Rapid Review of Radiology

APID

REVIEW

SHAHID HUSSAIN SHERIF A. A . LATIF ADR IAN D . HALL

Rapid Review of

Radiology Shahid Hussain MA, MB, BChir, MRCP, FRCR Consultant Radiologist Heart o f Eng land N HS Foundation Trust, Birming ham) UK

Sherif Aaron Abdel Latif MB , ChB, MRCS, FRCR Consul tant 1~1d i ologist Dudley Group of Hospitals NHS Foundatio n Trust, West Midlands, UK

Adrian David Hall MB , ChB , MRCP, FRCR Consultant Radiologist Dudley Group of H ospitals NHS Foundation Trust, West Midlands, UK

MANSON

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GENERAL INTRODUCTION

This book has been written primarily for senior radiology ITJinces preparing for final radiology exams and in particular fo r trainees studying for the Fellowship of [he Royal College of R3diology. With Ihis in mind, the cases presented herewith have been presented in the exact manner that the cases are presented in the Ro}'al College of Radiology FRCR 2B exam. In the exam, long cases and viva cases are presented with a very minimal bur highly relevant history and the required response is expected to be presented in a particular way. This format of reporting ' involves giving a Description/ Interpretation of the images; Diag nosis; Differential Diag nosis; and advice on Furrhcr Management. We have laid Ollt the answers here in exactly this manner and have included a Discussion [Q give in depth further information about each condition which wi ll enable the sntdent to answer any qucstions directed to him or her in the viva situation, By tollowing the RCR exam formal , thc candidate should be ideally prepared for t his e.l:am and for [he future as a Consultant Radiologist.

FILM TECHNIQUE Whatever the imaging modality, the radiologist interprets images using all the infonnation and clues available, to prod uce a differential diagnosis and/or advise on further investigation and management. Above all else, this mUSt be done in a SAFE manner, and this often requires one [Q be systematic in approach. Secondly, this process must be done in a SENSIBLE manner - it is easy to quote endless lists of differential diagnoses but if these are not refined for each individual case, the r.:tdiologisr's input is of little value.1l1e following discussion concentrates in particular o n perfo rming these tasks in the examination viva scenario. However, much of the advice is applicable to everyday practice [Qo, in particular the emphasis on a safe and sensible approach.

TYPES OF FILM The rypes of film one may enCounter in an exam/viva arc as fo llows:

The ' Aunt Minnie' There arc certain disorders that have a characteristic appearance on imaging that allows one to make an instant 'Spot diagnosis'. It \\~Il be assumed the candidate has come across it before, and thus t he best preparation here is

exposure to as many ofthese cases as Ix>ssible. Radiological atlases and film libraries provide ready access ro many of these classic cases, which can then be committed to memory. You can prepare a ready-made description of these cases for rhe viva. Ifro\! arc sure of the diagnosis, dispatch the film promptly with your preprepared 'speech' so that you can progress to the next case as soon as possible. Of course there may be 'Aunt Minnie' cases that yO ll haven't secn and rhis may present a problem, Such cases arc often not amenable to working out the diagnosis - you either know it or you don't . The o nly thing ro do is be methodical in your analysis and description of the findings so that at the very least you can suggest whether you feci an abnormality is li kely 10 be lo ngstanding and benign or orhenvise, and make appropriate suggestions on how you would proceed.

The 'test of observation' H ere, there is an abnormality prescnt that once seen, may wclllcad to an easy d iagnosis. The abnormality is subtle or hidden however, such that it tests the candidate's perception and approach [Q a case. Perceptual abi lity, however, is variable, not only between people bur also in the same obscn'er on diflcrcllt days (this is particularly [rue in examinat:ions where anxiety levels arc high). You must therefore be systematic in analysing each film if there is no ob\'io us abnormality [ 0 sec on firsr inspection. There arc mallYdiflerent s}'srcmatic approaches and it is beyond [he scope of this discussion to be morc prescriptive. Howcver, make sure you haye a system and usc it. Moreover, describe the process you are going through aloud in the viva so that the examiner knows that you arc practising safe radiology.

The 'jigsaw puzzle' This type of case presents several findings that once identified and considered together, lead to a specific or differential diagnosis. This nOt only tCSts perceptual skill and systematic approach, but also the ability ro mentally 'cross-reference' se\'eral diflcrential diagnosis lists for the various abnormalities identified , to find thc 'best fit' diagnosis. Whenever producing a differential diagnosis in an examination or real life, it is \'ital to produce a sensible list, not just a recital oflong lists learnt from books. To do this, you must use all clues available from the clinical histOry and film, and combine this information with knowled ge of the incidence of each possibility in a given patient population.

Generalln1rodueti o n

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  Cases 15, 16
  
  CASE 15 ory 68-year-old male presented with progrcssi\'c dyspnoca.
  
  CASE 16 ory --H)-year-old male presented with a
  
  g progrcssi\'c history of dyspnoca _more reecn! onset cyallosis.
  
  37
  
  Answer 15
  
  Chest Imaging
  
  ANSWER 15 Observations (15al This chest TJdiograph demonstrates bilateral lower zone imerstirial rcricular opacity with evidence of basal volume loss demonstrated b~' descent of bath hila. The interstitial opacit), gives the heart an irregular 'shaggy' border. These. appearances arc in keeping with basal fibrosi6. There is a calcified pleural plaque rcbred to the right hemidiaphragm. The combination of basal tibrosis with pleural disease would suggest asbestos exposure with pulmonal)' asbestosis and pleural plaques.
  
  Diagnosis PulmonarY;lsbcstosis.
  
  Discussion Pulmonary asbestosis is a chronic progressive fibrotic condition secondary to chronic asbestos exposure. eroeidol itc (blue ) asbestos fibres arc mOst commonly associated with malignant disease and pleural disease. Radiological features are of a fibrosing alveolitis that predominantly affects the bases and is indistinguishable from other causes. Fibrosis can progress to result in progressive massive fibrosis, but this again predominates at the lung bases. Pulmon:u), asbestosis has a latency period of -40 years and therdore pleural changes are usually seen prior to lung parenchymal changes. Othcr features of asbestos exposure include: Pleural efli.tsion - this is the earliest pleural abnormality, with a latency of - 10 years. Focal pleural plaques - have a btenc}' of 20-40 years. DitTuse pleural thickening Pleural calcification. Rounded atelectasis - this is also known as folded lung and :uises due to infolding of thickened plcurtl. with associated subscgmental atelectasis.oMost commonly seen in the lower lobes, it has the appearance of a rounded subpleural mass abutting
  
  15b CT image of the chest demonstrating a large intra parenchymal lung lesion that is abutting thickened pleura. Vessels appear to be radiating towards the lesio n as though pulled towards it.
  
  38
  
  thickened pleura, with linear bands extending from the mass into the lung (craw's feet ) ( I Sb ). Malignant mesothelioma - - 90% arc related to previous asbestos exposure Lung carcinoma - there is a brency of -30 years and occurrence is related to the dose of asbestos exposure and to cigarette smoking - which can increase risk by 100-fold.
  
  Practical tips Multiple pleur.l.l plaques arc characteristic tor previous asbestos exposure ( I Sc). Look for signs of m:llignancy in patiems with asbestos exposure - remember the increased risk of pleural and pulmonary malignancy. Pulmonary masses should be investigated with CT - ch:lfacteristic findings may permit a confident diagnosis of folded lung in some cases. Asbestosis is the 'odd one out' among the inorganic dusts causing pulmonary fibrosis. Thl: other fibrogenic dusts cause upper ZQlle fibr06is.
  
  Further management • Systemic sympwms, e.g. weight loss, shou ld be carditlly investigated to exclude mesothelioma or bronchogenic carcinoma, for which these patients are at increased risk. In cases where there is still clinical concern abom an area of possible tolded lung despitl: imaging, percutaneous biopsy may be requ ired to exclude a malignancy.
  
  Further reading Akira M, Yamamoto S, Yokoyama K, etal. (19 90 ). Asbeswsis: high -resolution CT-pathologic correlation. Radiolog'l 176: 389- 394.
  
  15c Axial image shows a right anterior calcified pleural 'plaque consistent with previous asbestos exposure . There is also subpleural reticulation representing fitirosis and appearances would be of asbestosis.
  
  Answer 16
  
  Case 17
  
  Chest Imaging
  
  AN SWER 16 Observations (16) This frontal chest rJdiograph shows extreme cardiomcgal~' . There is marked dilatation of the central and main plll .anary arteries with 'pmning' of peripheral pulmonary merics. No diffuse lung abnormality is seen T he findings arc indicative ofpuhnonary hypertension. Gj,.-c n the gross cardiomegaly, a kft to right shunt is the alIOS! likely cause. Howe\'er, cyanosis should not occur and lIS presence suggests the shunt has rc\-crscd, that is, the p;1[ienr has developed Eisenmenger's syndrome.
  
  Diagnosis Pulmonary arterial hypertension from an undiagnosed ,"cntricular septal defect (VSD ) progressing to Eisenmenger's syndrome,
  
  Discussion Pulmonary arterial hypertension is dilgnoscd by a sustained mean pressure >20 mml-lg (systolic >30 ITlITlHg, diasTOlic >1 5 ITlmHg ). Radiological fe:Hllfes on a plain chest r.td iograph that suggest the diagnosis arc: • Increase in size orthe main pulmonary artery. • Reduction in size of peripheral pulmonary arteries known as 'peripheral pruning'. • Right heart enlargement • Calcification ofthe central pulmonary arteries - a late but characteristk sign. • Parenchymal mosaic attenuation pattern seen on HRCT. Primary pulmonary hypertension is idiopathic. The condition can also arise secondary to pulmon:lTY disease or
  
  cardiovascular disease, either from an increase in overall pulmonary arterial resistance or from :In increase in the o' ·erall circulatory '·olume going through the pulmonary circulation. Increased resistance - pulmonary veno-occlusil"e dise:lse, chronic pulmonal)' thrombocmbolism, any chronic I'emil:ltory disorder leading to chronic hypoxi:l and resulting vasoconstriction in the puJmonal)' arteri:ll bed Increased flow - left to right shunts, i.e. ASO (atrial septal defect ), VSO (vemricubr septJ.J defect ), rOA (patent ductusarreriosus). In Eisenmenger's syndrome the pulmonary arterial pressure climbs until it c,"elltually exceeds the pressure in the left heart and the shunt re'·crscs. It is seen in those with pulmonary hypertension from :I left to right shunt.
  
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  can mimic pulmonar), aTlerial hypertension. Clinical should be subsequentl)' history here is vital and undertaken in the right clinical sening. The diameter of the m:lin pulmonal)' artery should bo less than that of the ascending thoracic aorta. Rc vc rsal of this ratio is a sign of pulmonary hypertension.
  
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  Furth er management Primary pulmonary hypertension has 110 cure :md a dism:ll prognosis. It is a diagnosis of exclusion so all underl ying causes of secondal)' pulmonary hypertension m ust be investigated. Cardiology referral with a I'icw to echocardiography would be required initially.
  
  CASE 17 History ..\ 50-year-old female presented with progressi,'e dyspnoea and intermittelH cyanosis of the fingcl1l.
  
  39
  
  Answer 17
  
  Chest Imaging
  
  ANSWER 17 Observations (17a) This frontal chest film shows abnormal rcticuhr interstitial op:Kiry at both lung bases, though there arc no features to indicate significant volume loss at the present time. A large area of calcinosis is noted in the soft tissues around the upper right hUlllerus. The combination of find ings and clinical history suggests a diagnosis of systemic sclerosis
  
  with lower zone pulmonary fibrosis and Raynaud's phenomenon
  
  Diagnosis Lower zone pulmonary fibrosis due to systemic sclerosis.
  
  Differential diagnosis For lower zone pulmonary fibrosis: • Idiopathic pulmonary fibrosis (I PF) (cryptogenic fibrosing alvcolitis - CFA). • Connective tissue disorders - systemic sclerosis, rheumatoid • Asbestosis. • Drugs - especially certain cytotoxic;, e.g. cyclophosphamide, bleomycin, busulphan, etc.
  
  Discussion This autoimmune d isease has also been known as scleroderma, \\~th a subgroup known as CREST syndrome Current nomenclature is systemic sclerosis wiTh diffuse or limited scleroderma, the latter being The equivalent of CREST syndrome. The condition is three times as common in females and typically presents in the 4th- 5th decades. A variety of autOantibodies may be present including ANA and rheumatoid Factor. Clinical features are many and varied bur include: • Musculoskeletal - thickened skin, soft tissue calcinosis, Raynaud's, erosi,·e arthritis (sec Case 152 ). Lungs -loll"er zone pulmonary fibrosis, aspiration. Oesophagus - hypotonia results in dilatation and dysphagia. lncompetence of the gastfo-ocsophageal sphincter results in reflux and consequent peptic stricture, aspiration, etc. Small bowel - dilatation and slow transit result in bacterial overgrowth and malabsorption. Barium studies show 'hidebound' appearance due to fibrosis pulling the vah>ulae closer together. Pscudosacculatiolls and pneumatosis in small and large bowel. T he CREST syndrome represents Calcinosis, Raynaud's, oEsophageal dysmotility, Sclerodactyly, Telangiectasia.
  
  Practical tips Once lower zone pulmonary fibrosis has been noted on the chest radiograph, examine the film for the following features that may indicaTe a specific diagnosis: • Dilated oesophagus - systemic sclerosis (sec Case 152 ). Erosions of the lateral ends of clavicles rheumatOid. Pleural plaqucs - asbestOsis.
  
  40
  
  • Soft tissue calcification - systemic sclerosis. • Signs of malignancy including bony sclerosis from myeloproliferative disorders - cyTOtoxic induced • Sympathectomy clips - systemic sclerosis ( 17 b ). Remember which disorders cause upper and lower zone fibrosis ( reler to the differential diagnosis abovc, and also that in Case 18 for npper zone fibrosis ): sarcoid is rhe classical upper zone disease, much as IPF is the classical lower zone disea~. Thereafter, remember that the upper zones arc better ae rated and the lower zones better perfused. So, diseases caused by inhaled dust (inorganic or organic, e.g. silicosis ~ and extrinsic allergic akeolitis [EAA] respectively) I ,,'" atlect the upper zones, while the lower zones will be '0"1 afkcted by blood ~orne disorders, i.e. drugs and autoimmune conditions. Unfortunately, asbestos is an exception and docs not obey this logic. As with many fibrotic lung conditions, theft; is an increased incidence of pulmonary malignancy in systemic sclerosis associated pulmonary fibrosis check for focalnodulcs/masses on the chest radiograph. Alternatively, tOcal airspace opacities may represent aspiration.
  
  Further management H RCT is the imaging choice in diagnosis and follow- up ofinrcrstitiallung disease. cr imaging findings of fibrosis include lung volume reduction, subpleural reticulation, interlobular septal thickening and tJ":."letion bronchiect.1sis.
  
  Chest Imaging
  
  Cases 18, 19
  
  CASE1S .... istory .~3
  
  r.:ar "Id 111J.1c I'n:scn1\:,j \\11h hol.:k Jnd p"('grc"'I"c d\'~pnoc.1
  
  :;'S E 19 "'11"Jlcprt"SCIllCd"1thkfl~rm
  
  4'
  
  Answer 18
  
  Chest Imaging
  
  ANSWER 18 Observations (18a) Plain radiograph of the chest shows changes of upper zone fibrosis with elevation of both hila and upper zone reticular opacit ies. In addition, there is a cavilY in the left uppe r zone containing a soft tissue de nsity mass with surrounding air crescent. These appearances would be consistent with a mycetoma. T he patieTlt also has a marked kyphosis with the head obscuring the lung ::apices. Moreover, on close inspection . there is a hint of syndcsmoph),tc formation along the right lateral aspeCT ofrhOr:lcic spine. T he combination of findings is consistent \\~th upper zone fibrosis associated \\ith ankylosing spondylitis. T here is myceroma formation in the fib rotic C:IVil}, in the left uppe r zone.
  
  Apical bullae and cavit"Jtioli . Paraseptal emphysema. Bronchiectasis . Cardiac fC:ltll res include ao rtitif; involving the ascending aona with aortic \·alve insutlicielll'Y. P13ill radiographic featu res of uppe r zone fibrosis include: EIc\':Ition of the hila. Tenting of the hemidiaphragms. Ele\':Ition of the horizontal fissure on the right (a good indicator). Increased lucency of the lower zone due to hyperexpansion. Reticular o pacilies ill the upper zones.
  
  Diagnosis
  
  Practical tips
  
  Ankylosing spondylitis.
  
  Differential diagnosis For uplX'r zone fibrosis (mnemonic - 'STRAD'): Sarcoidosis. TB. R.1diation. Ankylosing spondylitis. Dust inha13tion - inorganic (e.g. silica) and organic (i.e. chronic extrinsic allergic ah·eolitis).
  
  Discussion Ankylosing spondylitis is an autoimmune disease that most commo n l~' manifests as a seronegative ;lfthropathy, predominantly affecting the axia l skeleton (ini tially sacroiliac joints then thoracic and lumbar spine). It usually presents in the 2nd-4th decade and more fTcquently affects men (sex mtio of - 5: I ). As well as bone involvement, there arc respiratOry and cardiac m:llliteslations. Respi ralo!)' man itestations arc seen in - I % of cases and featu res include: UPlX'r lobe pu lmonary tibrosis. • Relicular/ reticulonodular opacities in lUllg apices.
  
  Clues to help limit the diOercntial d iagnosis for upper zone fibrosis include: Kyphosi' and 'bamboo spine' indicate ankylosing spondylitis. Egg shell nodal caldficalion suggests silicosis or S:trcoid. Associated calcified grJnulomatn suggest T B. A l wa~'s look fo r signs of secondary in fectio n/ mycetoma in fibrot ic cavities ( ISb ). Whcn pulmona!), fi brosis due to ankylosing spondylit is is suspected, look for signs of complications of d mg treatmcnt on the film: • Ihascular necrosis of humeral heads secondary to steroids. • Atypical dist ri bution of fibrosis may be secondary 10 drug treatment.
  
  Further manage ment M ultidiscipl inary management ll1ult isystem d isease .
  
  18b CT image in the same patient shows a cavitating lesion in the left upper lobe apical segment containing an Aspergillus fun gus bait.
  
  42
  
  is
  
  requ ired
  
  in this
  
  Answer 19
  
  Chest Imaging
  
  Case 20
  
  ANSWER 19 Observations (19) Two Spot images from an angiogram ;11I
  Diagnosis Subclavian steal syndrome.
  
  Discussion This is a condition that is usually acq uired and caused by ahcrosdcrotic d isc:!$!:. Stenosis of the su\xla,·i3.n artery results in stcaling of blood [Q the arm via rctrogradc flow m the ipsilateral "crlcbral artery. Othcr acquired causcs -:Iudc "asculiti (T akarasu ), cmbolism, aortic disscction, radiation fibrosis and chest trauma. Congenital causes arc ancommon. Clinical features include: lefT arm is more commonly im'oked than thc right. Reduced BP b~' up to 40 mmHg in the aflccted arm. • Delayed/wcak pulse in the aflccted arm.
  
  SubcJa"ian insufficicncy - pain, numbness and weakness in the arm that is brought on by cxercising thc limb. Necrosis ofthc fin gertips. Vertebrobasilar insuflicicncy - syncope can be prccipitatcd by exercising the arm due to the stealing of blood. Headaches, ataxia, vertigo, diplopia, homon ymous hcmianopi::a ::and hemiparcsis have ::all been reported.
  
  Practical tips Di::agnosis can be m::ade nonin\'asi\"d~' by US by idemif)~ng reversal of Doppler flow in the "cnebral arte ry.
  
  Further management CT can be useful to identify/characrcrizc c::alcificd atherosclerotic plaque in the subcl:lvian artcry (uncontrasted CT) and also the site/ degree of stenosis (arterial phase CT). Surgical referral is required for treatment wil h either balloon :mgioplast}' (+/ - stem insenion ) or surgical bypass (common carotid to subclavian artery).
  
  Further reading Chung ]W, Park JH , 1m JG, etnl. ( 1996). Spiral CT angiography ofthc thoracic aort:l. /{adioGmplJiCJ 16:
  
  81 1- 824 .
  
  CASE 20 History \ -I4 ' ye:lT-old male having a pre cmplo)'mcnt chest radiograph.
  
  43
  
  Answer 20
  
  Chest Imaging
  
  ANSWER 20 Observations (20a) This chest radiograph shows multiple fine, sand-like, tillY calcified lesions measuring Jess than I mill in diameter, spread throughout both lungs. Both lungs arc of normal \'olul11(. No olher abnormality is seen.
  
  question to the clinician such as 'how unwell is this patient? ' can be most helpful.
  
  Further management No further m;lIlagement is required in this benign condition.
  
  Diagnosis Ain:ol:lf microlithiasis.
  
  Further reading
  
  Oifferential diagnosis
  
  Brown K, j\'lund OF, Aberle DR, Batra P, tt (I/. ( 1994 ). Intrathoracic calcific3.tions; radiographic features and di ffe rential diagnoscs. RadioGraphiu 14; 1247-1261 .
  
  With pin-poinr high-density nodules, rhe possibilities afC Girl}' limited, as follows: • Inhaled illO%'3nic dusts such as silicosis. Nodules tend ro be a linle larger and arc predominantly in rhe Illiddle and upper zones. CoaicscclKc \0 torm larger lesions with cavir,uian and fibrosis occurs. Egg shell calcification of nodes. Other inoTbranic inhaled dusts such as tin oxide, limestone and marble. Slightly larger high -density opacities lead [0 a largcr ditlercntial in addition to the above: Varicclla pneumonia - pr~\lious inlection can appear radiologically with multiple calcified nodules measuring 1- 2 mm in size. No lymph node calcification isscen. Hist9plasmosis - hcaled inlection Gill also result in multiple tiny calcifications throughom rhe lungs. Associated 'I~rh mediastinal lymph node, liver and spleen calcification. Metasr:.uic calcinosis - focal calcification within the alveolar septae due to elevated senun calcium and phosphate b 'ds in conditions such as h}'PCrparathyroidism, multiple myelo ma, sarcoidosis, milk-alkali syndrome or hypervitaminosis D. There is upper zone prcdominancc and disease can progress to fOrtn airsp;lCc opacities, consolidatil'c appcarances and fibrosis (20b). Pulmonary haemosidcrosis due to mitral vall-e disease. Barium aspiration - hyperdense opacities in the lowcr zoncs morc common o n the right (20c).
  
  20b Chest radiograph showing bilateral upper zone airspace opacities of metastatic calcinosis.
  
  Discussio n This is a rare condition that aflccts adults in the 4th- 6th decades, resulting in calcification within the all'eoli. Usually these paticnts arc asymptomatic, howcI'er thcy can prcsent with dyspnoca on exenion. R.1diological appearances call be quite striking with diffuse tin y calcit1ed nodules < I mm in diamcter spread throughout borh lungs. The middle and lowcr zoncs arc preferentially aHee!ed. Serum GI1cium and phosphatc arc normal. DiAcrentiation from the causes below is usually made by thc normal biochemistry, characteriSlic radiological appearances and the p3.ucity of clinical symptoms rci3.1il'c to the lll3.rked radiological changes.
  
  Practical tips Clinical history is of vital imponance when narro"~ng down 3. list of ditlCrcnti3.1 diagnoses. Alveolar microlithiasis is 3. good cX:1mple of where markcd r:ldio[ogical changes :1re associatcd with a relative lack of symptoms. A simple
  
  44
  
  20c Chest radiograph showing hyperdense airspace opacities in the lower zones predominating on the right in a patient who aspirated during a barium swallow examination .
  
  Chest Imaging
  
  Cases 21, 22
  
  CASE 21 History .\ 28-year-old pariem prcscnlcd with oXrcriorating chronic d)'Spnoca.
  
  CASE 22 History A 45 -year-old
  
  1n3ic
  
  presented with
  
  ':,udiac arrhythmia and shortness of
  
  breath.
  
  45
  
  Answer 21
  
  Chest Ima ging
  
  ANSWER 21 Olao",atlo ns I2 I a)
  
  Prlt1 lc. ' l ip .
  
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  A nswer 22
  
  Ch est Imagi n g
  
  Case 23
  
  ANSWER 22 Ob" ,"'lions (22)
  
  I m'll"~
  
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  P,.ctIClllllps
  
  Discussion ~,
  
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  .. "' .... r <.. 01;..- "",·;'1 ~ .... n .. In _H" "f ",,;"n, •
  
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  Furthe, mln_gem.nl lo.M " CXIl M r<'I'~""'l' ,h ", ~" of .omoiJ .
  
  CASE 23 HillOry A 50. )< ... ,~J ,.,k
  
  p,.,,,,,,,,J
  
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  "
  
  Answer 23
  
  Chest Imaging
  
  ANSWER 23 Observations (23a) This chest radiograph shows evidence o f previous surgery - there arc sternotom y wires and :l metallic mitr:!l v,ll\'e replace m ent . The heart is enlarged with a cardio thor.lcic ratio of 2 1:32 . There is a ' double de nsity' seen through the right hean - a sign a flert atrial enlargement . Multiple, small ( 1- 5 mill ) calcium density nodules arc seen in both lungs with mid and lower zone predominance. Upper ZOn e venous diversion and a tiny light pleur:!] cffilsion suggest pulmonal)' veno us hypertension . Appearances ind icate that the patie nt has had surgical lllitr:ll valve rcpi3cemcllt, but there arc persisting Icaturcs of mitral vake disease and signs o f left heart failure. He should undergo furt her asscssmcnr o f " ake and lefr heart function . Th e high ·density pulllloll3ry nodules indicu c pu lmonary haemosidcrosis, a consequence of elevated pulmon3ry ve no us pressure o ver many ye3rs. Diagnosis Mitral ,'al\'e discasc. Discussio n The most co mlllOIl GUIse of mirral valve dise:lse is rheumatic h<.:arr disease, widl presentatio n most cumnlo nly seen in middk ,Igcd fcm;\k s. It results in kll ,ltriJI cnbrgemcnt J.nd pulmonary Vl!1l0US hypertension/heart f-ailure Radiographic SigllS of mitral \'al"c disease arc: ' Double density' behind the rig ht heart border due to left atrial enlargement. Splaying o rlhe carina by the large kft atrium . Oesophagus displaced to the right.
  
  23b Chest radiograph shows enlargement of the left atrial appendage in a patient w ith mitral stenosis.
  
  48
  
  ldt .anal "'PfICnd.agc CII\,U"ge lllenr. The norm;11 kIt mcdu!brul comour hJ.s tWO convcxities above th<.: \'C:IIUldC - rhc X>nK: arell 3nd the main pulmo nal)' MttT\ Enbrgcmc::nt of the k ft 3tri313ppend3ge produ.:cs.amlrd "bu mp' below the pulmon3ry 23b IS sometimes calk d the ' third mogul [he term mogul is o ne uscd by skie rs to d escribe bumps In [he snow!). Caktfiarioo of thrOlll bus in lile !cll: 3trium ( 23c). Right "'cntricular hypertrophy, Pulmoru.n \~nous hypertensio n , intcrstitial and pulnlOrUJ)" oedema. Pulfl"lOfUry h.lemosid<.:rosis - longstanding clev3tion of pulmonary "'eno us prl!ssure results in oozing o f serum into the incerstitium. Blood products within this will ultimately be broke n d own to ,.
  
  n..s
  
  Practical tips h is diAicult to distinguish trom plain lilm is mitrJ.1 "3[\"e stenosis o r n:gurgi tation eonscqu<.: ncC$ and signs arc t he S3111e and coc x i ~t. Howe"er, if there is gross kfl atrial ' rhcrc must he a component of stenosis prescnt. Further management Cardiac !'efen al with a " iew [(l c< h'"" "dio l~"'" assessmen t is required . Tr<.:almen£ il1\'ol\'es fo r arrh ythmi as (3£rial t"lbrillatio ll - AF ) and cardiac funnion . Surg ical intervcntion pCrelltall COllS v,llvc balloon dil at3tion (high r.lrcs), valvotomy and v:llv<.: repbc<.:mcllt.
  
  23c lateral chest radiog raph shows curvilinear calcification of the left atrial wall.
  
  Chest Imagin g
  
  Case 24
  
  u r·old f1ule W:I,S rdured for m"cstiguiofl rollowing m;!;:!.l g showing a right lower lobe k-I.iofl .
  
  I,.,.' .'
  
  49
  
  Chest Imaging
  
  Answer 24
  
  ANSWER 24 Observations (24) These PET scan images from 3. PET CT dcmonsnatc a 1;lrgc right lower lobe highly FDG ( 18 fluoro-2-dcoxyglucose) avid lesion. There is a smaller lett upper lobe FDG avid lesion. Thc.rc is also rigln hilar uptake as well as uptake in the subcarinal region. Uptake in the region of the oesophagus is also dcmonsu ;ucd. There is 110 FDG avid
  
  lesion in li"cr or adrcnals. No bony lesions arc demonstrated. Diagnosis l\'lct3static right lower lobe bronchogenic carcinoma. Differential diagnosis The left upper lobe lesion represent's either metastases or a synchronous lesion. Di scussion PET scanning rdics o n increased upl3kc of FDG in cell populations with higher metabolic turno\'cr. The main application is oncological imaging; other, less utilized applications include CNS and cardiac imaging. h is essemial that PET scans arc compared with cross-sectional imaging if no PET CT scan has been performed. Assessing only PC:f illl;lges alone can lead to diagnostic crrors, mainly incorrect st:lging of malignant disease, The images (not shown ) of this PET/CT scan demonstrated a right sided pleural effusion that turned out to be malignant. Pleural cffilsions do nOt demonstrate increased FDG activity. Among Other areas, normal FDG uptake is demonstrated in the brain , heart, sal ivary glands, liver, spleen as well as upper renal tracr and it is also excreted in the urine, Nomlal bowel uptake is also often demonstrated. Pirfulls include increascd FDG uptake in fut (brown f.my tissuc); this can simulatc malignant nodal diseasc. In the current study, the increased oesophageal uptake is due to a coexisting reflux ocsophagitis.
  
  cr
  
  rET scans can be false negative for small lung metaStases or solitary lung nodules, False positivc diagnosis o n PET scanning also occurs due to infection and intlammation - conditions that arc associated wid1 in creased glucose turnover.
  
  Practical tips In the UK, PET scanning is used mainly as a staging tool. Neurological and cardiac applications arc not utilized widely. Among others, PET is used for initial ~:~~::~~I~ll~~ rcstaging of bronchogenic or oesophageal Cancer networks arc increasingly utilizing PET prior [Q planning for curati\'c surgel)', A growing application is the assessment of sol itary nodules. This is particularly useful in centrallesiolls tor which histological confirmation is more challenging and the complication rate is increased. A well documcmed pitfull is a fu lsc negative scan for small IUllg nodules or mcmsmscs. Lesions measuring less than 7-8 mm do ha,'c a higher fillse negati,'c rate. Lymphoma staging is also widely undertaken. This is \'el)' useful for assessment of activity in residual lymphoma masses and also for assessment of early rcsponse to chemotherapy. Inflammatol)' or infected nodal mediastinal masses (histoplasmosis) can also give rise to false positive s~ans. Corrclation with cross· sectional imaging is importallt in all cases where FDG imaging is undertakcn.
  
  Further management PET CT resulrs should be discussed within a multidisciplinary team \,ith the purpose of deciding whether the patient would be a candidate for surgical disease clearance.
  
  248 Right lower lobe bronchogenic carcinoma (left) and likely metastasis (right).
  
  50
  
  25 fe male, prc\'iollsly - the USA, presented for a t chest mdiograph .
  
  E 26 ry :+-year-old male presented with gia .
  
  51
  
  Answer 25
  
  Chest Imaging
  
  ANSWER 25 Observations (25a) This iron tal chest rndiograph demonstrates widespread 1- 3 nUll diarnclcr miliary nodules seen Ihroughom barh lungs with no zonal predominance. The miliary nodules arc of cakifk density. Popcorn calcification ofthc kIt hilar lymph nodes is $Cen. In addition, there is amorphous calcification $Cen under the lell: hcmidiaphragm, which is likely to be in the $plccn.
  
  Diagnosis Histoplasmosis.
  
  Differe ntial diagnosis For increased density miliary opacities: • MiliarYlllcrastascs Pneumoconiosis - silicosis, si.dcrosis, baritosis.
  
  Varicclb-zostcr (25b ). H:lcmosidcrosis - due to chronic pulmonary venous
  
  hypertension, pu]mon:,uy haemorrhage, o r idiopathic. H istoplasmosis.
  
  and haematogenously to the spleen. Acme infection usually presents with few nonspecific symptoms, and radiological findings include gencralizcd lymphadenopathy, flitting nonscglllental bronchopneumoni:l, multiplc milia ry nodules, popcorn calcification ofhihlr/mediastinallymph nodes and splcnic calcification. Chronic hislOpiasrnosis is seen in patienrs with chronic obstructi\'c airways disease and has r:l.diological features of peripheral consolidation and apical fibrosis, Disseminated infcction can occur in immunocompromised patients. Nonpulmonary features of hisroplasmosis include pericarditis (5- 10%) and rheumatologic syndromes (-6%), c.g. arrhrnlgia, erythcma nodosum.
  
  Practical tips In cases whcre there arc multiple radiolosical findings, consider the diflcrential diagnosis liST for each finding and idemi!)' an ovcrlapping diagnosis (easier said than done in a \'h'asilllalion! ).
  
  Further management For popcorn calcification oflymph nodes: • Sarcoidosis. • Silicosis. Hisropbsmosis. • Coal workcr's pncumoconiosis «(,,,\VP). • L}'lllphoma - post radiotherapy . Forsplcnic calcification: • Tuberculosis. Histoplasmosis. In F.lrcts secondary to sickle cell discase. • H rd:nidcysts, • Haematoma.
  
  Discu ssion Histoplasma cnpSlllnwm is a fungus usually found in temperate clim:tres and most commonly in thc northern USA. Infection is by inhalation of air bornc fungal sporcs. These g..:rmin:ltc in the :l1vcoli and then spre;ld via the pulmonary Iymph:llics [0 the hilar/Illediastinallymph nodes
  
  25b Multiple calcified tiny nodules at the left lung base in a patient with old varicella pneumonia,
  
  52
  
  Clinicll/occupational history and I-IRCf can be useful to dillcrcntiate the possible undcrlying d!;\gnoscs. Respiratory referral with a \'icw to antifunSll trcatmcnt would be required ill the acute infcction.
  
  Further reading Brown K, Mund OF, Aberle DR, ct at. (1994 ). Imrathor;lcie calcifications: radiographic fcatures and diflCrential diagnoses. RndioGrnpIJies 14: 1247-1261. Whelt LJ, Wass J, Norton J ( 1984). Cavitary histoplasmosis occurring during tWO large urban outbrcaks, Analysis of elinical, epidemiologic, roentgcnographic, and Jaboratot)· fe:ltures. Mtdici"t ( Baltimore ) 63(4 ): 20 1-209. 'Vilear LJ, Connolly-Stringfield PA, Bakcr RL ( 1990), Disseminated histoplasmosis in the acquircd immune ddiciellC)' syndrome: clinical findings, diagnosis and trcat'lllcllI,and rcvlcwof\hclil'erarurc. Mcdicim (Baltimore) 69( 6 ): 361.
  
  er 26
  
  Chest Imaging
  
  ations (26a) IIOntal chest radiogr-aph dcmons[r.ltcs a right sided .arch. No left sided aortic knuckle is seen . The hean enlarged. No IOcal1ung parenchymal abnonnaliry is
  
  Case 27
  
  Practical tips Right sided aortic arch call gin: a notch in the posterior aspect of the upper oesophagus on COntr:lSt swallow examination .
  
  Further management No further managemem is required in this condition.
  
  _
  
  is can be contirmed wirh arterial phase contrast -ed CT chest (26b ). Right sided aortic arch can be ed with scveral congenital cardiac abnormalities but .I.lso be see n in p:nicms without cardiac abnorThe lau er grou p of p:l.IiclHS usually h:l\'c :J. right .Ionic arch with :In aberrant Jeft subclavian artery g as the most distal branch ort he ao rtic arch ), which behi nd the ocsophagll~. This can be seen on a swallow examination as a posterior indcmation in t:wium column of the mid oesophagus. Patients with sided aortic arch with mirror i1ll3gC branching, such the leti: subclavian arises as a branch of the first ,"esse! dlc Jo rtic arch, arc the group usually associated with tic heart disease. The aorta descends in the right crior mediastinum (although in a small proportion, .;; this is 011 the lett). 26b Axial CT image demonstrates the right sided aortic arch.
  
  e ASE 27 to ry 34 -year-old smoker presents with ressivel)' worsening shortness of ath.
  
  53
  
  Answer 27
  
  Chest Imil ging
  
  AN SWER 27 ObH rvllions l27"1 Th, ,h,,' , .. I"5''I'h
  
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  Chest Imaging
  
  CASE 28 Histo ry A
  
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  Answe rs 28, 29
  
  Chest Imag ing
  
  ANSWER 28 OI>& ..".lIono i2b. b._I m~
  
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  Chest Imaging
  
  Tbcsc same lindings 3fe visible all CT. Alveolar Ollid often ~ nrs as 'ground glass' opacity, which is an increased ::u.zincss/ attc.nuation in the lling - and which can be chy in distribution. 'Ground glass' opacity is a relatively ..onspccific imaging finding due [0 many conditions that .::aISC an overall increase in density within the segmellt of g displayed as a pixel on the CT image. There is a long ( remia! diagnosis including any cause of alveolar . j collsolidation.
  
  Case 30
  
  Practical tips A combination of cardiomegaly, pleural clTusion and airspace opacity suggests cardiac f."tiluTe with pulmonary oedema.
  
  Further management Medica! management with eXR radiological tollow-up as appropriate .
  
  CASE 30 iistory ~ 6 5 -vear-old male was referred tor :l ardiac MRJ to assess cardiac viability.
  
  57
  
  Answer 30
  
  Chest Imaging
  
  ANSWER 30 Observations (3Da, 30b) The first image (3 0a ) is;t shorr
  :md anterior walls. The visualized posterior, lateral and inferior walls show 110mlal enhancement and arc of nOnllal thickness . The second im:agc (3 0b) is a delayed lour chamber ,-jew showing delayed, prolonged cnhanccmcm of the septal, apical and 3lHcrohncr;11 wall of the left ventricle. These appearances :HC of an extensive left "cnrricular infurct wit h no evidence that inl"olved areas arc viable.
  
  Diagn osis Left \'cntricubr inrnrct involving Ihe septal/apical! amcrolatcralwalls.
  
  Discussion Cardiac MRJ has 311 expanding rolc and current uses include asscssmentof: Cardiac viability prior to re\'ascubrization. Cardiac congenital heart detect Cardiac tumours. Perieardialdiscasc. Canliomyopathics.
  
  Shorl a.'(is cine images arc acquired at first Pfl~ of a bolus injection of gadolinium to determine perfusion, Infurcted myocardium shows no enhalll::ement on lint-pass imagin! (as demonstrated in this case), In addition, first pass imaging can show: • Whether the infarct is transmural or subendocardiaL • Degrce of hypo/akinesia. Delllycn..cnhancel1lenr sequences al approximately 5 min show enhancement in infarcted tisslle sillce clear:lllce of contrast from fibrotic tiSSlle is slower lh:lll from normal nH'ocardium. · Ifthere is :lny uncert.linty regarding ditlercntiation of ischaemic from inf.ucted mnxardi um then cardiac MRI stress testing is per!armed \~'ithftNl-PIlJ1 images acquired at stress with adenosine and then repeated afTer 20 min al rest. Areas of hypocnhancement at stress that show recove!)' at rest represent areas of ischaemia rather than in&rction. .,\
  
  Practical tips Cardiac MR.I is a d~'namic test that requires assessment of cine inllges to make a subjecti\'e and objecti\'e assessment of1cfi \'enlricular function (ejection frnction ).
  
  Further management Assessing cardiac viability post 1l1~'ocardiaJ infarction is important since re\'ascularization of li\'e tissue reduces morbidity and morraliry. Previously cardiac pcrfilsion was assessed b~' cardiac nuclear medicine (lIHBG - Illetaiooobenzylguanidinc [scintiscan }) stress resting but there is now an increased role for cardiac M ItI.
  
  58
  
  Coronary artery atherosclerotic disease is ch:lr,lCterized using coronary angiography or corona!)' artery CT Patients with isehaemic but \'iable mYOi:ard iulll ma y be suitable for re\':lscularization with angioplasty or bypass gl'Jfiing,
  
  Chest Imaging
  
  Cases 31,32
  
  :.ASE 31
  
  Iis:ory - ~ ~-old
  
  female patient prcscmcd
  
  .:best pain.
  
  tASE 32 ry ':'i-ycar-oJd Illale presented with ~ naftcrminortral1111a.
  
  59
  
  Answer 31
  
  Chest Imaging
  
  ANSWER 31 Observation s (31a)
  
  Discuss ion
  
  This chest radiograph shows e\'idence of a pn:,'ious rigln llIastcctonw. !-lean :lIld mediastinal conrours are normal.
  
  '''ith breast cancer being such a common malisnan ~·. complications will present frequemly on plain radiographs so it is important to note :I mastectomy. Another 'tell tak' sign of pre\·iolts bre:lst cancer is the presence of a:;:illaTT clips from node sampling. The mastectomy may first be percei\'ed as a disparity between the o"eral1 densities of the tWO hemithoraces.. There arc many other causes for this, though sometimes may not be e:ls), deciding which side is norm:ll.
  
  No lung 3.b'llormaliry is seen. There is ditnlsc sclerosis of all visible bones, mOSt evident in Ihe ribs, Appearances arc of difli.Isc sclerotic mct:lSI":lSCS from a breast carcinoma primary.
  
  Diagnosis "hstcctomy and sclerotic bone metastases,
  
  Differential diagnosis For causcs of unibtcraJ hypcrtr.lllsr.1dbncy:
  
  Practica l tips
  
  • Chest wall abnormality - mastectomy, pecroral muscle
  
  In :lny c:lse with a history of breast cancer, the chcsr radiograph should be scrutinized for features of recurrence. These ~lre classical "iva films for exams :lnd also presc frequently in e"el)'ciay practice. Features to look fi include: Sclerotic/lytic bone metastases (31d ). Lung lllet~lstJses. Lymph:lngitis C;J.rdnomafQsa. Pleural effusion. A-.;illary lymphadenopathy. Ri ght hemidiaphragm ele":ltion secondary to li,·er metastases. • PUilllOnal)' pnelllllonitis/ ilbrosis from radiothcra~·
  
  atrophy (polio ) or absence (Pobnd's syndrome). Pleur-l1 almorm:tlit), - pncu1l1othor:lx,
  
  Lung abnormality - Swyer-James syndrome (consequence of bronchiolitis as a child resulting in a hypoplastic lung with air tT:lpping on expiration ), emphyscma, bulb!.', pulmon:ll)' embolus (3 I b ), For causes of unilater:tl hyperdensity: Chest wall :lbnormalil)' - gynaecomastia (unilater:tl in 40% of cases), breast impl:\l1I. • Pleural abnorm:llity - pleurJ.1 dfusion on supine film, pleural thickening. LUl\g abl\orma\it~, - ul\ilatera\ pu\mom.\}' oedema from lying on one side (3 I c), consolidation, lobar
  
  collapse (especi:llly left upper lobe ).
  
  31b There is a large central pulmonary embolus on the right with marked reduction in vascula r markings on this side.
  
  60
  
  Further management • In cases where clinical history and examin:ltion do revcal an olwious cause lor the rdati,"c diftcrenccs chest lucency - CT chest would be appropriate. Isotope bone SCJn may ident"if)r distant bone metastases.
  
  31c Unilateral pulmonary oedema with pleural effusion and airspace opacity on the right.
  
  An swers 31, 32
  
  Chest Imaging
  
  d Multiple poorly defined lytic lesions are seen ;;:TOug hout the ribs bilaterally .
  
  SWER32 Observations (32a)
  
  Discussion
  
  chest radiograph shows no ti.xallung abnormality and rib fracture or pneumothorax from the recent trauma. "e\"er, there is bilateral inferior rib notching involving 3rd- 8th ribs. The aortic knuckle is not well seen bllt is no other mediastinal conto ur abnormality. The ..art is not cnl:lrgcd. There is no evidence of previous ~Jiac surgery. No other specific bOlly or soft tissue
  
  The most common cause of inferior rib notching is coarctation of the aorta. This can be of [\vo types: • Preductal - the hypoplastic n~rro\\'ed segment is long These patients present in inf3ncy and early childhood with congestive c~rdiac fJilurc. Prognosis is worse than lor those who present with t.he postduct"al type . I'ostductal - this Ilsuallv consists of a short narrowed segment, immediately distal to the site of the ligamentum arteno sulll (32b). Presentation is llsllally in latef childhood, and is with hypertension, diftcrential blood pressures in the upper and lower limbs and / or a heart IllUfmur. ( COIlt.)
  
  ~rmaliticsarcsccl1.
  
  The most likely diagnosis is coarctation of the aorta p:nicnr's blood pressure should be checked in both and compared with that in the legs for confirmatory
  
  :Jiagnosis - 'or rib notching due to coarctation of the aorta.
  
  - ere ntial diagnosis inferior rib notching: ~\n:eriaJ :
  
  Coarctation of the aorta . • Subclavian obstruction after Blalock- Taussig shunt fo r tetralogy of Fallot . • \·cnous: SVC obstmction. Arteriovenous malformations (AVlvl ). :S-curogcnic, neurofibromatosis. superior rib notching: Rheumatoid arthritis, sckrodenna and SYSTemic lupus crythcmarosus (SLE ). H~~rparatbyroidism.
  
  :S-curofibromatosis. ~larfu n's syndrome. ~uicti\'e IUllg disease.
  
  32b Single angiographic im age shows postductal stenosis of the descending thoracic aorta, with postste notic dilatation.
  
  Answer 32
  
  Chest Imaging
  
  On chest radiographs, the focal Il:J.ITowing of the aorta is cbssically made more (vident because of pre- and posl'Stcnoric dilatation producing a classic ' tigure of 3' sign. Obscu ration orthe arch, as in this case, is also recognized. Ri b 1l00ching on the inferior surfucc of the 3rd-8th ribs can llsuall}, be seen in untrc:ltcd patients by 8 years. Rib notching occurs due [0 dilatation of the posterior imcrcostal arteries, which act as collateral vessels. Since the 1st and 2 nd posterior intercostal arteries arise from the COsfoccl"l'ical trunk of subcbvian artery rather than descendi ng aorta, they do nOt form a collateral path and hence do not cause rib erosion. Coarctation of the :10m is associated with sc\'eral orher congenital anomalies such as bicuspid aortic valve, patent ductus arteriosus (PDA), \'ctHricular septal dcfcC[ (VSD), tricuspid atrcsia and transposition of the gre;lt \·csscls. There is also an association with Turner's syndrome.
  
  When inferior rib nmching is noted check: The aortic comour for the 'figure of 3' sign. Is therc evidence of previous repair, e.g. thoracotonw scar? The hcart fo r e\'idence ofldi: ventricular hypertrophy, i.c. elevation of the apex . Arc there features to indicate neurofibromatosis, e.g. cutaneous soft tisslle nodules? When rib Ilmching is unilateral, suspect an aberrant $ulxla\'ian artery origin on the unaflected side.
  
  Further ma nage ment Surgical treatment for coarctation of the aorta in\,oh'es resection and end to end anastomosis, prosthetic patch graft, sulxlavian fbp aOrToplasry or balloon angioplasty.
  
  Practica l t ips When suspected from plain fi lms and clinical findings, t.'IR or CT angiograph~' has now largely replaced com'emional a n giograph~' as the next im'cstigatioll of choice (3 2c).
  
  32c A maximum intensity projection (MIP) image from an MRA exam ination of the thoracic aorta.
  
  62
  
  Chest Imaging
  
  Cases 33, 34
  
  SE 33 ory 38-year-old man was Ixing tigatcd fo r transient neurological
  
  ".
  
  CAS E 34 ory 51-year-old woman with oesophageal er presented complaining of chest
  
  63
  
  Chest Imaging
  
  Answers 33, 34
  
  ANSWER 33 Observations (33a)
  
  Further management
  
  There 3TC some incrc3.scd tubular sofi: tisslie dcnsitv opJCitics in the right lower zone, suggcstiyc of abnormal vessels, leading TO a faim pulmonary nodule. There is a funhcr nodular opacity in the right lIplXT zone, again \Iith Ihe suspicion of vessels running to it from the right hilum. The left lung is CicJT and the mediastinal outline normal. Given the clinical details, pulmonary ancrion:nous mallormations giving rise to paradoxical emboli and TIAs l1msr be presumed. Contrast cllh:lllccd CT would confirm.
  
  Angiographic assessment and treatment with embolization or balloon occlusion is now the preferred management.
  
  Further reading Coley SC, Jackson JE ( 1998). Pulmonary arteriovenous maltormations. C/jIJicn/ Radi%gy 53(6): 396-404. Pick A, Deschamps C, $tallSOIl AW ( 1999). Pulmonar)' arteriovenous tistula: presentation, diagnosis, alld treatment. World journal ofSllrgery 23( 11 ): IIIS- II22.
  
  Diagnosis Pulmonary :lrtcrio\'cnOllS m3ltonm.tion (AVM ).
  
  Differential diagnosis Single or multiple pulmon;uy nodules frOIll other causes.
  
  Discussion Pulmonary AVMs arc abnormal \'Jscular communications between pulmonary arteries and \·eins (95%) or systemic arteries and pulmon:lry veins. 1\10st commonl~', they arc of the simple type, with :I single artery feeding :I focal aneurysmal segment and a single dn)ining vein. Compkx lesions ha\"e more than one artery and/or vein. Figure 33b is a single angiographic image tll;lt demonstrates the large feeding vessel to a solitary AVl'vl Multiple pulmoll:lr)' AVMs may be associated with Osler-Weber- Rcndu syndrome. They arc usuall~' asymptomatic until the 3rd-4th decade when they can present with local effects - haellloptysis, d)'spnoca on exertion and cyanosis with clubbing (due to right LO lefi: shunt); or \\~lh distal eflects - cerebrovascular accident (CVA ) or brain abscess due TO paradoxical emboli. Lesions enlarge with age.
  
  Practical tips The 'finger in glm·e' appearance from mucoid impaction of cemfal ;lirways in allergic bronchopu lmonary aspergillosis can look similar in some ways to the vessels supplying an AVM. H owever, there is a nodule at the end of these \·cssels in AVI'.,.\
  
  33b Single image from an angiographic investigation demonstrating a large feeding vessel to the pulmonary AVM .
  
  ANSWER 34 Observations (34)
  
  Differential diagnosis
  
  T his chc:st radiograph shows a normal sized heart and clear lungs. There is, however, c\·idcnce of air within the mediastinum - best seen aTOund the leli heart border and aortic arch. Helow the medialldi: di;lphragm, a stent is just about visibic across the gastro-ocsophageal junction. It is likely that there has been oesophageal perforation from the stemed oesophageal tumour
  
  For causes of pneumomediastinul\J: • Al,'eolar rupture in acute asthma. • Oesophageal rupture due to malignancy, trauma, l"iolent vomiting ( Boerhaa\·..:>s syndrome - usually associated with a left sided pleural effusion). Extension from peritoneum - pneumoperitoneum. Iatrogenic - following oesoph ageal balloon dilatatioJl/stenting, bronchoscopy, ll1cdiastinoscopv. positil·e pressure \'emiJalion. .
  
  Diagnosis Pneumomediastinum oesophageal cancer.
  
  64
  
  from
  
  perforation
  
  of stented
  
  Chest Imeging
  
  n num is an uncommon condition that is m.nic but can present \I'ilh ncdcor ches!' 11 rarely leads to any complications bur it can dUgnostic sign for an underlying medical th.u does need trcarmen!. Although a ['an:: _ ~iuhs, it is see n in children and masa
  
  Case 35
  
  • Look for an cffilsion to suggest oesophageal injury, or oesophageal stem, as in this case .
  
  Further manag ement Chcst CT after the patient has drunk some water-soluble contraSt , o r a water-soluble contrast swallow, can confirm an oesoph:ageal tear and leak and help to idcntif}' the site.
  
  Further readi ng Gcrazounis 1\.\, Arhanassiadi K, Kalantzi N, Mousr:m ias M (2003 ). Spontaneous pneumomediastinum: a rare benign entity. J01I'-"(I/ of711omcic alld Cnrdiol'aJw/flT Surgery 126(3): 77~776.
  
  tips rlx' ribs for any sign of trauma. lAX lung fields for a lesion that might hal·C bronchoscopic/ surgical investigation. 4fr under the diap hragm to indicate ·toneum .
  
  .old male presented to A& E 1'a:Urrenr acute-on -chronic
  
  65
  
  Answer 35
  
  Chest Imaging
  
  ANSWER 35 Obse rvations (3Sa) There is a large area of increased lucency in the right mid and upper zone with no visible.:: lung markings. There is no visible lung edge, howeve r, to suggest this is a pneumothorax. Moreover, the re is crowding of"csscls in the lower zone. The appearances arc likely ro indicate a large upper lobe bulla. There arc no focal pulmol131")' opacities to indicate superadded infcClion . ComparisOI;I with an}' ;l\>;lilable old films should help confirm this interpretation. A general reduction in density in the left uppe r zone suggcSlS rhal similar pathology is del'doping here tOO
  
  Diagnosis Large right sided bulla.
  
  Discussion A bulla is a large dila ted airspace within the hmg Witll a \\"JJl less than I mill thick. They arc usuallY' produced byah'colar dcstruction in "'mphyscUlII, Impaired ventilation ofrhe rest of the lung can result in dyspnoea, as well as that due to rhe background chronic lung disease. Confusion Cln lrise when the absence oflung mukings leads to the erroneous diagnosis of pneumothorax. This
  
  35b Image demonstrates a small pneumothorax with no lung markings lateral to the lung edge.
  
  66
  
  can have dire conscquences if an intercostal chest drain is then mistakenl~' placed in the bulla! An expiratory fi lUl makes a pneumothorax easier to de tect ( because it enhances the cont rast di ffe rem ial between pleural air and lung parenchyma) but probably won't hel p resolve diagnostic confusion with a bulla. Patients with chronic lung disease may well ha\'e pre\'ious filrns that show a bulla TO be longstanding.
  
  Practi cal tips A pneumothorax leads to two findings: a visible lung edge and hypodensity with absent lung markings ilteral to this (35b). A bulla has absent lung markings but not the discre te lung edge. COIlI'ersciy, skin fo lds (seen mOST freqlleml~' in babies and the elderly) can produce a pseudo lung edge but the re \\~II be lung markings lateral to iT
  
  (35,). Further management If diagnostic uncertainty continues after expert review of the films, then occasionllly CT of the thorax may be needed to diflerentiate bullae from pneumothorax.
  
  35c Chest radiog raph demonstrates a skin fold giving an apparent lung edge but lung markings are visible lateral to it.
  
  Chest Imaging
  
  Case 36
  
  . . . lib Sequential chest radiographs taken 4 months apart.
  
  36c HRCT thorax of the same patient at a later dale.
  
