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proportion of soft-tissue tumors and the ma j ority of bone tumors appears in the first two decades of life (Fig. 2 1 . 1 .), and the patients often relate their complaints to sports injuries and traumas. In cases involving unexpectedly long-lasting and increasing symptoms follow ing a trauma, tumor is to be considered and X-ray tests must be performed. The period of the complaints is dependent on the rate of growth of the tumor and may vary extremely wide. In cases of a rapidly ex panding Ewing 's sarcoma or a highly malig nant central osteosarcoma it is often a few weeks or months, but in cases with less malig nant chondrosarcomas it can be many years. The benign tumors and turnor-like lesions usually grow slowly. Malignant soft-tissue tumors may remain undetected for a long time, especially if they are localized deep down, covered by muscles. In these cases, dull pain is the only symptom, which does not disappear even at rest. Those tumors that penetrate the superficial fascia are detected earlier, they cause large swelling, the skin is warmer above them and the venous network is expressed. The most significant symptoms of bone tu mors are also swelling, a warmer skin and pain. The latter does not depend on the load, in the beginning it is intermittent and pricking, later radiating, constant pain is perceived. Both benign as well as malignant tumors in the vicinity of joints may induce a restriction Benign �
of j oint motion and reactive synovitis with j oint fluid collection. If a soft-tissue or bone tumor expanding from the bone involves ves sels, the significant symptom is radiating pain and edema of the limb. Pathologic fractures may occur as the first symptoms in cases of benign tumors (e.g. enchondroma) or turnor-like lesions (e.g. ju venile bone cyst), which may have been pres ent for a long time and extenuated the cortex. The fracture arises spontaneously or in re sponse to a minimal trauma. Malignant bone tumors, bone destructive soft-tissue tumors usually start with pain. X-ray and other imaging procedures. In the diagnosis of soft-tissue tumors X-ray tests are of modest help. Only the fat-tissue tumors (lipomas, liposarcomas) can be differentiated as they are more radiolucent than the sur rounding muscle and bone tissue. If a primary tumor is discovered, antero posterior (ap) and lateral chest X-ray is man datory to detect possible lung secondaries. As bone tumors grow, they change the shape and the structure of the bone, producing typical (but not absolutely specific) X-ray changes, so X-ray therefore plays a decisive role in the preoperative diagnosis and treat ment planning. Benign bone turnors usually grow slowly and there is time of the shape of the bone to change, and for a demarcation reaction to de-
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X -ray features of benign and malignant bone tumors
2 1 . Tu m o rs of t h e m u s c u l o s ke l eta l system
velop. The border o f the intact and affected bone tissue is usually well-defined, and the le sion is often surrounded by a sclerotic margin (non-ossifying bone fibroma, osteoid osteoma) (Fig. 2 1 .2.). Occasionally the bone appears "inflated" (e.g. enchondroma in pha langes of the fingers) (see Fig. 2 1 . 1 5.). With malignant tumors, the border be tween intact and affected bone tissue is vague il-defined, the sclerotic margin is missing. If the bone shows fusiform widening (e.g. chondrosarcoma), this is a sign of slow expan sion. Nevertheless the cortex and also the
Fig. 21.3. a: A- P film of the right pelvis, with a lmost invisible changes the iliac bone is slightly more radiodense b: The a image shows a tumor originating from the iliac bone, interspersed with calcifications invading both the gluteus muscles and the pelvic soft-tissues
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neighboring soft-tissues are soon infiltrated. There is often a periosteal reaction, such as spiculum formation in osteosarcomas (Fig. 2 1 . 1 2.),laminated "onion peel" reaction in Ewing 's sarcoma (Fig. 2 1 .2 1 .) . Indirect signs of extraosseal invasion o f the tumor are shown o n traditional X-ray films, as calcified foci deposited in a hyaline matrix (Fig. 2 1 .3. b), or spiculum fOrmation in osteosarcomas. It is important that the penetration of cer tain types of tumors (giant cell tumor of bone, metastasis, bone fibrosarcoma) into the cortex is never accompanied by a periosteal reaction. The availability of modem non-invasive imaging modalities, such as CT and MR, angiography and arteriography have lost much of their significance, but they are still useful diagnostic or therapeutic tools in cases of soft-tissue tumors and expansive, infiltrative bone tumors. Angiography can provide information on the extent, blood sup ply of a tumor, its relation to important ves sels, displacement or invasion of vessels (Fig. 2 1 .4. a). Arteriography may be supplemented by therapeutic interventions. Cytostatic drugs can be inj ected intra-arterially in high concen tration, directly into the tumor, or the supply ing vessels can be embolized. In this way, the tumor temporarily shrinks, its removal is eas ier and the bleeding can be reduced to a great degree. Computer tomography (CT) is useful di agnostic procedure mainly in cases of bone tu mors, and less for soft-tissue sarcomas. Tomographies are done in a horizontal plane, and even a very small bone destruction, intra and endosteal propagation can be demon strated. CT is mandatory in tumors of the shoulder girdle, pelvis and spine, where single bones, bone structures are proj ected in tradi tional X-rays to cover each other, and thus the localization of a tumor can not be accurate (Fig. 2 1 .3. a, b). The most significant method is the MR (magnetic resonance) imaging, which is based on a non ionizing radiation source, and is therefore harmless to the organ ism. It is excellent for visualization of the intramedullary and of bone tumors, and their
188
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2 1 . T u m o rs of t h e m u s c u l o s k e l eta l system
intraosseal extent, metastases (skip meta stases) (Fig. 2 1 .4. b). Since it is less sensitive than CT depicting possible cortex break through by the tumor, it is often applied in combination with CT. Common isotope test used for bone tumors is the 99 Tc-polyphosphate bone scan. Pathologically high osteoblast activity is indicated by a high uptake of the isotope (Fig.
2 1 .5.). The test is extraordinarily sensitive, but less specific : Apart from tumors, in creased activity can be induced by degenera tive, traumatic or inflammatory changes. Its main indication is the early detection of multi plex bone tumors or metastases and observa tion of the effectivity of chemotherapy. Fol lowing effective preoperative chemotherapy the isotope uptake of the turn or decreases. Iso tope tests are of little value in the diagnosis of soft-tissue tumors . In the recent years ultrasound investiga tions developed considerably and became more sensitive. They play no role in bone tu-
a b
Fig. 2 1.4. a. Angiography: hypovascularized periosteal osteosarcoma dorsally on the d istal metaphysis of the femur, d islocating the a rtery b. Sagittal MR image of the same periosteal osteosarcoma: the tumor has broken through the fe mur cortex in the area marked with arrows and has spread into the intramedullary cavity
Fig. 2 1.S. Bone scan: apart from the growth plates, there is high isotope uptake shown in the proximal meta diaphysis of the right femur (Ewing's-sarcoma).
2 1 . T u m o rs of t h e m u s c u l o s ke l eta l system
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189
crease may be indicative of recurrence. This level is also high in disseminated cancer metastases, when high quantities of calcium and phosphorus dissolve in the serum from the diluted bone tissue and the ion level may be elevated. The acid phosphatase level is in creased in metastases of prostate cancer, while in cases of neuroblastoma the excretion of vanillic-amygdalic acid and homovanillic acid are considered characteristic. Important diagnostic indicator in myeloma patients are the appearance of Bence-Jones protein in the urine, and increased levels of serum proteins. Paraproteins are generally detected by im mune-electrophoresis. Sternal puncture can be performed to detect affected plasmocytes, however a normal bone marrow does not ex clude this condition.
Fig. 21.6. Ultrasonografic image: the base of the picture is the femoral cortex, seen as a white line, above it, a re current chondrosarcoma is seen in the vastus mus cle, the calcifications in the tumor su bstance are visi ble as white spots
mor cases, since bone tissue "blocks" out intraosseal processes. With soft-tissue tu mors, it is an essential non-invasive and al ways repeatable examination method, the size of the tumor can be identified in three dimen sions, and the tumor can be differentiated from fluid-containing cysts (Fig. 2 1 .6.). A connected use is the ultrasound guided needle biopsy taking from soft-tissue tumors. Laboratory investigations. Bone tumors do not initially cause bone metabolism changes, which can be detected by laboratory tests. An early sign is the increased sedimen tation rate, but this is not specific. The serum alkalic phosphatase level is characteristically high in osteosarcomas (it is excreted by the tu mor cells), its decreased level proves the effi cacy of chemotherapy, whereas a repeated in-
Biopsy, histological test. Regarding to the diversity and the relative rarity of bone and soft-tissue tumors, which hampers a differen tial diagnosis, establishment of the exact diag nosis and surgical planning, almost always re quire biopsy and histological examinations. Amputation is contraindicated without a histological opinion ! In cases of soft-tissue tumors aspiration cytology is possible, the specimen is obtained by needle biopsy. In the overwhelming maj ority of bone tu mor cases open surgical biopsy is performed, with adherence to strict rules. The scar should be positioned so that the surgeon might re move it during the definitive surgery (there is a risk of recurrence in the scar) . Regarding the numerous differential diag nostic problems close cooperation and con sultation is essential between the pathologist, radiologist and surgeon. Treatment. The variety of surgical inter ventions depends on a number of factors for both soft-tissue and bone lesions, the age and general condition of the patient, the localiza tion of tumor, and particularly the surgical stage of the tumor. Latent, active and aggressive forms of be nign tumors are differentiated. A latent tumor
190
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2 1 . T u m o rs of t h e m u s c u l o s ke l e t a l system
usually does not cause clinical signs, it ap pears coincidentally, it does not grow, or only very slowly, and occasionally it may heal spontaneously. Examples include lipoma, osteoma, etc. An active benign tumor grows slowly, does not extend from its anatomic sheath, has its own capsule. Examples are enchondroma, chondromyxoid bone fibroma. Aggressive benign tumors can expand gradu ally, or rapidly, emerging from their anatomic casing (fascia border, periosteum), but usu ally do not metastasize (or very rarely). Ex amples are desmoids and giant cell bone tu mor. The surgical classification of malignant bone and soft-tissue tumors is based on three considerations (Table 2 1 . 1 .) : 1 . The level o f malignancy o f the tumor (low malignancy: G 1 , high malignancy: G2
Table 21.1. , Surgical classification of benign and malignant tumors (Enneking) Classification by histological g rade
Classification by localization
GO G1 G2 TO T1 T2
Classification by metastasis
benign low malignancy high malignancy
=
=
=
=
=
=
MO M1
intracapsular i ntracompartmental extracompartmental
=
=
no metastasis regional or distant
Benign tumors
1. latent
GO
TO
MO
2. active
GO
T1
MO
T1-T2
MO
3. aggressive GO
Malignant tumors
LA I. B
G1 G1
T1 T2
MO MO
11. A 11. B
G2 G2
T1 T2
MO MO
Ill. A Ill. B
G l-2 G l-2
T1 T2
M1 M1
2 . The relation of the tumors t o their ana tomic compartment, intracompartmental forms (T l ) and extracompartmental forms that broke through (T2). 3. Absent (MO) or apparent metastases (M I ) at the time o f discovery. The above 3 factors have the highest prog nostic value, so there are significant differ ences between the surgical stages regarding the 5 year survival rate. The radicality of the surgical intervention should be chosen in ac cordance with this. Group I contains tumors of low malignancy (e.g. chondrosarcoma, fibrosarcoma, differentiated liposarcoma); If A: tumors have not broken out from the compartment (e.g. intraosseal chondrosar coma), or lIB : have broken out from the com partment (e.g. soft-tissue infiltrative chondro sarcoma). Group 11 includes lesions with high malignancy (osteosarcoma, Ewing 's-sar coma, malignant fibrous histiocytoma, etc.), which (IlIA stage) are intracompartmental, or (IIIB stage) have already broken out from their compartment. Group III includes those tumors of low or high malignancy, where the prognosis is very poor, since distant meta stases are already present at the time of diag nOSIS. Beside modem therapy (chemo-, ra diotherapy) complete removal of the tumor is essential . Intralesional curettage or marginal resection is permitted only for benign lumps, since tumor cells may be left behind. In cases of malignant tumors, wide (the incision is di rected in a few centimeters deep in the healthy tissue) or radical resection must be performed or if the tumor has already invaded the ves sels, amputation or exarticulation is to be per formed (Fig. 2 1 .7.). Now that effective che motherapy is available, limb-saving opera tions are done in more than half of the bone tu mor cases, resection of tumor being followed by reconstruction of the bone defect. The bone defect, which is usually large and in cludes a j oint, can be replaced with the pa tient' s own bone ( autograft), or bone from a bone bank ( homograft), or by modular tumor endoprosthesis system.
2 1 . T u m o r s of t h e m u s c u l o s ke l et a l syst e m
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Fig. 21.7. Type of surgical procedures according their radicalities. 1: radical intervention: amputation, exarticu lation, 2: wide resection, 3:marginal excision, 4: intralesional (cu rettage of the defect)
Bone cavities are filled with homologous or autologous ground bone, or bone cement (Fig. 5.8. a, b). If the tumor has destroyed the j oint sur face, the joint must be replaced or the defect may be bridged over by a bone plate to stiffen it (resection arthrodesis). This has an advan tage that, after the bony union, the limb will be stable and can bear weight, the disadvantages are the stiff joint and the poor quality of life.
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The defect can be replaced b y a n osteo articular graft from bone a bank, but integra tion is slow (6 months- l year), and both early and late complications are common (ligament insufficiency, bone collapse, fatigue frac tures, etc.). More recently the use of tumor endoprostheses has become popular, they have the advantage that the moving joint pro vides good function, but the prosthesis may work loose after some years and the bone de fect will then be even larger. Chemotherapy. The overwhelming ma j ority of bone tumors were earlier considered as chemotherapy-resistant. In the most recent decades it became clear that besides Ewing 's sarcoma and central osteosarcoma, which lat ter were thought to be resistant are indeed sen sitive to adriamycin and to high doses of methotrexate. As a result of the introduction of pre- and postoperative chemotherapy the numbers of lung metastases and local recur rences have diminished and limb-saving oper ations have become possible even in cases of osteosarcomas, that have broken through the bone (Fig. 2 1 .8.). The survival rates are not significantly worse than in the amputation group (Fig. 2 1 .9.), and the 5 years survival rate has elevated from 20% to 60-70%. Chemotherapy is also being used in the treatment of soft-tissue tumors increasingly frequently, however the results are not so clearly beneficial as in osteosarcoma. The maj ority of soft-tissue sarcomas are moder ately sensitive to chemotherapy, and a tempo rary remission or an increased survival rate can be achieved through the use of very ag gressive protocols. Chemotherapy can be used as palliative treatment in cases of lung metastases. Radiotherapy. Bone tumors are generally radiotherapy resistant, except Ewing 's sar coma, where irradiation of the aimed bone may be effective to support chemotherapy and surgical therapy. At one time the maj ority of soft-tissue tu mors were considered to be radiotherapy-re sistant. Recently however, the available so-
phisticated radiation sources (linear accelera tors, high energy gamma-ray beams) permits the attainment of a greater deep dose that is better focused with a milder skin reaction. In soft-tissue tumors radiotherapy is used as an adjunct of surgical treatment preopera tively, to decrease the volume of the tumor and postoperatively, to expose the tumor bed, to inhibit local recurrence, specially following margin�l resections. In some forms oftumors, irradiation of the regional lymph glands may be needed.
2 1.2. Primary bone tumors 2 1.2.1. Histological classification of bone tumors This classification, based on histogenetic aspects, was prepared by a working party of the WHO (2002), it is generally approved and it has an advantage of an internationally stan dardized approach (Table 2 1 -2.).
2 1 .2.2. Bone forming (osteogenic) tumors Table 2 1.2. Histological classification of bone tumors
I.
Bone-forming tumors A) benign
1. osteoma 2. osteoid osteoma 3. osteoblastoma B) malignant
1.
conventional osteosarcoma (central, high-malignant) 2. central, low-malignant osteosarcoma 3. parosteal Ouxtacortical) osteosarcoma 4. periosteal osteosarcoma 5. su perficial, high-malignant osteosarcoma 6. teleangiectatic osteosarcoma 7. small cell osteosarcoma 8. secondary osteosarcoma
2 1 . T u m o rs of t h e m u sc u l o s ke l eta l syst e m 11.
Cartilage-forming tumors
A) benign lipoma B) malignant liposarcoma
1. 2.
8) malignant 1. 2. 3. 4. 5.
chondrosarcoma (primary and secondary) juxtacortical (peripheral) chond rosarcoma dedifferentiated chondrosarcoma mesenchymal chondrosarcoma clear cell chond rosarcoma
X. Neural tumors Neurilemmoma
XI. Notochordal tumor chordoma
XII. Other tumors 1. 2.
1. solitary bone cyst 2. aneurysmal bone cyst 3. juxtacortical bone cyst (intraosseal ganglion) 4. metaphyseal fibrous defect (non-ossifying bone fibroma) 5. eosinophilic granuloma 6. fibrous dysplasia 7. "myositis ossificans "
giant cell tumor (intermediate) malignant giant cell tumor
IV. Bone marrow (hematopoietic) tumors 1. 2.
plasma cell myeloma(solitary, multiple) malignant lymphoma (reticulum cell sarcoma)
IVIA Ewing's sarcoma I primitive neuroectodermal tumor
8.
Ewing's sarcoma family 9.
V. Vascular tumors A) benign hemangioma B) malignant angiosarcoma
VI. Fibrohistiocytic tumors A) benign benign fibrous histiocytoma B) malignant malignant fibrous histiocytoma
VII. Connective tissue (fibrogenic) tumors A) benign desmoplastic fibroma B) malignant fibrosarcoma
VIII. Smooth muscle tumors A) benign leiomyoma B) malignant leiomyosarcoma
adamantinoma metastatic tumors
Tumor-like bone lesions
Ill. Giant cell tumor of bone (osteoclastoma) 1. 2.
193
IX. Lipogenic tumors
A) benign
osteochondroma chondroma (enchondroma, periosteal chondroma) 3. chondroblastoma 4. chondromyxoid fibroma
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2 1.2.2. 1 . Benign bone forming tu mors �
Osteoid osteoma
This is a benign, osteoblastic lesion, with a characteristic small size (ordinarily less than 1 cm), and reactive sclerosis surrounding the central radiolucent "nidus" (Fig. 2 1 . 10. a, b). The thickened hard cortex contains the nidus as a soft redd ish-gray a rea.
It is not uncommon, about 1 0 % of the be nign bone tumors. The tumorous origin of osteoid osteoma is not uniformly approved, some authors regard it as a reactive lesion. Symptoms. The disease starts usually at the age of 1 0-25 years. The special kind of pain is the leading symptom, it is severe, often occurs in the night and reacts well to salicy lates (salicylic test).
194
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2 1 . T u m o rs of t h e m u s c u l o s ke l eta l syst e m
About 1 0% are intracapsular, i n these cases a j oint-effusion, muscle spasm, cont racture are often present. Vertebral osteoid osteoma may result in an antalgic posture, and scoliosis. Femur and tibia are the most com mon sites of its occurrences. X-ray signs. The center of an osteoid osteoma is a radiolucent area 0,5-2 cm in di ameter ("nidus"), surrounded almost always by reactive sclerosis (Fig. 2 1 . 1 0. a, b). The nidus is often invisible due to the enhanced re- '
active sclerosis on regular films, in these cases a CT scan is advised. A bone scan shows a highly positive isotope uptake. Treatment. The aim is the total removal ofthe nidus. If this fails, the process may recur and the pain may persist. Surgical possibili ties: curettage or resection ofthe nidus. On the surface of the resected specimen the nidus is seen as a soft bluish spot. Very recently CT -guided drilling or radio-frequency thermo coagulation has been introduced. �
Osteoblastoma
This is a benign lesion, that is histologically equivalent to osteoid osteoma, but this is larger, exceeding 2 cm in the large tubular bones, and the sclerotic margin is usually missing or much less prominent.
Symptoms. This tumor occurs under the age of 20 years, it is much less common than osteoid osteoma. It is localized to the verte bral arches, long tubular bones, ribs, pelvis. The symptoms depend on the localization: the forms in the vertebral arches may cause neu rological complications, while osteoblasto mas in long tubular bones may be symptom less for a long time. X-ray signs. The X-ray image of osteo blastoma is not very distinctive. It generally causes extensive osteolytic destruction. The sclerotic margin is usually much less marked than in osteoid osteoma, occasionally it is mlssmg. Treatment. Osteoblastoma is one of the tumors that display active expansion. The choice of treatment should be curettage and filling with cancellous graft, or en-bloc exci sion in normal tissue. 2 1.2.2.2. Malignant bone forming
(osteogenic) tu mors Fig. 21.10.
a: A 16 year-old boy: osteoid osteoma in the metaphysis of the right femur b: a image: the intramedullary localization of the nidus and the surrounding sclerotic margin is clearly visible
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Osteosarcoma
This is a malignant tumor, distinguished by the direct bone mass and osteoid production
2 1 . T u m o r s of t h e m u sc u l o s ke l eta l system
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of the tumor cells. Osteosarcoma is the most common primary malignant bone tumor, comprising 25-30% of all bone sarcomas.
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It appears in many forms, its histological picture can be very variable. The conventional central osteosarcoma is most common, and is of greatest importance. In 60% of the cases, it appears in the first 2 de cades of life. It may involve any bone, but in about 50% of the cases it occurs around the knee, in the distal femoral or proximal tibial metaphysis (Fig. 2 1 . 1 1 .) . Symptoms. These are initially rather in significant: intermittent pain sets in, which later becomes persistent. The swelling ex pands rapidly, the skin is warmer, swollen subcutaneous veins appear. In an advanced stage pathologic fracture follows in app. 1 0% of cases. X-ray signs. Because of its fast progres sion, the osteosarcoma has usually broken through the cortex and infiltrated the soft-tissues even on the first X-ray film. Less frequently in forms producing a high volume of tumorous osteoid tissue, the involved bone is sclerotic, very radiodense (Fig. 2 1 . 1 2 . a, b). Fibroblastic, teleangiectatic or anaplastic osteosarcomas, that show hardly any bone production, induce osteolytic destruction. Most frequently mixed lytic -sclerotic forms occur. The border between the intact and in volved tissue is il-defined, with a "moth eaten" appearance. The growth plate, and the periosteum inhibit the expansion of the tumor somewhat. Similarly, the tumor destroys the joint cartilage and invades the joint only rarely. The pathological osteoblasts of the in, vasive tumor excrete osteoid in the soft tissues, this appearing as spiculum creation. eT and MR tests provide great help in as sessing the soft-tissue and intramedullary penetration (Fig. 2 1 . 1 2 . b, c). Pathology. The material of the tumor is determined by its osteoid content and by its calcification. Its texture may therefore vary from soft up to bone-hard. The cut surface often demonstrate visible bleedings, necrosis and cyst-like changes. At a distance of few cm from the tumor, inde pendent tumor islands, the so called "skip" metastases, are occasionally visible even macroscopically.
196
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2 1 . T u m o rs o f t h e m u s c u l o s ke l et a l syst e m
d e
a b
c
Fig. 2 1. 12. a: Sclerotic osteosarcoma invading the soft-tissue with spicula formation, located in the distal metaphysis of the femur b: a image showing the tumor invading to the joint in the level of patella c: MR image shows the soft-tissue extent of the osteosarcoma d: Histology of the osteoblastic osteosarcoma e: Limb-saving operation after resection of this osteosarcoma and implantation of a tumor prosthe sis (antero-posterior and lateral X-rays).
2 1 . T u m o rs of t h e m u s c u l o s ke l et a l system
The histological structure o f the tumor is variable, and often causes differential diag nostic difficulties. Osteoblastic (Fig. 2 1 . 1 2 . d), chondroblastic, fibroblastic, giant cell loaded, teleangiectatic, small cell and mixed forms are distinguished, depending on the dif ferentiation of the primitive connective tissue mesenchymal cells. Unfortunately, between osteosarcomas with a different tissue struc tures, the prognostic difference is negligible. Treatment. Central osteosarcoma is ra diotherapy-resistant, and accordingly chemo therapy and surgery are to be attempted. Cytostatic therapy is used both pre- and post operatively. Preoperative (neoadjuvant) che motherapy decreases the chances of the spread of tumor cell and metastasis formation during biopsy, the tumor shrinks, ("ripens"), and can be resected more easily.
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Thanks to the effective chemotherapy, the 5-year survival rate increased considerably, to 60-75 % (Fig. 2 1 .9.), and in more than half of the cases limb-saving procedures are success ful (Fig. 2 1 .8.). Some decades ago radical amputation pro cedures were almost always preferred, but nevertheless the 5 year survival rate did not exceed 20%. Today, surgical treatment, with wide removal of the tumor in the intact tissues is recommended, following preoperative che motherapy. Regarding metaphyseal location of the tumor the involved bone segment is re moved together with the j oint surface . Bone defects may be replaced by the patients own bone, or by bone from a bone bank, most often by tumor endoprostheses (Fig. 2 1 . 1 2. e).
Table 21.3. Other rare osteosarcomas
5 year survival rate
Diagnosis
Age Most frequent (years) Localization
X-ray appearance
Malignancy Therapy
Parosteal osteosarcoma
20-60
Femur, popliteal area, humerus, tibia
Intensely radiodense tumor on the bone surface
Grade 1
surgical
Periosteal osteosarcoma
15-30
Distal femur, tibia Blu rred, slightly (meta-diaphyseal) mineralized tumor on the bone surface
Grade 2
surgical 70% (chemotherapy ?)
Central osteosarcoma, low malignancy
15-45
Distal femur, proximal tibia
I ntramedullary lytic-sclerotic lesion, usually with a marked border
Grade 1
surgical
10-30 Superficial, highly malignant osteosarcoma
Distal femur, proximal tibia, humerus
Poorly mineralized superficial tumor, spicula formation
Grade 3
chemotherapy 55-65% surgical
Teleangiectatic osteosarcoma
10-25
Distal femur, proximal tibia metaphysis
Lytic destruction with blu rred border
Grade 3
Chemotherapy 65-75% surgical
Secondary osteosarcoma
30-60
Metaphysis of long tubu les bones
Lytic-sclerotic Grade 3 destruction with blurred border (+ basic disease Paget's disease, fibrous dysplasia, bone infarcts, postirradiation, etc.)
Chemotherapy 30-40% surgical
[-
-
90%
80-95%
198
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2 1 . T u m o rs of t h e m u sc u l o s ke l e t a l syste m
c
a: Parosteal osteosarcoma growing dorsally on the distal femoral metaphysis b: Operative specimen cut in half: it is clear, that the cortex is intact c: a images showing that the lump is situated on the outer surface of the bone, has not broken through the cortex.
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Parosteal osteosarcoma (j uxtacortical osteosarcoma) This is an osteosarcoma localized on the surface ofthe bone, from the parosteal tissues, with relatively better prognosis (Fig. 2 1 . 1 3 . a, c).
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Periosteal osteosarcoma This is located on the outer surface of the bone, in the periosteum, and usually in the diaphysis (Fig. 2 1 . 1 4.).
2 1 . T u m o rs of t h e m u s c u l o s ke l eta l syste m
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199
Fig. 21.14. Periosteal osteosarcoma on the surface of the tibia, the surgical specimen has been cut in half with a saw
Fig. 2 1. 15.
21.2.3. Cartilage-forming tumors 2 1 .2.3. 1. Benign cartilage-forming
tu mors �
Enchondroma
This is a benign tumor containing mature hyaline tissue. It is common, accounting for 20% of all benign bone tumors. It occurs in d h the 2n _4t decades, primarily involves the phalanges and the metacarpals of the hand.
Symptoms. It does not necessarily cause symptoms, and is often observed with un changed size for many years or expands only slowly. Stabbing pain may sometimes occur, or pathological fracture may be the first sign. The X-ray sign is specific, well delineated destruction is visible centrally in the meta diaphysis of the metacarpals, which inflates the bone without a periosteal reaction (Fig. 2 1 . 1 5.).
Lytic destruction in the base and mid-phalanx of the index finger inflating the bone -enchondroma
Pathology. Elevation of the cortical plate reveals a grey-white, debris-like material that fills up the medullary cavity. Micro scopically, this tumor is built up from hyaline cartilage, its structure is lobular. Occasionally it is difficult to differentiate the less malignant chondrosarcomas from the benign ones. One should not rely merely on the histology, but also consider the X-ray, the location and the clinical picture. It is essential to know that chondrosarcoma is very rare in the short tubu lar bones of the hand. It can present in a multi ple form as a systemic bone disease. In such cases, the risk of malignant transformation is higher, at 1 0%. Treatment. Excochleation and cavity fill ing the with autologous cancellous bone. In the active stage lesions may recur. About 0,5 - 1 % of solitary enchondromas produce secondary chondrosarcomas.
200 �
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2 1 . T u m o r s of t h e m u s c u l o s ke l eta l s y s t e m
osteochondroma may also be sources of com plaints . X-ray signs. Thin pedunculated or wide-based sessile forms are typical (Fig. 2 1 . 1 6, a, b). The peduncle bends in the direc tion of the diaphysis as a result ofthe dragging force of the neighboring muscles, resulting in the X-ray finding of a typical "hat-rack" shape. The size varies from a few cm up to 1 0 cm. Pathology. Macroscopically, the cortex of the bone continues in the shaft of the osteochondroma, and the end is widened as a butt, this area is covered by the hyaline cap. In the cartilage-bone border an ordinary enchondral ossification zone is visible. Treatment. The chiseling of osteo chondromas from their base ensures a perfect outcome. There may be a rare recurrence if parts of the cartilage or the covering connec tive tissue capsule are left behind. In less than I % of the cases, malignant transformation may occur in the substance of hyaline cartilage cap. The frequency is much
Osteochondroma (exostosis cartilaginea)
This is a bony lump covered with a hyaline cap, which p resents from the surface of the bone.
Osteochondroma is rather a hamartoma, than a real bone tumor, it is a cartilage bud that "migrates" out from the growth plate. It is quite common, giving 5 0-60% of all benign lumps. In 1 0% of the cases it is multiple and shows a familial accumulation, it is classified among the bone dysplasias (Chapter l 3 . 1 .7.). A solitary osteochondroma is usually di agnosed in the first decade, involving the metaphyses of long tubular bones. Distal fem oral, proximal tibial and proximal humeral metaphyses predominate. The osteochondr oma grows as long as the bones continue to grow. Rapid expansion with fast progression in adults is always a suspicious sign of malig nant transformation. Symptoms. At the beginning, there are al most no symptoms, the osteochondroma is discovered as an accidental finding. During its expansion it may compress the surrounding nerves and vessels, tendons snapping over the
a
b
Fig. 21.16. a: Ca uliflower-shaped osteochondroma in the proximal metaphysis of the fibula b: 3 dimension reconstruction a i mage of this osteochondroma
2 1 . Tu m o rs of t h e m u s c u l o s ke l eta l syst e m
�
20 1
higher, (ca. 5 - 1 0%) in multiple osteo chondromas, in multiple cartilaginous exo stosis. These patients must be thoroughly fol lowed up, and control X-rays are to be done. A sudden enlargement following bone matura tion is usually a sign of a malignant transfor mation, these should be excised immediately. �
Chondroblastoma
This is a rare benign tumor located in the epiphysis, next to the joint.
Symptoms. It appears chiefly on the tibia, femur and humerus of boys before the end of growth. The early symptom is swelling of the limb with a restricted range of motion of the neighboring j oint. Later a joint collection may follow with reactive synovitis. X-ray signs. These are very specific : oval or rounded lytic bone destruction in the epiph ysis, usually centrally with a sharp, occasion ally sclerotic border (Fig. 2 1 . 1 7. a, b). Treatment. The most common procedure is intralesional excochleation and autologous cancellous bone grafting. �
Chondromyxoid bone fibroma This rare benign tumor elicits hardly any symptoms . It presents in the second decade in the metaphyses of the long tubular bones. The X-ray image shows an eccentric lytic lesion with an uneven sclerotic margin, which may occasionally lead to bone protrusion. Treatment. Excochleation, autologous or homologous cancellous bone grafting. 21.2.3.2. Malignant cartilage-forming
tumors �
Fig. 21.17. a: Lateral X-ray of the knee: round lytic destructions (arrows) in the epiphysis - chondroblastoma. b: MR image: the extent of the chondroblastoma can be well estimated.
Chondrosarcoma
This is a malignant bone tumor containing hyaline cartilage cells in various stages of mat uration and a matrix.
There are many groups of chondro sarcomas, which differ in histological appear-
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202
2 1 . T u m o rs of t h e m u sc u l o s ke l eta l system
s u rvival among
1,0 1 - -1- - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - -� __q,�_5.. 0,9 0,8 0,7 0,6 0,5
\. J
5% 11 %
I I
11 %
11 1\
23 %
0,4 -':;'<;4\
0,3 0,2 0,10
0,1 0,0
°
___
12
24
36
grade 1 chond rosa rco ma
48
gra de 1 1 + 1 1 1 c h o n d rosarcoma
60
�»
m o u nth
(
Fig. 21.18.
�
12%
Survival among chondrosarcoma patients 1%
ance. It is important to be familiar with them, because biological behavior of a given tissue with the prognosis of the illness correlates closely (Fig. 2 1 . 1 8.). The chondrosarcoma is the second most common malignant bone tumor after the osteosarcoma. Males are affected slightly more often than females. Onset occurs pri marily in the 3_6th decade. It may be present in any bone, but it mainly appears on the prox imal part of the femur, pelvis, humerus, scap ula, less often the ribs and tibia, very rarely in the bones of the wrist and hind foot (Fig. 2 1 . 1 9.). Symptoms. Well differentiated chondro sarcomas grow slowly. Patients often observe limb swelling during many years. The less-differentiated forms break through the cortex early, and invade the soft tissue, and metastasize in the lung (mesen chymal and highly-malignant chondro sarcomas). X-ray signs. Intraosseal central chondro sarcoma is a process which swells the bone and causes lobular, lytic destruction (Fig.
L
10 %
5%
3%
Fig. 21.19. Incidence of primary chondrosarcoma
2 1 .20.). Calcification often develops in the substance of the tumor, giving a fine spotty, in other cases a massive radiodense X-ray im age.
