor in adults. Other common intraocular pigmented tumors include optic nerve head melanocytoma, retinal pigment epithelium adenoma and combined hamartoma of retina and retinal pigment epithelium. Choroidal nonpigmented masses include amelanotic melanoma, uveal granuloma, lymphoma, osteoma and choroidal metastases. Retinal non-pigmented masses include retinoblastoma and toxocara granuloma.

Choroidal Melanomas Small Lesions <1.5 mm height without high risk factors like juxta papillary location, presence of subretinal hemorrhage, presence of orange pigment are closely observed. Lesions of 1.5 to 10 mm height are treated according to its location. Peripheral lesions are locally excised. Central and mid peripheral lesions with size < 4 mm are treated with trans-pupillary thermotherapy (TTT). Lesions with > 4 mm of size are treated with plaque therapy or external radiation therapy with photons or protons. Lesions of >10 mm in height are treated with enucleation or external radiation therapy. Ruthenium-106 is currently the most commonly used isotope for plaque radiotherapy of choroidal

melanomas, although cobalt-60, Iodine-125, iridium192, strontium-90, and palladium-103 have also been used. Modern techniques for plaque brachytherapy involve suturing a shielded plaque containing seeds of the radioactive isotope to the sclera.8,9 This remains in place for a specified number of days in order to deliver the proper dose of radiation. Most melanomas are treated with a calculated apex dose of 70 to 85 Gy.10

Intraocular Lymphoma This is a rare variety of non-Hodgkin's lymphoma, large cell lymphoma being the most common histology. Uveal tract, retina, vitreous or optic nerves are usually involved. Vitreoretinal involvement is usually associated with central nervous system lymphoma. Diagnosis is usually done by vitreous biopsy. Usually they do not have systemic manifestation. Recommended treatment is external radiation therapy to a dose of 3600 to 4000 cGy at 1.8-2 Gy fractions.11

Retinoblastoma For over 100 years or longer the treatment of intraocular retinoblastoma has been enucleation. Various forms of radiation treatment have been used in the management of retinoblastoma since World War II. The goal of radiation has been to destroy the tumour, save the eye, and maximise visual potential. Since the radiation in the paediatric age group has its potential long-term side effects newer modalities like chemotherapy has been attempted. Since the introduction of platinum and etoposide based chemotherapy there has been tremendous improvement in tumor control and survival. Current standard treatment options for retinoblastoma include the following: 1. Cryotherapy: used in addition to radiation or in place of photocoagulation for lesions smaller than 4 disc diameters in the anterior portion of the retina. 2. Photocoagulation: occasionally used alone with small tumors. It is used for posteriorly located tumors that are smaller than 4 disc diameters, distinct from the optic nerve head and macula, and without involvement of large nutrient vessels or choroid involvement in patients with early-stage disease (in addition to radiation

350 Surgical Atlas of Orbital Diseases therapy) or when there is limited recurrence following radiation therapy. Thermotherapy delivered via infrared radiation is an alternative to laser photocoagulation.12 3. Chemoreduction: Systemic chemotherapy is used to reduce tumor volume with intraocular tumors making them suitable for treatment with cryotherapy or photocoagulation.13,14 Factors such as tumor location (macula), patient age, and tumor size correlate with responsiveness to chemotherapy.15,16 Most tumors are treated with combination chemotherapy, Inj.Vincristine, Inj. Etoposide and Inj. Carboplatin. The dose of these drugs depends on the age, stage and the intention of treatment, either chemoreduction or adjuvant chemotherapy (Table 1). They also require additional local therapy. Overall, the response rate is highest for tumors that are unilateral or unifocal and without vitreous seeding. 4. Subtenon (subconjunctival) chemotherapy: Carboplatin is administered by the treating ophthalmologist into the subconjunctival space. This modality is undergoing testing in phase I and II trials and is generally used in conjunction with systemic chemotherapy and local ophthalmic therapies for retinoblastoma with vitreous seeding. This approach offers some promise in this group of patients.17,18 5. Surgery (enucleation) is usually undertaken when unilateral disease is massive and there is no expectation that useful vision can be preserved. Careful examination of the enucleated specimen by an experienced pathologist

is necessary to determine whether high-risk features for metastatic disease are present. Postoperative external radiation therapy is indicated in the presence of optic nerve extension to transection, scleral infiltration and extrascleral extension. Systemic standard chemotherapy for six cycles is indicated for anterior chamber seeding, infiltration of iris, ciliary body infiltration, massive choroidal infiltration or optic nerve extension beyond lamina cribrosa. High dose chemotherapy for 6 cycles is used in patients with combined choroidal infiltration and optic nerve extension beyond lamina cribrosa or with scleral infiltration. High dose chemotherapy for 12 cycles is used in patients with extrascleral extension and optic nerve extension to transection. Vincristine, doxorubicin, and cyclophosphamide, or vincristine, carboplatin, and etoposide are the drugs in different dose schedules as described in (Tables 2 and 3). Intrathecal methotrexate is given if CSF is positive or there is radiological evidence of intracranial extension. 6. External beam radiotherapy: Retinoblastoma is generally a radiosensitive tumor. Presently, with effective chemotherapy drugs the indications for radiotherapy are minimized to avoid the late effects of radiotherapy, like facial deformities and second malignancies. External beam radiotherapy is a method of delivering whole eye irradiation to treat advanced retinoblastoma, particularly when there is diffuse vitreous seeding. Presently, the indications of external radiotherapy are residual disease after chemo-

Table 1: Chemoreduction regimen Standard dose regimen Inj. Vincristine 1.5 mg/m2

(0.05 mg/kg for children
Day 1

Inj. Etoposide 150 mg/m2

(5 mg/kg for children
Day 1+2

Inj. Carboplatin 560 mg/m2

(18.6 mg/kg for children
Day 1

(0.05 mg/kg for children
Day 1

(12 mg/kg for children
Day 1+2

(28 mg/kg for children
Day 1

High dose chemotherapy Inj. Vincristine 1.5 mg/m2 Inj. Etoposide 250 mg/m

2

Inj. Carboplatin 750 mg/m

2

Management of Ophthalmic Tumors: Role of Chemotherapy and Radiation Therapy 351 Table 2: Management of intraocular retinoblastoma group I to IV-B a. Focal therapy alone when appropriate b. Standard chemoreduction × 6 cycles + appropriate sequential focal therapy c. Chemocryotherapy at each cycle d. Standard chemoreduction × 12 cycles, when suboptimal regression with chemotherapy at 6 cycles and RT is not feasible (e.g. age < 1year) e. Alternative chemotherapy regimen or RT when there is no regression after 3 cycles of standard chemotherapy or recurrence during chemotherapy. Table 3: Management of intraocular retinoblastoma group V-A and V-B a. Unilateral – Primary enucleation b. Bilateral–High dose chemotherapy × 6 cycles + appropriate sequential focal therapy c. Chemocryotherapy at each cycle d. Periocular Carboplatin augmentation for group V-B e. High dose chemoreduction × 12 cycles when suboptimal regression with chemotherapy at 6 cycles and RT is not feasible (e.g. age < 1year)

therapy and local therapy, diffuse vitreous seeds, recurrent after chemotherapy, post enucleation (Scleral involvement, extraocular extension, optic nerve involvement). Externalbeam radiation with dose ranges from 3,500 to 4,600 cGy. Special expertise is very essential to treat pediatric ocular and orbital tumors. Newer methods of delivering external-beam radiation are being used in an attempt to reduce adverse long-term effects. This includes intensitymodulated radiation therapy (IMRT), stereotactic radiation therapy, and proton-beam radiation therapy.19 7. Brachytherapy with radioactive plaques. These are radioactive plaques in concave shapes, either beta or gamma emitting. The commonly used radioactive sources are Iodine125, Gold, Iridium192 and Ruthenium106. Ruthenium and 125I plaque therapy is preferred because of its favorable physical properties.20,21 They are used for either focal unilateral presentations or recurrent disease following previous externalbeam radiation. Indications of brachytherapy are lesion < 16 mm in basal diameter, < 8 mm in thickness, adjuvant to chemo reduction, failure of local therapy.