  6i
  
  Answer 36
  
  Chest Imaging
  
  ANSWER 36 Observat io ns (36a, 36b, 36c) This frontal chest rldiograph (36a ) demonstrates wide spreld, fairly symmetTicll lung plrcnchymll abnormality that is central in distribution and chnr.lo.:terized bv bronchial \\'all thicken ing and cystic bronchiectatic chl;'ge, Some larger soft tissue density nodules arc seen in the lower zones, which likely represent mucous plugs. Bibteral lobull!ed hibr enbrgement is seen with the right sided Ir mphadenopathy being more evident. Appearances arc of cystic fibrosis. Chest radiogrlph (36b) from the same plticnr t:l.ken at a latcr date demonSlrat"es more ad\'anced disease with more mucous plugging and hyperinfbtcd lungs secondary to air trapping. Peribronehill culling and thickencd 1.1Ontapering bronchi arc e\'idem. A right subclal'i;lIl central hne is noted, presumabl}' for the administration of IV antibiotics, A c r image (36e) of the chest on lung windows shows typical reatures o f CF with cylindrical brollchie([asis and a central distribution, peribronchial euning. mucous plugging :and lOCal :nclectasis. It also shows the most common complic:ation of:a pneumothorax on the lci't side.
  
  Di ag nosis Cystic fibrosis (CF).
  
  Di scussio n Cystic fibrosis is an :autosolll:ll recessi\'e Illultisystem condition that is chlrl(terized by exocrinr gbnd dysrunction due to mucous plugging arising sccondlry to a fuull in rell chloride transport. The condition aHects whites with a geographical distribution :affecting Europeans and Ashken:azi Jews. Di:agnosis is usually made in children, \\;th the majority being diagnosed within the first year of life with the clinical presentation of meconium ileus and respiratory symptoms. Pulmonary complic:ations are the predom inant cause of de:ath and sun'ival is limited to -30-40 rears.
  
  36c HRCT thorax of the same patient at a later date.
  
  68
  
  R:adiological features ofCF on a chest r.ldiograph arc: Cystic/cylindrical bronchiectasis. Peribronchial cuffing/thickening. Mucous plugging with secondlry lte1cctlsis due to obstruction. • I-lilar lymphadenopafhy. • Allergic bronchopul"lonaryilslX'rgil losis (ARPA).
  
  ~
  
  Respiratory complications or CF include pneumothorax. haemopt)'sis and cor pulmonale. Chronic pulmonary infecrion can lead to hypertrophic pulmonary osteoarthropathy ( H POA ). • Cystic fibrosis is a multisystem disease: as wdl as causing meconium ileus (in, chi ldre n ) and meconium ileus e(luivalent (in adults), It Clll also callse pancreatic insufficieney, biliary cirrhosis, portal hypel'lension, malabsorption due to gallbladder disease, cholelithiasis lnd dubbing (36d).
  
  Practica l tips • Typical presentation is of\\;despread pulmonary disease in a young patient. • Tunnelled cenrrallines in patient's arc used for long term drllg treatment with antibiotics or ":ichemotherapy. Again this is a clue 1'0 the underlying diagnosis, Look for complicltions ofCF - pneumothoraces and secondary infections. Though nor a common site, I-I POA Clll occur in the humerus and may thus be visible on the edge ofthr film.
  
  Fu rt her ma nage me nt Follow-up in these patients is bc5t lX'rformed with HRCf which can answer the important quest ions when lunf transplant is considered - is there coexistent Aspergill infection? Arc there complicating felttlTeS, e.g. pneurrx thomces?
  
  36d Sing le axial CT image d emonstrates significant splenomegaly due to portal hypertension and pancreatic calcificatio n secondary to recurrent bouts of acute pancreatitis.
  
  Chest Imaging
  
  Case 37
  
  - rar-old IV d rug use r presented with cough and rtncss ofbrcarh. He is nored to be signific:lOtly - on prescluation. This series of chest radiographs n at presentation, day 3 and day 7.
  
  69
  
  Answer 37
  
  Chest Imaging
  
  ANSWER 37 Observations (37a, 37b, 37e) The firs t chest film taken at presentation (373) shows no obvious radiologic:tl 3bnorm3lity. The next radiograph from 3 days later (37b) shows bilateral mid and lower zone rcricu!ar/gr:lIlular inrcrstitial infiltrate with no evidence of volume loss. The third film trom 7 days after presentation (3 7c) shows bilateral consolidarjvc change affecting both lungs. There is :J. pneumothorax on the left with subtotal collapse of the left lung. Surgical emphysema is noted. It is notable that the patiellt was initially vcry symptomatic with a relatively normal chest film and then showed rapid pulmonary changes over several days. The history of IV dmg abuse raises the possibility of background HIV infection and the sequence of radiological appearances is typical for Pllwllloeystis e""illii pneumonia.
  
  Diagnosis Pncumocystis carinii pneumonia (PCP). Discussion P. carillii pneumonia is the mOSt common cause of interstitial pneumonia in immullocompromised patients Radiological find ings can be very variable but the typical pattern of development is as follows: • Normal chest radiograph is seen in up to 40% of patients, especially carlyon in the infection. Bilateral diffuse perihilar airspace/granular/reticular opacities is the typical appearance. Progression to diffuse airspace consolidation with air bronchograms. Pleural effusions arc seen in -20% of cases. Response to treatment occurs over a period of - 1 week Atypical features (seen in - 5%) include upper lobe thin and thick walled cysts, IUllg nodules, mediastinal and hilar lymphadenopathy. Treatment with aerosolized pentamidine alone results in the d isease af1ccting upper lobes.
  
  70
  
  Complications of PC P arc common, e.g- pneumothorax and superimposed TB/fungal infections. Appearances on CT include a patchy mosaic/'ground glass' pattern with subsegmental sparing and coexistent thin walled cyStS and pneumatoceles. Appearance of nodules on CT imaging is suggestive of a second disease process being involved - metastases, l)'mphoma, septic emboli or Kaposi's sarcoma. Bilateral gallium uptake is seen prior to radiological changes being evident.
  
  Practical tips A strong index of suspicion for PCP should be maintained when an immunocompromised patient presents with dyspnoea. As in this case, symptoms can be OLit of proportion to the initial radiographic changes. Further management Diagnosis is confirmed with sputum cytology or bronchoscopy and lavage. Treatment is with IV cotrimoxazole. Further reading Kuhlman JE, Kavum M, Fishman E K, Siegelman $S ( 1990 ). PIIClllI/oeystis carinii pnellmonia: spectrum o f parenchymal CT findings. Radiology 175: 7 11 -7 14.
  
  Chapter 2
  
  ABDOMINAL IMAGING
  
  IN ABDOMINAL RADIOGRAPHS ays, a s)'Stcmltic approach is required to be able to lhc mOSt information from a radiograph. T he order • led is a guide and should be adapted to your own lliettnccs and adjusted for each individual case. :t
  
  -
  
  era l assessment r assess the qu:..ti t)' or the film, ensuring that there .kquarc cQ\'cragc - arc the hernial orifices cQ\'cred in p.uicm who appears to h:wc small bowel oOOmtetian? ..dtition, make a quick assessment of the film to exclude important di:tgnoses that arc surgical emergencies and rt'qui rc immediate management: • PtttOration. • Tone Illcgacolol.l ,
  
  es ufy any lines that arc present, e.g. postsurgical dnins, tu bes, urill:!r)' catheters and peritoneal dialysis ters. The pre$Cnce of peritOnea! dialysis catheters or rgical drains may offer a simple explanation for free .a.pcriwneal gas.
  
  forget 10 check for bowel loops extending be low the inguinal ligament indicative of hernia. Exclude bowel dilatation and wallthiekening. If there is dilatation, decide whether it is small or large bowel and then try to cst:l.blish the 'cut ·oW point. wter ClSCS illustrate specific features to check for when one suspeCtS small o r large bowel obstruction .
  
  < '-\
  
  Soft tissue organs Check the size and outline of the liver, spleen and kidnq'S. Also check the psoas outlines whic h ma ~' be obscured by retroperitoneal pathology .
  
  ~'\.,
  
  Calcification Look at the rest of the film for calcified densities such as gallstones ( onl~' 5- 10% visible on plain radiographs) and renal tract calculi (80-85% "isiblc on plain radiographs). Common incidental calciftc opacitics include mesenteric nodes, phleboliths , ,·ascular calcification and uterine fibroids. Calcification within the solid Ofb':lns may be more significant howeve r. Unusual calcified opacities may allow fOf a specific diagnosis such as a pelvic dermoid.
  
  Gas pattern erentiating sma ll from large bowel is \'ery difficult in ng chi ldren and one can usually only say whedler a k m is in d ll: pro.~i mal or distal bowel. In adults, raures uscd to distinguish the two include position in the o men; d iameter (I:uge bowel <5 cm, small bowel <3 ; \'alvube conni\'t:ntcs travcrse the whole \\~dth of small 1::1 but this is nor so with the colonic haustralions; solid es onl\' scen in colon. The ra~ge of ' nor mal' bowd gas pattems is rather wide, t from experience, o ne should quickly know whether amount and dist ribution of bowel gas arc. \·cry ormal. Unusually shaped collections of g:liS (e.g. ~ngular ) should raise suspicion of gas outside the GI :no- and lead rou to examine thc area very dosely. Check .:Mefully for gas in areas of the abdomen where it is not wormally seen, e.g. o\'er the liver (either as pockets of g3S 'JI"\\;thin the biliary tree ) or in the relroperiwncum. Don't
  
  Bones Assess thc bones of the ve rtebral column and pe lvis fo r incidental pathology or findings that might be rclc\'ant to intra· abdominal disease s uch as sacroiliac joint changes associated with inflammatory bowel disease.
  
  Periphery of the film Conclude by looking at peripheral stmcmrcs. For example, patholog), at the lung bases that might mimic abdominal patholoID', incident:l.llcsions in the abdominal wall.
  
  ABDOMINAL CT The first qucstion to ask yourself when looking at all abdominal is what type of comrasr has the patient been gh'en, i.e. oral/IV/ both and during what phase has imaging been carried Out. T he typical phase for abdominal
  
  cr
  
  71
  
  Abc!ominal Imaging
  
  imaging is portal-\'enous (60-70 seconds delay) bur other phases, e.g. pre-contrast, :mcrial, delayed, arc useful in characterizing spedfic lesions. Axial im::agcs will commonly be presented and you can proceed in one of two ways - either :lSSCSS all stmctures on each ax ial image then proceed to the next image, or, alternatively, assess each org:m in mm o\'er a series of slices before moving on to the next organ. in rcalit... , a combination of the tWO is often used but the J:mcr ensures nothing is missed. As well as searching for tOcal lcsions within the organs, other feature s to check include the fo llowing: • Li\'cr:
  
  O verall attenuation (e.g. reduced in fatty change) and enhancement homogeneity. Size :Illd comour (e.g. irregular contour in cirrhosis).
  
  Patency of normal vascul::lrstrucrures. $mte of intrahepatic bile ductS. Gallbladder - check for stolles, the density ofbilc, wall thickening and perieholecystic fluid. Carefully assess the extrahepatic bile ducts.
  
  72
  
  Pancreas - check lor calcification; the state of the pancreatic duct, pcripancreatic collections and infl:lnlmatofY changes. Spleen - check sizc and adjacent \'::Irices. Kidneys - is the :menuation orthe kidneys correct for that phase of scan? Is there:: collecting s~'Stem dilamtion? Check the adrenals abo\'e. Then systematically evaluate the non·solid organs: • Aorta - as well as o\'erall size, du:ck for dissection and patency of main branches. Check major venous patency at the same time, excluding deep vein thrombus. Bladder, ovaries/ut erus. Lymphadenopathy. Intra -abdomi nal fat - peritoneal soft tisslle deposits arc easily overlooked unless the abdominal fur is careful!vscrulinized. Colon, 'ileum and stomach. Free gas/flui d. Bones and lung bases.
  
  Abdominal Imaging
  
  Cases 38, 39
  
  - :car-old male presented with a of gradually progressive
  
  .,.
  
  SE 39 ory - -year-old female presellted with bkcding.
  
  73
  
  GENERAL INTRODUCTION
  
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  Answer 38
  
  Abdominal Imaging
  
  ANSWER 38 Observations 138a)
  
  Practical tips
  
  Single AP image from a double cammst barium swallow examination shows a short, smoothly i.lpcrcd narrowing in rhe lower ocsoph
  Previous CXRs call ~ useful in identifying a cause for a stricture - look for a tlll110UT that might have been irradiated or fratures of aspiration pneumonia/hiatus hernia.
  
  OCsoph3guS proximal to rhis h:l.S delicate tr,Ulswrse mucosal
  
  Furth er management
  
  folds, the so-called fcline oesophagus. This 3.ppe31"311CC is 35S0Ciatcd with gastro-ocsophagcal rdlux and leads to the conclusion that the shon stricrun: is a peptic stricmrc.
  
  GastrOenterology referral \\~th a view to direct \;sualization and confirmation of diagnosis with endoscopy,
  
  Di ag nosis
  
  Further reading
  
  Peptic stricture fcline oesophagus.
  
  Luedtke P, Lc\-illc MS, Rubcsin SE, tt nl. (2003 ). R.,diologic diagnosis ofbcnign esophageal strictures: a paltern approach. RadioGrnplJics 23: 897-909.
  
  Differential diagnosi s For oesophageal strictures: • Lower oesophagus: Peptic stricture secondary to gastro-oesophageal renux, Scleroderma - aflccted patients have an incompetent lower oesophageal sphincter and reduced pcrisl::Ilsis resulting in marked gastrooesophageal reflux. NG intubation - pre\'cnrs'closure orthc lower oesophageal sphincter. Zollinger-Ellison syndrome_ Upper and mid oesophagus: Harrett oesophagus - acquired condition characterized b)' columnar Illel::lplasia secondary to chronic gasrro-oesophageal rcflux/ oesophagitis. Prcmalignam condition with an increased risk of adenoearcinoma of the oesophagus. Caustic ingestion - usually long, smooth narrowing forms 1-3 months l)Osl ingestion. Mediastinal rJdiOtherap), - usuaJJy long, smooth narrowing forms 4-8 momhs post radiotherapy. Skin diseases - epidermolysis bullosa, pemphigoid, ery thema multi forme. Other less common causes of strictures include Crohn's disease, Candidn oesophagitis and Behr;el's disease.
  
  Discussio n Peptic strictures have this typical appearance of short (1-4 cm), smooth, tapered, concentric narrowing in the IOll'er oesophagus. Associated radiological findings include intramural pscudodi\'eniculosis (38b) and feline oesophagus (so called occause this is the normal appearance in cats). Longitudinal scarring can cause fixed [ranS\'ersc folds but these can be diflerentiatcd from fcline oesophagus sincc lhe), arc only seen in the region of the strictur;: and do not extend more than half way ac ross the oesophagus, giving a 'step bddcr' appearance.
  
  38b Contrast barium swallow shows flask shaped outpouchings with a narrow neck of intramural pseudodiverticulosis.
  
  74
  
  swer 39
  
  Abdominal Imaging
  
  rvations (39) image from a mesen teric angiogram examination an abnormal duster ofvcssds and contrast 'blush'
  
  Case 40
  
  Di agnosis can be made with selective mesenteric angiography or CT angiography. Both arc :a ble to idcnrif)' bleeding when the rate is as little as I mljmin. Three levels of abnormalit\, afC identified: In carly disease, a densel)' contrast filled dilated \'ein is seen within the bowel wall. As the disease progresses, a vascular tuft can be seen at the lesion site. Fmther progression shows an early filling vein during the arterial phase of scanning. ;vlcsenteric angiography has the adv,lntage of proceeding directly to treatment with embolization. '1.
  
  y-r ;
  
  _
  
  ysplasia is the most common cause of occult "ng in the large bowd, predominantly aflCcting the y population. The condition is characterized by ar ectasia of the colonic circulation, most commonly - :ring rhe caecum and ascending colon. The condition to chronic low-gr3dc blood loss but can also lead to cs of severe lower G I bleeding. There is an associa\\ith valvular heart disease, specifically aortic stenosis
  
  Practical tips J CT is exce.Hem at ider.1tit~'ing the bleeding point when "\V; there is actIve GI blcedll1g. Always perform a pre -contrast f scan prior to the arterial phase scan so that highcontrast intraluminal blood can be diftcrentiated from high -contrasT bowel tOad content/debris.
  
  Further management Surgical resection is the definitive treatment when endoscopic treatments have not controlled bleeding.
  
  SE 40 ory 35-year-old farmer presented with ache in the right upper abdomen sc...cralmonths.
  
  75
  
  ,
  
  Answer 40
  
  Abdominal Imaging
  
  ANSWER 40 Observations (40a) There is a large, well defined lesion in the right upper quadr:l!ll, which has thin, curvilinear calcification of its wall. This is projected over the li,'cr and is probably inrrahcp3tic, though a calcified gallbladder cannot be excluded from this
  
  film. This is a solitary lesion with no orher abnormality seen. Gi\'cn the appearances and paticnI's young 19C and occupation, a hydatid q'Sl of the li\'cr is most likely. Funhcr imaging with CT would help confirm the location of the lesion and the likcl~' diagnosis. Serological tests for hydatid disease should also be undertaken.
  
  There is a predilection lor the lower lobes and disease is Illore cOlllmonly seen on the right. CySts arc multiple in 2~ and bilateral in 2~ of cases. Figure 40b shows several left sided, well defined round intrapulmonary lesions. Calcification is rare. When air infiltrates between the layers of the cySt wall it can give the appearance of:a 'meniscus' sign, 'onion peel' sign and fin:atly thc 'watcr lily' sign, when there is complete separation of the endocyst from the pericysr. Rupture of the cySt can result in surrounding consolidation.
  
  Practical tips Diagnosis Hydatid disease.
  
  Differential diagnosis Ofca1citicd Ji\'crlcsion:
  
  Metastasis - especially colorcctal cancer. • Primaryli\'crlUmOl1T. • In fection - hydatid, TB.
  
  Discussion
  
  Further management
  
  Hyd~tid disease is ac~uired through infection by the parasitic tapeworm EcJllllO,o'CIIsgr(lllllloSlls. Dogs are the definite host with the human acting as an accidental host following lcddental ingestion of eggs from canine faeces. The li,·er is the mOSt commonly im·oln:d organ and presentation is with abdominal pain, jllllldice, bilia~' colic with eosinophilia in 20-50%. In the lil'er, inlection results in the fomlltion ofa cyst, which is more commonly found in thc right lobe; the size of the cyst ranges up to 50 Clll bur is - 5 em on average and mu lt iple in 20% of cases. The cyst is composed of three layers - the Outer pericyst, middle laminated ectocyst and the inner endocyst. Radiological features ;lfe: Variable appearance ranging frOIll a simple unilocular cyst to a complex hct"Cfogclleolls cystic mass. D:lIlghtcr cysts arc char;lCterislic bllt arc a r;1re finding Their presence is noted by a 'racemose' appearance. Initially
  )\bnagement can be cither medical (t\\"o benzimidazoles arc commonly used, albcndazole and mcbend:azolc) or surgical (cystectomy or partial organ resection).
  
  Complications arc of cySt rupture (50- 90% of cases) and infection. The lun gs arc th e second most common site of involvement in adu lts and the mOSt common in children.
  
  76
  
  • Benign liver cysts afe common, but all cySts should be closely inspected lor atypical features, e.g. h)'l)cTattenllating wall Of wall calcification suggestive of abscess/hydatid; poorly dcfined edgcs, which may suggest the lesion is in fact a metastasis. Cystic lesions involving lil"Cr and lungs should suggest infectivc/malignant underlying diagnosis until proven otherl\"ise.
  
  Further reading Pedrosa I, Saiz A, Arrazola 1, It nl. (2000). Hydatid disease: radiologic and pathologic features and complications. RndioGrnphics 20: 795-817. Polat P, Kantard M, Alper F, It nl. (2003 ). Hydatid disease frolll head to toc. R..ndioGrnplJicJ 23: 475-494.
  
  40b Patient with pulmonary hydatid presented with several large. well defined nodules in the left lung with no calcification.
  
  Abdominal Imaging
  
  Case 41
  
  CASE 41 History .\ 67-year-old, o\'crwcight female p,nicnt, with no past medical history, pr('scnrcd with \'ague abdominal pain, nausea and \·ol1li[lng.
  
  77
  
  Answer 41
  
  Abdominal Imaging
  
  ANSWER 41 Observations (41a, 41b) AI' :abdominal radiograph shows distended loops of gasfilled small bowd but absent colonic gas. TogClhcr with the clinical history, appearances 3fC consistent with small bowel obstruction. There is no evidence of free gas on these films but on the second image there is an abnormal collection of air o\'er the ce!Urnl liver that has a somewhat li near/branching configuration. T his is consistent with air in the biliary tree. In the right side of pelvis, there is a round opacity showing peripheral calcification - this is likely to ind icate an obstructing gJ.llstone.
  
  Di agnosis Gallstone ilcus.
  
  Discussion Gallstolle ileus is rchlti\'ely rare, accounting for 1-2% of all mcch;mic;ll obstructions (though more in the elderly). The most common scenario is of a stone eroding through from gallbladder to duodenum - the cholecysroduodenal fistula leads to pneumobi lia and the Stone then impacts in the Slllall bowel. T he fistula can also be !Tom the comOlon duct, and can enend to thc colon or stomach instead of the small bowel. Occasionally, the diagnosis can be made on plain films \\;th Rigler's triad t>f small bowcl obstruction, pneumobilia and ectopic gallstOnes. Oncn, however, the gallstone is nor seen on plain film since rhe stoncs frequently have a predominant composition of cholcsterol with little
  
  78
  
  calcilication. An axial CT scan of the abdomen (41 e conlimled a gallstone ilcus \\;th a 5 em diameter laminated gallstone found in the distal ileum. The bowel was collapsed disral to rhe site of stone impaClion. At laparotomy, the stonc was milked back to thc jcjunum and rcmo\·cd.
  
  Practical tips Always check for air in the biliary tree on the small bowd obstruction :lbdominal film . • Tilly loculcs of air in the biliary tree tend to be ccntl':llly located in the li\'(r (4Id ) compared with porral \'ein gas, which is seen in the periphery. • Bili::!')' tree gas can also be seen as a normal finding in paticlll~ who hal'l.: had a previous sphincterotomy or [allowing a recent ERe I' (end oscopic rctrogrndc cholangiopancrcatography),
  
  Further management 1\ lcchanic;rl Sl11all bowel obstruclion is a sm gic&.. emergency.
  
  Further reading Gurlcrik G, Gurlcyik E (2001 ). GallstOne ilclls: demographic and clinical criteria sllpponing preopcrati\'e diagnosis. UJI/S TrnJ'lIIn Derg 7 ( 1): 32- 34. Pangan JC, Estrada R, Rosales R ( 1984). Cholecystoduodenocolic fistula with recurrent gallstone ilcus. ArciJjJ'eJo/Surgery 11 9: 1201 - 1203.
  
  Abdominal Imaging
  
  Cases 42, 43
  
  ry n;:ar-old male is admitted with -encephalopathy.
  
  - SE43 ory llymptomatic 22 -),c;1r-old male nrcd w ith deTerioraTin g renal :rion
  
  79
  
  Answers 42, 43
  
  Abdominal Imaging
  
  ANSWER 42 Observations 142) Image from a double contrast barium meal examination shows multiple serpiginous filling defects in the lower oesophagus. Normal appearances of the g,tstric nmdus arc observed. Appcar:lllccs arc consistent with oesophageal varices and the distribution suggests that these arc 'uphill'. Diagnosis
  
  to IVC/hcp;uic vein/splenic vein thrombosis or obstruction. Downhill \'ariccs (found in the mid and uppe r oesophagus); these arc characterized by coll:ncral blood flow from the SVC \'ia the :azygous vein into the IVe, and arise due to SVC obstruction from conditions such as IUllg tumour, lymphoma and rc[rasternal goi tre.
  
  Ocsoph:agcal varices.
  
  Differential diagnosis Fo r ocsophagcal ,·ariees: • Varicoid carcinoma of oesophagus. For gastric variccs (i.c. CJUSCS of thickened gastric folds): • Hypenrophie gastritis. Mcnctrier'sdiseasc. • Lymphoma. • Splenic \'cin thrombosis,
  
  Discussion Oesophageal ,'a rices ha,'e a ,'cry typical appearancc o n contr;lst sw:tllow examination of dilated, smooth, se rpiginous filling defects. Varices collapse in the creer position and are beSt imaged with the patient pronto Thcre arc two types: • Uphill "arices (found in the lower oesophagus); these are charanerizcd by collatcral blood flow from the portal vein vb the azygous "ein to the superior \"ena cava (SVC ). These arise due to liver cirrhosis and duc
  
  Gastric varices arc secn in combination with oesophageal "arices in patients with portal hypertension. When seen.tn the absence of oesophageal "arices, splenic vein thrombosis should be suspected. Again, appearances arc of smooth. serpiginous or grape -like filling defects; most commonl~ seen in the gastric fundus.
  
  Practical tips Best images arc obtained with thc patient in a prone position, Further investigation with an ultrasound of the abdomen should be advised to look for cirrhosis and portal hypcrtension.
  
  Further management Treatment is aimed at controlling poml hrpcrtension \Vim medical and surgical (transjugular intrahcp:ttic ponosy~temic shunt - T IPS) means. Treatment of bleeding ":trices and preemptive treatment of non bleeding varices is achie,-cd with endoscopic banding and sclerotherapy.
  
  ANSWER 43 Observations (43) This pl:lin abdominal radiograph shows multiple small foci of calcification over both renal areas in thc region of the rCIl:J1 medulla rather than ren:J1 cort·ex. No stones arc seen elsewhere along the course of the renal tracrs.
  
  Renal papill:t!), necrosis. Hypelyir:Jminosis D, Milk-alkali syndrome, M:Jlignancy - bone metastases, multiple myeloma, p:trnneopiastic syndrome. Primary hypcroxaluria,
  
  Diagnosis Rell:J1 medullary llephrocalcinosis.
  
  Discussion Mcdullary nephroc:Jlcinosis represcnrs calcific:Jrion in the distal con\'oluted tubules, i.e. in the renal pyramids, There arc m:Jny causcs :tnd the underlying pathology can rarely be determined on a plain rndiograph - clinical history is fur more important here. Tlte C:JlISCS arc: Renal tubular :Jcidosis ( RTA ), Endocrine causes - hyperparathyroidism, hyperthyroidism, C ushing's. Medulla!)· sponge k.idne~' . Idiopathic hypercalcuna,
  
  80
  
  Practi cal tips • The most common causcs of symmetrical medullary nephrocalcinosis are hyperparathyroidism and RTl'.. • T he most common cause of asymmetrical medullary nephrocalci nosis is medulla!)' sponge kidney".
  
  Furth er manage ment Underlying causc must be identified - particubrly tre:ttJbk Furth er readi ng Dyer RB, Chen MY, Zagona RJ ( 1998 ). Abnorm:tl calcifications in the urina!)' trnct. RadioGraphies 18: 1405~ 1 424.
  
  Abdominal Imaging
  
  Cases 44, 45
  
  E 45 ry ·n=Jr-old male presented with dial bloating.
  
  81
  
  Answers 44, 45
  
  Abdominal Imaging
  
  ANSWER 44 Observations (44) This is a single A I' image from a double contrast small bowel barium examination. T here is focal, eccentric n:lrrowing of the second part of the duodenum, with predominant notching of rhe latc",! wall. Appearances of the duodell um superior and inferior to this arc completely normal.
  
  Clinical presentation can be with: • Pol)'hydramnios - i'l utero. • Persistent vomiting, "double bubble' - in neonates. • :-J:lUsca ~ ,'omiting and abdominal pain - in adults. The condition is complicated by 311 increased incidence 01 acute and chronic p3ncreatitis 3nd periampullary I"P';; ' J ulcer.
  
  Diagnosis Practical tips
  
  Ann ular pancreas.
  
  Differential diagnosis For 3.nnubr pancreas: • Sphincter orOddi oedema (secondary to impacted none or pallcrc3tilis)/carcinoma - usually produces an eccentric lesion but this is prcdominantl~' medially located. • Duodenal ade nocarcinoma - usually presems with an annular concemric I<:sion \\;th shouldering and utccr:ltion. There is an association \\~th G:lrdncr ~ syndrome and coeliac d isca~.
  
  Discussion Annular pancreas is a disOrder characterized by fuilure of rotation of the ventral bud of the pancreas resulting in pancreatic tissue encircling the duodenum. TIll: sccond pan of the duodenum is inmh'ed in 85% of cases, Usually patients are asymptomatic, but the condition can preSCn! at any age , with 48% of cases presenting in adulthood. There is an association with other congenital abnormalities when the condition presents in chi ldhood - tracheooesophageal atrcsi:l, duodenal atresia, irnperfor:ne anus, Down 's syndrome.
  
  Crtrcfully examine the fi lm for g:lllstones or sma ll fe;)tu res of cocli3c disease (small bowel flocculation of contrast , featureless smooth small IUlllen/ folds, jeju nization of ileal loops and peristalsis) to suggest :lI101her diagnosis.
  
  Further management
  
  · cr \\ill confiml pancreatic tissue encircling the duodenum. • ERCP (endoscopic retrograde cholangiopanereatography) or I'vt RCP (magnetic resonance cholangiopancre3togr:lphy) shows a nomlally located main pancre3tic duct in the body 01· the pancreas, :lIld 3 small duct in the head or the pancreas encircling the duodenum.
  
  Further reading Rizzo RJ, Szucs RA, Turner MA ( 1995 ). Congenital abnormalities of the pancreas and biliary tree in adults. l{nnioGmplJiu 15( 1 ): 49-68.
  
  ANSWER 45 Observations (45a)
  
  Discussio n
  
  Single image from a percutaneous chol3ngiogram is shol\1l. The percutaneous needle is seen with the tip in a proximal intrahepatic bili3ry duct. There is clcar abnormalit), of the commo n d uct, which has several strictures with duc t dilatation and bead ing. No filling defects arc seen to indic3te gallstones. Contrast is scen in the duodenum with no obstnlcting lesion scen at the lel'cI ofthc sphincter of Oddi.
  
  Prim3!,)' sclerosing cholangi tis is a progressil·e fibr·",... inflammatory condition 3fTecting both intrahepalic enrahepatic bile ducts. The condition is strongly as"""·,,,_ with inflammatory bowel dise3se (ulecratil·c colitis [l" round in 70%, Crohn's in 15%). Other assocbtions i'",""K re trope ritoncal and mediastinal fibrosis, chronic a hepatitis, Riedel's thyroiditis, p3ncrcaritis and Sjogn=fl s)'ndrome.frescntal"ion is Wilh progressive fatigue, ,,"". and jaundice . Biochemical changes :tn: round with clC"I SCrulll bilinrbin and alkaline phosphatase. • Im3ging feat\rres on cholangiogi.lphy (MRC P/ ERC P ): there arc mtrhifocal strictures aflccting intra- and extra hepatic bile ducts wilh s~ lesions. The classic p311em is or 3 'beaded' appe""... with alternating segments of ste nosis and dilat":ltia. ( 45b), • Im3ging features on CT: ducts have the 3ppcaran.x strictures, dilat;ltion, belding 3nd pruning. • Imaging on US: usually normal but may show d ua 1\';111 thickening,
  
  Diagnosis Primary sclerosing cholangitis ( PSC).
  
  Differential diagnosis ' For PSC: • Sclerosing cholangiocarcinoma. • Acute ascending cholangitis. • Bile duet carci noma - this G ill ran:I)' inl'oll'e the bili;u)' system in a difruse maImer producing multiple 1l1mour stric1l1res.
  
  82 •
  
  Abdominal Imaging
  
  Case 46
  
  of PSC include:
  
  ma nagement curative treatment for this condition is Ih'cr rion. Medical palliath'c care in\,oh'cs treatment toms of cirrhosis, parmi hypcrtension, chronic (pruritus and malabsorption ) and ductal ns such as strictures and ascending cholangitis. 'pHnary approach is therefore adopted requiring "'gists, transplant surgeons and inrcn'cntional 45b Single image from an ERCP examination showing multiple strictures with poststenotic dilatation gi ... ing a beaded appearance.
  
  - euoold male presented with ..n.aJ pain and vomiting.
  
  83
  
  Answer 46
  
  Abdominal Imaging
  
  ANSWER 46 Observations (46a ) This plain abdominal radiogr.lph shows a large, ahaustral gas~fillcd viscus arising fi-om the left side of the pelvis :llld extending into the upper abdolllen. The loop is projected OWf the left side of pelvis and descending colon with its apex under the left hcmidiaphragm. The medial walls of rhe loop form a sumrmuion line. There arc sc\'cral dilated loops of descending colol1 cvidem with absence of g.ts in the rectum. The features an: typical of sigmoid volvulus and there is no frc.c intflpcritoncal gas seen to indic,Hc pcrfor:uion.
  
  Diagnosis Sigmoid volvulus. Differential diagnosis For brgc bowel obstruction: • Colonic malignancy. Inflammatory strictures: Crohn's, ischaemia, divcnicujiris. Voll'lllus. Infectious processes; TB, amocbiasis. Extrinsic lesions: abscqs, bJadder/prostate/merine tul~lour, endometriosis.
  
  vake and call usually be differentiated from sigmoid volvulus bvsc\'eral teatures: Caecal ~'olvulus usually occurs in a younger agt~ group: 30- 50 years Dilated obstructed caecum often dilates to fill the left upper quadrant (although in many cases \'ertical rotation occurs with caecum still filling the right iliac fossa ). The main axis will be opposite that of sigmoid \'oh'ulus howe\'er, eXTending from the right iliac fossa towards the left upper quadranL Some haustral markings arc still el'ident, un!ike sigmoid \'okulus . • There mav well be small bowel dilatation bUT lhe rcst of the colon will be undilated, unlike sigmoid \'okulus,
  
  Further management Urgent surgical referral with a \'iew to insertion of a !latus tube to decompress the bowel. Further reading Burrel! HC, Baker OM, Wardrop P, EvansAJ ( 1994). Signiticant plain tilm findings in sigmoid \'olvulus. CiiJlicnl Rndiolo,9.1' 4 9 : 317- 319.
  
  Discussion Volvulus account for - 10% of Jarge bowel obstructions in the UK, the most common type being sigmoid volvulus This occurs more commonl)' in the elderly. The twisting of the sigmoid colon on its mesenteric axis is USU;\t!y a chronic problem with supcrimposed acute episodes, and represents a closed loop obstruction. Radiologically, the features arc of large bowel obstruction with a markedly dilared loop of colon seen arising from the left iliac fossa. The I'okulus is characterized by an ahaustral inverted V-shaped loop of colon. The media] walls produce a summ;uion line and together with the lines ofthc lateral walls create the classic 'coffee bean' appearance. Se\'cral radiological features have been doculllented as typical , though the most specific arc: • Apex of the loop under the left hemidiaphragm. Inferior convergence of the loop in the left side of the pelvis - the main axis of the loop therefore eXTends from left iliac fossa towards right upper quadrant. 'Left flank O\·erlap' sign - loop overlaps descending colon, Medial wall summation line. Other features described include 'li\'er ovcrlap' and 'pckic overlap' signs (where the loop overlaps li\'er and lefT iliac bone, respectively); apex ofloop above T! 0; an air to fluid ratio >2: 1. Diagnostic confusion can be resolved with a barium enema examination. This demonstrates a smooth, Tapered beak-like end of the barium column termed the 'bini's beak' sign. Treatment in\'olves the placing ofa rectal!latlls tube.
  
  Practical tips Caecal volvulus (46 b ) can someti mes be a confusing ditlerential. It represents twisting JUST above the ileocaecal
  
  84
  
  46b CaecaLvolvulus with a dilated caecum extend ing up into the right upper quadrant. Small bowel is dilated ' secondary to this obstruction but large bowel is collapsed helping to differentiate caecal from sigmoid volvulus.
  
  Abdominal Imaging
  
  Cases 47, 48
  
  E 47 ry ::;·'-car-old female presented with nal pain and vomiting.
  
  SE 48 ory 38-\'car-old woman underwent ruSt enhanced CT for further
  
  uarion of a lesion noted Ulld.
  
  page 88forcnsc answer)
  
  85
  
  Answer 47
  
  Abdominal Imaging
  
  ANSWER 47 Observations (47a) Multiple dilated loops of g3s-fillcd small bowel that measure morc than 3 em in diameter arc seen within the central abdomen. No gas is seen within the large bowel and appearances arc consistent with small bowel obstnlcrion. Surgical clips arc noted in the right side of pelvis along with
  
  choicc),stcClomy clips in the rig ht upper quad ram . Adhesions a TC therefore the most likely cause of the obstnlction.
  
  Di ag nosis Small bowel obstmction from adhesions. Differential diagnosis For small bowel obstruction; • Adl1(:sions - account for up • • • • •
  
  to 60% of s1l1:l11 bowel obstructions. Hcrni::a. Gallstone ileus. Small bowel or caecal malignancy.
  
  Intussusception. Malrotation and volvulus.
  
  Discussion Small bowel obstruction can have a varied prcsenr:uion on plain :abdominal f3diograph~'. The classical appeaf3nce is o f central abdominal small bowel loops dilated to :>3 em in diameter with :a paucity of gas in the large bowel. Other appe:af3nces C:all be of: • 'String of beads' sign due to small:air·fluid b 'c!s in fluid ·filled obstructed loops of small bowel (47b). • Absence of gas in the small bowel due to compleTe obstruction and complete fluid fillin g ofJoops (4 7e).
  
  47b Plain abdominal radiograph demonstrating the 'string of beads' sign.
  
  86
  
  Small bowd can be differemi:ated from large bowcJ using: the following fe:afUTes: • Presence of"al"ulae conni\'entes which extend :across the width of the bowel. Colonic haustf3 do not tran:rse the whole lumen. Dilated bowc! located in the centf31 abdomen f3ther than the peripher)' (47d ). Diameter of loops is <5 em. Absence of solid £leees.
  
  Practical tips Alw:ays check the film to tTy to identify the underlying: cause of the obstruction: Check herni:al orifices at the groin - there should Ix no bowcJ gas extending below the position of the inguin'llligamcnt (3 line from public tubercle to anterior·superior iliac spine ) (47e, 47f, 47g, 4 7 h ). Look for evidence of previous surgery, as in this ca~ Look for air in the biliary tree and radio·opaque gallstOnes outside the TerritOry of the gallbladder as indicators of gallstOne ileus. Examine bones for metastatic lesions, which can poim to malignancy. Alw:l.)'$ remember ro check for free gas secondary to perforation!
  
  Further managem ent CT is the inl'estigation of choice for small bowd obstruction and it can identify both the site and c:ause 011 obstmction and also the complications. Mcchanical sm bowel obstmetion is a surgical cmcrgency.
  
  47c Plain abdominal radiograph demonstrating complete absence of small bowel gas due to fluid filling.
  
  Abdominal Imaging
  
  47e Axial CT image shows that there has been a previous attempted hernia repair with a mesh noted in situ. There has, however. been a recurrence with dilated bowel going into the hernia and completely collapsed bowel emerging from it. This shows that this is the level of obstruction.
  
  479 Coronal CT reformatted image in the same patient demonstrates a right sided inguinal hernia.
  
  47h Axial CT image of a patient w ith small bowel obstruction.
  
  87
  
  Answer 48
  
  Abdominal Imaging
  
  ANSWER 48 Observations (48a) A l;ngc c(mral mass lesion is demonstraTed in th!.: left kidne y. This is slightly hctcrog..:ncous but hlS 3. prcdomi lllmly 6tty density. Appearances arc consistent with left renal angiomyolipoma.
  
  Diagnosis
  
  Appearance on CT: again, appeaT"J.nce is of a weI! ddined fat -collt:l.ining lesion with some areas of higher attenuation tissue. Identific ltion off.1t (He <-20) within a renal lesion is highly specific for an angiol1l)·olipoma. Appearances on ~·I RI: a En suppression sequence CaD be very liseful in confirming intralesional fu t conrenl
  
  Angiomyolipom3.
  
  Discussion Angiom}"olipoma is a benign lesion containing fur, blood \'csscls and smooth muscle. They Tend to present in two groups of patients' • Women in their 4th-7th decades where lesions arise spontaneously and tend to be iolitary allri I/J/I'/nfcml. Young paricms wirh TUberous sclerosis II'here lIIultiple nlld bilateral lesions an: seen in up to 75%of patients (48b), They arc also seen rarely in autosomal dominant polycystic kidney disease (ADPKD) and neurofibromatosis. • Appearance 011 US: typical appearance is of a we!! ddined edlObright lesion due to a high fin content (48c). There 'can be a variable degree ofredm:ed echogenicity depending on the ,unOUlH of smooth muscle and/or haemorrhage
  
  88
  
  The main complication of these lesions is haemorrhage the risk is rdated to size. Lesions greater than 4 cm diameter ha'·e a risk of spontaneous bleeding approximately 50%.
  
  Practical tips hkntification of f.Jt in a renal lesion is very specific angiom),olipoma.
  
  Further management Smail lesions « 4 cm ) arc usually asymptomatic but I >4 cm arc almost always sympromatic with pain and .. of haemorrhage. Lesion resection or nephrectomy s be consid<:Ted in these patients. Transcatheter art embolization is;ll1alrernarive.
  
  Abdominal Imaging
  
  Case 49
  
  89
  
  Answer 49
  
  Abdominal Imaging
  
  ANSWER 49 Observations (49a, 4gb) The twO CT images (493. ) demonstrate a large, well defined soft tissue mass in the central abdomen. This displaces 3djaccm bowcl loops and most likely originates in the mescntery. The si ngle imlgc (49b) from a double contrast barium enema examination shows multiple small , well defined mucosal filli ng defecTS througho UT the colon consistent with widespread colonic po lyposis. Multiple polyps throughout the colo n suggest an underlyi ng genetic condition.
  
  Diagnosis Familial adenomalOus po lyposis ( FAP) wi t h mesenteric desrnoid tumour.
  
  Discu ssion Familial adcnomatous polyposis is an autosomal dominant disease (chromosome 5 ) characte rized by multiple colonic adenol1latous polyps that inc\'it3bly progress to colorectal cance r within 20 years of dia gnosis. Treatment invoh'es prophylactic 1000al coleCTomy in carl)' ad ult life and genetic screening of r."lIllily memocrs from the second decade with a \'iew to prophylactic surgery. All patients ha\'e colonic polyps but small bowel and gastric adenomas arc also found (pcriampullaty cancer is the nltolsr COllllUOtl caliSC o(llcath o nce..col«tOftlY has-bccl1..pcrfunncd ). Othe r associated features include: Desmoid tUlllours. Mesenteric fib rosis. Gastric harnano mas. H ypen rophy of rctinal pigment epithelium. Gardner and Tun:ol.$),ndrollles arc \'Miants of-fAP . Gardncr syndrome also includes: O steomas of the skull and m ~l!ldibk. Dental abnormalities - dentigerous cySts, odontoma, hyperce mentollla, supernumerary tee t h. Soft tissue tlImours stich as fibroma, lipoma, leiom)'oma, neurofibroma. • Epidermal cysts. • Association with lh)'roid canccr.
  
  49b Multiple polyps.
  
  90
  
  TurcOt syndromc is FAP with assocbted CNS malignan..-, stich as medulloblastoma. Other polyposis conditio ns arc : • Peutz-Jeghers - autosomal dominant (AD ) hamartomatous polyposis condition with features of mucQeulaneous pigmentation (usually brown pigmented freckling on the mucous membranes oflips and g ums) and multiple polyps found prl!dominamly in the stomach and small bowel, \\ith lew also seen in rhe large bowel. Hamartomas ha\"e malignant potential but the condition is associated with all increased risk of lip per Gl tract malignancies.. Complieal'ions of the cOll(lition include: • o\hbbsorprion. • Transient intussusception • Carcinoma of the G I tract. • Carcinoma of breast, pancreas, ovary, endome and Testes.
  
  Cowden's s\'ndromc - AD condition char.lctcriud multiple ha;113rtOm:u ous polyps, breast and thyroid malignancy and skin lesions. Cronkhite-Carmda syndrome - hamanomatous colonic
  
  pol~'Ps
  
  arc a.ssoci:ltcd with alo(>l!cia., skin
  
  piglllcOI'3.tion and nail atrophy.
  
  Practical t ips • Images should oc carefull y inspected for a coexist colo nic tumour as well as cxtr:acolonic malignan . Intussusceprion in adu lts indicates an underhing. bowel pathology, whereas in children if can be idiopathic.
  
  Furth er management Surgical refe rral is required for prophylactic cokcr is referr.tl for genetic screening of rclati\·es.
  
  Further reading Galiatsatos P, Foulkes WD (2 006 ). Familial adcnOmatOllS polyposis. AmeriCfm jOllrnnl of
  
  Gnstrotllurology 101 (2): 385-398.
  
  Abdominal Imaging
  
  Cases 50, 51
  
  91
  
  Answe rs 50, 51
  
  Abdominal Imaging
  
  ANSWER 50 Observations (50a)
  
  Practical tips
  
  This single TI weighted coronal image shows a striking reduction in signal intensity throughout the H"cr parenchyma. This is likely 10 indicate iron overload. II is notable that the spkcn re tains normal signal intensity so the li\'(:r abnonm.li[~' is most likely due to hacmochromatosis.
  
  l\'l ultiplc transfusions for chronic haematological disordCG C:lnlead to iron overload, i.e. transfusion siderosis. j\IRJ wi ll show hypointensity in the liver find spleen in t condition. This helps diflerenti:lle it from haemo. eh romatosis where the spleen shows normal signal M IU.
  
  Diagnosis
  
  Further management
  
  i-Iacmochromarosis.
  
  In thc course offollow-up, ultl'J.sound monitoring may useful due to [he high risk of dC\'cloping cirrhosis hepatocellularcarcin0111a.
  
  Discussion Pri mary hacmochrOlTl3[Qsis is an aurosomal recessive
  
  condition characterized by increased absorption and ckposition of iro n within several organs illCluding [iva, pancreas, heart and pituitary gland. Patients arc usuall)' lsymptom:ttic until the 2nd dCC3dc, then they present with a varied clinical picture due [0 iron deposition in: • Skin - h)'PCrpiglllcllt:ttion. • U\'er - cirrhosis. lu:p:uomegaly. l)allcrc3S - diabetes. Heart - arrhythmias. dilated cardiomyopathy. Ml1sculoskclet31 - arthralgi:t. Pituira.ry - piruir-J.ry r.,ildrc with signs of impotence, tesf~eular atrophr. hair loss. Radiologically, imaging of the abdomen shows marked abllormality oCthe livcr. Accumulation of iron rcsults in the liver being of ditl'usel)' reduced signal on ;vIRl. The degree of iron deposition has been shown to correlate with the l\oIlU appearances. Uncnh:lIlced CT of the li\'cr demonstrates increascd :lUenuation (>75 HU). Follow-up in these patients is important due to the hepatic complication" of cirrhosis :tnd the ;nc.rc;eed risk. Oh hepatocellular carcinoma. Art.hropatAy of haemochromatosis is similar to that of calcium pyrophosphate deposition disease. Chondr~ calcinosis is a tcamre. T}VicaJl)', appearances arc of squaring of the mctacarpal he.lds due TO flatTening and peripheral small, hook-like SpUTS ( SOb ). Osteopenia is also common.
  
  SOb Radiograph of both hands shows fl atteni ng of the 2nd and 3rd metacarpal heads w ith loss of joint space, giving a squared appea rance. Early spur formation is seen on the lateral aspect of th e 2nd metacarpal of left hand.
  
  ANSWER 51 Observations (51 a, 51b)
  
  Discussion
  
  1111:1ges arc control and delayed prone abdo minal radiogra phs fro111 an [VU series. The control fi lm shows mediall y located appearance ofbolh kidneys and although the superior poles of both arc identifiable, inft:rior poles are not. The delayed IVU image (S ib) shows Illedial location of the pell'icalyeeal systems, which are anteriorly orientated. No filling defects arc identified.
  
  This is the most COlllmon fusion abnormality of kidneys. It is 1110re cOIll111only found in male patients has an incidence of I in 300. The kidneys arc joined tht:ir lower poles in 90% of Ca$CS, by a parcnehymal/fi isthmus band ( S I c). The long axis of the k i dn~ medially oricntated with anterior rotation, such th.;n renal pclvisesi are anteriorly located. Thc condiri complicated by urinar), stasis with renal stone fornua. infection and reflux . Vesieoureteric reflux hydronephrosis sd:ondary to ureteropelvic jlln obstruction arc common. There is :I reported incrc~
  
  Di agnosis H orseshoe kidney,
  
  92
  
  Abdominal Imaging
  
  Case 52
  
  enec of rCIl::ai adenocarcinoma, transitional cell m~
  
  and Wilms' rumour. In :I.ddition the kidnc" is
  
  cptib1c to injury following abdominal traUffi3. oc kidney is associated with: ourin:u)' ab'normaJitics: hypospadia, hidism, urctcr,ll duplication, bicornuate 'Jscular abnormalities .
  
  . al tips .an IVU, check for filling defeCTs, which could nt
  
  a retl;11 calculus or transitional cell
  
  51c Axial CT image demonstrates the horseshoe kidney with a narrow isthmus of tissue extending anterior to the aorta and IVC.
  
  -
  
  ~·old
  
  male willI weight loss.
  
  93
  
  Answer 52
  
  Abdominal Imaging
  
  ANSWER 52 Observations (S2a)
  
  Practical tips
  
  Single AI' radiograph of the abdomen shows a metallic
  
  • As with all radiographs that show e\'ide nce of likely primal)' malignancy. o nce this has been noted, look carefull y for metastatic disease elsewhere on the film , • Classicall~', coloreetal cancers metastasize to the li\'er due [0 the ,"enous drainage of bowel l"ia the portal \'enous system. Howe\,er, the \'enOliS drainage of therectum interfuces with the systemic ve nous drainage- . the 3nal canal and thus pulmona ry metastases are said 10 be more likely in rectal cancer than other colonic tumours. In reality, pullnonal)' metastases arc nOt :m uncommon finding in colon or rectal cancer.
  
  stem within the (cmtal pel\'is, which presumably lies inlhc rectum or distal sigmoid colon. [t is likely thaI Ihis stent has been inserted to rdicve symptolllS from a (olarectal
  
  1'lI1l1our. The rest of the bowd gas pattern is unremarkable wi t h no ("vidence of obslnlction. There arc, however, amorpholls, poorly marginated areas of calcification seen in the region of the liver and thc.:sc likely rcpn:scm calcified liver Illct:mascs. Ultrasound or cr
  
  should be undcrt3kcn, and a contrast cnl13nccd CT (52b) of this patient docs confirm the presence of calcified liver
  
  Further management Diagnosis Stcntcd rcct:l[ tumour with calcified liver mctast:tscs.
  
  Differential diagnosis For calcified [h'cr mctast:J.scs: Mucinous adenocarcinomas - colon, rectum , ovarian, brc:l5[ and s[Omach. Osteosarcoma. Endocrine pancreatic carcinoma. Med ullal)' carcinoma ofthr ro id . LUllgcancer.
  
  Discussion Colorcct:ll carcinoma is the third most COlllmon cancer diagnosed in the de"eloped world. Rectum and sigmoid arc the most common sites of1csio ns. When' surgical resection is not possible or appropriate, stems can provide symptomatic relief and prel'ent obstruction. The liver is the most common site for metastatic spread after regional lymph nodes,
  
  T Ni\'1 ( tumour- node- metas tases) staging lllust be accomplished as lor most tumours. Tumour staging rectal cancer is done with MR.I (52c ) (along with loc.ll nod al staging). The primary reason for MRl is ro as proximity of nUllour to the llle$Oreetal fuscia - this is thr. plane along which the surgeon dissects in a [ot"J.imcsorc excision (TME ) procedure. This boundary is rhus referm. 10 as the eireumfercmial resection margin (CRM ), Ifloc.. tumour spread extends close to it, the surgical margin Il\.Io well be contaminated with tumour with the attendant . oflocal recurrence. ldentif)'ing patients where the CR.:\! threatened in this way means they can be selected preoperati\'e radiotherapy or chemotherapy to reduce r risk. Distal nodal disease and metastases can be ass!: with CT or MRI but if the chest is also to be imaged exclude pulmonary metastases, CT is required for 1 component at least.
  
  52c Thin se~tion T2 weighted image shows a circumferential rectallumour with wall breach at the left anterolateral wall (12-2 o'clock) consistent with this being a T3 tumour.
  
  94
  
  Abdominal Imaging
  
  Case 53
  
  SE 53 c ry 35-year-old fe male presented \\~th inal pain and per rectal bleeding .3 months.
  
  95
  
  Answer 53
  
  Abdominal Imaging
  
  ANSWER 53 Observations (53a, 53b) Plain abdominal film of adult patielll shows marked wall
  
  thickening of the transverse colon wi th thlllllb-printing. The sigmoid loops show no such abnormalities and the rc(mlll contains fucccs. The double contr:lSt b:lIium enema film confirms extensive mucosal ulccrmion and a somewhat cobblestone "pp·caranee rhat extends from caecum 10 the descending colon. The colon distal to this is 1l0fllllL The appearances :Ire in keeping with colitis, and sparing the
  
  or
  
  more distal colon makes Crohn's disease morc likely than ulcerative colilis. It is not:lblc that the sacroiliac joints arc normal.
  
  Diagnosis Crohn'sdiscasc.
  
  Differential diagnosis For [emlina] ileal disease: • TB - uSlIllly has morc severe il\\'olvement of the caecum ( 53c). Thnc is oHen evidence of pulmonary TB. Radiation ileitis. Ycrsinia .
  
  53c T8 can also affect the bowel and appearances can mimic those of Crohn's disease. Caecal involvement w ith features of strictu ring and ulceration is more common than terminal ileal involvement.
  
  96
  
  For thumb·printing: • Inflammatol)' colitis - Crohn's, uJccl""Jtive colitis ( UC). Ischaemiccolitis. Infectious colitis/pseudomembranous colitis Divcrticulitis. Othcr causcs: cndometriosis, am)'loidosis, hereditary angioncurotic ocdcma, lymphoma.
  
  Discussion Crohn's disease is a chronic, inflammatory, granulomatOUl disease that can aflcct any part of thc bowel trona oesophagus to recrum. Small bowel is most common~ im·olved and the tcrminal ileum is iJwolvcd in ovcr 95% cases (53£1 ). I'rcscnt:ltion is usually in the 2nd-4th decldo with s)'ml)\ollls uf abdominal pain, diarrhoea, pcr ren bleeding, weight loss and fearurcs ofmabbsorption Radiological fcatures arc: • Aphthous ulccrs - shallow ulccrs with surrounding oedema. Fissures, sinu~s and fistulae - Crohn 's is the third most (0111111011 cause offisrulae aftcr idiopathic causa and di\·crticulitis. Thc tistulae can be bctwcen loops
  
  Ner 53
  
  Abdominal Imaging
  
  I, between inflamed bowel loops and otntcr nal "iscera, c.g. colo\'cskal or from bowel to tone mucosa - longirudinal and transverse
  
  Sl!'par.ttcd by ocdematous mucosa, ning of small bowcllolds. lion of small bowclloops due
  
  resection for C rohn's disease. There is noll' recurrence of
  
  disease in the ncotcrminal ileum with features of: Thickening of the bowel wall. M3rkcd stranding of the surrounding (,1[ due to inflammation.
  