2 1 . Tu m o rs of t h e m u s c u l o s ke l eta l syst e m
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203
a
Fig. 21.20. a: Chondrosarcoma destroying the proximal epi-metaphyis of the right humerus b: Surgical specimen of this chond rosarcoma - blu ish-gray transparent cartilage tumor, shining like mother of pearl c: Low malignancy chondrosarcoma: histological pic ture
The soft-tissue element of chondro sarcoma is generally lobular, the calcified spots clearly show the extent of the turnor (Fig. 2 1 .3. a, b). Peripheral chondrosarcomas do not initially involve the medullar cavity, only in a later stage. The well-differentiated chondrosarcomas are hypovascular, the less-differentiated ones are hypervascular. Pathology. Classical chondrosarcoma is a macroscopically grayish-white or blu ish-white, transparent, solid tumor, with a car tilaginous rigidity and a "mother-of-pearl" appearance (Fig. 2 1 .20. b, and c) Features of rare chondrosarcomas are listed in Table 2 1 .4. The treatment of chondrosarcomas. Low malignancy chondrosarcomas (Grade I .
•
chondrosarcoma, light cell chondrosarcoma, secondary chondrosarcomas) are radiother apy- and chemotherapy-resistant tumors. They grow characteristically slowly, meta stasize very rarely in the lung, the 5 -years sur vival is over 90%. Their treatment is chiefly surgical, depending on their location and ex tent. On radical or possibly wide resection, the lump should be removed with an intact mus cle envelope. Rarely, in an advanced stage, only amputation may be performed, however limb saving is very often possible. In cases of Grade H, IH chondrosarcomas, mesenchymal and dedifferentiated chondro sarcomas, the lump (which is usually quite ex tensive when it is first observed) can be re moved only by amputation to provide proper radicality. In these cases, surgical treatment is
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2 1 . T u m o rs of t h e m u s c u l o s ke l et a l system
Table 2 1.4. Chondrosarcomas Diagnosis
Age Most common (years) localization
5 year survival rate
X-ray appearance
Malignancy Therapy
pelvis, proximal femur, humerus, tibia, ribs
lytic-sclerotic tu mor, blu rred border, calcification
60% Grade 1, 40% Grade 2-3
Grade 1-2: surgical Grade 3: surgical, chemotherapy?
90%
Mesenchymal 15-40 chondrosarcoma
pelvis, rib, skull, femur
lytic-sclerotic tu mor, calcification
Grade 3
surgical chemotherapy?
30-50%
Dedifferentiated 35-75 chond rosarcoma
proximal femur, humerus, pelvis and scapula
sclerotic-lytic destruction, blurred border, calcification
Grade 3
surgical chemotherapy?
30%
Clear-cell 20-70 chondrosarcoma
femur, humerus epiphysis, pelvis, rib
lytic-sclerotic destruction, sharp border, calcification
Grade 1-2
surgical
90%
Juxtacortical 20-70 (periosteal) chondrosarcoma
femur, humerus, tibia
Hardly mineralized superficial bone tumor, spicu la, calcification
Grade 1-3
surgical chemotherapy?
Grade 1: 80-90% Grade 3: 30%
Secondary, 20-55 central chondrosarcoma
Short tubular bones, femur, humerus, tibia
lytic destruction, blurred border, calcification
Grade 1-2
surgical
75-95%
Secondary, 20-55 peripheral chondrosarcoma
metaphysis of long tubular bones, scapu la, pelvis
calcified tumor in Grade 1-2 soft-tissues + solitary / mu ltiplex exostosis
surgical
80-95%
Central 20-70 chond rosarcoma
be supplemented by irradiation of the regional lymph glands. Unfortunately, in spite of this the 5-years survival is quite low, between 30-35%.
2 1 .2.4. Bone marrow tumors •
Ewing-sarcoma / PNET
This is a malignant tumor, which presents at a n early age (5-25 years). Ewing's sarcoma and primitive neuroectodermal tumor (PNET) fam i ly include round cel l sarcomas, which exhibit various extent of neuroectodermal differenti ation. In 85% of the cases they show special chromosome translocations (t 1 1;22) and (q24;q 12).
30-40%
The Ewing' s sarcoma / PNET family are the 3rd most common primary bone sarcomas. The localized form involves a single bone, but not infrequently it is disseminated, can be de tected in other bones by needle biopsy and spreads to the lungs. This cases are to re garded as systemic. Symptoms. The swelling, pain, which are insignificant at the onset, progress rapidly, are soon accompanied by fever and leukocytosis. This condition can therefore be confused with acute osteomyelitis. The duration of the complaints is on aver age 3-6 months. The lump spreads rapidly from the intramedullary space to subperiostal area, and soon elevates the periosteum.
2 1 . T u m o rs of t h e m u s c u l o s k e l e t a l syst e m
X-ray signs. Ewing' s sarcoma / PNET ap pear in the diaphysis, dia- metaphysis of long tubular bones. They most often involve the fe mur, tibia and humerus, pelvic occurrence is also frequent. Originally there is an almost in visible fme spotty radiolucency on the diaphysis (Fig. 2 1 . 2 1 . a) . Later, the radio lucent areas link up, creating a larger region of spotty destruction. This is accompanied by a special lamellar, onion peel-shaped periosteal reaction. The Codman triangle is common at the edges. The extent of the tumor in either intra medullary or extraskeletal dimensions, is de tected by eT and MR tests. Pathology. The tumor comprises a gray ish-white, soft substance which exhibits bleedings, necroses. The substance of the in volved bone is necrotized, the cortex is soft ened or has disappeared. Microscopically, it contains uniform tumor cells lined up closely side by side with round or oval nucleus (Fig. 2 1 . 2 1 . b). Treatment. Ewing' s sarcoma is a radio and chemotherapy-sensitive tumor. The algo rithm of the treatment following the biopsy and diagnosis is as follows: 1 . preoperative chemotherapy, 2. irradiation of the tumor (to tal dose is 40-60 Gy), 3 . radical surgical inter vention and 4. postoperative chemotherapy. In axial locations (pelvis, spine, etc.) radio and chemotherapy without surgery is consid ered (survival is however less favourable). Some decades ago the 5 years survival rate of patients with Ewing-sarcoma was 5- 1 0% . With combination of radiotherapy, surgical and chemotherapy, this proportion increased to 50-70%. A new modality is the separation of stem cells after the removal of the tumor, then total body irradiation and return of the stem cells . Unfortunately, i n cases oflung metastases and local recurrences, the prognosis is still poor. �
Primary bone lymphoma Lymphoma rarely occurs in the primary form in bone, it may be seen at any age, but most frequently in middle age. Most often the pelvis, femur, tibia, humerus and ribs are in-
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205
a: Ewing's sarcoma in the proximal metaphysis of the fibula, typical onion-peel type periosteal reac tion b: Ewing's sarcoma: histological picture
volved. In contrast with Ewing' s sarcoma a long history, and the relatively good condition of the patient with no complaints are consid ered typical, even in cases oflarge tumors. Of ten a pathologic fracture is the first symptom. The X-ray picture reveals extensive bone de-
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2 1 . T u m o rs of t h e m u s c u l o s ke l eta l system
struction in the dia-metaphyses of long tubu lar bones, often without a periosteal reaction. Earlier, in cases of bone lymphomas the cho sen procedure was always amputation, but nowadays limb saving is coming into the fore ground, following local irradiation, surgical procedures and chemotherapy. Histologi cally, this bone lymphoma is similar to the lymphomas occurring in other lymphatic or gans. �
Multiple myeloma Multiple myeloma (multiple plasmocyt oma, myeloma multiplex) is generally classi fied among malignant hematological dis eases. This tumor elicits focal destructions in the bones, and the patients therefore often seek the help of an orthopedist, traumatologist with their first symptoms. The treatment of solitary forms and pathologic fractures of multiple forms requires surgical intervention. Symptoms. Theses usually present after the age of 40, often in the vertebrae, ribs, skull, pelvis and sternum. The illness often starts with a long period of no clinical symp toms, or vague back pain, which points to a spinal manifestation. In severe cases, the ver tebrae may be compressed, paralysis, par esthesia may be present. The immune system of the patients is usually impaired, banal in fections are frequent. The sedimentation rate is usually very high (over 1 00 mm / hour), se rum electrophoresis proves monogamma pathy and the bone marrow gained by sternal puncture contains a very large amount of pathologic plasma cells, confirming the diag nosis. The pathologic protein also appears in the urine (Bence Jones protein) . X-ray signs. Typical rounded, lytic bone destructions (as they were induced by a punch) are visible with sharp margin in the bones of skull, vertebrae, ribs, pelvis, Treatment. In solitary forms irradiation and resection-replacement of the bone seg ment involved is to be considered. In the most common multiple myeloma, the decisive role played by chemotherapy. The remission achieved by cytostatic therapy is usually tem porary, and the 5-years survival rate is low,
around 3 0-50%. In cases of impending or pathologic fractures, palliative osteosynthesis is carried out (intramedullary nail, plate, etc.) to ease the pain, and provide independence, chance of ambulation for the patient.
2 1 .2.5. Giant cell tumor of bone. (osteoclastoma, "brown tumor of bones") According to recent research this intermediate tumor evolves from the medullar stam-cells, osteoblast precursor cel ls. Mainly occurs in the relatively young in the 2 nd _4th decade. The epi-metaphyses of long tubular bones are usually involved, most often the distal femo ral, proximal tibial, proximal humeral, distal ra dial epi(meta)physis, and less often occurs in the sacrum, pelvis.
Fig. 21.22. Aggressive giant cell bone tumor, spreading from the tibia to the fibula (a-p and lateral X-ray).
2 1 . T u m o rs of t h e m u s c u l o s ke l et a l s y s t e m
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207
Brownish-red giant cell bone tumor in the proximal epi-metaphysis of the tibia surg ical specimen
Symptoms. The tumor usually grows rap idly, the patient reports vague local pain, swelling. X-ray signs. Giant cell bone tumors cause exclusively lytic bone destruction. The lesion is usually rounded or oval, eccentric, and the cortex is thinned, with no periosteal reaction (Fig. 2 1 .22.). It is more or less separated from the surrounding intact bone tissue, but has no sclerotic margin. It frequently invades the subchondral region of the joint surfaces, con sequently the surfaces may collapse in re sponse of load bearing. Pathology. Macroscopically, reddish brown, soft, occasionally fragile tumor tissue with haemorrhages is seen in the bone defect (Fig. 2 1 .23.) . Microscopically, the lump con tains a connective tissue-type fusiform cell population, and mononuclear cells, osteo clast-type giant cells. Treatment. The giant cell bone tumor is a classical semimalignant tumor: it grows ag gressively, breaks through the cortex, invades the soft-tissues, joints, and often recurs after inadequately radical treatment. On the other hand it very rarely metastasizes to the lung. Recent classifications put giant cell bone tu-
mor as intermediate in biological behavior, between the benign and malignant tumors . This essentially means that the histological image and biological behavior are not closely connected. The statistical data indicate, that 80% of these tumors is benign, 1 0% are pri marily malignant, 5% become malignant sec ondarily, and 3-5% give lung metastases (mostly through their recurrences) together with the repeatedly benign histological pic ture. Treatment is surgical, excochleation ofthe defect, which is then filled up with bone chips or bone cement. After this intervention, recur rence is not rare. Resection can also be per formed with intact tissue margin (if it is not necessary to sacrifice the joint surface).
2 1.2.6. Other rare bone tumors Adamantinoma and chordoma (the latter appears in the line of the embryonic dorsal chord) are classified here. Certain typical soft-tissue tumors also occur in bones, but very rarely. Apart from a few exceptions (adamantinoma, chordoma) clinical symp-
208
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2 1 . T u m o rs of t h e m u sc u l o s ke l eta l s y s t e m
I Table 21.S.1 Other rare bone tumors Diagnosis
Benign
Hemangioma
Desmoplastic bone fibroma Lipoma Malignant
Rbrosarcoma (primary, secondary) Malignant fibrous histiocytoma
Age Most common (years) localization
20-60
20-40 20-60
30-50 20-50
Liposarcoma
30-50
Adamantinoma
5-30
Chordoma
20-70
X-ray appearance
Malignancy Therapy
flat bones, skull, spine
oval lytic destruction, "tiger stripes" spiculum formation
latent, active
observation, su rgical
long tubular bones, metaphysis
lytic lesion, cortical destruction
active, aggressive
surgical
tubular bones, cuboid bones
lytic lesion, +/-sclerotic border
latent, active
surgical
femur, tibia metaphysis
lytic-sclerotic destruction, blurred border
femur, humerus, tibia
lytic-sclerotic destructive, blurred border
femur, pelvis, tibia, humerus
blu rred border lytic destruction
tibia (diaphysis)
Cystous, " " honeycomb-like , sharp bordered lesion
sacrum, cervical spine, skull
blurred border, lytic destruction, calcification
toms and X-ray appearance are not typical, they are pinpointed as an accidental finding following an injury and the final diagnosis is established only on the basis of the histology. Table 2 1 -5. summarizes their relevant fea tures.
2 1 .3. Bone metastases Oncology management is becoming an in creasingly more serious task in o rthopedic and trauma surgery. The main reason is that the increased average lifetime increases the
Grade 11-111 Grade 11-111
su rgical
surgical chemotherapy
5 year survival rate
30-70% 60-80%
Grade 11-111
surgical
40-60%
Grade I
su rgical
90%
Grade 1-11
su rgical
60-90%
number of diseases, and in the number of patients, who have solitary or mUltiple sec ondaries after the treatment of their primary tumor, but who can survive on cytostatic treatment for years.
The significance of the treatment of bone metastases is indicated by the fact that they are 80- 1 00 times more common than primary bone turnors . In about 20-40% of the cases, the secondary is discovered first, and the pri mary can not be found even with a compre hensive search.
2 1 . T u m o rs of t h e m u s c u l o s ke l eta l system
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209
After the lung and liver (primary filters), malignant tumors metastasize most often to the skeleton. Various tumors have very differ ent "bone affinities" as concerns their
metastases . 65-85% of bone secondaries are given by breast, lung, kidney and prostate cancer. The bones, most frequently involved in decreasing sequence : lumbar, dorsal, cervi cal spine, ribs, proximal femur, skull, pelvis, sternum and humerus. Tumors are very rare in the short tubular bones of the hand and foot (Fig. 2 1 .24.). Symptoms. The first symptom is usually a deep, intermittent pain that is independent of the movement and barely moderates in re sponse to painkillers . Such pain often presents weeks or months before the X-ray changes are detected. The involved area is tender, perhaps swollen, warmer. The case history and labora tory tests must be thoroughly evaluated. In 1 0-30% of the cases the first episode is a pathologic fracture. In the background there may be a kidney or lung cancer, giving mostly lytic metastases, or less frequently breast (Fig. 2 1 .25.), or thyroid cancer. Osteoplastic metastases of prostate cancer rarely break and have good propensity to heal (Fig. 2 1 .26.). Pathologic fractures occur most often in the vertebrae and load-bearing long tubular bones
Fig. 21.24.
metastasis with pathologic fracture i n the prox imal metaphysis of the humerus
Common localizations of bone metastases
2 10
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2 1 . T u m o r s of t h e m u s c u l o s ke l eta l system
Fig. 2 1.26. Osteoplastic metastases of prostate cancer in the pelvis
(the femur, tibia), less frequently in the hu merus. X-ray signs. In suspected cases, e.g. when there is local bone pain after history of turnor, an X-ray is taken of the area in question and eT, MR (occasionally PET) scans are added if necessary. A bone scan is also extremely important to decide if the process is single or multiple. The sensitivity exceeds that of the X-rays, the pathologic area shows an in creased isotope uptake months before the X-ray changes are detected.
In spine, in the opposite of inflammation processes the tumor involves a single verte bral body (Fig. 2 1 .27.), invading the inter vertebral space only in later stage. In the long tubular bones, the lesion may be central, though it is more often eccentric, involving the cortex, with a round or oval ap pearance. A periosteal reaction is rare. At the time of discovery metastases are usually mul tiple, only in 1 5-20% are solitary. There is im minent danger of fracture if the level of cortex destruction exceeds 50% or 2,5 cm. The laboratory parameters are less specific and may remain between the normal limits, especially for small solitary metastases. Differential diagnosis. Primary malig nant bone tumors usually present at a younger age, there is no history of tumor, and the tu mor is usually solitary and accompanied by a periosteal reaction. Similar X-ray changes are detected in cases of primary bone lymphomas, giant cell tumors, aneurysmal bone cysts. Treatment. Earlier a somewhat passive approach was favored. Today considering the possibilities provided by modem radio- and chemotherapy, and the marked development that have occurred in re constructive surgery (minimally invasive interventions, tumor endoprostheses) surgical treatment has came to the forefront. Aims of the surgical treatment: � � � � �
Fig. 21.27. Vertebral destruction caused by metastases of kid ney cancer (3D er i mage)
to prevent the imminent fracture radical removal of the lesion if the conditions are suitable in cases of fracture, reconstruction of motion, alleviation of the pain to ensure independence, mobility for the patients to ensure proper care and quality of life
In cases with a vertebral involvement (Fig. 2 1 .27.) in order to solve the neurological symptoms following a compression fracture, decompression is carried out with plate stabi lization.
2 1 . T u m o r s of t h e m u s c u l o s ke l et a l syste m A)
S)
Primary tumor is known and oncologically managed
I
Solitary
�
Primary tumor su rvery
+ ..
..
extra skeletal fracture chest, abdomen a. US surgical
.. Needle biopsy
Assessment of prognostIC factors
Surgery
Radical
Imminent fracture
no
..
.. Biopsy
+ Staging skeletal-extraskeletal i m minent fracture
1
Pall iative surgery
.. or chemotherapy
..
Stag ing extraskeletal
Assessment of general condition of patient
c hemoth erapyI radiothera py Palliative
Biopsy
Staging metastasis (solitary, multiple, imminent fracture)
..
1 1 a
..
Biopsy
Assessment of general condition of pt.
a -....." 11".at.ve --------surgic I
..
+
+
yes
..
U nsuccessful
+
.. I n bone (a. MR)
+
Successful
O ncological management of primary tumor
Staging skeletal-extra ske letal
Staging
I
+
M u ltiple/mu ltio rganic
..
211
Metastasis of unknown origin
X-ray, bone scan, PET-a. M R
+
�
.
. .. Planning surglcall
non- surglca I treatment
.
Fig. 21.28. Treatment algorithm in cases of suspected metastasis (before pathologic fracture)
In long tubular bones, the following proce dures are employed depending on the local ization of the fracture : intramedullary nailing, osteosynthesis fixed with a plate and curet tage of the defect, or insertion of a tumor endoprosthesis with cement fixation. Intra medullary nailing is advantageous, for it is stable weight-bearing, and even if the tumor progresses, loosening of the implant is not likely (Fig. 3.4. a, b). Surgical intervention can be supplemented by palliative radiotherapy and chemotherapy, to achieve pain relief and temporary remis sion. Amputation is very rarely performed for palliative purposes, only in cases of ulcerated tumors and unbearable pain. Surgery is not done if the expected sur vival is less than \ -3 months, if the general
state ofthe patient does not permit; or in cases of multiple metastases or multiorgan meta stases, if no imminent fracture is to be pre vented. In the latter cases palliative chemo therapy, radioisotope treatment (in certain thyroid, prostate, breast cancers), and hor mone therapy (in prostate cancers and in metastases of estrogen positive breast cancer) are preferred, and bisphosphonates are pre scribed in lytic and mixed-type bone meta stases. Prognosis. The life expectancy is good if the primary tumor is discovered and can be controlled oncologically, if the metastasis is solitary, appears years after the onset of the primary tumor, grows slowly, does not invade to the soft-tissues and no pathologic fracture
2 12
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2 1 . T u m o rs of t h e m u s c u l o s k e l eta l syst e m
has occurred. I n these cases (unfortunately only 1 0-20% of all patients) curative-type radical tumor excision is warranted, with limb savmg. The most sensitive prognostic factor is the origin ofthe primary tumor. In cases of breast, prostate, thyroid and kidney cancers, the ex pected survival time is much longer than in cases of lung cancers or bone metastases of melanoma. The life expectancy is poor when the pri mary tumor is unknown or unsuitable for sur gery, or when the primary is discovered at the same time as the metastases, if the metastases are multiorganic or multiple. In these cases surgery is palliative and the aim is to restore
the function and ambulatory capability with the least possible intervention, and in terminal case to ensure the chances of painless care.
2 1.4. Tumor-like bone processes The significance of tumor-like bone pro cesses (Table 2 1 .6.) is, that their X-ray ap pearance may mimic that of malignant bone tumors, which gives rise to differential diag nostic problems, since they are much more common than the real tumors . It is necessary to be aware of the possibility of misdiagnosis and of an unnecessarily radical intervention.
Table 2 1.6.' Tumor-fike bone lesions Diagnosis
Most common Age (years) localization
Symptoms
X-ray appearance
Treatment
Juvenile bone cyst
5-20
usually accidental or a pathologic fracture
pure lytic lesion with a sharp border thin cortex, swollen bone
depot-steroid, eXCDchleation, sclerotization
central or eccentric growth, blu rred margin, deformed bone
excochleation + cancellous graft, sclerotization
Aneurysmal 5-30 bone cyst
metaphysis of the humerus, femur, tibia
meta-epiphysis pain, swelling, of the long pathologic tubular bones fracture
Non ossifying bone fibroma
5-25
long tubular bones metaphysis
symptom less or subcortical, sclerotic margin, mild pain or a ..lobular" cystic thinning pathologic fracture
Fibrous dysplasia
10-40
metadiaphysis of long tubular bones, pelvis
mild pain, curvature of bones, pathologic fracture
5-25 Eosinophyle granuloma (solitaer) Myositis ossificans
5-30
in case of imminent fracture, excochleation, cancellous bone graft, or observation
blurred margin, often multiple, in cases of imminent "opaque glass" lytic defect fracture excochleation + cancellous graft
vertebra, long pain, tubular bones, eosinophilia Metadiaphysis of flat bones
a sharp border, a lytic bone defect, lamellar periosteal reaction
excochleation + a cancellous graft
long tubular bones, over metadiaphysis in the muscles
initially: oval lump showing ossification at its periphery, centrally radiolucency; later: a lump with uniform bone density, divided from the bone by a radiolucent layer
su rgical excision in mature stage, if it is a source of complaints (considerable contracture, pain)
initially: fever leukocytosis, pain, later: a solid lump, contractures
I ,
2 1 . T u m o rs of t h e m u s c u l o s ke l eta l syste m
�
2 13
Juvenile bone cyst (with pathologic fractu re) in hu merus proximal metaphysis .
Fig. 21.29. Non ossifying bone fibroma: multiple appearance in femur and tibia.
Regarding their progress tumor-like bone processes may be latent, like non-ossifying fibroma (Fig. 2 1 .29.), and juvenile bone cysts are ordinarily discovered accidentally, or at tention is drawn to them by a pathologic frac ture, due to the weakened cortex. In these a cases plaster cast is applied, which sometimes is enough for a bony consolidation of the le sion. If this fails, the non-ossifying fibroma is curetted and the cavity is filled with bone chips. A juvenile bone cyst (Fig. 2 1 .30.) is treated with injection of the crystalloid steroid to induce the bony restructuring, or similarly cancellous bone plombage is performed. Ifthe non-ossifying fibroma is small, observation is satisfactory with regular X-ray checks . Local aggressive growth is observed for aneurysmal bone cyst (Fig. 2 1 .3 1 . a, b),
which are regarded as intraosseal circulatory disturbances. This growth causes the bone segment to expand, weakening the cortex, causing risk of a pathological fracture. In about 25% of the cases, this lesion appears in association with other primary bone tumors (giant cell tumor, osteoblastoma, chondro blastoma, osteosarcoma), causing problems in differential diagnosis. Eosinophile granuloma appears mainly in the meta-diaphysis of long tubular bones as a central lytic lesion showing a thick sclerotic periosteal reaction. It may be solitary or mul tiple. In the former case, curettage and spongiosa plasty are performed. The latter case has a significantly worse, sometimes fa tal prognosis, chemotherapy can be at tempted. Fibrous dysplasia is classified to bone systemic diseases (Chapter l 3 .), it can mimic a bone tumor on the X-ray picture. It is very inconsistent as regards its extent in the bone : it varies from round small metaphyseal lesion to a "shepherd' s crook" shaped femur,
2 14
�
2 1 . T u m o rs of t h e m u s c u l o s ke l et a l system a
b
a. Multilobular aneurysmal bone cyst from the sciatic bone, impressing the medial side of the acetabulum b. Aneurysmal bone cyst destroying the d istal end of the radius (cut surface of the surgical specimen) a
Fig. 2 1.32.
b
Myosit iSOSsificans i n the form of oval calcification in the anteromedial side of the femur (ap (a) and lateral (b) X-rays)
2 1 . T u m o r s of t h e m u s c u l o s ke l eta l syste m
when the entire long tubular bone i s affected. In cases of latent, non-progressing forms a regular check-up is sufficient, in progressive forms, curettage, cancellous bone plombage is indicated. A good outcome was recently re ported following bisphosphonate treatment, which increases the strength of the surround ing bone. Myositis ossificans rarely occurs in a pro gressive form in infants; this progressive form develops rapidly and has a lethal outcome. This condition occurs in the overwhelming majority in young adults, metaplastic ossifica tion develops in the soft-tissues, muscles after a trauma or even without any injury (Fig. 2 1 .32.). It is often difficult to differentiate from malignant soft-tissue tumors or from bone tumors involving the soft-tissues. If the diagnosis is certain and the lesion is symptomless, removal need not be urged, as recurrence is common. In case of symptoms or a maj or loss of function excision is advised with a wide margin.
21.5. Soft-tissue tumors Classification the soft-tissue tumors . Ac cording to the WHO definition published in 2002, soft-tissue tumors are extra skeletal lumps originating from non-epithelial tissues, except those that develop from the reticulo endothelial system, the glia tissue and special connective tissues of the viscera and certain organs. Soft-tissue tumors are discussed by the WHO definition on a histogenetic basis. The basis of the classification is the tissue from which the lump develops and the tissue it produces. Soft-tissue tumors are relatively
Table 21.7. Classification of soft-tissue tu mors (simplified version) I.
Fat tissue tumors A) Benign lipoma B) Malignant liposarcoma
�
2. Connective tissue (fibroblastic, myofibroblastic) tumors A) Benign fibroma fasciitis nod ularis myositis ossificans B) Intermed iate (locally aggressive) palmar and plantar fibromatosis desmoid-type fibromatoses q Malignant Adult fibrosarcoma Myxofibrosarcoma
3. Fibrohistiocytic tumors A) Benign fibrous histiocytoma giant cel l tumor of tendon sheaths B) Malignant malignant fibrous histiocytoma 4. Smooth muscle tumors A) Benign leiomyoma B) Malignant leiomyosarcoma s.
Pericyte (perivascular) tumors glomus tumor
6. Striated muscle tumors A) Benign rhabdomyoma B) Malignant rhabdomyosarcoma 7.
Vascular tumors A) Benign hemangioma B) intermediate hemangioendothelioma q Malignant Hemangioendothelioma (epitheloid) Angiosarcoma
8. Soft-tissue tumors originating from
cartilage and bone tissue A) Benign soft-tissue chondroma B) Malignant extraskeletal chondroand osteosarcoma
215
216
�
2 1 . T u m o rs o f t h e m u s c u l o s ke l eta l system
9. Tumors of questionable or unknown etiology A) Benign intramuscular myxoma B) Intermediate ossificans fibromyxoid tumor q Malignant synovial sarcoma alveolar soft-tissue sarcoma epitheloid sarcoma clear- cell sarcoma of tendons and tendon sheaths extraskeletal Ewing/PNET malignant mesenchymoma
rare, but many types are known; the following table (Table 2 1 .7.) lists only the more impor tant ones. Malignant soft-tissue tumors comprise about 1 -3 % of all malignant lumps. 60-65% of these tumors are localized in the limbs, two-thirds of them in the lower limbs. De pending on the type of the tumor, local recur rence is common, 40-60%, distant secondar ies are detected in 40-60% of the cases, mainly in the lung. The symptomatology of the tumor is very indistinct, their detection depends mainly on
their location. Tumors lying near the superfi cial fascia are detected when they measure 0.5-2 cm, if they are located deep in the mus cles or next to the bone, detection is possible, when they exceed 5 cm in size. Unlike to the bone tumors, for the stage classification of soft-tissue tumors the TNM system is used (Table 2 1 .8.), which is useful both clinically and prognostically. This takes into account the size of the pri mary tumor (Tx: the size ofthe primary tumor can not be assessed, TO : no primary tumor can be detected, T l : the primary tumor measures less than 5 cm, T2 : the diameter of primary tu mor is more than 5 cm, T l a: it has a superfi cial localization, T l b: it has a deep localiza tion); the histological malignancy of the tu mor (G I : low malignant, G2 : highly malig nant tumors); to the involvement of regional lymph glands (Nx: can not be judged, NO: no metastasis in regional lymph glands, N I : me tastasis in regional lymph glands); and the dis tant metastases (MO: no distant metastases, M I : metastases are present). Benign soft-tissue tumors can also be di vided into latent, active and aggressive types (Table 2 1 .9.). Latent and active tumors may be marginally or pericapsularly excised, as concerns aggressive forms (e.g. desmoid) ex cision with a wide margin must be achieved.
Table 2 1.8. TNM classification of soft-tissue tumors Stage
Histological malignancy
Tumor size
Lymph gland involvement
Distant metastases
lA stage
Gl Gl
Tla Tlb
NO, NX NO, NX
MO MO
I B stage
Gl Gl
T2a T2b
NO, NX NO, Nx
MO MO
IIA stage
11 B stage
G2 G2
Tla Tlb
NO, NX NO, NX
MO MO
G2
T2a
NO, NX
MO
III stage
G2
T2b
NO, NX
MO
IV stage
Gl/G2 Gl/G2
Tl/T2 Tl/T2
Nl NO/N l
MO Ml
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2 1 . T u m o rs of t h e m u s c u l o s ke l eta l syste m
217
Table 21.9. Recurrence rate of some common benign soft-tissue tu mors Diagnosis
Stage Latent
Fibroma
+
Palmar and plantar fibromatosis (Dupuytren) Extraabdominal fibromatosis Rbrous histiocytoma Lipoma Angiolipoma Intramuscular lipoma
+ +
Hemangioma cavernosum Hemangioma arteriovenosum Hemangioma venosum
+
Giant cell tumor of tendon sheaths (synovioma)
Neurilemmoma
Aggressive Moderate
+
+
+
+
+
+
+
Regarding the malignant soft-tissue tu mors that commonly occur in the limbs, the time of onset, common localization and the histological malignancy is presented in Table 21.10. Therapy. The therapy depends o n the stage, the localization of the tumor, on the age of patient, etc. It is mandatory for these rare tumors to be managed in centers with a multidisciplinary approach. Optimal treat ment must be decided in consultations involv ing an oncologist surgeon, a pathologist, a ra diotherapist, a chemotherapist and a radiolo gist. It is optimal, if the orthopedist surgeon is specialized in oncological surgery, since tu mor resection should often be followed by re construction, and tendon and muscle trans plantation. Vessel replacements, and use of a vascularized musculocutaneous flap to cover
+ +
+ +
+
+
+
+
+
+
+
+
+
+
+
+
+
+
High
+
+
+
Neurofibroma, solitary
Myxoma, intramuscu lar
Active
+
+
Morton neuroma
Myositis ossificans
Recurrence rate
+
+
+
+ +
the defect is often needed, which may necessi tate to involve vascular- and plastic surgeons. Specimens for histology are obtained causing the least possible trauma, aspiration cytology, percutaneous core biopsy may also be appro priate if an experienced pathologist and spe cial test methods (cytogenetics, etc.) are avail able. In the surgical planning, the optimal inter vention selected should minimize the risk of local recurrence, besides ensuring the best quality of life. If the tumor involves many muscle groups, it must be excised with a deep margin extending in the direction of intact tis sues, some muscles may be cut in half so as to ensure some function. The surgical procedure is radical, if the entire muscle is cut out from its origin to its attachment (myectomy), when the tumor has not broken through the fascia. In case the tumor has reached the periosteum,
218
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2 1 . T u m o r s o f t h e m u s c u l o s ke l et a l system
Table 21.10. Localization and histological malignancy of some malignant soft-tissue tu mors Age decade
Localization
Diagnosis
Fibrosarcoma
3-6
Limb, trunk, head, neck
Myxo-fibrosarcoma
3-6
Lower-upper limb
I nfantile fibrosarcoma
1-2
Limb d istal
Malignant fibrous histiocytoma
4-6
Liposarcoma, well-differentiated
Malignant
I.
+
11.
+
+
+
Lower -, upper limb, trunk
+
+
3-5
Thigh, mediastinum
+
Liposarcoma, myxoid
2-4
Limb, thigh
+
Liposarcoma, pleiomorphic
4-6
Lower, upper limb, trunk
Leiomyosarcoma
4-6
Retroperitoneum, limb
Rhabdomyosarcoma, embryonal
1-2
Head-neck, genital
Rhabdomyosarcoma, alveolar 2-4
Limb, perineal, para nasal
Angiosarcoma
3-8
Lower limb, upper arm, neck
Synovial sarcoma
2-3
Lower limb, foot, popliteal, hand
Extra-skeletal myxoid chondrosarcoma
4-7
Thigh, upper arm
Extra-skeletal osteosarcoma
5-7
Thigh, bottom, shoulder girdle
Alveolar soft-tissue sarcoma
2-4
Lower limb, thigh extensor side
Epithelioid sarcoma
2-4
Forearm, hand, fingers
Clear-cell sarcoma
2-4
Limb, foot, ankle
+
+
+
+
+
+
+ +
+
+ + + + + + +
Grade
Ill.
+ + +
+ + + + + + + + +
it must likewise be excised, since it represents the "capsule" of the tumor. In case the tumor has eroded the bone cortex, or invaded the medullary cavity, the involved bone segment must be excised with the lump. Conditions of limb-saving operations are similar those listed in connection with bone tumors (Chapter 2 l . 1).
nancy. Following marginal resection of deeply seated tumors with high malignancy, neoadjuvant / adjuvant chemotherapy and / or radiotherapy is recommended. If the histology of the tumor was unclear at the time of the first surgery, and the remaining tissues were con taminated by tumor, wide excision of the tu mor bed is suggested in a second session.