Extraocular disease may be localized to the soft tissues surrounding the eye or to the optic nerve beyond the margin of resection. However, further extension may occur into the brain and meninges with subsequent seeding of the spinal fluid, as well as distant metastatic disease involving the lungs, bones, and bone marrow. High dose chemotherapy for 3 to 6 cycles are given and disease is reassessed. Enucleation is performed followed by orbital external beam radiation therapy. Further, high dose chemotherapy is continued for total 12 cycles. In extraorbital retinoblastoma, palliative therapy with radiation (including craniospinal irradiation when there is meningeal involvement) and/or intrathecal chemotherapy with methotrexate, cytarabine, and hydrocortisone, plus supportive care have been used (Tables 4 and 5). Table 4: Management of extraocular retinoblastoma a. Baselins CT scan or MRI; bone marrow and CSF cytology b. Intrathecal methotrexate if CSF is positive or there is radiological evidence of intracranial extension c. High dose chemotherapy for minimum three cycles and reassess d. Enucleation and continue high dose chemotherapy for 6-12 cycles and external beam radiation therapy (see post-enucleation protocol) e. If systemic metastasis at the time of presentation, modify timing of local therapy depending on the extent of tumor. Table 5: Post-enucleation adjuvant treatment protocol a. Systemic standard chemotherapy for 6 cycles for 1. anterior chamber seeding, 2. infiltration of iris, 3. ciliary body infiltration, 4. massive choroidal infiltration 5. optic nerve extension beyond lamina cribrosa. b. High dose chemotherapy for 6 cycles in patients with 1. combined choroidal infiltration 2. optic nerve extension beyond lamina cribrosa or 3. with scleral infiltration. High dose chemotherapy for 12 cycles is used in patients with extrascleral extension and optic nerve extension to transection. c. High dose chemotherapy for 12 cycles in patients with 1. extrascleral extension 2. optic nerve extension to transection.

352 Surgical Atlas of Orbital Diseases With emerging dose-intensive chemotherapy regimens and the use of high-dose chemotherapy with autologous stem cell rescue, clinical trials are ongoing to improve the dismal outcome for this relatively small group of patients. The agents used in the past included vincristine, cyclophosphamide, and doxorubicin; although they produce an initial response, overall survival has been less than optimal. Carboplatin, ifosfamide, and etoposide have shown more promise for remission and may be used in conjunction with high-dose chemotherapy followed by stem cell rescue. Patients presenting with extensive non-CNS metastases have been treated successfully with myeloablative chemotherapy with stem cell rescue.

Ocular Metastasis Intraocular metastasis is now considered the most common malignancy of the eye. The frequency of ocular metastasis varies significantly among primary sites. Ocular metastasis, and particularly choroidal metastasis, can precede the diagnosis of the primary malignancy. Lung cancer is the most common primary tumor (35 and 41%) detected in patients with no neoplasm at the time of ocular diagnosis followed by breast cancer, leukemia, lymphoma, multiple myeloma and sarcoma. Rarely metastases from malignancies of prostate, cervix, thyroid, skin, GI tract and kidney can occur. A number of options are available for the therapy of ocular metastasis, including observation, chemotherapy, photocoagulation, cryosurgery, surgical resection, or radiotherapy. The specific therapy chosen for a patient is an individualized process that considers the clinical condition of the patient. The most commonly applied treatment is external-beam radiotherapy. In general, 30 to 40 Gy in 10 to 20 fractions could be considered a standard course of radiotherapy. For patients with a long life expectancy, a higher total dose with lower dose per fraction can be considered.

Orbital Tumors Pediatric primary orbital masses include dermoid cyst, capillary hemangioma and lymphangioma, inflammatory lesions, lymphocytic and leukemic infiltrates, and pilocytic astrocytoma of the optic nerve, rhabdomyosarcoma and primary neuroectodermal tumor or Ewing's sarcoma. In adults, the

most common localized tumors of the orbit include cavernous hemangioma, fibrous histiocytoma, schwannoma, orbital pseudotumor, Grave's ophthalmopathy and hemangiopericytoma. The most common lacrimal gland tumors include pleomorphic adenoma and adenoid cystic carcinoma.

Rhabdomyosarcoma Orbital rhabdomyosarcoma is treated with combination of chemotherapy and radiation therapy after local excision or biopsy. Chemotherapy is usually given for 2 to 3 cycles prior to the initiation of radiation therapy, with the exception of patients with parameningeal disease and evidence of meningeal extension in whom radiation therapy generally begins as soon as possible. Vincristine, cyclophosphamide, doxorubicin, actinomycin-D, ifosfamide and etoposide are the drugs used for the treatment of RMS. Radiation therapy is effective for achieving local control of tumor for patients with microscopic or gross residual disease following biopsy, initial surgical resection, or chemotherapy. The radiation therapy dose depends predominantly on the extent of disease following the primary surgical resection. Patients with completely resected tumors (group I) of embryonal histology do well without radiation therapy but radiation therapy benefits patients with group I tumors with alveolar or undifferentiated histology.22-24 In general, patients with microscopic residual disease (group II) receive radiation therapy to approximately 4,100 cGy. 25 Patients with gross residual disease (group III) should receive radiation dose of 5,040 cGy. The treated volume should be determined by the extent of tumor at diagnosis prior to surgical resection and prior to chemotherapy. A margin of 2 cm is generally used, including clinically involved nodes. Precautions should be taken to limit the dose to the lens, cornea, lacrimal gland, and optic chiasm.

Orbital Lymphoma Most of the orbital lymphomas are confined to the orbit and are of low grade. Patient needs a staging workup (CBP, chest X-ray, USG abdomen, or CT scan of chest and abdomen, serum LDH, Bone marrow biopsy and CSF cytology) to rule out systemic lymphoma. Radiotherapy is a well-established treatment modality for orbital lymphoma. Primary chemotherapy has minimal efficacy in localized low-

Management of Ophthalmic Tumors: Role of Chemotherapy and Radiation Therapy 353 grade orbital lymphoma and thus is not advocated as a first-line treatment. There have been numerous series advocating low dose radiation for treatment of orbital lymphomas. In general, radiation dose of 3000 cGy is recommended for low grade lymphomas and 4000-4500 cGy for intermediate grade lymphomas.26,27 If it is associated with systemic disease, it is treated with chemotherapy constituting cyclophosphamide, doxorubicin, vincristine and prednisolone for 6 cycles followed by local radiation therapy.

Idiopathic Orbital Inflammation (IOI) Idiopathic Orbital Inflammation could be inflammatory, reactive lymphoid hyperplasia or atypical lymphoid hyperplasia. Corticosteroids have been the recommended initial drug (prednisone 1 mg/kg/d), Recently, antimetabolites (azathioprine, methotrexate, and leflunomide), T-cell inhibitors (cyclosporine and tacrolimus), and alkylating agents (cyclophosphamide and chlorambucil) were shown to be useful in the management of NSOI in different series.28 Use of low-dose external beam radiation 2000 cGy demonstrated a 50 to 80% efficacy in a previous series.29

Grave's Ophthalmopathy Severe exophthalmos may occur in some patients with thyrotoxicosis with involvement of extraocular muscles. Indications for therapy are corneal exposure which may cause corneal ulceration that progress to scarring, optic nerve compression. CT scan shows thickened extraocular muscles. Steroids and diuretics are the first-line treatment, administered for 2 weeks. Radiation dose of 2000 cGy in 10 fractions provides good symptomatic relief avoiding the need for further steroid therapy or surgical decompression.30

Optic nerve Meningioma The diagnosis of optic nerve meningioma is usually presumptive and based on the appropriate clinical picture supported by appropriate neuroimaging. Biopsy is not routinely advocated, as surgical intervention carries significant morbidity and mortality. Patients often undergo reimaging at 3 months, and they are followed radiographically at 6 to 12 month intervals after the disease has stabilized. Treatment strategy should be individualized.

Radiation therapy is recommended as soon as serial examination documents a new decline in acuity and/ or visual field. Tumor enlargement without loss of visual function, as determined by serial imaging, may also provide an indication for radiotherapy. Recommended dose of radiation therapy is 5400 cGy. 31 It should preferably be delivered via fractionated, 3-dimensional stereotactic and IMRT techniques that provide the most precise conformal application of the dose to affected tissues. Theoretically, this approach should reduce the risk of side effects to surrounding radiosensitive ocular and neural tissues.