  Engorged and dilated mesenteric \'csscls rclerrcd to as to
  
  inflammation
  
  or:dcmaofwalL gr:lIlubrity with < I 111m rounded mucosal
  
  'yps - inflammatory or h ~verpl astic mucosa. - often multiple. Jc:sjons willI discontinuous disease 3TC seen in
  
  of eases. g fea tures (53e ) are dcmollstr:ltcd in this case of _ male patie nt who has had a pre\'ious Terminal ileal
  
  the 'comb sign' due 10 the similarity in appear:lllce to the teeth of a comb. Skip lesions with tWO im·oked segments shown on this single axial image. MR imaging (small bowel emerograph}') fearures arc demonstrated in Figures 53f- 53i . The fat suppressed coronal/axial images ( 53f, 53g) show bowd wal l lhickening in the proximal ileu m.
  
  (eOlu. )
  
  C- image shows recurrence of disease in the ina I ileum post surgical resection. There is bowel wa ll thickening and oedema with alery change in the surroundi ng tissues.
  
  53f (coronal), 53g (axial) fat suppressed images showing bowel wall thickening of a loop of proximal ileum. Dynamic images are obtained and viewed in cine mode to see how this focus of bowel contracts.
  
  97
  
  Answer 53
  
  Abdominal Imaging
  
  The pre - :md post-contrast TI weighted corOlll! images (S3h, 53i) show cnhancmlcnt of an involvc.d loop of small bowel in the central lower abdomen. Treatment is both medical and surgical, with a high rate of fCCllTrcncc cI'cn alter resection (almost 40%), p;'1rticubrly in the ncotCfminal ileum following distal ileal resections
  
  Practical tips Terminal ileal involvement, skip lesions and multiple strictures arc the best signs for Crohn's disease. When assessing colonic disease, rememocr that UC aimosl always involves the rectum and has a continuou' distribmion withom skip lesions. Ho\\"cl·er, if a UC patient has had steroid enemas, the rectum may look nonmtl. Always look for signs of complications of Crohn's, i.e ade nocarcinoma ( risk increased up to 20-lold ), lymphoma, toxic meg:lcolon, pertoration, abscess, fistulae. Always look for signs of extrailltestinal manifestations
  
  53h (pre-contrast), 53i (post-contrast) coronal images of the colon showing enhancement in the thickened small bowel loops in the lower abdomen.
  
  98
  
  of disease all the film. Check lor sacroiliac joint disease, gallstones, hypcnrophic osteoarthropathy. Always look for signs of drug rfcatmcnr of disease on the film. Check femoral heads lor evidence of ;t,,:tscuhr necrosis from steroid trcatmclH. Further management Initial diagnosis in suspecTed cases is often confirmed with a Slllall oo\\'d barium study (iollow through Of emerod)'sis). Sometimes a more aCUTe presentation with abdominal pains mal' lcad to the diagnosis fim being suggested by CT. Capsule cndosco,,'~' is a !1 !:'1 illl"estigationllm Illay also first identify disease. OIKt" the diagnosis is made, follow-up imaging with MRI idea}.as this iucurs no rndiation risk oin what afC trequcntly young patients. CT imaging remains equally useful, howel·cr, when complications stich A abscess fonll~lrion arc suspccll:d. Trealllll:m includes medical and surgical discipli nes..
  
  Abdominal Imaging
  
  Cases 54, 55
  
  SE 55 Dry '::9-year-old male presented with ivc d}'sphagia.
  
  99
  
  Answers 54, 55
  
  Abdominal Imaging
  
  ANSWER 54 Observations (541 Single image from a double conrr3st barium meal examination shows multiple, small dense foci ofconlr:J.$t wit hin the aIHrulll and bod\' of the slOmach. These are surrounded by a lucent halo' rcprc.sc m ing oedema. There
  
  is some irregular thickening of the gastric folds, with thc targe t lesions appearing to be orientated along these.
  
  Diag nosis Erosive gastritis.
  
  Different ial diagnosis Fo r aphthous ulccr:Hion: Erosil'C gastritis. • C rohn 's disc;lSC. • Ihriurnprccipiralcancf.ICts .
  
  For gastric fo ld thickening: Erosil'c S;lstritis. Zollinger- Ellison syndrome. Crohn'sdisease. Malign:lIlc), - lymphoma, carcinoma. Benign rcani,'e lymphoid hyperplasia, Mcncuier'sdiseasc, •
  
  Discu ssio n Gastritis often has this apht hoid appearance ,,;da ":lriolirorm ulcers, consisting or a tin)' dense focus barium surrounded by a radiolucent halo or oedema gi,~ a ;rnrget lesion' appearance . Lesions arc usually multiple. The lntrum is preferentially affected with spread towarQ. the fundus, lesions appearing (Q be lon gitudin oriemated along the rugal rolds, Causes: In 50% or cases, no caus.ati,'e abnormality is identifi Peptic d iscasc, Dmgs - aspirin, NS AIDs, steroids, Alcohol. Infenion - herpes simplex, cytomcgalO\'irus (CM\' . C(mdidn, • Crohn's disease - lIslIall)' there arc signs ofCrohn's disease in other locations, most commonly the term inal ileum .
  
  Pra cti ca l tips On the contraSt examination look fo r features in oesophagus of an infecti,'c cause for the gamic appca
  
  o r for oesophageal varices pointing undcrlringcause.
  
  TO
  
  alcohol as
  
  Further management Gastroemerology referral with a ,'iew (Q endoscopy.
  
  ANSWER 55 Obse rvations (55) Single image trom a barium swallow examination shows a lesion in the lower oesophagus, just superior to the gastroocsophageal junction, The lesion is well defined with ;1 smooth edge, indenting lhe oesophageal IUlllen, No ulceration Of infiltration is seen. Ap pearances suggest a benign intramural mass, most likely a leiomyoma.
  
  Diagnosis Lciomroma orthe oesophagus.
  
  Differential diagnosis For smomh ocsophagel1 mass lesion: Neurofibroma. Li poma. • Hacmatoma, e.g. from instmmemation. • Duplication q'5t - can simulate an intramur::J.I mass,
  
  presentation is with dysphagia, odynophagia and pos.. haematelllesis. Radiological features are: Well defined, large, smooth intramural mass extending into the oesophageallulllcn. Forms an obtuse angle wilh lhe adjacent mucosagood sign ofa benign lesion. Usuall), found in the mid and lowef oesophagus, Calcification is sometimes seen and is vi rruall v diagnostic since it is the only oesophageal nll~our ealcif)'. Ukerationisrarc. May be multiple in -3%. Uniform contraSt enhancement on CT,
  
  Practica l tips Smooth, wdl defined, slow growing oesophageal afC likely 10 be benign.
  
  Di scussio n This is dIe mOst com mon benign tumour of the oesophagus. It is usually fou nd in young adul ts, being slightl y more common in males. Growth is slow and
  
  100
  
  Further management Gastroenterology refe rral \\;th a I';ew to direct ,;su with endoscopy.
  
  Abdominal Imaging
  
  Case 56
  
  101
  
  Answer 56
  
  Abdominal Imaging
  
  ANSWER 56 Observatio ns 156a, 56b)
  
  The All postmicturition imlge (56b) from 3n rvu series shows billteral dilat3tion of the disra.1 ureter with 3 ·cobra. held' lppelrlnce. There is a surrounding thick I1llo of lucency within the bbdder, represcnting oedema. These 3ppeara.nces are of bilatera.l ureterocelcs. The control film (56a ) demonsnates bilateral calculi in the peh'is that lie within these ureteroceles.
  
  Diagnosis Bilatcra.l ureterocdes.
  
  Differential diagnosis I:or radiolucent bladder filling defects on lVU: • Ureterocele. Hiaddertllmour. • Radiolucent calcu lus.
  
  • Sloughed renal plpilla. • Gas secondary to fistula, cystitis, idiopathic causes and trauml. • Island prostate - enl3rged central zone Cln appear as ~ ccntra.l bladder lucency.
  
  Discussion A simple or orthoropic ureterocele is a congenira.1 prolapse of the dist;!l ureter lnd its orifice into the bbddcr. It usu311 y an incidental ti nding in adu\[s and is bilatera.l in third of C3ses. Figure 56c shows how a ureterocele c produce l less spedfk type of filling defect when t.b.:. bladder is full and Figure 56d shows the typical ultra.sound. 3ppelra.nce. The main complication is of obstructi which can cause collecting system dibtatian and rc failure. There is also an increased risk of stone forma ' and it is therefore imperative to check the control film e,"ery patient-as in this case!
  
  56d US image of the bladder s h ows protrusion of the distal urete r into the bladd er, indicating that these a re orthotopic urete roceles and no t pseudoureteroceles.
  
  102
  
  Abdominal Imaging
  
  Case 57
  
  urcrcrocclcs can haxc a ·similar appearance and d bv obslrucl;on of:l normal ufetef. Dificrcllbcn\:ccn the tWO types can be made using or oblique films, which show no prmnlsion of into the bladder lumen with pscudourc[crocelcs.
  
  of pscudourcfcrocclcs include: .Jana ahhe distal ureter secondary to impacted • infection, radiothcrap~' . r tumour (56c:) ,
  
  to
  
  check the comrol film of an IVU.
  
  management I asymptomatic ureterocele Ill;l}' not require rec urrent urinary tract infcnion ( UTI ), calculi, obsrruct;vc uropathy arc indications for surgical
  
  56e Single axial T2 weighted image of the pelvis shows a pseudoureterocele secondary to a large bladder tumour.
  
  103
  
  Answer 57
  
  Abdominal Imaging
  
  ANSWER 57 Observations 157a) This frorHa] chest radiograph of an adult patient shows an added com'ex soft tissue density along the right mediastinal border and behind the heart. There is no normal gastric :ti l' bubble beneath the left hcmidiapllf:l.grn. The findings suggest d ilatation of the ocsoph:agus secondary to chronic distal ohstrunion, most likely due to achalasia. A barium swallow would confirm.
  
  Diagnosis Achalasia.
  
  Differential diagnosis Secondary achalasia due to a stricture at the gastrooesophageal junction, There will be normal pcristalsis however. C hagas' disease is esselltially the same as achalasia but the neurenteric plexus damage is due 10 Trypmlosomfl cYII::.iinfcction.
  
  Discussion Achalasia is a lllmilily disorde r that is idiopathic in aetiology, characterized br degeneration of Auerbach's plexus. This results in a fai lure of relaxation of the caudal oesoph:lgus at the g:lstro-oesophageal sphincter.
  
  Presentation is with progressivc dysphagia. investigat:ioe. is with a contrast swallow examination (57b), which mat" show featufes of: 'Vigorous achalasia' - multiplc tertiary contractions in the distal oesophagus Gill be the earliest sign. 'Bird's beak' deformity - symmetrical sLenotic segment of oesophagus at the gastro-ocsophageal junction. Imaging the patient erect allows best demonstration with contraSt forcing its way through the gastro-oesophageal sphincter when the hydrostatic pressure of the barium column in the oesophagus is sufiiciemly high . l'v lcga-oesophagus - dilatation of the oesophagus ttui call involve its emire length. Relaxation ofthe lo\\"er oesophageal sphinCler can be induced with amyl niTraTe inhalation. Achabsia is complicated by an increased risk of devcl~ an oesophageal squamous cell carcinoma.
  
  Practical tips Check lungs for pulmonary changes of previous aspiration. Check for evidence of pulmonary metastases from secondary oesophageal malignancy (or indeed if the di!:ncd oesophagus is due to a primary malignancy rather than achalasia).
  
  Further management • The condition is fi.lrther im'estigated with pressure measurements (manomet!)') and endoscopy. Treatment is most commonly by pneumatic dilatalil: of the gastro-ocsophageal sphincter. Surgical mrotomy is considered where there is disease
  
  57b Two images from a barium swallow examination show a dilated oesophagus contai food debris, which 'narrows down to form the classical 'bird's bea'k' appearance at the gastrooesophageal junction.
  
  104
  
  Abdominal Imaging
  
  Case 58
  
  105
  
  Answer 58
  
  Abdominal Imaging
  
  ANSWER 58 Observations (58a, 58b, SSc) Three im;lgcs from an JVU se ries arc provided bur no control film (which would normally be assessed prior 10 interpretation of the post -contrast films ). [Illages show uni lateral increasingly dense and persistent ncphrogr:un on the right'. The right kidney shows uniform smooth reduction in size when compared to the lefr. There is delayed excretion of comrasr by the right kidney on the 10 minute fi lm ( 58e ). These appcar:lI1ccs suggest unibtcTll
  
  right renal :trtcry stenosis. The young age of the patient makes fibromuscuhr dysplasia more likely than atherosclerosis as the underlying pathology.
  
  Diagnosis Renal artery stenosis.
  
  Differential diagnosis For persistent dense ncphrogram: • UnilaTeral: Obstruction - acu te obstruction is the most common cause of this sign. Renal artery stenosis/ ischaemia. Renal vein thrombosis. Acutc bacterial pyelonephritis. Acute papillary necrosis.
  
  Bi lateral: H }'potension/shock. Aelile tubular necrosis. • Acute glomerulonephritis . • Causes of un ilatera I change involving both kidneys.
  
  Fibromuscular dysplasia ( 10-20%) - usually in the mid and distal renal arter)'; allccts young adults and .::hildrcn; more common in women; bilateral in twothirds. Less com mon causes include vascu litis, arterial dissection and thromboembolic disease. Hypertension in neurofibromatosis is a consequence 01 phaeochromocytoma and/ or renal artery stenosis, \\~th .11 smooth stenosis seen in proxim:al ren:al :arter)'. In this p:arielU, a renal angiogram was performed and showed a smooth n:arrow stenosis in the mid portion of rhe right rena! artery (58d ) and this would be in keeping with the underl ying diagnosis of fibromuscular dysplasia of the renal ;lrter'\'. The eonsequ~nee of renal artCr)' stenosis is systemic hypertension as a result of o\'er;lCtivit}, of the reninangiotensin system. Treatment is aimed :at medically controlling hypertension and with renal arter)' :angioplas~
  
  Practical tips If there is bilateral delayed persistent nephrogram with absent or dec reased excretion, then the patient to ensure that contrast anaph ylactic sh"ock has not occurred. Magnetic resonance or CT angjogr~phy is the prcli:rred ilwesrig;ltion tor this condition in the moc\ernera.
  
  t..... needs to be immediatclv checked
  
  Further management Attempted renal arter)' angiography and angioplasry arc
  
  usually
  
  ad,~scd.
  
  Discussion 'Illere arc two main causes of renal artery stenosis: • Atherosclerosis (80- 90%) - usually in the proximal 2 cm of the renal artery; afrects older population >50 years; more common in men; bibteral in one-third.
  
  S8d Single image from a renal angiogram showing a ste nosis in the mid portion of the right renal artery.
  
  106
  
  Abdominal Imaging
  
  Case 59
  
  107
  
  Answer 59
  
  Abdominal Imaging
  
  ANSWER 59 Observations (59a, 59b) Single image from a barium follow -through examination shows smooth thickelled small bowel folds. Selected axial
  
  images from an IV comrast enhanced CT scm of the abdomen again dcmonsrr.l1cs smooth thickening ofbowcl folds in dilated loops offluid-fillcd small bowel. In addition, there is thrombus seen in the mid superior mesenteric vcill.
  
  Diagnosis Small bowel ischaemia secondary to superior mesenteric vein (SMV) thrombosis.
  
  Differential diagnosis For smooth thickened folds' Haemorrhage. Ischaemia: Acute - embolus, Hcnoch-Schonlcin purpura
  
  ( HSP). • Chronic - vasculitis, thromboangiitis obliterans, radiother.lPY· Oedema H ypoproteinaemia~ cirrhosis, nephrotic syndrome, protein -losing enteropathy. • Angioneurotic oedem,\.
  
  Lymphatic obstruction ~ lymphoma, mesenn:ric fibrosis, intestinallymphangiecrasia.
  
  Discussion Small bowel/mesenteric ischaemia can present very acutdy with symptoms of acute abdominal pain, ,'omiling, diarrhoea and recral blecding. This is usually due ro arterial thrombus, dissection or acute ,"enous Obstruction. Chronic ischaemia usually due to chronic arterial thrombus has a
  
  more indolent symptomatology with longstandi ng grumbling abdominal cramps, postprandial pain, weight loss and malabsorption. Other causes of ischaemia include \"asculitis, bowel obstruction , radiotherapy and aCUle: abdominal inflammation, e.g. pancreatitis, appendicitis. Radiological features of acure small bowel ischaema include: Bowel \\'all thickening/oedema. Enhancement pattern of the bowel wall can be incre3sed or decreased (5ge). Stranding of the surrounding f.1L Pneuillatosis intestinal is, which is a late sign and indicati\'e of necrotic bowel. Free imra-abdominal gas due to perforation of necrosed bowel. Portal venous gas is seen in preillorbid patients. In chronic ischacmia, there call be additional complicati of strictures (which tend to be long, smooth a symmetrical), ulceration and atrophic valvulae connivenro. The splenic flexure and the proximal descending colon most cOlllmonly affected due to the transition fr superior to inferior mesenteric arterial supply at this poiIIL
  
  Practical tips Look careftilly for linear gas shadows within the bowel wall indicative ofinrramllfal gas. Portal vein gas is usually seen in the pcriphery of the liYcr as well as centrally, in contrast to biliary gas, which is usually only central. This is a prcmorbid sip in adults.
  
  Further management hll'cstigation with CT C:tll both diagnose and complications of ischaemia.
  
  59c Axial CT image of the abdomen shows dilated flu id-fi ll ed loops of non-enhancing ischaemic small bowel.
  
  108
  
  idcnti~'
  
  Abdominal Imaging
  
  Cases 60, 61
  
  SE 61 - tory 57 -year-old m:alc prcscillcd with 'Cr abdomi nal pain.
  
  109
  
  Answers 60, 61
  
  Abdominal Imaging
  
  ANSWER 60 Observations (60) Single oblique radiograph of the pelvis from an IVU shows a small, shrunken, spastic trabeculated bladder with multiple diverticula, with a superiorly pointed dome. This is the so· called 'pinc trce' appearance of a neurogenic bladder. Diagnosis Neurogenic bladder. Differential diagnosis For Slllall bladder: Infection - schistosoilliasis/TB. Iatrogenic - postsurgery/ radiotherapy. Neurogenic. Transitional cell carcinoma (TCC ) - awmilletric bladder contraction with thick wall and filling defects. Extrinsic compression - usually gi\'es a pear shaped bladder appearunee.
  
  Discussion Bladder innervation is by the parasympathetic nervC$ 52-54. Injury to these nerves causes denervation oftbc detrusor muscle, giving this appearance of a shrunken. heavily trabeculated bladder, with an irregular thicken wall and multiple diverticula. Causes include: Congenital anomalies - myelomeningocele, spina bifida. • Spin:!1 trauilla. • Diabetes mellitus. • Infection - syphilis, herpes. • .Spinal neoplasm Practical tips Inspect IVU films carefully to look for asp' abnormality, which can help to determine the underl~ cause of the bladder abnormality. Further management In the absence of an easily identifiable cause, lumbo spine MIU can be useful.
  
  ANSWER 61 Observations (61) Single image from a barium enema examination demonstrates a well defined, Sillooth, eccentric filling deft."(;t in the distal sigmoid colon. This appears to be extraluminal in origin and lies on the mesenteric aspect of the bowcl A similar lesion is seen in the pouch of Douglas. Multiple lesions centred in an intrJperitone3110carion suggest the diagnosis ofimraperitone31 metaS[3Ses. Diagnosis Colonic mctastascs. Discussion Tumours sprc~ld to the mesentcry via four rolltcs: • Direct invasion along thc mesenteric \'essels and fut scen with gastric, pancreatic, colonic and biliary cancers; 40% of patients with adenocarcinoma of the pancreas have tumour cxtending along the mcscntcric root at diagnosis Extension \~a mesenteric 1}'mph3tics - colonic, ovarian, breast, lung, c3rcinoid and melanoma cancers C3n spread to mesenteric lymph nodes, though lymphomJ is morc common. Haematogenolls spread - commonly from melanoma, hrCJS! :lild lung prim3ries, met3st'3ses invoke lhe anti mesenteric bordcr of the bowd via sm311 arteries. They can act as the lead point tor intussusception. Up to 7.5% ofmehnoma paticnts show st1l311 bowel involvcment
  
  110
  
  Intraperitoneal seeding - brc:lst, 0\'3rian, panctc:lOi: and gastric tumours arc the most common primaries responsible for intraperitone31 'drop' seeding. "Thot lesions involve the mesenteric borders. Appcaranas arc of a focal mass or ditfuse stellate appearance 011 CT, but on double contrast barium im3ging, Jppcarances arc of a foc31 extrinsic indentation of bowel. This involves the pouch of Dougl3s (50'\ commonly the superior/anterior border of the sigmoid. Thc distal ileum and mediJI border ofd:.: caecum arc also often invoked
  
  Practical tips The most common underlying calise lor malignant diseJse is ovarian cancer. Further management Clinical review of patient to tty and loc3lize a likely tumour. CT sC3nning will confirm peritone31 di~ Illay well reycal the primary tumour. Further reading Shcth S, Horton KNt, Garland [\·tR, Fishman EK _ l'vlcsenteric neopl3sms; CT appe3rances of priffiX"l and secondary tumors and diffcrcntial diagno~ RndioGmphics 23: 457-473.
  
  Abdominal Imaging
  
  Cases 62, 63
  
  SE 62 ory 46-year-old
  
  Icmalc ,
  
  recentl y
  
  ted to the U K from Africa, - med with symptoms of urinary ncy and urgency.
  
  E 63 ry - ::'-\~ar - old
  
  diabetic presented with
  
  and abdominal pain .
  
  111
  
  Answer 62
  
  Abdominal Imaging
  
  ANSWER 62 Observations (62a )
  
  Practical tips
  
  This single coned view of the pekis shows curvilinear wall calcificatio n of a relatively normal capaci ty bladder, No calcifiGlrion of the lower ureter.; is seen. No discontinuity in thc calcification is.scen. TIle history of residence in Africa raises the possibilities of bladder TB and schistOsomiasis. The absence of gross bladder contraction makes the laner more likcl}" but it would also be helpful to re,;ew a full length alxlominal film
  
  ~~n!:; n~~l:t L~j;:,e~t~~~;u~~l~~ficadon. Transitional cell ::r
  
  Schistosomiasis il1\'ol\'("s bbdder and lower ureters and results in a calcified , nonshrunken bladder. Tn involves rhe kidneys and spreads via the ureters ro involve rhe bl:ldder. It is very unusual to have isolated bladder involvcment with TB and the degree of bladder contraction is more marked than in schistosomi:lsis Discontinuity of calcitic at ion in the bladder wall should arouse suspicion of bladder cancer,
  
  Further manageme nt Diagnosis SchistOsomiasis.
  
  Follow·up in these patients is required since the latency fw development of sq uamous cel l carcinoma of the bladder can be lip to 30- 35 rears.
  
  Different ial diagnosis For calcified bladder wall: Cancer - prirnaril}' transitional cdl carcinoma (TCC) but also other rarer bladder tlImour.;. • Radiotherapy. • Inlection - Tn and schistosomiasis. D is.cussi o~ .. ,. .. SChlstOSOlmaSIS IS one of the mOSt common paraslilc infcctions, affecting 8% of the global populatio n. Schistosolllff l)(Illllntobilllll is the female parasitc which aflccts the genitou rinary system. Unlike TB , which tends to affect thc kidneys first and then spreads caudally, schistosomiasis has a re,'erse involvement and uSlIall}' is confined to bladder and lower ureters. (Another case of bladder schistOsomiasis [62b] is shown - note the absence of upper tract calci fication that one might sec in TB .) Classically, the patient presents with urinary frequen c)" urgency and dysuria. Imaging findings arc of: • Calcification of the bladder - which results in reduced bladder filling capacity and increased posnnicturition residual volume. Calcitlcatio n of the lower ureters. Lower ureteric strictures. Ureteritiscystica.
  
  Complications of disease aAccting the genito urinary system include : Cystitis. Vesicourelerie reflux and subseq uent pyelonephritis. • Increased risk of squamous cell carci noma of the bladder. Other systems can be affected: Li\'er - oval migration results in portal hypertension and subsequent oesophageal varices. Respiratory system - diffuse granulomatous lung lesions.
  
  11 2
  
  62b Calcification of the bladder is seen in schistosomiasis with absence of upper tract calcification to help differentiate from TB.
  
  Abdominal Imaging
  
  Case 64
  
  R 63 tions (63) biominal radiograph demonstrates gas within the ~.i lyccal system and upper ureter. No intraa1 renal gas is seen. No gas is seen in the right o r in the bladder.
  
  There is an increased incidence of these conditions in patients with diabetes mellitus and women arc three times more: cOlllmonly affected than me n . Esc/)ericbia coli is the causative organism in 70%of cascs with Klehsiell", Proteus, Candida and PseudomOllflsorganisms also being found. In emphysematous pyelonephritis, small gas bubbles arc initially seen on plain radiographs in\"ol\'ing lhe renal parenchyma; this progresses to give a di ffuse mottling in morc ad,'anced disease and then progresses to produce a crescent of perinephric gas when there is extension into the perirenal fill.
  
  Practical tips _ nie - urinary dive rsion procedures . .' tract fistula to bowel due to innalllillation, Crohn's, diverticulitis or spreading malignancy.
  
  CT is the best imaging modality for assessing extent and location of gas.
  
  Further management Depends on ca use but obviously infective causes require prompt, appropriate antibiotic treatment.
  
  mamU5 pyelitis is a condition in which infective produce gas, which is confined within the renal :cc,aI system. In emphysematous pyelonephritis, gas JII'l
  
  \\ithin the renal parenchyma - a lifc·thrcatcnirrg that requires prompt di:agnosis and tn:amlcnt.
  
  Further reading Joseph RC, Amendola MA, Artze ME, et al. (1996 ). Genitourinary tract gas: imaging evaluation. RadioGrapbies 16: 295-308.
  
  E 64 ;u·old female presented with and epigastric pain .
  
  lIUlCty
  
  11 3
  
  Answ er 64
  
  Abdominal Imaging
  
  ANSWER 64 Observations (64a) Single image from :t double contrast barium meal cx:ulIi natio n shows :t lI'ell defined smooth walled ovoid mass lesion in lhe gastric JllIfIlm. A central smooth ulcer is present and no calcification is seen. Diagnosis Leiomyoma of the stomlcn. Differential diagnosis Fortargc! lesions: Ncurofibrolll3. Lipoma (64b). Ectopic pancreatic rest. MCl'3stascs - cOlll mon ly breast, IUllg, renal and malignant melanoma. H acm3ngioma
  
  Di scussion T his is the second mOST common benign gastric tumour after gastric pol~'Ps. Ukc ocsophagcallciomyoma, these aTC slow growing lesions and arc usually asymptomatic umil
  
  they incn:asc in size, when there may be epigastric p:lin and. bleedi ng. The gastric antrum and pylorus arc l he m common si tes "flected. T he majority of these lesio extend im raluminally (60%) and form well defi ned 0\' defects. They arc more likely ('Q ulcerate than ocsophag leiomyoma with ulceration scen in up to 50%. Calcifica . is rare. Complications include: Bleeding. Obstmction. Intussusception - tumour can act as a lead point. Malignant degeneration - s~cn in up to 15-20%m cases.
  
  Practical tips Smooth, well defined, slow growing gastric lesions likely to be benign. Further management Although radiological appcarances suggest this to be benign lesion, referral for endoscop~' +/- biopsy should made.
  
  64b Axial CT imag e demonstrating a well defined, smooth , rounded lesion in the stomach, which has clearly the same attenuation as intra·/extra·abominal fa t. This has appearances of a gastric lipoma .
  
  11 4
  
  Abdominal Imaging
  
  Cases 65, 66
  
  IJ8jorcnsenllsUler)
  
  11 5
  
  Answer 65
  
  Abdominal Imaging
  
  ANSWER 65 Observations (65a)
  
  Thickc nin~
  
  Single image from a double contrast bariulll enema exami nation shows :abnormalitv of the colon tha t extends from the rectum [0 the mid rrans\'crsc colon. There aTe (C3IUTes ofJuminal narrowing with mucosal irregularity, granulari ty ;1 11(\ shallow ukcr:ltion. The disease process appears cOll tinuous along the 3.flcctcd segment's with no fil rthcr lesions seen. Noml:!l appearances of the ikocacc:L1 region. Normal sacroiliac joints. T hc appearanccs arc in keeping with a colitis, most likely ulccrntive colitis.
  
  Significant bowel wall thicke ning can 1c3d classical 'thumb-printing' appearance. Widening ar thc presacral space.
  
  Diagnosi s Ulcc:rnti,'c colitis ( UC).
  
  Di sc ussion Ulcerativc colitis is an idiopathic inf1ammator~' bowel disease \Iith invokemcnt predominamly of the mucosa and su bmucosa or thc l:trge bowel. There a rc tWO peaks of prcscntltion - 3rd-5th dccades and 7th- 8th dccldes. The most commo n presentation is with blood)' diarrhoel and abdominal plin. The rectum is almost always involved (96% of cases) with continuous, co~centrif and symr~lelri.c invo~\'~mcnt of the colon more proxlillally. The tcrmlllalrku1l1 IS 111I"01\'ed in 10- 25% due 10 backwash ileitis. In acute inflammation there arc findings of:
  
  65b Single axial CT image shows continuous thickening of the colonic wall involving sigmOid colon. The surrounding fat is 'dirty' !i ncreased attenuation) due to inflammatory change. Appearances are of an acute active colitis though the appearances here are not specific for an underlying ca use.
  
  11 6
  
  o f bowd
  
  W:I!l
  
  (65b). to
  
  tht:
  
  Fine ll1uc<>S.11 granularity. Superficial ulceration. I'scudopolyps - islands of oedematous mucosa. Collar button ulcers (65c). Appcal'Jnces in the chronic stage: • Colon becomes ri gid with llllllinainarrowing due [( chronic inilamm:ltion, and loss or haustrations 'le3clpipc' colon. Coorsc grnn ul:tr mucosa. Inflam1l13tory polyps. Ibck\\'ashilcitis.
  
  Abdominal Imaging
  
  n is complicated by: n from toxic mcg~lCo[on in 5- 10% - thc ..:ommon cause of death (65d). .ldCIlOCarcinQm3 - this complicates Lip to 5% ~ ticnts with risk highest when there is or onsct at a young age( < 15 years) and \\;th chronicity of disease. The rectosigmoid 'TIOSI common location for ncopbstic [lOtlJlion. strictures - usually a single , shon, smomh is found, most cOlllmonly in the sigmoid . • .lS
  
  with Crohn's disease, there arc a variety of
  
  ..u.: complications which include iritis, pyoderma ~ m,
  
  chronic acrivc he patitis, sclerosing and seronegative arrhritis (Figure 65c ;lIes s;lcl'oiliiris with carly sclerosis of both oints).
  
  I tips riJtion ofCrohn's from UC is often possible the imaging tindings: thn's characteristically has multiple, eccentric,
  
  transmur:ll , skip lesions; preferential in\'ol\'ement of terminal ileum. FiStulae and deep ulcers afe common feature s . UC characteristicall\' has a continuous, concentric, symmetric i1l\'o l\,eniclH that eXl'ends proximally from the rectum :lIld only occasionally ilwokes the te rminal ileum. Fistulae, fissures and deep ulceration arc nOt fcatures. Remember that although the recnUll is always involved in UC, it Illa}, appear spared if steroid enemas have been used. Look for intestinal complicJtions of UC including malignancy, toxic megacolon, pneumatosis intestinalis and perforation . Look for exrJ";lim estinal complicat ions of UC such as sacroiliitis. Look lor compl ications oftreaUllellt, e,g. steroids causing avascular Ilecrosis (AV N ) Of osteoporosis.
  
  Further management A combined medical/surgical approach to disease !lIanage· ment should be taken.
  
  117
  
  Answer 66
  
  Abdominal Imaging
  
  ANSWER 66 O bservations (66a) Single image from a double contrast barium examination is shown. There arc multiple slIb1l1LlCOS:l ] lesions seell
  
  scattered throughout the (olon with no regional predominance. Close inspection shows that these aTC due to gas-filled cysts in the bowel wall. No free intraabdominal gas is seell to suggesT perforation. No linear gas collections ;\rc seen. No portal vein gas is seell.
  
  Diagnosi s Pnc1I1l1arosis cysroi(ics intcstinalis. Discu ssion Pneumatosis cystoidcs imcsrillalis is usually a benign condiriori of middle aged people who tend to be asymptomatic but elll present with symptoms of vaguc abdominal pain, diarrhoea and lllUCOUS discharge. R.adiological findings arc of multi pic small \ - 5 mm gas· tilled cysts in a subserosal!sublllucosal distribution. They are more cOlllmonly found on the mescnteric rather than anti mesenteric sidc of the colon. The cysts can rupturc
  
  leading to pneumoperitoneum but with no symptorm peritonitis. This cystic pneumatosis is usually a bem_ innocuolls condition and is associated with ch obstructivc pulmonary disease, perhaps dl,le to air t from ruptured alvcoli and along the mescntery \"i.l retropcritoncum. There is also an association with m disruption elsewherc in . the G I tract, e.g. peptic disease
  
  Practical tips Air in tht: bowel wall due to infarction typically · more linear (66b) and may be associated with
  
  gas in pre morbid cases. Further management Pncumatosis of the colon is usuallv not - the importance here is to trcat the patielll,
  
  
  Further reading Pear BL ( \998 ). Pnnllllatosis intcstinalis:
  
  :l
  
  review.
  
  /{nnio/Qgy 207 : 13- 19.
  
  66b Abdominal radiograph shows linear gas opacity withi n the w all of the transverse coler a patient w ith ulcerative colitis.
  
  118
  
  Abdominal Imaging
  
  Cases 67, 68
  
  119
  
  Answers 67, 68
  
  Abdominal Imaging
  
  ANSWER 67 Observations (67) Selected 'in and ou[ ofphasc' T1 weighted axial images of the abdomen arc provided, the ou[ ofphasc image lying to the right. There is en largement of tile left adrenal gland which appears of intermediate signal imcllsiry all Tl in phase and shows signal 'drop out' 011 the TI Out of phase image. Appearances on this chemical shift MRl art: consistent with fatty content and indicative of a benign
  
  % washOllt ..
  
  early-delayed ) x 100 ( carly-ullcnhanced
  
  Washout >60% has sensitivity and specificity - 9m. the diagnosis of adenoma.
  
  adenoma.
  
  Diagnosis Adrenal adenoma. Discussion Adrcnal1csions arc found in - 9% ohhe general population. Adrenal ldenomas arc the most common adrenal lesiol1. Imag ing feamres can be used to ditTcrcntiatc benign adenomas from primary malignant (carcinoma, phaeochromocytoma) or metastatic adrenal lesions. Imaging features for differentiation of an adenoma from metastasis are: Adenomas are mostly <2.5 em in size; lesions >4 em in ~~r~i~;~r;~~re likely to be ~etastases or adrenal Lesions showing no growth over 6 months arc usually benign. On unenhanced adenomas often contain intracellular f.1t", lowering the overall density on CT. When <10 Hounsficld units (HU ), this is highly specific for adenoma (96% specificity). Some adenomas contain less fat and so a lesion with H U > I 0 may represent adenoma or malignant lesion. On IV contrast enhanced both adenomas and metaStases enhance, but adenomas washoUT more rapidly. After a plain scan, acquire postcomrast scans at 60 s (early ) and 10 min (ddayed ).
  
  cr -
  
  cr -
  
  On chemical shift M JU - signal drop Oul on the OIl[ of phase imaging is seen with adenomas. Lesions without significant intracellular fut (e.g. metastases don't show any signal difference on in/our of phase: imaging. The sensitivity and specificity arc similar if nOt bener than, those of cOntrast enhanced CT. PET may be useful when CT and M lU fail to characterize an adrenal mass. Malignant lesions s increased uptake of FDG ( 18 fhroro -2 -deoxyglue (>Ii,'er activity) while benign lesions do not (
  Practical tips An incidcmal enlarged adrenal picked up on US/ CT should be furthe r irwestigated with a chest radiograpit. look for a lung neoplasm. Further management Adrenal adenoma should be followed up after 6 m Lesions that grow by over I ern in this time or those measure over 4 ern should be considered for su Hormonally active lesions may also be considered surgery. Further reading Mayo-Smith WW, Boland GW, Nota RB , Lee MJ (2001 ). State-of-the-art adrenal imaging. RndioGrnplJics 2 1: 995- 1012
  
  ANSWER 68 Observations (68) Single image from an JVU study is provided without a control film. It shows renal ectopia with both kidneys being located on the right side. This is crossed fused renal ectopia. There is no associated hydronephrosis. Diagnosis Crossed fused renal ectopia. Discussion Crossed re nal ectopia involves a kidney being located on the opposite side ofthe midline from its ureteral orifice. It is more common for the left kidne}' to have migrated to the right, with the crossed kidney lying inferior to the normal kidney. Usually the kidneys are fused and arc associated with aberrant renal ancries.
  
  120
  
  It is associated with: Renal calculi. Infection Reflux. Megaureter. Cryptorchidism. Urethral valves. Multicystic dysplasia
  
  Practical tips Try to identify complications of the condition such as scarring from recurrent infections, or hydronephrosis to obstnrction from stones/urethral valves. Further management No active management is required.
  
  Abdominal Imaging
  
  Cases 69, 70
  
  fC1ll::J.1c prcscnlcd with Sfc::J.torrhoc::J. for 12 months.
  
  ur-old 1l1::J.Ic W::J.$ involvcd in : J. accident.
  
  121
  
  Answers 69, 70
  
  Abdominal Imaging
  
  ANSWER 69 Observations (69) Single image from a small bowel enema examination is shown. The tip of the small bowd enema catheter is seen in the proximal duodenum. The examination dcmonstfltcs a reversal of fold pattern with an increased Ilumbcroffolds seell ill The ileum and a reduction in the number of smalJ bowel folds seen in the proximal jejunum. These features suggest malabsorption in dlC proximal s111311 bowel such as coeliac disease.
  
  Diagnosis Cocli3cdiscasc.
  
  Discussion Coeliac disease is a gluten-sensitive clltt:fopathy charaClt::rized histologically by villous atrophy. The duodenum and jejunum arc afl:ectcd more than the ileum because some proximal gluten digestion means that less reaches the disul small bowel. It classically presents with steatorrhoea and diarrhoea, but more commonly presellts in less specific ways such as fatigue, weight loss and abdominal pain. Malabso rption can also n::sult in anaemia, osteomalacia, neuropathy and oedema. Age at presentation call vary from early childhood to late middle age. lmagiog features of coeliac disease include· Smlll bowd dilatation (due to reduced motility) is the most common imaging finding. Reduced number of folds in the jejunum. 'Jejunalization' of the ileum - an increased number of ileal folds. This is a response to the reduced mucosal arca in thcjcjullum.
  
  'Moulage' sign - dilated smalJ bowel loops with d&ccmcnt, particularly in the jejunum and duodenum. T ransicm intussusception - the cause of abdom' pains.
  
  Floccubtion of the barium contrast due
  
  to
  
  Autoantibodies (e.g. antiendom),sial ) but diagnosis is made by demonstration on jejunal biopsy. This abnormality remo\·ed from the diet and recurs when it is condition is complicated by an inerea!iCd .risk adenocarcinoma of the small bowel, carcinoma and pharyngeal carcinoma) (;1
  
  Practical tips Folds in the jejunum arc normally thicker and numerous than the ileum. Expect to sec seven fi per inch in the jejunum lnd thrce to tour folds I:G inch in the ileum. A reversal of this pattern shaul.. raise suspicion of coeliac disease. If the radiological features deteriorate while on J glutcn-frce diet, raise the possibility Ii such aslymphoml
  
  Further management Confirmation of diagnosis is made with bowel biopsy.
  
  ANSWER 70 Observations PO) Single image from an IVU series shows contrast in the collecting systcms and in the bladder. The bladde.r has an abnomlal elongated configuration eXTcnding up om ohhe pelvis. There arc bilateral pelvic fractures through the acetabulum. No free COl11rast is seen leaking from the bladder.
  
  Practical tips Look for an underlying cause lor the abnormal bladder lppearance - pclvic fractures, i calcification in the walls of aneurysms, i ch;\nges in bone/small bowel to suggest un malignancy and l)'mphadcnopathy.
  
  Further management Diagnosis Pe;lr shaped hlematoma.
  
  urinary
  
  bladder secondary
  
  to
  
  Differential diagnosis For pear shalX'd bladdcr ( mnemonic - 'HELP' ): • }-hematoma External iliac artery aneurysms. Lymphadenopathy. Pelvic lipomatosis.
  
  122
  
  pelvic
  
  Further treatment will depend on the underlying this is a secondary finding pointing towards an problem.
  
  Abdominal Imaging
  
  Case 71
  
  E 71
  
  '"
  
  rear-old ICl1lllc with 3.n incidental kgo n de teCTed on ul trJsound.
  
  71 a T2 weighted MRI scans with a normal and extended TE.
  
  11b T1 weighted scans, in and out of phase.
  
  123
  
  Answer 71
  
  Abdominal Imaging
  
  ANSWER 71 Observations (71a, 71b, 71c) T2 weighted im:ages demonstJ"3.te :a well defined, sligluly lobulated m:ass in the right lobe of liver, which is of increased signal o n dIe normal T2 and UI[r.I-T2 weighted seans (7 13). This is the so-c:llled 'light bulb' sign. Axi:ll TI weighted images (7 Ib ) show thc lesion to be of reduced sign:al wit h no signal change in the Jj\·er on out of ph:asc sc:allS to indicate that the lesion represents foca l t:-arty sparing. D}'namic post-contrast images (7 1c ) show peripheral nodular enhancement with centripetal filling in o\·er time. These appearances arc diagnostic of cavernous haemangioma. Diagnosis Cavcrnous haem:tngiom:l. Differentia l diagnosis For hn>erechoic hepatic lesions on US: • Haemangioma. Focal nodular hypcrpl:asia (FNH ). !\dcnollla. t\'\c raSt:isis. Hep:atocell ular carcinolna. Lipom:l. Discussion Hepatic haemangioma is the most common benign tumour of tile liver, anccting up to 20% oCthe population. The~' :arc usually :asymptom:atic, being incidental findings 011 imaging studies, however large lesions c:an present with :acure haemorrhage and abdominal pain; 90% arc solitary
  
  124
  
  and 90% measure less than 4 em in diameter. Mul hacm:lIlgiorna may be associated ,\;th OsIcr-Wcbcrsyndrome. Some enlargement can be seen du pregnancy. • Imaging findings on ultrasound: well defined, uniform, h~l>Crcchok lesion is the Iypical ulmsou 3ppcar:lI1cc (71 d ). No Doppler signal is seen widUl' the lesion due [Q the low-,"c!ocity flow (7 1c). La lesions (;lll appear hn>occhoic and show flow ho\\'c,·cr. Imaging findings all cr: lobubtcd lowattcl1uati lesion, which is frequently peripheral in location. Calcification is not common. Following comr.m injection, there is peripheral nodubr cnhancelllcm with centripetal tilling. The lesion lIsuallj' fills in completely o\'(:r 3-30 min to become isodcnsc \\; II\'cr, though larger lesions ma~' show persisting central noncnhanccmcnt/ scar and small lesions Il\lII show immediate unitoml enhancement. Inllging findings on ~IRJ: haem:lIlgiomas show ~ signal on T2 weighted images [hat persist on mort" heavily T2 weighted scans. This is called the 'light bulb' sign and is indicative of cyst or haemangior-. The enhancemelU pattern with gadolinium is as described lor CT. • Imaging t1nding~on sulphm colloid lItan: cold
  
  Usually imaging is sufliciclit to make thc di agnosi.) where :uypical features :afe present, biopsy CnI undertaken prO\·ided therc is normal livcr between lesion and li\·cr capsule to pre\·ent haemorrhage.
  
  71
  
  Abdominal Imaging
  
  tips the illvcstig;ltion archaicc 10 characterize :ted hlcm:mgioma. A positive '\ight bulb' sign cs that the lesion is either a cyst or a gioma. However, depending on the specific iraj "t'
  
  allccS,I)()SI-contraSI SGI1lS rnaysti11 be as necrotic or cystic neoplasms can yield a
  
  'light bulb' sign.
  
  )If TV CQnlr.IS( - obtlin pre -conTrast scans, then .n 30 s (arrcrial phase ), 60 s (ponal phase ) and min (equilibrium ph:lsc ). centripetal enhancement is intense and nodular, hi~hJy specific for the di:lgnosis of
  
  Case 72
  
  Fu rther management These arc essentially benign lesions and don't require any furrhcr follow-up.
  
  Further reading
  
  VihnO\'3 Je, Bl fccl6 J, Smimiotopoulos }G , ct fli. (2004 ). Hemangioma from head [0 toc: MR imaging WiTh pathologic correlation. RadioGraphies 24 :
  
  367- 385 .
  
  gl01113.
  
  125
  
  Answer 72
  
  Abdominal Imaging
  
  ANSWER 72 Obse rvations (72a, 72b, 72c, 72d)
  
  cr
  
  ~
  
  Several axial images of the abdomen with IV contraSt cnhancctm:nt in the portal venous phase arc shown. There is significant stranding of the pcrip:Ulcrc~ltic fat indicatil'e ofacutc inflammation, which is prcdominamly around the pancrc:Hic tail. \Virhill the pancreatic tail, there is a moderate sized fluid-filled C)'stic lesion, which would be consistent with a pancreatic pseudocyst in a patient with pancreatitis. The local inflammatory change appears to extend to involn! the distal transvcrsc colon at the splenic f1exurc. In addition, there is central low atlclU13tion within the panal ,'ein extending into the proximal splenic vein in \ ,.,' 'r' ~ keeping with I.'Ortaljsplcnic vein thrombosis. The 1 pancreatic duct IS d ilated al 4 mOl diameter. While the whole b"
  Diag nosis Pancreatitis.
  
  In chronic pancrc:uitis (following sc\'CrJl bouts of 3( episodes ), the pancreas becomes atrophic, there llluldtOcal calcificltion, the pancreatic duct becomes dilJt and chronic pscudocysts can form (7 2c ).
  
  Practical tips Look for the two main C:lUSCS ofpancre:ltitis - alco'" and gallstones. US is required to look for b"
  Disc ussion
  
  \\
  
  Pancreatitis is an aCllte inflatnmatorv condition. There arc tWO main types of pancreatitis - oe
  126
  
  Further management Treatment is usually conserva tive although necrori pancreatitis may well need more aggressive manage involving surgery or percutaneous r:rdiologically gu drainage.
  
  Abdominal Imaging
  
  Cases 73, 74
  
  M-old male presented with -J. and chronic rcnal fuilurc _
  
  127
  
  Answers 73, 74
  
  Abdominal Imaging
  
  ANSWER 73 Observations (73) Ultrasound longitudinal image of the right testicle demonstrates multiple sma!) hypcrcchoic foci, with no posttriar acoustic shadowing, disrribmed c\'cnl~' throughout the. testicle. The testicle is otherwise of normal size and shape. No other focal mass lesion is identified.
  
  Diagnosis Testicular microlithiasis.
  
  Di scussion T esticular microlithiasis is a (ondition characterized by failure of clearance of degenerated rubular epithelium from the scminiferous tubules, which subscqucmly calcif)'. This is an asymptomatic condition and is an uncommon incidental finding. [mage findings o n US arc of multi pic small \ - 2 nlm hypc:rcchok foci with no acoustic sh:ad owing, disuiblilcd throughout both teSles. Atypical appearances of asymmetrical and unilateral foci arc also seen. There is an increased risk of testicular gcrm cell tumour in 40% and therefore follow-up \\~th 6 monthly-US is oflen ath
  Associations include: Cryptorchidism. Infertility. Klinefc!ter's. Down 's syndrome. Male pseudohermaphrodism. Al"eolar microlithiasis.
  
  Practica l t ips Identifying the disrriburion of calcific foci is im since a cluster of calcification within a hypocchOl":: suggests nunour or chronic testicular infarction. I can be due to [Onion, trauma o r sc,'cre cpididymim..
  
  Further management Urological referral with fo llow-up testicular US monthly intervals to screen for malignancy.
  
  ANSWER 74 Obse rv ati ons (74) Single AI' abdol11in ~11 r:ldiograph delllonstrntes amorphous, putty-like calcification within a shrunken right k.idney.
  
  Diagnosis Reml tuberculosis with autonephrectomy.
  
  Uretcral TB is found in 50% of gcnitourinary TB Radiographic features of involvement include filling defccts, calcification and strictures. Bladdtt presents with a shrunken, scarred, calcified bladder thick.ened wall and/or filling defects. Clinical p is with frequency, urgency, dysuria, mic haemaruria a.nd 'srcrilc' pyuria.
  
  Discussion The urogenital tract is the second most common site to be affcrted by TB, after the lungs. Renal TB is found in 5-1O'X. of patients with pulmonary TB, bur there is only rndiographic e"idence of pulmonary TB in fewer than 50%. The kidne y is usually affected first, via haematogenolls spread from a lung/bone/GI focus. There can then be contiguous spread of inl~ction to im'ol\'e the ureters and bladder. Renal TB is unilateral in 75% of cases and the most common appearance is of a small, shnmken, scarred, non fu nctioning kidney. Less common appearances arc of: • Cortical scarring. • Rcnal pelvis/infundibular strictures rcsulting in hrdrocalycosis and amputated calyx. Ncphrolithiasis. • Dystrophic parenchymal calcification.
  
  128
  
  Practical ti ps Urin;u)' tract TB spreads in an ante grade fashion. • Check chest radiograph for signs of pulmonary but this is only seen in about 50% of cascio
  
  Furthe r m anagement Medical dmg therapy for TB should be initiated.
  
  Abdominal Imaging
  
  Cases 75, 76
  
  cAr-old fe male presented with
  
  129
  
  Answers 75, 76
  
  Abdominal Imaging
  
  ANSWER 75 Observations (75) Supine abdominal radiograph shows a positiyc 'Rigler sign' suggest ive OfpnClItllOpcrironclim. There is also outlining of the falciform ligamcm and the left lateral umbilical lig:amcnt wilh a large aTca of free gas seen overlying the liver. These findings arc consistent with pneumoperitoneum.
  
  Diagnosis Pncumopcriwilcum.
  
  Differential diagnosis Of causes ofpncumopcritoncum: •
  
  Signs of free gas within the peritoneal ca"ity on a radiograph arc: • 'rootb:l]] sign' - large round lucency in central abdomen. 'Rigler sign' - air on both sides of the bowel w makes it unusually visible. 'Triangle sign' - gas within bowd doesn't no foml edges thercfo re such unusuall}' shaped collections of air should arouse suspicion of ITtt _ 'Unchus sign' - olllline of median umbilical ligament. • Outlining off.:tlciform ligament as well as the IDe and lateral umbilicallig-aments.
  
  Tr:lUllla.
  
  • l:nTogcnic ClUSCS, e.g. laparotomy - usually gas should hal'e resolved within 3 da)'s and its presence is suspicious after this; although it can take up to 3 weeks [0 resolve in some cases. Faster absorption occurs with CO 2 :lIId in obese p:l.ticms. Perforation of abdominal \iscus. Rupmrcd pneumatosis intcstinalis. Rupture of an abscess. Exrcnsion from chest, i.e. pneumomediastinum, broncnoplcural fistula, Through femalc gcnital tract, e.g. intercourse, waterskiing.
  
  Discussion T here :lfe a variety of signs th:lt aid in the detection of free int raperitoneal gas.
  
  Practica l tips If therc is an)' doubt about the diagnosis thcn m chest radiograph or a left knera] decubitus film abdomen should be acquired.
  
  Further management Free intraperitoneal gas is suggestive of:a perforated and is a surgical emergency - inform the surgiCJI immediatcl~'.
  
  Further reading Lc\'ine MS, Scheiner 10, Rubesin SE, n (II. (1991 Diagnosis of pneumoperitoneum on supine abdominal radiographs. Amc";wn jOIIrlJII! of Radiology 156: 731-735.
  
  ANSWER 76 Observations (76) Single image from a barium me:\l cxamination shows there is poor distension of the entire stomach, which is most evident in the bod}' and :lIltrum, with relative sparing of the cardia. Irregular gastric mucosa is seen within the fundus and bod}'. The duodenum is normal. The appearances arc {hose of linitis plastica :and a gastric tumour must be excluded in the first inst3.nce.
  
  Discu ss ion Gastric carcinoma lll:alignanc}' and shows an incre:lsed pre\'alcnce ill The scirrhous type of the tumour, which produco. plastica, accounts for 5- 10% of gastric cancer._ infiltration of tumour causes fibrosis and rigi reduction/ absence of peristalsis in affected area. fundus and body of the slOmach arc most in\'olved.
  
  Diagnosis Linitis plastica due to brasrric carcinOllla.
  
  Differential diagnosis For linitis plastica appearance (m nemonic - 'CALM RAGE' ), Cancer. Am~'loidosis.
  
  Lymphoma - usuall y non-Hodgkin'S type, most common pan ofGI tract ancCled. Metasmscs - from lung, breast and melanoma. Radiation. Alkalis and other corrosivcs. Granulomatous disorders, e.g. Crohn's, TB. Eosinophilic enteritis.
  
  130
  
  ~::e~!:l~:l~tt~'~he st~lach
  
  on CT is often \'cry due to poor distension; however, contrast afe much better due TO their d}'llamic nature.
  
  Furth er management Referral to a gastroenterologist for endoscopy. isappropriarc.
  
  Further read ing B:a-Ssalam:ah A, Prokop M , U!'tinann!\i, tt (I/. Dedicated multide[eetor of rhe sromach: spectrum of diseases. RadioGraphies 23 : 62-
  
  cr
  
  131
  
  Answer 77
  
  Abdominal Imaging
  
  ANSWER 77 Observations (77a , 77b) This fulllcngth film taken at 15 minutes in ;m JVU series shows multiple small, well defined, smooth rounded tilling defects within both ureters.
  
  Diagnosis Pyclonreteritis cystica.
  
  Discussion This is ;\ condition characterized by multiple subepithelial ftuid-nllcd CYSts in the wall of the renal and ureters extending into the ureteric lumen. Theft' association with recurrent urinary tract infections obstmction, with a predisposition in diabetic patients.. condition is asymptomatic and the lesions arc premalignant. Occasionally it presents with haema
  
  Differential diagnosis For radiolucent tilling defects in the ufeters: • Radiolucent calculi . • Transitional cdl carcinoma (Tee). Pycloufcrcritiscystica Blood dots. Ureteric polyps Sloughed renal papillae.
  
  Practical tips Although any part of t.he ureter n1JY be invoked, slight prcdilcction for the upper third.
  
  Further management ChJrJctcristic IVU JppeJrances may well pe ~ confidcnt diagnosis. H owever, if there is diJgDI uncertainty then ureteroscopy with biopsy may so be required. Antibiotic treatment may be unde though the response is variable.
  
  77b Multiple round filling defects.
  
  132
  
  Abdominal Imaging
  
  Case 78
  
  E 78 ry
  
  fnr-ol d fe male with diabetes trd \\1m renal failure.
  
  133
  
  Abdominal Imaging
  
  Answer 78
  
  ANSWER 78 Observations (78a, 78b) Two images from an IVU series arc shown. There is evidence of renal papillary swelling and enlargement. The intcrpoJar calyces on the right have a 'ball on ICC' appearance with 3 collection of contrast m~ucrial in the cemre of the papilla. A partial duplex system is also dcmonstr.ltcd on the right. There is a well defined intr:aluminal non-opaque filling defect in the upper ureter on the right, which repre$Cnts a sloughed papilla.
  