Adj uvant therapy. If a soft-tissue sar coma is superficial, it measures under 5 cm, and the wide margin excision succeeded with 2-3 cm-thick intact muscle envelope, the sur gical intervention is sufficient. Chemotherapy is not recommended for tumors of low malig-
2 1.5. 1. Some soft-tissue tumors of orthopedic significance �
Connective tissue tumors Connective tissue tumors comprise the largest and most colorful group of soft-tissue
2 1 . T u m o r s of t h e m u s c u l o s ke l et a l system
tumors. The most common i s the fibroma durum, which measures 0,5 - 1 cm and is usu ally located in the subcutis, sometimes pro truding from the skin as a pedunculated lump. Fasciitis nodularis is a reactive fibroblastic tissue mass rather than a tumor. It grows fast, reaches its final size, not ex ceeding 3 cm within a few weeks . It generally occurs in young adults . It may be found in any region in the body, in half of the cases occurs in the upper limb, mainly on the volar side of the forearm, in the superficial fascia, subcutis, rarely in muscles. Its rapid growth and histological picture may give rise to the suspi cion of malignancy, and particularly diagno sis of fibrosarcoma. Surgically, excision is the solution, but if the procedure is intralesional, it may recur. Superficial fibromatoses are plantar and palmar fibromatoses. The former is also called Dupuytren contracture (see chapter 27, Fig. 27.12.). They are sometimes both pres ent. Total excision is advised. One of the deep musculo-aponeurotic fibromatoses is the extra-abdominal desmoid (Fig. 2 1 .33.). Early recognition is essential to differentiate it from fibrosarcoma. The etiol ogy is unknown, the predisposing factors in-
Fig. 21.33. Aggressive desmoid infiltrating the left gluteus mus cle, and also the femur (MRI, frontal reconstruction)
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219
clude trauma, rapid abdominal wall contrac tion after delivery, a surgical procedure and scarring, hormonal factors may also play a role. It may occur anywhere, it is observed (in the sequence of decreasing frequency) : in the shoulder girdle, upper arm, thigh, pelvis and forearm. Treatment is surgical, wide-margin excision is advised, without sacrificing the vessels and nerves. Local recurrence is fre quent. Fibrosarcoma is a malignant soft-tissue tu mor that originates in the connective tissue (Fig. 2 1 .34.). It may occur at any age, usually
Fig. 2 1.34. Fibrosarcoma in the middle third of the left leg, showing pathologic vessel structure
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220
2 1 . T u m o r s of t h e m u s c u l o s ke l eta l system
h i n the 3 _ S t decade. Usually develops in deeper regions, next to tendons, aponeuroses, infiltrating the deep muscles. It is better de fined than the desmoid, it has a pseudo cap sule. Histologically in a typical case the bundles of connective tissue cells and colla gen fibers show "fishbone" pattern. There is a broad scale from the differentiated forms to anaplastic tumors, the latter display abnormal mitoses, and tumorous giant cells are also present. The recommended treatment in well differentiated forms is wide resection, in less the differentiated forms it is radical resection, perhaps supplemented with radiotherapy. a
�
Fibrohistiocytic tumors Malignant fibro-myxosarcoma is the most
b
common form of malignant fibrohistiocytic tumors
(Fig. 2 1 .35. a, b).
This lump is pre
sumed to originate from primitive mesen chymal cells, which later undergo partially histiocytic, partially fibroblast differentiation, and are capable of phagocytosis and for colla gen production. It occurs in the limbs, less of ten in the retroperitoneum, in the middle aged and in elderly. It usually grows deep between the mus cles, often causing cortical erosion on the neighboring bone. It expands rapidly, with typical clinical symptoms of fever, leuko cytosis, neutrophilia or eosinophilia. Its may reach S-20 cm in size. The cut surface is gray to yellow, depending on the fat content, inter
a: MK I mage of the middle third of the thigh: malig nant fibro-myxosarcoma in the substance of the rectus femoris muscle b: The same tumor after radical surgery (myectomy)
spersed with necrotic bleeding spots . Accord ingly, despite radical surgery, local recur rence rate is 40-S0%. The S years survival rate is low, at around 3 0-40%. This tumor most of ten metastasizes to the lung, or to the regional lymph glands . The therapy is mainly surgical : radical excision, often amputation. Irradiation of the regional lymph glands or adjuvant cytostatic therapy is also employed. �
Fat tissue tumors The most frequent mesenchymal tumor is
the lipoma, which contains mature fat tissue and has a capsule. It may be solitary or multi ple,
usually superficially in the subcutis,
rarely deep between the muscles. It measures
between 0 , S -20 cm. It is well separated from the surrounding tissues. Its cut surface is ho mogenous, butter-yellow, with a fatty shine. Lipomas often contain other tissues: angio lipoma,
pleiomorphic
lipoma,
angiomyo
lipoma, fusiform cell lipoma can be differenti ated. The lipoma is usually encapsulated. Sur gery comprises marginal resection. Liposarcomas are malignant tumors of the fat tissue the
(Fig. 2 1 .36. a, b).
They are the
second most common soft-tissue tumors after malignant fibrous histiocytoma. They occur at the 4-6th decade, in deep sites adj acent to the intermuscular fascias, aponeuroses. This tu mor most commonly occur in the thigh,
2 1 . T u m o rs of t h e m u s c u l o s k e l e t a l syste m
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221
popliteal region, and retroperitoneum. It usu ally grows slowly, and because of its deep lo cation its discovery is late . In consequence of the variable histological picture, many subdi visions are differentiated, the broad scale ranging from less malignant forms to forms of high malignancy. For the latter, the 5 -year survival rate is around 20%, whereas adequate treatment of the well differentiated forms leads to cure in 80- 8 5 % of the cases. Surgical procedure for the less malignant forms is wide excision, the highly malignant forms require amputation. The effectivity of chemo- and ra diotherapy is questionable. �
Tumors of muscle tissue The malignant turnor of the smooth mus
cle tissue is the leiomyosarcoma. It is a relatively rare lump, occurs in
a
adults, sometimes in the skin in the form of
b
small nodules, more often in the deeper re gions . Excision with a wide margin is recom mended. It rarely metastasizes, but local re currence is frequent, prognosis is fairly good. The malignant turnor of the skeletal mus cle tissue is the rhabdomyosarcoma. This is the most common soft-tissue sar coma in children. Many forms are known : e . g . embryonic, botryoid, alveolar, pleiomorphic rhabdomyosarcoma. The embryonic form is already present at birth, while the pleio h morphic type takes place in the 3 _ 5 t decade, chiefly in the muscles of the limbs. The lump is usually 2- 1 0 cm in diameter, and has no capsule, the cut surface shows bleedings, necroses. Earlier the prognosis was very poor because of the local recurrences and lung metastases . Recently combined radio-chemo therapy and surgical treatment increased the survival
in
a
great
extent,
the
5
years
symptomless survival rate now reaching 80%. �
a: H uge I I nrIC::Irrn nn::l b: Cut surface of the specimen: liposarcoma inter spersed with fatty, shining and necrotized spots
Vascular tissue tumors The hemangioma is a common localized
form of benign vascular turnors . Capillary hemangioma occurs predomi nantly in the skin and subcutis, in the form of
Cavernous hemangioma, which may be larger, occurs in deep tissues, often in the muscles.
It may exhibit local destructive
growth, which may cause complaints . Exci
typical purple - reddish nodules, it may be
sion or percutaneous sclerotization is neces
present at birth.
sary.
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222
2 1 . T u m o rs of t h e m u s c u l o s ke l et a l system
Arteriovenous and venous hemangiomas are rare, occurring in deeper tissues. The com pression of the surrounding tissues, nerves may induce severe pain. Intramuscular hemangioma may infiltrate the deeper muscles, the limb is swollen, the skin is hot. It is often large, excision with a wide margin may be difficult. If tumor tissue is left behind, local recurrence may arise. The angiosarcoma is a malignant vascular tumor.
Its
histological
structure
is
de
differentiated, it is usually impossible to de cide
if
the
pathologic
growth
contains
pericytes or endothelial cells. It takes place
a
primarily in the head, neck, less often in the
b
limb muscles. It requires a radical surgical procedure. The prognosis of the highly malig nant form is poor. �
Synovial tumors In orthopedic practice it is perhaps most
important to be familiar with tumors situated in the synovial lining embedding the tendon sheaths, bursae, j oint capsules. Many synonyms are known for the benign varieties: benign synovioma, nodular teno synovitis, nodular pigmented villous syno vitis, giant cell tumor of tendon sheaths, etc . Their histological pictures are similar or fully identical. Their etiology is unclear, some ex pert consider them to be as inflammatory, while others regards them as genuine tumors .
� may occur at any age, most often in the
The
3_5 1
decade. They appear primarily in the
small joints ofthe hand. The patients often no tice increasing swellings on their fingers . The lump measures
0,5-5 cm.
It is covered by cap
Fig. 21.37. a.: Synovial sarcoma on the left upper arm next to the elbow b.: MR image of the same lump in sagittal plane
sule. It has a lobular structure, it is ocher yel low on the cut surface. The lump should be excised with its capsule, if part of the tumor is left behind, local recurrence is frequent. A malignant variety of the synovial tumors is the synovial sarcoma
(Fig. 2 1 .37. a, b). It usually occurs in the 2_3rd decade, in the lower
cut surface exhibits necrosis, bleeding and calcification. Microscopically, it can be di vided into two maj or groups: biphasic and monophasic forms, which can mutually trans
limb, the foot, the popliteal area, the thigh,
form into the other form during the recur
rarely the hand. It is closely connected to the
rences. The growth rate of the lump is vari
tendons, tendon sheaths, bursae and joint cap
able, the patients occasionally notice the lump
sules. Macroscopically the tumor contains
for months, in other cases for years, but it may
cm cysts, and has a pseudocapsule. Its
then suddenly start to grow and attain consid-
5-20
2 1 . T u m o r s of t h e m u s c u l o s ke l eta l syste m
erable size. This tumor is classified a highly malignant sarcoma, it metastasizes early, of ten to the lung and to surrounding lymph glands. The histological picture and the prog nosis are not closely related, the 5 years sur vival rate is rather low, at 25-60%. Radical surgical intervention has recently been sup plemented with local radiotherapy. �
Peripheral nerve tumors Traumatic neuroma is a tumor-like lesion, occurring after the transsection of peripheral nerves. In these cases a small painful nodule may develop at the end of the proximal stump. Similarly the Morton neuroma is not a real tumor, but rather a fibrotizing process of the distal nerve. It is situated most often in the sole between the 3 rd and 4th , less often be tween the 2nd and 3 rd metatarsal heads . On ex posure, the division of the plantar nerve is fusiform, thickened. It causes painful attacks, which can be provoked by direct pressure. The involved nerve segment should be ex cised. Neurilemmoma (benign schwannoma) is a real benign tumor of the peripheral nerves. It often develops along the peripheral nerves, grows slowly. Pain, neurological signs are ob served only for maj or lumps. Marginal exci sion is advised.
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223
Solitary neurofibroma is also benign. It is more common, than its multiple form, the Recklinghausen 's neurofibromatosis. The lat ter often produces a malignant transforma tion. Solitary neurofibroma may occur at any location in the body, usually causing no symptoms. Malignant schwannoma is a malignant tu mor of the peripheral nerves. About half of the cases develops from Recklinghausen 's neuro fibromatosis. It is most common in the 2_5 th decade. It is often located in deep tissues, along maj or nerve trunks, closely adhering to them. The clinical symptoms are : referring pain, paresthesia, weakness. The cut surface of the tumor is grayish-white, with scattered bleedings and necroses. The histological pic ture is very variable. The prognosis of the sol itary forms is rather good, but in cases based on neurofibromatosis the 5 -years survival rate is less than 3 0-40% . The treatment is radical excision or rather amputation. Cartilage and bone producing malignant tumors also occur in soft-tissues, chondro and osteosarcomas of soft-tissues. The diag nosis is suspected by X-ray findings and con firmed by histological tests. The histological picture is the same as that for the forms occur ring in the bones. The treatment is radical sur gery.
22.
Zo lta n Cse r n a t o n y
Reflex dystrophy (Sudeck's syndrome)
Referred to in the literature as Sudeck's syn drome, Sudeck's reflex dystrophy, algo dystrophy, reflex sympathetic dystrophy, acute post-traumatic osteoporosis or acute focal bone atrophy, this is a regional vasomo tor and trophic syndrome that generally de velops in the limbs, causing painfu l and se vere functional disorders, affecting the skin, the subcutaneous tissue, and the articular and periarticu lar structures.
Etiopathology. The pathological basis of this disease is dystrophy, which generally en sues in areas rich in nerve terminals . The essence of the pathogenesis o f Sudeck 's syndrome is the vicious circle of the metabolic and trophic processes, associated with circulatory failure induced by pain, and with severe locomotor function disorders (Fig. 22. 1 .). Besides the classic example of trauma, there may be numerous factors provoking Sudeck ' s syndrome, though around 25% of the cases no cause can be demonstrated (Ta ble 1 .) .
Table 22.1. Possible causes of Sudeck's syndrome - Pregnancy and post partum condition (hip) - Bone fracture, frostbite, bu rns, or some other trauma (in 8.8% of all i nju ries and in 27% a mo ng women older than 60 years) - Suppuration of bones, joints or soft parts - Thrombosis - Certain rheumatic conditions (psoriatic arthropathy, dermatopolymyositis, lupus erythematosus, etc.) - Distortion of the cervical and lumbar spine. - Central or peripheral d iseases of the nervous system (cerebral thrombosis, epilepsy, radiculitis, herpes zoster, polyneuropathy, hemiplegia, cerebral tu mor, i njuries of the brain or the spinal cord) - Cardiopulmonary conditions (myocardial i nfarct, pulmonary synd rome, etc.) - Endocrine and metabolic diseases - Lyme disease - Psychic disorders Iatrogenic provoking factors:
Improper immobilization Impro perly adapted physiotherapy Vegetative hypersensitiveness and certain psychic disorders may be predisposing factors.
Evo king factor Pain Focal bone atrophy
Artriolar spasm Ti ssue hypoxia
Ti ssue acidosis
activity Pain
Fig. 22.1. Vicious circle of Sudeck's dystrophy
Clinical picture. The course of the clini cal picture is usually divided into three stages, defined by the concomitant symptoms. Radio logical alterations generally appear some weeks later, may initially be absent. �
Stage 1 This lasts from some weeks to three months. The first sign of the illness is gener ally the pain, which can practically converge
226
�
2 2 . Reflex d y st r o p hy ( S u d eck's sy n d r o m e )
with that, caused b y the inducing condition, but is disproportionately great. Local inflammatory symptoms (edema tous swelling and hyperemia) are observed; the skin is warm and dry, and often becomes hairy. The patient has no fever. Pressure, heat, movements, and emotional stress can increase the pain. �
Stage 2 In some cases, the disease begins directly with stage 2. It lasts for three to six months, during which vasoconstriction predominates, possibly leading to dystrophic symptoms. The skin is cold, moist and livid. Contractures can develop. In the X-ray picture (Fig. 22.2.) it can be observed that the focal atrophy of the bones at the certain area turns into diffuse os teoporosis at the end of the stage, the condi tion of the cortical bone remaining unaltered. �
Stage 3 This final stage, may last for two or more years from the onset. Severe pain may also be experienced in this stage. More or less perma nent tissue changes can occur: the skin of the
limb is shiny, silk-like, thin and pale, and the subcutaneous tissues are atrophic. Clinically, the limitation of articular mo tion is the outstanding feature. Radiolo gically, the symptoms of osteoporosis are ac companied by fibrous bones, due to the thick ening and parallel positioning of the trabeculae. Treatment. Apart from the specific ther apy of the basic disease, the treatment of the developed algodystrophy depends on the stage. �
Stage 1 The goal is to ease the pain and the symp toms of inflammation. The limb must be put in a resting position, or if necessary in a plas ter cast in a neutral position, immobilized, cooled, and treated with antiphlogistics. Non-steroid anti-inflammatory drugs can be administered as additional medication, to gether with calcitonin, and alpha- and beta-blocking drugs. In more severe cases, steroid can be given systemically for 1 -2 weeks.
Fig. 22.2. Anteroposterior picture of the hands: typical focal striped atrophy in the short tubular bones of the carpus and the hand.
2 2 . Reflex dyst ro p hy ( S u d e c k's s y n d r o m e)
A further possibility is sympathetic block ade, with paravertebral or ganglion stellatum lidocaine infiltration, depending on the local ization. Physiotherapy is significant, but it should not induce pain. If the lower limbs are affected, partial weight-bearing with crutches for some weeks, and before getting up, the putting-on of flexi ble stockings is suggested. Underwater exercises and alternating bathing, edema draining massage can also be applied. �
Stage 2 There is only a need for a resting position if the patient still suffers from pain. Physiotherapy is still necessary by the same principles. Careful warming of the cold limb is suggested.
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Stage 3 In this stage the developed contractures are relieved conservatively, with active and passive orthoses. If there is no other way, then an operation is required. Prevention of the disease is much easier than its treatment. Since Sudeck 's syndrome can evolve in the event of short-term immobilization, strict control of the patient and careful physiother apy are necessary. The therapy is mostly effective in stage I , and at the beginning of the stage 2 , but from the end of stage 2, there is a need for long-term rehabilitation, because of the prob lems caused by permanent deformities.
23.
Arpad Bel lyei
Aseptic bone necrosis
23. 1. Childhood bone necrosis ijuvenile osteochondrosis, juvenile osteochondritis, juvenile osteochondronecrosis) Bone necrosis generally refers to disorders which start in child hood. These disorders, which have a similar histological appearance, affect the epi- metaphysis, the apophysis, maybe even the accessory bones d u ring growth. These illnesses can occur at any site where enchondral ossification is taking p lace.
Currently more than 50 types of aseptic bone necrosis are known and depending on the localization and grade they can cause dif ferent complaints; however, regardless of the localization, all of these disorders are histologically the same. The more common ones are presented in Fig. 23- 1 . Initially bone and marrow cells necrotize in the affected area. This is followed by a revascularization process. The well-vascularized connective tissue gradually invade between the bone trabeculae, it dissolves the necrotized bone trabeculae and new bone formation com mences. The process is a slow one, and the de velopment of a normal structure takes months or even years . Resorption of the dead bone segments and the creation of new bone are usually a parallel processes (creeping substi tution) . Due to the breakdown of the balance between decaying and building, the bone in question may undergo distortion if exposed to even a normal load. Because of the histological alterations the term ' aseptic osteochondrosis ' appears opti mal since the cartilaginous area also takes part later in the process. The word "osteo chondritis" used in the English medical litera-
Fig. 23.1. Frequent localizations of osteochondrosis 1: Sternal epiphysis of clavicu le 2: Proximal epiphysis of humerus 3: Vertebra plana (Calve) 4: M. Scheuermann's disease 5: Os lunatum 6: Femoral head (M. Perthes) 7: Patella 8: Apex patellae 9: Tuberositas tibiae (Schlatter-Osgood sy.) 10. Apophysitis calcanei 11: Os nacicu lare (K6hler I.) 12: II-IV. metatarsal head (K6hler 11.)
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ture does not correspond with the pathological events since no inflammation occurs. The ex pression osteochondrosis not only implies the absence of any basic inflammatory event, but also indicates the occurrence of alterations leading to degenerative disorders . The attribute "juvenile" expresses the fact that this type of disease appears in childhood, during development. The forms occurring in adulthood, such as os lunatum malacia and id iopathic necrosis of the hip and knee joints, are completely different in appearance and course from juvenile osteochondrosis. In the case of Blount 's disease (tibia vara epi physarea), there a failure of ossification of the tibia in the epiphysis as well as in the metaphysis. Etiology. The cause of aseptic bone necro sis is not clear. According to Weis and Lindemann theories claiming that all such ill nesses have a mutual, definite cause are un founded. The many views can be condensed into 3 hypotheses. �
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The mechanical theory. Many believe that the primary cause is a mechanical dynamic overload, involving excessive pressure or traction, while others claim that the explanation lies within mutations caused by repeated trauma and micro trauma. Even though the partial role of mechanical factors cannot be ruled out, in themselves they do not provide an adequate answer. The vascular theory postulates that the illness is caused by a primary vein-supply disorder (thromboembolic mechanism) or external compression of the nutritional supply artery. The cause of the local circulatory disorder is still unknown. It is important to emphasize that the artery supply in the region of the epiphysis and the apophysis remains unstable till they connect to the meta-diaphyseal artery system during the closing of the developing cartilage. Constitutional theory. Many suspect that hereditary ossification disorders, or a
hormone dysfunction are to blame. The most accepted theory is that of Idelberger 's, who discusses genetically determined hypoplasia and hypo vascularization of the skeletal system. It is important to note that it is not osteo chondrosis itself that is inherited, but only the disposition. There are also different opinions regarding the categorization of sterile necrosis. Accord ing to Harbin and Zollinger consider that forms emerging during the first decade com prise primary necrosis, which should be dis tinguished from the secondary forms develop ing during the second decade. Hirsch 's defini tion includes local and generalized forms. Gofflabels deformities created by pressure (e.g. Perthes 's, Scheuermann 's, Kijhler 's 1. diseases) as real, while deformities generated by traction force as unreal necrosis (cal caneus, patella, trochanter, and sciatic bone apophysis). This is not a perfect distinction and ignores the fact that the maj ority of osteochondroses only have a mild effect and do not always lead to any consequences. Frequency. In childhood and adolescence sterile necrosis and associated pain syn dromes occur quite frequently, and have been increasingly observed in the orthopedic prac tice recently. This may be explained by the following factors : �
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Acceleration: the continuous increase in body height. This is accompanied by an increase in the power of the arms, the forces at the origin and attachment of the various muscles and ligaments therefore also increasing. The spreading of mass sport, which leads to higher pressure on the developing and cartilaginous ossification centers. The demands imposed by professional sport and the lowering of the age limits in competitive sport. Sport frequently becomes an existential factor even in childhood, and year round pain syndromes
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occurring at this stage cause serious problems for child, parent and doctor alike. The other extreme is when osteo chondritis and related pain syndromes with no medical consequences lead to the child being excused permanently from all forms of physical education (PE), even though this could be avoided by the use of brief activities coupled with non-steroid medication. Osteochondritis in childhood and adol escence is responsible for most of the cases of polypragmasia in orthopedic practice. Polypragmasia not only leads to the unfounded exemption from PE class, but also the incorrect mental treatment of chronic pain syndromes, with the parents tending to go from one doctor to another.
The most frequent forms of osteo chondrosis observed today are the following : - distal apex patellae syndrome, - proximal patella (main bone nucleus) syndrome, - Schlatter-Osgood 's disease, - Osteochondritis spina iliaca anteriorinferior, - Osteochondritis spina iliaca posteriorsuperior, - Scheuermann ' s disease, - Osteochondritis dissecans gen., - Perthes 's disease, - Kohler ' I-I!. disease, - Calcaneal apophysitis (Schinz 's disease) . X-ray signs. In these diseases the X-ray appearance can be divided into 3 stages: 1. The sclerotic stage. The affected bone nucleus becomes sclerotic, smaller than normal and shrunk, leading to the decaying of the ossifying center. 2. The fragmentation stage. Soft foci appear within the sclerotic bone nucleus and the shape of the fragmentation of the bone nucleus is visible. The radiolucent zones show the invasion of vascular shoots and the destruction of dead bone trabeculae.
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3. The remodeling stage. In cases of com plete recovery the contou r of the original bone nucleus is restored into the original trabecular structure. I n some cases however due to the mechanical forces it deforms, flattens, and certain bone segments sometimes separate.
The treatment. The treatment of sterile necrosis depends on localization, extent and age. The English and German orthopedic schools remain divided on this issue. The for mer considers sterile necrosis to be a self-healing illness, as an unstable boundary state, and provisional or symptomatic treat ment is recommended only when the condi tion actually causes an ailment. In contrast the German orthopedic school define it rather as an illness which should be treated as soon as it has been diagnosed.
23.2. Adu lt aseptic bone necrosis Adult bone necrosis resem bles childhood bone necrosis o n ly in the histological picture. The a ppearance and progression differ com pletely since the ability of decayed bone seg ments to repair comes to a close d u ring adu lthood.
The joints most affected are the hip, knee and shoulder. The most common types are femoral head necrosis in the hip joint, medial femur condyles necrosis in the knee j oint (Ahlbeck 's disease) and necrosis of the head of the humerus in the shoulders. A significant difference in localization is that necrosis does not occur in areas where the apophysis was situated earlier, but within the area of the epiphysis that bears all the static pressure, ir respective of the earlier epiphysis boundaries. Clinical symptoms. In adults the disease is considered irreversible since it destroys not only the relevant bone tissue, but also the en tire hyaline cartilage surrounding it. Another vital difference is in the symptoms : in some
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cases there are n o spectacular clinical symp toms or ailment zero or silent stage), then, as the disease progresses, the permanent syno vitis causes significant pain and immobility, which is initially temporary and gradually be comes permanent. Aggressive synovitis is caused by a mas sive surge of necrotic micro fragments into the j oint which in turn causes reactive synovitis. Early X-ray pictures usually do not indicate anything, whereas an MR test can already show the size of the deformity in stage zero. Diagnosing in stage zero is vital as in most cases this is a bilateral process including vari ous phases. After the diagnosis of active and painful necrosis on one side, it is imperative to check the opposite side, as minimal preven tion treatment is possible only if the diagnosis is made in stage zero.
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Chemical intoxication, causing enzymatic disorders in the liver, Long-lasting steroid treatment for systemic diseases (rheumatoid arthritis, non-differentiated collagenosis, organ transplants). Osteoporosis due to chronic steroid treatment causes micro fractures, then necrosis at the site most affected by mechanical strain (the femoral head). Necrosis following radiation treatment (radiotherapy) : A vascular disorder due to lasting radiotherapy and damage to the cells responsible for the bone metabolism could be the cause. Caisson disease this afflicts people work ing at constant high pressure, especially if they leave this too quickly, or in a defective sluice chamber). The viscosity of the blood changes and micro embolization disrupts the blood supply.
Differential diagnosis. Adult transitory osteoporosis (osteopenia) has very similar clinical symptoms; but its clinical progression and appearance are different. It occurs most frequently in the hip joint, causing pain on motion and synovitis. The native radiological picture is negative, while the MR images show a sterile necrosis-like state on the femo ral head. The only difference is that the pro cess affects not only the head, but the whole of the metaphysis. With this type of deformity, recovery is usually spontaneous and requires only observation. Etiology and types. Sterile necrosis oc curring during adulthood generally does not indicate any cause or source of the illness in which case it is classified as idiopathic. However, various systemic diseases and causes are known, including iatrogenic disor ders. �
Alcoholism or long-term alcohol abuse features in the history data in most cases of adulthood femoral head necrosis, there is a latent or manifest liver enzyme disorder that causes metabolic changes and local osteoporosis.
Fractured neck of femur treated earlier with a cannulated screw. One year later, necrosis of the head is visible, with a wedge-shaped crack.
23. A s e p t i c bone n e c r o s i s �
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Post-traumatic bone necrosis, which i s usually suffered b y older patients after a fracture of the femur neck. The reasons for this lie in the sensitive arteries supplying the femoral head (Fig. 23.2.). Iatrogenic, bone necrosis develops after surgery. A typical example is the meta tarsal head necrosis following osteotomies for hallux valgus.
Besides the epiphysis, bone necrosis may occur in the meta-diaphysis during adulthood. Its causes are generally unknown. It appears in X-ray films as irregular, dot-like calci fications (Fig. 23.3.), and sometimes difficult to differentiate from real bone tumors, like enchondroma, central chondroma of long tu bular bones, chondrosarcoma.
Fig. 23.3. An old lady, with extensive bone necrosis in the meta- and diaphysis of both femur
24.
Ta mas I l les, J6zsef La katos, Pet e r Pa l Va rga
Diseases of the spine
24. 1. Biomechanics, functional anatomy, functional units of the spine Bony transformation of the cartilaginous spine field starts in embryonic weeks 9 and 1 0 with the appearance of the ossifying centers. Each vertebra has three primary ossifying centers : one in the vertebral body, and one on each side of the vertebral arch (Fig. 24. 1 .) . The primary ossifying centers of the arches develop at the origin of the transverse pro cesses, and the ossification then starts in every direction (forward to the posterolateral part of the vertebral body, backward to the lamina of the arch and to the spinous process, laterally to the transverse processes, and up and down in the directions of the superior and inferior articular processes. The ossification of the vertebral bodies and arches is completed at the age of 3 years
for the cervical segment, and at the age of 6 years for the lumbar part. Further development of the vertebral bod ies originates from the secondary ossifying center, which appears at the edge of the upper and lower endplates at the age of 8 years . The spine must be stable and firm, and at the same time flexible and elastic. It can fulfil this double, antagonistic requirement thanks to its columnar functional units. The basic functional unit of the spine is the elementary moving segment (Fig. 24.2.). The basic moving segment contains two neighbouring vertebrae and the connective structures, naturally together with the muscles
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Fig. 24.2.
Fig. 24. 1. Primary ossifying centers of vertebrae
Elementary movement segment and its compo nents. a: Ligamentum longitudinale anterius, b: inter vertebral disc, c: ligamentum longitudinale posterius, d: facet joints, e: ligamentum interspinosum
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and ligaments attached t o the corresponding transverse and spinous processes. As concerns the function of the basic mov ing segment, there is a special connection be tween the two vertebrae. Because of their shape, the vertebrae can be regarded as levers, with the support point located in the facet j oints. The force sustained by the vertebral body is mitigated directly and passively by the intervertebral disc (Fig. 24.3.). Through the levers created by the vertebrae, the tension of the interspinous ligaments and muscles both indirectly and actively decreases forces that affect the basic moving segment. In this way, the compression forces in each basic moving segment are eliminated both passively and ac tively. The elasticity of the spine, ensured by the basic moving segments, is essential to elimi nate the rough quivering produced during walking. The physiologic curves of the spine also contribute to this elimination.
Fig. 24.3. Tasks of the elementary movement segment. The vertebrae are the levers; they act both directly and passively to decrease the forces acting on the verte bral body; the tension of the interspinous ligaments acts both indirectly and actively.
In the sagittal plane, the normal spine ex hibits a series of curves : cervical lordosis, tho racic kyphosis, lumbar lordosis and sacral kyphosis, with average angles of 300(± 1 0°), 40° (± 1 0°), 45°(± 5°), respectively for tho racic kyphosis, lumbar lordosis and lordosis for the lumbosacral transition. The sagittal curves of the spine are conse quences of the erect posture and are typical of the human race. The normal spine is a curve in a single plane which is balanced at rest and can be described by mathematic formulas. One extremely important function of the spine is to ensure the stability and balance of the body. The movements ofthe spine are the overall resultants of the movements of the basic seg ments (see Chapter 2).
24.2. Examination of the spine The successful treatment of diseases af fecting the spine often demands long and per sistent cooperation between patient and doc tor. The diseases of the spine are experienced as neck, back and lumbar pain, the restriction of movements, and deformities. Since the spine is the central axis of the body, its diseases may be accompanied by pe ripheral, neurological and vegetative symp toms . Consequently, in cases of spinal com plaints, an assessment of the spine itself is not sufficient: the body as a whole, and the indi vidual limbs and other organs should also be surveyed. The first step in the assessment is a de tailed history. The accent must be put on the pain that accompanies the disease of the spine (since the deformities and movement restric tions are easier to specify). Pain: The time of onset of spinal pain is not always the same as the time of onset of the disease. In degenerative diseases the pain is some times manifested only years after the disease has started. Chronic pain may create reflexes, which result in pain that persists even after the disease has ceased; such reflexes frequently
2 4. D i se a s e s of t h e s p i n e
are observed in chronic spinal pain syn dromes. During the evaluation of the com plaints, attention must be paid to the age, the constitution, the occupation and factors influ encing the psychological state. The type of the pain is important. Pain re lated to movement is usually a sign of a de generative or benign disease. Pain at rest or during the night may relate to a tumor or an in flammatory origin. The location and irradiation of the pain can be significant features in spinal diseases. Pa tients often complain of peripheral pain with out being aware of its significance, and do not associate it with a possible spinal disease. The doctor however must be fully familiar with the segmental structure of the spine and the spinal cord.
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tilt o f the shoulders can b e described i n terms of the difference from the horizontal, while the horizontal position of the pelvis can be de fined by the direction of the iliac spines and the cristae. If the pelvis is tilted, the next step is measurement of the sizes of the lower limbs, to discover functional scoliosis result ing from lower limb discrepancy. Part of the physical assessment comprises the determination of the type and localization of any spinal deformity. It is particularly es sential to describe the sagittal curves exactly; this it may be of substantial help in demon strating the etiology of a possible lateral curve. Determination of a compensated or de compensated state of the upper body is a fur ther important element in the assessment (Fig. 24.4.). If a plumb bob hung from the spinous process of vertebra C7 is situated in the mid-
24.2. 1. Assessment of the cervical spine Deformities of the cervical spine are rela tively rare. An antalgic posture, torticollis and scoliosis can be detected on inspection. During the assessment of pain a search is made for compression-induced pain. Pressure from above on the head, and tapping on the spinous processes may generate pain in the segments involved. The paravertebral muscu lature may also be painful and tender. Re ferred pain and occasionally radicular signs may be detected on the upper limb. In cervicobrachialgia, the upper edge of the tra pezium muscles, the lateral area of the shoul der joint and the lateral epicondyle of the hu merus can be especially tender. A detailed neurological assessment of the upper limb and a precise examination of the fine functions of the hand may be necessary. Assessment of the dorsal spine. As con cerns the dorsal spine, the early recognition of deformities is most important. The first step in the physical assessment is to inspect the standing patient from every di rection. Any asymmetry revealed by such an inspection must be accurately recorded. The
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L Fig. 24.4. Assessment of the decompensation of the spine with a plumb bob.