Optic Nerve Glioma Chemotherapy is the first-line treatment, followed by radiation if chemotherapy fails. Standard chemotherapy for optic-pathway gliomas consists of vincristine and carboplatin, whereas second-line therapy is often thioguanine, procarbazine, and vincristine. 32 When these fail, chemotherapeutic agents used in other progressive low-grade gliomas can be considered. These include cyclophosphamide, topotecan, and oral VP-16.Stabilization or improvement in visual function and tumor size is considered a response to treatment. Surgery has a limited role. Biopsies are performed when clinical and radiologic features are atypical. Radiation therapy dose above 5000 cGy is required for tumor control. Optic-nerve gliomas should be operated upon only when grossly proptotic and the eye is blind or near blind. In large series from the Mayo Clinic with a median followup time of 10 years, patients with glioma confined to the optic nerve survived almost twice as long as those with involvement of optic chiasm.33

Sequelae of Radiation Therapy 34-38 Postradiotherapy complications can be classified as acute (usually occurring within 3 months of treatment) or late (occurring many months to years after completion of treatment). Acute lesions generally affect rapidly proliferating cells, and most can be reversed by appropriate medical management. Such acute effects involving the ocular anterior segment include blepharitis, conjunctivitis, and keratitis. However, residual stromal lesions and interstitial fibrosis may follow. Late effects are primarily caused by permanent vascular damage and resultant

354 Surgical Atlas of Orbital Diseases ischemia. Retinopathy, cataract, and optic neuropathy are examples of such late effects. (Table 6) Keratoconjunctivitis sicca, another late sequela to radiotherapy, may often be clinically nonmanifest or insignificant but can result in ulcerative thinning and corneal perforation. Eyelid and periorbital skin radiation effects can be acutely controlled with topical corticosteroids, wound debridement, and antibiotic therapy. Occasionally, reconstructive surgery is indicated to treat lid deformities. Patients should be encouraged to wear ultraviolet protective sunscreens and avoid using harsh soaps and lotions. Nasolacrimal duct occlusion may require silicone intubation or dacrycryocysto-rhinostomy, whereas severe lacrimal punctal stenosis may necessitate a conjunctivo-dacryocystorhinostomy. Severe noninfectious inflammation may require a short course of corticosteroid therapy, but indiscriminate use of steroids should be avoided because it can promote extracellular matrix Table 6: Radiation effects on the eye and orbital tissues Eye lashes and Eyelid Spared by megavoltage (Cobalt, LA) Becomes thinner; function not altered Lash loss at 40–60 Gy Telangiectasia at 55 Gy Lacrimal system

Dryness in 8-25% pts at 30 to 45 Gy Dryness in all pts over 4-8 yrs at >55 Gy Atrophy at 50–60 Gy Stenosis at 65–75 Gy

Lens

EBRT

Single dose 2 Gy -Cataract Fractionated 8 Gy Cataract in 33% of pts > 11 Gy – Cataract in 100% of pts < 50 Gy – Cataract; vision not impaired > 60 Gy – Vision impairing cataracts

Plaque

50 Gy at limbus 33% cataracts

Conjunctiva

Conjunctivitis at 55–75 Gy Telangiectasia at 30 Gy

Cornea

Superficial keratitis- edema, epithelial defects at 30–50Gy Severe keratitis – Ulcer, scarring, perforation > 60Gy

Retina

40-60 Gy – retinopathy 10% > 60 Gy – retinopathy 30% CRA thrombosis may lead to edema and pallor of optic disc, retinal hemorrhages, blindness in 2-3 years

Optic nerve

Neuropathy at >55 Gy

breakdown. Prolonged ocular surface inflammation or ulceration frequently requires prophylactic antibiotics. Artificial tears and ointments are indicated for dry eye relief. It is important to recognize that the irradiated cornea often has a poor capacity to heal, despite neovascularization, because of the degree of epithelial toxicity. Mild punctate keratopathy needs aggressive lubrication. Tear replacement therapy with nonpreserved artificial tears and ointments facilitates epithelial wound healing. Infected corneal ulcers require prompt diagnostic and therapeutic measures, with initiation of broadspectrum antibiotics modified as needed on culture and susceptibility results. Although hydrophilic soft contact lenses can be used as protective bandaging to promote corneal healing, they may not be well tolerated in severe dry eyes; furthermore, they may increase the risk of an infection in patients who are often additionally immunosuppressed by chemotherapy. Gas-permeable glued-on contact lenses have been used to treat radiation-induced keratitis effectively, but experience with this is limited. If necessary, a conjunctival flap can control severe pain caused by persistent corneal defects.

REFERENCES 1. Jacobeic FA, Jones IS. Vascular tumours, malformations and degenerations. In: Duan TD, ed. Clinical ophthalmology, vol 2. Hagerstown, MD: Haper and Row, 1976;1-40. 2. Fitzpatrick PJ, Thompson GA, Easterbrook WM, Gallie BL, Payne DG. Basal and squamous cell carcinoma of the eyelids and their treatment by radiotherapy. Int J Radiat Oncol Biol Phys. 1984;10(4):449-54. 3. Luxenberg MN. Sebaceous gland carcinoma. Arch Ophthalmol 1988;106:119. 4. Tan KC, Lee ST, Cheah ST. Surgical treatment of sebaceous carcinoma of eyelids with clinico-pathological correlation. Br J Plast Surg 1991;44:117-21. 5. Pardo FS, Wang CC, Albert D, et al. Sebaceous carcinoma of the ocular adnexa: radiotherapeutic management. Int J Radiat Oncol Biol Phys 1989;17:643-7. 6. Meldrum ML, Tse DT, Benedetto P. Neoadjuvant intracarotid chemotherapy for treatment of advanced adenocystic carcinoma of the lacrimal gland. Arch Ophthalmol. 1998;116(3):315-21. 7. Tse DT, Benedetto P, Dubovy S, Schiffman JC, Feuer WJ. Clinical analysis of the effect of intraarterial cytoreductive chemotherapy in the treatment of lacrimal gland adenoid cystic carcinoma. Am J Ophthalmol. 2006.

Management of Ophthalmic Tumors: Role of Chemotherapy and Radiation Therapy 355 8. Journée-de Korver JG,Keunen JEE. Thermotherapy in the management of choroidal melanoma. Prog Retin Eye Res. 2002;21:303- 17. 9. Shields CL, Cater J, Shields JA. Combined plaque radiotherapy and transpupillary thermotherapy for choroidal melanoma: tumor control and treatment complications in 270 consecutive patients. Arch Ophthalmol. 2002;120:933-40. 10. Nath R, Anderson LL, Luxton G, et al. Dosimetry of interstitial brachytherapy sources: recommendations of the AAPM Radiation Therapy Committee Task Group No 43. American Association of Physicists in Medicine. Med Phys. 1995;22:209-34. 11. Jorge E Freire, Luther W. Brady. Jerry A. Shields, Carol L. Shields, Eye and Orbit, Principles and practice of Radiation Oncology, (4th ed), 2004;876-96. 12. Shields CL, Santos MC, Diniz W, et al.: Thermotherapy for retinoblastoma. Arch Ophthalmol 1999;117 (7): 885-93. 13. Friedman DL, Himelstein B, Shields CL, et al. Chemoreduction and local ophthalmic therapy for intraocular retinoblastoma. J Clin Oncol 18 (1): 12-7, 2000. 14. Shields CL, Honavar SG, Meadows AT, et al. Chemoreduction plus focal therapy for retinoblastoma: factors predictive of need for treatment with external beam radiotherapy or enucleation. Am J Ophthalmol 2002;133 (5): 657-64. 15. Lumbroso L, Doz F, Urbieta M, et al.: Chemothermotherapy in the management of retinoblastoma. Ophthalmology 2002;109(6):1130-6. 16. Gombos DS, Kelly A, Coen PG, et al.: Retinoblastoma treated with primary chemotherapy alone: the significance of tumour size, location, and age. Br J Ophthalmol 2002;86 (1): 80-3. 17. Abramson DH, Frank CM, Dunkel IJ: A phase I/II study of subconjunctival carboplatin for intraocular retinoblastoma. Ophthalmology 1999;106(10):1947-50. 18. Villablanca JG, Jubran R, Murphree AL: Phase I study of subtenon carboplatin I with systemic high dose carboplatin/etoposide/vincristine (CEV) for eyes with disseminated intraocular retinoblastoma (RB). [Abstract] Proceedings of the XIII Biannual Meeting of ISGED and the X International Symposium on Retinoblastoma, 2001, Fort Lauderdale, Fla. USA. 19. Krasin MJ, Crawford BT, Zhu Y, et al.: Intensity-modulated radiation therapy for children with intraocular retinoblastoma: potential sparing of the bony orbit. Clin Oncol (R Coll Radiol) 2004;16 (3): 215-22. 20. Shields CL, Shields JA, Cater J, et al.: Plaque radiotherapy for retinoblastoma: long-term tumor control and treatment complications in 208 tumors. Ophthalmology 2001;108(11):2116-21. 21. Merchant TE, Gould CJ, Wilson MW, et al.: Episcleral plaque brachytherapy for retinoblastoma. Pediatr Blood Cancer 2004;43(2):134-9.