  Diagnosis Renal papillary necrosis. Differential diagnosis Of causes ofrcnall':lpi llary necrosis (mnemonic - 'SAD ROPE'), • Sickle cell disease. • Analgesics. Diabetes. Renal ,-cin thrombosis. Obstructive uropathy. Pyelonephritis. Ethanol abuse.
  
  Discussion Renal papillary necrosis is caused by a variet), of disease processes that rcsuh in ischaemia of the papillary portion
  
  of the renal pyramids. There is a progression in appearances as disease progresses. Phases of de\'cl Papillary swelling/enlargement . Panial papilla')' sloughing - tract fomlation communicating with an irregular ca\·ity. On an I\ this is shown by the 'ball on tee' appearance \\itb contrast extending into a cemrall~' cavitated pa • Total papillary sloughing - which gives a 'signet appearance 011 IVU \\~ th the nonopacified slou_ papilla being surrounded by contrast in the calyx/ ureter.
  
  Practica l tips To diflerentiate between the various causes, ide whether the involvement is unilater-al (renal \'ein thrombosis, obSITucri"e uropadly) or bilateral (s}'sterniecauses). Diabetes is the most frequent cause (50%) . A classic 'cx;lm type film' to be aware of is the 1\1.: showing renal ,"'pillal)' necrosis and background abnormalities ofa condition that has been treated with NSAlDs, e.g. ankylosing spondylitis, or boo! changes of sickle cell disease. Furth er management Trcatmem ofthe condition requires identification (X unde rlying cause and control of this disease process
  
  C'
  
  "
  
  7ab Sloughed papilla (left) and 'ball on tee' appearance (right).
  
  134
  
  Abdominal Imaging
  
  Cases 79, 80
  
  CASE 80 History A 28-year-old female presented \\;th tipper
  
  abdominal pain , pyrexia and jaundice.
  
  135
  
  Answers 79, 80
  
  Abdominal Imaging
  
  ANSWER 79 Observations (79) Single image from;} barium swallow examination shows a shaggy, ulcerated Illllcosal pattern in the thoracic oesophagus typical of candidiasis.
  
  Di agnosis Calldidn ocsophagitis.
  
  Discussion
  
  CytOmegalol·irus (CMV) <>esophagitis - presenrs radiologicall)' with a single or multiple giant flat superfici;llulcer{s) ncar the gastro-ocsophageal junction. Less commonly, it can present as discrete small superficial ulcers as arc seen in herpes oesophagitis. H IV ocsophagiris - chamcterized by giam oesophageal ulcers, difficult to differentiate from O .'IV <>esophagitis.
  
  Cnlldidn ocsophagitis is the mOst COllllllon cause ot'
  
  infectious ocsophagitis with C. (I/bicfllIS being the most commonly ilWol\"cd org:lIlism. Immunocompromiscd pariems have a predisposition to infection. Prcscmarion is
  
  All affect imlllunocompromiscd patientS :lIld arc mrcly in immunocompctents.
  
  with gradual d)'Sphagia, odYllophagia and rCl.rostcrnal pain
  
  Practical tips
  
  Association with oral infection is seen in up to 80%, characterized by SlTcral pltchy, white plaques cOI"cring the mucosa. In the oesophagus there is predilection for the IIpper 1J(f/fQ!tlJe oesopllflg/IJ with the appear-Jllcc of multiple, shaggy, longitudinal mucosal plaques. There is reduction in primary [Xrisf"Jlsis and some narroll;ng oCthe lumen due to spasm and oedema. Stricture formation is a rare complication. Other infecti\·e causes of ocsophagitis include: Herpes ocsophagitis - the second most C0111mon cause of infectious ocsophagitis. Caused by herpes simplex virus ( HSV) type I and most commonly presenting in males in 2nd-4th decades. Usuall)' involving the mid oesophagus, there arc multiple small discrete superficial punctate ulce rs. T he intervening mucos.:J. is usually of normal appearance.
  
  Do 1l0tmistJke pseudodil'erticulosis of the oesophagus deep ulceration. This is a benign condition II oesophageal mucous glands become dilated and torm shaped outpouchings rrom the lumen. It is associated any $CI·ere ocsophagitis (sec Case 38 ).
  
  Further management Always consider immunocompromi~tion if not alreadyapparenr. Oropharyngeal candidiasis ma)' be a pointer to th~ nature of oesophageal ulceration but upper Gl endoscopy is still often needed to confirm via bi and brushings. Antifungal or amiviml drugs tor treatment as appropriate.
  
  ANSWER 80 Observations (80a, 80b) Axial CT image and coronal reformats ofthc liver with IV contrast in portal ,·cnolls phase are shOll'n. There arc multiple, well defined low attcnuation cystic structures seen throughout the liver. These appear to be communicating lI'ith the bile ducts and are better demonstrated on the coronal reformatted imagcs (SOb), No calculi are scen in the ducts. The CT appearances arc highly suggcstil'e of Caroli's disease and the clinical detJils suggest cholangitis has de\'clopcd.
  
  Complications include biliary stasis, stoncs, hepatic abscess. There is an increased risk carcinoma in patients ,,;th Droli's disease. Biliary . and porral h)'I>Crtension arc 1/{Jf;complications.
  
  Diagnosis
  
  Practical tips
  
  Caroli'sdisease.
  
  Diagnosis is dependent on showing that the communicate lI'ith the biliary tree - coronal and reformats call be essential for this.
  
  Discussion Caroli's disease is a rare autosomal recessive disorder characterized by multifocal, cystic dilatation of the intrahepatic bile ducts. The differential diagnosis for this condition is polycystic li\·er disease, however the two can be discriminated by identif)'ing the communication betwcen 'cysts' and bile ducts in Caroli's.
  
  136
  
  Associations orCaroli's disease: Medullary sponge kidney (80'it). Polycystic kidney disease. • Congenit;ll hepatic fibrosis. • Choledochal cyst.
  
  Further management If there is anI' concern as to whclhcr the communiCating, MRCP (magnetic resonance pancreatography) is a noninvasive method I
  
  Abdominal Imaging
  
  Cases 81, 82
  
  137
  
  Answers 81, 82
  
  Abdominal Imaging
  
  ANSWER 81 Observation (81a, 81b) Two MIU images are presented - T2 weighted sagittal and axial images through the pelvis. TheS(: images show bulky enlargement offhc uterus. The junctional zone is widened and poorly defined and the re aTC l11ultipk high signal fixi within the myometrium in keeping with ccropic
  
  endometrial tissue These appearances arc typical of adenomyosis.
  
  Diagnosis Adenomyosis.
  
  Di sc ussion Adenomyosis is a chronic condition affecting women during the re productive years. There is III incrclscd incidence in multiparous women, suggesting that uterine trauma plays a role in the development of this disease. The disease is characterized by ectopic endomerrial tissue spreading into the myometrium resulting in myometrial hyperplasia. The condition is benign and although it is associated with endometriosis, there is no increased risk of de"cloping endometrial/utcrine malignancies. The disease can be either fOCal or difli.tsc. It can sometimes be difficult
  
  on US to differentiate focal forms of the condition fr uterine fibroids. US appearances afe of poorly defined hypoechoic area within the myometrium with multiple small, 2-4 nu:a, ectopic endometrial cysts seen withill the myometriu There is thickening of the anteriof and posterill nwometrialwaiJs . . Ivl Rl appearances arc \'ery similar - myometrial \\ thic kening; high signal (on T2 weighted images) ect endomctrial cySts in the myometrium and focal/di thickening of the innermost layer of myometrium kn as the junctional zone. Symptomatically pjticnts present with menorrhap.;.. pelvic pain and dysmenorrhoea and the condition can to infertility.
  
  Practical tips Widening of the junctional zone with eCTopic cndom cysts arc the best M RI features for diagnosis.
  
  Further management Hysterectomy is the only management option to rc symptoms.
  
  ANSWER 82 Observations (82a) Single AP radiograph of the abdomen demonstrates a large mass lesion in the right upper quadrant, which shows clllyilinear calcification of the wall. Appearances would best fit with this being a calcified wall of the gallbladder. No discontinuous areas in calcification jre seell.
  
  Diagnosis Porcelain gallbladder.
  
  Differential diagnosis Similar appearalll.:es on US can be due to: • Emphysematolls cholecystitis. • Gallbbdder filled with stones • Normal gas-filled bowel
  
  Discussion This is an idiopathic condition that was coined to describe the blue discolouration and brittle naUlre of the gallbladder at surgery. The condition is associated with gallstones in 90% of cases and is more frequently seen in women (sex ratio of 5: 1). Patients are usually asymptomatic and diagnosis is made incidentjlly on plain films or US imaging. Radiographic appearances mirror the histological findings of dystrophic calcification in a chronically inflamed gallbladder. T he caleification can be of twO types: • Curvilinear calcificjtion in the muscularis (82b). • Punctate calcification in the mucosa/glandular spaces.
  
  138
  
  Imaging features on US arc of curvilinear/pun calcification in the lhickened wall oflhe gallbbdder, \\ can be local or affecting the whole wall , wi th Illi jcoustic shadowing. On oral cholecys tograph y_ gallbbdder is found to be 1l0nh.lIlctioning. The illl of identiF,'ing the condi tion is the risk of den'l gj!lbladdcr carcinollla, which occurs in 10-2 0% of Appearances of galibladder carcinoma in this simation be of a luminal tilling defect, infiltration cjusing focal thickening or a localized filllgating mass. Cholecyst is therefore recommended e\"en if the paticlU asymptomatic.
  
  Practical tips • Ultrasound and CT scans should be scrutinized far secondary gallbladder malignancy when porcelain gallbbdder is noted • A discontinuity in the calcification whcn the whole gallbladder wall appears to be calcified may be a to gallbladder carcinoma.
  
  Further management CholeeystectolllY is advised due to the high risk of tuml
  
  Further reading Kane R, Jacobs R, Katz], Costello P ( 1984). Pore gallbladder: ultrasound jnd Radi%gy 152: 1"37- 141.
  
  cr jppearances
  
  Abdominal Imaging
  
  Case 83
  
  139
  
  Abdominal Imaging
  
  An swer 83
  
  ANSWER 83 Obs ervations (83a, 83b, 83cl Three MIU images arc shown - a sagittal T2 weighted image (833 ), axial Tl (83 b ) and axial Tl weighted lilt suppressed image (8 3c ). There is a large 4 em lesion idcnti licd within the left ol'ary, which is of high signal 011 both T I and T 2 weighted images and shows no fut suppression. On the sagittal T2 weighted image, there is el'idence of layering of the contents of lhis mlSS. These appearances would be entirely consistent with blood produC[s and the lesion represents an cndomeniorna.
  
  Diagnosis Endonletrioma.
  
  Di scussion End ometriosis is a chronic condition characterized by ectopic deposits of endometrium outside the uterine cavity. It aflccts women in the 4th-6th decades and is characterized by chronic pe!\'k pain, dysmenorrhoea, menorrhagia and dyspareunia. Common sites for endometrial deposits arc the ovaries, fallopian rubes, pouch of Douglas and uterosacralligarnents, however more dist'Jl spread can occur to the bladder/bowel wall with even further distal spread to the lungs and plcur.l. Spread to the G I tract most commonly occurs TO the distal sigmoid :llld the rect'osigmoid wall and deposits can result in peritoneal ad hesion and subsequent obstruction. Repeated bkeding of the ectopic endomet.rial deposits results in fibrosis and pain.
  
  Endometrial deposits can be focal or diffuse. In case, the cbssical ap pear:mcc of an endometrioma 'chocolate cyst' is shown with a 1- 5 em cyst contai layered blood products of high signal on T l and _ weighted images. There is no signal loss on fut supprl!SSl( sequences. The diffuse form is more common, how("l with multiple smaller scattered cysts seen in se\ locations. Complications depend on the site of the deposin can result in: InfertiliTY (due to involvement of the fallopian tubes causing adhesions/fibrosis ). Bowel obstruction (due to fibrosis/stricturing oflbt: bo\\'clwall). Pneurnothor.lx (due to spread to the pleura ).
  
  Practical tips Endometriomas can be diOcrcnriat'cd from de rmoid by using 3. fur suppressed MRj sequence. Both will high signal on TI and T2 weighted images endometriomas will still be high signal on fa t supp sequences.
  
  Further management This wndition can be difliculr 10 COntrol. management with hormonal suppression of menst is often attempted but surgical excision may be req
  
  S3e l esion remai ns high signal on the T1 fa t suppressed image suggesting that it represents blood .
  
  140
  
  Abdominal Imaging
  
  Case 84
  
  -old female presenlcd .tbdominal pain.
  
  141
  
  Answer 84
  
  Abdominal Imaging
  
  ANSWER 84 Observations (84a) This single image from a double contrast barium enema shows abnormality of the sigmoi d colon with poor distension and apparent e:\ternal compression of the bowcl loops inferiorly. Thn:e well (Idined eakifie densities an: seen adjacent to the affected segment and ha,'e the appearance of teeth. Diag nosis Ovarian dermoid. Discussion Ovarian dermoid cyStS arc benign lesions, which arc relali,'cly common, accounting for -20% of all o"arian neoplasms. These mostly present during the reproductive years, with a mean age of presentation of 30 years. They contain tissue from all three germ cciliayers - ectodermal (ski n, brain), meso
  142
  
  Appearances on plain radiograph: teeth occur in 56 of cases and arc readily identifiable. Mass effect \\ith bowel loop displacement is otten seen (84b). The image shows a cemral lucent demit}' rcprcscntatin ' f:tt in the right side of the pelvis, which is dispbcing sigmoid colotl. Appearances on us/crjf\'lIu ::t well delincd high cchogcniciry lcsion is identified in the o\'ary due to high fur content (84c). Again teeth arc easily identified with posterior acoustic shadowing, \\idUII the solid Rokit3nsky nodule. Fat is readily iclcntifiUo 011 CT and J\'I RJ b~' virtue of its low density :lIld hi_ signal.
  
  Complications arc of torsion, rupture and degeneration (seen in 2% of cases).
  
  mali~
  
  Practical tips Some films uscd in radiological exams rely on the can nOling an incidental lesion at the edge of the film. in ovarian dermoids arc a classical example of an 'ed~ the film'case!
  
  84
  
  Abdominal Imaging
  
  ma nagement the re is still clinical concern after l.lltrasound, useful in completely characterizing these Figures 84d-f arc axial T2 weighted (84d ), ghted (84c) and fut satur.llcd (84f) axial _ of the pelvis showing dermoid cysts in both . The right most cyst shows signal void rly on all image sequences in keeping with --,nion :l1ld complete f.1 t suppression confirms the inam fat coment ofthcsc lesions. ani to gynaecologist for surgical excision. ~
  
  Fu rther reading OutW3tcr EK., Siegelman ES, HllIlI JL ( 2001 ). Ovarian teratomas: tumor I)'pes and imaging characteristics. RndioGrnp/)jcs 2 1: 475- 490.
  
  84d-84f Axial T2 weighted (84d), T1 weighted (84e) and fat saturated (84f) axial images of the pelvis showing dermoid cysts in both ovaries. Loss of signal on the fat saturated image confirms the high fat content.
  
  143
  
  Abdominal Imaging
  
  CASE 85 History Incidental li'"cr lesion W,\S nOfcd on abdominal ul trasound scan ora 26-ycarold female
  
  Case 85
  
  Abdominal Imaging
  
  Case 85
  
  145
  
  An swer 85
  
  Abdominal Imaging
  
  ANSWER 85 Observations (85a. 85b) Selected prc- and post-contrast l'vIlU images of the liver. Prc-contr:lSt images (853 ) demonstrate:l brge, well defined lesion in segment 4 of the liver, which is of slightly reduced signal on Tl weighted images. Out of phase Tl weighted images show 110 significant alteration of liver signal [0 suggest the area is due to focal f.ltty sparing. The lesion is slightly hyperintense on T2 weighted scans. On dynamic post·conrrast imaging (85b ), the lesion ShOII'S marked enhancemem during early arterial (25 s) phase with a central hypointcnse scar ,·isible at this stage. There is subsequent wash out of contrast in the portal (60 s) and equilibrium (3 min ) phases to become isointense with liver. The central scar shows enhancement in the equilibrium phase. T he contrast agent used is gadolinium BOPTA (GdBOIYfA) and the image taken at 90 min shows persisting contrast uptake within the lesion indicative of hepatocyte content. These appearances and pattern of enhancement would best fit with the diagnosis of focal nodular hyperplasia.
  
  Diagnosis Focal nodular hyperplasia (FNH ). DiHe;e ntial diagnosis For hepatic lesion with central scar: • FNH. Adenoma. Hepatocellular carcinoma H aemangioma. Fibrolamellar carcinoma. Di sc ussion Focal nodular hyperplasia is a benign hepatic lesion that occurs mOst commonly in young/ middle aged \\"omen Usually these patients arc asymptomatic, with diagnosis made as an incidental finding, although some patients can present with vague abdominal pain. They are solitary (85-95%) vascular lesions containing normal hepatic elements - Kupffer cells, hepatocytes and bile ducts Imaging features arc very similar to those of hepatic adcnomas. Unlikc hepatic adenomas, development is not associated with the oral contraceptive pill, though ocstrogens can exert trophic ctlccrs Focal nodular hyperplasia is dil'ided into typical and atypical groups, with approximately 50% in each. Typical lesions show the signal characteristics, enhancement pattern and central scar as described below. Atypical lesions may show signal heterogeneity, with more marked '1'1 and T2 hyperintensity, and no centr.ll scar . • Imaging Icatures on cr and J\HU: classical FNH is isodense on CT, while MRJ shows mild Tl hypointensity and mild '1'2 hyperintensity. There is prominenr enhancemellt in the arterial phase following IV contrast, the central s..::ar being conspicuous at this point due to nonenhancement. Contrast washes out rapidl). in the portal and equilibrium phases to become similar to normalli,·er, though there may be scar enhancement in the equilibrium phase.
  
  146
  
  Liver specific MRI contrast agents can help further.. In this case, a gadolinium agent with additional li, specific properties (Gd-BOPTA) has been used.,\[ gadolinium chclates in current usc arc cxtraecll agents that arc excreted \·ia the kidneys. Gd-BO PT. and Gd-EOB-DTPA arc tll"O agents cUTrently in that ;1]SO show a proportion ofhepatie excretion. Tbnbeha'·e the same as conventional extracellular age during the arterial, portal and equilibrium phases of&. MRI, but also ilhlS[rate funcrioning hepatocyte ti on delayed illl;lges. Since FNH contains hepal00 and abnormal bile ductules, such agents arc rctai within FNH on delayed images {20 min to 2 depending on the agent}. Other non·gadolinium liver specific MRI contrast agents can similarly characterize FNH on the basis of the fu nctio hepatic tissue elements it contains. Imaging features on angiography: these lesions usually ha'·e a central supplying artery with centri fu~ filling giving a 'spoke wheel' appearance; this can difl"crcntiation from an adenoma, which has several peripheral supply vessels. Imaging features on sulphur colloid scan: usuall y these lesions show normal activity due to the pre ofKllpflercclls.
  
  Practica l tips • Sulphur colloid SC:lllS h:w(' Tr:lditionally been used' help confirm FNI-I suspected on CT and M IU by virtue of the pn:scncc of Kuptler cells. Liver spccili.: contrast agcnts ,.1s described above lllaY .1l0W 0 this in many cases. Hepatic adenoma is a cOlllmon ditlcrcntial when FN H is considered and there is evidence that ag~ such as Gd-BOPTA can help ditfcrcnti:J.tc FNH . hepatic adenoma h), dClllonstr:lting the presence at functioning liver tissue in rhe fonner.
  
  Further management Difl""erentiation frolll adenoma and hepatocC" carcinoma can sometimes be difiicult el·en Ilith histol inlormation. Excision is then carried out. Further reading Schneider G, Grazioli L, Sai ni S (2005 ). MRI oftlJ( Liller. Springer, Nell' York
  
  Chapter 3
  
  CENTRAL NERVOUS SYSTEM, HEAD AND NECK IMAGING D MRI HEAD imaging, if:ln abnormality is nOT obvious at first ..Deck all arcassyst'cmatically: h if IV contrast has been given. les - check the configuration and size, ensuring is appropriate for the other CXf!.l-3.xi:J.1 CSF such as the sulci. hemispheres, brainstclll and cerebellum - on in particular, there is a lot of information to tc for focal masses, signal abnormalities and ral abnormalities! In examination \ 'iVa5, it may lIIlrC'alistic to expect the candidate TO quickly detect .lhnormalitics so you may well be gi,"cn some cc or JUSt presented with selected images. A ~",lluation of FU\JR (fluid anenuatcd inversion :) or PD (proton density) sequences may .' lead you to pathology on M RI .
  
  -axial spaces - carefully check for normal size, c of blood or other collections. is - chcck for enhancemenr on cr and normal \Xtids on i\'IIU {including vessels at the skull periphery - assess orbits, sinuses, temporal Iskull base and skull vault. e has their own blind spots bur here arc some ed areas 1'0 check when all else seems normal: tempo ral lobes and posterior fossa 011 ges of the teml>oral loocs arc often degraded by artefucr and pathology easily missed. The same to a lesser extent ill the posterior fossa. base and petrous bone - pathology GI.Il be easily JUiooked, especially on M Itl where there is a lot to
  
  cr -
  
  -
  
  ~.
  
  signs of stroke - the
  
  earl~'
  
  changes ofinfurction
  
  be \'ery subtle. Carefully check for subtle focal 'ng and reduced grey-white matter rcntiatio n. The Imer is beSt detected by using I11II:I'\' 'narrow windows' on • c:Oous sinus thrombosis and subdurnl empyema arc disorders with significant morbidity and mortality t can Ix easily overlooked. When presented with an ~ntly no rmal of a patient with a history of cDmincing acute imracrnnial pathology, always double .:Deck for increased density in the venous sinuses and mbtle subdural collections. Administrntion of IV ~urast may help confirm either. - Hxmorrhage - subtle subarachnoid or subdural tiood. must not be missed. C heck carefull~' for small
  
  cr.
  
  cr
  
  amounts ofsubarachlloid blood layering in the ~> occipital horns or around the medulla close [0 the fornmen magnum. Pituitary - this is well sccn 011 sagittal MRI scallS, so do not forget to chcck it. A notc on mass lesions: \Vhen confronted ,\~th an intracr:lIlial mass lesion, assess the following: • Location - cemin tumours ha\"{~ a predilection for certain areas of the brain . • Age - this has a strong influence o n the differential diagnosis. Certain rumours are common in the paediatric age group while metaS[;lses always have to be considered in the older adult population. Is the mass intra- or eur.t-axial - that is, does it originate within brnin substance or nOt? The distinction is very important in fomling a differential diagnosis and though it seems obvious, can sometimes be difficult to establish on in particular. An eurn-axial mass causes crowding ofthc subcortical white matter fronds that extend between gyri. Com'erscly, an intrn-axi31 mass stretches the grey matter around it and consequently displaces the fronds of white maHer 3parr. Mass eflcer and oedema - check ror midline shift and effacement of normal CSF sp3ces such as sulci and cisterns. If extra-axial CS F spaces arc obliterated, there is risk or death due to br:lin herniation th rough dural al>c rt ures. !f rou see such features, then indic3te the degree of urgency required in seeking neurosurgical evaluation and intervention. • Assess the enhancement pattern of the lesion. This is often nonspecific but certain patterns such as the intense, homogeneous enhancement of meningioma ma~' allow a confident diagnosis.
  
  cr
  
  CT AND MRI NECK C ross-sectional imaging of the neck requires excellent an3tomical knowledge! Familiarize ~'oursclf with the anatomical divisions of the upper aerodigesth'e traer, the fascial Sp3Ces ofthe neck along which tumour and infection track, and the nodal stations of the neck. With this knowledge, you can make a meaningful interpretation for the E?-..TT surgeon . T2 weighted scans with fu! suppression arc particularly useful to eV31uate at first inspection if these arc avail3ble, for detecting both primary pathologr and
  
  147
  
  eNS, Head and Neck Imaging
  
  nodal disease. Thereali-cr, sinmltaneous examination of the Tl weighted scans can help to better delineate the precise anatomical location of disease.
  