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Fig. 24.5. Assessment of rib hump.
dIe of the buttocks, the spine is compensated; if there is any deviation, it is decompensated. The most important part of the physical as sessment is the hyperflexion test, the aim of which is to determine whether a deformity is
Fig. 24.6.
accompanied by structural changes. A charac teristic feature of structural changes is the presence of vertebral rotation. This can be assessed most easily with the patient in a standing position, with a 90° hip flexion and the upper trunk bent forward (Fig 24.5.). Hyperflexion resulting in a rib hump on the convex side proves vertebral rotation and hence structural changes in the spine. Spinal mobility is an important character istic as concerns structural changes in the spine. During bilateral flexion of the spine (bending test), the ability of the spine to achieve correction can be examined, while ax ial distraction can characterize the rigidity or mobility of the spine (Fig. 24.6). If a deformity with tenderness of the spinous processes and paravertebral muscles has been discovered, a search must be made for possible pain, even though deformities do not typically cause pain. If the shoulders are pushed down, the pa tient may experience pain in the dorsal seg ment. If a patient standing on tiptoe transfers
Bending test. With the patient bending lateral ly, the mobility of the curves can be assessed.
2 4. D i s e a s e s of t h e s p i n e
the body weight to heel, that may also elicit pam. The cause of pain elicited above and be tween the spinous processes may be chronic muscle tension, or contact with the spinous processes (Baastrup 's phenomenon, inter spinous arthrosis). Pain may be provoked in the paramedian line and in the facet j oints by lateral move ment of the spinous processes. Tenderness is frequent even more laterally due to the lasting, reversible tone increase of the paravertebral muscles. Myogelosis involves well-outlined, mo bile nodules in the musculature. The most common localizations are the shoulder, the neck and the lumbosacral area. Its cause is muscle fiber hyaline degeneration and conse quent connective tissue deposition. Movement of the dorsal spine is difficult to judge separately. When the trunk is bent forward, the distance between vertebrae C7 and D2 increases by 4-6 cm in normal cases (Giinz 's sign). It is important toe assess of the mobility of the dorsal kyphosis, which is per formed as follows : the forward-bending pa tient is requested to hollow the back and at the same time to lift the upper limbs and head. Normally, the dorsal kyphosis is then elimi nated. Assessment of the dorsal spine includes a consideration of the costovertebral joints, which are best described by the expansion of the chest. The expansion between full expira tion and inspiration, measured in the line of the nipples, is normally 7-8 cm or more. The general assessment comprises the de termination of various features : •
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The time and circumstances of the onset of the given deformity, and of any other deformities in the family. The general appearance of the patient (weight, height, and facial asymmetry), the condition of the joints, muscles and skin, and hyperpigmented spots on the skin (cafe au lait) . The neurological status. A reflex assessment (patella and Achilles reflexes),
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and particularly a n assessment o f the abdominal or cremaster reflexes, may disclose causes of neurological origin (syringomyelia or Chiari malformation),
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Fig. 24.7. Risser's sign. Depending on the development of the iliac crest apophyses, stages 0-5 are differentiated (al. This X-ray shows stage 3 (bl
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where the first appearance o f the spinal cord manifestations may be a scoliosis considered to be idiopathic. The time of the first menstruation. The current stage of sexual development and the characteristics of secondary gender features, according to Tanner 's signs. In girls, this means observation of the development of the breasts, and the appearance of pubic and axillary hair; in boys, besides the facial, pubic and axillary hair, it involves determination of the size of the testicles. Establishment of the phase of ossification, related to Risser 's sign (Fig. 24.7.), is based on observation of the ossifying centers of the iliac crest, and the presence or absence of the ossifying centers of the vertebrae. The ossifying center of the iliac crest appears in the anterior-superior iliac spine, progresses to the posterior, and then gradually unites with the iliac crest.
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Risser ' s stages 1 -4 denotes the proportions (in percentage) of the full length of the crest, while in Risser 's stage 5 the ossifying center is already fused to the iliac crest. The biological age revealed by Tanner 's signs and the bone age indicate the general level of development of the patient, accurate estimation of which is essential for the prognosis and for the decision concerning the treatment plan (see later).
24.2.2. Assessment of the lumbar spine The lumbar spine is the most mobile seg ment of the spine, but its range of motion is difficult to describe in degrees. The extent of forward bending of the lumbar spine is deter mined by Schober 's sign (Fig. 24.8.), via measurement of the distance between two
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Fig. 24.8. Schober's sign. On measurement of the forward flexion of the lumbar and dorsal spine, the marks drawn on the skin move apart.
2 4 . D i s e a s e s of t h e s p i n e
marks on the skin. The caudal skin mark is drawn at the S I level, with the cranial mark 1 0 cm cranially, and the expanding distance is measured. During forward bending, this dis tance usually increases to 1 5 cm; in the vent of restricted movement it is less. Lateral bending is 20°, while rotation is 1 0° . It should be noted that the extent of rotation movement is great est at C l -C2 (80°) and gradually decreases caudally. Below L l , the level of rotation be tween two vertebrae is only 1 -2°. This is quite important for an understanding of degenera tive problems, where the first clinical sign is an increase in the rotation, i.e. rotational insta bility. Passive displacement can be provoked in the sacroiliac joint; if this elicits pain, it points to a disturbance of the j oint. Bilateral com pression of the iliac crest may provoke pain in these cases (Fig. 24.9.). If there is sacroiliac joint involvement, hyperextension of the hips in the prone position may also be painful (sa croiliac Mennel ' s sign) . The lumbosacral Mennel ' s sign is utilized to examine the lumbar facet joints . The lum bar spine of the prone patient is fixed on the examining couch, the lower limbs and thighs are then lifted, and the pelvis is pulled dor sally. In a positive case, the patient feels pain in the lumbar spine, which is a sign of irrita tion in one of the lumbar segments, most probably in the lumbosacral segment. During the assessment of local pain, ten derness and pain are searched for in median
Fig. 24.9. Compression assessment of the sacroiliac joint.
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and paramedian lines and along the sciatic nerve (Valleix ' s points). Referring pain and radicular signs. One of the most important radicular signs is Lascgue ' s sign (Fig. 24.5 1 ) . With the patient lying supine, the lower limb is gradually flexed at the hip, with the knee extended. Normally, such flexion is possible to an extent of 90%. If the patient feels sharp pain in the flexion internal 3 0-80°, Lascgue ' s sign is pos itive; the pain is due to the tension and patho logic compression of the sciatic nerve. When Bragard' s sign is examined, the hip is flexed until the onset of pain and the foot is then dorsiflexed: the pain increases. While Lasegue 's sign is positive for any root in the sciatic nerve, the femoral sign may be positive in cases of root compression of the higher lumbar spine segments. If the knee of a patient in the prone position is flexed, severe pain is experienced at a certain degree of flexion, blocking further flexion. The knee flexion can be extended only after the pelvis is lifted or af ter a significant flexion of the hip. During examinations of the radicular signs, motor, sensor and reflex changes are searched for that correspond to the individual roots . The most common symptoms are re lated to roots L4, L5 and S I (Fig. 24.48., 24.49., 24.50.). The assessment of the lumbar spine in cludes an assessment of the lower limbs, as it is a common dilemma to decide whether com-
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plaints are o f spinal i n origin o r whether some hip disorder plays a role in their appearance.
24.2.3. Radiological assessment Basic examinations. The X-ray assess ment of spinal disorders demands antero posterior and lateral exposures ofthe involved segment. Much more practical, however, is a total spine film (30 . 90 cm) including the pel vis and the skull, taken with the patient in a standing position. Standing is necessary, be cause the curves resulting from loading of the spine are easier to consider. If there are tho racic spine complaints, the end-points of the possible curvature are located on the antero-
posterior X-ray film. The upper end of the structural curve is the vertebra, at which the upper endplate displays the greatest tilt in the direction of the concavity, and the lower end of the structural curve is the vertebra, at which the lower endplate has the greatest tilt in the direction of the concavity. The supplementary angle to the perpendicular lines drawn to the endplates of the end vertebrae shows the ex tent of the structural curves (Fig. 24.1 0.). The tip of the curve, decided from the X-ray film, is normally the vertebra located farthest from the axis ofthe body, which also has the largest rotation. The extent of the rotation is usually estimated from the contour of the vertebra, and from the projection of the pedicles and laminae that deviate from symmetry. Two in ternationally approved methods (those of Nasch-Moe and Perdriolle; Fig. 24.1 1.) are used to measure the rotation of the vertebrae, from which the neutral vertebrae of the curves can be determined. These are located next to the extremities of the curves without rotation. From a therapeutic aspect every vertebra with a similarly directed rotation is considered a component of the curve. Lateral films are taken for detection of the borders of the sagittal curves and their possi ble changes.
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Fig. 24. 11.
[Q-HH
Measurement of vertebral rotation with the method of Nasch-Moe and Perd riolle.
2 4. D i s e a s e s of t h e s p i n e
To summarize: In the course of the normal X-ray assessment, information is obtained on the presence of scoliotic curves, and on the presence or absence of possible concomitant changes (congenital abnormalities, traumas, tumors, dystrophic bone diseases, etc.).
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Additional examinations Bending films: Information is usually acquired on the mobility of the curve and on the exact borders of the main and compensatory curves. This information is needed primarily for the planning of therapeutic interventions (Fig. 24. 1 2.). Dittmar 's views: These special, oblique X-ray views are mainly used to visualize the foramen and the interarticular part of the vertebral arch (see: spondylolysis). In the event of deformities, these views are used to determine the anteroposterior and lateral aspects of the markedly rotated vertebrae. This may be of great help particularly for the recognition of possible
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congenital abnormalities located at the level of the punctum maximum of the curves. eT examinations: These are needed in special cases for the assessment of bony structures . They help to reveal congenital abnormalities. The 3D reconstruction picture accurately determines the localization of wedge vertebrae. In degenerative diseases, eT scans are performed to detect prolapsed discs, spinal stenosis, or possible bone tumors. MRl examinations: These are informative if there is a suspicion of pathological processes involving the soft tissues and the spinal cord. In cases of neurofibromatosis, MRI examinations of the entire spine are mandatory to exclude spinal cord mani festations and a paravertebral soft-tissue mass, and also when congenital vertebral abnormalities are present, to exclude concomitant congenital spinal cord abnormalities (syringomyelia, diastemato-
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Fig. 24.12. Bending X-ray. The mobility of the spinal curve can be estimated. a: Normal posture, b: Bending to the rig ht, c: Bending to the left
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a
b
Fig. 24.13. Myelography. A definite stoppage of the contrast flow is visible. a: Anterioposterior view, b: Oblique view
myelia, tethered cord, and Arnold-Chiari malformation), and in degenerative spinal disorders to evaluate the state of the intervertebral discs. Scintigraphy (bone scan) : This is most frequently performed when the suspicion oftumoral changes (e.g. osteoid osteoma),
metastasis, or inflammatory disease arises. Since scoliosis is rarely painful, in cases of painful scoliosis the bone scan is indicated to discover the cause of the pain. Myelography: X-ray pictures taken following the inj ection of contrast material into the epidural space can reveal ex-
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Fig. 24. 14. Discography demonstrating a healthy (a) and a diseased (b) discogram.
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24. D i se a s e s of t h e s p i n e
pansive processes (herniated discs, spinal cord tumor). They can be highly infor mative when combined with a CT scan (Fig. 24. 13.). Discography: X-ray pictures taken following the inj ection of a known amount of contrast medium into the disc furnish information on the state of the disc and the pain-inducing effect of the inj ected medium (Fig. 24. 1 4.).
24.3. Classification of deformities of the spine Deformities in one plane, localized to the sagittal plane �
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Hyperkyphosis - Functional (nonstructural) hyperkyphosis - Postural hyperkyphosis (negligent posture) - round back (dorsum rotundum) - saddle back (dorsum kypholordoticum) - Structural hyperkyphosis - Scheuermann 's disease - Calve 's disease - Neuromuscular diseases - Congenital hyperkyphosis - Trauma - Metabolic disease - Osteoporosis - Tumors - Iatrogenic hyperkyphosis - Postoperative - Post-irradiation Hyperlordosis - Functional (nonstructural) hyperlordosis - Postural lordosis - flat back (dorsum planum) - Hyperlordosis related to hip flexion contracture - Structural lordosis
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Deformities in 3 dimensions : �
Scoliosis - Functional (nonstructural) scoliosis - Postural scoliosis - Scoliosis related to limb length inequality - Hysteriform scoliosis - Scoliosis related to root irritation - Scoliosis related to disc hernia - Scoliosis related to tumor - Scoliosis related to inflammation - Appendicitis - Structural scoliosis - Scoliosis of unknown origin (idiopathic) scoliosis - Scoliosis of known origin - Congenital scoliosis - Neuromuscular scoliosis - Neurofibromatosis - Mesenchymal diseases - Trauma - Extraspinal contractures - Osteochondrodystrophies - Metabolic diseases - Tumors
24.3. 1. Characteristics of deformities in one plane During the demonstration of deformities, it is essential to differentiate the functional and structural curves, since absolutely differ ent treatment strategies must be employed for the two curve types. Functional curves are extremely mobile, and well correctable. They never become rigid, and they are not accompanied by struc tural changes of the vertebrae. Sagittal deformities are curves that usually originate from increased or decreased physio logic curves. Physiologic sagittal curves de velop as the individual learns to sit, stand and walk, in parallel with the development of the trunk muscles. The spine of a neonate is characterized by the intrauterine position, with kyphosis of the
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entire spine. I n the course o f individual devel opment, the first curve to appear is cervical lordosis. This is observed when the child turns onto the tummy and lifts up the head. In the other segments of the spine at this stage there is a ventrally concave curve, kyphosis of the entire spine. The kyphotic spine first straight ens when the individual begins to stand and walk (at 1 3 months), and lumbar lordosis then develops. From the age of 3 years, mild lordosis is visible, which attains the final form at the age of 8- 1 0 years . Any external factor which disturbs this spontaneous process, depend primarily on the level of development of the muscles, will im pair the further development of the child. The most common error is to force a child to sit and walk too early before the appropriate development of the necessary muscles. In many children, overfast vertical development is imposed, instead of ensuring a spontaneous, horizontal motion which provides a more healthy static development course. If the child
has enough room following turning onto the tummy, the demand for motion will be ful filled by moving horizontally (creeping or crawling) without sitting up. Standing and walking will start only when the musculature has properly developed. The advantage of horizontal as compared to vertical motion de velopment is that each static developmental stage takes place with the properly developed musculature without external assistance. Thus, the child sits rarely or not at all, avoid ing the posture which imposes the highest passive load on the spine. The normal sagittal curves become auto matic and characteristic qualities of the indi vidual at the age of 8- 1 0 years. 24.3. 1 . 1 . Fu nctional hyperkyphosis The most typical example is a negligent or im proper posture. Its development is related to the diminished load-bearing capability of the dorsal and trunk muscles, but psychological factors may also contribute.
The following types can be distinguished (Fig. 24. 1 5.): �
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Fig. 24. 15. Correct and improper postures. a: Normal postu re, b: Round back, c: Kypholordotic (saddle back), d: Flat back
Round back (dorsum rotundum) : The enlarged dorsal kyphosis is enhanced, while the lumbar lordosis is physiological. The hyperkyphosis usually involves the upper lumbar section. It is a consequence of inequality between the development of the muscles and the gravitational load, but a lack of the willpower, needed for the proper posture also plays a role in its onset. Saddle back (dorsum kypholordoticum) : Both the dorsal kyphosis and the lumbar lordosis are larger than normal. The most common form occurs at the age of 6- 1 0 years, i f the muscle development i s not proportionate with the growth. The increase in lumbar lordosis is most probably is compensatory.
A negligent posture may be balanced by active gymnastics and muscle training exer-
24. D i s e a s e s of t h e s p i n e
cises, whereby its progression may be stopped. An effort must be made to discover the origin, since a negligent posture is fre quently a symptom of a general weakness of the muscular system. A negligent posture is often accompanied by generalized j oint lax ity, drooping shoulders, and a protruding ab domen. Such children are often easily fa tigued and reticent, which suggests a psycho logical cause. Treatment. The etiology must be discov ered and the cause must be eliminated, as an irreversible postural deformity can develop in a neglected case. 24.3. 1.2. Functional hyperlordosis An increased lumbar lordosis is generally compensatory. It serves the aim of balancing the spine in the sagittal plane when the dorsal kyphosis is enhanced. Normalization and straightening in a lying position are typical of compensatory hyperlordosis.
Besides compensating dorsal hyper kyphosis, hyperlordosis may somewhat cor rect an ill-positioned pelvis. In consequence of the flexion contracture of the hip the pelvis tilts forward, and the sa cral slope is greatly increased, reaching even 90°. To compensate this, lumbar hyperlor dosis develops, in order to maintain the nor mal, balanced bearing. In parallel with the weight gain of the em bryo in pregnancy, the sacral slope increases, and hence the gravity line of the body runs in front of the hip j oint. A precondition of straight standing is the compensatory increase of the lumbar lordosis, which may explain the frequent low back pain during pregnancy. Af ter delivery, the hyperlordosis declines, and accordingly this low back pain alleviates. Ex tremely marked lumbar hyperlordosis devel ops in cases of paralysis of the abdominal and long dorsal muscles. Flat back (dorsum planum) : This is an other characteristic sagittal curve abnormal ity. The dorsal segment is almost flat, since
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the dorsal kyphosis disappears and relative or absolute dorsal lordosis develops. The lumbar lordosis is also greatly diminished, the spine becomes expressively straight, and as a result the back becomes flat. The Delmas 's formula indicates that, if the number of spinal curves diminishes, the sta bility also decreases, and consequently the spine becomes insufficient and unstable; this IS a common source of low back pain in adults. 24.3. 1.3. Struct u ral kyp hosis The most frequently observed structural changes o f t h e p hysiologic curves in one plane are kyp hotic changes. There are two well-differentiated forms: reg u lar and angular ky p hosis (Fig. 24.16.).
Regular hyperkyphosis : The most typi cal example of thoracic hyperkyphosis devel ops as a result of Scheuermann 's disease (Fig. 24.1 7.). The extent of the kyphosis increases
Fig. 24. 16. Regular and angular kyphosis.
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along the full length o f the thoracic spine, and the punctum maximum is located distally. In a parallel process, the cervical and lumbar lordosis also increases considerably, so as to compensate the increase in the thoracic curve. After the end of growth, progression is not likely. �
Scheuermann 's disease (osteo chondrosis j uvenilis dorsi, kyphosis dorsal j uvenilis)
This d isease develops because of damage to the secondary ossifying centers of the in volved vertebrae; it is mai nly localized to the dorsal section, and involves many vertebrae. In a typical case, the anterior third of the af fected dorsal vertebra l body loses its height, and consequently an increase in the regular dorsal ky p hosis fol lows. The intervertebral discs become thinner, and display degenera tive signs. Typica l p henomena are the rigid dorsal hyperkyphosis and in advanced cases, back pain. The treatment is chiefly conserva tive; surgery is rarely performed (Fig. 24. 17.).
Etiopathology. This condition was first described as juvenile dorsal kyphosis by Scheuermann in 1 920, referring to the aseptic necrosis of the secondary ossifying centers of the vertebral bodies. The ossifying distur bance involves chiefly the front half of the epiphyses, and the vertebral bodies assume a wedge shape. The epiphysis areas with weak ened resistance may crack, and nucleus pulposus substance may protrude through these fissures to the cancellous mass of the vertebral bodies (Schmorl 's node) . The X-ray changes point to the absence of normal growth rather than a destructive process. In the period of active growth, the mechanical overload due to the physiological kyphosis further hinders the development of the verte brae, leading to a further diminution of height. The compression of the anterior part of the vertebrae causes the dorsal kyphosis to in crease, enhancing the mechanical load on the ventral edge of the vertebrae. This vicious cir cle results in Scheuermann' s hyperkyphosi s.
Fig 24.17. Kyphosis dorsalis juvenilis.
Dominant inheritance factors also play a role in this ossification disturbance, since the likelihood of multiple occurrence in the fam ily is 50%. Incidence : Various sources report the in cidence in puberty to be 0. 5 - 1 1 %. The gender distribution also varies. According to clinical and X -ray surveys, the Hungarian incidence is 1 1 %, and there is no difference between the two genders. Localization. This condition is most com mon in the dorsal vertebrae, and it involves more than one. Less frequently, it is seen in the dorsolumbar section, where it likewise in volves the epiphyses of a few vertebrae. Even more rare is a lumbar localization, when it in volves only the epiphysis or end-plate of a sin gle vertebra, resulting in a large Schmorl ' s hernia. Apophyseal osteochondrosis should be classified as a separate pathological entity. This also involves lumbar vertebrae, and frag mentation and the disturbed growth of the
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apophyseal bone centers forming the anterior edge of the vertebrae can be observed. The etiology is unclear, as for the epiphyseal type. Clinical symptoms: The disease starts at around the age of 1 0 years ; the explanation is the onset of secondary bone centers activated from the age of 8 years. The most important clinical symptoms are hyperkyphosis and pain. Hyperkyphosis : The chief clinical symp tom is the fixed, absolutely rigid and in creased dorsal kyphosis, which also causes se rious cosmetic problems . In Scheuermann ' s disease, the kyphosis i s decreased to only a very small extent or not at all . Apart from the rigid, fixed dorsal kyphosis, another important symptom may be distalization of the punctum maximum of the kyphosis. A conspicuous sign is the hyper pigmentation of the skin over this segment, resulting from the increased dorsal kyphosis in response to the pressure against the back-rest of a chair or bench . Another clinical sign is the increase in the lumbar lordosis. This is compensatory and therefore especially mobile; it may be accom panied by spinal pain because of the stretched ligaments of the lordotic spine . In about one-third of the cases hyper kyphosis is accompanied by moderate, usu ally functional or mildly structural dorso lumbar scoliosis, which is never progressive. In general there are no neurological signs and radicular pain. Pain: Besides the cosmetic problem con cerning the spine, the pain is an important clinical sign; its appearance and intensity vary with the age, the stage of the disease, and the localization and severity of the kyphosis. In the early phase, pain is usually not experi enced. The patient seeks out the doctor be cause of the visible deformity. At around puberty, the frequency of pain may increase, but in only 1 0-20% of the cases. The muscular pain in the interscapular region can be characteristic, and as can the tender ness of the involved vertebrae, which in creases in response to physical activity and the pain if these vertebrae are tapped on. This
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type of pain usually ceases at the end of growth, unless there is a severe residual defor mity. After the end of growth, however, if a se vere residual deformity is present, the pain caused by the degenerative changes will be the leading clinical symptom, paradoxically localized in the area of the compensatory curves. In lumbar Scheuermann 's disease the most important clinical symptom is the accentu ated, severe pain localized to the involved vertebrae. Since the deformity in this location is difficult to discover (initially only a de creased lordosis is seen, and manifest kyphosis can be detected only in advanced cases), the source of the pain is often confused with inflammatory diseases (vertebral osteomyelitis or spondylitis tuberculosa). X-ray signs: Besides the physical findings the diagnosis of Scheuermann 's disease is based on the X-ray examination. The typical vertebral changes are the following (Fig. 24. 1 8.):
Fig. 24.18. X- ray signs of Scheuermann's d isease.
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- Trapeze-shaped vertebrae: One o f the most significant X-ray findings is the wedge formation of at least three ver tebrae. The criterion of the wedge shape is that the end-plates converge to the front by more than 5 ° . Trapeze-shaped vertebrae lead to the development of regular hyperkyphosis in excess of 50°. The intervertebral spaces diminish, particularly anteriorly, due to the hyper kyphosis and the trapeze-shaped vertebrae. - Schmorl 's hernias: These changes are considered to be a diagnostic criterion for Scheuermann' s disease. Small hypo dense, pearl-like indentations are visible under the end-plate fissures, often surrounded by a sclerotic margin. - End-plate irregularity: The secondary ossification centers are fragmented, and the end-plates therefore display a zigzag irregularity.
Two rare, atypical radiological signs are observed in the lumbar spine : - Epiphyseal form: A huge Schmorl ' s hernia is visible in a single vertebra, usually in the anterior third of the body. This differs from the common Schmorl 's hernias, for it may attain a considerable size; it is connected widely to the intervertebral space, and the edges are markedly sclerotic. - After the end of growth, in the late phase of Scheuermann ' s disease, besides the radiological features outlined above, degenerative changes may appear in the involved and in the compensatory curves, with the most typical osteo phytes . Clinical sequence. Three stages are dis tinguished: The first stage starts between the ages of 1 0 and 1 2 years, with a negligent posture and a mildly increased dorsal kyphosis, usually with no complaints. In this period, the in creased dorsal kyphosis is mobile, and can be
eliminated through active muscle power. The child is taken to the doctor because of the bad posture. The second stage starts at the age of 1 2 and lasts until the end of growth. The spinal seg ment becomes rigid. The progression may ac celerate in teenagers, and the deformity and the complaints intensify. 1 0-20% of the pa tients seek the doctor' s help for these com plaints . Apart from the visible deformity, the interscapular pain and vertebral tenderness are the leading signs. The symptoms may be come stronger on physical activity, but they usually cease at the end of growth. The third or late stage, after the end of growth accompanies the patient throughout life. However, large proportions of the pa tients remain symptom-free. In the event of a severe residual deformity, the early spondylosis progresses. Pain will be strong in the compensatory curves, mainly in the cervicothoracal junction and in the form of headache. In the lower lumbar spine, the com pensatory curves may lead to the appearance of lumbago at a relatively young age. Treatment. Although three stages are dif ferentiated in Scheuermann' s disease, the his tory is not well known. Both conservative and surgical treatment are based on empirical fac tors. An adolescent with mild hyperkyphosis (50°) without rapid progress does not gener ally need treatment, but check-ups should be regular, with continuous training of the active elements of the spine (exercises and swim ming). These patients have a good chance for a symptom-free life following the end of growth. The basic treatment of Scheuermann 's dis ease is conservative. The principles and indi cations of therapy are as follows : - Rapidly increasing dorsal kyphosis. - Vertebral changes, and progressive wedging. - Severe dorsal kyphosis and pain that does not respond to medication or conservative measures. - Breathing disturbances due to vertebral deformities.
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With timely therapy, the overwhelming majority of the patients respond well to corset treatment. Indications are : - A curve less than 70° - Risser ' s sign less than 3 . - More than 40% o f the curvature can be corrected passively. - Minimal vertebral wedging is seen. The most important precondition of effec tive corset treatment is spinal flexibility, the chances of the success of corset treatment be ing particularly good when treatment is started in the first stage. In this benign condi tion, surgery is rarely justified. An indication for surgery is a curve exceeding 80°. If the de formity is accompanied by severe pain, which hinders the everyday activities and is not al\e viated by conservative means, surgery may be considered with dorsal kyphosis of 60-80°. The strategy of the surgery is different in the second and third stages. In the second
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stage, operative correction may be performed from a posterior approach; in the third stage, after the end of growth, the surgery is carried out in two steps: anterior release and posterior correction (Fig. 24.1 9.). Angular hyperkyphosis: This is a strictly sagittal deformity, where the changes involve only a few (sometimes only a single) levels, and consequently the harmonic sagittal align ment of the spine is badly distorted with an an gular deformity. This deformity has two prin cipal consequences: the risk of instability, and the possibility of marked secondary deformi ties, depending on the extent and location of the primary deformity, to maintain the com pensation of the spine. These two complica tions are closely related, the increase in defor mity may enhance the instability. A number of diseases may result in angu lar kyphosis, e.g. Calve 's disease, neuro muscular conditions, congenital vertebral ab normalities, trauma, metabolic diseases, such
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Fig. 24. 19. A 16-year-old boy with Scheuermann's disease. Preoperative hyperkyphosis 74° (a). Following surgical correc tion 44° (b). No lateral cu rve (c); the correction is in only 2 dimensions. (d)
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a s osteoporosis, certain tumors, iatrogenic conditions, and after surgery or irradiation. The risk of instability associated with an gular kyphosis is evidently dependent on the etiology and also on the level and/or the state and spatial location of the connected func tional units. The most severe instability may arise following trauma. Similarly, congenital anomalies, such as a dislocated spine or poste rior hemivertebra lead to kyphotic deformities and hence to potential (occasionally immedi ate) instability. The outcome may be similar in infectious diseases of the spine, where one or more discs and vertebral bodies may be destroyed, lead ing to stable angular kyphosis. The most important elements of the treat ment strategy are the restoration of the bal ance, the normal geometry and the bio mechanics of the spine, and the ensurance of stability. These aims are achieved either by a conservative approach, or by surgical means, depending on the etiology. �
Clinical symptoms. Typically, dorsal pain sets in, which increases in response to ac tivity. Insignificant angular kyphosis is ob served at the painful segment, and the spinous processes are tender. Since the segmental nerves are irritated, mild neurological signs may arise in the corresponding dermatome. The movements of the spine may be restricted in every direction. X-ray signs. In the lateral view, the bony center of the vertebra involved is hyperdense, sclerotic and flattened (Fig. 24.20.). The ante rior part of the ossifying center becomes pa per-thin (silver dollar vertebra). Both below and above, the intervertebral spaces exhibit compensatory widening.
Calve 's deformity or vertebra plana (vertebra plana, osteochondrosis vertebrae)
This is a condition localized to a single verte bra and caused by damage to the primary (central) ossifying center. In a typical case, the ossifying center of a dorsal vertebra is flat tened and the anterior two-thirds of the ver tebra becomes paper-thin.
During recovery, the height of the verte bral body is partially restored, but it never reaches its original height. The discs adj acent to this condition remain intact. The etiopathology is uncertain. Calve classified this affection as a sterile necrosis of the ossifying centers of the developing organ ism, on the basis of the radiological signs and the benign outcome. Histological studies, however, demonstrated that the reason for vertebra plana was eosinophilic granuloma. It is not excluded, that there are two different etiologies which give a similar clinical picture. ..
Fig. 24.20. Calve's deformity. The D6 vertebral body is paper thin (on the left). After 2 years, almost total restitu tion has been achieved (on the rig ht).
Differential diagnosis. Eosinophilic granuloma must be excluded. Sternal punc ture usually facilitates the diagnosis. It must be differentiated from tuberculotic vertebral diseases, the latter involving the neighbouring discs. In Calve ' s disease the ESR (erythrocyte sedimentation rate) and the result of other lab oratory tests are normal. Therapy. This condition usually heals spontaneously, and is not progressive; it does not cause instability at the spine. In the acute, painful stage, some days of bed rest, a reclin ing corset or a temporary reduction of activity is needed.
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24.3.2. Spinal deformities 3 dimension: Scoliosis 24.3.2. 1. Fu nctional (nonstruct u ra l) scoliosis Functional curves are quite mobile, well- cor rectable cu rves; they never become rigid. On lateral bending, they are fully and sym metrically corrected, so they can never be identified from bending X- ray views. No structural changes are present in the verte brae; according ly, no rotation or related rib hump is detected in the hyperflexion view.
Etiopathology. This entity is a lateral spi nal curvature of unknown origin. There is generally a simple postural deformity in the background. The most frequent reason is the weak back musculature, the lateral bending of the spine being related to the usually poor muscular state. An important factor is the im paired balance between the active and passive elements of the spine. It sets in when all-day studies and an inactive made of life mean that the musculature does not develop appropri ately, but weakens instead, not following the development of the passive elements of the spine. This deformity may also be the first in dication of psychic problems, and it may also turn attention to root compression (a juvenile disc prolapse). It is almost always present when there is a limb length discrepancy. Incidence. It occurs mainly in girls, to gether with the first signs of puberty, in about 1 5% of the population. Localization. In 90% of the cases a ' wide-arched, left convex thoracic or thoracolumbar, very mobile curve is seen. Clinical symptoms. There is a lateral curve, and a badly negligent posture, and the shoulders are drooped forward. Abdominal breathing and other signs of joint laxity are frequent. Flatfoot is also usually present. The children are asthenic, and their activity is di minished.
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The lateral curve is quite mobile, and well correctable with active and passive muscle power. When the individual is lying or bend ing forward, the curve disappears and no rib hump or any structural change is detected. X-ray signs. Since the deformity disap pears in a lying position, it can be detected only in a standing X-ray, as a wide arched bend showing no structural signs. Therapy. If the functional scoliosis is due to a limb length discrepancy, the curve is cor rected by equalization. Functional scoliosis is caused by appendicitis, or disc herniation: if the etiologic factor is eliminated, the bend ceases. Regular swimming and physical exer cises are recommended to strengthen the back and belly muscles.
24.3.2.2. Struct u ra l sco liosis Scoliosis is the best known of the general ized 3D deformities of the spine. Structural scoliosis is such a deformity, characterized by frontal bending, an anteroposterior, almost exclusively lordotic deformity and horizontal vertebral rotation around the gravitational axis. 24.3.2.2. 7. Idiopathic scoliosis
This is the best-known structural deformity. Idiopathic structural scoliosis is a 3D deformity of the spine, characterized by frontal bending, an a nteroposterior, almost exclusively lordotic deviation and vertebral rotation in the hori zontal plane around the gravitational axis. The presence of vertebral rotation is a diagnostic criterion of structural bends. A number of subtypes are differentiated within idiopathic structural scoliosis as regards the onset, local ization and prognosis. Some of them appear early, and progress rapid ly, causing severe spinal and chest deformities, whereas others progress more slowly.
Etiopathology. The cause of idiopathic structural scoliosis is unknown, as are the fac-
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tors responsible for the differences i n onset, location and progress ofthe various bendings. Many factors are suspected as being primary causes of idiopathic structural scoliosis. The more important ones are summarized below: Genetic origins: There are certain facts, such as the similar appearance in twins, and a familial accumulation, which turn the atten tion to genetic factors . However in spite of the indisputable presence of genetic factors, the exact course of inheritance is not clear. It is certain that scoliosis inheritance is not con nected with chromosome X. The presence of a dominant gene is suspected, but polygenic in heritance is not excluded. The Scoliosis Re search Society has declared scoliosis to be a genetic malformation. It must be emphasized that idiopathic structural scoliosis is an iso lated developmental disorder. An isolated manifestation is not typical of monogenic (dominant, recessive and gender-related) in herited disorders, but rather of polygenic in heritance (see hip dislocation and clubfoot) . Thus, instead of a multiple disorder, we are faced with the consequences of the distur bance of a given developmental process. It is also a fact that there are no maj or dif ferences in geographic incidence, which sug gests that environmental factors do not play a significant role in the onset of idiopathic structural scoliosis. Growth. The connection between growth and idiopathic structural scoliosis has been known since the start of the 1 9th century. Growth is a complex process that depends on genetic, hormonal and environmental (health and feeding) factors. Not much is known of the complex regulation of the process. An analysis of the connection between growth and idiopathic structural scoliosis reveals some striking connections : - Growth is needed for the development of structural scoliosis. - The progression of an existing spinal deformity accelerates in the period of ra pid growth. - Small, mild curves attain a steady state following the end of growth.