22. Maurer HM, Beltangady M, Gehan EA, et al.: The Intergroup Rhabdomyosarcoma Study-I. A final report. Cancer 1988;61(2):209-20. 23. Maurer HM, Gehan EA, Beltangady M, et al.: The Intergroup Rhabdomyosarcoma Study-II. Cancer 1993;71 (5):1904-22. 24. Wolden SL, Anderson JR, Crist WM, et al.: Indications for radiotherapy and chemotherapy after complete resection in rhabdomyosarcoma: A report from the Intergroup Rhabdomyosarcoma Studies I to III. J Clin Oncol 1999;17 (11):3468-75. 25. Raney R, Hays D, Tefft M, et al.: Rhabdomyosarcoma and the undifferentiated sarcomas. In: Pizzo PA, Poplack DG, Eds.: Principles and Practice of Pediatric Oncology. Philadelphia: JB Lippincott, 1989;635-58. 26. Esik O, Ikeda H, Mukai K, Kaneko A. A retrospective analysis of different modalities for treatment of primary orbital non-Hodgkin's lymphomas. Radiother Oncol 1996; 38:13-8. 27. Jorge E Freire, Luther W. Brady. Jerry A. Shields, Carol L. Shields, Eye and Orbit, Principles and practice of Radiation Oncology, (4th ed) 2004;876-96. 28. Cockerham KP, Hong SH, Browne EE: Orbital inflammation. Curr Neurol Neurosci Rep 2003;3:401-9. 29. Smitt MC, Donaldson SS: Radiation therapy for benign disease of the orbit. Semin Radiat Oncol 1999;9:179-89. 30. Sandler HM, Rubenstein JH, Fowble BL, Sergott RC, Savino PJ, Bosley TM, Results of radiotherapy for thyroid ophthalmopathy. Int J Radiat Oncol Biol Phys. 1989;17(4):823-7. 31. Tsao MN, Hoyt WF, Horton J, et al. Improved visual outcome with definitive radiation therapy for optic nerve sheath meningioma. Int J Radiat Oncol Biol Phys. 1991;45S:324-25. 32. Jahraus CD, Tarbell NJ. Optic pathway gliomas. Pediatr Blood Cancer 2006;46:586-96. 33. Rush JA, Younge BR, Campbell RJ, et al. Optic glioma: long-term follow-up of 85 histopathologically verified cases. Opthalmology 1982;89:1213-9. 34. Brady LW, Shields J, Augsburger J et al. Complications from radiotherapy to the eye. Front Radiat Ther Oncol 1989;23:238-50. 35. Servodido CA, Abramson DH. Acute and long-term effects of radiation to the eye in children. Cancer Nurs 1993; 16: 371-81. 36. Merriam GR, Szechtzer A, Focht EF. Theeffects of ionizing radiations to the eye. Front Radiat Ther Oncol 1972; 6: 346-85. 37. Monroe AT, Bhandare N, Morris CG, Mendenhall WM. Preventing radiation retinopathy with hyperfractionation. Int J radiat Oncol Biol Phys 2005; 61: 856-64. 38. Bhandare N, Monroe AT, Morris CG et al. Does altered fractionation influence therisk of radiation-induced optic neuropathy? Int J radiat Oncol Biol Phys 2005;62:1070-7.

356 Surgical Atlas of Orbital Diseases

26

CHAPTER

Carotid-Cavernous Fistulae: Role of Interventional Radiologist D Ravi Varma, D Radhika Varma

The cavernous segment of the carotid artery is unique, as it is the only anatomical location in the body where an artery is completely surrounded by a venous structure. Carotid-cavernous fistulae (CCF) are spontaneous or acquired communications between the carotid artery and the cavernous sinus without an intervening capillary bed. The arterial supply to these lesions may come from the internal carotid artery (ICA), from the dural branches of external carotid artery (ECA), or from both. The cavernous sinus, being at the crossroads of the cranial and facial

venous circulations, has rich communications with the facial veins through the superior and inferior ophthalmic veins, with the transverse and sigmoid sinuses through the superior and inferior petrosal sinuses, with the cerebral cortical veins through the sphenoparietal sinus and with the pterygoid plexus (Figure 26.1). Thus these lesions can have a wide range of clinical presentations, the severity of which is dependent not only on the volume of flow across the fistula site, but also on the adequacy and preferred pathway of venous drainage.

1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18. 19.

Common carotid artery, Internal carotid artery, External carotid artery, Dural branches of external carotid artery supplying the cavernous sinus, Cavernous segment of internal carotid artery, Dural branches of internal carotid artery (meningiohypophyseal trunk and inferior cavernous sinus artery), Cavernous sinus, Superior petrosal sinus, Inferior petrosal sinus, Superior ophthalmic vein, Inferior ophthalmic vein, Facial vein, Pterygoid venous plexus, Sphenoparietal sinus, Superior sagittal sinus, Deep venous system, Transverse sinus, Sigmoid sinus, Internal jugular vein.

Figure 26.1: Arterial and venous anatomy around the cavernous sinus

Carotid-Cavernous Fistulae: Role of Interventional Radiologist 357

A

B

C

(A) Type A CCF: Internal carotid angiogram in lateral projection reveals opacification of cavernous sinus (arrow) and retrograde flow in the superior ophthalmic vein (double arrow), through a rent in the arterial wall. Note that there is immediate opacification of the entire cavernous sinus and superior ophthalmic vein during the arterial phase as is common with these high flow lesions (B) Type B CCF: Internal carotid angiogram in lateral projection reveals opacification of cavernous sinus (double arrow) through small dural branches (arrow) arising from the cavernous segment of internal carotid artery. In these lesions, there is a significant delay in opacification of the cavernous sinus and its draining veins – suggesting a slow flow across the fistula (C) Type C CCF: Right external carotid angiogram in frontal projection reveals opacification of the right and left cavernous sinuses (arrows) through multiple small dural branches of the middle and accessory meningeal arteries (double arrow). Though the CCF was located on the right side, this middle aged lady was symptomatic in the left eye as the predominant venous drainage was through the left superior ophthalmic vein. Type D CCF has arteriovenous fistulae with supply from dural branches of internal and external carotid arteries Figures 26.2A to C: Barrow classification of CCF

The widely used system to classify CCF is based on their angioarchitecture. Barrow in 1985 classified these lesions based on their arterial supply1 (Figures 26.2A to C). Type A CCF are direct fistulae between the ICA and the cavernous sinus, and are most commonly secondary to trauma. Type B, type C and type D CCFs, are low flow indirect fistulae where multiple microfistulae are located within the wall of the cavernous sinus and drain into it. The arterial supply to these lesions may originate from the dural branches of ICA (Type B), dural branches of ECA (Type C) or from dural branches of ICA and ECA (Type D). (The salient features differentiating direct and indirect CCF are depicted in Table 1).

PATHOPHYSIOLOGY The elevated pressure in the cavernous sinus leads to dramatic orbital changes as the superior and inferior orbital veins are devoid of valves. Though most obvious manifestations of CCF are reflected in the eye, we should not forget to look for other less evident changes which may be vital clues to the diagnosis, or may result in disaster if overlooked.

Symptoms such as headache may indicate elevation of cerebral cortical venous pressure and may result in potentially fatal complications such as cerebral edema, intracerebral or subarachnoid hemorrhage. The pathophysiological mechanisms behind the common clinical symptoms are detailed in Figure 26.3.

Clinical Features CCF that have principal drainage into the superior and inferior ophthalmic veins, present with predominant orbital symptoms. 2 Though most patients have orbital symptoms on the same side as the fistula, we have seen several patients with unilateral CCF in whom the orbital symptoms were present on the contralateral side, or even on both sides, depending on the available paths of venous drainage (Figure 26.2C). The symptoms and clinical signs of CCF and their relative incidence are listed in Table 2.2,5 The clinical signs may be difficult to differentiate from other sequelae of craniocerebral trauma such as orbital hematomas and cranial nerve injuries. There should be a high index of suspicion when a patient

358 Surgical Atlas of Orbital Diseases

Figures 26.3: Pathophysiology of CCF

presents with orbital symptoms after a head injury. The low flow at the shunt and nonspecific nature of symptoms makes indirect CCF even more difficult to diagnose. A useful clinical test to identify these

lesions is to look for reduction of symptoms with digital compression of the carotid artery in the neck.