  ~~~IIN
  
  RADIOGRAPHS
  
  T here arc a very limited number of pathologies that arc likely to be shown on a skull radiograph:
  
  ~
  
  Trauma As well as skull fractures, check for indirect signs of fixture such as fluid leyels in sinuses and intracranial air. Remember 10 look at all the bones on the film - the fracture of odontoid peg at the edge of the lateral skull film is a basic exam type of case. Calcificatio n Know the normal intracranial calcifications such as pineal and choroid plexus so that you can ditlerentiatc from pathological calcification such as that associated with meningioma. Pituitary Size and shape of the pituitarr fossa should be assessed.
  
  ~
  
  Vault Several diffuse processes have characteristic changes on the skull radiog raph and make for 'spot diagnoses', e.g. acromegaly, fib rous dysplasi;l, th3lassacmia, hyperparathyroidism ;md myelon13.
  
  Spine As always, there arc many potential pathologies to sec on plain films of the spine but trauma, infection and neoplasia arc particularl y common.
  
  -0.
  
  148
  
  Tra uma When ;lssessing cen'ical spine films' • First ensure that the complete cervical spine has been imaged down to C7- Tl level On the lateral film, look at the anterior spinal, posterior spinal and spinolalllinar lines to ensure continuity (I.e. imaginary lines drawn along the anterior and posterior vertebral body cortices and along the anterior limits of the spinous processes).
  
  Look at the sofi- tissues of the anterior cen'ical space. which should be no greater than a third of the width ofa vertebral bod" for C I- 3 and no more than the width ofa \'ertebr:al body for C4-7 . Check the imen'al between the anterior arch of atlas and the
  
  ~ ~~1~~1;ol~~n;~~ ~d~i:s s~~r~ 1~~~I~~a~~;i~r~I~. greater
  
  On the AP film :J ]ook for misalignment of the vertebral spinous processes in a \'Crtical plane, which would suggest a flcet joint dislocation. Ensure that the AP 'peg' view adequately excludes a C2 peg fracture. When you arc suspicious of an unstable fracture, nak this clear so that adequate steps arc taken to prote..., the cervical spine until such time as this has been confirmed or excluded with CT. When assessing thoracic and lumbar films, carefully chco. alignment and \'ertebral body height. On the AP fil m the thoracic spine, look for widening of the paravcrte stripe suggestive ofhaematoma. Mali gnancy Bony involvement is one of the most common sites metasratic tumour spread along with the lungs and Ihu
  
  ,-~o~I~~rl~~~~ol~i~~II~~:'~I~~~~~~,i~;,n:e~~~~l~~~~~::e~~ spinous process for lytic deposits. As ever, pay attention the soft tissues - a paravertebral soft tissue mass may ~ you to a subtle bony lesion. Do not lorget that \\ mrcloma classically produces foc al bony lucencies possible resulting vertebral collapse, a significant nu . of cases show only diffuse oSleopenia, which can III osteoporOSIs. Infection Spinal infection is usually centred on the disc and the there will be a reduction in disc height with i1l\'olvc of the superior endplate of the vertebral body belo\\ inyoh·ement of the inferior endplate of the vertebral above. Added sofi- tissue d\le to abscess is likely to be and in the cervical spine will be seen as a preyertebral tisslle Im.SS on the lateral cervical spine film; in the [h spine this will be seen as a widening of the paravert stripe; and in the IUl11b::lf region there may be a abscess
  
  eNS, Head and Neck Imaging
  
  Cases 86, 87
  
  ~d presented with bilateral dcafilcss.
  
  149
  
  ANSWER 86 Observations (86a) Single high-resolution axial CT image of the skull at the level of the petrous bone. There is bilateral enlargement of t he vestibular aqueduct. Preci se measurements have not been documented but the aqueducts afC dearly significantly wider than the horizonta l semici rcular canal seen on the right.
  
  Diagnosis Enlarged vestibular aqueduct syndrome. Discussion Enlarged vestibubr aqueduct syndrome presents clinicall)' with sensorineural hearing loss. Hearing is usually prescnt :\f
  
  birth and then deteriorates from age of -3 years.
  
  Deterioration is often in a stcP\\~SC manner, associated with episodes of minor head trauma. There is a suggestion of an inherited recessive genetic link and 50% of cases arc bilateral. A degree of cochlear dysplasia is present in 75% of cases. The normal endolymphatic duct o riginates from the vestibule, via the common crus and extends posterolaterally in the bony vestibular aqueduct to t he endolymphatic S.1C. The endolymphatic sac li ~s on the posterior aspect of the petrous temporal bone. Enlarged vestibular aq ued uct syndrome is diagnosed when the diameter at irs midpoint
  
  exceeds 1.5 mm. Figure 86b indicates the dil:J.ted aqu in rhiscase. Diagnosis of the condition is important due to the _ results achie\'cd with cochlear implantation.
  
  Practical tips A quick assessment of the vestibular aqueducts can made by comparing the diameter at midpoint to thc= diameter of the adjacent posterior semicircular ca~ the aqueduct should not be larger. When identified , check the cochlea for e"idence of dysplasia.
  
  Further management Advise on a\'oiding head trauma where possible, t .~ no contact sports. • Hearing can be improved with cochlear implants.
  
  Further reading Dahlen RT, H arnsberger HR, Gray SD etal. ( 1997 ). Overlapping thin -section f.1st spin-echo MR oftht large \'estibular aqueduct syndrome. American jOltrlml of Neuroradiology 18: 67- 75. Valvassori GE ( 1983). The I:J.rge vcstibul:J.r aqueduct associated anomalies of the inner car. Otola171lgo _ C/illicsofNortbAmeriCll 16: 95- 101 .
  
  86b Axial CT image demonstratin g an enlarged
  
  vestibu lar aqueduct.
  
  150
  
  eNS, Head and Neck Imaging
  
  187) que image from a parotid sialogr-anl with no iiIm provided for comparison, The parotid duct is calibre with no obstructing stones or stricture. florid pUllerate dilatation of the _ _ _""'I" ",,,,,,,,1,,· and acini in keeping with punctate
  
  Case 88
  
  Althollgh sialography is the primary method of diagnosis, US is useful for tollow-up as it avoids thc radiation burden and invasive nature of sialography. At US, the gland is swollen with :I hettrogencous appearance and multiple hypo<:choic tOci within it. lntraglandular reaerivc lymph nodcs may be noted
  
  Practical tips lnrraglandular lymph nodes arc seen in the parotid but not the submandibubr gland, which becomes encapsulated before lymph node de,"dopmellT occurs embryologically.
  
  Further management recurrent sialadcnitis is llSU;\lIy associated with ,[rietllTc causing duct obstruction. These causes, • Me rare in children and chronic infhlmmation is JlTlmonJy idiopathic in nature. The parotid is more than the submandibular gland, perhaps due par.ltivcly smaller salivary Olltput. Clinically, present with repeated artacks of swelling of tile associated fevcr. Age of onset is typically 3- 6 symptoms usually rcsoh'ing sponranl"Ously after
  
  Although sia1cctasis most commonly idiop;\thic, Sjogren's syndrome can also cause this appearance and shou ld be excluded with laboratory investigations. I'/J ?
  
  Further reading Yam KL, L1U C, Li CK ( 1997 ). Primary Sjogren syndrome prescnring as rccurrclll parmitis. HOllg KOIIDJolmlal ofPaediarriu (Ncw Series) 2: 47-50.
  
  151
  
  Answer 88
  
  eNS, Head and Neck Imaging
  
  ANSWER 88 Observations (88a) CorOIl:ll Tl weighted MR il11agcsofthc orbit show llllrkcd bilateral swelling of the muscle bellies of the interior, medial and superior rc([Us muscles with sparing of the tendinous insertions. This disrribution of extraocular Illuscle swelling is typical ofth~'roid ophth:tlmoplthy.
  
  Diagnosis Thyroid ophthalmopatlw.
  
  Differential diagnosis Orbital pSCudotlllllOur - usually ilwokcs both muscic belly and tendillo/ls jmcrtioll; is pnillflll compared (0 painless thyroid o pluhalmopathy and is morc commonly IIlIilateral.
  
  Discussion Thyroid ophthalmopathy is a disease of the orbit characterized by deposition ofmucopolys:tCcharidcs \\ithin the muscle bellies of the intraorbital muscles. [t tlsu:tll y presents in adults and is more common in \\·omel1. Thc paticllt is usually hyperthyroid (although 10% of cases arc found in cuth yroid patients) with ocular presentation within I year ofhypcrthyroid symptom onset. Se"erity of the cye disease, howcver, is not rela ted to thc sC"crity of thc th yroid hormonc imbalancc. Radiological fcatures arc as follows Majority of cases arc bilateral although therc is often asymilletrical diseasc. About 10% arc unilateral lnft~rior and medial rectus arc most coml11only affected, with the lateral rectus being lc,\st likely to be affected.
  
  Musele bellies arc ani..~cted with swollen appearance and relative sparing of the tendinous insertions. Axix CT images (88b) show swollen medial recti with t)'pical sp:lring of the tendinous insertions Proptosis, lid retr:lctiOIl and reduced eye mo'"eIllCnlS are features. Usually rhe disease resolves spontaneously but ste trcatmCnt is prescribed to treat the swelling :lnd [0 red intraorbit:ll pressure, which can otherwise cause optic n and ophthalmic "ein compression. Surgical decompressil is sometimes required when there is acute swel ling threatellcd visual loss.
  
  Practical tips The order of muscles involved in thyroid ophthalmopathy is ( from most to least frequent ) IntCrior>]\kdial>Supcrior>LaterabOblique - Mllen - I'M SLO(w).
  
  Further management Thyroid ophthalmopath)' with visual disturbance req prompt evaluation and treatmcnt gi"cn the potential to visual function.
  
  Further reading Hosten N, Sander B, Cordes M, etn/. (1989). Gr.JXCS ophthalmopathy: Mit imaging ofthc orbits. RfldiologJ' I72: 759- 762.
  
  88b Selected axial CT images through the orbits show thickening of tbe medial rectus muscles with their tendons.
  
  152
  
  eNS, Head and Neck Imaging
  
  Case 89
  
  89 \-car-old lll ale presented with al headaches.
  
  89a Axial PO (top left), axial and sagittal T1 weighted (left), axial and coronal T2 weighted (right) MR images.
  
  153
  
  Answer 89
  
  eNS, Head and Neck Imaging
  
  ANSWER 89 Observations (8ga) Selected MIU images demonstrate a round lesion in the amcrosupcrior aspect of the 3rd ventricle. The lesion is mildly hypcrimcnse on Tl weighted images, with variable signal on T 2 weighted images and a graduated fluid !clod appearance. It is likely that the COntents are proteinaceous flu id and the findings arc consistent with a colloid cyst of rhe 3rd vcnrriclc
  
  Diagnosis Colloid
  
  C)'SL
  
  Differential diagnosis For 3rd vcntricular lcsion: lvleningioma - these arc not usually hyperintense on T I . Meningiomas commonly caJcit)r and show diffuse enhancement with cOntraSL Ar.lchnoid cyst - these arc usually isodense on CT and isoimense with CSF 011 MRi Dermoid cyst - usually found in the midline. These contain fut and arc therefore usuall~' hypode115e on CT and have mixed signal on J'vllU. Ependymoma of the 3rd ventricle - these arc "ery r.lre. Imaging featllres i'nclude cystic areas, necrosis and calcification and the y show diffuse uniform enhancement. Basilar tip aneurysm. Discussion Colloid cysts account for approximately 0 .5-1 % ofCNS 'tll11l0u rs' . They usually present in young adults and arc more commonly seen in males. T he cysts arise from the interior aspect of the septum pellucidulll and extend into the 3rd vCllIricle. Presentation is usually secondary to obstructed CSF flow at the leld of the 3rd ventricle with features of positional headache due to tr.lnSielll obstruction, distllrb:lIlces of gait, reduced consciousness lcl'el and papilloedcma. The lllajority of cysts ( 80%) contain mucinous material while 20% contain fluid similar to CSF. Typical features on CT: well defined thin walled cyst in the 3rd veillride of slightly increased attenuation with no en hancement (8 9 b ). Can cause erosion of the sella Typical features on MRI : mucinous material contained within the cyst produces a well defined lesion of increased signal intensity on TI and T2 weighted images.
  
  154
  
  Practical tips A large basilar tip aneurysm can be mist:J.kcn for a colloid cyst on uncnhanccd CT scan Jnd this rcq carly identification and treatment. Need to assess whether or nor there is hydroccp - look at the degree ofdilarariotl of the temporal horns oCthe lateral ,·cntriclcs and look lor depT ofthc brainstcm
  
  Further man agement Although these arc benign lesions they can present with CSF flow obstruction resulting in headache, k consciousness al1d death, so a clinical review tor evi elevated intracranial pressure and neurosurgical re\~ appropriate.
  
  eNS, Head and Neck Imaging
  
  Cases 90, 91
  
  - '"Car-old male presented with \isual field blurring.
  
  155
  
  Answ er 90
  
  eNS, Head and Neck Imaging
  
  ANSWER 90 Observations (90a) This contrast enhanced CT image of the brain demo!lStr;ncs mul tiple ring enhancing lesions with surround ing ":lsagellic oedema in the left frontal lobe. Though no lesions arc seen in the right hemisphere on this single image, JPparcnt vasagenic oedema in the right frontal lobe suggests that there may be fu rther lesions on this side that arc not visualized. The diHcrcntial di:tgnosis for ring enhancing lesions is long, but the history of pyrexi:t points towards cerebral abscesses being most likely. Diagnosis Cerebml abscesses. Differential diagnosis For ring enhancing CNS lesions: Cerebral abscess. (I Metastases. Toxoplasmosis. Demyelination. Lymphoma. Mu lticentric glioma l'v lultip.le inrnrcts . • Resolv1ng haematomas Discussion Patients who arc immullocompromised, on steroids or have dbbctes aTC more susceptible to CNS abscess formation. The most common SOU Tee of infection is haematogenous spread, though direct spread trom intected paranasal sinuses can also occur. Clinical presentation is with headache, seizure and pyrexia. Focal infection pre..:eding abscess tormation is ceTcbritis, and appears as foeallow-density change on CT or high T2 signal on MRJ (90b, 90c). This
  
  proceeds to capsulation/abscess lormation at 10-13 with the ring enhancement illustr:Hed. When arising hacmatogenous spread, the classic3l location is at grC)'-whire nlltter interflce, and most commonly in middle ..:erebral artery territory. The me.dil l wlll is thinnest (due to better perfusion of the grey m laterally than the white matter medially) and results . tendency to rupture on this 3spect into the "cntricle
  
  Practical tips Dif'tcremiation ofthc above list call be difficult and history is "ery important to identify immune statm... foreign travel and clini..:al presentation Look for complications of absces$Cs such as rnpflln" causing meningitis Of ventriculitis, radiologically identified bv enhancement of these structures or sigl1,\1 in thi: sulci on FLAJ R MR images. Wall enhanccmcnt of cerebral abscess is typically unifOfm - thick, im:gubr wall enhancement sho raise suspicion of ..:erebral metastases. A peripheral ring on the unenha nced scan is more common in metastasis than glioma Assess paranas;"ll sinuses for a primary source of infection and ensure a chest radiograph has been 10 look for sourccs ofinfcct'ion and potential rigb;: left shunts. Further management J'vI!U with diffusion weighted imaging can solllcri mo ditlcrcntiate tumour/metastases from abscess. Rest11' ditfusion of pus in an abscess cavity meJns that an is high signal Oll OWl Jnd low signal on ADC diffusion coefficient ) mapping. O pposite findings with llccrorictlllllours.
  
  90b, 90c Axial T1 weighte d postcontrast a nd coronal T2 we i g ht ed~M RI im ag e s of left tempo ral lo be abs ce sses . The ax ial T1 postContrast im age s hows a n e n ha ncing rim w ith s ur rounding low s ignal oed e ma. The coro nal ima ge s ho ws a h igh signa l abscess with s ignificant high sig na l s urro undi ng oedema c ausing a m od e rate d eg ree of mas s effect a nd midline shift.
  
  156
  
  eNS, Head and Neck Imaging
  
  Case 92
  
  91 Di scussion Optic drusen arc focal accumu lations of hyali ne material
  
  in the region of the optic nerve head, which commonly calcify. Aetiology is thought to be either a dc\'clopmcnral abnor!ll;llit)' or a degene rative process and it is histopathologically separate from retinal drusen deposits that call be a nOTmallinding or associated with age related
  
  macular degeneration. Clinically. patients arc usually asymptomatk bur can present with reduced \'isu:al acuity, migraine-like headaches and pscudopapillocdema. Di:agnosis is made by the absence of ad\·ersc imaging features such as abnormal cnhanccmcnt, optic nen'C thickening, mass dlcCI or postcrior globe solid lesions. Ocular ultrasound can be particularly usefu l in evaluating, and confirming this is not anuall). papilloedema. Optic disk haemorrhage is a very rare complication. The majoriry of cases (75%) arc bilateral.
  
  Practica l tips
  
  •
  
  \.omcg:a]ovirus (CMV).
  
  • Herpes simplex.
  
  • 'lpticdruscn. • _ rhisis bulbi.
  
  Bilateral calcification docs not necessarily suggest a benign disc::lsc process. The nonherirable form of retinoblastoma (66") presents at - 24 months \\irh usually unilatcnl disease; but the heritable form (33%) presents ::II - 12 months with often (66%) bil::atenl disease.
  
  • lninal detachment • ~rinopathy of prematurity. • thpcrcalcacmic states - hyperparathyroidism, ..an:oidosis, chronic renal failure.
  
  Further management Ophthalmologica l assessment is required 10 confi rm the diagnosis and check viSllal fields and acuity.
  
  g ad ult m:lle prescnted with and panlysis of upward b'"lZC.
  
  157
  
  Answer 92
  
  eNS, Head and Neck Imaging
  
  ANSWER 92 Observations (92) This midline saginal Tl
  
  elderly. Germ cell tumours arc the most common tum ofthe pineal region, accounting for over 50% of all p. masses. The most common subtype is germinoma, which histological1y similar to testicular seminoma. Pi germinomas arc well defined midline masses that arc more common in m3les than females. T hey afe norma seen in children and young adults. The lesion enh avidly. Oue to the anatomical location of the pineal these lesions compress the aqueduct ofSylvius produ hydrocephalus. The superior col1iculus of the brain ma~' also be compressed producing Parinaud's synd paralysis of upward gaze. Less common germ cd! ttl of the pineal gland include teratOmas, which are m seen in young children. Choriocarcinomas and emb~ cell tumours arc even less common and arc hi_
  
  post-contrast ,'vIR image demonstrates a large, slightly lobulated but well defined mass in the pineal region. The mass enhances homogeneously, compresses the superior colliculus and causes dilatation of the 3rd velUride. The most likely diagnosis is a pineal germinoma causing Parinaud's syndrome and obstructive hydrocephalus
  
  Diagnosis Pineal germinoma.
  
  Differential diagnosis Of pineal region masses: • Germ cdl tumours (>50%): Germinoma Teratoma - extremely heterogeneous mass. Occurs in young children Choriocarcinoma. Embryonal cell tumour. Pineal parenchymal tumours (25%): • Pineoeytoma - well demarcated, calcified, slow growing tumour in1middle ,'ged adults. • . Pineoblastoma - si milar to medulloblastoma. Allects young children. Enhances avidly :llld is nOl usually well circumscribed.
  
  rn~lignant
  
  Practical tips The pineal region is in the midline and masses in region may compress the aqueduct ofSyivius, so
  
  ~. ~t;;~~~ ~~~~~;n~~S~I~~~~~~~r~~h~l~~~~al gland
  
  Others: • J'v lcningioma • Epidermoid or dermoid. • Arachnoid cyst - CSF density/signal • Pineal cySt - common; fluid density or signal though contents can be proteinaceous on MlU. • Lipoma
  
  Discussion The pineal gland is a midline structure situated behind the 3rd ventricle and responsible for biorhythm. It is calcified in most people over the age of 15 years and in almost all
  
  92 Pineal germinoma.
  
  158
  
  exceeds I em in diameter a pathological pineal p should be suspected. Calcification in germinoma when present is ccntnIL. pincobbst'Oma pniphaal. In ~'ollng children think of teratoma (which are \heterogeneous masses), pineoblastoma and a vein Galcnancurysm In young adults, the most common solid lesion is germinoma.
  
  Further management Surgery is difiicult due to the central location in The main rolc for surgery is in obtaining a possibly debulkins of tumour to relieve symptoms. However, serminomas arc radiosensitive and therefore radiotherapy is the of treatment.
  
  eNS, Head and Neck Imaging
  
  Case 93
  
  na PO and T2 weighted axial (top), T2 weighted coronal and T1 weighted sagittal (bottom) images.
  
  159
  
  Answe r 93
  
  eNS, Head and Neck Imaging
  
  ANSWER 93 Observations (93a) Selected J\Ht images of Ihe brain demonstrate hyperintel1sity in the righr trans\'ersc sinus on the T I and T2 weighted images \\lrh an abse nce oCthe nomlal \'enOliS sinus flow \'aids. There is high-signal abnormality seen withi n the right mastoid air cells on T2 weighred images, which is intermediate signal on Tl imaging - this is likely to indicate infection/innammato~' change, Appearances arc consistenr wirh a diagnosis of \'enous sinus thrombosis secondary to mastoiditis. No imracerebral/ccrcbellar h ~\emo rrh age or inlarer is demonstrated on these images.
  
  Di ag nosi s Venous sinus thrombosis.
  
  Discussion There arc a variet), of underl )'ing ellises of \'enous si nus thrombosis, which include trauma, in/ccrion, idiopathic and h)'percoagulablc states, i.e. oral contracepti\'e pill, anti phospholipid syndrome, parancoplastic tumour s)'ndromes, antithrombin III deficiency. Presenting srmptoms can often be \'ery nonspecific, meaning that diagnosis is often OI}ly made radiologically. Symptoms include headache, nausea, vomiting and drowsiness, The superior sagit tal sinus is most commonly aflccted, followed by trans\'ersc and sigmoid sinuses
  
  IUdiological appearances :ITC: Uncontrastcd CT may show a hypcrdcnsc \ '(nOllS sinus due to thrombus (Figure 93b shows a thrombosed superior sagitt:J.J sinus). Contrast enhanced CT shows a filling delcct within the triangular lumen outlined by a sillall rim of (oml"$ the 'empty delta' sign. • MRI demonstrates :lIlabscncc afOaw \·oid within vcnous sinus, with local eflects of oedema, subcom infurction, suteal effacement and haemorrhage. • [n the acute phase, thrombus can appear as isoinrcnsc on Tl weighted imaging and hypointcnsc on 1'2 weighted imaging. TIlis hypointcnsity on T2 can sometimes be mistna. tor flow \'oid and thcn::forc phase-contrast MR.o\. which shows /low, is beneT at identification. • I n a chronic thrombosis, the venous sinus appea hypcrimcnsc on T I and '1'2 weighted images w extracellular methaemoglobin .
  
  Venous infarcts arc idenrifiablc by their nonconformity \\ltb arterial territories and haemorrhagic tendency, Figure 93b demOIlstr.u a \'enous inf.,rct that docs nOt conform to the anatomical area sl1pplied by the vessels of posta. circulation,
  
  93b Selected axia l CT images demo nstrate a fo cal area of low attenuation involving grey and with appearances consistent with an infarct. The distribution, however, does nol conform to arteria since this rep resents a venous infarct.
  
  160
  
  eNS, Head and Neck Imaging
  
  Ca se 94
  
  sinus thrombosis call present in a '"cry • manner and has signific3m morbidity and umrC31cd. When assessing scans of acutely _-call)' unwell' palicnts, alwa)'S keep it in mind the scan carefully. Similarly, beware of g infan':t$ that show features as described :itham questioning the possibility of an rutg wnous sinus thrombosis. J.ranccs oflhrombus o n MRl are complex ~ \\;th the age o[[hrombus. Moreover, patent sinuses can show absence of tlow \'oid when in certain planes. Making the diagnosis of smus thrombosis from MRI can therefore be 1('d. Modern Illultidctcctor CT scanners h3YC t speed and spatial rcso]urion to image the Sinuses and provide an allcrnari"c that (an be interpret, thromb us appearing as a filling Ul the lumen Oflhc otherwise enhanced '"CnOllS Figure 93c is a coronal reformat of a CT ~l showing thrombus in rhe lefi- tranS\'crsc ~ an ;cmpty dclt.l' sign. 93c Coronal reformat CT image shows thrombus in the left transverse sinus.
  
  Answer 94
  
  eNS. Head and Neck Imaging
  
  ANSWER 94 Observations 194a)
  
  Epidermoid ~ 5% ofCP angle lesions.
  
  Single T2 weighted Mit image of [he brain at the kycl of the internal ;ludirol)' Illcarus. There is a large , well defined soft tissue mass lesion in the Tight ccrcbcllopontinc (C P) angle with extension intO the internal auditory canal. Widening of the canal is seen. No dural tai l is c\'idclH. Acoustic neuroma is m OST likely.
  
  Arachnoid CYST. Posterio r circulation aneurysm. Metastases.
  
  Di ag nosis Acousticncuroma.
  
  Differe ntial diagnosis
  
  orer angle lesions:
  
  • Acoustic neuroma accounts tor 75% orcp angle
  
  masses. Meningioma is the mosllikdy diHi.:rclltial at 10% (94b ). Lesions arc extra-axial and tend 10 be cxtracanalicular and commonl y calci!},. Dunl rails arc seen in up [0 6O'X.oftumours on MRJ.
  
  Discussion Acoustic neuromas arc the most common tumours of C P angle and internal auditory (anal. They typicall~ from the \'estibular di\'ision of the 8th nefl,c and sh. p·erhaps be morc accurately termed \'cstibular sc llomas. These lesions prescll! in the 4th-7th decades arc more frequently seen in females. Prescntation at a rounger age in patients \lith type 2 neurofibro with prescntation in thc 2nd decade. Bilateral aCf' neuromas arc virtually pathognomonic of fibromatosis typc 2, whilc solimry tUlllours arc seen to 25% of cases. Presentation is with sympl scnsorincural hearing loss, tinnitus, vertigo and
  
  94b Axial PO, T2 and T1 weig images w ith three post-contras: coronal images demonstrate a defined, extracanalicula r, enhanci ng lesion in the angle representing a
  
  162
  
  swer 94
  
  eNS. Head and Neck Imaging
  
  ging features: arise ill the internal auditOry canal (lAC).
  
  cause ~nlargcmcnt/c:rosion oftlte lAC. LCSions extend imo rhe CP angle.
  
  L.olt'gcr tumours (>3 em) have central areas of necrosis md haemorrhage. ~ificati on
  
  n
  
  is llOt a feature. cr lesions arc usually imensc with uniform anccmcnt. MRI, lesions arc low sigmll on TI, high signal on
  ieal tips 'nguishing the main C P angle masses from each Ilhcr:
  
  • Bmh acoustic neuroma and meningioma show uniform enhancement so this is unhclphLI. • Acoustic neuroma - expands the lAC, causing flaring of the poms acousricus. May be bright 011 T2 unlike meningioma. Makes an acute angle \\ith petrous bone. • Mcningiolll::l - dural rail of enhancement, obtuse! anglCJwith petrOlls bone. Relatin:ly linle tissuc in the lAC compared to acoustic neuroma. • Epidermoid vs arnchnoid cyst - both appear to follow the density and signal ofCSF on CT and T I md T2 weighted j\'IRJ. Howc\"er, the epidermoid shows increased signal on PO, FLAIR and diffusionfl weightedMI{:l .
  
  Case 95
  
  Detection of acoustic neuroma is largely done with M RJ when possible - thin section T2 weighted sequences are used in the main, with COntrast reserved for difficult cases. When contT:lindicated, use contrast enhanced CT. A subgroup of acoustic neuroma show rel;llively rapid growtb that necessit3tes trea tment while others show extremely slow growth and can be managed morc conservatively, Follo\\;ng initial diagnosis, a repeat
  
  ~~l~~~~~~~~~~~:i:l~:~~n~ ~~o~:~~\,n;;~i~~I~I~~e~~~~~ that can be managed more conservatively. Neurofibromatosis type 2: bil:lteral acoustic neuroma is a recognized diagnostic criterion for this condition. also known as the MlSME srndrome ( multiple inherited schwan noma, meningioma and ependymoma), In the 'real world' mos t acoustic neuromas arc unrelated to neurofibromatosis typc 2. However, in exam "i\'as (and cenainl~' when bilateral), hal'e a high index of suspicion for the meningioma elsewhere on the scan.
  
  *
  
  Further management Therapeutic options involve surgical excision, stereotactic radiation and conservative ma nagement. Which o ne depends on the particubr circumstances of each patient, but also whether the tumour is fast growing o r not. A repea t sean a few months after the first can help identit}' the group of f.1Ster growing lesions that require a more aggressivc approach.
  
  SE 95 ory 30-ycar-old male: -opanlomogram ) done for denral
  
  163
  
  Answer 95
  
  eNS, Head and Neck Imaging
  
  ANSWER 95 Observations (95a) The OPG shows multiple missing teeth in the upper jaw and to a lesser extent the lower jaw LOO. In the riglu body of the mandible, there is a well cin:ulllsuibcd, thin walled, unilocular lucency measuring sen:ral ccmimclrcs in diamete r. 111crc is no associated bon}' dC$truClion. The Ll'St
  
  is 3ssociatcd with the crown of an unerupted molar and
  
  appcar:lIlccs afC consistent WiTh a dentigerous cyst. Diag nosis Dentigerous cyst. Differenti al diagnosis Of cYStic mandibular lesion : P~riapical/radicular cyst. Dentigerous cySt. OdonlOgenic kcratocyst. AmcloblaSIOlll:l.
  
  Aneurysmal bone cyst. Simple bone cyst. Metastasis/ myeloma. Brown tulllour. Fibrous dysplasia. Man}' orher 'small print' lesions - nasopalatine cySt, Pindborg's tumour, Statile's bone cyst, medi:tn mandibular cyst, etc.
  
  Discussion Dentigerous cysts arise from the ctown of an unerupted tooth. The typicall}' aftccted age group is 10-30 years, with a male predominance. The cysts arc slow growing and usually an incidental finding, but occasionally cause pain, swelling or becomc secondarily infected. The majority occur in the posterior mandible. They arc \'ariable in size, and ha\'c a thin walled, unilocular appearance. TIlt associatio" ",ilh all ll1uyupud CYOII"1 is lilt key to diaguosis. The diAcrential diagnosis of cystic lesions of the mandible is vcry long bl![ most of the causes arc so r;tfe that they can be dismissed most of the time. The emphasis will consequently be on common lesions and important rare ones. It is Ixst to assess lesions according to visible association with demition, unilocular \"$ multilocular nature and age of the patient. Note that although not visibly dentition related , lesions such as ameloblastOllla and odolltogenic keratocysr do arise from dental-related tissue.
  
  Dentition-associated cysts: Periapical/radicular cyst - secondary to pulp necrosis of a carious tooth. V"i/ow/ay cyst associaud with thr Toot of a tooth. Can Ix dCStrucli~'e when large:, but noc expansile. Most common lesion seen , Likely to be painful. Dentigerous cySt - IIlIilow/aT cyst associaud Il'ith lIlt cyon", of all murllpted tooth No visible association with teeth: Ameloblastoma/ adamantinoma - rare, locally aggrcssh'e (but non -merast:l.sizing) lytic [Umour th~1 is slow growing and often painless. This is all t.'
  cr
  
  95b OPG s hows large lytic ameloblastoma in the right mandible with areas of internal septation producing a 'soap bubble' appearance.
  
  164
  
  eNS, Head and Neck Imaging
  
  Case 96
  
  If brown tumour is suspected look. for other signs of hypcrpar:uhyroidism such as resorption ofthc lamina dura
  
  orthe teeth producing 'floating teeth' and generali zed dcmincmlization ofrhc mandible and ll1;uilb . Furth er management Though often aS~'ll1ptomatic, dentigerolls cyst can cause pain and swelling and may predispose to pathological merure. Surgical excision (including the uncruprcd tooth) may therefore be undertaken.
  
  Further reading with demal associ,uian: tCd \Iith tooth apcx :md pain . I>crbpical cyst. tcd with unerupted tooth _ dentigerous cyst \'Can and associated with crown ) or JStO lll3 (>30 years). l....'St
  
  Scholl R., Kcllw H , Neumann D, Lurie A ( 1999). Cysts and c"sric lesions of the mandible: clinical and radioiogk-histopathologic review. RndioGrnplJics 19: 1107- 11 24 .
  
  C\ost wit h no dcnral association:
  
  ~.n· _ OKC, simple bone cysr.
  
  -
  
  I~ars
  
  - ameloblastoma, mct3stases o r myeloma.
  
  
  165
  
  Answer 96
  
  eNS. Head and Neck Imaging
  
  ANSWER 96 Observations (96a)
  
  Practica l tips
  
  This occipiwn1cnral radiograph shows a large calcified lesion in the right fromal sinus. The lesion has \'cry well defined margins and a slightly lower density cemre. No fluid is seen within the sinus and there is no evidence of local bony destruction. The appearances arc those of a frontal sinus 'iYory Ostt-oma', h 'ol)' osteoma.
  
  • "Yhen multiple osteomas arc seen always consider Gardner's syndrome and irwcsligarc the fami ly hist and tor the presence of colonic polyposis. • Paran:lsal sinus osteomas may cause local pressure erosion. A coronal CT rcconsmlCtion (96c) dcmonstrJtcs a left cdulloid sinus osteoma eroding: the lamina papyracc;l and extending into the left orbit. Background sinus inOammatory changes 3« present.
  
  Discussio n
  
  Further management
  
  Osteomas an:: benign tumours of membranous bone. These arc round, well defined lesions of bony density that arc usually found incidentally. They arc commonly found in the paranasal sinuses, p:l1"ticularly the frontal and ethmoid sinuses. Other coml11on si tes include the calvarium and mandible. A CT coronal reconstruction (96b) in the same patient confimls that the lesion lies within the right frontal sinus.
  
  Incidental finding requiring no further management
  
  Diagno'sis
  
  96b Coronal CT reconstru ction demo nstrating a right frontal sinus osteoma.
  
  166
  
  eNS. Head and Neck Imaging
  
  Ca se 97
  
  lid fem ale presented with w.l loss; and an episode of mthsago.
  
  167
  
  Answer 97
  
  eNS, Head and Neck Imaging
  
  ANSWER 97 Observations (97a . 97b) Selected MR images ofrhc brain - FLAIR axial and T2 weighted sag;!!al - arc presented. These scans show multiple foc:!] O\'oid signal abnormalities in the pcri\'ctltricubr white matter, which 3rc orientated perpendicular to the long ax is of dIe ventricles. Further lesions invoke the corpus callosum. No associated oedema. No evidence of hydroccph:tlus. Given the clinical derails, it is likely that the patient has multiple sclerosis and is currentl y experiencing optic t~Cllli t is.
  
  fi ndings on MRl with se\'eral aetiologies. Maki ng a specific diagnosis of dem),clination is often nor possible from imaging alone though multiple IcsiOOl. il1\'ol\;ng lhe corpus callosum arc characteristic for 1.'15. Coexisting lesions in the brainsl'cm, cerebellum and spinal cord also increase specificity. Be wary of making a conclusive diagnosis of M5 M RI unless feat ures arc highly specific and there is J. supporting history - the consequences for the pa arc significant. O ften , one can only offer a ditlcrcn diagnosis for ' UBOs' that includes ,'v15.
  
  Diagnosis Multiple sclerosis (MS).
  
  Differential diagnosis Of white matter lesions on MR]; Acute disseminated cnccphalolll),clitis (ADEM ). Vasculitis. Ischaemic disease (97c).
  
  Further m anagement M RI of the spi nal cord is freq uently also undertak complete assessment. Though MRl findings may be _ suggesti\"e , careful neurological assessment is req uira. correlate the imaging findings \\~th clinical and Jahora. findings before making the diagnosis.
  
  M igraine.
  
  Further reading
  
  Ncurosarcoid.
  
  Runge VM, Price AC, Kirshner H5, ct at. ( 1986). evaluation of multiple sclerosis by magnetic reso imaging. RfldioGrnpbiC!6: 203- 212.
  
  Discussion This is the mOSt common chro nic demyelinating disease and'is characterized by multiple lesions spread in tillle and space. T ypically, it has a remitting /rdapsi ng course. The onset of symptOms is usually in the 3rd-4th decades and thcre is a sli ghtl y increased predominance in remales. I ncrcascd prevalence is nOTed in areas of temperate climate. Clinkal presentation is with focal neurological signs, com monly including optic neuritis. Imagingfcaru resare: Lesions arc classicall~' ]x:ri\'entricular in location, O\'al in shape, with their long axis perpendicular to the lateral ventricle walls - 'Dawson's fin gers'. Common locations for plaques include peri\'entricular white matter, corpus callosum, internal capsule, centrum scmiovale, optic ner'I'C/ tracts, cerebellu m. Lesions on the inferior aspect ofrhe corpus ca llosulll arc characteristic. • Acute lesions can ha,'c mild surrounding oedema and can enhance with com raSt. • Chronic lesions ha"e no mass effect/ oedema and don'tcnhancc. • Lesions arc hyperintense o n T2 , hypo/ isointense o n
  
  Tl. Mull'ip1c sclerosis is also the most common demyelinating process aflccting the spine - the cervical spine being most frequently affected. It is characterized by plaques orientated alo ng the axis or the spinal cord. Funher clinical e\'3lua[ion im'ol\'es: Lumbar punctme with CS F analysis for ol igoclon:tI bands. Electrophysiologic;).1 studies.
  
  Practical tips Focal while matter signal ;).bnorm;).lities in cerebral white marter (sometimes referred to as unidentified bright objects - UBOs) are common incidental
  
  168
  
  97c Sing(e axial MRI FLAIR image shows periventricular high signal with scattered high signal w hite matter foci. Appearances are of related small vessel disease.
  
  n.
  
  eNS, Head and Neck Imaging
  
  Case 98
  
  169
  
  ANSWER 98 Observations 198a, 98b) This later:)l image of the skull (98a ) shows elongation of the jaw (prognathism ), frontal bossing, cnlargcl1lt:nr of the frontal sinuses and t hickening of the calvaria. There is marked enlargement of the pituitary fOssa with e,·idcncc of expansion bur no erosion. Selected sagitt;'l] 1' 1 weighted illlages (98b ) of the br.l.in connml the plain IiIIll findings of enlargement of the lTontal sin uses, frontal bossing ;'ind thickening of the c;l]"aria. There is a mass lesion within the pituitary fossa with appearances consistent with pituitary macroadcllol1la. Enlargemen t of the tongue is also noted.
  
  Plain radiogr:tphic image findings :l.rc: Enlargement of the sella (98c). Mandibular enlargemcnt. Increase in sizc ofrhe frontal sinuses with promin afthc supraorbital ridge. Enlarged hands with spade -like appearances o f the terminal phalanges (98d ). Thickening of the calvaria. Dur:tl ect:l.sia :l.nd posterior \"crtebr:l.1 body scallapln:Increased heel pad thickness( >25 tllm) (98c). Prcm:lture ostcO:lrthritis (OA).
  
  Pract ical tips Diagnosis
  
  Normal dimcnsions of thc pitu itary fossa on lateral films arc a length of < 15 mill and height of < 12 mm.
  
  Acrorncgal~' .
  
  Discussion
  
  Further management
  
  Excess growth hormo ne secretion by the anterior lobe of the pituitary gland results in a variety of nlllsculoskclet;ll abnOfm:l.litics.
  
  !\'ianagcmcnr of this condition is both medical (soma statin/brollloc riptine ) and surgical ( trallssphe n hypophysectomy).
  
  98d Ra diogra of the hand shows marked soft tissue en largement givi ng it a sp like appeara nc:e. In addition th is w iden ing of terminal tufts.
  
  g8e Magnified image from plain skull radiograph shows enlargement of the sella.
  
  98e Soft tissue lateral radiograph demonstrates thickening of the heel pad, which measu res >25 mm in thickness. .
  
  170
  
  eNS. Head and Neck Imaging
  
  Case 99
  
  -. weighted axial (top left). thin section T2 weighted axial orbits (top right), axial and coronal T2 weighted I MR images.
  
  171
  
  Answer 99
  
  eNS. Head and Neck Imaging
  
  ANSWER 99 Observations (99) The axial images of the orbit show fusitorm e nlargement of the posterior righ t optic l1en'e, which also il1\"okes both sidcs of the optic chiasm. The coronal scan confirms thickening of the posTerior aspect of bOTh optic nen'es. Axial T2 weighted images also show a round tixus of high signal in The righ t cerebellar hemisphere and a second lesion in the posterior aspen of right midbrain that produces convexity to the margins of the cerebral peduncle. The appearances arc likely to indicate optic nerTe glioma involving the chiasm and both opril": nen'es along with hamartomas in the cerebellum and midbrain due to neurofibromatosis type I. No cutaneous neurofibromas arc seen on these images.
  
  Diagnosis O pTic chiasm/nen'e glioma due 1 ( N F l ).
  
  LO
  
  neurofibromatosis type
  
  Differential diagnosis ror optic nerve thickening: • Optic nen'e glioma - 80% under 20 yean, variable enhancement, calcifjcation I""Olre, buckling of nen"e, oflen asymptomatic. • 'Meningioma of optic nem: - middlc aged women, 'tramtrac k' enhancemelll, calcification in 20- 50%, straight nen"e, visu al impairment early. Sarcoidosis. Multiplcsclcrosis Lymphoma, kukaemia and metastatic disease. Intracranial hypertension - enlarges the perineural CSFspace.
  
  Discussion Optic nen'e glioma typically presents in childhood, only 20% manifesting beyond the age of 20. Rclati\"cly slow growing and benign ill children, lesions presenting in adults often show morc rapid malignant growth with intracranial spread. T hough often asymptom:uic, prescntaTion can bc with visual loss and strabismus. BHater:!1 tumours herald NF l . The tumour causes fusiform or tubular cnlargement of the optic nen"e sheaTh complex and shows variable enhancement with IV contrasT. The majority of lesions occurring in the orbital portion of the nen·e do not extend
  
  172
  
  intracranillly. Some optic nen'c gliomas haxc ext!: associated thickening of rhe surrounding meninges, Ie ar.lchnoidal hyperplasia, which is often seen in patients 'Il NF - on T 2W M IU , Ihis is seen as:I ccntrallow-si tumour surrounded b~' a higher-signal rim that call M like a dilated perineural CSF space. Approximately 25% of paticlHs with optic glioma NFl and it is one of the diagnostic critcri:l listcd for condition, of which two or more arc required: six or IDI 'cafe-au -lait' patches; twO or morc Lisch nodules; rn
  
  p.
  
  more neurofibromas or one plexiform ncurofibr axillary freckling; optic glioma; bone dyspb.si~ pseudarthrosis; fint degree relati,'c with NF l} In this the coexisting lesions in the cerebellum and midbrJin one to the overall diagnosis of NF l . These hig h T2 si~ toci arc seen in the brainsl'em, basal gangl ia, cerci peduncles, cerebellum and the stLprarenrorb.1 whiTe They arc usually thought of lS I lnd often decrease in size with . to as[f(xyrom~ in the cerebmm, and telling the difference ma~' be i enlarges O\'er time or shows possibility of astrocytoma must be considered. choroid plexus calcification and hydrocephalus aqucduct stenosis arc o ther intrncranial features . NFl is sporad ic in 50% and autosomal dominant' (chromosome 17).
  
  Practical tips Tl weighted post-contraSt scans of the orbit sh be performed with fut suppression in view of the adjacemorbitai fur. Meningioma is the main differential diagnosis for optic ncn'e thickening and the features liSTed abomay help dis tinguish the tWO. Whenever NFl enters the diflc rential for anv ~ I""Oldiological study, al\\':lYs check for evidence of cutaneous nodules that 'clinch thc diagnosis'.
  
  Further management Treatment depcnds on the size ofnrl1lour along \\ age and gener:!] condition ofthc patient. Options surgcry, radiotherapy and chemotherapy.
  
  eNS. Head and Neck Imaging
  
  Case 100
  
  173
  
  Answer lOa
  
  eNS, Head and Neck Imaging
  
  ANSWER 100 Observations 1 11008) Axial T2 weighted image demonstrates high signal in the lateral aspect of right medulla. This is bright on dIe DWI
  
  indicating restricted water molecule: diffusion probably from cell swell ing due to leutc inf:lfction. Together with the clinical details, findings arc consistent with latcrallllcdllll~' syndrome due to in furction in the territory of the right posterior inferior cerebellar artery ( PICA).
  
  Observations 2 (100b) These axial and coronal 1'2 weighted images show a focus ofincrcascd signal in the right side of the midbr:l.in. Then: is no associated mass dice! and the lesion lies in the region of the red nucleus. The clinical symptoms arc compatible with 3n infurct affecting the red nucleus and causing Claude's syndrome.
  
  Diagnosis' Right PICA infarction (Wallenberg's syndrome).
  
  Diagnosis 2 Ri ght midbrain infarction :lflccting the red nucleus (Claude's syndrome ),
  
  Discussion There arc various specific panems ofbrainstem infarction, oftell having unusual eponyms. Othe rs include Weber's, Nodlnagel's, "
  100a High signal in latera l aspect of ri ght medulla.
  
  174
  
  as thrombosis, infuretion can result from dissection of ,'crtebral artery, Claude's syndrome is due TO a lesion of the red nu of which inF.lrction is one example. T he consequences ipsil:tteral oculomotor pals}' and contralateral tremor araxia,
  
  Practical tips • T hese arc examples of comparati\'ely rare and \'e~ specific infarcts but they do make for an interestin.... test ofne uroonatomy understanding! • OWl depicts reduction in Brownian motion of\\ molecules, Cytotoxic oedema in acute infarction produce this and present high signal on the 0\\1 scan. It is ,'cry sensitive, depicting infurclS JUSt 30 or SO from onset. • The DWI image also has inherent T2 weighting 'shine through' ), that is, thc signal is a combin.lD ofT2 weighted and reduced diffilsion , As such. ma~' not distinguish an older infurcr (T2 hyperi from a recent one (reduced diffusion ) as both \\ appear bright. An alternati\'e dep i~tion that can diflcrentiate acute and nonacute infurcts is the apparent diffusion coefficient (ADC) map. Th~ no T2 component and shows reduccd diffusion acute infurct) as low signaL An older infarct th.Jl: also bright on DWI b}' virtue of T 2 'shine th ~ will be bright on AOC. Ischaemia is not the on l ~' cause of reduced di Others include seizurc, trauma, h}'\>oglycaemi..l. abscess.
  
  Further management DWI is !\lore scnsiti"e in thc carl}' d etection of ' when compared to CT. However, when throm being considered, is more than adequate to haemorrhage alone.
  
  cr
  
  100b Right midbrain high signal.
  
  eNS. Head and Neck Imaging
  
  Case 101
  
  101 C1r-old male presented with _lIlrrollcd epilepsy_
  
  175
  
  Answer 101
  
  eNS, Head and Neck Imaging
  
  ANSWER 101 Observations 1101a. 101b) The axial CT image of the [)",in ( lOla) showsscvcliIl small calcified lesions in the subepcnd ymaJ region of the txxIy of the lateral \·cntricks. The T2 wciglucd MR im:agc of the brain ( 10 I b ) conlirms sc\'cml small subcpcndYlllalnodulcs with associated low signal representing calcification. These appearances arc consisTcm with slIbcpcndymal hamartomas.
  
  There arc abnormal widened gyri in the left paricml lobe and right frontallobc seen on Ihe MR image, which arc likely to indicate cortical h:lI11lrtom3S.
  
  Diag nosis Tuberous sclerosis.
  
  • Distinguishing lubcrs/ h:lrnarto111as from giant c astrocytomas: tubers and subepend ymal hamar! C:lll show some enhancement on MRI but CT is usually sensith·e enough to show this. Because cell astrocytom3S show more enh:lllcement, this be :lppreeiable on CT a.nd should a.rouse suspici
  
  Further management Tuberous sclerosis has a high mortality with 70 before the :lgc of 24 ~'ears. A multidisciplinan approach is required with follow -up imaging im Mltl brain, renal ultr3Sonography ( monitoring myolipom:ls :lnd looking for re nal cell c:lTcino cchocardiography (50% of patients ha\·c rhabdom~
  
  Discussion TuberOLis sclerosis is an inheri ted autosomal domin:llll disorde r of the neuroectoderm that is characterized bv mul[isystem abnormalities. The classical triad of features arc mental retardation, seizures and adenoma scbaceum. CNSfcarures: Subepcndymal hamartomas - most commonfy seen along the ventricular surfuce of the caudate nucleus. r...l ultiple small sy.bependymallcsions which calcify in 80%. Figure I Ol c shows another case where nodules have not calcified, but produce :I 'w:lI'y' border to the ,·entriclcw:llls. Corric:ll tubers - appearances arc of large widened :ltypical gyri with reduced attenuation ce!lues on CT. T he)' :Ire usuall}' multiple and can show rim calcification in 50%. Heterotopic grey matter is13nds - these :lppcar as 13rge hypodcnsc focal islands of tissue \\ithin the cerebral whiremattcr. Giant cell astrocytOlll:ls - occur around the foramcn of J" lonro, and can cause hydrocephalus. J\-blign:l11t potential is low. Multisystem illvokement: • Ocular - ocular phakomas, oplic nerve gliomas. • Renal - angiomyolipoma, cysts, incre:lsed risk of re n:ll ccllcarcinoma. Respiratory - Iymphangiomyom:ltosis-like features with cystic lung disease, spontaneous pneumothoraces ~lnd chylothorax. Cardio,"asctdar - rhabdomyoma, aonic aneurysms. Skin - :ldenoma sebaceum ( red/ brown small flat skin lesions distribUled sylllme tricall ~' o\"er nose and checks), sh:lgreen p:ltches, ash lcaflcsiolls, subungual fibromas.
  
  Practical tips The phakornatoses ( neurocutaneous disorders), o f which tuberous sclerosis is an example, make great eX:l111 G 1SCS because of the multitude of radiological signs to 'piece together' - know them well!
  
  176
  
  Further reading AJtman Nit, Purser RK, Post MJ ( 1988 ). Tuberou. sclerosis: characteristics:lt CT and J\'IR imaging. Radi%gy 167: 527-532.
  
  101c
  
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  . /~-" >"'"'" -.~ ,_
  
  . U_'~ 4' ~ ~
  
  . ,..,
  
  i,
  
  ,
  
  '.~.',; '. ' ,
  
  --_,
  
  !
  
  ,
  
  f f:.",.
  
  eNS, Head and Neck Imaging
  
  Case 102
  
  177
  
  Answer 102
  
  eNS, Head and Neck Imaging
  
  ANSWER 102 Observations (102a) Axial 1'2 (left) and gradient echo T2 (right ) images dcrnonstr,nc a lesion in the lllcdi:ll right Icmporallobc with a vcry low signal rim representing hacmosidcrin. This is ch:u :lctcristkally more prominent on the gradicm echo T2 sequence with 'blooming' artcf.tcr. The cenlre of the k-sion shows T2 hypcrimcnsity and the o\'crall shape is round. The fcamrcs are consislcnr with a C:l\'crnoma.
  
  Diagnosis Ca\'crnoma (ca\"ernous angioma or ca'"crnous mal formation )"
  
  Discussion
  
  where flow ,ooids with complcx flow patterns aTe Figurc I 0 2c demonstr.ltes an example o n an axial _ weightcd image with an AVM in the right frontal producing the characteristic (';l1lglc of now voids. C:wernous malformations (ca,"ernomas) arc thin walled sinusoidal vessels representing congenital hamartomas. Unlike other vascular malforl11 atio~ then: is no brain parenchyma Ixtwecn the \"3sculM spaces. The appearancC$ on CT arc rathe r nons but i\'IIU shows vcn' characteristic features, as illustrated. The celltral high signal represe nt's lllcthaemogiobin and the outer low signal rim haemosiderin. They presem with focal seizures (W small parenchymal haemorrhages. Venous angiomas arc uS ll all~' asymptomatic and anomalous ,"eins that dmin rhe normal brain. Classically, an enhancing stellate venous mallo is seen extending TO the ventricular or conical
  
  Vascular malformations arc a common cause of parenchymal brain haemorrhage and should be excluded when young patients present with spontaneous haemorrhage. They de,"elop from congenitally abnormal \'ascular connections, whieh may incrcasc in size. with rimc. R..1diological features of\'ascular malformations arc: Practical tips • AVMs typically h;1\'c ;t tangle oflow-signal flo\\ Arteriovenous malformations (AVMs) afe the most common type and arc eucntially an abnormal ":\ on ,\-IRI best seen on T2 and PD weighted ima_ • 10% of AVMs develop an :lssod:J.tcd :lllclll)'Sm OJ( collection of an cries connected dircctlv to ' "cins with no imen'ening capillaries ( I 02b). Thc' vast majority alw:l\'S look. for this. • afe supratentorial. On CT thc)' arc of mixed density Gradient ccho T2 is an excellent technique for and mav have calcifications. Enhancement is also secn. idcntif)'ing haclllosidcrin 011 MIU, which is oltca !-Iowcver, the classical appearance is found on MRl in vascular llultOrm:uions.
  
  102b Large right cerebral AVM demonstrated on a CT cerebral angiogram.
  
  178
  
  102
  
  eNS. Head and Neck Imaging
  
  idcrin will also be seen in an old haemorrhage itwoking an underlying vascular ation. Figure l02d dcmonSlrarts an old
  
  KC~sari ly
  
  age in the kft imernal capsule/thalamus .. would be cas}' to confuse this with c;;wcrnoma the axial image. Howe"er, all old haemorrhage to form ::I slit-like cavity unlike the rounded of cavernoma, and the coronal scan illustr.n cs diffe rence. NOte also the local \'olume loss due to
  
  Case 103
  
  gliosis resulting in some enlargemen t orlhe adjacent [cft [atcra] vcntridc.
  
  Further manageme nt Often these arc aSYffipromatic and call be monitored with imaging to 3SSCSS change in growth. There is, however, an up to 2% risk of bleeding and surgcry/slcrcoractic radiosurgery arc treatment options.
  
  03 Axial and coronal T2 weighted scans of orbits (left/middle) and a 'time of flight' MR angiogram of the rluacranial circulation !right),
  
  179
  
  Answer 103
  
  eNS, Head and Neck Imaging
  
  ANSWER 103 Observations (103) T2 weighted images demonstrate dilatation of the left superior ophthalmic vein \\'ith presence of flow \'oid and no focal compressi\'e mass lesion. The "time of flight' MRA demonstrates normal arteri:!l intracranial anatOIlW \\'ith signal in the dilated left ophthalmic \"cin indicatil;g f.m flow. The find ings arc in keeping with a kft caroricoC;ll'crnousfisrub.
  
  Diagnosis CaroriCOC3'I'CrIlOUS fistub.
  
  Differe nti a l diagnosis For superior ophthalmic rein distension: • Ca"ernous sinus thrombosis. Superior ophthalmic "ein thrombosis. Pseudotllmour. Graves' disease. Obstructi,'c orbital mass. Discu ssion Fistulous communication betweell the internal carotid artery and the ca"ern?us ~inus can ariS<,' secondary to head trauma or rupture 01 an mternal caroud artery aneurysm, The condition olien occurs spontaneously, however, due [0 atherosclerosis. In addition, fisllllae can occur \\ith dural branches of the external carotid artery. Orbital bruit is found in - 50% of patients due to turbulent arterial blood flow, T he increased arterial pressure in the l'e!lOllS system and orbiral "cin congestion result in the symptoms of: • Pulsatik proptosis. Chemosis. • Reduced visual acuity due to impaired retinal perfusion - scvere/ rapid visual loss requires angiographic im'cstigation and listula closure as an emergency (0 preserve function.
  
  103 Dilated left superior ophthalmic vein.
  
  180
  
  Cranial nerve palsies - most commonly 6th and 3rd nervcs. This is thought to be due to either impaired \"cnous drainage of the ncn"c or direct compression Ilcr'I'CS bv distended ' "cins. Contral~tcraJ srmptoms arc secn in - 10% duc to [h~ presence of conllcniOtls between the two GI\"ernous sinuses. Imaging features on h'IRI arc: • Dilated superior ophthalmic vcin with flow ,·oid. Enlargement of the ea,'ernous sinus. • Swelling of the extraocular muscles, which can rcsuli in limited ere mo,·ements. Angiograph~' shows c;\fly opacification of the supe ophthalmic rein when contrast is injected into intc carotid artery. Earl~' opacification of the veins C011llTI eating with cavcrnous sinus may also be noted.
  
  Practical tips Early radiological changes arc of enlarged ocdemat extraocular muscles and dilatation of the superior ophthalmic I·cin. More chronic changes arc of eniargeillent of the superior orbital tissure and scll:ar erosion. Clinically, pulsatile exophthalmos susgeslS this condition. Further management When imaging features of a caroticQCa"ernous fistu.b associated with decreased "isua! acuit,)" cmergency mcnt is required to relieve intraoclllar pressure.
  
  eNS, Head and Neck Imaging
  
  Case 104
  
  181
  
  Answer 104
  
  eNS, Head and Neck Imaging
  
  ANSWER 104 Observations (104) SeleCTed '\'IR images ofthe brain show a large lesion in the
  
  cerebellum, which is predominantly cystic in narure (hn>erimcnsc on T2 and hypoimensc on Tl weighted images). Following IV colltrnsr there is cnhancemcm oftlle
  
  \\':1.11 of the cystic component and an associan:d solid nodule posteriorly. S.1gittal image shows some filllncss of the btcral
  
  Typical appeara nce is of a well defined cystic I containing fluid of CSF density with a solid perip nodule, showing uniform a"id enhancement. Th~ commonly haemorrhage or necrosis in the solid n but it rarely calcifies. In up to 30% of cases the lesions be emird)' solid wilh no cystic component.
  
  "cmridcs and ObStTUcri\'c hydrocephalus is likely with
  
  Practical tips
  
  compression at thc level of the 4th '·(lUride.
  
  When forming a differential for the posterior fossa mass with enhancing peripheral nodule, the foil
  
  Diagnosis
  
  generalizations apply: • Children - piloc)'tic astrocytoma>
  
  Hacmangioblastoma.
  
  Differential diagnosis • Juvcnilc pilocytic ;lstrocytoma - can be "cry difficult to diflcrentiatc from a haeillangioblastonll. This is the mOSt COlllnlon infratentorialtulllour in children. There is an association wi th ncurofibromatosis typc I , Lesions arc of an ident ical appearance, with a predominant cystic component and enhancing solid peripheral nodule. Metastasis. Arypical mcdulloblastoma,
  
  Di scussion H aemangioblastoma is a benign vascular rumoull aflccting the CNS. It is the most common primary infratemorial nUllour in adults. The majority (80%) present in adults in the 3rd-6th decades, although there is an association '\ith "on H ippel- Lindau (V HL), which sees these tumours presenting in childhood and in this case may be multiple.
  
  hacmangioblutoma. • Younger adults - hacmangioblasroma most com • Older adults - consider cystic mctaS[3sis
  
  Furth er management When haemangioblastoma is suspected, do n't forg:... raise the possibility o f VH L (4-20% of haemangiob occur in the COllleX I of VH L and multiple lesi diagnostic ofVI-IL). VH L is an autosomal dominant inherited condition characterized
  
  spectrUl:n:~:o~rl:~"'::~m:~o~"~,~,:i~~::~~~j~i~:~:
  
  blastomas, develop a cardiac pheochromocytomas, pancreatic I cell mmours and haemangioblaslomas, li"er and retinal angiomas.
  
  104 Enhancement of the solid posterior nodu le on post-gadolinium im ages.
  
  182
  
  eNS. Head and Neck Imaging
  
  Case 105
  
  183
  
  Answer 105
  
  eNS. Head and Neck Imaging
  
  ANSWER 105 Observations (105) The selected images demonstrate diffuse cerebral atrophy exceeding that cxptctcd for the patient's young age. There is:I large focal area of signal abnonm.liry in the central pons Ih:1I is hyperintense on T2 weighted and hypointcnsc on TI wei glued sc:ms. 111crc is no associated mass effect. The signal changes would fil with central pontine myelinolysis and the clinical dCt3i1S and cerebral atrophy suggest that chronic alcohol abuse may Ix the underlying cause.
  
  Clinically this results in pseudobulbar palsy, terraplc~ cOll\'uisioIlS, acute confusion and progression to comL is mOSt commonly confined to the pons but changes also be secn in the basal b>;lnglia, caudate, thalamus suocortkal white matter. Radiological presentation is fairly wdl defined low attenuation lesions on CT in central pons. On M IU , lesions aTe oflo\\' intensity on and high signal imensity on T2 imaging.
  
  Practica l tips Diag nosis
  
  Differenti al diagnosi s
  
  Standard MR/CT im::aging (::an be normal for up to days after symptolll onset, lagging considerably behind clinical presentation. Rest ricted diffusion on D however, is found wirhin 24 hours.
  
  • Pondnc glioma. • Inf.,rcrion.
  
  Further management
  
  Central pontine myelinolysis.
  
  Di sc ussion Central ponline myelinolysis res ults from the des trllction of m~'c1in sheaths, dassicall~' in patients with rapidly corrected hypo n:ttracmia. Cases are reported in patients with: • Chronic alcohol abuse. • Chronic liver d isease. ' • $c"ere m::alnutrition. • Wilson's disease, chronic rell::al fitilure, diabetes, acute m)'clogcnous leukaemia . In the c::ase demonstrated there is diffilse glob::al (erebral ::atrophic ch::ange suggestive of chronic alcohol abuse.
  
  • Early MIU appearances can be similar for a b::asibr inElrcr and M RA (::an be useful to look for :lrteri,;d thrombus. • Sc::arch for underlying cause if not readily app::arcOL
  
  Furth er rea ding Ruzek KA, Campe::all NG, Miller GM (2003 ). Earf!; diagnosis of central pontine myelinolysis with diffusion-weighted imaging. American JOllnHlJ Nmrorndiolo!JY2 5 : 210-213. Stadnik nv, Demaerd P, Lurpae rt RR., It nl. ( 200.; Imaging tutori::al: differemi::al di::agnosis of bright lesions on diffusion-weighted MR images. RadioGraphies 23(1): e7.
  
  105 Focal area of red uced Signal on T1 weighted (left) and increased signal o n T2 weig hted (right) images.
  
  184
  
  eNS, Head and Neck Imaging
  
  Cases 106, 107
  
  -old fCIll:a1c patient prescnted clles.
  
  185
  
  An swer 106
  
  eNS, Head and Neck Imaging
  
  ANSWER 106 Obse rvations (106a )
  
  cr
  
  This non contrast enhanced image demonstrates a large midli ne mass extending into the 3rd ventricle, which hlS both solid :lIld cystic compOllcnrs with central calcitication.
  