A discussion of growth necessitates men tion of the widely investigated hormonal fac tors. The extensive female predominance in idiopathic structural scoliosis points to the role of the estrogen metabolism, as does the observation that, in severe, progressive cases, hypogonadism is detected in girls (late devel opment of secondary gender characteristics, late menarche) . However, no disturbance of the secretion of sex hormones has been identi fied. The suspected disturbance of the estro gen metabolism does not explain either the scoliosis occurring in boys or the progressive scoliosis appearing at a younger age. Connective tissue causes: The compo nents of the viscoelastic connective tissue in clude collagen and proteoglycans. The me chanical properties of the connective tissues are determined by the distributions of these components and the orientation of the fibrils. Qualitative and quantitative changes in both the collagen and the proteoglycans may be recognized in idiopathic structural scoliosis, and these connective tissue defects of genetic origin are considered by many experts to be the primary reason for idiopathic structural scoliosis. It has not yet been determined whether the changes perceived are causative factors or secondary changes due to the effect of the developed curvatures. Neuromuscular factors : It is generally ac cepted that postural reflexes are the most im portant factors in the stability of the axial skel eton. A clear understanding of the muscular function, and especially that of the para vertebral muscles, is essential for familiarity with the development of the idiopathic struc tural scoliosis. Any neurological or muscular disturbance in a growing subject is a factor predisposing to scoliosis. Accordingly, any subclinical neuro muscular dysfunction could be a primary etiologic factor in idiopathic structural scoliosis. Maschida presumed a central neuro muscular anomaly or asymmetry in the proprioceptive reflexes as a causative factor. Biomechanical factors: Many bio mechanical factors may be considered to be the primary signal in the development of idio-
24. D i s e a s e s of t h e s p i n e
pathic structural scoliosis. The most impor tant is a decrease of physiologic curves, the cessation of dorsal kyphosis, which is the ba sic, primary etiologic factor of idiopathic structural scoliosis. From the Delmas formula it is clear that, in parallel with a decrease in the sagittal curves, the stability of the spine de creases, and if the load increases (an overload triggered by fast growth), the response will be lateral bending, i.e. scoliosis. To summarize: Idiopathic structural scoliosis is a genetically determined disease. Both biomechanical factors and a genetic pre disposition play roles in its development. If these two factors are present, hormonal and/or connective tissue changes in the period of growth may lead to the development of struc tural scoliosis. Incidence. It is most common in child hood, before puberty. Curves exceeding 200 are observed in 0.5% of the population. It is 8- 1 0 times more common in girls than in boys. Depending on the time of onset of the curve, idiopathic structural scoliosis can be subdivided into groups, this traditional classi-
Fig. 24.21. Mehta's costotransversal angle. The greater the dif ference between the two angles, the more likely the progression of the scoliosis.
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fication possibly hinting a t the likelyhood o f worsenmg. Infantile scoliosis develops before the age of 3 . The curves with the best prognosis here are those without curvature extending to the spine overall, while those with the worst prog nosis are the very short ones involving only 1 -2 segments. The former of these two curves may be re garded as a manifestation of intrauterine com pression syndrome (the siebener, number 7 syndrome) and may be observed together with the other symptoms of the intrauterine com pression syndrome : torticollis, skull asymme try, hip dysplasia, pelvic asymmetry, foot de formity, scoliosis and thoracolumbar kypho sis. These symptoms disappear by the age of 6, without treatment. The second, much less common type is malignant scoliosis. Huge curves may de velop by the end of growth, exceeding 1 000, with associated cardiorespiratory, and often neurological problems. It is difficult to differentiate the two groups by means of simple observation. Mea surement of the costovertebral angulation (Fig. 24.2 1 . a) may help identify the progres sive variant. Malignant, untreated curves may end up in severe neurological symptoms, which practically never evolve in idiopathic scoliosis. Thorough clinical tests may dis close the etiologic factor in a large proportion of the curves. Juvenile scoliosis develops between the ages of3 and 1 0 years . These curves follow an evolution scheme closely connected with the onset of the curve. They dramatically worsen in the period of puberty, and therefore a regu lar check-up is essential, every 3-6 months. This is particularly important for double curves, where the two curves compensate each other and for a long time do not seem to elicit maj or morphologic changes. Adolescent scoliosis occurs after puberty. It has a better prognosis, and the picture is not so severe, as in the previously discussed groups . However, it should be noted that ado lescent scoliosis undergoes a mild worsening until ossification is complete.
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Localization. Various manifestations are listed, the sequence reflecting the incidence (Fig. 24.22.). The thoracic curve is the most common type, with the worst prognosis. It is situated between Th6 and 1 2, causing a severe chest deformity and breathing problems. It is gener ally right convex, and often accompanied by marked decompensation of the upper trunk. Combined scoliosis has a right convex dorsal curve and a left convex lumbar curve, which usually compensate each other. This is
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Main localizations of idiopathic structural scoliosis. a: Right convex dorsal, b: Right convex dorsolumbal c: Left convex lumbal, d: Right convex dorsal and left convex lumbal scoliosis
not striking, because of the compensated state, and it can therefore be undetected for a long time. At its discovery, a large curve may already persist. The observation of sagittal curves is particularly important. The detection of kyphosis in the spinal segment between the dorsal and lumbar curves is an indication of a particularly bad prognosis as regards the curve progression. Otherwise, this type has a moderate prognosis as regards the breathing function and its progression. A lumbar curve is usually left convex with the apex located at L2- 3 . It has a good progno sis in childhood. It is never accompanied by a breathing function impairment, though if it is extensive, a marked pelvic tilt may occur. From the age of 40, in the presence of even mild curves, degenerative changes may occur, causing severe pain and disability. The thoracolumbar curve with apex at Th l l - 1 2 or L l -2 is generally right convex, and has a somewhat better prognosis, though a third of them exhibit marked progression. Breathing function impairments are not typi cal. Clinical symptoms. In childhood the de formity is the only symptom. The first note worthy sign is the flattening of the physiologi cal curves, leading to a flat back. The lateral spinal curvature is notable only if it is exces sive. The height of the shoulders, the scapu lae, the trunk-arm triangle and the position of the iliac crests are asymmetrical. On the con vex side of the curve, the shoulder and scapula are higher; the scapula is elevated. The trunk-arm triangle is thinner and shorter, and the iliac crest is less prominent (Fig. 24.23.). The upper trunk is often decompensated. Structural scoliosis is always accompa nied by a rib hump, which can easily be de tected and measured in the forward bending position. A smaller rib hump is visible in front, on the concave side, often causing dif ference in appearance and size of the breasts, evoking a marked psychological problem for girls in puberty (Fig. 24.24.). The movements of the spine are apprecia bly restricted; the change is proportional to
24. D i s e a s e s of t h e s p i n e
the extent of the curves. It can be assessed by bending the spine laterally (bending test), and by the axial traction of the head. In childhood, the deformity is not related to any pain. If pain sets in at an early age, some cause other than scoliosis must be searched for. At an older age, the frequency of pain increases due to the secondary degenera tive changes. This is typical in lumbar curves. Changes in pain intensity following pu berty are also related to hormonal changes. During pregnancy and after menopause a pain increase is to be expected. One of the most important clinical signs is an impairment of the breathing function. This is significant for severe curves, when rest dyspnea is often observed, due to two factors : the chest rotation caused by the spinal defor mity, with the consequent lung compression and restricted breathing motions, and the seri ous increase in pressure in the pulmonary cir culation, leading to a cardiac insufficiency.
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Fig. 24.24. Appearance of vertebral rotation-, torsion and rib hump in scoliosis.
Because of these two factors, in severe cases, when the curves exceed 60°, the life expec tancy may be even 30% less than in the nor mal population. Most of these patients die from heart failure.
Fig. 24.23. Chief characteristics of scoliosis.
X-ray signs: For the documentation of scoliosis, bidirectional standing films (3 0x90 cm) are needed, including the pelvis and the head. In the standing position the curves of the loaded spine are easier to determine. The ex tent of the structural curve is measured on anteroposterior films by Cobb ' s method. The rotation is determined with the technique of Nasch-Moe or Perdriolle. Assessment of the shape of the vertebrae allows conclusions on the structural changes due to deformities, which are proportional to the extent of the curve. The vertebrae are higher on the convex side due to the growth asymmetry, and lower on the concave side, where degenerative changes follow because of the increased pres sure. On the lateral films, the borders of the
258
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2 4 . D i s e a s e s of t h e s p i n e
normal sagittal curves and possible changes are detected. To summarize: In the course of the normal X-ray assessment, information is acquired on the appearance of the scoliotic curve and pos sible associated changes (congenital anomaly, trauma, tumor, bone dystrophy, etc.).
this period. The rate of growth o f spine can be observed from the appearance of the ossifica tion center of the iliac crest (Risser 's sign). The end of ossification corresponds to the end of the growth of the spine, and the end of the rapid progression of the scoliotic curves. It is essential to discover these curves early, and to start the treatment as soon as pos sible, ignoring the arbitrarily outlined treat ment threshold (35-40°), in the hope of a better outcome. Idiopathic structural scoliosis can be treated by conservative and surgical means, the indications of which differ:
Therapy. Every step in the physical as sessment has the aim of the most precise diag nosis possible related to expected progression of the curve. Many experts have assessed the natural history of scoliosis. It has been clearly estab lished that the possibility of the development of scoliosis is already present at birth. The de formity emerges only in childhood, and its se verity is individually variable. Some curves never worsen, whereas others undergo a dra matic progression at puberty. The third group is, where the worsening is linear, but the progress speeds up in puberty. The cause of this dramatic progression at puberty is simple. The peak in the growth of the upper trunk and the spine at that time (Fig. 24.25.). Due to the rapid growth of the spine, the worsening may reach 7-8° in a month in
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Conservative treatment: Various corsets and plaster fixations are used. The indications are as follows: - Curves in the interval 1 0-40° - Risser' s stages 0-3 - If surgery is contraindicated up to skeletal maturity. The various forms of conservative treat ment were introduced in the leading scoliosis centers around 1 950, and both the techniques and the results of corset production gradually improved. growth/year . o o -tO
extent of curve .---.
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Fig. 24.25. Connection of the progression of scoliosis with the growth and sexual development.
2 4. D i s e a s e s of t h e s p i n e
Corset treatment is currently recom mended in three groups of patients: - Infantile scoliosis: In cases of malig nant, progressive curves, corset treat ment is the only therapeutic modality as a supplement of plaster corsets until the age when surgery can be performed. - Juvenile and adolescent scoliosis: Corset treatment may stop and/or decrease the progression, leaving a curve that can be tolerated well in adulthood. - Adult scoliosis: Their role is exclusively functional.
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The aims of corset treatment: - To stop or decrease the worsening of the curve, to decrease or eliminate the increasing asymmetry of the individual vertebrae. To enhance the compensation of the entire spine, to transform a decompensated single curve into two combined, well compensated curves, which are better tolerated in the long run.
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Derotation is strived for with by posterior pads in the horizontal plane. Derotation is simple correction of the rib hump, which also influences the sagittal and horizontal curves. Basic types of corsets . The Milwaukee corset was devised by Blount and Schmitt in 1 946 (Fig. 24.26.) for the postoperative fixa tion of scoliosis, and particularly that due to poliomyelitis . It was introduced around 1 95 0 and was increasingly used for idiopathic scoliosis too . The Milwaukee corset is the prototype of active corsets . The rigid head-holder creates a continuous stimulus to make autocorrective movements; if the pa-
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Fig. 24.26.
259
T o act o n the entire tru nk so a s to achieve both cosmetic (rib hump, and shoulder equalization) and functional (breathing function) improvement.
Boston corset.
Milwaukee corset.
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Fig. 24.28. Cheneau corset.
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tient relaxes the trunk muscles, the head bumps against the head-holder. The Milwaukee corset is used mainly to treat infantile and juvenile scoliosis under 40°, since it does not affect the normal devel opment of the chest and does not harm the breathing function. It is recommended in ado lescent scoliosis if the apex is above vertebra Th6. The Boston corset was created by John Hall in 1 97 1 . It is posterior-opened, with one layer, and is symmetrical in the sagittal plane; it decreases lordosis (Fig. 24.27.). This type of corset is used in thoracolumbar and lumbar curves where the apex is below vertebra Th l O . The Cheneau corset (the official name i s CTM Cheneau, Toulouse and Munster, where it was first introduced) is suitable for the treatment of any curve under 40° (Fig. 24.28.). The Charleston corset is used at night; it overcorrects the spine into a convex direction. It is indicated in highly mobile curves under 25°. Similarly, the Spine Cor dynamic corset i s recommended i n highly mobile curves under 20° and it uses elastic straps for correction. In tensive exercises are necessary. Corsets are used for 23 hours daily, and re moved only at the time of washing. Exercises are a necessity. The aim of loosening exercises is to de crease the rigidity of the curve and of the rib hump and to create dorsal kyphosis : =
- Breathing exercises : These increase the vital capacity and the mobility of the ribs, and promote the symmetric development of the chest. - Overall upper trunk correction: The patient must be aware of postural ab normalities and practice auto-correction in order to create and actively protect the physiologic curves. - Elimination of the disadvantages of the corset. General muscle training, kypho sis and breathing exercises. - Psychological support for the child.
The value of physiotherapy is outstanding in conservative treatment; no other method can replace it. An alternative is regular swim ming, since the forces of gravity are then less ened and the muscles of the trunk and belly are trained symmetrically. Exemption from school exercises is not justified. �
Indication and stages of surgical treatment The first scoliosis operation was carried out by Jules Guerin in 1 839 . He suspected that the condition was of muscular origin and performed percutaneous myotomy of the paravertebral muscles. The first fusion was done by Russel Hibbs in 1 9 1 4 . His procedure and its modifications were the accepted therapeutic methods for about 5 0 years (Fig. 24.29.). After loosening therapy for 1 year, in situ spondylodesis followed without further cor rection. To protect the result, external fixation was used until bony consolidation was achieved ( 1 year). The outcome of this 2-year-Iong procedure was the prevention of progression. No correction was accom plished. The first real breakthrough was due to Paul Harrington, who reported his procedure for Heine-Medin scoliosis in 1 962. His opera tion (Fig. 24.30.) has become the gold stan dard for the treatment of spinal deformities and especialJy scoliosis. He set the basic for all operative corrections with his instrumenta tion. After 1 -2 months of active preparation with traction, the curves are corrected surgi cally with distraction hooks inserted into the end-vertebrae on the convex side. The correc tion may be enhanced by using compression on the concave side. The postoperative treat ment is aimed at protecting the correction achieved in the operation: 6-8 weeks of bed rest in a plaster bed and a corset for 1 year. The average correction achieved is 40-45%. The greatest problem was the neurological complications (6-8%) originating from the distraction of the spine; moreover this device permitted only a 2D correction, and thus flat back frequently occurred.
24. D i s e a s e s of t h e s p i n e
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Fig. 24.29. Bony fusion without an implant in Barta's mod ification principle of the operation in drawing (a). The consid erable cu rve (b) could be red uced by prolonged traction (c). Fusion was then achieved done in situ without an implant and with a bone graft (d). I n spite of the fusion and the lengthy conservative treatment, signifi cant scoliosis may still be observed 10 years after the operation (e).
Yves Cotrel and Jean Dubousset, moved a great step forward by introducing a multi segmental corrective system in 1 98 3 , which furnished completely new possibilities for the treatment of spinal deformities. The basic fea-
ture in the Cotrel-Dubousset 3D corrective technique is the correction of rotation, not only in the horizontal, but also in the frontal and sagittal planes (Fig 24.3 1 .) . This can be achieved in a single procedure.
b
c
d
Fig. 24.30. The essence and the result of the Harrington operation (a). Preoperative (b) and postoperative a-p (c) and lateral (d) radiographs.
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Three-dimensional (3D) correction of scoliosis. Frontal, sagittal and horizontal correction of scoliosis in a single proced ure. Through use of the lever arm principle, the sagittal correction can be increased.
Fig. 24.32. Correction of scoliosis with a double cu rve in a 14-year-old girl. 54° dorsal right convex and 52° lumbar left convex dou bled cu rved (a) scoliosis corrected in the dorsal segment to 10°, and in the lumbar segment to 4° (b). The preoperative irregular sagitta l curves (c) are seen to be corrected postoperatively (d).
24. D i s e a s e s of t h e s p i n e
During the operation, the strategic points of the curves are determined and fixed with staples. A corrective rod, bent do as to con form to the expected postoperative curves, is then inserted beside the spine and rotated by 90°, so that its curves turn into the sagittal plane and the scoliosis is corrected. Using the principles of the lever, the thoracic distraction further corrects the dorsal kyphosis, while the lumbar distraction further corrects the lor dosis. Derotation decreases the rib hump by changing the position of the costotransversal joints and ribs, indirectly proving the 3D cor rection. The Cotrel-Dubousse 3D correction nor mally does not require preoperative loosening treatment. In the event of a normal bone struc ture, the rods inserted on both sides of the spine provide such a stabile fixation, that postoperative external fixation is unneces sary, the patients are out of bed on the second day and can resume school after 2 weeks (Fig. 24.32.) .
Indications of operation: - A Curve exceeding lumbar 30°; in other localizations, 40° - More than 1 0° progression yearly. - Risser's stages 4-5 . Ifthe indication is well based, but the bony age has not reached Risser 's stages 3 -4, ven tral corpodesis is performed. This is also the recommended method at any age for curves exceeding 90°. In consequence of the extensive 3D cor rection during surgery, paralysis of the lower limbs may occur because of cord or root in jury. There are two ways to avoid this during the operation: to wake up the patient tempo rarily at the time of correction in order to check on the movement of the lower limbs, or to check the perioperative evoked electric po tential. In cases of motor abnormalities, the correction must be decreased. The re transfusion of peri- and postoperative maj or blood loss by autotransfusion (Cell-saver, Solcotrans) may decrease the complications . Antibiotic is necessary to prevent infection.
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24.3.2.2.2. Scoliosis of kno wn etiology
Here only those forms of scoliosis are in cluded which may be important for a general knowledge of medicine. Spinal deformities are frequent symptoms of various congenital syndromes, and teratogenic and inherited dis eases (Marfan' s syndrome, general joint lax ity, neurofibromatosis, infantile cerebral pa resis, myelodysplasia and certain mucopoly saccharidoses). About 70 multiple develop mental anomalies are known where scoliosis may be one of the signs. �
Congenital scoliosis
Congenital scoliosis can occur as a conse quence of growth asymmetry caused by a congenital developmental anomaly. The word congenital is slightly inappropriate, since at the birth only the vertebral developmental anoma ly is definitely present; scoliosis is not a lways seen.
Incidence. The exact incidence is not known, but it is presumed to be relatively rare. The occurrence of known wedge vertebrae in the dorsal spine is 0,05 %. , but it is probable that the true occurrence is around 1 %. Etiopathology. The cause of this spinal deformity is known: growth asymmetry con sequent to a vertebral developmental error. The reason for the vertebral developmental shortcoming is unknown; it takes place be tween weeks 3 and 6 of pregnancy as a result of some unknown noxa affecting the embryo. The development of the central nervous sys tem, urogenital organs and heart also starts in this period, and these systems too may be in volved. The main types of vertebral developmen tal anomalies are the disturbances of segmen tation and formation. These two disturbances are sometimes combined (Fig. 24.33.). The disturbances of segmentation may be uni- or bilateral. Defective unilateral segmentation results in a unilateral block vertebra. On the side of the bony union the vertebra does not
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a
b
c
d
e
g
Fig. 24.33. Types of wedge vertebrae. Segmentation disturbance: a: Unilateral block vertebra. b: Bilateral block vertebra. Formation distu rbance: c: Total wedge impression. d: Incarcerated wedge vertebra. e: Partially formed wedge vertebra. f: Slight wedge vertebra. g: Combined segmentation and formation disturbance.
grow, whereas on the contralateral side the growth is normal . Depending on the number of segments involved, maj or curves may evolve, finally reaching 50-60°. Bilateral seg mentation disturbances result in a complete block vertebra, leading to a symmetric loss of growth, but no curve. Formation disturbances produce wedge vertebrae. These may be fully developed wedge vertebra (normal disc is present below and over the wedge vertebra), incarcerated wedge vertebrae (an atrophic disc is present both below and above the wedge vertebra), or partially developed and insignificant wedge vertebrae. The growth potential below and above the fully developed wedge vertebra is normal, so the expected growth on the convex side is double relative to the concave side. This may result in a severe curve (50-70°) by the end of growth. Incarcerated wedge vertebrae do not pro duce such severe curves, since the growth po tential of the atrophic discs is less. The partially developed wedge vertebrae do not give rise to a maj or curve worsening, since a single normal disc is located below or above with a normal growth potential. The in significant wedge vertebrae are not divided from the normal vertebrae by discs.
The worst prognosis is expected in cases of combined developmental anomalies, if a unilateral loss of segmentation is associated with contralateral wedge vertebrae even in multiple segments. This may lead to a 1 00° curve. Clinical symptoms. Any segment may be involved. The most common sites are in the lumbar and cervical parts . Depending on the number and location of the segments, huge curves may develop, or the state may be fully compensated. In the thoracic segment rib synostosis may accompany the spinal changes, but this does not influence the clini cal outcome. Therapy. The treatment of congenital anomalies is surgical. Conservative therapy is only justified only in exceptional cases and in order to gain time. In cases of unilateral block vertebra, contralateral fusion is to be performed at the time of the diagnosis because of the expected rapid progression. Cases of complete block vertebrae need only observation. It is most important to decide whether fully-developed or incarcerated wedge verte brae are present. Fully-developed wedge ver tebrae demand surgical excision because of the expected rapid progression (Fig. 24.34.). In cases of incarcerated wedge vertebrae, sur-
24. D i s e a s e s of t h e s p i n e
gical intervention must be considered, de pending on the growth potential. The worst prognosis is to be expected in cases of com bined developmental anomalies. Each devel opmental anomaly most be evaluated individ ually. �
Neuromuscular scoliosis
Neuromuscu lar deformities occur, when the balance between the active and passive stabi lizing elements of the spine is disturbed.
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The occurrence and progression of the de formities are similar in this group, despite the diverse etiology. Neurological or central and muscular or peripheral subgroups are to be differentiated. The neurological subgroup, which involves damage to the central nervous system, is subdivided into conditions associ ated with upper or lower motor neuron dam age. The following abnormalities can result from upper motor neuron damage : ICP, syringomyelia, myelomeningocele and spinal cord tumors. Lower motor neuron damage causes are present in (Heine-Medin 's) polio myelitis and spinal muscular atrophy. b
a
c
d
e
f
Fig. 24.34. Wedge vertebra in an 8-year-old girl. ThlO wedge vertebra (a) and 3D CT reconstruction (b). After removal of the wedge vertebra (c) segment fusion (d). The spine in saggittal plane before (e) and after (f) the correction.
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The muscular o r peripheral subgroup com prises the deformities due to arthrogryposis or muscle dystrophies (e.g. progressive muscle dystrophy) . These patients are often unable to walk and are wheelchair-bound. Both groups are characterized by C-shaped thoracolumbar deformities affect ing the sacrum, which start at an early age and progresses rapidly, ending up involving the entire spine. Their progression does not cease at the end of growth, which is a marked differ ence from idiopathic scoliosis. Deformities of central origin are large arched, very rigid curves. The curves related to peripheral factors are also large-arched, but remain very mobile for a prolonged period. "Collapsing spine" frequently develops, which is difficult to manage despite the mo bile spine. The management of neuromuscular defor mities is far from simple. The aim of conser vative treatment is to ensure normal sitting,
a
b
and additionally to control the curve, in an at tempt to delay the surgical intervention, which is often unavoidable. The most important aim of surgery is to form a balanced, stable spine which will re tain its stability in spite of the missing active elements. If the basic condition creates an in creased muscle tone, a corset is needed post operatively for I year. In paralytic cases, a postoperative corset must also be considered, which may help the patient regain balance. �
Neurofibromatosis Neurofibromatosis is an autosomal domi nant progressive disease involving the ecto-, meso- and endodermic tissues. Its incidence of 1 in 3 000 is the same in the two genders with a constantly high penetrance, but with different expressivities. Genetically, there are at least 4 types. 90% of the cases are classified into the classical (van Recklinghausen 's) group. Typically, subcutaneous neurofibro-
c
d
Fig. 24.35. Neu rofibromatosis in a 13-year-old girl. 108° dystrophic scoliosis (a) and 72° kyphosis (c), corrected surgically to 35° (b) and 44° (d), respectively.
2 4. D i s e a s e s of t h e s p i n e
mas, pigmented skin lesions (cafe au lait spots) and pigmented iris haematomas (Lisch 's nodules) are detected. Besides the neurocutaneous manifesta tions in neurofibromatosis, skeletal changes may occur in 1 0-50% of the cases, producing mainly spinal deformities. The deformities vary. On the basis of the bone dystrophy, they are divided into two groups, differing in prog nosis and therapy. The nondystrophic curves are very similar to those in idiopathic sco liosis, so their management is identical. The dystrophic curves are short, involving 4-6 ver tebrae, and are extremely severe. Apart from dystrophic bone changes (hypoplastic and/or missing pedicles, tiny spinous, transverse pro cesses and excavated bodies), they are accom panied by severe kyphosis. The extensive early lesions progress irresistibly, leading to cardiorespiratory and neurological symp toms, despite the wide spinal canal. The treat ment of these deformities can be effective only, ifthe early aggressive surgical treatment is commenced in multiple steps, with ventral corpodesis and posterior corrective spondylo desis (Fig. 24.35.). A corset is contraindicated in this condition. �
Mesenchymal diseases / Mar/an 's syndrome Marfan 's syndrome is an autosomal domi nant disease with differences in expressivity and high variability. It is caused by qualitative and quantitative changes in fibrillin, a glyco protein component of the microfibrillary fi bers. While the connective tissue is involved in the entire body, the main clinical signs are present in the eye, and cardiovascular and skeletal systems (Chapter 1 3). The incidence of spinal deformities is high (40-80). Scoliosis is most often observed, but other changes (spondylolysis, spondylo listhesis, spina bifida and facet subluxation) are also possible. Mar/an 's scoliosis differs from the idio pathic form. The gender distribution is equal. Infantile and j uvenile forms are frequent. Rapidly progressing double curves most often appear (right convex dorsal and left convex
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lumbar), with severe rigid deformities. The physiological curves are frequently inverted, causing severe breathing failure. Deformities in Mar/an 's syndrome should be treated with early combined surgery: ven tral release and corpodesis and posterior cor rective spondylodesis. Conservative treat ment is justified only if surgery is contraindi cated. �
Metabolic diseases / Rickets scoliosis Rickets scoliosis is a spinal deformity in rickets and osteomalacia due to a disturbance of the vitamin D supply, absorption or metab olism (see Chapter 1 4) . The developing bones lose their solid structure, and various other spinal skeletal deformities may arise: caput quadratum, craniotabes, Harrison groove, 'rickets rosary' rib changes, sword-sheath tibia, etc. The spinal deformities first appear as lower dorsal and lumbar kyphosis (sitting hump). Scoliosis follows. The earlier the rick ets starts, the more severe, short and rigid the curve produced. The trunk becomes shorter due to compression of the vertebral bodies. In severe cases, the disproportion between the trunk and the limbs is noticeable. Com pression of the anterior or lateral elements of the vertebrae damages the growth plates and the ossifying centers , so the curve therefore progresses. Rickets due to a vitamin D deficiency is currently rare; it can be cured by means of vi tamin D treatment before the deformities de velop.
24.4. Congenital abnormalities 24.4. 1. Spondylolysis, spondylolisthesis I n spondylolysis the thinnest part of the ver tebral arch, the interarticular part, exhibits a uni- or bilateral division. In the event of bilat eral interarticular lysis, the involved vertebra is divided into two parts. The frontal part com prises the vertebral body, the adjacent parts of the arch, the transverse processes and the
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Fig. 24.36. Spondylolysis i n pars interarticularis.
superior articular processes. The posterior part contains the posterior a rticular processes, the spinous p rocesses and the corresponding part of the arch (Fig. 24.36.).
bral arches, creating interarticular lysis. This theory is supported by only a few data. Due to the erect posture, the interarticular part is exposed to an increased load and is overloaded, and this is further aggravated by possible hyperlordosis. This may explain why Scheuermann' s disease is accompanied by spondylolysis and spondylolisthesis in 50% of the cases. Again due to the erect posture, the interarticular part may be secondarily overloaded and, because of the repeated microtraumas, a fatigue fracture may occur, causing spondylolysis and spondylolisthesis. Repeated microtraumas occur in individu als who repeat extreme movements that affect their lumbar spine (extreme flexion - exten sion) : competitive gymnasts, kayak - canoe competitors and athletes. The disease was classified be Wiltse into 5 types (Fig. 24.37.) : �
Spondylolisthesis may occur, when a vertebra l body (together with th e spinal segment above it) slips forward on the vertebra below. The most common cause of spondylolisthesis is bilateral spondylolysis. Rarely, the slip may take place backward direction; this is cal led retrolisthesis.
Incidence. The incidence of spondylolysis and spondylolisthesis varies in different geo graphic areas. The incidence in Europe is 6%, among Eskimos it is 26-27%, and in certain isolated ethnic groups it may even reach 40%. The incidence also varies with the age. In Eu ropean newboms, spondylolysis is not de tected, whereas the incidence at the age of 6 is 5%, and by the end of growth it attains the fi naI 6%. Etiopathology. Many theories have been put forward to explain the development of spondylolysis and spondylolisthesis. In the oldest theory, the tension exerted on the interarticular parts because of a developmen tal anomaly obstructs the normal ossification from the double ossifying centers of the verte-
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Dysplastic type: A multiple develop mental anomaly of the lumbosacral seg ment is present as a predisposing factor. The spondylolysis develops as a con sequence of the insufficient development of the proximal part of sacrum (cupola formed, rounded, hypoplastic base of the sacrum), and of the inferior articular processes of vertebra L5, promoted by the enhanced pelvic incidence accompanied by the increased sacral tilt (sacrum acutum) . Besides the dysplastic inferior articular processes of vertebra L5, occult spina bifida is often detected. This type is more common in girls and is detected around the age of 6. Isthmic type: This is the most common type of spondylolisthesis and can be further divided into 3 subtypes. The lytic subtype is a fatigue fracture of the inter articular parts. Elongation of the inter articular part without lysis may also occur. The third subtype involves an acute fracture of the interarticular part. The isthmic type is more common in males, and has a worse prognosis in females with a greater likelihood of progressing. The isthmic type often causes lumbar in-
24. D i s e a s e s of t h e s p i n e
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stability. The earlier the deformity occurs, the more severe the clinical symptoms are. Degenerative type: This deformity is usually a limited slip, observed as a consequence of chronic segmental in stability due to disc degeneration (spon dylosis) and facet joint changes (spondyl arthrosis). Retrolisthesis is also frequent. It is fairly common in women over 50 . Traumatic type: Spondylolisthesis i s caused b y a vertebral fracture a t some other site than the interarticular part. It is rare.
1
2
3
5
4
6
7
Fig. 24.37. Types of spondylolisthesis. 1: dysplastic, 2: degenerative, 3: trau matic, 4: pat ho logic, 5: pars interarticu lar lysis, 6: elongated, but intact pars interarticular, 7: trau matic pars inter articular fracture.
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Pathologic type: This is due to weakening of the interarticular part in localized (tu mor) and/or generalized (osteoporosis or inflammation) bone diseases.
Localization. Spondylolysis and spon dylolisthesis may occur at any age and affect any spinal segment. Most often (80%), L5 slips on the sacrum. L4 slips occur in 1 5% of the cases. Slips at L3 and L2 have a frequency less than 1 %. In the other segments, the rate of occurrence of spondylolisthesis is around 5%. Clinical symptoms. Spondylolysis and spondylolisthesis often present without symp toms . The symptoms mainly occur in child hood and adolescence because of the changes in the dynamics of the lumbar spine, resulting in segmental instability, and present as acute or chronic lumbago. The most typical symp tom is low back pain after long standing or af ter extensive flexion - extension of the lumbar spine; this pain increasing in response to physical activity. The pain is not radicular pain, but often radiates to the buttock or the thigh. The paravertebral muscles, the hip ex tensors and the hamstrings are very tight, re sulting in a special gait with short steps, with externally rotated legs, as a consequence of the limited hip flexion. Neurological signs, radicular pain or signs, and reflex changes de velop only in cases of excessive slip or disc herniation. Pseudoradicular pain may occur regularly. Clinically, increased lumbar lordosis is found at the location of slip step formation. The spinous processes of the involved verte brae and the paravertebral area may be tender. In one-third of the symptomatic cases, minor lumbar scoliosis may be present. The symptoms may worsen with age, but the radiological signs do not demonstrate close correlation. The patients do not tolerate load-bearing well : a sudden, chiefly rotational motion may produce heavy pain. This may be related to the segmental instability. X-ray signs. The anteroposterior and lat eral views are not always sufficient. The
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Fig. 24.39. Dittmar's view of spondylolysis.
Fig. 24.38. Severe spondylolisthesis: the picture resembles a re verse Napoleon hat.
anteroposterior film shows the inverted Napo leon ' s hat projection of the vertebrae only if the slip is severe (Fig. 24.38.). Other develop mental anomalies are often seen, e.g. spina bifida. The lateral view unequivocally reveals the slip (spondylolisthesis) and tearing of the interarticular part is often seen. The increase in the axis of the sacral slope induces hyper lordosis, and a hypoplastic, rounded sacrum base is frequent. The region of the interarticular part and the tear is observed in oblique, Dittmar ' s views (Fig. 24.39.). The borders of the tear may be irregular, with a relatively narrow gap indica tive of a fresh lesion. When the defect has per sisted for a long time, the gap is wider, and the edges are rounded and sclerotic, proving the presence of pseudoarthrosis. To establish the extent of slip, Mayerding 's scale is used, in which the sacral plateau is divided into 4 equal parts. The dis placement in grade I is 0-25%, in grade 11 26-50%, in grade III 5 1 -75% and in grade IV 76- 1 00%. In the most serious cases, the
slipped vertebra is located in the pelvis in front of the vertebra below it, this is called spondyloptosis (Fig. 24.40.). Treatment. Spondylolysis and non progressive insignificant spondylolisthesis are often symptom less and do not require treatment. Spondylolysis cases discovered in
Fig. 24.40. Spondyloptosis, lateral view.