Table 1: Comparison of direct and indirect CCF Sex prediliction Etiology

Pathology Hemodynamics Arterial supply and Barrow Classification Type Symptoms

Spontaneous closure

Direct

Indirect

Male (Higher incidence of trauma) — Trauma (commonest) — Ruptured cavernous ICA aneurysm — Surgical trauma — Ehler’s Danlos syndrome — Fibromuscular dysplasia — Arterial dissection — Idiopathic Rent in the wall of the cavernous segment of internal carotid artery Usually high flow Cavernous segment of ICA (Type A)

Commoner in female — Spontaneous (commonest) — Cause unknown — Predisposing factors • Cavernous sinus thrombosis • Pregnancy • Trauma • Sphenoid sinusitis Abnormal small dural arterio-venous shunts in the wall of the cavernous sinus Usually low flow — Dural branches of ICA (Type B) — Dural branches of ECA (Type C) — Combination of dural branches of ECA and ICA (Type D) Usually insidious onset, with slow progression

May be abrupt following trauma or may present after a delay of days or weeks. Usually progress rapidly. Extremely rare

May be achieved in 34-60% by conservative management

Carotid-Cavernous Fistulae: Role of Interventional Radiologist 359 Table 2: When to suspect CCF Past History • Craniocerebral trauma • Cavernous sinus thrombosis • Connective tissue disorders Symptoms • Swollen red eye • Orbital pain • Diplopia • Progressive vision loss • Headache • Pulsatile tinnitus

Signs • Proptosis • Ptosis • Chemosis • Cranial nerve palsies • Elevated intraocular pressure • Papilledema • Optic nerve atrophy

Rare presentations • Neurological deficits • Intraparenchyma / Subarachnoid bleed • Epistaxis

CASE ILLUSTRATION (Figure 26.4) Radiological Investigations

In a patient with clinical features of CCF, contrast enhanced CT scan is most often adequate to confirm the diagnosis. In addition to the characteristic orbital and cavernous sinus features (Figures 26.5A to C), skull base fractures and bony spicules are demonstrated. Though CT angiography, MRI and MR angiography (Figures 26.6A to C) elegantly demonstrate the abnormality, they add little in term of diagnosis or treatment planning, as the exact site of fistula and hemodynamics of flow across it are

A

B

Figure 26.4: Clinical features of CCF. Mr. P (same patient as in Figure 26.2A) was referred to us with proptosis of left eye that progressed over 4 months following a road traffic accident. Note the proptosis, orbital congestion and conjunctival chemosis. He had elevated intraocular pressure and had lost vision in the left eye. As is common in most patients with CCF, his presenting complaints were limited to the orbit. Only on specific queries, he admitted that he often heard a pulsatile "whooshing" sound in the left ear and that he had severe headaches that appeared after the head injury

rarely demonstrated. Duplex Doppler studies of the orbit are useful in follow-up of lesions that are on conservative management or have undergone partial occlusion. Definitive planning of treatment requires digital subtraction angiography with selective injections of the internal and external carotid arteries on either side. Information regarding the arterial supply, site and size of the fistula, volume of flow across the fistula, patency of cavernous sinus, pattern of venous drainage and adequacy of collateral circulation at the circle of Willis can be obtained on this study.

C

Figures 26.5A to C: CT scan findings in CCF Plain (A) and contrast enhanced (B) axial CT scans of the orbit in a 24-year-man with posttraumatic direct CCF, reveal proptosis (arrow), enlarged extraocular muscles (arrow heads) and prominent cavernous sinus (double arrow) on the left side. Dilatation of the superior ophthalmic vein (double arrowhead) should be specifically looked for in such cases (B) as it may be the only indicator of an underlying vascular abnormality. This characteristic appearance has been termed as the "Hockey stick" sign. Coronal CT scan (C) in another patient with post-traumatic direct CCF, reveals enlarged extraocular muscles (arrow heads) and dilated superior ophthalmic vein (arrow) in the right eye. In subtle cases, comparison with the normal structures in the contralateral orbit will help in identification

360 Surgical Atlas of Orbital Diseases

A

B

C

Figures 26.6A to C: MR and MR Angiographic features of CCF. 21-year-old man with history of head injury presented with pulsatile proptosis. Axial T2 (A) and T1 (B) weighted MR scans of the brain reveal dilatation of the superior ophthalmic vein (arrow) and prominence of cavernous sinus (double arrow) on left side. On MRI, high velocity blood flow produces "flow voids" within blood vessels that usually appear black on all sequences. Basal projection of MR Angiogram (C) reveals flow from the left internal carotid artery (arrow) into the left cavernous sinus (double arrow). The cavernous sinus is seen to drain into inferior petrosal sinuses (arrow head) and superior ophthalmic veins (double arrowhead) on either side

Management of CCF Management of CCF must start with treating its secondary manifestations such as glaucoma. Aggressive medical management of the elevated intraocular pressure with adrenergic blockers or acetazolamide should be started while definitive treatment should be directed towards closing the fistula. Surgical measures such as lateral canthotomy or tarsorrhaphy may be used to decompress the orbit and to prevent exposure keratopathy.

Direct CCF High flow lesions such as Type A CCF usually progress rapidly and may result in vision loss, ophthalmoplegia, elevated intracranial pressure and intracranial hemorrhage. Spontaneous thrombosis is extremely rare and these lesions must be managed without delay. The indications of emergency treatment4 of CCF are listed in Table 3. Surgical ligation of the carotid artery is ineffective in treating these lesions and may infact worsen the neurological symptoms, as the fistula steals blood from the intracranial circulation. On the other hand, endovascular management seals off the fistula and preserves the patency of the carotid artery.

Occlusion of the fistula using silicone or latex detachable balloons is the treatment of choice for direct CCF3 (Figures 26.7A to F). These balloons are negotiated through the rent in the wall of the artery, into the cavernous sinus and are inflated so as to seal off the fistula. After confirming satisfactory position of the balloon and verifying the patency of the carotid artery, the balloon is detached (Figures 26.8A to L). Though these balloons eventually deflate, they occlude the fistula long enough to cause thrombosis within the fistula and cavernous sinus. Transarterial balloon embolization has been reported to be successful in 80-90% of cases. Table 3: Indications for emergency management of CCF Clinical features • Epistaxis • Elevated intracranial pressure • Progressive proptosis • Diminishing visual acuity • Intraocular pressure > 40 mm Hg • Transient ischemic attacks Imaging features • Presence of cortical venous drainage • Pseudoaneurysm / cavernous sinus varix

Carotid-Cavernous Fistulae: Role of Interventional Radiologist 361

Figures 26.7A to F: Technique of detachable balloon embolization of CCF. The delivery catheter (A) is 165 cm long. The stiff proximal shaft is 0.86 mm in diameter while the distal supple part is 0.57 mm in diameter. (B) The valve of the detachable balloon is threaded onto the tip of the delivery catheter and the assembly (C) is introduced into the carotid artery. Once the balloon crosses the fistula and enters the cavernous sinus, it is inflated by injecting contrast medium into the hub (D), so that the balloon takes the shape of the cavernous sinus (E) and occludes the orifice of the fistula. Once satisfactory occlusion of the fistula is achieved, the balloon is detached by gentle traction on the delivery catheter (F)

Figure 26.8A: 29-year-old man who was operated for a traumatic extradural hematoma, presented 8 months later, with proptosis, diplopia and right sided headache. He had loud bruit on auscultation over the right orbit. Note the ptosis, proptosis and lateral deviation of eyeball on the right side

B

C

Figures 26.8B and C: His brain CT (B) revealed a prominent superior ophthalmic vein (double arrowheads) on right side. Diagnostic right internal carotid angiogram (C) showed opacification of cavernous sinus (bold arrow), with absent flow into distal branches of internal carotid artery. From the cavernous sinus, blood was seen to flow retrogradely through the superior ophthalmic vein (double arrowheads) into the orbit, into the inferior petrosal sinus (long arrow) and into cortical veins (double arrows). As he had high flow towards the orbit and significant flow into the cortical veins, we planned emergency endovascular management. Such large hole direct CCF are best treated with trans-arterial embolization using detachable balloons

362 Surgical Atlas of Orbital Diseases

D

E

Figures 26.8D and E: We threaded a detachable balloon (9 x 14 mm size) onto a delivery catheter (D) and introduced the assembly (arrow) through a guiding catheter (arrow heads) into the internal carotid artery (E)

F

G

H

Figures 28.8F to H: We negotiated the balloon across the orifice of the fistula and placed it in the cavernous sinus (arrow head). We slowly inflated the balloon with contrast medium, with frequent check angiograms (F). On complete inflation of the balloon, though we could achieve cessation of flow in the cortical veins, flow persisted in the superior ophthalmic vein as seen on check angiogram (G) and follow-up Doppler study (H)

I

J

Figures 26.8I and J: We negotiated another detachable balloon (arrow head) through the fistula into the anterior part of the cavernous sinus (I) and inflated it so that flow in the superior ophthalmic vein was arrested (J). The ophthalmic artery (arrow) and intracranial branches of internal carotid artery (arrow heads) are seen to fill now

Carotid-Cavernous Fistulae: Role of Interventional Radiologist 363

K

L

Figures 26.8K and L: In the weeks following the embolization we confirmed the absence of flow in the superior ophthalmic vein on followup Doppler studies of the orbit (K). Though Doppler gives us information only about the orbital component of CCF, it is an inexpensive and reliable modality to confirm the efficacy of treatment. Plain radiograph of the skull performed a month after treatment (L) showing the two balloons within the cavernous sinus

Indirect CCF

multiplicity of the microfistulae and their propensity to parasitize additional arterial supply after embolization, these lesions are relatively difficult to eliminate. Cure rates of 60-80% have been reported in literature. Occlusion of the cavernous sinus using detachable coils is another treatment option in cases where the above techniques fail. Extremely soft platinum coils are introduced into the cavernous sinus through arterial or venous routes, so as to completely occlude its lumen. Complications such as transient cranial nerve palsies usually resolve over a few months. Permanent complications occur in less than 5% of cases.