  There is dibtation orthe temporal and anterior horns of the "cntridcs but normal :tppcarancc to the 4th ventricle findi ngs arc in keeping with obstructive hydrocephalus at the lc,"el of the. 3rd ventricle secondary to a mass lesion. The mixed density and calcification arc \ '(1)' suggcstive of a craniopharyngioma and M IU is suggested to further e"aluate.
  
  Diagnos is Craniopharyngioma.
  
  Differe ntial diagnos is
  
  or suprascllar/intrascllar mass lesion:
  
  c,wcrnolls sinus ( 3, 4, 53 and 6 ) and hypopitui resulting from compression of normal pituitary tissue Compared to craniopharyngioma, pitu itary adenomas arc predominantly solid and show morc i uniform enhancement. Calcification is rare, craniopharyngioma. Suprasellar ex tension produca 'snowman' configuration due to w:lining at the I the diaphragm sellae. Figure I 06b shows prc- and contrast Tl weighted images of a pituitary macroa with such features. lbthkc deft cyst is the other main diAcrcntiai di.a_ wi th 70% in\"olving sellar and suprasellar regions. benign cySt arising from remnants of Rathke's pouu can be differentiated by the following features' regular and ovoid shape, smaller size ( <2 !TIl ), cystio;: 110, or minimll cnh:lllccmcnt ofthe thin wall.
  
  • Pituitary macroadcnoma.
  
  Discussion
  
  • Craniopharyngioma • R.1thke cleft cysr.
  
  Craniopharyngioma account for - 4% of int neoplasms, with a slight predominance in males, peaks ofincrcascd incidence: • Firstly in children in 1st-2nd decades, Wh("fC account for 50% of suprasellar turnouTS. • Secondl y in adults in 5th - 6th decades.
  
  Other masst:s in the sellar region include meningioma, metastases, chordoma and intenla!l'arOlid mer)' aneurysm. Pituitary macroadenor{KI is the most common lesion involving the sellar and suprasellar regions. Microadenol1las (20%) an: defined as being less [han 10 mm in size and usu:llly present with sympl"Oms due to hormonal secretion. Macroadcnoll1as (80%) measure over 10 mill in size and arc usually endocrinologieally inaeri,'e. The~' present with symptoms secondary to mass effeer such as hydrocephalus, bitemporal hemianopia from optic c hiasm compression, involvement of cranial nerves tr:wdling in the adjacent
  
  These arc benign tumours arising fro m rem 111thke's pouch. They grow from a suprasellar ori_ the base of the 3rd vemricle. lmr:lsel lar extension in 2 1%. Involvement of the bon)' sella can be seen radiographs and o n CT bOlle window imag("S demonstrate appearances or J-shaped sella, enl and then ultimatel y erosion and destnJcrion.
  
  106b Pre- and post-contrast T1 weighted MR sagittal images demonstrate a large pituitary adenoma with uniform enhancement with contrast and with no cystic elements or signal voids to suggest calcification.
  
  186
  
  106
  
  eNS, Head and Neck Imaging
  
  IT appca r,lIlccs arc 5ho'l\'11 in this casc \\;th
  
  ,,:ystic clements and calci fi cuion (which is -90% of cases). Following comrasl there is amen! of the solid compOllcnrs and the CYSt
  
  .\tRI findings reflect CT features with a cystic InCn! following fluid signal (although high >my be seen on Tl weigh ted images depending
  
  Jttin/ blood content). The solid componcm is o n T 1 and shows cnhancclllcllI with
  
  Practical tips Look for the complications of masslcsions in this area: • H ydrocephalus :lIld optic chiasm compression. • Lateral extension to invoke thc cavcrnous sinus ( seen in up to 10 % of pituitary macro~dcnoma.s ) , which can lead to thrombosis :lIld cranial nervc palsy. Lateral extension beyond the lateral wall of the internal carotid is rare wilh Rathke eleft cySt :lnd may help diflcremi:lte it frolll the other two conditions listed.
  
  Fu rthe r management +/- posropcrath'e radiotherapy.
  
  Treatment is surgical
  
  106c shows '1'1 sagittal pre- and post-IV contrast d T2 axial and 53gitt.ll images (left). These Lu-gc su prasellar lesion with intnsclb.r extension. solid and C}'stic clements wj th enhancement of component'S and cyst wall. Complications of 'C hydrocephalus, pituitary st;}lk and optic chiasm n can be appreciated.
  
  .I
  
  Further rea ding Choi S H , Kwon 81, Na DG, ct al. (2007). i'imitary adenoma, craniopharyngioma, and Rathke cleft cyst ilwolving both intr.tsellar :lIld suprasellar regions: ditlcrcntiation llsing 1-Ht!. CIi"icn! Radio!ogy62:
  
  453-462 .
  
  106c T1 sagittal pre· and post-IV contrast and T2 axial and sagittal images demonstrate a large suprasellar lesion w ith solid and cystic elements with enhancement of the solid components and cyst wall. The lesion is causing obstructive hydrocephalus, pituitary stalk and optic chiasm compression.
  
  187
  
  An swer 107
  
  eNS. Head and Neck Imaging
  
  ANSWER 107 Observat ions (107a) T1 sagittal and T2 axial and coronal images of the brain dcmonsu':uc: complete :agenesis of the corpus callosum \\ith no callosal [issue identified. There is increased separation of the 13tcral \'cmodes on the a.xial images crc;uing a 'b:J.t's
  
  wing' appear-uKe. Coronal image demonstrates clc\'3tion of the 3rd ventricle .
  
  Prenatal detection can be made by US investigatic.. Diagnosis cannot usually be made before 22 wee gestation. Equivalelu image findings arc of: • Absence ofthe septum pdlucidum. • Dilated/elevated 3rd ,·cnrricle. • Enlargement of the occipitJI horns of the lateral ,·cntrides.
  
  Di agnosis
  
  Practica l t ips
  
  Agenesis ofthc corpus callosum.
  
  Ditlcrentiating bcrween dysgenesis and ischacmie da is done b~' de("ermining which parts of the corpus call arc abnormal. With ischaemie injury the rostrum nill present but :ltrophic.
  
  Discussio n The corpus callosum usually develops by 20 \\'ccks' gestation. T he genu and the body of the. corpus callosum develop first and the posterior bod)' and spleniulll develop b ter. The rostrum is the last part of the corpus callosum TO develop. Idcntil}'i ng the parts of the corpus callosuill present call help to diflcrcntiatc octwccn dysgenesis (absent genu/splenium ) and destruction, most commonly dlll~ 10 ischaemia (genu present but may be atrophic ). Corpus callosal agenesis is usually associated with reduced intellectual function and can be associated with a varier\' of c;NS abnormalities in~ludin~ hydr~ephal.us, mid[il~e itpoma , Oandy- 'Valker cySts, mterheilltsphenc arachnOId cysts, neuronal migration disorders and Arnold- Chiari malformation. Classical imaging appearances on CT/M IU arc with: ' Bat 's wing' appcar""Jnce of the lateral ,'entriclcs due to parallel lateral "entricles with marked separation ( 107b ). ' High riding' 3rd ventricle - can be seen at the b 'd of the Jateml ventrieles. Enlarged foramen of t-.'ionro.
  
  Fu rther ma nage ment Somctimcs this fin ding is made incidentally on imaging in later lire and no fu rther in\·estigatiOD required.
  
  Fu rther reading Babcock OS ( 1984 ). The normal, absent, and abno corpus callosum: sonographic findings. Rndioio..!!! 15 1: 449-453. Oa\'idson HO, Abraham R, Steiner RE ( 1985 ). of the corVllS callosum: magnetic resonance Rndiolog., 155: 371 - 373.
  
  107b Axial CT images of the brain demonstrating 'bat's wing' appearance of the lateral ventricles in a with agenesis of the corpus callosum.
  
  188
  
  eNS, Head and Neck Imaging
  
  Case 108
  
  189
  
  Answer 108
  
  eNS, Head and Neck Imaging
  
  ANSWER 108 Observations (108a. 108b) Axial CT images ( 1 08a - axial C T scaJl at skull base with IV contrast, seen on bone and soft tissue windows) oflbe brain demonstrate a poorl}' defined enhancing lesion in the jugular foramen, which is extending superiorly to in\'ol\'e
  
  rhe middle car and is causing permeative erosion aflbe pClrous temporal bone at the juguhlf foramen. The T2 weighted MR image ( l OSb) confi rms this mass lesion, which appears hrpcrintcnsc with serpiginous flow ,ooids indicating vascular flow.
  
  Diagnos is
  
  destruction seen with skull base metasuses. Vasculu ,ooids and foci of haemorrhage are beller demonstnt MRI - flow produces black signal I"oids and haem produces hyperintensc foci on T I weighted images combination of black holes and white dots leads to called 'salt and pepper' aplXlrance commonly dcscril Angiography is sometimes required for smaller tha t don't demonstra te the classical imaging appe and rel"eals a hypc r\'ascular lesion with a dense mmourstain. The re is a small ris k of maligna nt transfo rmaci in -2% of cases.
  
  Glomus jugulorymp::lIlicum tumour.
  
  Practica l tips
  
  Description Glolllus jugulare rulllOUrs afC paragangliomas :lIld afC rhe most common jugular foramen lesion. Trpically, these lesions arc soliury with a peak incidence in the 5th-6th decad es and increased incidence in females. There are fumi lial associations in which multiple paragangliomas present in conditions such as multiple endocrine neoplasia ( M EN). Glomus jugubre rumours usually extend imracranially and can inl"oh·e the m iddle car, as demonstrated here. Clinifal presentation ma), be related to illl·oll'cment oCthe 9th, 10th and 11 th cranialnervcs. When there is extension to im"oh"e the middle ear (glomus jugu10tympanicum ) then pulsatile tinnitus is the classical presentation. These tumours arc highly vascular and enhance al·idly wi th contrast on CT and MRI. O n CT , permeati\"(: bone erosion is classical, differing from the coarser lytic
  
  When suspected on MRI , cr can still help make.a conclusive d iagnosis in many cases by demonstra classical permeati,", pattern of erosion at the foramen.
  
  Further management Depending on the size of the tumour and the in extension, a combined ENT / neurosurgieal proc pcrfomled.
  
  Further reading Caldemeyer KS, l'-'lathews vr, AzzarclJi B, Smith ( 1997). The jugular foramen: a rC\"1cwofan;a[ masses, and imaging ch:aracteristics. RadinG,.. 17: 11 23- 1139.
  
  108b Hyperintense mass lesion w ith flow voids"
  
  190
  
  eNS, Head and Neck Imaging
  
  Case 109
  
  19 1
  
  Answer 109
  
  eNS, Head and Neck Imaging
  
  ANSWER 109 Observation s (109) These sclcclcd MR im3gcs show an expanded sphenoid
  
  Appc:ar.mccs on MRJ :lrC \'3rbbk depending 011 fluid/ protein CQrHcnt of the mucocele but impo
  
  sinus filled with abnormal signal rather th:a11 air. The (amelUS show mild uniform hyperintcnsiry on Tl weighted and T2 weighted images. The walls of the sinus :appear slllomh and intJct with no ob\iou$ evidence of dcstmctiOIl.
  
  imaging findings arc of a nondcstructi\·c, slow gra...
  
  The findings arc consistent with a mucocele of the sphenoid si nus, the signal indicative ofprotcinaccolls fluid contents. Sagin;)'l Tl and coronll 1'2 inl:lgcs dcmonstr:I\C displacement of the optic nerves br the expanded sinus, presumably causin g compression of the atHl:rior optic pathway gi\'en the hiSTOry of\'isuaJ disrurbance. Diagnosis Sphenoid sinus mucocele
  
  Discussion A mucocele arises due
  
  to chronic obstruction of a sinus, which then becomes filled with mucus. This collection acts like a slow growing mass c:lUsing expansion of sinus bony walls withom frank bOil\' dl:slruction. Vessels and other srmcrures arc displaced ";ther than being encased, as with a tumour. Usually these lesions are asyrnptomaric untillhcy becomc large when the}' can cause optic nel""\'C compression, proprosis and headache. Secondary infection of the mucocele can supen'ene, but this is a rare complication. Ninet), per cent of lesions arc found in rhe frontal and ethmoid sinuses with sphenoid sinus rnucocdes being anI}' rarely seen.
  
  lesion.
  
  Practical tips CT is good lor showing bony expansion with the •
  
  absellce of bone erosion. ~'IlU is good for identil)'ing the exrelll/size of tile lesion and looking for complications of optic nel'\c compression ( usually from postcrior ethmoid lesi
  Further management Referral to ENT for consideration of endoscopic surgery.
  
  109 Superior displacement of optic nerve (left); lateral displacement of cavernous sinus by large sinus mu cocele (right).
  
  192
  
  eNS. Head and Neck Imaging
  
  Case 110
  
  193
  
  Answer 110
  
  eNS, Head and Neck Imaging
  
  ANSWER 110 Observations (110a, 110b) The selected plain CT images ( iIOa) show a poorly defined, hypcrdcnsc midline lesion eXTending across the body of the corpus callosum. There is no significant surrounding oedema. Pre- and polit·contrast Tl weighted MRimagcs ( 1IOb ) from the same patient show a multilobulatcd lesion with uniform cnhanccrncnr crossing the midline in the body of the corpus callosum. No further lesions lre seen \\~thin the brain p arcnchym~1. Incidental note is made of cavum sepnull pcllucidum. lllc findings arc of a 'buuerfly' tumour in t.hc corpus callosum.
  
  Diagnosis HutTcrfly g liobhlSloma multiformc (GBM ).
  
  Typical imaging appcarnnces of a butterfly gIl of a poorly defined lesion that enhances uniformly fomlation, necrosis and haemorrhage are seen in a of C:1SCS and c:1kilication is rare and usu:llly :1SSOCiatcd chemo/ r.ldiOlher.lpy. The corpus callosum is mack dense, tightly packed white matter tr.lcrs and th mass efleet and vasogenic oedema arc m Hemispheric glioblastomas conversely have quite mass eHect and surroundin g oedema.
  
  Practical tips GBM and lymphoma arc the two likeliest diagnose.. bUrTerfl)' lesions ill\'olving the corpus callosum. Al diflicult to diffe remiate radiologically, cavit:\tiOll necrosis arc rcl:ltivcl y uncommon in lymphoma (ex AIDS patients).
  
  Differential diagnosis For lesions crossing the midline in the corpus callosum: • GBi\·1. • Lymphoma. • Dcmyclimuing disease - multiple selerosis.
  
  Discussion This is the mOSf common :ind the most malignant primary brain (Umour. [\ accounts for >50% of brain tumours \\;th :I \\~de :lge distriburion pc:alcing :It the 7lh-8th decades. The most common location is within the white muter of centrum scmiovalc with increased incidence in the frontal lobes. Other patterns of distribmion include callosal extension giving this appearance of a butterfly gHom:l, postcrior fossa lesions and multifocal distribution (seen in 2-4%). Tumours sprcad b), direct extension im·oh·ing white maller traClS, such as the corpus callosum and cerebral peduncles or vi:l the CSF « 2% of C:ISes). Haematogenous spread can also very rnrcly occur.
  
  Further management T2 hypoilltensiry is sometimes present with C:"S lympholl1:1 and thereforc this imagc sequencc CD sometimes hclp to diffcrentiatc lymphoma from glioblastoma. Ncurosurgical c\·alu:ltion.
  
  Further reading BOllrekas EC, Varakis K, Bnms D, et nf. (2002 ). of the corpus callosum: ~'I R illl:lging and di!fe considcrations in :ldults and children. Alllerimll JOlln1n1 ofRndiology 179: 25 1- 257. Rees JI-I, Smirniotopollios JG, Jones RV, Wong K ( 1996). Glioblastoma multitormc: radiologic· pathologic correlation . RadioGrnphies 16: 6.
  
  110a lobulate d lesion crossing the mid line in the body of the corpus callosum.
  
  194
  
  eNS, Head and Neck Imaging
  
  presented with
  
  Case 11 1
  
  ,
  
  lllb
  
  -.~ ••- "'~& •.
  
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  '.'.
  
  . . .
  
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  ......
  
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  e -- ,~.
  
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  195
  
  An swer 111
  
  eNS. Head and Neck Imaging
  
  ANSWER 111 Observations (111a, 111b. 111c) These STiRsagitt:l1 ( l il a), STIR coronal ( illb) and T2 weighted axial ( i l le) images of the spine demonstrate posterior vertebral scalloping, particularly at the T il , TI2
  
  and L2 b'ds. There is also a thoracic scoliosis. AI
  
  approximately no le"el , coronal images show a right sided high·signallesion projecting laterally from the spinal canal,
  
  Neurofibromas - enhancing dumbbell-shaped lesi Scoliosis and kyphosis. Enlarged vertebral foraminae - due to ne urofibrOI1ll or lateral meningocele. H ypoplasia of the pedicles, Irans\'erse and spinous processes.
  
  presu mably through the intervertebral foramen. This
  
  Practical tips
  
  follows CSF signal and is probably a lateral thoracic meningocele. The axial 1'2 weighted image shows a second lateral thoracic meningocele on the Jeft side, in a similar region ofthe spine. The combination of findings is strongly suggestive of neurofibromatosis type I and multiple highsignal cutaneous nodules on the sagittal images confinn this diagnosis.
  
  • Alwars look for the presence of subcutaneous nod on the image when NF l is suspectcd. • It Illa}' be difficult to diflCre.ntiate between a lateral thoracic meningocele and a 'dumbbell' neurofib H owever, on MRI the former will be ofCSF de on all sequences, Le . high signal on T2, whe.reas latter will have a hyperintense periphery on T2 mQ hypointense core. In a case such as this, one might also comment rn.. there arc no obvious adrenal Illasses or renal as)'mmetry (implying association with phaeochromocytollla and renal artery stenosis, respectively).
  
  Diagnosis Neurofibromatosis type I (NFl ).
  
  Discussion Neurofibromatosis Iypc I is a neurocutaneous disorder, of which 50% is inherited in an autosomal dominant manner and 50% is sporadic. Classical features in the spine include: • L.1teral thoracic meningocele (dysplasia ohhe meninges resulting in di\"enicula of the thecal sac, whieh extends through the neural foramina ). Posterior vertebral scalloping - also due to dural ectasia.
  
  Further management Neurofibromas can undergo malignant tr:l.Ilsform;!.tli 2-3%; therefore a rapid rone of growth or new onset should be thoroughly investigated.
  
  1118 Posterior vertebral scalloping.
  
  111b l ateral thoracic meningocele.
  
  196
  
  eNS, Head and Neck Imaging
  
  Case 112
  
  197
  
  Answer 112
  
  eNS, Head and Neck Imaging
  
  ANSWER 112 Observations (112a)
  
  Selected uncnhanccd axial cr images ofrhc bi.lin show a well defined low attenuation lesion in the left fronrallobc
  
  causing minimal mass effect. The contents arc oflowcr attenuation than CSF but gh'en the absence oftrauma it is llnlikcl~' that this is due to intr.lcr:lnial air. It is likely that instead, it represents fur, which could be easily confirmed by adjusting the CT windows or taking a direct dcnsit~, measurement. A calcified nodule is seen at the posterior aspect of the lesion with furthe r mural c:l.lcific:l.Iions elsewhere. Further small loculcs of f'at arc seen in the :Interior hom of the left lateral ventricle and sc\'cr:al sulci.
  
  Typical appearances on MR.I: well defined lesion which is high signal imensit),on T I weighted il1lagi • \\ith signal \'oid at points of calcification. No enhancement with comrast. CySt rupture leading TO chemical meningitis is occasional complication, as in this c:ase. Release of globules can produce f.u-Iluid levels WiTh CSF. Fi 112b demonstrates typlc:al appcar.mce of:a dcmlOid II; 6t-containing lesion of high signal on TI weight im:aging. Some strands of mesodermal tissue arc n within it. A f.lI -fluid level is seen in the [:ateral \·emrick to rupture into the subarachnoid space.
  
  Diagnosis Ruptured dermoid cyst
  
  Discussion Dermoid cYstS arc fairly Ullcommon CNS lesions, usu:ally presenting before the 4th decade. They arise due to inclusion of epithdial elements :at the time of closure of the: neural rube and therefore contain ectodermal and mesodermal components, i.e. hair, sebaceous glands and skin . Derrnoids arc usually locpted ncar the midline and C:all be found within hrain parench}'m:a , vcntricles, CSF sp:aces or within·hom:. Dc\'elopmem :and g.rowth arc \'ery slow and presentation is therefoTt: late. • Typical appearances on Cf: low-densit), lesions containing fat with no contrast enhancemem. j\·l ural or focal ccntral calcification can be seen.
  
  198
  
  Practical t ips Posterior fossa dcrmoids arc usually found in the midline and show no contrast enhanccmem on CT/MIU. On TI weighted MRI high signal is produced ~ Fat. Blood products ( methaemoglobin). • Proteinaceous fluid. • Contrast.
  
  Further management
  
  cr
  
  MRI is bencr than for identif)~ng nlprurc of a CYSt and the consequent chemical meningitis.
  
  eNS, Head and Neck Imaging
  
  Case 11 3
  
  199
  
  Answer 11 3
  
  eNS, Head and Neck Imaging
  
  ANSWER 113 Observations (113a) These T2 weighted axial brain images show :J. diffuse increase in signal affecting the right Icmporallobc. This in\'o!\'cs grey and wllite maner, eXTending to the cortical surncc . Sulci in the aAcctcd aTea arc dfuccd. Similar signal changes extend along the cingulate gyrus ,md also affect
  
  the contralateral temporal lobe
  
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  a lesser extem. The
  
  duration of the history and distribudoll ofillvoil'cmcnt :lfe in keeping wi th encephalitis, mos\ likely due to herpes simpkxvims.
  
  Diagnosis Herpes simplex (HSV) encephalitis.
  
  Differential diagnosis Right middle cerebral territory infilTct.
  
  Discussion Encephalitis is the term generally u.sed to describe a diffuse
  
  cerebral inflammatory process of \'iral aetiology. Clinical presentation is with confusion , headache and seizures progressing to coma. Fe\'er is almost always prcsem . The most common organism is HSV. infection being either primary or due to vinls reacti\~ltion. The resulting cytotoxic oedema manifests as high signal on T2 weighted, FLAIR and DWI t.'IRI. The temporal lobe is typically affected , often with the inferior frontal lobe and cingulate gyrus. Unlike other viral infections, the basal g:mgli:!. arc usually spared.
  
  Bilateral temporal lobe im'okement is virtually pathognomonic of HSV infection. Features that atypical and should arouse suspicion of other organisms include basal ganglia i1l\'ol\'ement and isolated im'oh-ement of other lobes withom [c ~ lobe ilwolvemellr. Cytotoxic oedema (seen in stroke and encephali
  
  ~' ~~~~:Ii~o~~el:,~:r~~~~' \~.:~~!l~lI~l~~~l~:I:~s~:sss, atlects\\"hite matter only, Without clinical history, one might mistake the :\ changes of HSV encephalitis for a middle ccrebr.JI artery (MeA ) infarct. Howevcr, notc that the posteromedial temporal lobe affecTed by HSV is spared in an MCA infurct as this lerritory is sup by the posterior cerebral arIel)' ( PCA). Figure II shows a typical MCA infJrct wilh lowanenuatioo change in the right [emporallobe but sparing the described posteromedially (this scan also happem show a hypcrdense MCA due to lhrombus). FOf" comparison, Figure 113c shows a PCA infurct \\ the posteromedial rempomllobc affected. Neonatal herpes-related encephalitis is due to H type 2, probably acquired via the genital tract perinatally. The imaging features arc different fnr those due to HSV type I described above.
  
  Further ma nagement Urgent treatment with alHiviral drugs - mort untreated I-ISV encephalitis can be as high as 65%,
  
  Practica l t ips • HSV encephalitis has high Illonalit}, and morbidity rates bur is treatable with acyclovir SO a high index of suspicion is needed, especially on cr, which may well be the first imaging done - signs of oedema and swelling arc likely to be comparati\'cI}' subtle compared to MRI (if prcscllt at all).
  
  11 3b Axial pla in C7 brain shows a ri middle cerebral a: territory infarct hyperde nse artery sign.
  
  200
  
  eNS. Head and Neck Imaging
  
  Case 114
  
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  Answer 11 4
  
  eNS, Head and Neck Imaging
  
  ANSWER 114 Observations (114al This single CT im::agc shows diflusc low-attenuation change in the frontJl and tcmponl lobes and br:linstem with loss of the grey-white matter diflcrcllliation. The cerebellum is bener preserved. Generalized sulcal cff.1ccmcm indicates ccrebr.tl swelling. This widespread reduction in brain density makes the normal ccrcbr:tl vessels and dural rcflccrions appear spuriousl ~' conspicuous. Close inspection confirms thal this is a pcrccplUal 'abnormality' - rhe basal cisterns arc of normal CSF density so there is not acute subarachnoid haemorrhage. The ffndings arc suggcstil'c of global ccrcbr:l.l ischaemia and oedema.
  
  Diagnosis Global cerebral anoxia. This particular casc turned ou t to be secondary to asthma· induced cardiorespiratory arrest.
  
  Discussion Global ischaemia can occur following prolonged hypoxia, which may be sccondary to litting, aspiration, smothering, strangulation, etc. The CT,findings arc distinni\'e, with loss of grey- white matter differcnriation and changes due to cercbr:al oedcllla including sulcal eflaccment. Generalized low density of the cerebral COrTex de\"elops. The cerebellum
  
  is more resistant to hypoxia and therefore appears of hi_ densit), in comparison with the low-density cerebral cona: the so·called 'rC\"ersal sign' (sec also Case 163 ). The conspicuity of the vessels and dura may lead to the erroneous diagnosis of acute subarachn haemorrhage (SAH ) on first inspection. An example or"_ real SAH is shown ( 1l4b ) - note high density in suprasellar cistern representing acute haemorrhage and nornlal density cerebral cortex with preserved grey-\\ marter differentiation. There is also dilatation of temporal horns of the lateral ventricles indicati ng e obstructive h~'drocephalus. Trauma is the most COIllrn. cause of SAH. cr is 90% sensith'c in the detection of ;'\!.. in the first 24 hours following prcsentuion. Howcvcr. decreases to 50% at I week and continues to fall ther Small SAH may not" be seen on CT and will onl~ deTected on lumbar puncture (LP) as xanthochromia. r.cause of75% of spontaneous SA H is berry ancurysms these arc mostly found sprouting from the circle of\\ An exam pic of a large SAH in :I patient with :I rup aneurysm is shown ( 1 14 c). Note the cxtcnsi\'c amoWl[ blood filling the sulci and Sylvian fissures. Blood has entered dlC vemricuiar svstcm and C.lIl be seen in the- ' and 4th ventricles. The'large ;lIleurYSlll appears to from the origin of lhe left middle cerebral artery.
  
  ' 14b Axial plain CT of brain showing SAH with blood in suprasellar and basal cisterns.
  
  202
  
  eNS, Head and N eck Imaging
  
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  Answer 118
  
  Musculoskeletal Imaging
  
  ANSWER 118 Observations (118a)
  
  Discussion
  
  A below knee ~unpllt;ltion has previously been performed. lllcrc is a pcrmcati\'c lucency with a ,\ide zone of transition affecting the tibial stump. Periosteal reaction is visible along the posterior cortex ;lIld there is llso a hint of this along the fibub. runhc:mlOTc, fuint luccncics arc suspected along the :anterior cOrtex of the distal femur. These arc rhe appearances of an aggrcssi\'c lesion. The reason for :Imputation and the time: inrerval since ha\'e nor been stared - this would help dari/)' the likely diagnosis. From the radiological findings alone, the main possibilities arc that rhe amputation was for a distal malignancy such as bone lymphoma that has now recurred, or that rhe current findings arc due 10 osteomyelitis in the stump following amputation to r an unrelatcd reason. However, suspectcd im'olvcment of the distJI femur without joint destmction f"lxOUI"S the former option.
  
  Another example of a lymphoma is seen aAi:cri~ tibia of a parielH in Figure 118b; a peTmeariH: present with e\idence of cortkal destruction. Permeative bone dest"mnion on radiogra~ the presence of aggressive p;nhology with fa potential. The pcrmcath'c lesion has a ditfusc: eaten' appearance (l i Se). The zone oftransi in other words the lesion is poorly dcmu.impaceptibly merges with uninvolved bone, lesions where the zone of transition to no narrow. PeriOStcal reaction rna)' or may not Ix finding of a r~ef[m::ati\'e lesion on imaging usu.ll. malignant pathology; however. infection can s,1me appearallce.
  
  Diagnosis Lymphoma recurrence following amputation.
  
  Differential diagnosis Of permc:ui)'c bone lesions: • Metastases. Myeloma. Lymphoma. Leukaemia. Osteomyelitis
  
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  119,120
  
  Musculoskeletal Imaging
  
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  brion producing a 'cobweb' appearance. poisoning causes dense rnct;lphyscal bands.
  
  management treatment 'l\'ailab1c tor osteopet rosis is bone trans plamation. Patients arc morc prone to than the normal popul.uion.
  
  rvations (120a) Aexion and extension views of the cel"\~cal spine arc m. On flexion there is significant adanlOaxial joint .ation. The odolHoid peg is not clearly demarcated ~ likely to be parti:J.II~' eroded. The remainder of the - -..a.I spine is quitc wcll prcscn·cd. The most likely is in a patiem of this age is rheumatoid arthritis. toaxial subluxation in a patient \\"ith rheumatoid tis ( RA).
  
  erential diagnosis mJ.moaxial subluxation: .. Jt.-\. .. Psoriatic arthropathy. .. ·U'·cnilc idiopathic anhritis.
  
  11ge Forearm of a patient with fluorosis showing diffuse osteosclerosis and prominent ligamentous insertion calcification.
  
  Ankylosing spo ndylitis. Systemic lupus erythematosus (SLE ). Down's syndrome . J"lorquio's syndrome. • Retropharyngeal abscess in a child .
  
  Discussion Atlamo.uial subluxation occurs when the dist-lIlcc octween the posterior aspect ohhe arch of the alias and the ;lIltcrior aspect of the odontoid peg exceeds 3 mill in adultS and 5 mm in children. Erosion and destmction of the OOomoid peg may also be seen, particularly when the process is caused by an inflammatol)' arthropathy. Several causts arc described in the differential diagnosis list but RA is the most common cause in adults. Synovitis with pannus fo rmation causes erosion of the o dontoid peg and atlantoaxial ligamentS and consequcnt subluxation. This (CO/It. )
  
  2 17
  
  MuSculoskeletal Imaging
  
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  Answer 121
  
  Musculoskeletal Imaging
  
  ANSWER 121 Observations (121a, 121b) AI' and l:ltem1 r.ldiographs of the left- knee show lobulated calcified masses in the soft tissues anterior to the left knee. Taking into consideration the age and cthnicity of the paticm, ru!l1or.ll calcinosis is the most likely diagnosis.
  
  Diagnosis Tumoral calcinosis.
  
  cause pain and limitation of mo\"ement, with o\·erl~· .:: ulcer:nion and the de\·clopmelH of a sinus tract d (halk~' nuid. In fuct, the masses m3y contain fluid lel"cls on imaging that ha\"e a milk of eakiulll (on The periarticular region orlhe hip is the most COlllla aHccted site. The masses can grow to 3 ,"cry large sill: there is a tendency to r recurrence if the lesions complctelyexciscd.
  
  Differential diagnosis
  
  Practical tips
  
  Ofpcriarticular soft tissue calcification: • Hacmatoma. Myositisossificans. Crystal arthropathy. Sclcrodcm13 ( I 21c - note the characteristic dist:ll tutt resorption in the index tinger). Dermatomyositis. SyIlO\'ilJ osteochondromatosis. • Tumornl calcinosis. • SYIl0\;alurcom:t ( 121d, 121 e, 12 1f, 121 g ).
  
  The radiological appearances of the ,'arying CJ periarticular calcification can be quitc different: • T umoral calcinosis - lobulated dense tl13sseS, fluid le\"els. • Myositis ossific3llS and synovial sa rcoma may I similar but withom fluid Ic\"els. • DefmatOll1l"0sitis - shects OfC:lkificatioll. • S)'no\'ial oSTeochondromatosis - se\"eral foci of calcification with lucent centres that 3fe essen loose bodies within the joim.
  
  Discussion
  
  Further management
  
  Tumonl calcinosis is a rare, 1x:nign condition characterized b}' the presence of progressively enlarging perianieular calcified soft tissue masses. It usually affects roung black patients and there is a fumilial tendency. The masses Illay
  
  Patients with tumor31 calcinosis arc usuallv treat phosphate depiction. Surgical excision is 3150 a option but needs to be meticulous as recurre na: OCCLIrs with incomplete excision.
  
  121c Radiograph of the hand in a female adult with scleroderma shows the typical soh tissue calcification . Note the distal tuh resorption affecting the index finger, which is characteristica lly see n in this condition.
  
  121e, 1211, 121g Axial n , T2 and fat saturated images of the synovial sarcoma shows a fairly well de soft tissue lesion which is of tow signal on T1 and in homogeneously high signal on T2.
  
  220
  
  Musculoskeletal Imaging
  
  Cases 122, 123
  
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  221
  
  Answer 122
  
  Musculoskeletal Imaging
  
  ANSWER 122 Observations (122a)
  
  Practica l tips
  
  All and latCr:l1 radiographs of the right knee and upper tibia
  
  Ostcopoikilosis tends to be distriburcd around whereas multiple sclerolic !l\Clastlscs will not confin<.!d.
  
  and fibula demonstrate marked cortical thickening of the diaphyses ohlle tibia and tibula, which has the appcarnncc of'dripping candle wax', This is typical ofmclorhcostosis. There arc also multiple small sclerotic lOCi within thc mctJdi:tphysis of the distal femur and the proximal libi:! , which h:1I"c thc appearance of oSlcopoikilosis.
  
  Diagnosis
  
  Further management No further mallagement is normally necessary. conditions arc usually found incidenrall)•. The ~ is to ensu re thai a patielU with multiple sclerotic has osteopoikilosis and not sclerotic metastases.
  
  Mclorhcostosis and ostcopoikilosis.
  
  Differential diagnosis $dcrQ[ic metastases in a paricllI with mclorhcostosis; this is much less likely. H owt:I'cr in patients with multiple sclerotic foci alone, sclerotic metastases must always be considered.
  
  Further readi ng Levine S, Lambiasc It, Petchprapa C (2003 ). G::rT lesions of the tibia: characteristic appearances. coLII'entional r.ldiograph)'. RndioGmphics 23: 15 7- 177.
  
  Di scussion Melorheostosis is a nonhereditary disease of unknown aetiology that often presents as an incident-al finding. II is usually discovered in childhood where it has a rapid progression, but it occasionally presents in adults where it has a slow c.hronic course. The limb ill\"oked often demonstrates joint pain , swelling and limitation of movement. Males and fem:lles arc equally aficcted. Radiological signs ofmclorheoslOsis arc as follows: • Conical hyperostosis in one or multiple tubular bones with streaks of sclerosis beginning at the proximal end of the bone and extending distally, This produces the chamcteristic 'dripping candle wax' appearance. This is shown particularly well in Figure 122b, where the right humerus and scapula arc aficcted in allother patient. Predominantly aflccts the diaph)'Sis of the bone, The lower extremities arc mo re commonl\' aficcted than the upper. . Although a single bone may be involved, contiguous bones of an extremity afe mOTe often aflccted. Hilateral signs arc cxtremcly rare and should prompt consideration of other causcs of sclerosis. • Limb length discrepancy is also a feature and the sclerosis may cross the joint and result in joint nLsion. The skull, spine and ribs arc rarely invoh'ed. !\klorheostosis is associated with osteopoikilosis, osteopathia striata (asymptomatic disease consisting of lo ngitudinal striations along the metaphyses of lo ng bones ) and arteriovenous malformations, OSleopoikilosis is an autosomal dominant disorder that is more common in Lll~ks. II is asymptomatic and consists of multiple o\'oid bone islands parallel to the h;:lJlg axis of the bone. These bone islands normally measure 2- 10 mm and arc found at the metaphysis and epiphysis, rarely extending into the midshaft. It usually aficcts rhe peh'is, wrist and ankle and Tarely affects the skull , ribs and mandible . The differelltial diagnosis or this condition, which often leads to clinical con fusion and concern, is disseminated sclerotic metaStases.
  
  222
  
  122b AP radiograph of melorheostosis affecting the humerus ~nd scapula demonstrating the typical 'dripping candle wax' appearance,
  
  A nswer 123
  
  MuSculoskeletal Imaging
  
  Case 124
  
  ANSWER 123 Olao,."ations 1123) n~
  
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  Answer 124
  
  Musculoskeletal Imaging
  
  ANSWER 124 Observations (124a)
  
  Further management
  
  There is difruse, coarse trabeculation of the visible bones causing a 'cobweb-l ike' appcar.:lllCC. Erlenmeyer fla sk deformity of the distal femurs is presen!. The features arc in keeping with thalassacmia major.
  
  Death usu311y occurs within the first decade-. b)' multiple tra nsfusions, howe\'cr as exp discussion this tOO leads to skeletal abnorrn
  
  Diagnosis
  
  T yler P, Madani G, Chaudhuri R, ct (I/. (2006 radiological appearances ofth:llassnemia,
  
  Further read ing Thalassacmia major.
  
  Rndiology 6 1( 1):
  
  Discussion Thabssacmia is an inherited disorder of haemoglobin synthesis charncfcristically seen in Mediterranean patients. The homozygous form, thalassacmia major, is mOTC sncrc. The radiological signs result from marrow hypcrpl:J.sia and expansion due [Q cnramcdullary h:u:rnatopoicsis. Evcry part of the skeleton may be aflcctcd in patients with untreated disease. R.1diologicaJ fcamrcs of the complications of treatment such as recurrent transfusions and iron chelation thcrapy may also be seen. In fuet, ab normalitit:s secondary to iron chelation therapy arc now more com mon than those due to lllarrow hyperplasia. T he radiological signs to look for depend on the site of the bod~' imaged' • Peripheral skelerun: Coarse trabeculation e3using 'cobweb' appearance ( 124a ). • Loss of conca\'ity ofwbular bones ( 124a). • Er1cnmerer flask deformity of metaphyses of long bones ( 124a). • Arthropathy and chondroca1cinosis as a result of haemochromarosis secondary to hypertral\sfllsion. • Fraying of metaphyscs and dense metaphyseal bands secondary ro iron chel3tion therapy. • Skull: 'Hair on cnd' appearance. Frontal bossing (124b) due to diploic expansion. O bliteration of paranasal sinuses (except for ethmoid sinuscs) due to marro\\' h)'pcrplasia ( 124b ). • A-.;i:tlskekton: Co3rsc trnbeculation causing 'cobweb' 3ppe3rance ( 124<). BiconC3\'C "ertebr.lc. 'Bone within bone' appearance of spine and ribs. Paraspinal masses (due to extramedullary hacmatopoiesis). Expansion of the ribs posteriorly, panicularly at the costochondral junctions, due to marrow hyperplasia ( 124c).
  
  Practical tips • T he only sign may be a difruse but subtle coarsening ofthe bony trabecu lae. On an AX It look for evidence of hepatosplenomegaly (a result of extramedullary haematopoiesis) and gallstones. On a exit look for cardiomcgaly secondary to an3emia, 3S well as a paraspinal mass due to eMr.lmedullary haematopoiesis.
  
  224
  
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  MuSculoskeletal Imaging
  
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  Musculoskelet';l Imaging
  
  125
  
  Case 126
  
  Furthe r ma nagement i':lRI is helpful in assessing local spread :llld :lids planning of surgical resection.
  
  benig.n or aggressive? ysis, metaphysis or epiphysis?
  
  Fu rther reading
  
  old is the patient?
  
  ~'Iurphcy
  
  "olmg patient with an aggressive lesion such as m[cd, the twO main primary tumours to Me Ewing's and osteOsarcoma. The tWO can :ult to differentiate radiologically, and can also 1Ullilar clinical features such as fevcr and 'fosis. T he features shown in Table 2 may help,
  
  M, Robbin M, i\kRac G, tt al. ( 1997 ). The many fuees of osteosarcoma. RadioGraphies 17: 1205':' 12 31.
  
  _ none arc pathognomonic .
  
  .able: 2 Dljji:YC1Iti(ltillgfCfl/IlYn of Ewillg'r S(lYCOIIJ(l «lid ortcosnYCOIII(l Ewing's sarcoma Children More common in diaphysis More commonly lucent or permeative To bone
  
  O steosarcoma Older children and young adults 1\-10T": common in metaphysis More conHllOnl}' sclerotic To lung
  
  227
  
  Answer 126
  
  Musculoskeletal Imaging
  
  ANSWER 126 Observations (126a) There is a tnllswrsc fracture at the medial third of the clavicle. which is li kcl\' to be an insufficienc\' fracturc as Ihere arc Looser ZOllC~ at the b \cral aspect oCthe cl:J.I'iclc :tnd the b tcr.tl border of the body ofth, scapula. T he bones arc generally oSlcopcnic with marked thinning of the coniccs. T he findings arc consistem with a diagnosis of osteomalacia
  
  Diagnosis Osteomalacia.
  
  Discussion Ostcomabcia is a disorder of insuHkicnt osteoid mineralization causing bone softening. Aetiology can be due to dictary deficiency, dccrclscd absorption or deficient metabolism of vitamin D. Radiological fc;trurcs of ostcomalacia include.: Generalized ostcopcni:t. Cortical thinning. Bowing of long bones. Protmsio acetabuli. Coarse trabecular patrc.rn ( 126 b ). Looser zones: • _ Pseudofra([ures that consist of tranS\'erse luct':nt clefts \Iith sclerotic margins . • These arc mostly seen in the pekis, femoral necks ( 126c) and scapula. Insutlicicncy fraCTUres.
  
  228
  
  Practical tips Osteomalacia is related TO renal failure and hyperparathyroidism therefore: On an abdominal radiograph look for a perit dialysis catheter or a Hillnelled femoral haem line. On a chest radiogr.lph look for a centr.ll line and eroded lateral cla"icles. Promincnr vascular and soft tissue calcification secn. Brown TUmours ma~' be present and appear as lesions, which mal' also be associated with pathological fr.lc t ~Jre.
  
  Further management Treatment involves rCI'ersing the cause of Ii deficiency.
  
  Musculoskeletal Imaging
  
  Cases 127, 128
  
  prcsclHcd with ing dcform it), of right leg.
  
  229
  
  Answer 127
  
  Musculoskeletal Imaging
  
  ANSWER 127 Observations (127a) There is marked angulation deformity of the diaphyses of the lower tibia and fibula with the formation of pseudarthroses. The bones aTe also generally ostcopcnic. The most likcl~' diagnoses arc nonunion of prc\'ious fractures, ncurotibromatosis or osteogenesis impcrfccta.
  
  Di agnosis Neurofibrom:ltosis.
  
  Differential diag nosis Nonunion of a fracture. Osteogenesis imperfecra. • Fibrous d}'spbsil. • Congeniral.
  
  Discussion Congenit:ll - :lllcCls the middle to lower third ofthc tibia and tibula. H alf of congenital pseudarthroses present in the first }'c:ar of life and later on there ma}, be cupping of thc proximal bone end and pointing ofthe distal bone end. Neurofibromatosis rype I (NF l ) is a common genetic disorder ;1I\d in addition 10 cutaneous and neurological :abnormalities, osseous lesidns :are also seen. There rnav be an terolateral bowing of the ribi:a with or witho~t a hypoplastic fibula. Focal n:lrrowing and intramedullary sclerosis or cystic change:at the :apex of the angulation is due to hanlarlOnlatOus fibrous tissue, typically at the junction of the middle and distal third of tibia. Pathological fracture with nonunion often results in pseudarthrosis of the tibia and fibu la, with 'pencil pointing' of the bone fragments. Prophylactic bracing of limbs with bowing deformit), may pre"ent the de\·clopment of pseudarthrosis. OSleommy with bone grafting and pinning ( 127 b ) is the treatment of choice if the pseudarthrosis has alread~' occurred.
  
  Osteogenesis imperfecta - all four types can r bowing of ,he long bones due to bone softening multipte fractures. Bowing typically involvC$ all the
  
  bones and can resull in pseudarthroses. Again OSt and pinning arc the preferred treatment alth: bisphosphonalcs n:l\'C been shown to produce success. Cleidocranial dysplasia is associated with (on_ pseudarthrosis of the femur. Ankylosing spondylitis can lead to pseudarthrosis spine.
  
  Practical tips Severe osteopenia and multiple fractures ofdi ;lges that hal·e exuberant callus fo rmation su&..£cIII osteogenesis imperfecta. Remember that SOIlK osteopenia may result trom disuse, howe"er, c!" fracture nonunion. Look for soft tissue nodules indicative of neurofibromatosis. A 'ground glass' density lesion associated \\ith pscudarthrosis is suggestive offibrous dyspl.lSi.l.
  
  Furthe r management As in this case, osreotomy with bone is the treatment of choice.
  
  g~'''''g
  
  Furth er reading Cheema 1, Grissom L, Harckc H (2003). characteristics of lower·exrrcmit")' bowing in
  
  RndioGrnpbics 23: 871 - 880.
  
  127b Radiog raph of the same patie nt pos with pins.
  
  230
  
  """-....
  
  Musculoskeletal Imaging
  
  Case 129
  
  dislocations. Hypoplasia orthe capitellum and !';ldial head may also be pn::scm and this can kad to all increase in the carrying angle: at the dbow. Similarly, genu valgus can <x:eur due ro asymmetrical den:lopmcnt of the femoral condyles. Some patients may ha\'e a short 5th metacarpal.
  
  'Aunt Minnie'. Fo ng's discasc, also known as and ostco-onychodysplasia, is a nTC [ disorder characterized by sym lllcrric:ll and mesodermal anomalies. Patients lend to ab no rrn31ities of nai l dysplasia that Gill spooning and splitting of the fingernails or ("\'ell or aplasia. This particularl}' aflc cts the thumb finge rnails. Pat ient s lll;lY al so h:1.\"c lbnorm:tl of [he iris. ~ nce ofbilatcrnl poSTerior iliac horns is seen in ~ and is diagnostic ofdlC condition. Hypoplasia JDtc:rior half of the ilia can result in drooping of the . The other major finding is aplasia or hypoplasia d bc, which frcqucntl~' results in recurrent lateral
  
  Practical tips Look for a perironeal dialysis eMheler or femoral runnelled dialysis line on the pclvic radiograph, :ls Ihese palients ma ~' have rcnal failure. If the knecs arc included on the radiograph, look for hypoplastic or absent patellae and genu valgus deformity. Further management The mOSI serious association of thi s condition is renal dysfunction, which occurs secondary to abnormality of the glome ru lar basemenr membrane leading ro protei nuria , hacm3ruria and renal f.lil urc. The CX~lcr mechanism for this is unknown , bur renal f.1ilure tends to <x:ellr in later life , This is an important poim ro note when diagnosis of this conditio n is made, often incidentally.
  
  23 1
  
  Answer 129
  
  Musculoskeletal Imaging
  
  ANSWER 129 Observations (129a)
  
  Practical tips
  
  There is a 'hair on end' appcarnncc to the skull vault and the diploic space is widened. This docs not aHe" the calvaria below the lel'el of the imcrnal occipital protuberance. T he mlxilbry sinuses arc oblitcr.ttcd. The most likely C:IllSC is thabSS3cmia major.
  
  • Thalassacmia major and sickle cell disease arc the most common causes of'hair 011 end' skull. To diflcrentiate the twO look at the maxillary sinusothey arc obliterated this suggests thalassaemia. docs not occur in sickle cell disease. Is {he appearance diffuse or localized? IflocaJizo:. consider neoplastic causes such as haemangiolll.l ( 129b). Haemangioma [ends to have a 'corduroy' ap i.e. thickened and coarsened rather than thin \ trabeculation due t'O \'ascubr channels. This is demonstrated in Figure 129b.
  
  Diagnosis TIIl13.ssacmi:J.major.
  
  Differential diagnosis Of'hair on end' appc ar;111CC of skull: The mnemonic is 'STAN' and is c:\sy to remember when VOli think of Sun Laurc! 's hair! . Sickle cell disease. Thalassacmia major. Anaemia (other an3cmias ): Hereditary spherocytosis. • Glucose-6-phosphatc dehydrogenase deficiency. • Severe iron deficienc}' anaemia.
  
  Further management Thal:tssacmia major has a poor prognosis with aHccted children not surviving past the first Treatment is by repeated transfusions.
  
  Further reading Hollar 1"1 ( 2001 ). The hair-on ·end sign. Rndi
  
  Neoplastic:
  
  • Hacm:lIlgioma (J29b). • Neuroblastoma metastases in children.
  
  Discussion The 'hair on end' sign is a finding that can be seen in the diploic space ofthe skull on r:tdiogr:tphs, cr and MIU, and has thc appearancc oflong, thin, venical striations. On plain radiogl'.)phs and cr the appearance is caused by alternating thickened tr:abeculae and radiolucent marrow hyperplasia. On 1'\-\ RI, the alternating bands of h}'POintense tr:abe..:uiae and h}'pcrimense marrow producc the distillct striated p;\ttem . Es.scmially the effect is due to marrow hyperplasia. The diploic space widens and the outer table thins and can become obliterated. With regard TO anaemic causes, the marrow hyperplasia begins in the frolHal region and can aflcct the entire calvaria excluding that which is below the internal occipital prolllbcr:tllce, since there is no marrow in this area. Marrow hyperplasia in thalassaemia major is more marked than in any other anaemia and may cause hyperplasia of the fucial bones resulting in obliteration of thc paranasal sinuses. Howc"cr the ethmoid sinuses arc spared as they do not contain marrow. Thc 'hair on cnd' appearance can also be seen in se"ere childhood cases of iron deficiencyanacmia. The medical literature is split as TO whethcr the 'hair on end ' appea rance may be re"ersed following treatment of the anaemia. Some authors have reported that resolution of the appearance occurs with rrCatmelH, although the diploic space may remain wider than normal. Howe\'er others h:we reported that the appearances persist without regression e\'en O\'cr a follow-up period of approximately 20 years.
  
  232
  
  347-348.
  
  Musculoskeletal Imaging
  
  Cases 130, 131
  
  auk presented wil It left forsc\"crnlwccks. He
  
  233
  
  Answer 130
  
  Musculoskeletal Imaging
  
  ANSWER 130 Observatio ns (130a) ,"Iultipk bony C.\ostoscs arc secn arising from the ribs on both sides of the chest. 011 the lett, there is a large associated soli tissue mass projected O\"er the btcr:tl thor.lx Gi"cn the history of chest pain, sarcomatous tf:msformation must be suspected. Mon:oyer, there is a small leti: pleural rc:tetion, a large pulmon:lI)' nodule in the left upper zone and possible pulmonary nodules ill the leti: lower zone and rightcostophrenic recess. The combination of findings suggests sarcomatous tr.J.t1sformarion ill diaphyseal aclasis wirh pulmonary
  
  exostoses poillt away trom the nearest joint, as illustr;J..tel image 130b. A pscudo-Madclung ddormity may de\' ( 13 0b ), where there is ulnar shortening with bo\\ · _ the radius, and ulnar tilt of the distal radial articular [n tcwcr than 5% ofpatknts, malign:llll transform.m into chondrosarcoma or ostcos:m:oma call occur. A example is shown ( 1 30( ) where a patient with dia~ acbsis developed a chondrosarcoma aHccting the fi The exostoses may also cause neurological comp
  
  due
  
  to
  
  ncrve compression or entrapment.
  
  Practical tips Diagnosis Diaphyseal aclasis with sarcomatous transformation. Discussion Diaphyseal aclasis is an 3urosomal dominant condition ch:!racterized by multiple exostoscs (osteochondromas). Osteochondromas 3fe benign cartilaginous tumours. Diaphyseal aclasis is usually discovered in ch ildhood and short stature may occur due to the development of exostoses :It the expense of nornul bone growth. The exostoses arc usually multiple and bilater.:ll and mostly afii:cT the linlbs though ribs can be atli:CTed, as in this case. The
  
  130b AP and lateral radiographs of the ulna demonstrate multiple exostoses. Note how they point away from the joint.
  
  234
  
  'Diaph)"scaJ' aclasis is a misnomer as the exostoso ;lTise from the metaphyses. They are otten multiple and bilateral and point from the nearest joint. Thc carrilagc cap of the cxostosis Ill:!}' be calcificu.
  
  Further management In cases of neTye entrapmenr, surgical excision possible. The crucial f..1ctor is to f degeneration when iT occurs; sllspiciol1s signs i and growth of an exostosis :lfter physcal closun. thickening of the cartibginotlS cap by greatcr than I :;. This is best delincated 011 MRL
  
  130c AP of rlght fem ur demonstrates extensive flocculen t calci fication in a huge chondrosarcoma, which arose from the exostosis at the medial femoral condyle.
  
  Musculoskeletal Imaging
  
  Case 132
  
  131 . ... ""atii,' ns (1 31a , lateral radiographs of the [cfi knee re"cal a well in the l:lIcral aspect orthe medial femoral . A scpantc bony fragment lies within the defect. rhose of osteochondritis dissccans.
  
  _1"-"'"""
  
  Practica l tips • In the adolescent knee with symptoms bur no obvious abnormality on first inspccrion, check for the subtle osteochondl""J.l defec!. The' LAM E' mnemonic identities the classical location. • When idemitied, check for radiological evidcncc of joinr eflllsion and loose bodies. • Suggest MRI forfurthere'"aluation.
  
  Fu rther management
  
  KOC hondritis dissccans is svnon"mous with hondrosis dissccan s and ostcocho~dra! fracture ~lb ) . The cardinal fcaHirc is fragmentation ora portion the articular cartilage and underlying bone, which may Ie to fonn a loose body \\ithin the joint. It is thought o.:cur due to subchondral btiguc fr:tcturc as 3. result of lUring from rotatory impacrion torees. Though times asymplOmatic, presentation is often with p:lin <J.\"Jtcd by movement and/ or limited movement. r,uients are mOSt cOlllmon!\, aficctl-d in adolescence with
  
  aficctcd morc than fcn~ales. The ~"picallocation of uochondritis dissecans at the knee is the Lateral Aspect ~lcdial fcmoral Epicondyle (usefully remembered by the ...\.\1£' mnemonic). The condition can be bilateral in up 30% of cases. Other commonly aflcctcd sites include the eral head , capitellum and talus. ,vllU is useful in cmlining whether the osteochondral fragment is loose ("\;denced b~' a rim of fluid around it on T2 weighted _ or a rim of contrast around it on i'lIRl anhrography.
  
  Identification and trcannent arc important to prevent the dc\"eloprnem of osteoarthritis. Arthroscopy and remol·al o r pinning of the detached fragment is the treatment of choice when conservative management with reST and NSAIDs!:lils.
  
  .,~.~ f ~~~~
  
  131b
  
  131b T1 and T2 weighted sagittal MRI images of the knee show a traumatic osteochondral fracture in the anterior aspect of the lateral femoral condyle
  
  CASE 132 istory middle aged female prcselllcd lI'i[h nful hands.
  
  235
  
  A nswer 132
  
  Musculoskeletal Imaging
  
  ANSWER 132 Observations {132aj T his radiograph of both hands re\'eals soft tissue swelling at the meracarpopha!:mgeal joints bilarerally and to a lesser extent at the proximal interphalangeal joints, This is not associated with any erosions, ho\\'e\'cr there is subluxation of sc\'eral joints including the metacarpophalangeal joint of the right index linger and the right first carpometacarpal joint. Degencrati\'e ch'lllges arc also noted at both wrists. In summary, there is a bibtcralnollcrosi\'c anhropathy \\ith evid ence of joint subluxation, The differen tial diagnosis includes collagen \'ascular disease, Early rheumatoid anhritis sholl id also be considered.
  
  distribution is olien bikuel'JI and symmetrical but t:bc f.tctor excluding rheumatoid arthritis is of co absence of erosions. Se\'eral difterent diseases m~ similar appcal":lIlces as outlined in the differential _ Soft tissue calcification Illa\' be seen \\'ith sclerod Ehlcrs-Danlos sHldrome: )accoud's arthritis is nonerosi\'e anhr~pathy aflccting the hands and patients lollowing rheumatic \'ah'e disease. Figurt shows an example of this demonstrating cha subluxation at the metacarpophalangeal joints ulnar de\'i.uion orthe digits. T here is also subl the first carpometacarpal joint, Note the a erosions.
  
  Diag nosis Systemic lupus erythematosus (SLE).
  
  Differe ntia l diagnosis Of nonerosi\'c deforming arthropathy: • Collagen "asc uhlf disease. • SLE. • Ehlers-Danlos syndrome. • Scleroderma. Jan:oud's arthropathy. H}
  236
  
  Practica l tips T he o nly find ing lI'ith the lIonero si\'e arthr Illay be periartku b r soli tissue swelling in the stages. Subluxation of joints is the major linding and Illostly ;IAcCIS the metacarpophalangcal and carpometacarpal joints bib tcrall}'. The appearances tend to be bilateral and s~ Scleroderma ( 132c) and EhJcrs- Danlos syn may be associated with soli. tissue calcificatioo. hand radiogra ph,
  
  Discussion
  
  Further ma nage me nt
  
  O \'cr 90% of paticnts with SLE dc\'clop arth ralgia, Nonerosi\'c arthropathy is characteristically present, the main fcatures ofwhieh arc soft tissue swelling and joint subluxation. In particular, the subluxation aftects the first carpometacarpal joi nts and ;llso the metacarpophalangeal joints leading to u lnar de"iation of the fingers. T he
  
  SLE is, as its name suggests, a systemic although patiems can CXpeCi a long lifc death occurs frOIll renal failurc or cardiomyopathy. supporti\'e with the aim of suppressing the .1. element oftlte discase.
  
  Musculoskeletal Imaging
  
  Case 133
  
  male prcS(:ntcd \\;rh left
  
  237
  
  Answer 133
  
  Musculoskeletal Imaging
  
  ANSWER 133 Observations (133al AP and axial radiographs ofthc left shoulder demonstrate a well defined lesion ill the upper hUllleral epiphysis. This has a thick sclerotic borda and central lucency. T he lesion has nonaggrcssi\'c features including a narrow zone of transition and the likely diagnosis consideri ng the agc of the patiellt and the loc1tion of the lesion is chondroblastom:t.
  
  importam to check whuher the paticm has unfuso:l. epiphyses 35 GeT will not normally be seen in this group. If doubt still c.~ists as to the nature of an cpiphya lesion, MR.!
  
  tl1J)'
  
  help. There is typiclll)' lTIarked
  
  oedema seen surrounding a chondroblastoma bUi rarclyaroundaGCT
  
  Further management Diagnosis Chondroblastom:l.
  
  Chondroblastoma is a benign tumour that m3Y bco: loc3H), aggressive. Treatment is by curettage and bone grafting.
  
  Differential diagnosis Of epiphyseal lesions' • Chondroblastorna - well defined sclerotic border, calcification in 50%, may have periosteal re;letion. Giant cell tumour (GCT) - closed epiphyses, abuts articular surfuce, eccentric, no marginal sclerosis. No periosteal reaction unless fracturc present Geode - will be other signs of arthropath~' ; older patients • Metastases and myeloma • Infection - llSUllI), :netaphyseal rather than epiphyseal.
  
  Discussion Chondrobbstoma is a rare, cartilage contlining tumour that llmost always occurs in the epiphyses of long bones. Patients aflccted arc under 30 years of age and tend to present with localized plin. The lesion is usu311y well defilled with" sclerotic border and occurs most commonl" about the knee joinT. Calcification within the lesion is see;l in approximately 50%. The tumour rardy metastasizes but m3Y be locally aggressive ( 133b, 133c). As with all focal bone lesions, an 3ssessment of benign \"s aggressive tClTures, the patiem's age and the location of the lesion within the bone arc criticli in forming a meaningful differential diagnosis. Fortunately, the dilferenti31 for epiphyseal lesions is fairi)' short! One condition that deserves brief discussion is GCT as this can somet.imes look similar. The \·ast majority occur in long bones, most commonly ar the knee. Age at presentation is 20-40 years, often with pain. Classical features ofGCT arc presence in a fused skeleton, epiphyseal subanicular location, ecee.ntric position and absence of margin31 sclerosis. Only when III these features are present can one confidently predict GeT from rhe plain r.lciiogr.lph. What is impossible to sa)', ho\\·e\·er, is whether the lesion is benign or malignant. Figure 133d is a radiograph of the knee in a patienr with a GeL Note how tht· lesion 3buts the articular surfuce but has no marginal sclerosis unlike the ehondroblastoma illustrlted ( 133a ). The distinction l11;ly not always be this clear howe\'er, and further 3.(h-ice is ollercd in rhe practical tips section.
  
  133b, 133c Plain radiograph of a lucent tibial epiphyseal lesion and axial T2 weighted MRI' of the same case, which shows a well defi ned hypointense lesion in the tibial epiphysis co with a chondroblastoma . The high signal th the tibial plateau is in keeping with marrow This reactive oedema is a common feature d chondrobfas toma and can lead to overesti the aggressiveness of the lesion .
  
  Practical tips • With regard to epiphyseal lesions in yOllng p;nients, the two main possibilities lre chondroblastoma and GCT. However, man}' ehondroblastomas will not have the prominent internal calcifications and sclerotic border seen in Figure 133a. In such ClseS it is
  
  238
  
  133d Radiograph of knee demonstrating a lucent subarticular lesion typical of a GeT.
  
  Musculoskeletal Imag ing
  
  Cases 134, 135
  
  239
  
  Answers 134, 135
  
  MusculoskeletlOllmllglng
  
  AN SWER 134 Obsarvllions l1341
  
  ,0«
  
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  Case 137
  
  Conventional osteosarcoma affecting the metaof the femur in a child.
  
  ory - -year-old female presented wilh pain.
  
  243
  
  Answer 137
  
  Musculoskeletal Imaging
  
  ANSWER 137 Observat ions (137a) There is homogeneous sclerosis of the L4 \"Crlchral body producing the appearance of an i\"ory vertebra. No significam expansion or trabeculation of the \"crtebral body is seen. The remaining n~rtcbr3C and bony skeleton 3TC nonn al in appearance. 111C most likely diagnosis in a female patient is SclCfOlic metastases from a breast carcinoma primary. T he diHcrcntial di:lg nosis includes lymphoma.
  
  Dia gnosis Sclerotic metastases from breast carCin01ll3.
  
  Different ia l diagnosis (J\'lncmonic - 'Mc:ts ... LP HI M' ): Lympho ma.
  
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  M3StOCYlosis.
  
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  137c Sagittal fat saturated MAl imag e shows multiple sclerotic and lytic m etastases throughout the visuallized spine,
  