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a
b
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c
Fig. 24.41. Isthmic reconstruction (Jakab's screw). a: Preoperative X-ray; the lysis is clearly visible. b: Postoperatively after the insertion of screws. c: Screws i n oblique view.
children must be followed up clinically and, depending on the individual findings, X-ray images are taken to check on the progression of the slip. The complaints present as instability-in duced lumbago. The treatment is mainly con servative. Nonsteroidal anti-inflammatory drugs and muscle relaxants are prescribed and, following a temporary reduction in phys ical activity, back and belly muscle exercises are encouraged. Physiotherapy may also be employed. Physical education at school is al lowed, but overloading and heavy spinal flexion - extension movements should be avoided. The treatment is naturally dependent on the extent of the spondylolisthesis. In cases in which there is only a minor slip, conservative methods predominate with the use of an antilordotic corset. This device decreases the lordosis and possibly the angle of the sacral slope. If the complaints are not alleviated by con servative methods, the slip progresses; if the slip is extensive (grades Ill-IV), surgical treat ment must be contemplated. The progression
of spondylolisthesis is likely in early adoles cence, in women, in dysplastic slips, in grade Ill-IV cases and when there is a high sacral slope (sacrum acutum) . The essence of surgical treatment is to eliminate the instability induced by the spondylolysis and spondylolisthesis. Reduc tion of the slip is not attempted. In mild slips, isthmic reconstruction is em ployed: direct compressive arch synthesis is achieved with a malleolar screw introduced through the lysis of the interarticular part, fol lowing resection of the pseudoarthrosis (Fig. 24.4 1 .) . Instability may also be cured by in situ fu sion ( Wiltse 's method) without internal fixa tion. Fusion between the sacrum and the arches and transverse processes of vertebrae L4-5 is performed with bone grafts taken from the iliac crest. The patient is mobilized in a corset 3 weeks after the operation. Bony fu sion is expected in 3-6 months. Fusion can also be effected with implants. The vertebrae involved are fixed with trans pedicular screws and rods; stable fixation en hances bony fusion (Fig. 24.42.).
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a
b
Fig. 24.42. Spondylolisthesis, segment fixation. a: Preoperative X-ray. b: Postoperative X- ray.
On use of the implant, reduction of the spondylolisthesis is possible, but this is not the primary aim of the operation. When the spondylolisthesis is to be reduced, the poste rior arch of the reduced vertebrae must be re moved in order to avoid nerve compression. In cases of posterior lumbar interbody fu sion, discectomy is carried out from a poste rior approach, retracting the cauda equina, and interbody fusion is accomplished with a bone block(s) inserted in place of the disc.
24.4.2. Sacralization, lumbarization The lumbosacral region, called the "rest less point" of the spine, is the most frequent site of developmental abnormalities. In sacralization, the transverse processes of vertebra L5 are longer and wider, like but terfly wings, and the vertebra is partially or completely fused with the sacrum. Bilateral, symmetric fusion is usually symptomless. However, due to the uneven load-bearing uni-
Fig. 24.43.
Sacralization. The enlarged transverse L5 process touches the base of the sacru m on the right.
24 . D i s e a s e s of t h e s p i n e
lateral change, more often leads to spondy losis in the upper levels and complaints. In partial sacralization, the common cause of the pain is the neoarthrosis created between the enlarged and extended transverse process and the sacrum lateral mass (Fig. 24.43.). Lumbarization is present when the first sa cral segment is not ossified with the other parts of the sacrum but remains partially or fully separated; its appearance is similar to that of the vertebra L5 (Fig. 24.44.). The con dition may be symptomatic because of the intersacral neoarthrosis or the improper load ing that induced lower lumbar spondylosis. Since this defect may be symptomless for de cades, it is not generally agreed whether the symptoms are caused by the lumbarization or by other factors .
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Clinical symptoms. In both abnormali ties, the complaints usually appear gradually as lumbago in middle age. The pain is boosted by physical activity. No neurological signs are detected. Typical lumbago may be present. The paramedian points are usually tender, mostly unilaterally. In sacralization, local ten derness is felt uni- or bilaterally over the at tachment of the transverse process and iliac crest (5-6 cm laterally from the midline. This pain increases during bending to the involved side and on rotation of the trunk (Baastrup ' s sign). Therapy. In most cases, conservative treatment is needed, with temporary rest, nonsteroidal anti-inflammatory drugs, muscle relaxants and pain killers. The complaints are improved by physiotherapy too. As soon as the pain has subsided, regular exercises are recommended. Temporary use of a lumbar corset may alleviate the complaints. In the complaints are stubborn and conservative treatment fails, surgery must be considered. In sacralization causing neoarthrosis, an en larged transverse process may be removed. In other cases posterolateral fusion (spondylo des is) is recommended, with or without inter nal fixation.
24.4.3. Occult spina bifida The relatively common developmental anomaly is generally discovered accidentally. It usually appears on vertebra L5 or S I . On S I , this anomaly has no clinical effect, since it can not disturb the stability of the firm sacral bone. Occult spina bifida on L5 is generally presumed to exert any clinical effect either, but some claim that any variation or weaken ing of the static ally overloaded vertebra L5 may lead to complaints. Conservative care is justified. If this fails, the cause of the back pain may be different (see myelodysplasia). Fig. 24.44. Lumbarization
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24.5.
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Other diseases
24.5. 1. Baastrup syndrome (interspinous arthrosis) The spinous processes are in contact with each other, mostly in the lumbar section, and the development of neoarthros leads to arthrosis. The pain sets in on rotation and rec lination of the spine. The secondary form may occur in spondylosis (see lumbar spondylo sis). It reacts well to conservative treatment; the tip of the spinous processes may be trimmed in some cases.
24.5.2. Sacrum acutu m Opinions concerning to this entity differ. Sacrum acutum is present if the slope axis of the sacrum greatly increases, and it becomes almost horizontal. The cause is presumably the chronic and increased tension of the liga ments and of the disc at the lumbosacral level. This deformity may accompany other defor mities (see spondylolisthesis dysplastic form). The treatment is conservative. In the pain-free period, exercises are advocated with a view to decreasing the lumbar lordosis.
24.5.3. Coccygodynia This is a condition involving coccygeal pain, which may originate in various ways: �
�
While very thin individuals are sitting, the prominent coccyx may chronically irritate the skin and periosteum. A fall onto the buttocks may result in local contusion and periosteal irritation, or ventral dislocation may take place if a liga ment is tom in the sacrococcygeal j oint.
Clinical symptoms. Pain felt in the sacro coccygeal j oint, increases during sitting. There is often some direct trauma in the his tory. Defecation sometimes is extremely pain ful. Lying and walking do not cause is symp toms .
Examination reveals tenderness and pain in the sacrococcygeal joint. At times, the pain increases on passive movement ofthe coccyx. The X-ray picture sometimes indicates a fracture or ventral displacement of the coc cyx. Most often, however, the X-ray result is negative. Its main use is to exclude other pa thologies (tumor or infection) . Rectal exami nation is advised in every case. Treatment. The complaints frequently cease spontaneously or following conserva tive treatment. Individuals are often recom mended to use a ring cushion. Local physio therapy, nonsteroidal anti-inflammatory drugs, and a lidocaine - local steroid injection may succeed. In stubborn cases, surgical re moval of the coccyx may be the choice.
24.6. Degenerative disorders of the spine The importance of degenerative disorders of the spine in medical practice is due to two important and typical facts : 1 . Either acute or chronic spinal pain may lead to a disability, and impair the quality of life. 2. The risk of damage to the spinal chord and radices, be cause of their special anatomical characteris tics in spinal diseases. The common occur rence of pain and neural signs urges both pa tient and doctor to seek the effective and suc cessful treatment of these conditions. As far as the epidemiological significance is concerned, in the USA in 2002 spinal con ditions were listed as the third most common among the conditions that require health care. As regards health care expenses, they have been in a lending position for years. Modem spinal surgery combines orthope dic and neurosurgical techniques, but also makes use of the instrumentation of general, thoracic, vascular and plastic surgery. It is generally available in well-organized opera tive centers . The characteristic features in clude the extensive use of implants (metal or plastic devices), advanced medical devices (special motors, computer-guided operative
24. D i s e a s e s of t h e s p i n e
tools, endoscopes, lasers, neurostimulators, etc.) and products of the biotechnological in dustry (produced from natural or artificial sources and tissue culture).
24.6. 1. Degenerative spinal disorders The spine sustains structural changes in the course of aging. These changes progress at a segmental level, and the sum of the changes of the individual segments leads to the typical degenerative deformation of certain segments and ultimately of the entire spine. 24.6. 1 . 1 . Degenerative p rocess
of the disc The lumbar disc in healthy young adults, with its well-hydrated (physically "perfect gel") nucleus pulposus, the densely colla gen-fibered annulus fibrosus and the well-de fined cartilage-covered end-plates, fulfils its purpose from every aspect, providing an elas tic and firm junction between the vertebrae, which react properly to the axial load (mainly
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gravitation). At the end of the third decade, fine, concentric (sometimes radial) fissures appear in the lamellar system of the annulus, indicating that the nucleus pulposus can no longer distribute the circular pressure prop erly. The "perfect gel" of the nucleus pulpous is gradually dehydrated, and during the fourth decade the water loss is significant. As the age progresses the nucleus - annulus border be comes ill-defined, and the inner rings of the annulus loosen because of the numerous ra dial fissures. If the fissures between the lamellae reach the outer rings, fibrous tissue proliferation starts in the center of the disc. This scar tissue fills the interlamellar space and the entire nucleus pulposus, and nerve ter minals are introduced. The loosening of the lamellar system of the annulus fibrosus elicits changes in the me chanical properties of the disc. During flexion - extension and rotation, the tension of the lamellar system decreases, its motion-limiting role also decreasing. The dehydrated nucleus pulposus and the annular system with its im paired elasticity are decisive factors contrib uting to a loss in height of intervertebral space. This loss has two consequences: 1 . the relative tension of the longitudinal ligaments diminishes, and 2. the j oints of the segment exhibit first hypermobility, and then sub luxation (Fig. 24.45.). 24.6. 1.2. Disc prolapse, hernia If the elements of the disc are detached from their original position and invade the spinal canal, the resu lt is a herniated disc. Depending on the relation with the spinal anatomy, cen tral, mediolateral, foraminal and extraforaminal hernias may be defined (Fig. 24.46.).
Fig. 24.45. Degenerative process of the movement segment. a: Osteophyte formation (arthrosis). b: Osteophyte
If a piece tears off the disc completely, the process is called sequestration. If the pieces of the destroyed lamellar system do not enter the canal, but press against the posterior longitu dinal ligament and narrow the canal, it is called protrusion.
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a
tures of the segment, and by the movement limiting role of the uncinate processes. Spinal cord compression may result in an irreversible loss of function within a few hours. Fortunately, this complication is rare. From a practical medical aspect, lumbar hernias play a prominent role. Their occur rence in the individual lumbar segments var ies considerably. The most common sites af fected are the lowermost two segments above the sacrum: herniation of discs L4-5 accounts for 60-0% of all lumbar hernias. Herniation above L3 is very rare.
24.6. 1.3. Degenerative spine instability
b
Fig. 24.46. Herniated disc types by anatomical localization. a: Central hernia, b: Mediolateral hernia
A herniated disc (either protruded or se questrated) is regarded as a cause of compres SlOn. This condition is accompanied by pain, movement restriction and occasional neuro logical signs. The sequestrum first elicits in flammation of the perineurium (edematous thickening), and may then may disturb the neural function. The sensory quality may change, the motor function of the muscles may diminish, and the reflexes may change. At the same time, the short interspinal mus cles may suffer a reflex (lasting) spasm, seem ingly to protect the segment from movement. Herniated discs in the neck and back may cause symptoms by direct compression of the spinal cord. Herniated discs in the dorsal seg ment are rare (this segment has limited mobil ity) . The modest symptoms in the cervical segment are explained by the special struc-
Due to the structural changes caused by degenerative processes, the movement of a segment may exceed the physiological limits. This is called instability. Segmental instabil ity is usually chronic (disc degeneration), but may appear in acute form (herniated disc or, vertebral fracture). Segmental instability activates the resta bilizing mechanisms of the body. As a result of these processes, canal stenosis, and irrita tion or compression of neural components may arise, causing clinical symptoms. Types of lumbar spinal stenosis (by Arnoldi) 1. Congenital developmental anomalies 1.1. Idiopathic 1.2. Known etiology (achondroplasia, etc.) 2. Acquired types 2. 1. Degenerative 2.2. Combined degenerative and congenital 2.3. Spondylolysis 2.4. Iatrogenic 2.5. Post-traumatic 2.6. Others (metabolic, tumor, etc.) �
Degenerative spinal stenosis By the time of skeletal maturity, the sagittal diameter of the bony canal has at-
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tained its final size (Fig. 24.47. a). This is the distance from the posterior wall of the verte bra to the lamina. This distance is permanent and never changes. This is the anatomic diam eter. The functional diameter is the distance between the posterior wall of the intervertebral disc and the ligament flavum system (Fig. 24.47. a). This length naturally changes during movement: it increases in flexion (kyphosis) and decreases in extension (lordosis). The degenerative processes pro duce excessive tissue mass (protrusion, liga ment thickening, etc.) at the height of the disc, resulting in a decrease in the functional diam eter (Fig. 24.47 b). The degeneration of intervertebral discs progresses without any clinical symptoms, until the spatial demands of the intraspinal neurogenic elements begin to inhibit the phys iologic function.
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If a functional block vertebra is created at the end of the process in such a way that both the dural sac in the center of the spinal canal and the nerve root laterally can fulfil their tasks, then the process ends without symp toms . However, in the course of degeneration, disproportion can occur in any phase between the spatial demands of the neurogenic ele ments and the space narrowing induced by the process. According to the modem approach, a her niated disc is considered to be a special form of degenerative spinal stenosis, where the nar rowing of the spinal canal occurs relatively rapidly, and the symptoms are therefore ac centuated.
24.6. 1.4. Clinical exam ination of
degenerative spinal d isorders
a b
Fig. 24.47. a: Normal physiological diameters of the spinal canal (1. anatomical diameter, 2. functional diameter ) b: Fu nctional diameter of the spinal canal (3) spinal stenosis.
Pain of spinal origin. In the examination of a spinal patient, the first step is the thorough recording of the his tory. One of the most important points is to verify the circumstances of the onset of the pam. Besides its onset and its duration, spinal (and low back) pain is characterized by its lo calization and radiation. Low back pain is called local if it is present in the middle or lat eral parts of the lumbar spine, including the lumbosacral area and the sacroiliac j oints. The radiating pain starts in this area and involves one or both lower limbs. In the assessment of local pain, tenderness and pain are sought over the spinous pro cesses in the median or paramedian line and over the path of the sciatic nerve. Valleix' s points are located between the buttock and the greater trochanter, where the nerve leaves the pelvis; in the middle third of the thigh posteri orly; and behind the fibula head correspond ing to the peroneal nerve. Tenderness at these points is an indication of root irritation, but these are not genuine radicular signs. The sa croiliac j oints, the sites of origin of the gluteal muscles, may also be tender.
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The pain syndromes involving the neck, shoulder girdle and one or two upper limbs are referred to as cervicobrachialgia. Lumbago is a Latin word meaning low back pain. If it is accompanied by radiating pain to one or both lower limbs, it is called lumboischialgia. One of the most frequent reasons why the patient consults the doctor is low back pain. Three types may be distinguished on the basis of the etiology: - Vertebrogenic - Extravertebral, somatic - Extravertebral, psychological low back pam. Vertebrogenic low back pain is most often a symptom of a degenerative process. It inten sifies in response to physical loading (e.g. a standing posture), and is alleviated at rest (an unloaded state). It is important if the patient is disturbed by pain while sleeping. Pain in creasing at night excludes a degenerative ori gin, and inflammation or tumor is possible. This is particularly the case when the pain is accompanied by general symptoms (fever, malaise, loss of weight, etc.), and conven tional pain-killing methods are not effective. If local pain is accompanied by radiating pain, an accurate assessment can provide im portant information. If the radiation corre sponds to any dermatome, typical radicular pain is present, and symptoms of ·nerve in flammation are found. (Such patients have a typical sign, functional scoliosis, the so called "antalgic posture".) Radiculitis is caused most often by some form of spinal stenosis, and if radicular pain is accompanied by sensory or motor loss signs, there is usualJy a herniated disc in the background. Extravertebral, somatic low back pain is usually caused by some abdominal problem, e.g. a nephrolith, pyelitis, a renal tumor, or in flammation of the gall bladder, colon or ova ries. Among women, low back pain is very frequent during the periods. Aneurysm of the abdominal aorta may also cause long-lasting, excruciating low back pain.
Extravertebral, psychological low back pain is one of the most characteristic manifes tations of psychosomatic conditions. These patients may have already experienced real somatic low back pain, and later the recollec tion of their suffering is their way out of a psy chological conflict. Inspection. The appearance of an un dressed patient may be highly informative. If the patient is able to stand, the antalgic (pro tecting) posture, may be striking as may be an expressive paravertebral muscle spasm if the patient is recumbent. In lumbar stenosis, the lordosis is smoothed, fixed lumbar kyphosis is sometimes seen. Degenerative spondylo listhesis sometimes produces a step sign. In activity atrophy may be marked in the lower limbs. The gait of the patient should be observed with special care. The cause of a limp (claudication) may be a blood supply loss (ar teriosclerosis), or a lower limb joint distur bance (e.g. hip osteoarthrosis). A typical sign of lumbar spinal stenosis is the spinal ("neurogenic, intermittent") limp. The motor power of the lower limbs undergoes a diffuse deterioration after a certain walking distance. The patient first starts limping, and is then forced to stop in order to avoid falling. Fol lowing a short period of leaning forward or sitting, the muscle power returns and the pa tient can carry on walking. Neurological state. Following the inspec tion and the observation of the gait of the pa tient, and the recording of the cervical and lumbar (active) ranges of movement, the neu rological state of the trunk and the limbs is as sessed. This means a basic assessment of the sensor, motor and reflex functions. Symptoms of monoradicular lumbar defi cits with low back pain point to nerve com pression (Table 24. 1 . and Figs. 24.48., 24.49. and 24.50.) . The presence of neuritis is demonstrated by stretching provocation of the nerve. In the case of L4 neuritis (the main bundle of the femoral nerve), forceful bending of the knee
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Table 24. 1. Symptoms of mono radicular lu mbar Level o f root involvement
Frequency Pain
Reflex deficit
Para-, hypesthesia
Motor change (deficit, paresis)
5 1 root (disc prolapse L5-S1)
37-43%
posterior thigh, lateral leg, v. toe
Achilles
5 1 dermatome (lateral leg)
flexor hallucis longus, triceps su rae, tiptoe is d ifficult
L5 root (disc prolapse L4-L5)
53-59%
anterolateral leg, big toe
none, oce. weaker or missing tibialis posterior jerk
anteromedial leg
m. extensor hallucis longus, m. extensor digitorum, walking on heels is d ifficult or impossible
L4 root (disc prolapse L3-L4 or L4-L5)
1-2%
anterior thigh, medial leg, femoral nerve sign
patella reflex is weak or missing
anterior thigh, medial leg
m. quadriceps atrophy, extension of knee is weak, knee instability
Central protrusion of higher lumbar discs - cauda equine syndrome
0,5- 1%
radicular pain radiating both lower limbs
reflex deficit both lower limbs
paresthesia in area of rectum, private parts
d istu rbance of urinary and defecation
of the patient in a prone position provokes pain in the inguinal region (this is the femoral test). Radiculitis of roots L5 and S I , the main bundles of the sciatic nerves is called sciatica. The stretching test of the sciatic nerve relates to the classical Lasegue sign (straight leg rais ing; Fig. 24.51 .). With the patient in the su pine position, the hip is gradually flexed with the knee extended. This flexion is normally possible to 90°. The tension of the sciatic nerve increases in direct proportion to the ex tent of flexion. In cases of inflammation, rais ing of the straight leg through an angle of 1 5-20° may already produce pain, which be comes more severe on further flexion. In Bragard' s test, the straight leg raising test is performed until the pain limit, and the foot is then passively dorsiflexed to increase the ten sion of the nerve, producing even worse pain. An increase in abdominal press are (coughing or sneezing) also increases the pain. Complaints related to defecation or urina tion should be evaluated with special care. Overflow incontinence, urine retention or
complaints in connection with the tension of the full bladder may refer to cauda equina compression (many or all descending roots are damaged). The cauda equina syndrome is character ized by: - severe radicular pain - flaccid paralysis, with the loss of all sensation in a riding-breeches distribu tion - total stool and urine incontinence - impotence Additional examinations of degenerative spinal disorders, and differential diagnosis: Neuroradiologic examinations furnish in formation on the structural state, the localiza tion of defects, and space-reducing processes. CT, MRI tests, myelography, discography and bone scan are the other choices (Fig. 24.52.). Neurophysiologic studies are not carried out as a routine. EMG is indicated as a differ ential diagnostic tool (especially to differenti ate peripheral neuropathies).
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i
j{J 8
Q�.y
Fig. 24.50. Fig. 24.48.
S 1 root signs. Motor change: m. peroneus. Reflex change: Achilles reflex. Sensory change: Outer edge of foot.
L4 root symptoms. Motor change: m. tibialis anterior. Reflex change: patellar reflex. Sensory change: inner edge of foot.
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Fig. 24.49.
LS root symptoms.
Motor change: m. extensor hallucis longus. Reflex change: medial ankle reflex. Sensory change: mid part of foot.
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The psychologist and/or psychiatrist has an important role to play in the assessment of therapy-resistant chronic pain syndromes. Be fore spinal operations, the patients routinely perform a series of tests compiled by the psy chologist.
"Fig. 24.51. Assessment of Lasegue's and Bragard's signs.
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Fig. 24.52.
Herniated disc in segment L5 51 on MRI. -
Even without treatment in the overwhelm ing majority of acute back pain cases there is an alleviation within 3 weeks. 24.6. 1.5. Conservative treatment
of a herniated disc and degenerative spinal stenosis When the treatment strategy is being de cided, the history must be carefully consid ered. During the process of herniation, the water content of the hernia decreases and the hernia shrinks. The tom-out sequestrum loses half of its original volume in about 6 weeks, and the space-reducing effect fundamentally de creases. This is why the hernia-elicited symp toms regress spontaneously even within a few days, and the pain ceases totally in 3-6 weeks without treatment. Sensory changes have a similar likelihood of being resolved, but mo tor changes (paralysis) may signal the risk of permanent harm. When the patient exhibits no, or only mild motor effects the basic strat egy is nonoperative, conservative treatment. This conservative treatment is intended to resolve the neuritis. The patients are given steroids via an infusion or an epidural cannule or paravertebrally, or nonsteroidal anti-in flammatory drugs are administered. Symptomatic pain reduction may also be used (local cooling and pain killers). Muscle relaxants too are popular, though it is not cer tain, that they are needed in every case.
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In the first few days after an acute onset, in bed rest is advised; after 3 -4 days, a gradual vertical posture and the resulting load-bearing is encouraged. In optimal cases, a consider able improvement starts around day 3 - 5 , and from around day 1 0 physiotherapist-guided exercises may commence. The total recovery time depends on the original symptoms and the extent of the neurological deficit. Within 6-8 weeks most patients (with no motor in volvement) regain a painless state. In degenerative spinal conditions, the complaints and pain may recur.
24.6. 1.6. S u rgica l treatment of degenerative spinal d isorders
Surgical treatment must be taken into con sideration if the complaints do not lessen in response to conservative therapy, or even worsen. If the first assessment reveals exten sive paresis accompanied by neuritis and pain, surgery may be decided as the first choice. The cauda equina syndrome is an absolute in dication for surgery. The aims of the operation in degenerative spinal disorders are to solve the compression, to eliminate the pain, to support the natural restabilizing processes in cases displaying various stages and forms of instability, and ul timately to create block vertebra with fusion. Percutaneous minimally invasive oper ations. In these procedures, via a relatively small exposure, the skin is penetrated with a trocar. A working channel (a 2-4 mm wide, 1 50-300 mm long tube) is advanced to the disc or to the epidural space. To remove the content of the hernia, mechanical devices can be employed, but a laser beam may also be used to vaporize certain parts of the hernia (Fig. 24.53.). Decompression. In open surgery, the spi nal canal is exposed, the nerves are decom pressed, and the sequestrum fragments are re moved. This type of operation is indicated when there is no rough instability in the given
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Fig. 24.53. Percutaneous approach for removal of herniated d isc.
segment, and the decompression itself is ex pected to resolve the pain and the radicular symptoms. a
Decompression, fusion and stabiliza tion. In cases of advanced degenerative changes, following decompression in the un stable segment, it may be necessary to create an artificial block vertebra (fusion), which constitutes the ultimate solution (Fig. 24.54. a, b). The fusion procedures (creation of a block vertebra with a bone implant) are highly ac cepted procedures for the surgical treatment of degenerative spinal stenosis. The following indications are approved by a broad interna tional professional consensus: - In cases of mono segmental invol vement, fusion is the choice, if de compression leads to definitive segment instability - In cases of degenerative spondylo listhesis, especially in segment L4-5 , where the incidence of degenerative spondylolisthesis is 5 times higher than in all the other segments. - In cases of polysegmental decomp ression, the possibilities for recreation of the spinal balance (physiologic lordosis) must be carefully planned. In these cases, implants are necessary to ensure the stabilization of the vertebrae in the physiologic shape of the spine, by creating the appropriate bony fusion. Disc prosthesis. In modern spinal surgery, innovative techniques have been introduced
b
Fig. 24.54. a and b: Transpedicu lar lumbar intervertebral fusion.
to protect the movement of the segment. In se lected cases, the damaged disc is replaced by a prosthesis in either the cervical or the lumbar segment (Fig. 24.55.).
24.7. Spinal changes in osteoporosis Aging presents a special sequence of prob lems as concerns spinal disorders .
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c
Fig. 24.55. a: Outline of cervica l disc implant. b and c: Biplanar X-ray after insertion of a cervical disc implant.
Morphologic characteristics of the ag ing spine. The morphologic appearances de scribed in the previous sections in which the degenerative changes were discussed, be come much more marked as aging progresses, and emerge in all spinal segments. Block ver tebra formation occurs mainly in the lower segments of the cervical and lumbar spine, as a result of the bony union of collar-shaped os teophytes localized at the end-plates of the vertebral body. In the intervertebral j oints, ankylosis develops, and the intervertebral space is filled with a fibrotic mass. The end-plates of the vertebral body (mainly the subchondral layer) become scle rotic, and the loosening trabecular system is observed below it, in the cancellous bone. These changes may lead to segmental de formities (segmental scoliosis, degenerative spondylolisthesis, retrolisthesis, etc.). Degen erative changes in many segments give rise to the characteristic (senile) deformation. Physi cal examination reveals retroflexion and ret roversion with decreased lumbar lordosis, and occasionally lumbar kyphosis and an increase in the dorsal kyphosis (see Chapter 1 4 . 1 ).
These processes cause the height to decrease and the typical (stooping) posture of the el derly develops. Treatment tactics of spinal complaints of elderly. If there is acute spinal pain (but no neurological deficit), the patient is relieved from weight-bearing, and pain killers and anti-inflammatory drugs are prescribed. The background of the acute spinal complaints of the elderly involves a pathologic fracture in most cases. Accordingly if the pain is not alle viated within a few hours, an urgent diagnos tic scan is required in order acquire get infor mation on the structural integrity of the spine. It must not to be forgotten that in this age group, the pathologic fracture of the vertebra, and the morphologic diagnosis may therefore be urgent. Operation is contemplated only after the failure of long-lasting conservative treatment or when neurological signs appear. In a recent technique, bone cement is injected into the fractured vertebral bodies (vertebroplasty) to decrease the kyphosis and protect the stability of the spine.
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24.S. Tumors Primary extradural tumors of the spine are very rare.
24.S. 1. Primary tumors Benign tumors, osteoid osteoma, osteo blastoma and osteochondroma in the spine are rare (Fig. 24.56.). Vascular tumors of the ver tebral bodies (hemangiomas), however are relatively common. They are generally mani fested before the teenage years ; a late onset is rare. They grow from the vertebral arch to the canal or to the surrounding soft tissue. The main complaint is the local pain at rest (sometimes at night) . They may cause nar rowing while they expand to the spinal canal, and may produce signs of a neural deficit by compressing the spinal cord or the spinal nerves. Bone changes induced by an aneurysmal bone cyst (classified as a tumor-like change) or disturbances of the bone metabolism (e.g. Paget' s disease) are extremely rare in the spme.
a
Fig. 24.56.
Osteoid osteoma in the arch (U axial image)
Of the primary malignant bone tumors of the spine, the following sarcomas are note worthy: osteosarcoma, chondrosarcoma, Ew ing ' s sarcoma, etc. (Fig 24.57. a, b, c, d). They expand aggressively and destructively, and do not respect the surrounding areas, and their size and location do not allow their com-
b
Fig. 24.57. a, b: Osteosarcoma on the cervical spine (MRI) c, d: Total segment resection, bone graft and metal plate fixation on biplanar X- ray.
2 4. D i s e a s e s of t h e s p i n e
plete surgical removal even at the time of their discovery. They usually occur in children. Following a complex oncological assessment, the operative removal must be supplemented with chemo- and radiotherapy.
24.8.2. Metastases The most common form of tumor involve ment of the spine is undoubtedly the onset of metastases (secondaries).
It is believed that almost all kinds of tu mors may metastasize to any bone, and most often to the spine. The reason is the special blood supply of the vertebrae : the tumor emboli captured in the end-arteries running to the end-plates may relatively easily embed into the cancellous bone and start to grow thanks to the good blood supply. The most common localizations of the metastases are the dorsolumbar segment and the bodies of the two upper lumbar vertebrae. In the first stage, the metastasis infiltrates the medullary cavity of the spongious sub stance, and the destruction of the bone
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trabeculae leads t o compression (pathologic fracture) . If the vertebra is displaced and the vertebral canal is deformed due to the tumor, instability and a tumor-induced deformity are threatening (Fig. 24.58.) .. In the event of these pathologic fractures as urgent MRI assessment is needed. The im ages provide information on the extent of the tumor, the involvement of the spinal canal, the state of the spinal cord, and the tumorous in volvement of the surrounding soft tissues and spinal segments. Severe spinal cord compres sion demands immediate surgery: by six hours after the onset of flaccid paralysis, the chance of full neurological restitution has been lost. The operative goals are decompression of the neural elements, full or partial removal of the tumor, and reconstruction of the spinal stability. It must be borne in mind, that these interventions are intralesional from an oncological aspect (Fig. 24.59.) . The incidence of tumors is currently in creasing, while the diagnostic and treatment procedures are undergoing constant improve ment. Accordingly, an ever increasing num ber of patients are reaching that phase of their
Fig. 24.58. Pathological stages of vertebral metastasis: a: tumorous invasion of medullary cavity b: impression of the endplates (pathologic fracture) c: segmental instability due to the vertebral fracture with spinal stenosis and compression of the neural ele ments.
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restricted. Typical signs are gibbus formation, the destruction of two or more vertebrae on the X- ray image with a pre- or paravertebrally enlarged soft-tissue projection and a cold ab scess.
b c
Fig. 24.59. Total segment resection on lumbar spine. a: metastasis in L3 b, c: Following total segment resection, metal pros thesis bone cement, and transpedicular fixation im plant; biplanar X- ray.
oncological disease, where spinal metastasis is likely. Observation, adequate musculo skeletal screening of these patients, and the immediate assessment of early complaints that point to spinal metastasis (local night pain) afford some opportunity for the early treatment of spinal metastases. Surgical inter vention is only one (radical) mode of treat ment. A complex oncologic approach makes use of various chemo- and radiotherapeutic procedures in cases involving bone meta stases.
24.9. I nflammations of the spine 24.9 . 1 . Spondylitis tubercu losa (Pott's disease) Spondylitis tuberculosa is tuberculotic inflam mation of the vertebrae. The i nvolved spinal segment is painful, and its movements are
Incidence. Spondylitis tuberculosa is one of the most common forms of musculo skeletal tuberculosis. Mostly the dorsal and lumbar spine are involved, but it may appear in the cervical segment too. In the past few years, its incidence has shifted in the direction of the elderly. The incidence of pulmonary in volvement, and also that of newly identified extrapulmonary cases increased even in those countries that have an advanced preventive network. Etiopathology (see Chapter 20). This in flammatory process typically occurs in the frontal edge of the vertebral body, next to the disc. The disc itself is already involved in the early phase of the disease; an early X-ray sign is the narrowed disc space. Following the total destruction of the disc, the process destroys the end-plates of the neighbouring vertebrae. The two neighbour ing vertebrae collapse on each other because of the partial destruction of the vertebral bod ies, the development of cavities follows, and sequestra and a gibbus are produced. The for mation of an abscess (cold abscess), is com mon. The dorsal spine is surrounded by pus in the shape of a fusiform abscess, which some times breaks through to the surface between the ribs as a sinus. When the abscess is formed in the lower back, in the lumbar segment, it sinks in the caudal direction among the psoas muscle fi bers (psoas abscess), and it may appear as an inguinal or femoral sinus. The abscess, sequestrum and gibbus formation may lead to spinal cord compression symptoms and para plegia. The explanation of the neural damage is the mechanical compression by the abscess and the sequestrum, and the toxic, vascular damage induced by the process.