In contrast, indirect CCF are low flow lesions and usually progress slowly. These lesions must be carefully followed up with periodic clinical examination, measurement of intraocular pressure and angiographic studies as required. Special attention should be paid to changes in the angioarchitecture and quantum of flow on angiography. Patients with visual deterioration, elevated intraocular pressure, obtrusive diplopia, intolerable bruit or headache and malignant proptosis with exposure keratopathy require definitive management. Table 1 summarizes and compares direct and indirect CCF.

Prognosis

Indirect CCF have multiple tiny arterovenous shunts in the wall of the cavernous sinus. These are usually embolized using polyvinyl alcohol particles, which not only mechanically occlude the lumen of the fistulae, but also incite an inflammatory reaction in the vessel wall (Figures 26.9A to F). Owing to the

With advances in imaging techniques and development of newer interventional techniques, consistently good results are being achieved in the management of CCFs. The key to effective management of these lesions however, is early diagnosis by maintaining a high index of suspicion.

364 Surgical Atlas of Orbital Diseases

A

B

C

Figures 26.9A to C: Diagnostic left external carotid angiogram in frontal (A) and lateral (B) projections, reveal multiple arteriovenous fistulae (arrows) along the wall of the left cavernous sinus. Selective angiogram of the feeders (C) shows the fistulae and opacification of the cavernous sinus (arrow) as well as superior ophthalmic vein (arrow heads)

D

E

Figures 26.9D and E: The feeders were embolized using polyvinyl alcohol particles (D) and (E)

Figure 26.9F: Postembolization check angiogram shows absence of opacification of the fistulae and cavernous sinus

Carotid-Cavernous Fistulae: Role of Interventional Radiologist 365

REFERENCES 1. Barrow D, Spector R, Braun I, et al. Classification and treatment of spontaneous carotidcavernous sinus fistulas. J Neurosurgery 1985; 62: 248-56. 2. Debrun GB, Lacour P, Fox AJ, et al. Traumatic carotid-cavernous fistulas: etiology, clinical presentation, diagnosis, treatment, results. Semin Intervent Radiol 1987; 4:242-8.

3. Higashida RT, Halbach VV, Tsai FY, et al. Interventional neurovascular treatment of traumatic carotid and vertebral artery lesions. Results in 234 cases. AJR 1986; 153: 577-82. 4. Halbach VV, Hieshima GB, Higashida RT, et al. Carotidcavernous fistulae; Indications for urgent treatment. AJR 1987; 149: 587-93. 5. de Keizer R. Carotid-cavernous and orbital arteriovenous fistulas: ocular features, diagnostic and hemodynamic considerations in relation to visual impairment and morbidity. Orbit 2003; 22: 121-42.

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27

CHAPTER

Ocular and Systemic Associations of Proptosis Subrahmanyam Mallajosyula, Mohd Javed Ali

Some orbital lesions are known to have other ocular features and also associated with other lesions elsewhere in the body. It is always essential to have a knowledge of these conditions, so that we can investigate accordingly and detect the disease in a very early stage and sometimes in asymptomatic stage,and help the patient. If needed we should refer them to the respective specialists to prevent complications and ensure overall care of the patient. In this chapter an attempt is made to list ocular and systemic associations of common orbital disorders presenting with proptosis. Some others like leukemia, lymphomas are dealt with separately elsewhere in this book.

Capillary Hemangiomas (a) Ocular features: Strawberry nevus of the lids, proptosis, ptosis, astigmatism, amblyopia, optic atrophy and exposure keratitis. (b) Systemic associations: • Kasabach-Merritt syndrome (multiple capillary hemangiomas + thrombocytopenia) • Mafucci syndrome (multiple capillary hemangiomas + multiple enchondromatosis) • Phaces syndrome (posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta, eye abnormalities and sternum abnormalities).1,2

Neurofibromatosis (a) Ocular features: Eyelid neurofibromas, prominent corneal nerves, glaucoma, Lisch nodules, congenital ectropion uveae, iris mamillations, choroidal naevi, retinal astrocytomas, optic nerve gliomas, optic nerve meningiomas and spheno-orbital encephalocele with pulsating proptosis. (b) Systemic associations: Chiasmal tumors, hypopituitarism, spinal and gastrointestinal neurofibromas, pheochromocytoma, juvenile xanthogranuloma, capillary hemangioma, Wilms’ tumor, rhabdomyosarcoma, scoliosis, macrocephaly, aqueductal stenosis and seizures.3,4 Diagnostic Criteria for NF-1 NF 1 is diagnosed if 2 or more of the following group of seven conditions are met • Six or more caif-au-lait spots > 5 mm in diameter in prepubescents or > 15 mm in postpubescents • Two or more neurofibromas or one plexiform neurofibroma • Axillary or inguinal freckling • Optic N glioma • Two or more Lisch’s nodules • Sphenoid bone dysplasia or thinning of long bone cortex, with or without pseudoarthrosis • First degree relative with NF-1. [Source: NIH Consensus development conference. Arch Neurol. 1988;45:575-580]

Ocular and Systemic Associations of Proptosis 367

Neurofibroma type 2 Features Meningioma, glioma, schwannoma, vestibular schwannoma, posterior subcapsular lenticular opacities, hamartoma of retina and retinal pigment epithelium. NF2: Criteria for Diagnosis Presence of any one of the following features: Bilateral vestibular schwannoma First degree relative with NF2 plus unilateral vestibular schwannoma < 30 years. First degree relative with NF2 plus any 2 of the following: • Meningioma • Glioma • Schwannoma • Juvenile posterior subcapsular lenticular opacities • Juvenile cortical cataract. [Source: National Institute of Health Consensus Development Conference Neurofibromatosis: Conference Statement. Arch Neurol 1988,45:,575-78.] Diagnostic evaluation for NF2: MRI for neurological and neuro-otologic evaluation is mandatory, since nearly 90% of NF2 exhibit bilateral vestibular schwannomas.10

Thyroid Orbitopathy (Grave’s disease) ( a ) Ocular features: Lid retraction, chemosis, proptosis, superior limbic Keratoconjunctivitis, keratoconjunctivitis sicca, diplopia, optic neuropathy and choroidal folds. ( b ) Systemic associations: Thyroid acropachy, plummer nails, tremors, fatigue, tachycardia, atrial fibrillation, pretibial myxoedema, alopecia, vitiligo, high output failure and myasthenia gravis.