  137b Sagittal fat saturated MAl image shows a sclerotic metastasis in the T5 vertebral body.
  
  244
  
  Musculoskeletal Imaging
  
  \'crtcbra sign is less cOlllmon in children and . the result oflymphollll, nunonly, oslcobbslol1la, ncuroblaslOm:l, rna or medulloblastoma deposits can cause nee.
  
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  Case 138
  
  thickcningjdil:muiorl and hepatosplenomegaly, which may be sccn on the spine r.ldiogr.lph. Decreased disc Sp:lCC height and cndplatc changes point 10 infection.
  
  Fu rther management The underlying Clllse G ill be dClcmlillcd from the age of the patient, ancillary signs on the radiograph (as described abovc) and thc history and cxamination . An isotope bonc scan may be required to loc:tre further dqx>sits in a p:tticllt with mct"ast"ascs.
  
  Furth er reading Graham TS (2005 ). rhe ivory vertebra sign. R.adiology 23 5: 614-6 15.
  
  245
  
  An swer 138
  
  Musculoskeletal Imaging
  
  ANSWER 138 Observations (138a) There is marked abnormality at the midtarsal joint with dislocation. sclerosis and debris forma tion. Prominent vascular calcification is noted. The appearances arc those of a Charcot joint and the most likely cause in \'lew of the \'ascubr ca1cillc:ltion is diabetes mellitus.
  
  Diagnosis Diabetic ncurop:llhic 100L
  
  Differential diagnosis Of causes of neuropathic joint: • Shoulder and upper limb joints: • Syringomyelia. • Congcnit:ll insensitivity to p'lin
  
  • uprosy. • Syph ilis.
  
  Spine' • Trauma. • Tabes dorsalis.
  
  Hip and knee: • Tabes dorsalis.
  
  • Steroids. Ankle and foot: Di:lbctcsmcllitus. Alcoholism. • j\'lyclolllcningocdc. • Congenital inscnsiri\"ity to pain.
  
  Discu ssion Neuropathic arthropathy is a traumatic arthritis associated with loss of sensation ;lnd proprioception of an affected limb. When encoun tered clinically it is also known as a C harcot joillt. The decreased pain sensation produces repetitil'e trauma leading to eventual destruction of the joint. T here is olien no history of trlullll and the patient Ill l}' present with a swollen Wlrrll joint with normll inflammatory markers. A third h:we pain at presentation although there is ustlllly a decreased response to deep pain and proprioception at this stage. Because the patient is still using the limb there is no juxta-articular osteoporosis, in fJct the bones arc sclerotic. The exccption to this rule is in patients with superadded infection, which is not uncommon in diabetics. Repeti tivc trauma leads to destmction, dislocation and dcfomlity \\irh multiple loose bodies within the joint. The likely underlying pathology depends upon the site of the joint and the age of the patient. When considering the ankle and foot, thc most common causes in adults arc diabetes mellitus and alcoholism, whereas in children the most common causes arc myelomeningocele and congenital insensitivity to pain. Examples arc shown of a Charcot joint secondary to diabetes mellitus ( 138b ) and a Charcot elbow in a patient with syringomyelia ( 138e).
  
  246
  
  Radiological features of a Charcot joint include: Dense (i.e. sclerotic) bones. Destmction and fragmentation of articular Degeneration. Debris (loose bodies). Dislocation. Deformity. Joint cffitsion. Exeessin:callus formation.
  
  Practical tips Look for vascular calcification on the radiograpr will otten be present in patients with diabetes hence point to the underlying eausc. Fu rther management Trcatment is stl.pporril'e. Ampuration may be severe progress101l.
  
  Musculoskeletal Imaging
  
  Case 139
  
  247
  
  Answer 139
  
  Musculoskeletal Imaging
  
  ANSWER 139 Observations (139al Irregularity, sclerosis and loss ofhcight of the right femoral head arc seen on the pelvic radiograph. There is no signitic:l!lt abnormality ohhc acetabulum and the lefi: hip is unremarkable. The findings arc consistent with avascular necrosis of the right hip. Bilateral sacroiliac joint fusion is also present, though more prominclH 011 the riglH side. This suggests a background seronegative arrhropathy. It is therefore likely that the avascular necrosis is drug induced by treatment for the seronegative arthropathy, or perhaps associated inflanllllawrv bowel disease. No bowel abnormality is seen on tl;is plain film [0 confirm the latter hypothesis
  
  Diagnosis Avascular necrosis (AVN ) in a patient on steroids for seronegative arthropathy. Differential diagnosis For causes of avascular necrosis (mnemonic - ' DRIED HI P' )' • Diaoctes and other metabolic conditions, e.g. hypedipidaemia, gout, pancreatitis. R.adiotherapy. ' l;ltlammatory disorders, e.g. rheumatoid, SLE, scleroderma . Endocrine disorders, e.g. Cushing'S. Drugs, e.g. steroids, anti-inflammatory and immunosuppressi\'e drugs, alcohol. Haematological disorders, e.g. sickle cell, hacmophilia, polycythaemia, Gaucher's. Infection and injury, e.g. fractures, burns and f.1t embolism. Penhe's disease (idiopathic AVN in children ). Discussion Avascular necrosis is a consequence of interrupted blood su pply [Q bone with death of cellular c1cmclHs. The many causcs arc listed in the difierential diagnosis and folloll" the mnemonic 'DRIED HIP'. The femor.tl head is the most common site at'tected. Other common locations include the humeral head and femoral condyles . The earliest radiological sign is subtle relative sclerosis seCOnd;H)' to resorption of surrounding bone. A radiolucent crescent par.tllel to the articular surface may appear. Flattening, fr.tgmentation and sclerosis then ensue. Subchondr.tl cysts and collapse lead to early osteoarthritis of the aflccred joint. An AP afthe pelvis ( 139 b ) shows the subtle crescent sign in the !cft hip of a child with early avaseubr necrosis. This is mo re clearly seen on the frog lateral vicw of the sallle patient ( 13ge).
  
  248
  
  Musculoskeletal Imaging
  
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  transplant kidne y.
  
  Further management The underlying cause should be sought and treated. Man ~' patients will de\'elop debilitating second ary arthritis and go on to require replacement of the affected joint. with steroid theral')'. e.g. thumb-printing presence ofa SrDm3 or sacroiliitis
  
  249
  
  Answer 140
  
  Musculoskeletal Imaging
  
  ANSWER 140 Observations 1140a)
  
  Dis cu ssion
  
  T here is diffilSC osteosclerosis 3Hccting the \'isibk skeleton. There is also ll1:tssivc splcllot1ll!galy making the likdy (iiagnosis That of myelofibrosis. Lymphoma is another possibility and less !ikcly, m:lslCX:YlOsis.
  
  This rnrc1oprolifcrativc disorder results in progfC'1ll marrow n:placcmcm by tibmsis ;lIld consequent an extramedullary h3cm:lTop0ics;s and sp1cno mcga[ ~' massi,'c). T~vical age ofansc! is O\"er 50 )·cars.
  
  Diagnos is
  
  Practical t i ps
  
  r.lyclofibrosis.
  
  • Osteosclerosis is on-en missed when the appcaranct blamed on the qU31ity orthe film, i.e. when it is thought to be due to the I';1diograph being underpellCrrJted. Assessment of the intervertebra discs should be made - if the" arc visible then the: :lppearanee is likcl~' to be real. This is well demonstrated in Figure 14 0e: there is diffuse osteosclerosis aflcctin g all the bones on this exit patiellt with osteopetrosis. Note how the intervertebral discs arc clearly seen, reinforcing: f.let that the radiograph is Ilot underpcnelratcd. P.lticnrs with renal osteodrsrrophy may ha\'e a haemodialysis line on CXR or peritoneal di al~~ on A.XR. There may be evidence of subpcrioslcM bone resorption, soft tissue and vascular calci and 'nigger jersq" spine. When sclerotic met"JstJses 3Te suspected on a cheek rhe bre;lsr shadows: in female patienl5 may be 3 mastectomy, while in males enlarge breast tisslie Illay be seen due 10 hormone th prostatic carcinoma, Figure 140d shows sueh-:- sclerotic prostate lllet3St:lses and gynaeco
  
  Differential diagnosis Of dimISI: bony sclerosis and splenomegaly: • Lymphoma.
  
  • M:lStocyrosis. Of generalized osteosclerosis in ;Idults: • Sclerotic metastases (es pecially breiS! or prostate carcinoma).
  
  Lymphoma. • A'lyclo/ibrosis.
  
  • Paget's disease (140b). • Renal osteodystrophy. • Sickle (ell disease. M;lstocrtosis. Osteopetrosis. • L~rknodysostosis • Fluorosis Osteopetrosis and pyknodysosTOsis have onset in the paediatric age group.
  
  140b Axial CT image of the pelvis shows sclerosis of the right hemipelvis with cortical thickening and thickened internal trabeculations typical for Paget's disease.
  
  140c CXR of an adult with osteopetrosis demonstrates diffuse dense osteosclerosis.
  
  250
  
  Musculoskeletal Imaging
  
  c splenomegaly on the AXR suggests -brosis atrophy (possibly with calcification ) sli ggest's cd] disease, and there may be mher signs of this ~ gallstones, avascular necrosis of the femoral .Ind cndphuc inf:lfClions causing H -shapcd I bodies. megal), and small bowel thickening suggest ·'osis. the" AX R of a patient with osteopetrosis a izcd 'bone within bone' lppcar.mcc Illay be md the \'encbral bodies may h;1\'( densely 'c cndplatcs producing 'sandwich \'crtcbr:J.c'. (,"cr flask dcfarmin' of the femurs should also cd for. Figure 140c is an orthopamomogr.un ad ult patient with osteopetrosis demonstrating osteosclerosis and supernume rary teeth '5 disease c;m also produce a 'bone within bone' nee, howe"er coarse trabeculatio n and cortkal ning normally diflcrcnti:lle this from other
  
  Case 141
  
  for spleniI.' enlargement that causes rC\:llrrc nt painful episodes, SC\'\:re thromlxxytope ni3 or an llnaccepmbly high red blood ce ll transfusion requirement. Median survival from time of diagnosis is approximatel y 5 years. End stage myelofibrosis is a wasting illness characterizcd by gencral disability, Ii,'cr failure and bleeding from thrombocytopenia.
  
  is is associated with ligamentous insertion
  
  management is no specifi c rreatillem for myelofibrosis. omy is not routinely performed, but is indicated
  
  140e OPG of an adult with osteopetrosis; this demonstrates diffuse osteosclerosis and supernumerary teeth, which is an associated finding.
  
  251
  
  ANSWER 141 Observations (141a) There is a large, cxpansilc, mixed lytic/sclerotic destruct!,'C lesion involving t he right anterior hcmipclvis. There is marked cortical destruction with a soft tissue component. The lesion has a wide zone of transition. This is an aggn.:ssi"c lesion and the most likely diagnosis in a patient of this age is a primary malignancy such as lymphoma or chond rosarcoma. Metastasis should also be considered.
  
  Diagnosis
  
  greater than I.S cm then malignant transformation be suspected. Again, flocculent chondroid cakificao characteristic tor malignant degeneration.
  
  Practical tips It call otten be diflicult to diflcrentiate an e ncho from an intr;\medullary chondrosarcoma, howc'a" larrer is more likely to present with pain and enchondroma will often demonstrate periosteal and cortical bre.\kthrough.
  
  Chondrosarcoma.
  
  Further management Differential diagnosis • Lymphoma • Metastasis.
  
  Discussion Chondrosarcoma is the third most common primary bone malignancy with multiple myeloma being the mOSt common and osteosarcoma following second. Chondrosarcoma ca n be primary, or secondary following malignant translorrnation in ;1 pre -existing skeletal lesion such as an osteochondroma, enchondroma or a parosteal chondroma. C hondrosarcoma most commonly presents in the 4th or 5th decade of lite with a male predilection of 2: I. Radiographs typically re\·cal a mixed lytic and sclerotic appcarance. The scleroric areas represent chondroid matrix mineralization and arc seen in 60- 78% of lesions. The characteristic appearance of mineralized chondroid matrix is a 'ring and arc' pattern of calcific:uion that can co..11esce to form 'snowflake- type' calcification. T his characteristic chondroid calcification usually ;\lIows confident radiological di;\gnosis of a cartilaginous lesion. R.,diological signs· Mi xed lytic/sclerotic lesion. Wide z:onc of transition. 'Ring and arc' and 'snowflake' calcification. Cortical destruction. Soft tissue mass. C hondrosarcomas arc also characterized as central or peripheral. Central chondrosarcomas make up the majority and arc intramedullary in origin, although they may erode through the co rtex into the soft tissues . Central chondrosa rcomas lIsuall y arise in the pelvis or lemur and arc often expansile with the characteristic calcification described . Endosteal scalloping is often seen and call help diflcrcntiate low-gr;\de chondrosarcomas from enchondromas. Clinical sympwll1s arc nonspecific, with pain being the most frequent symptom . Peripheral chondrosarcoma is also termed exostotic chondrosarcoma and refers to malignant degeneration in an cxostosis (i.e. is a secondary chondrosarcoma). An cxample is shown in Figurc 141 bnote the flocculent calcification of the chondrosarcoma, which had arisen from the exostosis at the lateral femoral condyle. This should always be suspected when there is growth o f an exostosis after skeletal maturity or if an exostosis becomes painful. The cartilage cap of a suspicious exostosis can be measured 011 M lU, and if the thickness is
  
  252
  
  As with most primary bone t um ours, a su chondrosarcoma should only be biopsied in a bone tumour centrc so as nOt to seed tll contaminate the su rgical field.
  
  Further reading Murphey lvi , Walker E, Wilsoll A, et at. (200 3). of prim.\!)' chondrosarcoma: radiologic-path correlation. /{ndioGmpbics 23: 1245- 1278.
  
  141b Flocculent calcification from a chonrlrosarcoma that has arisen from the exostosis at the lateral femoral condyle.
  
  Case 142 A 45-year-old male presented with severe back pain for several weeks.
  
  253
  
  Musculoskeletal Imaging
  
  Answer 142
  
  ANSWER 142 Observations (142a, 142b) AP and hlteral images ( 1423 ) ofthc thoracolumbar spine shol\' marked deformity at the TI2/ LI level with erosive destruction of the opposing ,"enebral endplates. There is anterior slip at this level with anterior angulation (gibbus deformity)_ Appearances arc in keeping \'ith a discitis. Axial and coronal T2 weighted MR images ( 142b) demonstrate high signal in the TI2/ Ll intel"Yertebral disc. Signal change extends throughout the adjacent ,·ertebrae, and there is endplate destruction and partial vertebral coll:tpse at T12. Til ,·ertebra has increased marrow signal suggesting it is also in\"ol\"ed. The axial image demonstrntes high -signal fluid collections in both psoas muscles consistent with bilateral psoas abscesses.
  
  Diagnosis Tuberculous spond~'litis ( Pan's disease ) with associated psoas abscess.
  
  Di scussion The spi ne is the most common bony location to be in,'olved in TH. Presentation is often late since initial sympl"oms :are of ,"aguc back pain and stiffness. The most common location IS the upper lumbar/ lower thor:acic region, particularly around L I leyel. The anterior aspect of the venebral body is mOSt typically aftectcd. The disk spacc thcn becomes involved via cxtension along the anterior or posterior longitudinalligamelll or directly through the endplatc.
  
  R:adiological features of disci tis include: Reduction in height of the intervertebral disc, is usuall~' the first sign of a disciris. Erosion of the "ertcbral endplates. Involvement of the vcrtebrallxxlies leads to and resulting gibbus deformity. Spread of infection into adjacent soft tissues in psoas/ paraspinal abscesses. • Neurological im'ol\-ement arises due to intraspim. spread of infection and vertebral body colla~ occurs much more commonly whe n the infecri"fo_ organism is TH.
  
  Practical tips • Discitis will cause bon)' abnormality of the su and inferior endplates of the adj:acent ,·enebral i.e. abnormalitv will be centred at" the level of • Thc vertebral disc space is m:aintained longer ill. spondylitis than in pyogenic discilis. • Look at the paraspinal regions to look for s infection. Infection can present as leg pain/s-duc 10 rracking of collections dOWI1 the iIi muscle. • Look at, and around the spinal cord for ex collcctions :and spinal in\"olvemem.
  
  Further ma nagement Treatment is with antituberculous medication. of associated psoas or epidural abscesses may be
  
  142b (left) Vertebral body collapse w ith abnormal marrow signal in the vertebral bodies above and below it. Bilateral psoas abscesses (right ).
  
  254
  
  Musculoskeletal Imaging
  
  Cases 143, 144
  
  male presented with hand following :J.
  
  255
  
  Answer 143
  
  Musculoskeletal Imaging
  
  ANSWER 143 Observations (143a) There is a bilaterul symmetrical erosive arthropathy ltlecting the distal interphabngell joints. BOlle density is preserved and there is 'pencil in cup' ddormity with bony proliferation. At the left little finger there arc signs of ankylosis of the distal interphalangell joint. The appearul1ces lre consistent with psoriatic arthroplthy.
  
  Diagnosis Psoriltic arthropathy.
  
  Differential diagnosis • Ankylosing spondylitis. • Rheumatoid arthritis (RAJ.
  
  Discussion This is a seronegative arthropathy aflecting synovium and ligal11entous attachmcnts, and aflects fewer than 5% of psoriasis pltients. In approximately 15%, the arthrop~1thy Cln predate de\"clopmem of skin changes by SCI'aal years The pattern of disease is \'arilble, though the case illustruted is classical, i.e. asymmetric erosive oligoarthritis aflccting the distal joints of hands and feet. New bone formation is charucteristic, and Illay i"esult in ankylosis of interphalangcal jOllHs, as in this case. Resorption of the distal phalangcal tufts nu y Ix seen with accompln)'ing nlil changcs. Spondylolrthrop;lthy is another pattern of disease, often with sacroiliitis and parul"ertcbrul os.~iticltions. Other disease paTtefllS include symmetrical pol\'arthritis mimicking rheulllatoid, lllotloarthriris and arthritis mlltilans, a grossly destructivc pattcrn that may progress to forlll 'opera glass hand'
  
  143b Radiograph of bot h hands of a patient with rheumatoid arthritis. Bilateral symmetrical erosive destruction is present affecting the metacarpophalangeal joints and the wrists. Note the telescoping of bone ends and the periarticular osteoporosis .
  
  256
  
  Practical tips When presented with a hand radiograph with C'\ of erosil'l' arthropathy, certain diflt~remiatill g can help identify the most likcly aetiology. T he mdiological differentiating learures of the more common crosive arthroplthies arc as tallows: • Psoriatic arthropathy: - Usually (/IyJllmctrical aosh"e. - lntaphalangeal joints, particularly the dist:a. interphalangeal joints, aTe a!lecred. - ROilY prolijcrfltioJl/ periostcal reactioll ani prcsCJ1'cd bom dCllsit'ycharacrerisric. - 'Pencil in cup' ddormity - Ankvlosis . It' - Bilateral symmetrical crorlJ'e arthropathy. - Metacarpophalangeal and proximal interphalangeal joi!HS affected, Le. prOD JIIlfll/joj'ltsojdigilJ. - Margillal erosions. - Subluxation with ulnar deviation of digiD. -
  
  Decreased bone demity.
  
  - Ankvlosis. . - Figure 143b is a rudiograph of both patient with rheumatoid arthritis bilateral synllnetrical crosil'c dcstructX.. llletacarpophabngeal joints. There is genemlized decreased bone density and arthropathy at rhe carpal joints and rail. joints. Figure 143c sholl'S similar ch _ feet of a rheumatoid patient with m~ erosions and symmetrical subluxation .. metara.rsophalangeal joints bilaterull~
  
  143
  
  Musculoskeletal Imaging
  
  • Gomy arthropathy: - .\symmetrical crosh'c arthrop:llhy. - Erosions arc juxta-articular with DVttiJnllgj"g
  
  ("gu. - Preserved bone density and joint space. - Bony proliferation/periosTc:ll reaction. - Goltr) tophi causing sort tissue masses in 50%. - Figures 143d and 143c arc radiographs of goU[ de mo nstrating an asymmetrical eros;ve arthropath y with 'punched out' erosions. These ~rosions have o\'crhanging edges, which arc best seen at the proximal imerphalangeal joints orthe index fingers bilaterally and the mcucarpophlbngcal joint o f the right thumb. Further hrgc 'punched out' juna-articular erosions with associated tophi arc seen around the base of the right big toe in particular. Note the preservation of bone density.
  
  •
  
  It is impossible to distinguish thc sponrlyloonhropathy of psoriasis from Reiter's syndrome though it is notable that in psoriasis the hand is most affected and in Reiter's, the foot. Spond)'lo.1.rthropathy of psoriasis is diflcrcnt r:tdiographic~lll)' from ankylosing Sl)()nd)'litis - thc paravcrtebral ossification is asymmetrical and nOT d uc to true s)'ndesrnophytes.
  
  Further manage ment Givcn thc inflammatory nature of the disease, treatment consists of anti -inflammatory and immunosuppressive drugs as for other infl3mmatory anhrop:lthic.::s.
  
  Radiog ra ph o f both ha nds d e m o nstrates , 'punched out' e rosio ns w ith overhangi ng and associated soft tiss ue swelling in a patient gout.
  
  '43e Radiograp h i gout with characteristic 'punched o ut' erosions aff ecting the big toes.
  
  257
  
  Answer 144
  
  Musculoskeletal Imaging
  
  ANSWER 144 Observations (144a) Il:adiogr.:tphs of the lett hand fCveal ::I lucent cxp:msik lesion ofthc diaphysis ofthc 5th Illcrac:upal. There is thinning of the cortex but no conical breakth rough or periosteal
  
  reaction. No pathological fracture
  
  hlS
  
  occurred and no
  
  fraCl11rc is seen elsewhere. The lesion has a narrow zone of tr:l.l1sition and nonaggrcssivc fcamrcs and is consistent \\ith
  
  Pract ica l tips Chondroid pattern calcification, onen seen in enchondroma, is howe,"er frequelllly absent in of the tubular bones of the hands and feet. A painful enchondroml without pathological should be suspected of having undergone m tr:lnsformation IIntil pro\'en o therwise
  
  an enchondroll1a.
  
  Diagnosis Enchondroma.
  
  Further manag ement There is no specilic treaTment for enchondronu .. benign bony le~ion That is oflen picked up inci when a pathological fr:lCture occurs.
  
  Differential di agnosis For lytic lesion in the digits (mllemonic - 'SEGA GAME 1"), • Simple bone cyst - r;uc in the hand. Enchondroma is by fur the most likely lesion with this appcar;l1Iccat this site. <.Ibnt cell ttlrnQUT of tendon sheath. Ancun'smaJ bone C"Sf - mrc in the hand. GIOllll'IS tumour -I)ainful. Abscess/ osteomyelitis. Mcmstascsalldllwc!oma. Epidermal inclusion cyst (imr:l.Osseous) - distal ph3.bnx; history oftr:luma, e/,\·tlu:m3. 3.nd swelling m3.\' . . be prescnt. Fibrous d~'Splasi:J. - r:'Lre in the h3.nd. Di scussion Enchondroma is a common benign eartibginous tumour most collllllonly seen in the tububr bones (50% in hands and feet ) ofpatiellIs under the age of 30 years. The C:J.Tdinal radiologicallCalmes arc listed below: • Centr:'Lllesion within the medullary canal. • LueelH expansion of bone. • Narrow zone oftr:lnsition. • Thinning of the cortex but no cortical breakthrough. • No periosteal reaction unkss pathological fr:'Leture. • Stippic(] calcific;ltion llKI)' be presem • Cortical endoste;\1 scalloping. • Alleets small tububr bones. Oilier's disease occurs when multiple enchondromas arc present ( l 44b). The enchondromas arc most commonly see n in the (emur, tibia and hands in an asymmetrical distribution. In the long bones, presentation is with asymmetric limb shortening due 10 impairment of epiphyseal fusio n. The lesions do nor incre3.se in size after ccss.lrion of normal growth. Malignant rr:'Lnslormarion to chondrosarcoma can occur in up to 25% of patients with Oilier's disease by the age of 40. Malignant change is more common in centr:'Llicsions and should be suspected when there is abnormal cominued growth with pain 3.nd swelling and /or imaging findings of growth in a pre\'iously stable lesion, bony erosion and new or increasing calcification "·Ialli.lcd's s)'ndrome describes multiple enchondromas wi th multiple soft tissue haemangiolll3.s, which presem on imaging as multiple phlebolilhs in the soft tissues. It prescnts bl"cr, usually after puberty, and has a much higher incidence ofmalignalll tr:'Lllsfonmuion.
  
  258
  
  144b Multiple lucent expansile lesions of the tubular bones of the hand represent e nc hondromas in this patient with Oilier's disease. Note that the absence o f ph leboliths excludes the main d ifferential diagnosis, Maffucc i's syndrome.
  
  Musculoskeletal Imaging
  
  Cases 145, 146
  
  presented wilh heel pain.
  
  259
  
  A nswers 14 5, 146
  
  Musculoskeletal Imaging
  
  ANSWER 145 Pract ica l tips
  
  Observations (145 ) Tllerc: i ~ diflllsc- osteoscicr(>sis w it h J 'bO lle wi {hiLl hOll e' ,1Pl'C.'1rJL1ce, The most likely cii.lgnosis is ostt'opelros is,
  
  Dia gnosis Osteopetrosis cau sing a 'bone wit hin hellle'
  
  ap~~Cdr;Ll1Ce,
  
  Different ia l di ag nosis Of ' bone wit hin bOll e' appcJr;lIJcc (mJ1C'lll o nic ,- 'SHA RPS POOL ') : SicklC' cdl ,iiscasc, .. H ypervil~ Lllinosis D, • /\cromegaly, '" Rickets, '" r;lget's dis n~e Sc nrvy'" Post- radi,llioJl tberapY/Lh ol'Ouast/hell'Y metal <)
  
  Q
  
  G
  
  poiso ning, C)!'ieope tfos is ;md 0\;]1 05i5,
  
  '" Osteol11vc liti.s, '" LeL1kaem id,
  
  Di scussion ' Bone \vithin bOll e' clcsc ribcs til,' r.ldi o logi cal ,1 ~'lxar .mcc ",hell' o ne bone ,1l' ~' CMS w ari,~ with in al1orh<:r , lL Cc\1l be seell ,IS P;UT ofnofnd dn'c lor 11l C\1t, espeCi ;llly in th c spinc, G I'c>wth ,\I:r esr may ;ll so C;HISt' xb e ,lppnr:1I1ci:: hlll' tile re ;He 5('\,('['.11 p,ltholos,iul ,all SCS as lislt:Ll , The di(Tll sc os teosc lerosi s ;md :lgC o l'the pClticllt in rhi s (~\St' 114;; ) ],oilll to t llC correct di ,l g ll os i ~ ,
  
  The di ltcrt'11ti ,11 di ag nosis el 'bOI K \l'il'hin hOl K' <1lll),',d l-an cc is 1;1]-ge blll rh ere MC (('rr~ il\ fe ,l lllrcs lil e Llilderkin .. di s(:a~e ~'r oc('ss that lll~ y poim 10 tile specitic Cduse: ~ O s r eo ~)nl'os is l1uy 11;1\'(: ErknnKyn' ihsk ddnnnit\' oj the (' nd s of long bones and sclerotic vULL' bral ' endplalt's producing \;llJci" ie h vcrl'c brJt", ., T here lllay he ;m c iJl,llY Sigll<; of~ idd e (e lJ C\i:'e,lse Oil the radi ugr:lp h sll ch as ;\\',lSC\t! ;lr n t'crm is of Ie men,)" and hLim era l he.1ds, gdlbtollCS, H -s lu pcd vc:nehrac du e rn en dphtc: intar([io l\ ,111d sl,lcnic arropliy Ol' ca\C iti c ~tivJl , e Acromq;'l ly is ,w:;oci;1tcd WiLh rCClangular-s h
  or
  
  Further m an ageme nt The o lll v t reaimelli ,\\':lihb le Jor oqeopetfosis is bon e Jl1.\lTOW 1r.1 1l " I '1.1J\t~lljOlL 1 );J[jel1r~ .1re ))lore pro n e rn Tr;lctl1reS rh dll th e nonn .lll)(>PL1LHitllL
  
  Further re ading VVi lliam s H , Da\ies l\ , C hap man S (2004 ), HOll c wiThin J hone', CIiIi1I:t7! }{,:r-iiirJ/ogr S9: 132 - 1.44 ,
  
  ANSWER 146 Observation s (146) "Vithin th e bu,iv oC lhe C;t!c'1J1 t llS thne i ~ 'J \\'d l ' LlcJll1cd ILlc~ nt Jesio L1 \\'i~ h.l Ilarrnw 'Z,(lne nl lTansi liOll , Th e les ion h,1 S ::t vcr y rhill sc leroti c bo rdt' l' ,1 11ci celllrJ ll v w itiJin the lesi,o ll i, a ( lu mp of L~ ;li ci tJ C ar l O n, Therc is 110 l'c ri os Lc.ll l'e;1Crion ur o verly ing soft [is~uc m ;lSS, T he lesion h,1 ~ ,1 n O IJ~ggre ssiv e :tppeJl'<1l1ce dnd rile findillgs .liT n "p ical o t ;lll iniLlOSStC1l1s lil' O Ill ~L
  
  Dia gnosis In traosscous iipolll J, Differenti al diagnosis e
  
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  l1ni ca l1lnal hone c"st POsL -tfa Ulllari c CIf<;\' , Gia11l ce ll fl 1111 0 11i' \ GeT) , D es moplastic Ii brOIll ,oL
  
  illtraoss(ouS li poma, "irh llO ge nrln I)lcdilcni oll , Thert' is an assoc iari l)n w ich hypc rli popro[c in~eLlli.l, TIle: n'p i("i radi o log ic,ll Je-arllr,s are ;1/1 eX I'3 ns iJe , n Oll agg l-cssiYe r;)d io lu,'(L1r Icsioll \I,ill!
  :\11
  
  intraosseom lip o \Jl;l
  
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  Di scussion is t il e lli OSC CO lllDl ClLl IOCl rioll l() r all iiltI;lOSSCOlI S lip o mJ Ori1n sire' S o t inl' o lw:menr inclu d e rhe proximal te lllUL' , tib ia, hlli ll c ru~, pe lvis , 1ll.1l1,li bl. c alld vend,]'«c , \Nhcn in\'o h' illg tllbllLtr bo nes, ir is Il <; l\.11 1\, loca ted at rhe IllCUp l l\',~ i ' . P,lli" lltS L,f an y ag,c ill.1\' dn 'eioi,
  
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  260
  
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  Furth er management T his i~ ,\ 1X: llig ll bt' ll )' Inil)ll i, n ccc ~<;,lI'\"
  
  and
  
  I ll) lll nil,r rn ,ll);l gern fnt
  
  MuSculoskeletal Imaging
  
  Case 147
  
  CASE 147 History ~ .lO·}~,,·'~,1
  
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  Answe r 147
  
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  MuSculoskeletal Imaging
  
  107< Rod;"g"ph ollne 1001 io 11>0 .. me po,;enl os in
  
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  ANSWER 148 Observations (148a)
  
  or
  
  There is an area of dense sclerosis aflccting the shaft the femur with associated cOrtical thickening. Within this is an area of ccmral111cency. This likely represents a nidus and the tcatufcs arc therefore consistent with all osteoid osteoma. Depending 011 other clinical features, infection might be considered as a diHcrential diagnosis.
  
  Diagnosis Osteoid osteoma.
  
  Differential diagnosis • Infixtion. • Eosinophilic granuloma.
  
  Discussion Osteoid osteoma is a rclati\'cly common, benign skeletal neoplasm composed of woven and osteoid bone, with loose intervening fibrovascular tissue. The lesion itself rarely exceeds 1.5 em in maximum dimension and there arc three main types: cortical, cancellolls and subperiosteal. Cortical lesions arc the most common and have characteriseic findings. Radiographs show dense reactive sclerosis that aHccts the sha'ft of a long bone, especially the tibia alld femur. The lesion itself, howc"er, is a radiolucent area within this area of osteosclerosis known as the nidus. This is more clearly set::n on tht:: CT images of the same patit::nt (148b ). Cancellous osteoid osteomas have a sitc predilection for the femoral neck, posterior clements of the spine and the small bones of the hands and feet. By comparison, the sclerosis associated with a cancellolls lesion is usually mild
  
  or moderate and may be distant from t he lesion. C the classical cortical osteoid osteoma, the cancellous may not necessarily be situated at the centrc oj sclerosis, making treatment more diflicult. The subperiosteal type is rarest, and typically I the medial aspect of the femoral neck or in the h~ feet. It produces a soft tissue mass immediately adjao:::a the affected bone rather than osteosclerosis. Can ... and subperiosteal osteoid osteonl<1S typically arise intra-articular or juxta-articular location. O steoid osteomas most commonly occur in efl(and tibia, where they arc usually diaphysci metadiaphyseal. The most commonly affected area spine is the neural arch of the lumbar spine ..\;: locations include the skull, ribs, mandible and Intra-articular lesions arc most commonly found in The affected population is young, with abol.. presenting between the ages of 10 and 20 years._ all patients arc Caucasian with a male prcdominaocc is the llsualmode of presentation and is worse at is thought to be related to the vascularity of the Symptoms may be present before the lesion is ica11), visible and approximately 75% of patients repmof pain after salicylates. CT can aid plain radio_ identifying tht: nidus. When the history is a~ location unusual or radiographs unexpected l~ bone scinrigrapby can be very helpful. The finding is a 'double dmsit}" sign, in which I area of intense radionuclide activit), w'","'on,1inr nidus, superimposed on a second larger area tracer accumulation due to reactive sclerosis.
  
  148b CT reconstruction in the same patient clearly shows the lucent lesion with surrounding sclerosis and central nidus.
  
  264
  
  Musculoskeletal Imaging
  
  Case 149
  
  Fu rt her reading
  
  cr scan to confirm and
  
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  suggest 3. the nidus.
  
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  Gangi A, Guth S, Dictclllanll j, Roy C ( 2001 ). Interventional Illuscu[oskclctll procedures. RndioGrnplJics 2 1(3): I. Knnsdorf ,\>I, Stull M , Gilkey F, Moser R (199 1). Osteoid osteoma. RndioGrnpbjCJ 11 (4 ): 67 1- 696.
  
  pat'icnt's::J.gc arc strongly an isotope bone scan if
  
  and CT arc unrc\·caling.
  
  of com pi ere rClllO\,:t1 of the nidus. treatment is su rgic31, however lllany cases 3rc
  
  ted radiologically wi rh CT·suidcd laser ublion. Greater than 90% Sllccess rates arc ";;th this technique.
  
  265
  
  Answ er 149
  
  Musculoskeletal Imaging
  
  ANSWER 149 Observations (149a) There is all extensive, cxpansik [liceill lesion involving the length orlhe lett humerus, pn:dominJntl)' the mctlphyscs and diJphysis. RClllo
  Diagnosis Fibrous dysplasia.
  
  Discussion Fibrous dyspbsia is a benign tiura-osseous dc\-c!opmcllt'al :l1lolll.l1y of bone whereby the medullary cavity becomes replaced by an im l11atlll'c matrix of .:ollagcn and \\"OI'CI1 bOllc. Males and females arc equally affected and 75% of patients develop the disease belore Ihe age of 30 years. The monostotic form, which mOSI commonly lflects rhe ribs, proximal femur and craniofacial bones, accoullIs lor 80% ofcascs. Thc polyostotic fonn (i.e. in\'ohing more than one sire) is seen in.ll~c n:mai.ning 2o:',~nd prcdominlntl~' aflec~ the femur, tibia, pelvIS and 1;1clal boncs_ A subtyiX 01 the pol)'oSlolic form is McCune- Alb right syndrome , where polyostotic unil:tleral fibrous dysplasia is associlted with 'catc ·au -lait' SPOtS and precociollS puocrty in young girls. Radi ologicall~', a 'ground gbss' density !csion in the medullary cavity is the characteristic fcaturc_ Thc 111ct;Jphysis
  
  14gb Occipitomental radiograph of skull in a patient with leontiasis ossea demonstrating hemicrania I sclerosis and expansion.
  
  266
  
  is thc primary site of in\'ol\'ement with extension into diaphysis and the lesion may undergo calcitication_ The crlniofucial torm is termed leontiasis ossel hcmicranial, unlikc Paget's discase. Thc frontal sphenoid bones arc most commonl y invoh·ed . Sclet" overgrowth of the fucial bones and cllvari:t results in deformity, cxophthalmos lnd visull implirmenl obliteration ofthe sinuses. Sclcrosis of the skull bast nlrrow thc neurll toraminl clusi ng cranial s~'mptoms. Frontal and lateral skull radiographs 1 149c) of l paticm \\;th leontiasis ossca show these Ii Complications of fibrous dppiasia includc pat frncmre, and malignant transformation into osteos.t!i fibrosarC01l1l or malignant fibrous hisliocytoml in 1% of cases. Osteotibrous dysplasia is ;111 cmity that was Illis the past lor fibrOllS dyspJasi:t. It is almost cxcl conlincd to thc diaphysis of the tibia and is seen in children. The lpp~arallces are vcry simillr to dysplasia with a nonaggressi\<e e <~pansilc.lucent/ ·_ glass'lcsion. Enlargcmcnt ofthc tibia occurs \\ith bowing. As with fibrous dyspllsil, pathologic:t.l c0111monly occur. The lesion often rcgresses spon with age. An eXl1llpic is shown ( 149d ) that illus typical appearances ;11ld mid-di:lphyscallocation in", child. Th~ radiological featurcs of fibrous dysplasia ~ Lucent/'gro und glass' lesion in medullary CJ.\ • Expansilc.
  
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  Answer 149
  
  Musculoskeletal Imaging
  
  • ;\ lctadiaphyscallocation. Remodelling/alteration in bone architcaurc. Endosteal scalloping. 'Shepherd's crook' deformity when :aflCcting proximal femur. Limb length discrepancy. Pathological fractun:. No cortical destruction. Nonaggrcssivc features.
  
  Practical t ips
  
  Case 150
  
  149d Radiographs of the leg in a child demonstrate a lucenV'ground glass' lesion in the diaphysis, which has nonaggressive features and is typical of osteofibrous dysplasia.
  
  The appearances of fibrous dysphsia on'rlap with those of many benign bone lesions and it is reasonable to include it on rhe differential diagnosis list in many cases. As a result, • is often the firsl differential prmidcd by r:ldiolog)' trainees In a viva even when the lesion in question hlS characteristic katu rcs of something clsc~ Only if a lesion has the cturaCfcrisric features should fibrous dysplasia be the tirsl Jiagnosis mentioned - morc often, il should be further down the list.
  
  Further management There is no specific tre;lImelll for fibrous dysplasia. uhologieal fracUlres often occur and may necessitate urgieal fixation.
  
  267
  
  Answer 150
  
  Musculoskeletal Imaging
  
  ANSWER 150 Observations (150a, 150b) A'\:ial images oflhc wriSt with Tl weighting ( 1503) and T2 fur saruration ( l SOb) arc shown. Inflamed tcndon sheaths an: of high signal on T2 &t sattlr;lfCd images and low signal on Tl weighted images. In addition, there is palm:!f bowing of the flexor retinaculum. The median ncryc is of increased signal on the T2 weighted imlge. This combination of features is consistent with a diagnosis of carpal tunnel syndrome.
  
  Diagnosis Carpal tunnel syndrome.
  
  On M RJ thcse same findings can be appreciated: • Median nervc swelling proximal to the carp~ termed pscudoncuroma. Palmar bowing of the flcxor retinaculum. Featurcs of tcnosyno\"itis - which appear as signal on 1'2 wcightcd images around the fI tcndons. Increased signal in thc median nelYC on TI imaging. Median nervc enhancemcnt following contra injection is va riable and thcre can be marked enhancement due to oedema or absence of enhancemcmdue to ischae mia.
  
  Discussion Carpal
  
  W Hile!
  
  syndrome is a disease that arises due to
  
  chronic pressure on the median ncrve as it passl"S \\~thin the carpal tunnel, usually associ:ttcd with repetitive wrist Illovelllents. It is lllorc commonly seen in females with a ratio of 4:1 and is bilat eral in 50% of cases . Ustla]])' d iagnosis is Illade by cli nicians with positi\·e findings o n clinical examination , electromyelography and nerye conduction studies. lt1diological ex;;ullina[ions havc a roll.' in the few cases where these ilwestigations are inconclusi\"c. Ult rasou nd is usually the first examination undertaken althoug h M IU has been s hown to bc morc specific and sensitive. US findings arc of: • Akdian nervc swelling in the proximal mnncljlc\"cl of the distal radius. Nervc flattening in the distal nlllncl . Bowing of the flexor re tinaculum.
  
  Practical tips Assessment of nerve swelling can be made the size of the 111edbn nervI.' :It the level radius and at the ham:lte.
  
  Further management Methods to decrease oedema and swelling \\i c:lrpal tIInnel such as treatment of hypo thy " restriction of fluid illlakc can help relieve sy Howeyer the gold standa rd treatment for carpoa syndromc is surgical decompression .
  
  150b High signal of median nerv e on T2 weighted image (upper arrow); palmar bowing of flexor retinaculum (lower arro,;",).
  
  268
  
  Musculoskeletal Imaging
  
  Case 151
  
  269
  
  Answer 151
  
  Musculoskeletal Imaging
  
  ANSWER 151 Observations (151a, 151b) AI' and lateral Tadiogr;\phs of dlC lumbar spine show posterior \'cttcbr.tl sGllloping of the lower lumbar vertebrae \\'ith a narrowed a nteroposterior canal diameter. The AI' film ( 151a ) shows th:Jt the inrcrpcdicu lar dist:lIlcc is abnormally narrowed at L5 level and there is squaring of the iliac wings. The features arc consistent with achondroplasia.
  
  Diagnosis Acl1ondroplasia.
  
  Differential diagnosis O f posterior \'crtcbr:J.] scalloping: • Prcssurt" dlccr - tUIllOurS in spinal c:l nal, syringomyelia and cOllllllunicating hydrocephalus. Dural cctasil. This occurs in ncurofibrolll:atosis. Mar/;IIl's and Ehlcrs- Danlos. Acromegaly. Achondroplasia.
  
  Congenital disorde rs sllch as Illucopolysaccharidoscs, e.g. Morquio's syndrome. Discussion Achondropbsia is an autosomal dominant d isease of defcctive enchondr.tl bone formation relatcd to advanced
  
  151c Macrocephaly and bulging forehead on lateral skull radiograph.
  
  270
  
  p;uemal agc. Those \\1th thc homozygous form still born or die in thc neonatal pcriod. Tho hcterozygolls :tchond roplasia can h;l\"e a long we normalill1elligcnce. R.1diologicll fc:tturcs to look lor includc the Macroccphlly with bulging forehead ( 151 c narrow fora men ntlgnum ma~' be associ.uc-d hydrocephllus. Small J-shlpcd sella due to flarrcnin g ofru sellae. Short flared ribs. • llc)stcrior vertebrnl scalloping. • Anterior-inferior \·enebr.tl body beaks. • Short pcdiclcs and caudal narrowing ofintc distance (this should normally increasc in the dircction ). These abnormalities can Icad to stenosis. Squared ilbc wings. 'Champagne glass' pchic inlet ( 15 Id ). H orizontal sacrum. Shortening and bowinS of long bones with.l 'trumpet' appearance due to disproportionate mel"Jphyseal flarin g ( 15 1e). Brac hydactyly leading to short smbb), fingers I ~
  
  151d AP pelvis in an achond ro plastic typical 'champagne glass' pelvis with iliac wings. The interpedicular distance nar caudally and the sacrum is horizontal in orien and difficult to visualize.
  
  Answer 151
  
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  Answer 152
  
  MusculoskeletlOllmllglng
  
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  Musculoskeletal Imaging
  
  Cases 153, 154
  
  CASE 154 History An 15-yc3r-old male presented with
  
  back pain.
  
  273
  
  Answers 153, 154
  
  MusculoskeletlOllmllglng
  
  ANSWER 153 Obsorvatlo nsl153)
  
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  Musculoskeletal Imaging
  
  tips ttncbral body affected has a uniformly flat 'coin edge' 3ppcarancc. ;-encbral discs arc typically spared with normal ~ccs.
  
  posterior clements arc spared. generalized Os[copcnia suggests osteogenesis
  
  Case 155
  
  from binh. Pbtyspondyl)' may be generalized, aflixting all
  
  the \'crlcbral bodies, multiple, affecting some but not all the vcrtcbrnl bodies, or localized, in\"o]ving just one \·crtcbn.l body.
  
  Further management The underlying caUSe should be determined ifpossibk. Eosinophilic granuloma is the most common cause in children and usually n:sokes spontaneously wilh age.
  
  \'ertcbra plana is the te rm used to describe of a previously normal vertebral body, refers to flattening of the venehral bodies
  
  275
  
  Answer 155
  
  Musculoskeletal Imaging
  
  ANSWER 155 Observations (155al
  
  Practical tips
  
  Three well defined densities in the right uppcr quadrant 3TC projected outside the comour of the right kidney and arc likely to represent gallstones. There is mild, diffuse osteosclerosis and increased trabeculation of the bony skeleton with I'encbral cndplate infarctions causing H shaped lumbar vertebrae. Sclerosis and flancning ofthc left femoral head suggest avascular necrosis. The spleen is noted to be atrophic :md calcificd. Ol-crall the features arc consistcm with a diagnosis of sickle cell disease.
  
  Ancillary signs of sickle cell disease on the radiogt2l' depend on the area ofrhe body imaged: • Abdominal radiograph: H -shaped vertebrae due to endplate infurctiom.. Avascular necrosis ofthe femoral heads causim!: flattening and fragmentation. Gallstones (secondary to haemolytic anaemia Splenic arroph)' and calcification . Renal papillary necrosis may be caused by siekkcell disease due to slo ughing of papillae from infurcrion; a sloughed papilla mal' be seen widm: the renal calyx (on an IVU) ( I SSc).
  
  Diagnosis Sickle cell disease.
  
  Discussion Sickle cell disease is an inherited disorder mostly seen in Afro-Caribbcans. The sickling arthc red blood cells leads to increased blood viscosity, occlusion of small I'essels and bone infarction leading to necrosis. Chronic haemol~,tic anaemia also ensues. Marrow hyperplasia leads to coarsen ing of the trabeculae and may cause diffuse osteosclerosis. A CXR in a l7-year-old female with sickle cell disease is shown ( I 55 b ). This demonstra tes diffuse osteosclerosis with H -shaped thoracic vertcbrae (due to endplate. infarctions) and cardiomegaly (secondary to chronic anaemia ).
  
  155b CXA in a patient with sickle cell disease demonstrating diff use osteosclerosis with cardiomegaly and Hshaped thoracic vertebrae.
  
  276
  
  Chest radiograph: H-shaped vertebrae due to endplate infurniom. • Avascular necrosis of the humeral heads • Cardiomegaly (due to chronic anaemia). Skull radiograph: 'Hair on end' appearance of skull vault due marrow h)'perplasia. • Widening of diploic space.
  
  [0
  
  Further management Treatment is genera lly supportive with m transfusions being necessary. There is a high ineid infection of bone and lung. Skeletal pain can occur only from osteomyelitis but also from bone inf..1rction
  
  MuSculoskeletal Imaging
  
  Case 156
  
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  Case 158
  
  CASE 158 Histo ry .\ 14 -ycar-oJd male presented with .mll pain.
  
  281
  
  Answ er 158
  
  Musculoskeletal Imaging
  
  ANSWER 158 Observations (158) Within the midshaft or the diaphysis of the humcms there is a rdativciy well defined Irtic lesion that has a narrow zone
  
  of transition. T here is endosteal sca lloping and smooth J:uncllalcd periosteal reaction. The lesion has nonaggrcssi\'c
  
  features and in this age group eosinophilic granuloma or infcclion is the most likely diagnosis. Diagnosis Eosinophilic
  
  gra nu l om~1.
  
  Differential diagnosis Osteomyelitis. Fibrous drsplasia.
  
  • Leukaemia
  
  Radiographic aplX'aranccs arc \'aried. Lesions typi appear lytic but may have reacti"e sclerosis. l"largins ' oc well demarcated o r poorly defined and they may C" ha\"e a pcnneati\'c appearance. A lamcllated periost reaction is often seen. ilwasioll of overlying soft tissue result if rhe lesion penetrates through the corte.~. In skull, the lesion is often round with a punched appearance and une\'en destruction of the inner and skull tables results in a 'double contour' or 'ocvelled c appearance. If the re is more than one lesion, these coalesce producing 'geographical skull'. In the spine, \'crtebral bod,' is thc most common site of lm'oke Lung in\'olvc~lelU occurs in 10%. Treatmcnt of the lesions consists of conscn'ati,'e therapy treatment such as curettage or excision .
  
  • Lymphoma.
  
  Practica l tips Discussion Langcrhans cell hisriocytosis is a spectrum of disease characterized by idiopathic proliferation of histioc),tcs prod ucing focal or systemic manifestations. Eosinophilic granuloma is the term used [0 describe the disease when limited ro bone and is mostly seen in patients aged octween ;; and 30 years, The elinical 'and radiological fC:Hurcs may mimic infecrion as well as mher benign and malignam diseases. The cause and pathogenesis of the condition arc u nknown. Clinicli m:mitCSl'ations relate to thc atfected bone with local pain, tende rness an d masses COlli manly observed . Patients may ha\'c low'grade fe"er or ele"ated inflammatory markers, which eonnlscs the clinical picture with infection, The diseas~ may occur in any bone, although there is a predilection for the flat bones widl more than half occurring in the skull, mandible, ribs and pelvis. Lesions arc solitary in 50- 75% of cases. Approximately one, third oflesions occur in the long bones, most commonly the femur followed bv the humerus and tibia. Most lesions occur in the diaphysis, and in general the growth plan: acts as a barrier to extension.
  
  282
  
  AJways keep this cond ition in mind when fo diflcrential diagnoses for lytic bone lesions in the ~ patient - the appearances are varied and the clinical ' may be confusing.
  
  Further management The prognosis of eosinophilic granuloma is excellent spontaneous resolution ofbon~' lesions occurring in b-1 months.
  
  Further reading Lc\'inc S, Lambiase R, Pelchpr:tpa C (2003 ), ConiQ1 lesions of the tibia: characteristic appearances at conventional radiograph}', RtJdioGrnplJitJ 23: 157- 177. Stull M, Kransdorf M , O,,'aney K ( 1992 ). L1ngerhm cell histiocytosis of bone. RndioGrnphitJ 12( 4 );
  
  801-823.
  
  Musculoskeletal Imaging
  
  Case 159
  
  CASE 159 story :!4-ycar-oJd male prcscms with knee inst:lbiJity ing a football injury 2 l110mhs previously.
  
  283
  
  Answer 159
  
  Musculoskeletal Imaging
  
  ANSWER 159 Observations (159a) S:lgillal T2 weighted imagcs or the knee demonstrate a rupture orrhe anterior cruciate ligament, no imact fibres being demonstrated . In keeping with this, there is mild anterior tibial translocation. The posterior cmciate ligamcnr (PCL)is intacr. There is loss ohhc normal 'bow tic' appearance ohhe laterll meniscus with non-visualization or the bodv and much orthe posterior horn. Funhcrmore, abnorm;llow signal tissue is present in the intercondylar region just lateral 10 the PCL. These findings are indicative or a 'bucket handle' tcar of the lateral mcniseus with a fragment of mcniscus displaced mediall~' . A joint emLsion is also present. Diag nosis Anterior cruciate ligament (ACL) rupture with 'bucket h;mdle' tear of the lateral meniscus. Differenti al diagnosis Nonc. Discussion The ACL is best evaluated on Tl weighted images and fibres shou ld run parallel to the roof of the intercondylar notch (159b). ACL tears most commonly leave no nonm.l residuallibres visible on MIt! . Sometimes, residual fibres of the ACL arc seen, but following a more horizontal course than normal. Sagittal images of normal menisci show a 'bow tic' lPI>carlrlce on at least two contiguous slices (159c). This is because the normal meniscus is approximately 9 mill in width and the sagi n al images arc 3-4 mill in thickness. Thus, at least two sagittal slices should pass through a contiguous section of meniscus. 'Hucket handle' tears constinHe about 10% of Illeniscal tears. The vert ical tear through the inner edge produces a
  
  284
  
  mobile fragment that flips through approximately 18 much like a handle flipping from o ne side of a bucket the other. In such circumstances, the residual pan: meniscus will be reduced in thickness and will not be Stta on the usuaillumbcr ofsagirral scans. If the 'bow tic' seen on less than two contiguous SJgirral images, a 'bu handle' tear must be excluded. The mobile fragmalll' should then be sought elsewhere in the joim, c.g. me in the intercondylar region ; anterior to the postCfl" entdatc ligament ( peL) producing the 'double pel' (I 59d ); in the anterior joim, in from of the anterior of the meniscus. Other t}'PCS of mcniscal tear include: Oblique and horizontal - linear signal change \\1m. the meniscus th:lt extends to the inferior or superior surf.,cc. R.1dial (also known as 'parrot beak' tear) - a vcrrial (ear through the rrcc edge will produce an absent 'bow lie' sign similar to a 'bucker handle' tcar. Howcn:r, the derect is only small and so the ddea the 'bow tie' is much smaller.
  
  As an aside, if the 'bow tic' appeamnce of the m is seen on more than two saginll images this C.Do indicative of a discoid Illeniscus. This is prob congenital abnormality where the meniscus has J disc -like sh3pe than the normal 'e-shape' due to II thll1 nomlal body. They arc mon: prone to rcaring 3.IlIr.i be symptomatic e\'en without being torn.
  
  Fu rther management • A complete ACL tcar causes instability that is by surgical repair with a prosthetic o r tendon grwo.. Menisclltears often require arthroscopic debridement.
  
  Musculoskeletal Imaging
  
  Case 160
  
  CASE 160 istory kma lc patient presented with joinr
  
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  285
  
  Answer 160
  
  Musculoskeletal Imaging
  
  ANSWER 160 Observations 1160a) T here is subperiosteal resorption of the r:ldial aspect ofthc middle phalanges of the index and middle fingers. No c\'idcncc of marginal erosions or brown tUllIours is seen though the trabecular pattern is coarsened. The findings arc consistent with h)'perparathyroidism.
  
  Diagnosis Hyperparathyroidism.
  
  Radiological features of h),pcrparathyroidism arc 10110ws: BOlle resorption: Radial aspect middle ph,)Janx of211d and 3rd fingers ( 160 .. ). Terminal phalangeal tufts ( 160b ). Disral end of cla\"idcs and superior aspect of ribs
  
  onCXIL "ledial aspect proxill1:l1 tibia ( 160c). I'Ikdial tcmorll and humeral necks.
  
  Discu ssion The uneOnlrollcd production of parathyroid hormone in h)'Pcflxtr:lthyroidism is primary, secondary or tertbry. Primary hyperparathyroidism is causcd by a parathyroid adenoma. Thc raiscd parathyroid hormone lel·cls lead to reso rption of bone and hypercalcaemia. Secondary h)'perp:Hathyroidism is usually a consequencc of renal insuflicienc)" where chronic hypocalcaemia leads to parathyroid hyperplasia. Some patients with secondary hnxrp:lrathyroidisrn then go on to del·clop the tertiary form , whereby a parathyroid adenoma :Irises within :I chronically ol'erstinllliated hyperplastic parathyroid gbnd. The cardinal radiological feature is subperiosteal bone resorption. The dillerent sHes aflccted arc shown l>elow, the d:lssical location being the radial aSlxct of the middle phalanx of the index and middle fingers. Figure 160b shows :lnothcr pattern of erosion - band -like zones in the middle orthe terminal rults. Bone soltening mal' result in wedged vertebrae, kyphoscoliosis :lnd bowing of long bones. Parathyroid hormone-stinllllated focal osteoclastic activit)' can cause brown tumours, which arc characteristieall)' expallsile, lytic, wdl demarcated lesions. These can be the solit:I.TY sign ofhYlxfl>arathyroidism in 3% ofc:lscs. Figure 160c sho\\"~ a pathological fracture through a bro\\TI
  
  160b Left hand radiograph demonstrating lucent bands of resorption across the mid-portions of the distal phalanges of the first three digits.
  
  La.mina dura of skull :Illd teeth producing 'fl
  
  teeth' , 'Pepper-pot' skull due to trabecular n:sorplion ( 160d ). Pseudo-widening of joillls, e.g. sacroiliac jointi.. • Margill~\1 cro5ions of the h;lIlds.
  
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  160c AP radiograph of both knees demonstrates subperiosteal resorption at the medial aspect of both tibial metaphyses.
  
  286
  
  MuSculoskeletal Imaging
  
  A nswer 160
  
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  Case 166
  
  Paediatric Imaging
  
  Answer 166
  
  ANSWER 166 Observations (166a) This axial comrast enhanced CT image shows bilarcrnl and symmetrical low density in the b::lsal g:lIlglia, in\'Oh-ing thc putamina and thalami. T here arc scveral possible causcs for this appearance. Hypoxia and hypotension should be self-
  
  evident. If not present, le\'l.'ls of glucose and carbon mo noxide should be checked urgcllIly.
  
  Diagnosis Low-d ensity basal hypoxia/ ischaemia.
  
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  matter (thalami, putullin:t and br:tinstclll nuclei), the pcn rolandic g~'ri and conicospinal tr.tc[S. This case illustrate such a pattern . In oldcr childrt.'n, $t.',-erc hypoxic-ischaemic injury fill" produce a diflcrent pattern, that of global cerebral injunT he resulting cercbral oedcma :lIld loss of grcy-whl marta diflcrcnti:ttion with sparing of tht.' brainstem ccrcbdlulll GIll prod uce the acute rCl'crs:t1 sign on descriocd elsewhcrc (sec Case 163 ). As a comparatil-c aside, high-dcnsity b:tsal ganglia d to calcification arc illustrated (l 66b ) and the diffcren diagnosis listed.
  
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  Differential diagnosis Of low-density bas:tl g:l.Ilglil • H ypoxia H ypotension
  
  HypogJycacmi:l Carbon monoxide poisoning WiJson'sdiscasc Of basal g:lI1glia ca1cificltion (mnemonic 'PIE MAPS' ): • Physiological - rhe most common calise and i ll crc;l ~ingl y so with agc, . • InfectIon - c)'to1l1cgalOVlnlS (CMV), toxoplasma, cong('ni~1 1 rubdla, HI V. Endocrine - hypoparathryroidism (and pscudoj pseudopscudohypoparathyroidism ), hypcrparatllyroidism, hypothyroidism. lI'lceabolic - Leigh discase, Fain disease, Wilson's disease. Anoxia - at birth , ccrebro"ascular accidcnt (eVA). Poisoning - Clrbon 1I10noxide, lead. Syndromes - Down's, Cocbyne's syndrome, ncu rofibromatosis.
  
  Discu ssion Apan from \Vilson's disease, the listed ditlrrcnti:J.I diagnoses lor this :tppe:n:l1Ice afe of :tWIC disorders that ul timatcl}' result in reduced cerebral oxygenation or glucosc provision. T he dlcc t of hypoxi:tjh)'potension on the infant brain dcpends on whcthcr the infant is tefm or prem3ture, and whether the insuh is mild or scvere. In thc prem:tUlrc infant up to about 34 "'cch, it is thc dccp whitc martel" Thai is most vulnerable and hypoxie-ischacmie injury rcsults in peri,"cnrricular Icu komalada (PV L) with sparing of subcortical wh ite mane r and cortex. Since the cOfTicospinal tr:\Ct fib res pass through this area, there is usually rcsulting 1Il0torimpairmcnt. In the term inf:tn t , the pattern of susceptibility is diftcrelH. A mild insult results in isch:tcmia of the 'watcrshed' :treas oftlie cerebrum where blood supply is most tenuous_ These arc the boundaries OCfwcen the areas supplied by the anterior, middle and postcrior cerebral arteries. Vit:11 ;Ifeas of the brain arc protected by redistributio n of blood flow. Aftc r a sc,'cre insu lt, howc"cr, "ieal arcas of the brain can no longer be protcctt.'d, and it thc most met.lbolicall)' actirt.' areas at this timt.' of lift.' that arc aHccted. Thus, ischacmic damage occurs in the deep grcy
  
  298
  
  Practical tips Comparativc dcnsities of grey and whitc matter in tlr infJnt brain arc variablc on CT dcpc nding o n stage myelill3lion. Norrnaliry ofthc basal g:mgli:t dcnsi~ can oc confirmcd by comp:trison wit h othcr grey matterstntcturcs. MRI is thc most sensitivt.' mod:tlity for dctecti ng hypoxic-ischaemic injury but may be logistically difficult in the acutclr unwcll inf.mt. Ultrasound llUO be practically the casiest imaging option bm has reduccd scnsiti,"ity.
  
  Further management Urgent correction of hypoxia, h)"potcnsion hypoglycaemia is required followcd b~' exclusion of
  
  Paediatric Imaging
  
  Cases 167,168
  
  ory mature neonate prcscllIcd with ina! distension and sepsis.
  
  CASE 168 History .\0 adolescent mate presented with a hiswl)' oftralima.
  
  299
  
  Answer 167
  
  Paediatric Imaging
  
  ANSWER 167 Observations 1167a )
  
  Necrotizing enterocolitis (NEe ).
  
  Pneumoperitoneum should be carefully looked for, as. this necessitates immediate surgery. In the supine position. free gas often collects anteriorly as a large rounded lucency in the central abdomen producing the 'football' sign. When perforation occurs ill utero, meconium within the peritoneal cavity calcifies and can be seen on plain radiography. An example is shown in a neonate ( 16 7b where there is peritoneal calcification (best seen over the inferior tip of liver ) with a pneumoperitoneum causing ~ positive Rigler sign, i.e. visualization of both walls of tbc bowel.
  
  Discussion
  
  Practical tips
  
  A cord c!:i mp is noted indicating that the neonate is no morc than a few davs old. T he bowel is abnormal with gaseous distension and a bubbly appearance to the bowe! wall indicating lllllrll gas. A brgc pneumoperitolleum is present with most of the gas adjacent to the li\'cr. The features arc consistclH with necrotizing enterocolitis and perforation. No gas is seen within the portal veins.
  
  Diagnosis
  
  up to 80% of cases arc n:latcd to prematurity and NEe is the most COlllmon gastrointestinal emergency seen in premature blbics, lIsllaIly occurring in the tirst 2 weeks of life. Ischaemia of the bowel is thought to occur secondary to perinatal stress, hypoxia or infection. Presentation is \\~th d iarrhoea or bloody stools. Radiographic features consist of distended thick walled bowd which has a bubbly :lppearance due to submucosal gas, i.e. pneumatosis intestinalis. Gas mar track from the bowd into the portal ve no us .s~'s tem and can be ,seen on ~lain rad!ogra~h~' (branchlllg gas extends tow:lfds the penphery of the h\'er unlike air in the biliary tre!,;, which is central ). Such a finding in adults is an ominous and usually premorbid sign, bur in NEe, this is nOt the case at all. Bowd strictures arc a potem ial long term complication.
  
  The earliest radiological sign on plain film is bowel di[,ltation (due to ileus). If a cOntrast enema is required to exclude obstructioa then water·soluble contrast should be used - barium is contraindicated.
  
  Further management Mortality rates arc dependent on the degree prematurity, with rates quoted at 5% in term infunrs 12% in premature newborns. Initial treatment is supportJ\" with bowel rcst bm if serial radiographs or clinical fea show progression or per/ofation then surgical resection nccrotic bowel is required.
  
  167b Abdominal radi ograph in a newborn de mo nstrates visibility of both sid e s of the bowel wall, i.e. a positive Rigle r s ign indicating pneumoperito neum . Fle cks of calcification in the right abdo men confirm the diag nosis of ante natal meconium pe rito nitis .
  
  300
  
  swer 168
  
  Paediatric Imaging
  
  ervations (168) bonr injul)' is $Cen on this skull r.ldiogr:lph. There arc pie wormian bones, which at this age is abnormal. is a large differential di:agnosis but the most likely is idiopathic.
  