24. D i s e a s e s o f t h e s p i n e
Clinical symptoms. The disease starts with uncertain symptoms, subfebrility and fa tigue. The dorsal or lumbar pain increases in response to load-bearing. The spinal move ments become restricted in every direction due to the protective muscle spasm. The pa tient tries to substitute the painful spinal movements, and to lean with the hands on the thighs so as to unload the spine. The spinous processes are tender, and gibbus may occur. The muscle power of the lower limbs may weaken. The cold abscess may be palpable. Laboratory signs. The sedimentation rate is increased, and lymphocytosis is common. The Mantoux test may be positive. The feared complication of spondylitis tuberculosa is paraplegia, which demands immediate inter vention (see below) .
Fig. 24.60. Spondylitis tuberculosa. The D7-8-9 i ntervertebral spaces narrow. The end-plates are destroyed. On the left, there is marked soft-tissue mass widening.
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X-ray signs. The first X-ray sign is the narrowed disc space. The involved vertebrae become atrophic, and the end-plates are un even. As the process advances, the anterior parts of the vertebral bodies are destroyed, cavities and shadows of sequestra appear, and the neighbouring vertebrae collapse on each other. The X-ray films reveal an enlarged soft-tissue proj ection pre- or paravertebrally. This is due not only to edema, but also to the development of a tuberculotic abscess con taining thick pus that resembles to condensed milk (Fig. 24.60.). The X-ray film shows the disappearance of the psoas muscle edge in the lumbar seg ment, or widening of the psoas proj ection in dicative of the presence of the abscess. The sclerotization of the bony structure and de marcation seen in the X-ray picture are signs of the consolidation of the process, together with the diminishing or disappearing para vertebral soft-tissue mass. A bony union be tween the neighbouring vertebrae, or block vertebra formation means consolidation, and the healing of the process (Fig. 24-6 1 .) . CT and MRI examinations facilitate deter mination of the extent of the process, and the spinal cord involvement. MRI also helps in the differentiation of spinal osteomyelitis and tuberculosis. Since the clinical picture can vary consid erably, establishment of the diagnosis is not always simple. The abscess and vertebral focus may be punctured with a CT-guided biopsy tool, and specimens can be gained for bacteriology (an imal inoculation) and histology. In the differential diagnosis, all those dis eases must be considered which cause spinal pain and vertebral destruction (osteomyelitis, spinal tumors, certain cases of spondylitis ankylopoetica, spondylodiscitis, eosinophilic granuloma, and compression fractures) (Ta ble 24.2.). It is important to note, that tuberculosis usually involves two or more vertebrae and discs. The vertebrae collapse on each other, abscess formation follows, and neurological complications may occur.
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Treatment. With the exception of para plegic cases, the diagnosis is followed by therapy with combined anti-tuberculotic med ication for an appropriate period to relieve the toxic symptoms and decrease the activity of the process. In the event of regression of the clinical, X-ray and laboratory signs, if the im aging assessments (X-ray, CT and MRI) do not prove demarcation and abscess, and if sequestra are present, operative intervention may be justified. The surgical aim is focus re section and stabilization. When there are threatening neural signs, paraplegia may ne cessitate emergency intervention. Surgery is always accompanied by anti tuberculotic medication. Combined anti tuberculotic treatment must continued for months, since the process may flare up from the calcified abscesses, and hidden foci.
24.9.2. Pyogenic spondylitis (spinal osteomyelitis) Fig. 24.61.
The clinical processes and X- ray signs, of the inflammations of the vertebrae caused by pu rulent bacteria are so diverse that their recog nition is difficu lt. The vertebral infection may appear as a tempestuous, often life threatening spinal osteomyelitis, causing fe ver, and a toxic state, or it may be a slowly progressing form mimicking spondylitis
Angu lation and gibbus because of the collapse of the vertebrae. The process has healed.
Pott ' s triad involves - gibbus - cold abscess - paraplegia. Table 24.2.
Differential d iagnosis: vertebral tuberculosis, osteomyelitis and tumor Tuberculosis
+
Narrow disc space Destruction
One vertebra Severa l vertebrae
Paravertebral mass
Unilateral Bilateral
Extension of psoas projection Bone proliferation Destruction of arches and ribs
Osteomyelitis
Tumor
+
-
+
+-
-
-
-
-
+-
+
+-
+-
+
+
-
+-
+-
-
+
+-
-
+
24. D i s e a s e s of t h e s p i n e
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tuberculosa. The course of the disease is influ enced by the general state of the patient, the viru lence of the agent, its possible resistance to the medication, other drugs that may have been taken earlier, and the efficacy of treat ment.
Etiopathology. The most common agents are staphylococcus, streptococcus, pneumo coccus, and sometimes other strains of bacte ria. They approach the vertebrae via the blood circulation. The history may include purulent diseases, postoperative complications, fever, disc operations or local paravertebral lido caine inj ections, or conditions that may often seem unrelated to the spine, e.g. pelvic infec tion, or inflammations of the kidneys, genita lia, gall bladder, etc . A purulent focus leads to vertebral destruction, which does not respect the end-plates of the neighbouring vertebrae, so that at least two vertebrae and an inter vertebral disc are destroyed. The picture may be reminiscent of vertebral tuberculosis, and differentiation can be problematic. The process may give rise to abscess and sequestrum formation, and (less frequently than for tuberculosis) may break into the spi nal canal, inducing neurological complica tions.
Fig. 24.62. Bacterial vertebral osteomyelitis. The involved verte brae are sclerotic, with X-ray signs of repair; cavity formation is moderate; in spite of the collapse, the vertebrae retain their heig ht (no gibbus formation).
Clinical symptoms. Vertebral osteo myelitis is characterized by fever, malaise, and severe pain in the involved spinal seg ment. The excruciating pain often results in an inability to walk. The laboratory results demonstrate the changes in acute osteomyelitis: an increased sedimentation rate, leukocytosis, a blood pic ture shifted to the left, and a high C-reactive protein level. Chronic vertebral osteomyelitis in adults may be similar to spondylitis tuberculosa, but the clinical picture is more dramatic : higher fever, greater pain, the progress is faster, and the laboratory changes are more marked. X-ray signs. The first X-ray sign is again a narrowing of the disc space. This is followed by the evidence of undefined end-plates,
Fig. 24.63. MRI shows the borders of the process and the extent of edema.
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usuration, more extensive destruction, cavity formation, and occasionally sequestrum for mation within the cavity. The paravertebral soft-tissue mass may widen because of the ab scess or granulation tissue. The surroundings of the vertebral destruction are more sclerotic than in tuberculosis. A typical sign of restora tion is the osteophyte formation at the edges. The vertebral bodies may collapse onto each other, but the process does not lead to a sub stantial decrease in the total height of the two vertebrae; accordingly, in contrast with tuber culosis, no gibbus formation is seen (Fig 24.62. and 24.63.). The structure of the vertebral body be comes sclerotic in the X-ray picture, as a con sequence of the treatment applied, the de struction is demarcated, the cavity dimin ishes, the edge osteophytes unite, and the two vertebrae undergo mutual healing, forming a block vertebra (Fig. 24.64.). A supplementary examination procedure is conventional tomography, which visualizes the sequestra hidden in cavities, and also the extents of the cavities. In the period of disc space narrowing, a bone scan may contribute to the correct diag nosis .
Fig. 24.64. The block vertebra is evidence of healing.
eT shows the extent of the condition, while MRl demonstrates that the widening of the paravertebral soft tissue mass is more moderate, and the structure of the vertebral body is more homogenous in vertebral osteomyelitis than in tuberculosis.
24.3. Differential d iagnosis concerning vertebral tuberculosis, vertebral osteomyelitis, spondylodiscitis and vertebral tumor Tuberculosis
Osteomyelitis
Spondylodiscitis
Tumor
History
Tubercu losis in the past
Banal infection
Banal infection or none
Primary tumor
Fever
Subfebrility
Fever
Fever or subfebrility or none
Not typical
We
Moderate
High
Moderate or high, or normal
Occasionally high
(-reactive pro- Normal tein
High
Moderate or high
Not typical
Leukocytosis
Lymphocytosis
High, left shift
High or low
High or normal
Radiological destruction
Extensive
Moderate
Not typical
Involves one vertebra
Gibbus
Typical
Not typical
Not typical
Not typical
Structu re
Atrophic
Sclerotic
Sclerotic
Lytic or plastic
24. D i s e a s e s of t h e s p i n e
Diagnosis. When any vertebral process is detected, the most appropriate and modem bacteriologic and histological tests (im age-guided aspiration and sophisticated bi opsy techniques) must be employed to estab lish the diagnosis (Table 24.3.). Treatment. In vertebral osteomyelitis, in contrast with tuberculosis, conservative ther apy may result in healing of the process. There are a few exceptions : sepsis, abscesses, sequestra and threatening neurological com plications . Immobilization in a bed or a plaster bed, depending on the age, is the obligatory method with which to treat inflammations. Broad-spectrum antibiotics which ensure a therapeutic concentration in the bone are given first, followed by an antibiotic selected on the basis of sensitivity tests. Indications of effective therapy may appear within a few days : the fever and the pain subside, and the laboratory parameters improve. The improve ment in the sedimentation rate is slower than that in the C-reactive protein. Regression of the X-ray signs is much faster, than in tuberculosis, but it occurs only after the clinical improvement. When the clinical and radiological regres sion of vertebral osteomyelitis have taken place, the patient is mobilized in a few weeks. A plaster or plastic corset is fitted for external support. The appearance of a block vertebra signifies healing. Spinal processes that result in abscesses, sequestra or threatening neurological compli cations demand not only medical but also sur gical treatment. The abscess is exposed and the focus is cleaned and drained, similarly as described in connection with the treatment of spondylitis tuberculosa.
24.9.3. Spondylodiscitis Spondylodiscitis is a benign, inflammatory condition of the discs or vertebral end-plates.
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Apart from the pain syndrome, the nar rowing of the intervertebral space is typical, without maj or destruction. Moderate fever and an elevated sedimentation rate may be ob served. Most experts consider it to be outcome of a spinal infection. It may be a torpid osteo myelitis, where the agent invaded the disc secondarily from the end-plates without the typical picture of acute osteomyelitis. It occurs in children and young adults. The intervertebral disc is a bradytrophic tissue. The poorly nourished disc is unprotected against infection and is destroyed in a long, torpid process, and the space narrows. The richly vascularized vertebral body resists a not highly virulent agent, and therefore is not (or only slightly) involved in the process. Clinical symptoms. The processes de scribed above often persist undetected be cause of the insignificant local and general symptoms. However, pain may be experi enced in the spinal segment involved. Bending forward may be hindered because of the paralumbar muscle spasm, and the child therefore avoids doing it. Local tenderness may be detected. X-ray signs. Because of the slow progress of the disease, the X-ray picture may initially be negative. Later, the narrowed inter vertebral space becomes visible with usuration of the end-plates, and intrusion of the disc at the lumbar spine into the vertebral body, even in the form of Schmorl 's hernia (Fig. 24.65., and 24.66.) . Lymphohematogenic infection may create space narrowing over a long spinal segment. A bone scan may facilitate the diagnosis. CT and MRI tests provide more informa tion earlier than the analogous X-ray image. Treatment. Chronic antibiotic and rest (bed rest, and a corset) cure the process within a few weeks. With the exception of the narrow disc space, which persists, the symptoms re solve.
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It has been reported that mild cases of discitis in childhood may heal spontaneously, regardless of the treatment. Since this condi tion is presumed to be a mild, torpid infection, antibiotics are advised.
24. 10. Chest deformities The anatomy, biomechanics and vital function of the chest make it the most compli cated unit in the organism.
24. 10.1. Pectus excavatum (funnel chest) Fig. 24.65. A 16-year-old girl with spondylodiscitis mimicking Schmorl's hernia, fever, and a high erythrocyte sedi mentation rate.
This is a congenital chondrosternal impres sion, where the mass of the sternal bone and the attached rib cartilages bend backwards, creating a funnel-shaped impression, the deepest point being the xyphoid process (Fig. 24.67.).
The extent of the impression of the chest wall can differ. In a severe case, the lower end of the sternum, the xyphoid process, almost reaches the vertebral bodies. In girls, the de velopment of the breasts may accentuate the virtual extent of the midline impression.
Fig. 24.66. MRI reveals that the d isc protrudes into the cancellous su bstance of the vertebrae.
Etiopathology. This has not been fully identified. It often occurs in association with other developmental disorders : Marfan ' s syn drome, flat back syndrome, etc. The structural changes in the chest may disturb the thoracic organs. The lower section of the sternum and the ribs move backward on inspiration, and protrude on expiration: paradox breathing takes place, with an impaired vital capacity. The decrease in the sternal - vertebral distance elicits dislocation of the mediastinum. The apex beat of the heart may be palpable in the axillary line for the displacement and rotation of the heart. Thus, the QRS complex may change, with R vector deviation to the right, and T wave inversion.
24. D i s e a s e s of the s p i n e
Fig. 24.67.
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Fig. 24.68.
Orcu mscribed, symmetrical impression on the fron tal chest wall. Pectus excavatum.
Lateral chest X-ray: At the deepest point of the im pression, the posterior su rface of the sternum ap proaches the ventral surface of the spine.
Clinical symptoms. The children are usu ally asthenic and lean. Their trunk is slightly tilted forward, the spine is mildly kyphotic, and the shoulders are turned forward. In re sponse to a physical load, fatigue, dyspnea and tachypnea may arise. The performance of these children is inferior to that of their con temporaries. Their respiration is often para dox. They often suffer from influenza, bron chopneumonia or asthma. In adult cases, physical loading may lead to suffocation and compressive thoracic pain. Neither the children, nor the adults like to get undressed in front of others, and they avoid public places such as swimming pool because of their deformity.
The chest CT appropriately visualizes the extent of the deformity, and the distance be tween the deepest point of the sternum and the ventral contour of the vertebrae. The severity of the deformity is indicated in terms of Keszler 's index (Fig. 24.69.), as follows :
X-ray signs. The traditional postero anterior chest X-ray reveals the displacement of the heart and mediastinum, and sometimes the accompanying spinal deformity. The lat eral view clearly shows the distance between the posterior edge of the sternum and the ven tral contour of the vertebrae (Fig. 24.68.).
sternum - vertebral distance ------
posteroanterior chest frontal diameter
· 1 00
With this index, the extent of the defor mity is graded as mild (30-3 5 %), moderate (20-30%) or severe « 20%). Treatment. As conservative treatment, breathing exercises and abdominal and dorsal muscle training are recommended from early childhood to decrease the deformity, to pre vent paradox respiration and to practice cor rect thoracic breathing. Swimming and athlet ics may improve the vital chest functions and capacity.
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I·
b
_
:::: _c
---t--:C----_-_-_-,'"
_ _ - _ - -
>------
I
c:+-=J
-
... --
·1
- -
Fig. 24.69. Keszler index
=
a/b x 100
If the complaints or the deformity worsen, cardiopulmonary changes are observed, or the deformity causes psychological complica tions, surgery indication may be contem plated. About 40% of the patients are operated on between the ages of 4 and 1 0 years, and 50% under the age of 20.
24. 10.2. Pectus carinatum (pigeon chest) I n contrast with funnel chest, pigeon chest is a protrusion deformity. Less attention is paid to this deformity, but it may a lso occur in a d is figuring form that causes a major disturbance in the function of the thoracic organs. It may appear in combination form with funnel chest, with the predominance of either.
Etiopathology. The etiology is unclear. The deformity occurs in early childhood and becomes more sever at puberty (around the age of 1 0) . In the view of Lester, it may origi nate from rickets, but, similarly as for pectus excavatum, the congenital etiology prevails. Anomalies of the diaphragm may play a role, with the difference that the centrum ten-
dineum is widened and the tension of the lat eral fibers creates the deformity. The antero lateral part of the diaphragm is hypertrophied; this pulls in the rib arch, and paradox breath ing results. Clinical symptoms. These are similar to those described for funnel chest. The breath function test proves a reduction in the vital capacity, especially if the chest ex pansion is decreased by the expressed Harri son groove and the paradox breathing. The condition may be accompanied by a kyphotic back or kyphoscoliosis. The cosmetic and psychological problems are similar to those in cases of funnel chest. X-ray signs. In pectus carinatum ob liquum, the protrusion is obligue, whereas pectus carinatum arcuatum it is arched. It does not displace the heart. A chronic pulmonary circulation failure is detected as a late compli cation of severe deformities. Treatment. The surgical therapy and postoperative care of protrusion deformity cases are similar to those for pectus ex cavatum.
25.
Jeno Kiss
Disorders of the neck and the shoulder girdle
25. 1. Functional anatomy and biomechanics of the shoulder girdle The main function of the shoulder and the elbow, via the upper arm and the forearm is to adjust the main human working tool, the hand into the wanted position. Any operational dis turbance of these two large joints of our upper limb may impair the working capabilities and the regular daily activities. The degree of the function depends on whether the dominant or not dominant side is involved. A young la borer or a competing athlete has different ex pectations relative to the elderly, who mainly use their upper limbs for self-maintenance.
The movements of the shoulder girdle are combinations of the glenohumeral, acromio clavicular and sterno-clavicular movements . An additional function is the motion of the shoulder blade on the trunk, the three most important components of which are the eleva tion (lifting the arm in the plane of the shoul der blade), external rotation and the combina tion of extension and internal rotation which result in the ability to reach behind the back. This motion is ensured by the function of the thoraco-humeral, thoraco-scapular and scapulo-humeral muscles. The shoulder itself, the glenohumeral j oint has the largest range of motion of any joint of the human body, and at the same time it is the most unstable joint.
g ----��----
c
��----- b
d
Fig. 25. 1. Anterior aspect of the shoulder region: a: n. suprascapularis, b: n. axillaris, c: plexus brachia lis, d: hu merus, e: processus coracoideus, f: acromion, g: clavicula, h: lig. coraco-acromiale, i: m. subscapularis, j: m. supra spinatus, k: biceps tendon.
296
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e
a
� _
7
': ,
·,
k
g
_ -
---..I\ SH=l-tS .
v
•
I
b
-+--+- c
"
/
.'
>
h
Fig. 25.2. Posterior aspect of the shoulder region: a: n. supraspinatus, b: n. axiliaris, c: humerus, d: spina scapulae, e: m. supraspinatus, g: m. teres minor, h: m. teres major, i: m. deltoideus, k: m. infraspinatus.
Special significance is attributed from an orthopedic aspect to the rotator cuff (Fig. 25. 1 ., 25.2.). This comprises the tendons of four muscles that originate in the frontal and dorsal surfaces of the shoulder blade : the subscapular, supraspinate, infraspinate and teres minor muscles. Besides assisting the various rotational motion of the humerus, the most important task of the rotator cuff is to en sure the central position of the humeral head against the j oint cavity, in the glenoid fossa, in any position of the arm.
25.2. Examination of the shoulder girdle The most common complaints are pain, weakness, motion disturbance and instability. The onset of the complaints, and the circum stances of the possible injury must be ex plored. The details of the profession, possible sporting activity and the dominant side are also important. During the inspection atten tion should be paid to an abnormal posture, deformities, muscular atrophy, possible skin
redness, swelling. The assessment of the range of motion starts with the range of active motion, and in case of a deficit, it is compared with the range of passive motion, since impor tant conclusions can be drawn from the differ ences. The stability of the glenohumeral joint is evaluated via the various stability signs (sulcus sign, apprehension tests, drawer sign). The individual muscle and muscle group functions are checked. The muscles of the ro tator cuff are to be assessed extremely care fully. An indispensable part of the physical assessment of the shoulder girdle and the up per limb is the evaluation of the motion of the cervical spine and the neurological state of the upper limb. This may be supplemented if needed by connectivity testing of the upper limb nerves (ENG) and the muscles (EMG). The radiological evaluation requires bi directional films, one a-po and one lateral view. Depending on the actual disease a num ber of directed examinations are to be used. A cheap, swift and reliable procedure is ultrasonography test to evaluate the soft tis sues and especially the rotator cuff. The next step can be in assessment a native or con-
2 5 . D i s o r d e r s of t h e n e c k a n d t h e s h o u l d e r g i r d l e
trasted e T and MR assessment of the changes in the soft tissues, and bones, j oints. In certain cases, arthroscopic examination of the glenohumeral, subacromial or subscapular area may be required, these procedures are mainly used as the introductory phase of an endoscopic or open therapeutic method.
25.3. The congenital and acquired disorders of the of the shoulder girdle •
Torticollis, muscular congenital (wry neck)
The asymmetric posture of the head is caused by the shrinkage of the sternocleidomastoid muscle. The head is tilted lateral ly and rotated in different directions, the face is asymmetri cal, the sternocleidomastoid muscle is tensely prominent. From the age of 1-2 weeks a pain ful swelling is observed in the muscle. This oc curs in both genders, and is often associated with other developmental anomalies. The symptoms are usually observed as early as 1-2 weeks. This is the 3rd most common musculoskeletal disorder after hip dislocation and clubfoot.
Etiopathology. This is a congenital disor der. According to certain theories injury, or tearing occurs in the substance of the ster nocleidomastoid muscle during birth, leading to the scarring of the muscle. Others postulate the role of intrauterine in congruence, the neck is forced into an asym metrical position, this causes the shrinkage of the muscle. A further view consider the intra uterine ischemia as a causative factor. Besides the exogenous factors the role of endogenous, hereditary factors have also been supposed. In certain families, the wry-neck occurs with in creased frequency, often together with other hereditary disorders, such as hip dislocation, clubfoot. Clinical symptoms. In the substance of the sternocleidomastoid muscle a hazel nut-plum sized tender lump is palpable at
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one-two weeks of age. Later the lump disap pears from the muscle. It is palpable only ex ceptionally after the first or second year, but the shrunken muscle may still be detected. The shortened muscle is as tense as a tight string and protrudes under the skin. The head tilts to the involved side, while the face turns in the opposite direction. The shrinkage may involve one or both muscle parts. The shrink age of the sternal part causes the rotation, while the clavicular part tilts the head (Fig. 25.3.). If the torticollis persists for a long pe riod, the skull will become asymmetrical . The involved part of the face will be smaller, scoliosis may affect the cervical spine. Differential diagnosis •
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Congenital scoliosis of the cervical spine. The X- ray shows the possible vertebral anomalies. Ocular torticollis. I n cases of certain vision anomalies the child may hold the head asymmetrically to compensate. Torticollis caused by i nflammation. Various inflammatory processes may occur in the cervical spine. Painful motion restriction may occur in the neck related to about of common flu, which resolves within a few days. Traumatic torticollis. Pain commonly develops in children after sporting activity, during fighting, in adults following sudden jerks (e.g. looking backwards), causing an oblique posture of the neck. Rheumatoid torticollis. This occurs together with other rheumatoid symptoms. Spastic torticollis. This is a the possible consequence of neural illnesses, such as encephalitis, chorea. Scar related torticollis. This is caused by scars after burns, or various i nflammations. Torticollis may be caused by the individual's occupation. Torticollis may a lso be due to psychological reasons.
Treatment. If the disorder is detected in infanthood, conservative treatment may be commenced. The shortened muscle is to be stretched by means of passive exercises, the
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Fig. 25.3. Right sided torticollis. The right m. sternocleido mastoideus is tense, the head is tilted to the right and rotated to the left.
parents must be educated to do these. The in fant should be positioned in such a way as to have to make an active correction to see the environment: e.g. in the case of right sided torticollis the light and sound stimuli must be located on the right side and above, forcing the baby to look to the right and upward. The baby is to be fed in such a way that the head must move in the direction of correction. De pending on the severity of the disorder opera tion is advised at the age of 1 -2 years . The tense part of the muscle is incised-in some cases the entire muscle is detached from the sternum and the clavicle. Some experts pro pose the recession of the upper attachment of the muscle from the mastoid process. Surgery performed at an proper time usually results in complete correction. If the operation is de layed, the facial asymmetry may persist. Rehabilitation. Postoperatively soft col lar support in young individuals is used, or plaster of Paris fixation in older patients or in cases of severe deformity. The head is posi tioned into overcorrected state. After 2-4 weeks of rest, the neck exercises commence.
Fig. 25.4. Sprenge/'s deformity. The left shoulder blade is
higher.
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Klippel-Feil-syndrome syndrome)
(short
neck
The neck of the patient is very short, and the range of motion is restricted. The deformed vertebrae may resu lt in the mechanical irrita tion of the cervical nerve roots and occasion ally of the spinal cord, neural signs may there fore appear.
Etiopathology. This characteristic defor mity is caused by a multiple developmental anomaly. There is no unified etiology, so it is recommended to consider this anomaly as a syndrome. The basic disorder is a disturbance of segmentation, or the unification of the bi lateral half-segments of the vertebrae. Symptoms. In mild cases the X-ray done for some other reasons uncovers the disorder of the cervical spine, which is localized to a few vertebrae and symptomless. In severe forms of the condition, the short neck is spec tacular, which may be due to the extreme shortening of the neck, or it may also be vir tual due to the higher location of the scapulae.
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The neck motion is restricted, torticollis may develop. The X-ray shows the vertebral changes listed above. Treatment. Only the complaints and neu rological symptoms due to the degenerative changes in adults are to be treated. �
Elevated scapula (Sprengel's deformity, congenital high position of the shoulder blade)
This is a shoulder blade anomaly occu rs in both genders. The shoulder girdle with the shoulder blade is higher on one side or rarely on both sides, while the shoulder blade is de formed, hypoplastic and often rotated (Fig. 25.4.)
Etiopathology. During the development, the shoulder girdle does not descend in the usual way from the height of the cervical seg ments. The periscapular muscles are short ened due to the position of the bone, their path changes, in certain muscles fibrosis is ob served. From the upper apex of the shoulder blade a cartilaginous-bony bridge (omo vertebral bridge) is connected to the lower cervical spine in about one third or half of the cases. Symptoms. The shoulder blade and the shoulder are located higher, the upper angulus
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of the scapula protrudes in the line of the neck and shoulder, the tip of the shoulder is dis placed anteriorly. The elevation of the arm is hindered because of the restricted motion of the scapula. The X-ray shows the shoulder blade positioned higher than the opposite one, in some cases the bony bridge connected from the angulus superior to the lower cervical spine is also observed (Fig. 25.5. a-b). Treatment. This condition typically does not disturb the function of the limb, is not painful, therefore its correction is usually jus tified from a cosmetic point of view, primarily in unilateral cases. Quite a few procedures are recommended, one of the most simple and rel atively common interventions is incision of the omovertebral bridge. If the angulus supe rior protrudes considerably, a good cosmetic result may be achieved by excision of this part. �
Scapula alata (flying shoulder blade)
This scapula disorder may take place for vari ous reasons. In mild forms during elevation, in severe forms even at rest, the shoulder blade lifts off the trunk. Since the function of the upper limb and the u nlimited motion range of the shoulder girdle require stability and harmonic movement, the pathologic state of the shoulder blade may cause severe func tional loss.
a b
Fig. 25.5. a: X- ray: The right shoulder blade is higher. I n the cervicodorsal junction at many levels spina bifida is present. b: The shoulder blade limits the elevation of the arm.
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Etiopathology. The main motors and sta bilizers of the shoulder blade may weaken one by one (serratus anterior or trapezius muscle palsy) or simultaneously. The cause of serratus anterior muscle palsy is a lesion of the thoracic longus nerve, which may appear after a viral infection, lifting heavy obj ects, trac tion, direct contusion or iatrogenic injury. The accessory nerve may be injured in a similar way leading to palsy of the trapezius nerve. Symptoms. The shoulder blade lifts off the trunk on elevation of the arm. In severe forms, this sign is detected even at rest. Be cause of the disorder of the shoulder blade, the elevation of the arm is weaker or very limited (Fig. 25.6. a-b). In mild cases, only fatigue, shoulder girdle pain are detected. In case of facioscapulohumeral dystrophy (FSHD) com plex shoulder girdle weakness is observed. Treatment. In cases of isolated serratus anterior palsy, conservative treatment is ap plied first, including shoulder girdle exer cises, selective current stimuli and vitamin D . If conservative treatment is ineffective after 6 months, surgical exploration and neurolysis is to be considered. In cases of total palsy of the scapula stabi lizers the fixation of the shoulder blade to the trunk, the scapulo-thoracal fusion provides
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Neuromuscular compression syndrome of the upper limb ( " thoracic outlet " syndrome)
This is a distinctive, complex syndrome caus ing complex sensitivity, innervation and circu lation disturbances in the upper limb. It is in creasingly referred to as the "thoracic outlet syndrome" (TOS). It is most common in mid d le-aged women.
The typical cause may be a cervical rib, when the brachial plexus and the subclavian vein and artery are all compressed at their exit from the chest, before they reach the upper limb (Fig. 25.7. 25.8.). Symptoms. These are due to sensory, mo tor and circulation changes. •
Sensory changes: Painfu l paresthesiae, often
marked pain, burning sensation appears in the ulnar part of the hand and fingers. Hypoesthetic zones may be present in areas supplied by the ulnar nerve.
b
Fig. 25.6. A young female with facioscapulohumeral dystrophy (FSHD): bilateral scapula alata (a), limiting the elevation of the arm (b).
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Fig. 25.7. Pathomechanism of the thoracic outlet" syndrome. " a: a. vertebral is, b: m. scalenus medius, c: m. scalenus anterior, d: a. su bclavia, e: v. subclavia, f: m. pectoralis minor.
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Motor changes: Paresis, muscle atrophy may take place mainly in the small muscles of the hand. The g rasping force may be diminished. Vasomotor changes: Cyanosis may appear in the limb, atrophic changes may appear on the fingers. The radial pulse may weaken or disappear if the limb is abducted and externally rotated.
Treatment. In mild cases conservative treatment is successful. It is important to per fonn exercises to strengthen the shoulder musculature and improve the posture. In stub born cases, an operation, removal of the 1 st rib or cervical rib may be needed to improve the compression signs.
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Fig. 25.S. Cervical rib (white arrow) and extended processus costotransversarius (black arrow).
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Cervicobrachial syndrome Detailed description of this clinical picture is to be found in the chapter of spinal disorders (Chapter 24). The symptoms are often similar those to of other upper limb illnesses, and therefore an assessment of the cervical spine is essential, occasionally with X-ray examina tion in cases of any upper limb symptoms, and a basic neurological assessment of the upper limbs. �
Obstetric paralysis (paralysis of the lower limbs after birth)
During birth, the brachial plexus or roots of the p lexus are injured leading to various ex tent of paralysis of the upper limb muscle.
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The involved upper limb o f the infant lies motionless, rotated internally beside the trunk. Later, the clinical picture depends on the extent of the paralysis. Three forms are distinguished, regarding the anatomy of the plexus : - Erb-Duchenne 's form involves the upper arm - Klumpke 's form involves the forearm - the third form involves the entire upper limb. Symptoms. The infant is lying with the floppy, motionless upper limb, internally ro tated beside the trunk. Later the extent of the paralysis and of the possible anesthetic, hypoesthetic areas are dependent on the type of the paralysis. These changes can usually be established only in older babies. In larger chil dren and adults a growth disturbance of the upper limb is observed, while the humerus or in more severe forms the entire limb is shorter. Treatment. In the first week after birth the limb is put to rest. The limb is usually posi tioned with the shoulder abducted to prevent contractures. The simplest way is to tie the shirt sleeve with a ribbon to the swad dling-clothes (Fig. 25.9.) . In the second week, passive exercises and electrical stimulation are commenced. After the 2nd year an im provement is not expected from further con servative treatment, the function of the limb may often be improved surgically.
25.4. Disorders of the shoulder In the common meaning shoulder joint is simply taken as the glenohumeral joint. How ever, in a wider sense, the shoulder joint refers to a joint performing all of the movements be tween the trunk and the humerus. �
Subacromial impingement
This syndrome is caused by the abnormal contact with and rubbing of the acromion by the g reater tubercle and the attached supra spinate tendon. Typical symptoms are the painful motion arc during lifting of the arm and the pain radiating to the upper arm, and increases at night. This syndrome is character istic of the individuals between 40-50 years.
Etiopathology. These symptoms may be caused by a number offactors. The most com-
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Fig. 25.9.
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The paralytic upper limb is fixed in an abducted po sition to the swaddling-clothes or to the bed.
Fig. 25.10. Painful arc, typical of the middle range of the eleva tion (a: painless, b: painfu l motion)
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mon are the anatomical changes o f the acromion as the age advances. Inflammation of the subacromial bursa for any reason, but usually after repeated trauma, tendinitis of the rotator cuff, may lead to impingement by nar rowing the subacromial space. Paralysis or only weakness of the rotator cuff, contracture due to shrinkage of the tendons or of the cap sule of the glenohumeral joint may cause im pingement by displacing the humeral head cranially. Symptoms. A typical complaint is the pain in the middle range of arm lifting, the so called painful arc (Fig. 25.1 0.). The night pain is also typical, the patient wakes many times and is unable to lie on the involved side. Apart from the painful arc examination reveals crep itation during elevation and rotation, and weakness of the muscles of the rotator cuff due to the pain. X-ray shows the possible changes in shape, osteophytes of the acromion, and scle rosis of the greater tubercle (Fig. 25. 1 1.). Ul trasound or MRI shows thickening of the sub acromial bursa, fluid collection, edematous thickening or degenerative thinning of the supraspinatus tendon. Treatment. Conservative treatment is first applied, depending on the causative fac tors . The possibly decreased range of motion
Fig. 25.11. Calcified attachment of the coraco-acromial liga ment leading to impingement.
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must be restored with active exercises of the rotator muscles. The pain and inflammation are alleviated with NSAID, physiotherapy and a few (at most 3) local steroid inj ections. The working environment, sport and occupa tional activity are to be adjusted. If the conser vative method fails, an operation may be con sidered. Subacromial decompression may be done with an open exposure or arthro scopically. The operation is followed by a number of month rehabilitation period, with the aim of restoring the motion rhythm, range and the muscle power. �
Tearing of the rotator cuff
Any tendon, but most often the supraspinate, may be torn partially or in total thickness, typ ically near to the attachment. The clinical pic ture is characterized by a painful arc during lifting of the arm, the pain, increasing at night and radiating to the upper a rm, and the func tion disturbance, depending on the muscle in volved.