Craniofacial Dysostosis Crouzon Syndrome ( a ) Ocular features: Proptosis, hypertelorism, V pattern exotropia, hypertropia, optic atrophy, exposure keratitis, megalocornea, glaucoma, colobomas, aniridia and blue sclera. (b) Systemic associations: Wide cranium, midfacial hypoplasia, parrot-beak nose, frog facies,

mandibular prognathism and acanthosis nigricans.5,6

Apert Syndrome (a) Ocular features: Shallow orbits, proptosis, hypertelorism, exotropia, antimongoloid slant, exposure keratitis, optic atrophy, keratoconus, congenital glaucoma and ectopia lentis. ( b ) Systemic associations: Oxycephaly, midfacial hypoplasia, low set ears, high arched palate, cleft palate, syndactyly, mental handicap and anomalies of the heart, lungs and kidney.5,6

Encephalocele (a) Ocular features: Pulsatile proptosis, dystopia, medial canthal swelling, microphthalmos, colobomas and morning glory syndrome. (b) Systemic associations: Neurofibromatosis, hypertelorism, broad nasal bridge and cleft palate.7

Wegener’s Granulomatosis (a) Ocular features: Nasolacrimal duct obstruction, dacryocystitis, scleritis, peripheral ulcerative keratitis, nonspecific orbital inflammatory disease and occlusive retinal periarteritis. (b) Systemic associations: Necrotizing granulomas of upper respiratory tract, necrotizing glomerulonephritis, perforation of nasal septum, saddle shaped nasal deformity, nasal-paranasal fistulae, vasulitis of the spleen and adrenals, polyneuritis and meningioencephalitis.7

Wyburn-Mason Syndrome (a) Ocular features: Arterio-venous malformations of the conjunctiva, lids and retina, orbital A-V malformations causing proptosis and bruit, vitreous hemorrhage and neovascular glaucoma. (b) Systemic associations: A-V malformations of the CNS causing headaches and seizures, cranial nerve palsies, motor and sensory deficits.7

Langerhans Cell Histiocytosis (a) Ocular features: Proptosis, ptosis, periorbital swelling, localized pain. (b) Systemic associations: Osteolytic lesions of skull, ribs and long bones, diabetic insipidus and soft tissue lesions of liver and spleen.8

368 Surgical Atlas of Orbital Diseases

Hurler’s Syndrome

Hemangioblastoma

(a) Ocular features: Corneal clouding, papilloedema, shallow orbits causing proptosis. ( b ) Systemic associations: Stiff joints, coarse face, chest deformities, dwarfism, hepatosplenomegaly and deafness.9

Von Hippel- Lindau's disease

Nonspecific Orbital Inflammation Syndrome • • • • •

Crohn's disease, Systemic lupus erythematosus Rheumatoid arthritis Myasthenia gravis Ankylosing spondylitis.

Sclerosing inflammation of the orbit • • • •

Riedel's thyroiditis Mediastinal fibrosis Sclerosing cholangitis Fibrosis of parotid gland, lacrimal gland and lung.

Osteoma • Gardner's syndrome (Familial polyposis of large bowel, plus osteoma of skull or jaw plus epidermal and subcutaneous cysts) • Turcot Syndrome (Familial adenomatous polyposis plus CNS gliomas).

Fibrous dysplasia McCune-Albright syndrome (ployostotic fibrous dysplasia + sexual precocity + cutaneous pigmentation).

Orbital Hamartoma (tuberous sclerosis) Ocular : Retinal hamartoma Visceral : Pulmonary lymphangiomatosis Renal angiolipoma Cardiac rhabdomyoma Skin : Facial angiofibroma CNS : Intracranial astrocytomas, ependymoma

• • • • •

Retinal hemangioblastoma Cerebellar and spinal hemangioblastoma Renal cell carcinoma Pheochromocytoma Others: Pancreatic tumors, cystadenoma of epididymis.

REFERENCES 1. Haik BG, Karcioglu ZA, Gordon RA, Pechous BP. Capillary hemangioma. Surv Ophthalmol. 1994;38:399-426. 2. Kushner BJ. Hemangiomas. Arch Ophthalmol. 2000; 118:835-36. 3. Beauchamp GR. Neurofibromatosis type 1 in children. Trans Am Ophthalmol soc. 1995;93:445-72. 4. Listernick R, Charrow J, Greenwald MJ, et al. Natural history of optic pathway tumors in children with neurofibromatosis type 1. J Pediatr.1994;125:63-66. 5. Cohen MM. The child with multiple birth defects. 2nd ed.New york:Oxford 1997:178-96. 6. Gorlin RJ, Cohen MM, Levin LS. Syndromes of the head and Neck. 3rd ed. Newyork Oxford; 1990. 7. Kanski JJ. Clinical Ophthalmology:A systematic approach. 6th ed. Butterworth - Heinemann. 2007. 8. Huang F, Arceci R. The histiocytoses of infancy. Semin Perinatol 1999;23:319-31. 9. AAO. Pediatric Ophthalmology and strabismus. Section 6. Ocular findings in inborn errors of metabolism. AAO publication 2006. 10. Arun D Singh, Bertil F Damato,, Jacob Pe'er, A. Linn Murphee, Julian Perry: Clinical Ophthalmic oncology, first edition, 2007 Saunders Elsevier,Philadelphia.

Index

A 3-D reconstruction of orbit 84 Adenoid cystic carcinoma 194 clinical features 194 imaging 195 management 196 pathology and pathogenesis 195 basaloid variant 195 comedocarcinoma variant 195 cribriform (glandular or swisscheese) pattern 195 sclerosing variant 195 tubular (ductal) variant 195 prognosis 196 Angiosarcoma 76 Applied anatomy of orbit 3 cavernous sinus 8 extraocular muscles 9 globe 8 lacrimal system 13 lids 10 nerves of the orbit 14 optic nerve 14 parasympathetic innervation of the orbit 16 sensory innervation of the orbit 14 sympathetic innervation of the orbit 16 orbital apex 6 orbital osteology 3 periorbita 6 Auscultation 51

B Basal cell and squamous cell carcinoma 348 B-cell lymphoma 174 Benign tumors of orbit 103 capillary hemangioma 103

cavernous hemangioma 103 hemangiopericytoma 103 lymphangioma 103 meningiomas 104 Bone tumors of orbit 180 case-illustration 181 aneurysmal bone cyst 184 cholesterol granuloma 184 chondroma 183 chondrosarcoma 186 Ewing's sarcoma 186 fibrous dysplasia 181 giant cell lesions 184 Langerhans cell histiocytosis (lCH) 186 mesenchymal chondrosarcoma 186 myeloma 186 ossifying fibroma 182 osteoblastoma 182 osteogenic sarcoma 184 clinical presentation 162 clinico-pathological classification of primary orbital bone disorders 180 osteoma 180 Bony lesions 62 Bony orbit 60

C Capillary hemangioma 348 Carotid-cavernous fistula 39 Carotid-cavernous fistulae 356 direct CCF 360 indirect CCF 363 management of CCF 360 pathophysiology 357 clinical features 357 prognosis 363 radiological investigations 359 Cell cycle and the principles of antineoplastic therapy 347 Cephalocele 201

radiological finding 202 treatment 202 Choroidal melanomas 349 Classification of orbital tumors 102 primary 102 hemopoietic 102 lacrimal gland 102 mesenchymal 102 miscellaneous 102 neural 102 secondary 102 direct extension 102 Cystic lesions of the orbit 78 Cysts of the optic nerve sheath 203 Cytology smear 91

D Dacryocele 205 Decision making 271 apical conal lesions 279 intraconal lesion 273 lesions of superior peripheral space 279 thyroid associated orbitopathy 285 Dermoid and epidermoid cysts 200 investigations 200 MRI 200 treatment 201 Developmental lesions of orbit 98 dermoid and epidermoids 99 fibrous dysplasia 99 hamartoma 99 meningioencephalocoeles 98 neurofibromatosis 98 sphenoid wing dysplasia 98 Diagnosis of orbital tumors 98 oculomotor paresis 98 optic neuropathy 98 pain 98 papillary abnormalities 98 proptosis 98 Duplex Doppler 56

370 Surgical Atlas of Orbital Diseases

E Enlarged extraocular muscle 69 ENT approach to proptosis 300 etiological factors 300 clinical manifestation and evaluation 300 diseases of the lacrimal apparatus 300 infection and inflammation 300 tumors of the orbito-sinual disease 300 Epithelial cyst (dacryops) 190 clinical features 190 management 191 pathology and pathogenesis 191 prognosis 191 Etiology of proptosis 53 Evaluation of a case of proptosis 53 External radiotherapy in ocular tumors 346

F Functional endoscopic sinus surgery (FESS) 309

G Graves’ ophthalmopathy 353

H Hand-Schuller-Christian syndrome 133 Hematic cyst 203 investigations 203 treatment 203 Hertel's exophthalmometer 35 Hertel's exophthalmometry 37 Hutchinson's sign 15 Hydatid cyst of orbit 217 investigations 217 management 217

I Idiopathic orbital inflammation (IOI) 353 Inflammatory lesions of orbit 100 orbital cellulitis 100 idiopathic orbital inflammation 100 orbital infections 101 aspergillosis 101 cysticercosis 102 neoplastic lesions 102 tuberculosis 101

Internal radiation therapy (brachytherapy) 345 Intraocular lymphoma 349 Intraocular tumors 349

K Kilppel-Trenaunary syndrome 153 Kimura's disease 132

L Lacrimal gland tumors 76 Langerhans histiocytosis 133 Letterer-Siwe disease 133 Leudde's exophthalmometry 35 Lid retraction 46 Lymphoma of lacrimal gland 77 Lynch-Howarth's operation 304