  - erential diagnosis worrnian bones, with common causes underlined cOlon ic ' PORKC HOPSI'):
  
  • • • • • • • • • •
  
  Pyknodysoslosis. Osteogenesis imprrtccrJ Rickets in healing. Kinky hair syndrome ( Menkes). CleidOCranial dysostosis HmOIhyroidjsm /hypophosphatasia. OLOpalatodigital syndrome , Pachydcrmopcriostosis. Syndrome of Down's. Idiopathic - norma] in first \'Car of Hti;
  
  Case 169
  
  found in the lambdoid, posterior sagill3l :md tcmporosqu:lITIosal sutu rC$. The), arc consid ered abnormal when seen after 1 rear or large ;\Ild numerous (> 10 ;n num ber and larger than 6 x 4 01111).
  
  Practica l tips Because oflhc wide di lTcrential diagnosis, il is diilicult to idemify a specific cause wilhout Ihe aid of a good dinic:al hisrory. Diffuse ostcopeni:a will be present on the skull radiograph in cases o f rickets and ostcogenesis impcrlccta. Pyknodysostosis, o n the other hand , will be 3ssociated with diffuse osteosclerosis on rhe film.
  
  Further management t.hnagcmelll is dependcllI on the underlying causc.
  
  Further reading Cremin B, Goodman H , SprJllge r j, Beig hton P ( 1982 ). Wormian bones in osteogenesis iml>crfccta and other disorders. Skeletnl Rndi%g:v 8( I ): 35-38.
  
  Discussio n 'onni:an bones arc essentially small bones occurring in the rures of the calvaria. These intrasururJ.1 ossic1cs arc usually
  
  CASE 169 History .l,.
  
  child with a hist0'1' of partial seizures.
  
  301
  
  ANSWER 169 Observations (169a) Axial T2 weighted MR image at the level of thc latera] vcnrric1cs. This demonstrates ;1 large cleft extending through the full thickness of the left cerebral hemisphere from thc surface of the br~lin to the left lateral ventricle. The cleft is lined by grey matter and is filled with CSF. The findings arc consisten t with schizcnccphaly.
  
  Diagnosis Schizcncephaly. Discussion During gestation, Il('uranes migr3tc out\v,lrds from the pcrivcntricular germinal matrix to form the norma] cerebral cortices. This migration can be interfered with by several causes including chromosomal abnormalities, but mostly the reason is unknown. The result is brain tisSLle lying in the wrong place, typically grey maner. Schizcnccphaly is a cleft extending through tbc full thickness of cerebral hemisphere from the ependyma-lined wall of ventricle to the brain surtaee. It is lined by pia ;md grey matter that usually shows polymicrogyria, and is often located around tile $ylvian fissure. The lateral end of tile cleft may be open and readily apparellt (open lip type ) but can sometimes be closely opposed ;md easy to miss (dosed lip type ). However, even the closed lip type will show a small irregularity in the wall of the "entricle at the site of
  
  16gb Axial T2 MRI of the brain shows a small area of subependymal heterotopic grey matter adjacent to the left occipital horn. Note how it is isointense to the cortica l grey matter.
  
  302
  
  the cleft. It is not certain whether this condition is d ue an ischaemic insult leading to germinal matrix infarcno. or whether it represents a focal cortical dysplasia. There arc diHcrent manitcstations of the conge neuronal migration anomalies which result in \'a ~ - _ degrees of mental retardation and/or seizures. 11It following further patterns are recognized: • Heterotopic g rey matter - when small collections of the neurones arrest on their way to t he cortex the\' can be seell as discrete nodules '( most commonly in ~ subependyrnal location ) or as a subcortical band . Thus there arc nodular and band heterotopias. Thor: are isointense to grey matter and show no enhancement. \-Vbile so metimes asymptomatic, seizures and developmental delay can ensue, espe . with band heterotopia. An example of nodular heterotopic grey matter is shown in an axial T2 MR image of the brain ( 169b ), where a small area of heTerOtopic gre~' maner is seCl1 in a subependymal location adjacent to the occipiral horn ofrhe left lateral ventricle. P01Ylllicrogyria - sometimes neurolles may migrate the cortex but arc abnorm,\lly distributed, produ . _ a bumpy appearance to the cortical gyri termed polymicrogyria. An example is shown in a coronal ~ weighted MRI ( 16ge ), where polymicrogyria atlccting the right temporal lobe produces a burnp:r
  
  Paediatric Imaging
  
  A nswer 169
  
  Case 170
  
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  Paediatric Imaging
  
  ANSWER 170 Observations (170a) An cndotr.lchcal rube has been pllecd juSt above the carina. There is also all NG tube passing inro the stOffilCh. There arc bilateral diffi.1SC patchy opacities in both lungs indican,'c of widespread atelectasis and patchy consolidation. However, thc lungs appe3T hypcrinflatcd and there arc 5111311 pleural cnusions. In vicw of the history, the :appearances arc likely 10 be due to meconium aspiration syndrome.
  
  Diagnosis Meconium aspiration syndrome.
  
  Discussion A'\cconiulll aspiration syndrome is the most common cause of respirator}' distress in ncwborns bom at 1'11][ or post reml. T he brgc size oflhc fetus makes ddive,)' diAinLit. Perinatal hypoxia and feral distress lead 10 meconium defecation ill lIlero. Aspiration of the meconium into the trac heobronchial tree then causes obstruction of small peripheral bronchioles (though only a minority of fetuses exposed to meconium stained amniotic fluid develop respiratory symptoms). This resu hs in une\'enly distributed areas of subsegmental ateledasis with alternating areas of air trappi.ng. The chest radiograph usually begins dearing within a few days with no long teml radiographic sequelae in the lungs.
  
  The radiological features on CXR are: Bilateral patchy atelectasis and consolidation. No air bronchograms. Hypcrinllation with areas of air trapping. Small pleural effusions. Spontaneous pneumothorax and pneumomediastinum ma~' result from the air trapping ( 170b).
  
  Practical tips Most common cause ofrespiratory distress in term babies: meconium aspiration. • Most common cause of respiratory distress in preterm babies: hyaline membrane disease. Furth er manag ement Almost all neonates with meconium aspiL.ltion s~'ndrome make a full recovery of their pulmonary function. Upper ainl'ay suction may be employed and vcntilatory suppon may be required in more scvere cases of rcspiL.ltory distress...
  
  HOb CXR in a neonate with a large pneumomediasti num. Air outlines the thymus producing an 'angel's wings' appearance.
  
  304
  
  Paediatric imaging
  
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  Furth , m a n .~.m. n l S"'1Ii<, 1"'P';' ~ "'~" .... ". TIt ... ~'n '" I"" ' "mri"" ,,1 hr , .. ,,,amofi< k'k, """'Ph'S,,1 " ,;.,,,'" '" '~ n '''m,1
  
  """ il;'1" ,,,.I, i, g ..
  
  P"'""""';"
  
  J)' r ~'I\'"
  
  ' nJ/"" "r""""
  
  Answe r 172
  
  Paediatric Imaging
  
  Case 173
  
  ANSWER 172 Observations (172) This MCUG study shows a transverse filli ng defca at the _ terio r urcthr:l. with distension of the proximal posterior ;arcthra. The findings arc collsisrcnr \\~th posterior urethral lQh·cs.
  
  Dia gnosis Posterior urethral vakes.
  
  posterior urethra may be seen during voiding and vesieouretera! reflux is present in 50%. Bladder trabeculation and a significant post void residual volume may be noted. Prognosis depends on the duration of obstruction prior to corrective su rgery and is worse if associated with \'esicoureteral reflux. Approximately rhrcequarters of cases will have been discO\'ered in the first year of life, though occasionally it call be first noted in adulthood.
  
  Discussion Congenital presence of thick folds of mucous membrane in the posterior urethra is the most common cause of
  
  urinary tract obstrunion in boys. The condition is often suspected on prenatal US where it can lead to oiigoh)'clramnios, hydronephrosis, prune belly and urine lSCitcs or urinoma due 10 leak. I f obstruction occurs carlv in IItero then multicysti c dysplastic kidney may result. Aftc"r
  
  birth, MC UG is the investigation of choice to outline the tr:Insvcrse fi lling defect caused by the thick mucosal folds. Distension and elongation of the proximal part of the
  
  Practical tips Note how diagnosis is still possible when the catheter is ill sim during the. voiding phase of the MCUG ( 172).
  
  Further management Urological surgical intervention is required with initial treatment aimed at relieving bladder outlet obstntction and abl:tting the valves. Secondary trcatment may be required for vcsicoureteral reflux, urinary tract infections, urinary incontinence and renal dyshlilctioll.
  
  CASE 173 History A newborn presented with abdominal distension and failure to pass meconium.
  
  307
  
  Answer 173
  
  Paediatric Imaging
  
  ANSWER 173 Observations (173a) An NG tube is in the stomach. There arc multiple dilated loops of bowel in the abdomen. No fluid lc,"cls arc seen within the bowel suggesting that the ;lppcaranccs 1l1:1~' be dlle to meconium ileus. However, mher p:uhoiogics such as Hirsc hsprung's discasl: ;lIld impcrfor:llc anliS should be considered.
  
  should be performed using Gastrograffin as this has a therapeutic eflect, helping to cle:tr the meconium b~ drawing water into the gut. With Hirsehsprung's dise:tse, the contr:tst enema will demonstrate dilated bowel with :t transition zone to a dist1l. aganglionic segment.
  
  Practica l tips Diagnosis ,\.tcconiulll ileus.
  
  Differential diagnosis Hirschspnlllg's disease. Impcrlor:uc3.nlls. Ilcatatrcsia • Inguinal hernia.
  
  ·
  
  Discussion Meconium ileus is the term used to describe small bowel
  
  obsmu.:rion in neonat . :s secondary to inspiSS:J.tcd meconium pellets impacted in the disnl ileum. The \'35t majority prm"c to [m"e cystic fibrosis and this is the earliest manifcstation of the disease. The diagnosis is confirmed by performing a contrast enema, which demonstrates multiple round filling defects (the inspissated meconium ) in the distal ileum and proximal colon ( 173b ). The colon may be very narrow on the contraST STUdy if it has been unused due to antenatal ubsrnleTion, whereby it is Termed a microcolon. The enema
  
  • On all A...,{R of:t baby it is almost impossible TO tell if dil:tled loops ofbowcl arc l:uge or small bowel. The presence or absence of ,·omirins/passage of meconium are more helpful to know with regard to assessing if there is high or low bowel obstruction. The hernial orifices should be checked for :tir suggesting an inguin:tl hernia. A 'soap bubble' appeamnce m;I ~' be seen on AXR in llIeconium ileus due to the mixlllre of gas with meconium. Fluid levels arc not usually presem in meconium ileus because the bowel COntentS arc ,·ery viscous. Look at the sacrum on the AXR, as imperforate anlU is associated with sacral agenesis.
  
  Furth er mana gement • 'V:tter-soluble contrast enema can be useful for both diagnosis and treatnletH. All patients with meconium ileus should have a 'SIl"CZ test' to exclude underl ying cystic fibrosis.
  
  173b Gastrograffin enema in a newborn shows multiple filling defects in the ascending colon and terminal ileum, which represent inspissated meconium. Note the dilated small bowel loops.
  
  308
  
  Paediatric Imaging
  
  Cases 174,175
  
  CASE 174 Hist ory _\ 4-yur-old child presenred with ~'cr and abdominal pain.
  
  CASE 175 Histo ry .\ 4 -month o ld child presented ",lil pcr,;islent irrit'Jbilit)'.
  
  309
  
  Answer 174
  
  Paediatric Imaging
  
  ANSWER 174 Observations (174a)
  
  Axial cr of the :abdomen with oral canTmst and IV contrast in poml phase. There is a large slightly hcrcrogcl1wlls mass arising from the. right kidney. The mass docs not enhance as much as the rellal parenchyma. There is local mass effect with displacement, but no 100';'\siol1 ofthc right lobe ofli\'cr or encasement of"csseJs. A small mass of similar density is seen ncar the hilum of the left kidney. The appearances suggest bitateral Wilms' tumours.
  
  Diag nosis
  
  Almost all neuroblastomas contain calcification whereas 'Only up ro 10% of Wilms' rumours calcify. Both tumours call cross the midline and look similar on imaging, appearing illsep3.fablc from the kidney; howcver, neuroblastoma rends to encase surrounding ,·esscls such as the aorta whilc Wilms' tumours tend to displace surrounding tissucs and stmcrures. An examplc of a neuroblastoma is shown ( 174b) cncasing the aom and coeliac axis vessels. Neuroblastoma may also extend into the spinal canal through the neural f'Or."tmina.
  
  Bilateral Wilms' [Umours.
  
  Differe nti al diagnosis Neuroblastoma.
  
  Discussio n Wilms' tumour (ncphroblastoma) is the most common abdominal malignancy in young children, mOSt commonly prc.scming at ase 3-4 years. The most frequent presentation is with abdominal mass, though hypertension, pain, IC\'er and h:u:maturia also occur. The tumour usuall~' grows to a large size, often measurin~ over 10 cm. Radiological fcatures includc: Exophytic mass displacing rather than encasing adjacent structures. Less contrast enhancement than norlllal rella] parenchyma. Cystic/necrotic areas give it a heterogeneous appearance. 111\':1sion ofthc ren:1l \'ein and inferior ,'c na ca\':1 may occur in up 1'0 10%.
  
  Look for evidence of 1l1e[3static spread; 70% of neuroblastomas have malignant spread at presentation c'Ompared to just 10% of Wilms' tUlllOUrs. Also, \-Vi!ms' tumours spread to lung, whereas neuroblastoma sprcads to bone.
  
  Further management Treatment is ,lith radical nephrectomy and chemother."t~ I'reopcrath'c chemotherapy is advocated in cases ofbilarer.al Wilms' tumours and whcn therc is IVe ex tension at tumour (occurs in - 5%).
  
  Tumours arc bilateral in 10% and this indicates background nephroblastomatosis, a state of persistent nephrogenic blastema that is a precursor to Wilms'. Wilms' tumour is associated with the Beckwith- Wiedem:lnn s}'ndromc (macroglossia, visceromegaly and omphalocclc). Other associations include aniridia and hemihypcrtroph}'. The main differential diagnosis is neuroblastoma, a comlllon malignant tumour of the neural crest that presents in a similar way to Wilms' tumour ,lith a painful abdominal mass and fe'·e r. Typical age of present'alion is slightly earlier howe\'er (under 2 years). Hormone secretion from the tumour (such as catecholamines) may cause o ther signs, including hypertension and opsoclonus (chaotic jerky eye mo\'eme[1[s). It can arise an~'whcre in lhe sympathC[ic ncural chai n including the adrenal gland and the abdominal s}'lllpathetic chain.
  
  Practica l tips • Always check for bilateral tumours when Wilms' is suspected. Differentiating between neuroblaStollla and Wilms' tumour can be difficult radiologically but then: are some specific features that can help:
  
  310
  
  174b CT of a neuroblastoma. The huge mass displaces the spleen and left kidney and shows typical encasement of vessels, in this case the aOt:i and coeliac axis.
  
  Answer 175
  
  Paediatric Imaging
  
  Case 176
  
  ANSWER 175 Observations (175) There is bilateral symmetricll thick, smooth periosteal reaction aAccting the diaphyses of the long bones. There
  
  is no fraying or splaying of rhe metaphyses to suggeSt rickets. The most likely diagnosis at this age is Cail'{fs disease, ho\\'c\'cr other possibilities such as lcuk:".cmia need to be considered.
  
  Diagnosis Caftcy's disc:lSC.
  
  Differential diagnosis Of bilateral dinusc periosteal rcacrion in childhood: • Norni:ll variant belare the age of 4 months_
  
  C lflcy'sdiscasc. Leukaemia. Scurvy.
  
  Rickets. H ypcn"iraminosis A. Non accidcnt:ll injury (NAI ).
  
  6 months. Irritability and fcn:r arc thc prcscnring symptoms and are associated with soft tissue swelling O\'cr thc bones. Bilateral synll11ctrical thick periosteal reaction is the cardinal radiological feature and most commonly alleets the m,mdible, elal'icle and the long boIlCS. It usually involvcs thc diaphysis ofthc bone. In the majority ofe:lSCS, [here is spontaneous complete reeon:ry by the age of 3 years.
  
  Practica l tips If there is ditfusc periOSTeal reaction with tractures of
  Further management Whcn NAI is considered the n a (.-arcnli analrsis ofpn.:vious radiographs, the clinical presentation and consult":nion with a specialist paedi3trie radiologist must be carried our because of the repercussions of a misdiagnosis.
  
  Discussion Inf.l.Iltilc conic:J.l hypcrostosis (Caffey's disease ) is a prol ifcrJtivc bone disease seen in patients under the age of
  
  CASE 176 History None available.
  
  311
  
  Answer 176
  
  Paediatric Imaging
  
  ANSWER 176 Observations (176a) There is widespread bilateral decreased bone density with healing insuflicicncy fractures of the r:tdius lnd ulna bilaterally. These arc associated \\~th thick smooth periosteal rC;lcrioll and there is !Taying and spla)~ng of the mCl.lphyscs. The fcarurcsarc char.tctcristic ofrickclS.
  
  Diagnos is Rickers. Differential diagnosis Hypophosph3t:J.sil.
  
  Discussion Ri ckets is mOST commonly due to insuflicicnt biologically actil'c I'iumin D, though impaired calciulll absorption or excessive phosphate excretion call occasionally be to blame. In the western world, pure dicta!)' deficiency ofl'itamin 0 is ra rcl~' the sole cause; morc ofren it is due to malabsorption or impaired vitami n 0 mct:J.bolism in the Ih'cr or kidney. RickelS is 'essc: ntially osteomalacia during enchondral bone growth, Portions of the skeleton that ha"e already matured show features of osteomalacia, but loss of normal maturation and mineralization of cartilage cells at the
  
  176b AP radiograph of both legs shows typical features of rickets with fraying of the metaphyses.
  
  312
  
  growth plate lead to the additional distinctive radiological featmes of rickelS. Osteomalacia is discussed elsewhere in the book but the radiologieallcatuTes arc due to excessive unminel"".llized osteoid producing Looser's zones, osteopenia, cortical tunnelling, indistinct trabeculae and finally bowing and fl"".letures due to softened bones. In addition, the following features arc seen in rickets: Widened growth plate - loss of normal chondrocyte maturation and minel"".llization result in cdl build up here . Metaphysc:s arc irregular/ frayed , splayed and cupped - impaired miner.1lizarion causes the frayed irregular appear.1nce while build up of chondrocytes at the physis indems the metaphysis producing cupping and splaying. Epiphysis oSleopcnic and irrcgubr. I\:riarticularsofttissue. swelling. Apparent periostcal reaction due to subperiostcal ullminer.1lized osteoid. Delayed maturation and growth. Figure 176b demonstrates ricketS of the lower limbs note the typical changes around the metaphr~!s in the tiw and also bowing of the fibula, Figure ] 76e is a CXR of.a child with rickets showing spla)'ing of the anterior ends 01
  
  Answer 176
  
  Paediatric Imaging
  
  Case 177
  
  the ribs and the metaphysis of the right humerus. The
  
  Further man age ment
  
  ~arancc
  
  Rickers is now usuall y idcllIificd early :llld treated with I·iramin D supplements. Significant pch'is deformity and gait disturbances arc now rarel y seen in the dC\'ciop
  of the anterior rib ends is due to changes at the
  
  cOSlochondr.d junction growth "blcs :and is tcnncd rhe 'rachitic rosa')",
  
  Practical tips • The earliest sign of rickets on the plain film is a widening of the growth plate . • i..,(X)ser's zones aTC ..ire in rickets compared 10 ostcom:tbci:t in the filscd skeleton.
  
  CASE 177 History ~onc ;lvaib blc.
  
  3 13
  
  Answer 177
  
  Paediatric Imaging
  
  ANSWER 177 Observations (177a) There is panial n lsion of an extra digit with the mct:lClrpal of the IinJc finger. This c ssc n tial1~' represents polys)'lldact)'ly, the possible causes of which include idiopathic, Ellis- van Crc\'c1d syndrome and C:arpcntcr syndrome. Diagnosis Ellis-van Crcn:ld syndrome.
  
  Discuss ion There arc scveral causes ofsrndactyly (filsion of digits) and polydactyly (supernumerary digits ), which arc both congcnit:ll abnonnalitics. Ellis- van C rcvcld syndrome is also assOCiated with carpal fusion, as is Apcrr's syndrome. This is characterized bv features in the skull: notabk craniosynostosis of the ~oronal sutures, hypoplastic mid filee and enlargement of the sclb. . All of these features arc dt~lllonstratcd in the lateral skull radiogl':lph in a child with Apert's syndrome ( 177 b ).
  
  Differential diagnosis
  
  or causes of polydactyly:
  
  • Idiopathic. Ellis- van Crevcld syndrome. • Carpenter syndrome. • Polysynd:lCtyly syndrome . O fca llscsof s),ndaCfyly: • Idiopathic. Apcrr's synd rome. Carpemer syndrome, Down's syndrome. Poland's syndrome . NClirofibrolllarosis.
  
  Practi cal tips Some exam cases will have an obvious abnormality as part of a syndrome that you do n ' t know - Slating that you would seek help from a te.u book o r specialist colleague is a reasonable answer. You ca n't know everything! Further management Po ly/syndactyly will be parr of a syndrome with multiple ab norrll ~l 1ities.
  
  177b Lateral skull radiograph of a child with Apert's syndrome demonstrating craniosynostosis of the coronal sutures, hypoplasia of the midface and enlargement of the sella.
  
  314
  
  Paediatric Imaging
  
  Case 178
  
  CASE 178 History ~onea\'ailablc.
  
  315
  
  Answ er 178
  
  Paediatric Imaging
  
  ANSWER 178 Observation s (178a, 178b) There is bowing deformity ohhc tibia, fibula and humcms The metaphyses of the bones arc widened producing an Erlenmeyer flask deformity. The metaphyses arc also rclati\'cly lucent when compared with the diaphysis, which is sclerotic. There 3fC no specific leatures 10 indicate lead poisoning, ostcopo.!tTOsis or thalassacmia, so the diflcrcnti:ll diagnosis lies between Pyle'S disease and lipidoses such 35 Gaucher's or Niemann- Pick disease.
  
  Diagnosis Pyle's discasc.
  
  Differential diagnosis [11 rhiscasc: Craniomct'aphyscai dysplasia. • Niemann-Pick disease. • Gauchcr'sdiscasc. Of Erlellmeyer flask deformity (mnemonic - 'Lead GNOME'): • Lead. G3uchcr's. Niemann- Pick disease - looks like Gaucher's but withouJ avascular necrosis. Osteopetrosis Mctaphyse;11 dyspbsia ( Pyle's) and craniollletaphyseal dysplasia (same as Pyle's discase: but there is a history of cranial nelyepalsies). 'E'malOlogical!! - thalassaemia.
  
  Discussion Pyle's d isease is also known as metaphyseal dyspbsia. It is a rare autosomal reccssi,"c disordcr characterized by flaring of the ends of long bones with relative constriction and sclerosis of the ce mral ponion of the shafts. Affected patients arc usually as~' mptomatic and genu valgus
  
  deformity is often a featu re:. The expanded metaphyses tend to be lucent and have the appearance of an Erlenmeyer flask (narned after the wide necked laboratory flask bearing the name of this German chemist). Craniometaphyseal dysplasia essentially has the samc features but in addition there arc cranial nCIYe palsies due to sclerosis of the skull base. Gaucher's disease is a hereditary disorder oflipid st.oragc common among Ashkenazi Jews. It is characterized by hepatosplenomegaly with flask-shaped long bones and generalized osteopenia with strikingly thin corticcs. Avascular necrosis is also a feature.
  
  Practical tips Erlenmeyer flask deformity, the metaphyseal expansion of long bones, is also discussed in Chapter 5. AdditionaJ differentiating ICaturl..""S can be found on [he r:ldiograpn as to the specific underlying cause of Erlenmeyer flask. deformity: • Diftllsc sclerosis and sclerotic vertebr:ll endplates producing 'sandwich vcrtebrae' indicate osteopetrosis. With Pyle's disease, there will be relative sclerosis at the diaphysis and lucenc)' of tne metaphysis. Gaucher's disease will also be associated with lucency and osteopenia but there may be signs of anscular necrosis of the femoml or humeral hcads (loss of height and Ii-agmentation ) and on an AXR massi,"e hepatosplenomegaly may be seen. Thalassaemia is associated with coarsened trabeculation producing a 'cobweb' appearance. Lead poisoning causes dense metaphyseal bands as well as Erlenmeyer flask deformity. Further management This condition is usually asympromatic and requircs n... direct management.
  
  1788 Metaphyseal widening with increased lucency.
  
  3 16
  
  Paediatric Imaging
  
  CASE 179 Hist ory _'onc3vailabk,
  
  Case 179
  
  Answer 179
  
  Paediatric Imaging
  
  ANSWER 179 Observations (179a , 179b) The lateral skull radiograph ( 179 b ) demonstrates thinning of the calvaria with multiple wormian bones. Bowing dctormirics afC seen w atTcC[ the lim bs (1 79a ) and there Ut several frlcturcs of diHcring ages, mostly seen at the metaphyses of the long bOlles. Thac is geller,\iized oSTcopcnia of the skeleton with marked thinning of the con ices. The fcanm::s arc consistent with osteogenesis irnpcrfccT:t
  
  Diagnosis Osteogenesis impcdccta Differential diagnosis Of lI'oTmian bones with common causes underlined ( mnemonic - 'PORKCH OPSI' ):
  
  • P),knodysostosis Ostcogcm:sisinmcrfccra. Rickets in healing. Kinky hair s)'lldromc (I'''knkcs). Cleidocranial dysostosis. Hypothyroidism/hypophosphatasia. Otopalatodigital syndrome. Pach),dermoperioslosis. • Syndrome of Down. I~!iopa[hic nOrllwl in first ,"car of life.
  
  318
  
  Di scussion Osteogenesis imperfecta is a connective tissue disorder characterized by fi.lgile bones and blue sc1eme. Type I is comp:ltiblc with life. Type 2 is the lethal form associated with perinatal death. The principal radiological features include: Diffuse osteopcnia with thinning of cortices. Multiple fractures of differing ages with pseudarthroses and bowing deformity. Fractures arc associated with exubcmllt callus fOfmation. Biconcave yerrebi.ll bodies. Multiple wOTmian bones in the skull POOf dentition. Practical tips Multiple fractures in children should i.lise suspicion of non accidental injury (NAI) and sometimes diflcrentiating this from osteogenesis imperfecta can be difficult. Predomin:lJ1t1y osteogenesis fractures are diaphyseal compared with metaphyseal NAI fractures but this is nOt always the casco Further management Early medical inteIyention to increase bone mineral density and surgical intcrvention to treat/correct scoliosis and treat fractures mean that a multidisciplinary ap proach to the ongoing treatment is required
  
  Paediatric Imaging
  
  CASE 180 Hist ory A ch ild presented vomiti ng and .uaxi:!.
  
  with
  
  CASE 181 Hist ory A 2-l11omh-old baby presented with cyanosis. (Ste pnge 322 [01- e(lse (lllslI'cr)
  
  Cases 180, 181
  
  Answer 180
  
  Paediatric Imaging
  
  ANSWER 180 Observations (180a) A series of axial MRI images of skull show a well defined hypcrdcnsc mass in the cerebellar ycrmis causing compression and anterior displacement of the 4th ventricle with obstmctil'c hydrocephalus. A small alllount ofocdcma su rrounds the mass. The teatures arc typical of a medulloblastoma Diagnos is Medulloblastoma. Differential diagnosis Of posterior fossa tumour in children: Medulloblastoma: Predominantly midline posterior to 4th vcmriclc. Hypcrdcnsc on CT with oedema. • Avid cnh:I.llCCl11CllL • 20%calc;!)', 50% necrose. Juvenile pilocytic astrocytoma: Typically paracentral, posterior to 4th ,'cntriclc Majority arc cystic with an enhancing mural nodule, the remainder arc solid. 20% calci!)'; oedema is rare. Epend)'llloma: Arises within 4th ve ntricle. Heterogeneous appearance and enhancemem • SO%c;\lcify.
  
  H ydrocephalus is ott·en communicating type due to protein exudate obstructing CSF resorption Spreads through the exit foramen of fourth \"emride and wraps around brainstern ('plastic growth'). Sagittal and axial T2 weighted MR images (l 80b ) demonstrate a high signal lesion arising within the 4th Yemricle, wrapping around the brainstem and spreading via the foramina of Luschka and Magendie. Hydroceph.llus is present and a syrinx orthe upper cervical cord has de,-doped. Hrainstem glioma: Within pons, possibly causing pontine expansion or 4th 'Tntride displacement posteriorly_ Iso· or hypodense to brain so may be easily missed. Sagittal TI weighted MIl! with contrast ( i SOc) dcmonstrates a large pontine glioma. Note how the lesion is nonenhancing and almost the same signal as surroundi ng brain. Smaller such lesions can easily be missed due to such imaging characteristics. Enhancement often absent or minimal H),droccphalus uncommon (because present with focal neurology before this occurs).
  
  Disc ussio n ,\kdulloblastoma is the second most common paediatric brain tumour and the most common in the posterior fossa. It is a tl'pe ofprimiti\"e neuroectodermal tumour ( PNET)
  
  180b Sagittal and axial T2 MRI of the brain demonstrating an ependymoma in the 4t h ventricle, which extends into the foramina of l uschka and Magendie and causes obst ructive hydrocephalus. A syrinx of the upper cervical cord has developed .
  
  320
  
  Answer 180
  
  Paediatric Imaging
  
  arising from the roofofthe 4th,\·cntriclc. The majority of patients affected are under the age of 15 years, with 80% of lesions arising from the cerebellar vem}is, and the rest lying more laterally in the cerebellum. This lateral location is marc C01111110n in older ch ildren. They arc typically hyperdensc on CT due to dense cellularit)" and show avid, homogeneous enhancement. There is usually surrounding oedema, 20% show calcification and up to 50% show necrosis/cystic change. They arc highl~' malignant and spread occurs via the CSF in up to a third. Medulla· blastoma is rardy associated wi th Gorlin's syndrome - an autosomal dom inant disorder cha racterized by multiple Cluaneous basal cell carci nomas during childhood with mandibular ker:l.tocvsts and enensin: intracranial Gllcificarion of the fal~ and tentorium. At least 50%of primary brain IllmOUfS in children occur in the posterior fossa. Brainstem glioma tends to present with focal neurology due to invoh'ement oflhe long tr:J.cts and cr:J.nial nerve nuclei, while the olher three differential diagnoses listed present br way of mass effect and obstructive hydrocephalus with headache , vomiting and at3xia . The salient imaging reatures of each arc listed. Ir is important when staging paedi,lIric posterior fossa tumours to remember the potential ror CS F spread with medulloblastoma in particular, but sometimes with ependymoma too. POSt gadolinium scans should therefore include the whole spine as well as brain to pick up such deposits ('drop
  
  metastases' ). A sagi nal TI MlU post-contrast ( l SOd ) demonstrates an enhancing medulloblastOma in the posterior fossa causing obstructi"e hydrocephalus. CSF spread of tumour has occurred with a metastasis in the prepontine cistern.
  
  180e Sagittal T1 weighted MRI post IV contrast that shows a large pontine glioma.
  
  180d Sagittal T1 MRI of the brain with gadolinium show ing an en hancing posterior fossa mass, w hich causes obstructive hydrocephalus. A metastasis is seen in the prepontine cistern. This proved to be a medulloblastoma.
  
  Practical tips Make sure the post COntr:J.St scan looking for drop metastases is done preopcrarh'dy as postoper:J.ti\'e haemorrhage and granulation tissue can cause connlsion. Haemangioblastoma is primarily a tumour of adults but can be seen in adolescents in the posterior rossa when part of\'on Hippcl- Lindau syndrome. It is typically a cystic mass \\~th enhancing mUr:J.1 nodule, SO has similarities with pi lacyt ic astrocytoma.
  
  Further management M RJ of the spine with intr:J.venous contrast enhancement should be undertaken to look ror 'drop metastases'. Neurosurgical assessment is then dearly appropriate.
  
  Further reading Koeller K, Rushing E (2003 ). From the archives of the AF IP: medulloblastoma: a comprehensive review \\~Ih radiologic-pathologic correlation. RadioGraphies 23: 16 13- 1637.
  
  321
  
  Answer 181
  
  Paediatric Imaging
  
  ANSWER 181 Observations (181a) There is a right sided aortic arch, The heart is boot shaped indicating right ventricular hypertrophy. The lungs are not plethoric, in fun there is a reduction in the calibre of the pullllOnary vessels. Ol"crall, the !caturcs Jre suggestive of Ell10r'S Tetralogy
  
  Diagnosis Tetralogy of FalJot. Differential diagnosis Of conditions associated with a right sided aortic arch: • Truncus arteriosus. • Tetralogy of Failor. • Transposition of great vessels. • Pulrnonarvatrcsia • VClltricul~r septal delcct (VSO ). Discussion Tctralob'Y of Failor is Olle afthc most common causes of c)lanotic congenital hean disease and is composed of the following: obstruction of right \"cntricular outflow, brgc VSD, right ventricular hypcnrophy and an o"erriding aorta. Radiological features on CXR arc· Conca"ity in tIle region of the pulmonary anery, which is small. Enlarged aona
  
  Normal sized hean. Boot shaped hean due to right ventricular hypertrophy. Right sided aortic arch in 25% of cases Decreased calibre of pulmonary ,"essels. Right sided aortic :lfch may also be seen in paticnts with another cause of neonatal cyanotic congenital hean disease - transposition of the great ,·essels. However unlike Fallot's there is increased pulmonary vaseularity and the heart has an 'egg on its side' appearance on the CXR due 10 Ihe f::lct that the mediastinum is narrow because of the abnormal relationship of the great vessels. An example is shown ( 18 1 b ), though in this particular casc the aortic arch is left sided.
  
  Practical tips • Fallot's tetralogy, pulmonary stcnosis and tricuspid atresia cause cyanosis with oligaemic lungs. Transposition ofthe great vessels, tmncus arteriosus and lotal anomalous pulmonary venous drainage cause cyanosis with plethoric lungs. Further management Cardiolo~' referral for echocardiography and consideraTion for a palliati,·c shunt or complete surgical repair.
  
  181b CXR in a child with tra nsposition of the great vessels. There is pulmonary vascular congestion and the heart has an 'egg on its side' appearance.
  
  322
  
  Pae diatric Imaging
  
  CASE 182 History ,\n II ·m,.., " ·,"1"" .. I ~-.h ",~
  
  .... J
  
  <><,.."s.,g~. 1
  
  ,b,,,,,," . 1; ,;. ~
  
  1"1'"
  
  Case 182
  
  Paediatric Imaging
  
  Answ er 182
  
  ANSWER 182 Observations (182) The posterior fossa is enlarged with absence of the cerebellar vermis, hypoplasia oCtile cerebellar hemispheres and the presence of a large posTerior fossa cySt, which is in continuity with the 4th ventricle. A vcntriculopcritoncal shunt is seen in the right l:trcral ventricle. There is \'cntriculomcgaly but the sulci arc. not clfJccd. The features arc consistent with Dandy- Walker malformation Diagnosis Dand).-Walkcr malformation
  
  Discussion The Dandy-Walker malformation is a congenital malformation whcreb}' the posterior fossa is enlarged and the tentorium ((rcbelli is elevated, howc"cr the cercbcll;u' hemispheres arc hypoplastic. Absence or hypoplasia of the cerebellar vermis is present with a posterior fossa cyst directly connecTed to the 4Th ventricle. Ventriculomegaly and dysgenesis of the corpus callosum arc associated findings. Most affected patients die in infancy. The less severe form, Dandy- \Valker variant, is more common and is not associated with enlargemelH of the posterior fossa. T he associaTed posterior fossa cySt is smaller and the cerebellar vermis is hypopl;lStic rJther thJn ;lbsent.
  
  There Jre numerous associated eNS anomalies, fo r example corpus callosum dysgenesis, holoproseneephaly, gyral dysplasia , grey matter migration anomalies and encephalocele. Associated anomalies outside the eNS include cleft palate, polydactyly and cardiac defe([s.
  
  Practical tips • A mega cisterna magna may mimic Dandy- Walker malformation, howe\'er there is no cerebellar vermis abnormality, continuity with or abnormality of the 4th I"entricle. • A posterior fossa arachnoid cySt may also mimic these appearances. If the posterior fossa is not enlarged and the cerebellar vermis is h~'poplastic rather than absent, consider Dandy- Walker variant rather than malformaTion.
  
  Further management The associated eNS abnormalities can be better identified on i\.j RI. T rearment often involves inscnion of a ventricular shunt, as in this case, to relieve hydrocephalus. Genetic counselling may be appropriaTe for the f.1mil)'.
  
  182 Absence of cerebellar vermis (left): sulci are not effaced (bottom left); ventriculomegaly (right); hypoplasia of cerebellar hemispheres (top right).
  
  324
  
  Pllediat,ic Imaging
  
  CASE 183 History mmmo&.
  
  Case 183
  
  Answer 183
  
  Paediatric Imaging
  
  ANSWER 183 Observations (183) Dextrocardia is present with situs solitus. A right femoral line is noted. An NG tube is present in Ihe stomach, which is dilated with air. There is a 'double bubble' appearance of the dilated stomach and duodenal cap with no gas seen distally. T he findings afC consistent with duodenal atresia
  
  Diagnosis Duodenal :nrcsi3, possibly pan ofVACTERL syndrome. Differential diagnosis Of ' double bubble' on abdominal radiograph: • An nuJarpancreas. • Duodenal diaphragm. • Peritoneal band. • Choledochal cyst.
  
  Discussion Duodenal atresia is due to failure of rccamllizatiOIl of the duodenum at around 10 weeks and is the most common cause of congenital duodenal obstmction. The other major cause is an nular pancreas, and both arc associated with Down's syndrom~. The ob~ (ruct i on is just beyond the ampulla in the majority of cases and the 'double bubble' results from gas-fluid levels in the first part of duodenum and stomach. Gas may be seen more distally in the bowel if there is duode nal stenosis rather than complete atresia (though atresia is twice as common).
  
  326
  
  Duodenal atresia is associated with the VACTERL syndrome, a non-random association of congenital abnormalities aftccting multiple systems, summarized by the mnemonic 'VACTERL'. Three or more of the associated defects arc required to make the diagnosis. The mnemonic is as follows· • Vertebral anomalies • Anorectal anomalies - imperforate anus. • Cardiovascular :momalics - most commonly endocardial cushion ddects. • Tracheo- Esophageal fistula • Renal anomalies - ma}' be associated with a single umbilical artery. • Limb anomalies - e.g. radial dysplasia, polydactyly, syndactyly. The characteristic cardiac abnormality is a septal defect but dextrocardia, as in this case, has been described.
  
  Practical tips Always check the 'double bubble' radiograph for VACTERL associations, e.g. vertebral anomalies on the film Further management Fluid and electrolyte imbalance must be corrected along with decompression of the stomach \~a NG tube insertion. Surgical correction is then required, usually with good
  
  Paediatric Imaging
  
  CASE 184 Hist o ry ~
  
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  Answer 184
  
  Paediatric Imaging
  
  ANSWER 184 Observat ions (184a) Lateral spinal radiograp h shows ve rtebral bodies arc fla ttened with ccnll':li bcaking anteriorly. There is also widening of the intcrvcrtcbr.l! disc spaces and posterior ,'ertcb ral scalloping_ T he find ings arc suggestive of Morquia's synd rome.
  
  Diag nosis Morquia's ~yndrom c.
  
  Differential diagnosis • Hurler's syndrome. • Achondroplasia.
  
  Disc ussion Morquia's syndrome is a rare metabolic disorder classified as one of the rnuco polys3C(ha ridoscs. It is autosomal recessive and presents in childhood wi th characteristic skeletal dcfonnity and dwarfism. Patients also ha\'c deafness and card iac dysfunction, howc"cr Ihey may well Ii"c [Q adul thood . Atlantoaxial subluxation is a feature and there may be absence o f thc odontoid peg. Radiograph of the
  
  cervical spinc in the same p;]tienr (I 84b) shows mul tiple flattened vertebral bodies and abscnce of the peg. H urler's syndromc (another of the mucopol yS3ccharidoscs) and achondroplasia can have similar radiological features; in particular, both ma y causc posterior ,'enebral bod)' scallopin g and anterior venebral body bcaking T he radiologic;l l fca nlres of Morq uio's s)'l1drome arc described below: Spine: Posterior ,·crtebral sc;]lIoping. \Videning of intervene braI disc spaecs. Congenital flattening of the ,·enebral bodies ( plar)"spond)"l)'). Ante rior bcaking of ,·crtebral bodies. Atlantoaxi;ll sublux;]lion. K~'phoscoliosis .
  
  Pelvis: • Fragmem";]tion and flatten ing offemoml hcads ( 1.4<). • Flared ili;]c wings ( 184c).
  
  184c AP radiograph of pelvis showing fragmentation and flanening of the femo ral heads with fl aring of the iliac wings.
  
  184b Lateral cervical spine demons trating absence of the peg w ith flattenin g and posterior scalloping of the ve rtebral bodies.
  
  328
  
  Answer 184
  
  Paediatric Imaging
  
  Lower limbs: • Sloping of superior margin of tibial plateau laterally ( 184d ). • Genu I'algus deformity ( 184d ).
  
  Hands and feet" • Proxirm.l tapering o f the metacarpal bones producing 'bullet-shaped' tllcrac3.rpJls ( 184e). • Short \\;dcncd mbular bones with metaphyseal irregularity ( 184e).
  
  Practical tips It may be vcry difficult on imaging to diAcrcntiatc
  
  Morquio's from achondroplasia or dle olher mucopol~'­ sacchlridoscs. Some featu res may help radiological diflcrentiation: Cau(bl narrowi ng of the spinal callal is not a Icarmc of the mucopolysaccharidoscs however it is present in ac hondroplasia; therefore assess the intcrpcdicubr distance on the All of the spine . • The 31ltcrior vertebral body beaks in 1\1orquio's tend to be in the Middle of the "enthral body whereas in Achondroplasia and Hurler's syndrome the y arc Ameroinfcrior.
  
  If the spine radiograph includes the craniocer\'ical junction always assess the peg as this ma ~' be absent in ~'I orquio's and there may be atlantoaxial subluxation. On a peh'ic radiograph flaring o flhe iliac wings "iJJ be seen in achondroplasb and the mucopolysaceharidoses, howC\'er in achondroplasia the sacrum may be horizontal in orient-J.tion therefore appearing abscnt (see Case 15 1).
  
  Further management Mortality/ morbidity arc related to atlantoaxial instability due to odonlOid peg hyperplasia. In addition, respirarory complications arc common due to chest wall deformity.
  
  Further reading \Vakely S (2006). The posterior vertebral scalloping sign. R.ndiology 239: 607- 609.
  
  184d AP radiograph of both knees shows genu valgum with sloping of the superior margins of the tibial plateau.
  
  329
  
  Chapter 6
  
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  Breast Imaging
  
  CASE 186 History A 56~ycar· old woman with a firm mass in the ccmrallcft breast.
  
  Case 186
  
  ANSWER 186 Observations (186a, 18Gb) Bilateral mammograms show that in the upper central and hucral aspects of the left breast there is extensive pleomorphic microcalcification. Within the left axi lla there is a 21 mill diameter rounded node. The right breast appears normal. Ultrasound of the superior central left breast demonstraTes an irregular solid mass. The extensive microcalcification is of a configur:ltion suspicious for malignancy in the central left breast. There is possible metastatic spread ro the large axillary node. The ultrasound demonsrrates a probable malignancy. Furrhcr investigation with urgent biopsy of the lesion is required.
  
  Diagnosis Ductal carcinoma ill situ ( DC IS ) with involved axillary nodes.
  
  Differential diagnosis For pleomorphic calcification on mammograms: • DCIS. Atypical ductal hyperplasia. • Fat necrosis. • Fibrocystic change.
  
  Look carefully for signs of an invasive focLis within the ca1cifieltion - search for a spiculate mass or stromal deformity. If not identified, assess further with ultrasound and possibly MR imlging.
  
  Further management Further imaging assessment is suggested : mammographic magnification orthogonal views (typically craniocaudal and mediolateral ) may evaluate morphology and distribution of the microca1cification. Ultrasound guided biopsy of the solid lesion lnd mlmmographic stereotactic biopsics to Obtain a sample containing calcification should be performed. The sllspicious node should be sampled under ultr;l.sound guidance. In this case, the biopsies showed : ultrasound solid lesion - invasive carcinoma; stereotactic carl'S - DClS; axillary node - malignant cells. Once a diagnosis of malignancy has been established, surgical rcferral is required. In this case the pltien r underwent a mastecromy and axilbry lymph node clearance. Histological findings ,,·ere of a 12 mm I DC in thc superior ccntral breast with extensive (7 cm) intermedi,He grade DCIS; 4 Ollt of 15 axillary nodes were involved with tumour.
  
  For enlarged axillary nodes: • Ipsilateral breast malignancy. Infection/inflammation of ipsilateral breast or arm. Collagen vascu lar discase/rheumatoid arthritis. Lymphoproliferative diseases: lymphoma and leukaemia . Metastases (melanoma, lung, contralateral breast). HIVadenopathy.
  
  Discussi on Pleomorphic microcalcification may be defined as irregular calcifications of varying sizes and shapes, usually <:0.5 mm in size. Orthogonal mammographic views may darif)' the characteristics - for instance, lilH.:ar and segmental distributions suggest that the calcification is ductal in origin, whereas regional or diflilsc multiple bi!ater.ll groups are less li kely to represent a ductal process. A small percentage of mllignant lesions arise from the stromal elements of the brelst. Ninety per cent of breaSt cancers have cellular features thlt arc similar to ductal epithelium and arc consequently classified as ductal cancers. When confined ro the duct thev arc te rmcd ductal carcinOllll ill sitll ( DCIS ). When the cells have breached the basemenr membrane around the duct and invaded the surrounding tissues, they arc termed invasivc ductal carcinoma ( IDC). The diagnosis of DCIS is associated with the possibility of associated invasive disease. If no invasive focus is identified on mammograph y further assessmenr with ultrasound and possibly J\lR imaging with a view to potentially finding an invasive component may be performed.
  
  Practical tips Not infi·equently, microcaJcification is ofequi\·ocal configuration - if there is doubt there is a low threshold to proceed to biopsy.
  
  336
  
  186a Left MlO: axi llary lymph node (upper arrow) and extensive microcalcification (lower arrow).
  
  Breast Imaging
  
  Cases 187, 188
  
  CASE 187
  
  CASE 188
  
  Hi st ory A 33-year-old woman with a soft mobile smooth left breast lump.
  
  Hi story A 64-\'car-old wom:all with a swollen CI)1:hC;'l\:UOllSright breast.
  
  33j
  
  Answers 187, 188
  
  Breast Imaging
  
  ANSWER 187 Observations (187a. 187b) The breasts arc heterogeneously dense. There arc scmiovoid low-density opacities in bmh breasts. There is a large dominant lesion in the upper Ollter left breast. Ultrasound of the left breast lump demonstrates:I wel! defined smooth margined anechoic mass with through transmission. The appearance of the lesion assessed with ultrasound is in keeping with a benign simple cyst. Diagnosis Benign simple cySts.
  
  Differential diagnosis Of:l smooth low-densi ty lesion on mammography: • Simple cyst.
  
  · Oiteyst. Fibroadenoma. • - 1.5% of circumscribed round lesions Illay be malignancies.
  
  pc:rimc:nopausally in many women, but can be found in women of all ages. Cysts may develop after commencing oestrogen (hormone ) replacement therap~'. On mammography, cysts appear as semi-ovoid masses with variable margins and density. There may be a peripheral halo and/or rim egg shell calcification. On ultrasound, cyStS usuall)' appear as well defined, anechoic masses ,\~th posterior acoustic enhanccmem. In some, high specular echoes shift in position as gain is increased ('gurgling' cysts) . Posterior enhancement is not always demonstrable, particularly if the cyst is small or dose to the chest wall. If there is an y question as to the cystic narure of a lesion, aspiration is recommended. On occasion lesions appear cystic on ultrasound, but aspiration is unsuccessful - thick prOTeinaceoUs fluid may be tOO gelatinous to be aspirated.
  
  Practi ca l tips
  
  Of an echoic lesion on ultrasound: • Simple cyst. Complicated (proteinaceous) cyst. Duct ectasia. • • illtr:J.dl!craljintracysric papilloma - look carefully on ultrasou nd for a mural lesion.
  
  • Avoid satisfaction ofsul"\'ey: look for other, more suspicious lesions. CyStS often recur after aspiration. If the lesion is dearly a simple: cyst, aspiration is not required unless the symptoms of the mass arc distressing. Irlhere is any doubt as to the nature or the cystic lesion, core bioI'S), is suggested.
  
  Discussion
  
  Further manage ment
  
  Cyst's arc asymptomatic in many women. Presentation is l'ariable. A palpable mass or masses may develop rapidly and is/arc associated with tenderness. They del'elop
  
  Ir there is any doubt about the mammographic appearances, further el'aluation with ultrasound should be undenaken.
  
  ANSWER 188 Observatio ns (188) T here is diffilsc tr.lbecular prominence throughout the right breast which is or generalized increased density. T he skin is thickened. There arc enlarged nodes in the right a .~ iIla. The left breast is normal.
  
  Di ag nosis Probable inflammatory right breast cancer nodal illl'oll'emcm.
  
  \\~th
  
  axillary
  
  Differential diagnosis For diffusc trabecular/skin thickening: • Post radiotherapy change. Progressive systemic sclerosis. Obstruction ortht' superior I'ena cava. Lymphoma. infection/ inflammatol)' mastitis - most common in lactating women. • Tr:lllma. • Generalized oedema due to causes such as congesu"e hean f.l ilure or nephritic syndrome.
  
  Di scussion Uhr:lsound or MR imaging may be used to find a discrete
  
  338
  
  invasive focus which could be biopsied. The diagnosis could also be obtai ned from skin punch biopsy or rrom image guided core biopsy of an axillary node. Inflammatol)' breast cancer may be defined by clinical diagnosis dependent on findings of oedema, el)'lhema and 'pcau d'orange' or on histological findings of metastatic breast cancer in dermal lymphatics. The definition is debatable: not all women with clinical findings suggcsti,'c or inflammatory breast cancer have iOl'olved dermal lymphatics and ~ot all patientS with tumour cells in the dermal lymphatics present wi th signs of inflammation . Inflammatory malignancies account for 1%of all breast cancers and up to 40% of locally adl'anced breast cancers. The diflcrentiation between mastitis and inflammatol)' carcinoma may be ditlicult.
  
  Practica l t ips Patients often undergo neoadjuvam chemothcrapy prior to mastectomy. Consider infla!J1matory breast cancer when an inflamed breast fui ls to respond to a brief course of antibiotics.
  
  Breast Imaging
  
  CASE 189
  
  CASE 190
  
  History A 42-rear-old mail with a soft mobile
  
  History
  
  tender Icft breast lump.
  
  Cases 189, 190
  
  Screening mammograms in a 57 -rearold womall.
  
  339
  
  Answers 189, 190
  
  Breast Imaging
  
  ANSWER 189 Observations 1189) In the left brC:lst there is l full shaped density emanating from the nipple which gradually blends into surrounding lany tissue. The right breast appears normal Diagnosis Gynaccornasria.
  
  Differential diagnosis O f a breast lump in a male: • Gvnaccomastia. !\Ialc breast cancer (circuillscribed or spiculate mass usually evident; often eccentric to the nipple ). Pscudog}'naccomasria (f.1rty cnbrgcmctlt with no ductal o r stromal prolitcration; secondary to obesity). Di:lbctic mastopathy (firm mass in patient with longstanding type I diabetes mellitus ). Abscess (cITthcma; acute histOry). Discussion Gynaccomastia usually appears as a fan shaped density emanating from the nipple, gradually blending into surrounding fat. Three mammographic patterns of gynaecomastia have bce.n described: nodular, dendritic and dirfuse. Tllere may be prom inent extensions into the surrou nding fu t and, in some cases, an appearance similar to that ofa hetemgeneollsly dense tcmalc breast. Although
  
  there arc characteristic mammographic features that ailow breast cancer in men to be recognized (round/spiculate subareolar mass typically eccenTric to the nipple), there is substantial overbp between these features and the mammographic appearance ofbcnign lesions. Male breast cancer is rare, accounting lor < I % of all male cancers. Gynaecomastia is characterized by hyperplasia of ductal and stromal clements of the male breast . It manifests clinically as a soft, mobile, tender mass in the retroareolar region. Gynaecomastia has been associated with an increased semm b·cI of oestradiol and a decreased level of teSTOsterone - this may occur with physiological changes at puberty and senescence and be caused by endocrine disordcrs, systcmic diseases, neoplasms and certain drugs including anabolic steroids, cimetidine, spironolactone and marijuana
  
  Further management On the diagnosis of gynaecomastia it is important to corrdate the imaging findings with the clinical histOry. Many cases of gynaecomastia are idiopathic bur underlying causes should be investigated - senUll hormone b ·cls should be taken. Ask for a drug history and the presence of signs of chronic renal insufficiency, cirrhosis and a testicular mass; other imaging im·estigations perr:a ining to the patient may raise one of these possibilities.
  
  ANSWER 190 Observations (190) 1n the antelior aspects of both breasts there arc smooth \\"ell defined spherical calcifications with lucent cen tres. No abnormal masses or distortion arc seen. Diagnos is Bilateral calcificatio n of benign configuration. Differe ntial diagnosis For benign calcification: • Vascular - usually secondary to medial atherosclerosis . Mav be. associated with diabetes and hYl;erparathyroidism. Often demonstrates a characteristic 'train track' configuration. Fat necrosis - peripheral calcification in a lucent mass; histOry oftraullla or surgery. Fibroadenoma involution - 'popcorn ~ like' calcifications usually beginning at the periphery and the n ill\'olvi ng the central portion. 'Milk of calcium' - a benign process that call be diagnosed with magnification views of orthogonal projections: on the CC view, calcifications appear poorly defi ned and sllludgy; when imaged on the MLO view, rhe calcifications are seen as sharply defined and cresccnt shaped or linear. Plasma cell mastitis an d duct ectasia - large rod-like calcifications orienled along the axes of the duct:al
  
  340
  
  system. T hese calcifications h:nd to be coarser and larger (usually >1 mill in diameter) than malignan t calcifications. Skin or dermal - usually spherical and lucent-centn: calcifications at the periphery of the breast. Suture - usually seen :at a known surgical site. The calcifications ma~' be linear or tubular.
  
  Discussion Calcification is a frequent finding on mammograms. The arrangement of calcification aids categorization as to whether it is benign or malign.ult. Clustered (occupying a ,·olume
  Breast Imaging
  
  Case 191
  
  CASE 191 History ~ ~"~ ,n, m,oid kif on ",,' in ..."" ,.,.,." ~ ,,"
  
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  Further Reading AidJ to Radiological Diffirmtia/ Dif/gllosis Stephen Chapman , Rich:ml Nakidny \VB S;1UllrlCrs, 4th edn , 2003
  
  NtIITOTfldi%m- TIJe ReqlliIiteJ Robert I Grossman , D:a\'id M Youscm Mosby, 2nd edn , 2003
  
  Clillicnllmngillg: An Arlal of J)ijfirwtiai Diagnosis Ronald L Eisenberg Lippincott Williams and Wilkins, 4th cdn, 2002
  
  A Barko\'ich
  
  Diagnostic lI"aging Head fllld Nuk ltic Harnsbcrgcr Amirsys, 2004
  
  Radi%m Rtl'itw M all lla/ Wotfg:ang Dahncrt Lippincott WitlianlS and Wilkins, 6th (dn, 2007
  
  FlIlIdfllllClltnls o/Dif/gIlMt;, Radi%!J.Y William E Brant , CI),de A Helms Lippincott Williams and Wilkins, 3rd cdn, 2006
  
  Sc/f-Al!Cl!JIICllt C% m- Rtl!iew of NtIITOilll(Jgillg Ki rsten Forbes, Sanjay Shetty, Michael Lev, Joseph H cis(rll1:an J\'lanson Publishing Ltd , 2008.
  
  Grail/oer (Illd AlliJon's Diagllostic R adiology: A Textbook of Medical /lIJflgjllB
  
  Ronald G Grainger, David J Allison , Adrian K Dixon Churchill Livingsto ne, 4th edn, 2001
  
  lmagill!] of DisenJeJ of tile Chest D~l\'id M H:mscll , Peter Armstrong, David A Lynch, H Page McAdams j\'losby, 4[h cdn , 2004
  
  Pfledifltrit NtllTOiwflgi"g
  
  Lippincott Willi:ams and Wilkins, 4th edn, 2005
  
  Sdf-Al!el!lIItI" CO/OIIT Rel'iell' oj 'nJorfl cie Imflging Sue Copley, DJvid M . H ansell, Nestor L. Muller Manso n Publishing Ltd, 2005 Textbook of Rfldi%g:y fllld Imflging David Sutton, Ro dney Reznck, Janet Murfitt Churchill Livingstone , 7th (dn, 2002
  
  Mnglltlic Resollflll 'e IlIIng;II9 o/the Brf/ill and Spille
  
  SconWAtias Uppincorr-R.wen, 2nd edn, 1996
  
  343
  
  Index of Differential Diagnoses , .1.;;f"'.. ..,. (" .,;. _JI " 'h,I",;, !7
  
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  General Index pncumalOsis (tontinlled) cystoides intestinalis 66 intestinalis 65, 167 ,.. pneumobilia 41 pneumoconiosis 6, 7 PlIelfmotystis tan"ii pneumonia ( PCP) 37 pneumomediastinum 34,165 meconium aspiration syndrome 170 pneumonia, aspiration 171 pneumoperitoneum 34,75 necrotizing emerocolitis 167 pneumothorax 3 1 bulla diflerential diagnosis 35 cystic fibrosis 36 endometrioma 83 Langerhans cell histiocytosis 27 meconium aspiration syndrome 170 PllwmotyJtis tnrjlljj pneumonia 37 positive pressure \·entilation complication 165 polycystic kidney diS(:asc 48 polydactyly 177 polyhydramnios, annular pancreas 44 polymicrOb..yria 169 portal hypertension, oesophageal varices 42 positive pressure ventilation 165 positron emission tomography (PET), merasl';nic bronchogenic carcinoma 24 posterior cnlciate ligament 159 double PCL sign 159 postcrior inferior cerebellar artery infarction 100 posterior urethra! vah·es 172 Po u 's disease 142 prematurity hyaline membrane d isease 165, 170 necrotizing enterocolitis 167 primary sclerosing cholangitis 45 primitive neuroectodermal tumour ( PNET) 180 progressive massive fibrosis 7 prostate carcinoma, metastascs 140 Protws, emphysematous pyelitis 63 pmne belly, posterior urethral vah·es 172 pscudodi\·erticulosis, intr.lmural 38 pseudohyperparathyroidism 147 pS(:udo-Maddung deformity 130, 153 PsclldomollaJ, emphysematous pyelitis 63 pseudoureteroceles 56 psoas abseess 142 psoriasis 11 6 psoriatic arthropathy 143, 152 pulmonary arterial hypertension 16
  
  pulmonaryarterim·enolls malform:uion 33 pulmonary asbestosis 3, 15 pulmonary embolus 31 pulmonary fibrosis dmg-induced 18 lowcrzone 15, 17 upper zone 15,17, 18 pulmonary haemosiderosis 23 pulmonary interstitial emphysema (PIE) 165 pulmona!)' malignancy asbestosis 15 systemic sckrosis 17 pulmona!)' metastases, cavitating 14 pulmona!)' nodules follo w-up 6 mitral vah'e disease 23 oesophageal tumour 14 rhcumatoid lung 6 sarcoidosis I pulmona!)' oedema 3 1 heart failure 29 pUlmOlla!)' opacity barium aspiration 20 ground glass 29 Icft upper lobe tumour \,~th lrmphangitis carcinomatosa 12 lung lobe collapS(: 14 metastatic calcinosis 20 pleural pscudotumour II puhnona!)' sarcoidosis 1 pulmonary \·cnous hypertension mi tral \'alvediscasc 23 pulmona!)' oedema due to heart failu re 29 pyelitis, emphyscmatous 63 pyclou reteritiscystica 77 pyknodysostosis ! 19, 168 Pyle's disease 119, 178
  
  renin-angiotensin system, o\'eracrivity 58 rcspirarory distress, meconium aspiration syndrome 170 reversal sign 114, 163 rheumatic heart disease 23 rheumatoid arthritis 120,143 rheumatoid factor 17 rheumatoid lung 6 rheumarologic syndromes, histoplasmosis 25 rib. bilateral cervical 28 eXOStoses 130 lesions 9 notching 32 rickets 168, 175, 176 fig ht ventricular hypertrophy, mitral valve disease 23 Rigler sign 75, 167 Rigler's triad 41 Rokitansky nodule 84
  
  Rathke cleft cyst 106 Raynaud's phenomeno n 17 thoracic Outlet syndrome 28 rectum st'ent'ed tumour with calcified liver metastases 52 ulcerative colitis 65 Rdter's synd rome 116, 143 renal adenocarcinoma 5 1 renal arte!)' stenosis 58 renal ectopia, crossed nlsed 68 renal lesions, fat 48 renal medullary nephrocalcinosis 43 renal obstmction, hydronephrosis
  
  sialectasis, juvenile punctate 87 sicklccclidisease 129, 140, 155 siderosis, transfusion 50 sigmoid colon, displacement 84 sigmoid mlvulus 46 silicosis 18 simple bone cySt 115 Sj6grcn's syndrome 87 skin folds, lung edge art:efuct 35 skin nodules, neurofibromatosis type I 8 slipped upper femoral epiphysis (SUFE) 117 small bowel adenoma 49 ischaemia 59 loop dilatation 69 strictures 59 thickening in Crohn's disease 53 small bowel obstrucdon 4 1 adhesions 47 mechanical 4 1,47
  
  S sign of Golden 14 sacral agenesis 173 sacroiliac joint disease Crohn's disease 53 ulcerative colitis 65 sacroiliac joint fusion 139 sacroiliitis 116,143 sandwich ,·enebrae 140 sarcoidosis cardiac sarcoid 22 pUinlOna!)' 1 schistosomiasis 62 schizcncephaly 169 sclerpdcrma 132, 152 sclerosis Charcot joint 138 dilYuS(: 119 sep~\1 thickening, interlobular 29
  
  68 renal osteodystrophy 11 9, 140 renal papilla!)· necrosis 78 renal scarring, infections 68 renal stones, horseshoe kidney 5 1 renal lUberculosis with autonephrecromy 74 renal tubular acidosis 43
  
  351
  
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