M Malignant conjunctival tumors 349 Malignant tumors of orbit 104 adenoid cystic carcinoma 105 Graves disease 106 histiocytoma 105 lymphoma 105 metastasis 105 optic glioma 106 rhabdomyosarcoma 104 Management of ophthalmic tumors 347 Medical management of proptosis 337 nonspecific inflammations of the orbit (NSOIS) 337 chronic granulomatous infections 338 nonspecific lacrimal inflammation 337 nonspecific myositic inflammation 337 orbital cellulitis 338 parasitic infestations 338 rhino-orbital mucormycosis 338 specific inflammations of the orbit 338 structural lesions 340 Tolosa-Hunt syndrome 339 vascular lesions 339 vasculitis 339 Meningioma of optic nerve sheath 74 Mesenchymal tumors 170 histiocytic tumors 175 fibrous histiocytoma 175 malignant tumors of uncertain type 175 rhabdoid tumor 175

mesenchymal soft tissue tumors 170 striated muscle tumors 170 rhabdomyoma 172 rhabdomyosarcoma 170 Microphthalmos with cyst 202 Mucocele 202 Müller's muscle 46

N Neurofibroma 75 Neurosurgical approach to proptosis 309 Non-Hodgkin's lymphoma 147 Nonspecific orbital inflammatory syndrome (NSOIS) 131

O Ocular and systemic associations of proptosis 366 capillary hemangiomas 366 systemic associations 366 ocular features 366 craniofacial dysostosis 367 apert syndrome 367 crouzon syndrome 367 encephalocele 367 fibrous dysplasia 368 hemangioblastoma 368 Hurler's syndrome 368 Langerhans cell histiocytosis 367 nonspecific orbital inflammation syndrome 368 orbital hamartoma (tuberous sclerosis) 368 osteoma 368 sclerosing inflammation of the orbit 368 Wegener's granulomatosis 367 Wyburn-Mason syndrome 367 diagnostic criteria for NF-1 366 neurofibroma type 2 367 criteria for diagnosis 367 diagnostic evaluation 367 features 367 neurofibromatosis 366 ocular features 366 systemic associations 366 thyroid orbitopathy 367 Ocular metastasis 352 Optic nerve glioma 353 Optic nerve meningioma 353 Orbital amyloidosis 129 Orbital diseases 97 classification 97 developmental 97 endocrine 97

Index 371 inflammatory 97 miscellaneous 97 neoplastic 97 traumatic 97 vascular 97 Orbital exenteration 318 complications of exenteration 320 indications 318 management of the exenterated socket 320 myocutaneous flaps 320 patient preparation 318 prosthesis 320 skin flaps 320 skin grafting 320 spontaneous granulation 320 surgical procedure 318 types 319 anterior exenteration 319 lid sparing exenteration 319 radical exenteration 319 total exenteration 319 Orbital fractures 220 anatomy 220 examination 222 floor fractures 233 general operative considerations 229 antibiotics 230 decision repair or not repair 232 timing of surgery 231 imaging 226 implant materials 227 late and secondary fracture repair 239 lateral wall and zygomatico-maxillary fractures 238 Orbital infections 120 demographic profile 120 diagnosis 121 emergency department care 122 etiological causes 121 bacterial infections 121 fungal infections 121 parasitic infections 121 protozoal infections 121 imaging studies 121 risk factors 121 Orbital lymphoma 146 modified Rye's classification of Hodgkin's lymphoma 147 classic HD 147 nodular lymphocyte-predominant HD 147 revised European American lymphoma classification (REAL classification) 146 leukemias and lymphomas of T-cell origin 146

WHO classification of NHL 146 B-cell neoplasms 146 T-cell neoplasms 146 Orbital lymphoma 352 Orbital prosthesis 327 assemble the prosthesis 331 casting 329 fabrications of ocular prosthesis 331 impression of the orbital defect 328 moulding 330 preparation of the patient 328 sculpting 329 types 327 adhesive retained prosthesis 327 magnetic retained prosthesis 327 partial prosthesis 327 spectacle mounted prosthesis 327 using the desired material 331 Orbital tumors 352 Orbital tumors of neurological origin 162 optic nerve glioma 162 optic nerve meningioma 163 orbital schwannoma (neurilemmoma) and neurofibroma 165 Orbital xanthogranuloma 134 Orbitotomies 288 approaches 289 general principles 288 lateral orbitotomy 290 Stallard-Wright lateral orbitotomy 290 swinging lower lid flap 289 transcarcuncular approach 291 transfrontal orbitotomy 291 complications 291 postoperative management 291 transnasal endoscopic approach and transantral approach 291

P Parasitic cysts of orbit 207 investigations 208 treatment 208 Patterson's operation 305 Perception of color vision 41 Peripheral surgical space 91 Plaque radiotherapy 346 Pleomorphic adenoma 191 clinical features 191 imaging 192 management 192 pathology and pathogenesis 192 prognosis 192 Pleomorphic adenoma 76 Proptosis 28 axial proptosis 29

down and in displacement of the globe 32 down and out proptosis 29 measurement of proptosis 35 upward displacement of globe 35 Pulsations of globe 37

R Radiation therapy delivery methods 344 Reese-Berke's incision 273 Retinoblastoma 349 treatment 349 chemoreduction 350 cryotherapy 349 photocoagulation 349 subtenon (subconjunctival) chemotherapy 350 surgery (enucleation) 350 Rhabdomyosarcoma 77, 352 Role of cytology in orbital lesions 85 fine needle aspiration/sampling technique 85 intraoperative-operative diagnosis by squash and imprint cytology 85 squash or imprint cytology 85 Rosai Dorfman disease 133

S Sarcoidosis 130 Sebaceous carcinoma 348 Secondary and metastatic orbital tumors 244 malignant melanoma of eyelid 256 metastatic orbital tumors 263 orbital extension of conjunctival tumors 258 malignant melanoma of the conjunctiva 259 squamous cell carcinoma of the conjunctiva 258 orbital extension of eyelid tumors 252 orbital extension of intracranial tumors 257 orbital extension of intraocular tumors 244 orbital extension of lacrimal sac tumors 250 orbital extension of medulloepithelioma 246 orbital extension of nasopharyngeal tumors 262 orbital extension of retinoblastoma 244 orbital extension of tumors of the nasal cavity and paranasal sinus 260

372 Surgical Atlas of Orbital Diseases orbital extension of uveal melanoma 247 sebaceous carcinoma of the eyelid 253 squamous cell carcinoma of the eyelid 255 Sequelae of radiation therapy 353 Sinus diseases causing proptosis 301 allergic fungal sinusitis 301 extensive nasal polyposis 301 frontoethmoidal mucoceles 302 mucormycosis 301 purulent infections 301 treatment 301 Soft-tissue lesions 72 Sphenoid wing meningioma 45 Steps of Reese-Berke's approach Steps of superior lid crease incision 275 Sturge-Weber syndrome 152 Subconjunctival hemorrhage 51

T Teratomas 201 Thyroid-associated orbitopathy 111 course of disease 114 incidence and epidemiology 112 management guidelines 118 pathogenesis 112 risk factors 112 visa classification 114 appearance/exposure 117 application of the visa classification 118

inflammation/congestion 116 strabismus/motility restriction 117 vision/optic neuropathy 114 Transcranial approach with resection of the orbital roof 311 Transpalatal approach to remove postnasal tumors 307 Trauma 65 Tumors of lacrimal gland 348 Tumors of paranasal sinuses causing proptosis 302 fibrous dysplasia 302 hemangiopericytoma 302 juvenile nasopharyngeal angiofibroma 303 malignant tumors 303 adenoid cystic carcinoma 303 esthesioneuroblastoma 304 non-Hodgkin's lymphoma 303 rhabdomyosarcoma 303 squamous cell carcinoma 303 various approaches for tumor removal 304 Caldwell-Luc operation 304 external ethmoidectomy 304 intranasal ethmoidectomy 304 Jansen-Horgan operation 304 lateral rhinotomy/medial maxillec tomy 305 total maxillectomy 306 Tumors of the eyelid 347

V Val salva 51 Vascular anatomy of the orbit 17 arterial supply 17 outflow 19 paranasal sinuses 20 venous 19 Vascular lesions of orbit 151 malformations 151 cavernous hemangioma 152 lymphangioma 151 orbital varices 152 other congenital malformations 152 shunts 153 angiosarcoma 155 capillary hemangioma 154 carotid-cavernous fistula 153 hemangioblastoma 155 hemangioendothelioma 155 hemangiopericytoma 155 Kaposi's sarcoma 155 new growths 154 Vascular pulsations 39 Von Recklinghausen disease 75

W Wegener's granulomatosis 132 Whitall's tubercle 12 Wyburn-Mason syndrome 153