This disorder, most common around the age of 40, is due to the degenerative changes of the tendons. Its frequency increases with the age. It may occur rarely at a younger age (throwing athletes, laborers) . Etiopathoiogy. A number of factors may play a role. The tendons of the rotator cuff, and specially the supraspinate have a hypovascular zone in the vicinity of the at tachment. The impaired circulation is accom panied by collagen degeneration due to aging, repeated microtrauma relating to the occupa tion or sporting, occasionally a significant trauma. The tear initially is rarely total, but usually a partial thickness tear (Fig. 25. 1 2 . a-b), and i t later becomes complete gradually or after a major injury. Tears are also de scribed regarding to their extent as well. When the tear is larger and persists longer, the possibly intact residue of the rotator cuff loses its centralizing effect on the humeral head. The humeral head is displaced cranially and gradual secondary degenerative changes take place.
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may be chosen. Active exercises are impor tant to strengthen the remaining components of the rotator cuff and other muscles of the shoulder girdle, and to improve the active mo tion. Pain and inflammation can be reduced with NSAID, physiotherapy or a few, (at most 3) local steroid injections. Operation is con sidered in older patients after failed conserva tive therapy, at an active age or even as pri mary treatment. In partial tears, open or ar throscopic acromioplasty and removal of the tom periphery are indicated. In cases of a total tear, rotator reconstruction, reinsertion of the tendon(s) to the bony base is recommended (Fig. 25. 13. a-b) .
a
b
Fig. 25.12. Partial tear of the supraspinate tendon in the prox imity of the joint (a) and the bu rsa (b)
Symptoms. This condition is also charac terized by the pain presenting in the middle range when the arm is elevated, the so called painful arc (Fig. 25.1 0.). Night pain is also common. The patient may experience pain and cracks, rubbing in certain movements. Active elevation of the arm is possible even in cases of massive tears, but examining the muscle functions one by one, the weakening is detected. In case the lesion has been present for a long time, the supraspinal or infraspinal fossa will be excavated, signaling the inactiv ity atrophy and degeneration of the muscles. X-ray shows the possible changes in shape, the sclerosis of the acromion, and the sclerosis of the greater tubercle. In more se vere cases, thinning of the subacromial space and secondary osteoarthritis of the gleno humeral j oint are observed. Ultrasound or MRI shows thickening of subacromial bursa, fluid collection, and degenerative thinning in cases of partial tear, and the loss of the supraspinate tendon in cases of total tear. Treatment. Depending on the age, occu pation, daily activities of the patient, and also on the extent of the tear, conservative therapy
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Adhesive capsulitis (frozen shoulder)
This is a typical syndrome caused by fibrotic tissue changes in the soft tissues surrounding the shoulder. The range of motion is limited, painful, but no significant X-ray changes are detected.
It is a common affliction mainly in middle aged adults, women are involved one and a half times more often. It is more common in association with diabetes mellitus and other metabolic diseases . The condition may be uni or bilateral with different severity and pro gression. Etiopathology : a number of factors may play role. Two basic forms are distinguished. - Primary adhesive capsulitis: the etiology is unknown. - Secondary adhesive capsulitis: the clinical symptoms are similar and the cause is known : injury of the shoulder, operation, intraor periarticular inj ection, infection, etc. Symptoms. Regardless of the etiology pain radiating to the upper arm is typical. The pain is present at rest, often it is worse at rest. The onset of pain is soon followed by a grad ual restriction of motion. As time goes by, the pain may decrease or disappear, but the re stricted motion persists for a long time.
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limitation of both active and passive shoulder motions the native X-ray does not prove any maj or changes. Treatment. Typically in a large propor tion of the cases this disorder heals without any particular therapy. This healing may last for 1 -3 years, and while the pain ceases and the motion improves, some restriction in the range persists. The aim of the conservative therapy is to relieve the pain. At the same time, gradual restoration of the range of mo tion should commence with exercises per formed a few times every day. In the over whelming maj ority of the cases conservative treatment results in a satisfactory improve ment, an additional procedure could be the manipulation of the joint under anesthesia. Some prefer operative treatment. a �
Glenohumeral arthrosis (osteoarthritis of the shoulder)
This condition causes a painful limitation of motion of the g lenohumeral joint it may be primary or secondary (following inflamma tion, degenerative, traumatic changes, infec tion, etc.). The primary form involves mainly elderly a bove the age of 60-65. The secondary forms may take place at any age, depending on the etiology.
Fig. 25.13. Reinsertion of torn supraspinate tendon with transosseal sutures (a: a-p view, b: upper view).
On examination typically severe restric tion of elevation and rotation is found. The ab solute diagnostic criterion is that besides the
Etiopathology. In primary arthrosis, the changes are similar to those for the other large joints (Fig. 25.14. a) . The rotator cuff is usu ally intact. In secondary osteoarthritis the outcome of the changes depends on the etiologic factor (Fig. 25.14. b, c, d) . Symptoms. Common symptoms of the primary and secondary osteoarthritis are the pain and the limited range of motion, which usually develops gradually. Because of the chronic instability or the rotator cuff tear, muscle atrophy takes place around the shoul der. In contrast with adhesive capsulitis radio logical changes are always detected in cases of arthrosis. Treatment. Since the load-bearing de mand on the upper limb is less than that on the
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c a
d
b
Fig. 25.14. Primary arthrosis of the shoulder (a), post-trau matic arthrosis (b), avascu lar humerus head -necrosis induced arthrosis (c), massive rotator cuff tear induced arthrosis (d).
lower limbs, conservative treatment can be applied for a long period. If an operation is necessary, it may be a j oint-preserving or a j oint-replacing procedure (arthroplasty). Joint-preserving methods include arthroscop ic lavage of the shoulder, endoscopic removal of cartilage debris, osteophytes, tom pieces of labrum and tendons, and in rheumatoid pa tients arthroscopic or open synovectomy. In severe cases, implantation of a shoulder pros thesis will improve the quality of life. De-
pending on the type and severity of the illness merely the head of the humerus (hemi arthroplasty) (Fig. 25.15. a), or both the head of the humerus and the glenoid surface can be replaced (Fig. 25.15. b). When both the glenohumeral joint and the deltoid muscle are destroyed, glenohumeral arthrodesis (Fig. 25.1 6.) could be the only solution to ensure pain relief and it provide an acceptable func tion by rotation of the scapula over the trunk.
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a b
Fig. 25.16. Glenohu meral arthrodesis, osteosynthesis with plates.
athletes and heavy laborers, is due to the phys ical overload and the special intra-articular path of the biceps caput longum tendon. If conservative treatment fails, arthroscopy is advised, this may show fragmentation or fi brillation, which may be smoothened arthro scopically. In cases of a severely degenerated tendon proximal detachment and tenodesis in the sulcus or more distally can be considered. Fig. 25.15. Shoulder hemiarthroplasty (a) and total arthroplasty, (b) X-ray picture.
25.5. Other painfu l shoulder conditions briefly �
Tendinitis of biceps brachii muscle Pain and local tenderness radiate from the area of the bicipital sulcus to the upper arm. This disease usually observed among young
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Calcifying tendinitis This is usually observed in women over 40, who experience very heavy shoulder pain without any antecedents. The diagnostic crite rion is a radiodense structure over the greater tubercle, which is less dense than the mature bone, but well circumscribed, corresponding to the calcium crystal deposits (hydroxy apatite) in the substance of the tendon, which proves an inflammatory process in the area. The treatment is basically symptomatic, since the condition heals spontaneously. Pain kill ers, NSAID are prescribed. A dramatic im-
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provement may b e achieved from a few ste roid inj ections into the subacromial space (not into the tendon ! ) . A further conservative tool is ultrasound shockwave therapy. When con servative therapy is unsuccessful, or extensive depositions lead to subacromial impingement, the focus is to be removed arthroscopically or by open surgery. �
Arthrosis of the acromio-c1avicular (AC) j oint A localized pain develops in the AC j oint mainly in middle aged or older patients, radi ating typically toward the neck. The local ten derness over the joint is also distinctive. Simi larly to other joints the AC changes may be caused by primary or post-traumatic arthrosis. The treatment is basically symptomatic, con servative. In stubborn cases resection of the lateral end of the clavicle and removal of the degenerated disc are justified. �
Disorders of the sterno-c1avicular (SC) j oint These are relatively uncommon. Instabil ity occurs as a part of general joint laxity, which rarely requires treatment, or as post-traumatic instability, when surgical sta bilization is considered. General j oint inflammations and degener ative processes may involve the SC j oint. There are some conditions of unknown origin, that cause deformity of this joint or the medial end of the clavicle (Tietze ' s syndrome, osteitis claviculae, etc.), which require con servative treatment in cases of complaints.
25.6. Glenohumeral i nstability, shoulder dislocation Symptomatic pathologic displacement of the humeral head relative to the g lenoid surface is cal led glenohumeral instability. Partial dis placement of the humeral head is subluxation. If the humeral head leaves the g lenoid fossa in its entirety, dislocation occurs.
The humeral head is usually luxated ante riorly and caudally, and can be reduced in the beginning only with medical assistance, after multiple repetitions, it can even reduce spon taneously. The younger the patient, the higher the probability recurrence of the shoulder dis location. About 96% of the dislocations are anterior or antero-inferior, the remaining 4% are posterior or multidirectional. Etiopathology. The stability of the shoul der is ensured by different static and dynamic factors. The static, passive stabilizers are the thickening of the joint capsule (glenohumeral ligaments), the glenoid labrum, the negative joint pressure and to a lesser extent the joint surfaces. The dynamic, or active stabilizers are primarily the muscles of the rotator cuff, secondarily the shoulder blade stabilizers and ultimately all the muscles in the shoulder gir dle. Shoulder instability may develop for many reasons, the type of the instability also varies. Because of the wide range of etiology the shoulder instability is classified in many dif ferent ways: �
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By the extent of the instability - subluxation - luxation By the direction of the instability - anterior - caudal - posterior - multidirectional By the time of the instability - acute - inveterate - recurrent By etiology - traumatic - habitual - voluntary
History. The majority of the patient men tion some kind of traumatic dislocation and recurrent luxations after that. The circum stances of the injury are essential. The most common anterior luxation typically develops
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) Fig. 25.17. Assessment of shoulder instability with su lcus sign.
following an extension-external rotation movement, while the posterior dislocation is induced by anteflexion and internal rotation. The first anterior luxation most often requires reduction performed by the doctor, later the patients are themselves able to reduce the shoulder. The first dislocation following a real injury may recur later even following the movements of the everyday life (dressing, washing, reaching backward, etc.). After many repetitions, the patient can even pro duce the luxation, this is not necessarily iden tical to the voluntary dislocation, which is usually related to psychiatric problems. Symptoms. Initially the dislocation causes a major sensation, with painful swell ing, elastic blockade of motion and often paresthesiae in the upper limb. If the shoulder is dislocated, the acromion protrudes, a de pression is seen under it and the humeral head is palpated caudally and frontally (anterior dislocation) or toward the back (posterior dis location). In cases of anterior dislocation, if the shoulder is externally rotated in 90 degrees of abduction, at the end point of the range the pa tient indicates that this motion causes discom fort with the feeling that the shoulder will dis locate during the assessment (anterior appre-
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hensive test). In cases of posterior dislocation, anteflexion and internal rotation provoke a similar sensation (posterior apprehensive test) . A further diagnostic sign is the subluxability of the humeral head caudally (sulcus sign, Fig. 25.1 7.), which is tested by axial pulling ofthe relaxed arm. A typical fea ture of multidirectional instability is the mo bility of the humeral head anteriorly or poste riorly (anterior and posterior drawer sign). X-ray signs. The direction of the disloca tion is detected by traditional X-ray test. After reduction, the native film gives information on the shape and direction of the glenoid cav ity, and on the possible bony B ankart' s lesion, (Hill- Sachs ' s lesion, Fig. 25.18. a). In case CT scan can also be done (Fig. 25. 1 8. b, c), CT arthrography is needed to clarifY the state of the bone anatomy and the labrum. MRI or ultrasonography may provide data on the con ditions of the labrum and the rotator cuff. Treatment. In case of acute luxation re duction and fixation of the shoulder in internal rotation for 3 -4 weeks results in lasting symptomless outcome only rarely. Following repeated dislocation the mode of treatment depends on the type of instability and the etiologic factors. If dislocation is intentional, the cause of the personal identity disturbance must be identified (psychiatric illnesses, family or school conflicts) and possibly cured. Disre garding the etiologic factors, ignoring the in stability complaint (the so called skillful ne glect) may be helpful, when the patient' s at tention is turned away from the significance of the instability. In cases of traumatic dislocation rehabili tation care for at least 6 months is essential, the most important elements of which are strengthening of the rotator cuff and scapula stabilizer muscles and improving the proprio ception in the shoulder area. Surgical treat ment is indicated only after failed conserva tive therapy, and the circular loose cap sule-ligament system is tightened (lower capsuloplasty) .
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Fig. 25.18. a: I n a case with rig ht habitual luxation the defect caused by the tear of the glenoid labrum is visible together with the a nterolateral head defect. b: Impression fracture at the posterior part of the humeral head (Hill-Sachs lesion): a image. c: Fracture of the anterior glenoid rim (bony Bankart's lesion) a image.
In case of real recurrent traumatic disloca tion operative procedure is the primary mode of therapy. The type of surgical method de pends on the type of injury and the direction of instability .
The postoperative rehabilitation is deter mined by the technique used. The patient may return to full physical activity (physical work, sport) usually after 6 months. The modem surgical procedures prevent recurrent luxation in 90-95% of the cases.
26.
Jeno Kiss
Disorders of the elbow and forearm
26. 1. Functional anatomy of the elbow and forearm While the movements ofthe glenohumeral joint can be compensated by the sliding of the scapula on the trunk, and similarly the motion restriction of the scapula can be compensated between certain limits by the glenohumeral joint movement, the flexion - extension of the elbow can not be replaced by any other func tion. Pronation - supination of the forearm can be compensated with the elbow extended ?� the movement of the shoulder. At all ages, It IS of utmost importance for every individual �o be �ble to reach both orifices of the gastro mtestmal tract. In case of a severe extension elbow contracture, the mouth is out of reach' in the event of a considerable flexion elbo� contracture, the patient is unable to reach the perineal area. Besides flexion - extension pronation - supination of the forearm is als � important, which is around 90° each. To per . form this complex movement, the integrity of the forearm bones, the attached interosseal membrane, and the proximal and distal ra dio-ulnar joints is essential . In consequence of the shape of the joint surfaces and the complexity of the ligament system, the elbow is a very stable joint. At the distal end of the humerus, the joint between the trochlea and olecranon ulnae ensures flexion - extension, while the joint between the capitulum humeri and the caput radii chiefly plays a role in the rotation and the sta bility of the elbow. Further important compo nents of the stability are the processus coronoides ulnae and the medial - lateral col lateral ligaments. The extension of the elbow is car:� ed ou� � y a single muscle, the triceps brachll, and It IS flexed only by the m. biceps
brachii and m. brachioradialis, but to some ex tent by practically all the muscles that origi nate over the elbow and are attached on the forearm or the wrist. The forearm is rotated by the pronator and supinator muscles with the assistance of the extensors and flexors . It should be pointed out th �t the biceps i � not only a flexor, but also a �Ulte strong supmator. When the palm is fac mg forward, the axis of the extended elbow positi ?ne � ? eside the trunk is 5-1 5 ° of valgus. It IS cntlcal from a surgical anatomical as pect that all three nerves and blood vessels are in the close vicinity of the elbow.
26.2. Assessment of the elbow The basic assessment criteria and methods are fully identical to those described in con nection with the assessment of the shoulder girdle (see details in Chapter 25 .2). In the course of the examination, the direction of movement and the anatomical features of the elbow j oint must naturally be taken into con sideration. A complex upper limb examina tion must always comprise such an assess ment.
26.3. Significant developmental anomalies �
Congenital dislocation of the radial head
This is a com plex developmental anomaly with axial deviations of the radius or the ulna, and the radial head is in a dislocated position. It is relatively rare, and may occur isolated or
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in association with other developmental anomalies.
Clinical symptoms. The deformity is rarely a source of complaint in childhood, and is mostly recognized accidentally. The pronation and supination of the forearm are restricted. In adults, the symptoms are arthrotic in character. Treatment. Reduction should be supple mented with correction osteotomy, and occa sionally with shortening of the radius or the ulna. When the symptoms present in adult hood, resection of the radial head is justified. �
Clinical symptoms. It is typical that the pronation - supination movements are miss ing. Treatment. Attempted restoration of pronation - supination movements through plastic surgery interventions usually fails, fol lowing removal of the bony bridge, even if a soft tissue interpositum is created, recurrence of the synostosis is common.
Congenital radioulnar synostosis
Because of a disturbance in the differentiation of the cartilaginous substance of the forearm bones, the separation of the two bones is in com plete. The synostosis between the radius and u l na is usually proximal to various extents (Fig. 26. 1.).
Fig. 26.2. Fig. 26.1. Bilateral radioulnar synostosis.
Bilateral Madelung deformity; clinical (a) and X- ray pictu re, demonstrating bending of the radius in the volar and ulnar directions (b), the dorsal protrusion of the ulna is clearly visible (c).
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Madelung's deformity
Mode/ung's deformity is a g rowth disturbance
of the distal epiphysis of the radius, resu lting in volar and ulnar bending of the distal radius. Its occurrence is often familial. It is more com mon in girls and often bilateral.
Clinical symptoms. The radius is short ened and the distal end of the ulna therefore protr�des over the wrist to the dorsal surface of the forearm (Fig. 26.2. a-c) . The deformity increases with growth. The symptoms are caused by the restriction of the extension and radialduction movements of the wrist, and by the restriction of the supination of the fore arm. When the deformity is severe, the grasp ing force of the hand may also diminish. Treatment. If the deformity is extensive, osteotomy on the distal metaphysis of the ra dius may correct the curvature of the bone. The ulna is shortened at the same time, or its distal end is resected.
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26.4. Acquired disorders of the elbow �
Arthrosis (osteoarthritis) of the elbow
Arthrosis is a painful restriction of the move ment of the elbow, with radiological changes, which can be primary or secondary (outcome of inflammation, degenerative, trauma, infec tion, etc.). Primary osteoarthritis involves mainly individuals over 40 who are engaged in heavy labor. Secondary changes may occur at any age, with varying incidence.
Etiopathology. In primary arthrosis the changes observed are similar to those in other large j oints . The changes primarily affect the j oint surface; the involvement of the radio humeral j oint precedes the ulnohumeral changes. In the late stages, osteophytes are typical at the tip of the olecranon and the coronoid process, obstructing the movements. In secondary arthrosis the type of the changes is dependent on the etiology factor (Fig. 26.3.). Clinical symptoms. Pain initially appears only after the exertion of physical effort, but it
Fig. 26.3. Elbow destruction caused by rheu matoid arthritis (lateral and anteroposterior X-ray)
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later becomes permanent. Early restriction o f the extension and supination is typical, but later movement is restricted in every dimen sion. Cracks van be heard and friction felt while moving the joint. The osteophytes sur rounding the ulnar nerve may cause the com pression of the nerve. In case of rheumatoid arthritis, swelling and occasional instability are also observed. Treatment. In any form of elbow osteo arthritis, conservative treatment is applicable for a relatively long period. Conservative treatment is employed to relieve pain, to maintain motion range and to protect the mus cles. In cases of systemic diseases (rheuma toid arthritis, hemophilia, gout, etc.) treatment of the basic disease is essential. If operative therapy is needed, joint- preserving and j oint-replacing procedures are available. Joint-preserving surgery involves arthroscop ic lavage of the joint, removal of inflamed synovial villi, and in rheumatoid arthritis ar throscopic or open synovectomy. Predomi nantly in post-trauma cases, the scarred j oint capsule and the hypertrophied callus that in hibits movements can be removed (arth rolysis). In severe cases, an elbow endoprosthesis is implanted to improve the quality of life (Fig. 26.4. a, b). This endoprosthesis ensures proper movement and stability, but the pa tients must refrain from excessive physical activity. �
during work (tennis players, typists, etc.). It is common among heavy laborers. Both variet ies are most frequent between the ages of 40 and 50. Lateral epicondylitis is 5 times more common than the medial form.
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Epicondylitis humeri lateralis et medialis (tennis elbow, golfers' elbow)
Lateral epicondylitis of the humerus is called tennis elbow, and medial epicondylitis is cal led golfers' elbow. The pain presenting at the lateral epicondyle is a consequence of overloading of the forearm extensors, while the pain occurring at the lateral epicondyle is the outcome of excessive use of the forearm flexors.
The symptoms are often detected in young adults who overload the extensor muscles of the wrist and fingers while playing sports or
Fig. 26.4. X-ray pictu res of an elbow following prosthesis im plantation in rheu matoid arthritis. Anetroposterior (a) and lateral view (b)
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Etiopathology. Repeated trauma and de generative changes in the area of the origin of the muscle result first in microscopic tears, then, in about 30% of the cases, macroscopic tears are observed. Clinical symptoms. In tennis elbow, the patients complain of gradually developing pain in the lateral part of the elbow. The pain initially occurs during active movements of the wrist and when grasping obj ects, but later it may be present even at rest. The pain in creases when the forearm extensors are ac tively stretched. The pain can be so intense that the patient is unable to grip, and drops grasped and lifted obj ects. The lateral epicondyle and the extensor muscles are ten der to palpation. Golfers ' elbow causes simi lar symptoms in the medial epicondyle area, and the medial epicondyle is tender. Differential diagnosis. In the case of ten nis elbow, radial nerve compression, cervical root compression and j oint ailments are to be considered. In golfers elbow, the compression syndrome of the ulnar nerve, collateral liga ment disorders and also cervical root com pression must be differentiated. Treatment. Conservative treatment, usu ally lasting for a number of months, proves successful in 90% of the cases of both disor ders. This includes the avoidance of overload ing movements, resting the wrist and hand if necessary, padding applied distally from the nonsteroidal ongm, anti muscle inflammatory drug creams or tablets, local lignocaine + steroid injections into the painful areas, and physiotherapy. If conservative treatment does not bring relief within a rela tively long period, various surgical interven tions are to be considered. �
Olecranon bu rsitis
Inflammation developing in the bursa over the olecranon is a relatively common condi tion. The inflammation is caused by chronic ir ritation of the tip of the elbow, metabolic dis ease, a blow in the elbow region, or hematogenous bacterial infection.
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Clinical symptoms. Depending in the eti ology, in acute cases moderate or severe swelling of the bursa, fluctuation and tender ness occur, in septic inflammation, hyperemia and warmth are detected. In case of chronic bursitis, the swelling varies, tenderness is moderate, and fluid accumulation, thickening of the wall of the bursa, and loose bodies in the bursa are palpable. Treatment. In case of sterile inflamma tion, the usual anti-inflammatory methods, and occasionally the drainage ofthe bursa and steroid inj ection are considered as conserva tive therapy. In stubborn cases, surgical re moval of the bursa is recommended. �
Instability of the elbow
Slack col lateral ligaments, usually acquired, but rarely of congenital origin, or deformed bones of the elbow result in instability of the joint.
Etiopathology. The elbow is a relatively stable j oint, nevertheless, elbow luxation due to high-energy injuries is the second most common dislocation after that of the shoulder. The instability may be acute or chronic. Chronic instability is relatively rare. With re gard to the direction, it may be medial, lateral, anterior or posterior, or usually some combi nation of these. Clinical symptoms. Acute elbow disloca tion is usually not difficult to diagnose, con sidering the obvious deformity and elastic block of movement. It is more difficult to prove chronic instability, which causes only subluxation. Even an experienced examiner can only establish proper diagnosis after sum marizing the circumstances of the onset of the complaints, the results of careful physical as sessment and an X-ray examination. Treatment. If no fracture is involved, an acute elbow dislocation, requires closed re duction and depending on the degree of insta bility following reduction, transitional immo bilization ( 1 -3 weeks), followed by early functional treatment. In cases of chronic insta bility, depending on the characteristics and
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extent and also on the physical activity and age of the patient, conservative treatment is advised: modification of the sporting and vo cational activities, strengthening of the mus cles bridging the elbow and occasionally ex ternal orthesis. Surgical procedures depend on the cause and type of the instability. �
Acquired axial deformities
In consequence of very frequent elbow frac tures in child hood, varus deformity is com mon due to the damage to the growth plate.
a b
A mild deformity is usually merely a cos metic problem. In more severe cases, how ever, the entire function of the upper limb is altered, especially when the varus deformity is complicated with a flexion contracture of the elbow. The diagnosis is unmistakable in view of the characteristic clinical and radiological signs. Milder cases do not need treatment. In more severe cases of varus with a flexion contracture, correction osteotomy in the hu merus distal metaphysis may be justified (Fig. 26.5. a, b) . �
Tunnel syndromes of the elbow
These clinical entities are characterized by pain and occasional paresthesia radiating from the elbow to various areas of the fore arm or of the hand. The ulnar nerve is most commonly involved. Compression of radial nerve is less frequent, and the median nerve compression is rare.
Fig. 26.5. 40° post-traumatic varus deformity of the rig ht el bow of a 10-year-old child (a), and following correc tion by humerus osteotomy (b)
Etiopathology. Nerve compression may take place where the nerve passes certain ana tomical locations and is compressed by a scar, an abnormal muscle belly, an osteophyte, a callus or a tumor. Stretching or tension of the nerve may be caused by severe post-traumatic or degenerative deformity of the elbow (see also in Chapter 1 7) . Treatment. Conservative therapy may be successful only in minor cases. Rest and use
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of a night splint in attempt to decrease the edema caused by the compression of the nerve. In more severe cases, surgical decom pression of the nerve may be necessary.
26.5. Volkmann's ischemic contracture of the forearm and the hand This clinical picture arises following injuries or the application of a tight plaster of Paris ban dages. The typical contractures are produced by the ischemic necrosis of the forearm mus culature. The syndrome was first described by Volkmann.
Etiopathology. This syndrome usually appears following a supracondylar humerus fracture in childhood, elbow luxation, or use of a tight plaster of Paris bandage applied for some other reason, but it can also be caused by a misplaced tourniquet cuff. Ischemic necro sis develops in the foreann musculature, re sulting in scarring of the muscles. In certain cases, damage to the ulnar, radial and median nerves is observed. Clinical symptoms. Early symptoms may be identified merely a few hours after the in jury. The hand is cool, swollen and purplish blue. A change in sensitivity and loss of mo tion to various extents may occur in the fin gers. The developed Volkmann contracture displays a typical picture: flexion contracture of the elbow, pronation ofthe foreann, flexion and a mild ulnar deviation contracture of the wrist, extension in the metacarpophalangeal joints, flexion in the interphalangeal joints, and an opposition contracture of the extended pollex. The fingers exhibit a claw hand defor mity (Fig. 26.6.) .
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Treatment. Prevention is essential. The risk of an ischemic contracture must be borne in mind in cases of elbow injuries, and reduc tion and positioning of the plaster of Paris must be carried out accordingly. A circular plaster must not be used in elbow injuries. The circulation must be checked regularly, and the attention of parents must be drawn to this. I f a minimal circulatory, sensory o r motor distur bance is detected in the hand, the bandage must be loosened immediately and close ob servation must follow. An important clinical symptom is the pain on passive flexion of the fingers, due to the increasing pressure in the muscle compartments. If this state does not improve quickly, fasciotomy of both flexor and extensor compartments on the elbow and the foreann must be perfonned immediately in order to decrease the pressure in the com partments and restore circulation. When a Volkmann contracture has already developed, the treatment is long-lasting. Contracture of the fingers may be improved by exercises, or in more severe cases by me chanical methods. In mild cases, these meth ods may even result in full recovery. In severe cases a surgical solution may be needed. De pending on the extent of the defonnity, the hand function may be improved by tenotomies, muscle transfers or wrist arthrodesis.
Fig. 26.6. Typical clinical picture of the Volkmann's contractures
Fe r e n c M a d y
27.
Disorders of the hand and the wrist
27. 1. Functional anatomy of the hand and the wrist Beside its motor function, the hand also acts as a sensory organ. The bones of the hand are the carpal bones, the metacarpal bones and the phalanges. Special attention should be paid to the first metacarpal bone due to the � a?dle joint and the metacarpophalangeal Jomt. The other metacarpals are characterized by the shape of their distal articular surface (ball joint). As concerns the joints, the loose ligaments ?� the saddle joint (the first carpometacarpal Jomt) and the eccentric location of the liga ments of the second to fifth metacarpo phalangeal joints should be taken into consid eration. The muscles of the hand are divided into three groups: the thenar, mesothenar and
Fig. 27.1.
a
hypothenar groups . These muscles provide the fine (but weaker) motions of the fingers . The peripheral nerves (the median and the ulnar nerve) reach the hand through pre formed anatomic tunnels (the carpal tunnel and Guyon ' s tunnel). Both nerves are of mixed type. There is a separate nerve on both sides of each finger. The blood supply of the hand is provided by the end-branches of the radial and the ulnar arteries. The anatomic structures of the hand are covered by the palmar fascia . Clinical examination of the hand. The easiest and quickest way to examine the hand is to apply the scheme described by Erik Moberg, which consists of four steps (Fig. 27.1 a-d) :
b
The Moberg's scheme: a method for rapid assessment of the hand-function.
d
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a) Examination of the integrity of the long flexors and extensors. The patient is re quested to extend the fingers fully, and then to flex them slowly while keeping the metacarpophalangeal joints extended. If this can be accomplished properly, the long flexor and extensor muscles and ten dons are intact. When the superficial flexor tendons are examined, the fingers are kept in extension, and the proximal interphalangeal j oints are then flexed one by one. If there is a lesion of the superficial flexor tendon, the finger cannot be flexed in the proximal interphalangeal joint in this way. b) The motor function of the ulnar nerve can be checked by adduction of the thumb and ab- and adduction of the long fingers. A weakened thumb adduction indicates a de teriorated function of the ulnar nerve. The patient is requested to hold a sheet of paper between the thumb and the edge of the palm. If the adduction function preserved, considerable force is required to remove the paper sheet. The abduction strength of the fingers is tested against resistance. The closing strength of the fingers is checked by attempting to open them. If the patient resists well, the innervations of the intrin sic muscles are intact. c) The median nerve is responsible for the opposition-reposition of the thumb. When testing the opposition, the patient is re quested to form a circle with the thumb and the little finger and to keep them tightly together. If the fingers can be sepa rated easily, the opponent muscle strength is weakened. d) The last step is to test the sensitivity of the hand according to the supply area of the three nerves . A small blunt obj ect is to be used for the examination. If the superficial sensation is preserved, the patient reports good sensation in the palm, in the lateral aspects of the fingers and in the palmar as pect of the fingertips . The limit between the sensory areas of the median and the ul nar nerves is the midline of the ring finger toward the wrist crease. The sensory areas
on the dorsal aspect of the hand differ from those in the palmar aspect. Distally from the proximal interphalangeal joints, the ar eas are the same, but the rest of the dorsal aspect is supplied by the radial and ulnar nerves. The border between them is the midline of the middle finger. More accurate methods for examining the sensitivity of the hand are the two-point dis crimination test; the touch test, the pain- and the pick-up tests. Recognition of objects is en sured by the simultaneous activity of the ther mal, pain and touch receptors. Ifthese are sev ered, the patient is not able to recognize smaller obj ects without eye control. If there is only a minor impairment, the two-point dis crimination increases from the normal 1 -2 mm up to 5-6 mm or even more. In case of more severe nerve damage, all the sensory functions are affected and the function of the hand deteriorates considerably. Both the regulation of the perspiration and the sensitivity of the hand are linked to the digital nerves. This can explain the phenome non that, if the digital nerve is severed, the skin is dry and the perspiration is impaired. On percussion of the damaged peripheral nerve, electric shock-like pain is evoked at the site of the nerve injury or nerve compression ( Tinel' s sign). The site of the nerve damage can therefore be accurately determined. The blood supply for the hand is provided by the radial and the ulnar arteries. The interosseal artery also provides some branches flowing from the wrist. These pres ent several anatomic variations and do not provide sufficient blood supply for the hand by themselves. The neutral 0 method is used to describe the range of movement of the hand. The joints are in the 0 position when the fingers are ex tended. The functional position of the hand: The optimal grip position is the best for the function of the hand (Fig. 27.2.). The functional position is borne in mind when the hand is immobilized: the articular ligaments adapt quickly (even within one or
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Surgery on the hand is performed under re gional or general anesthesia and with the use of tourniquet bloodless . The hand may be affected b y numerous or thopedic disorders . However their incidence can be different.
27.2. Congenital anomalies of the hand Fig. 27.2. Functional position of the hand. The metacarpo phalangeal joints are flexed at 60° in the second and 90° in the fifth finger. The wrist is in 30° dorsiflexion. The proximal interphalangeal and distal inter phalangeal joints are bent at 20-30°. The best grip is provided in this position of the hand.
two weeks) to the length they had during im mobilization. This explains the fixed deformi ties developing during immobilization. These deformities can be prevented by immobiliza tion in the functional position.
The upper and lower extremities develop simultaneously between the fourth and sev enth week of the embryonic life . The hand de velops through the growth and differentiation of the limb buds. If the embryo is exposed to genetic or direct toxic effects, various devel opmental failures may occur in the hand (and often in the foot) . �
Syndactyly In syndactyly, two or more fingers are par tially or completely j oined. Sometimes only
Fig. 27.3. Types of syndactyly a: Sketch of an X-ray image indicating that in cutaneous syndactyly the bones are not affected and separated as in a normal hand. b: In osseous syndactyly the bones are missing or joined. c: In spoon-hand, all the fingers have a common skin glove; certain bones are missing or deformed.
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Fig. 27.4. Cutaneous syndactyly. The middle and ring fingers are joined in their entire length. Due to the growth of the fingers, the otherwise longer middle finger bends ulnarward. After separation, the fingers continue growing proportionately.
the skin is common (cutaneous syndactyly), but in more severe cases, the bones are also common (bony syndactyly) . In the most se vere case, all five fingers are involved (spoon hand) (Fig. 27.3. a-c). In cutaneous syndactyly, the otherwise separate fingers are covered by a common skin (Fig. 27.4.). The nail may be deformed at its base. Possible variations of the blood ves sels should be clarified before surgery is de cided on. Angiography is to be performed to exclude any vascular abnormalities. Separation of the fingers needs an experi enced surgeon. A preferred age for such inter ventions is at the age of 2-3 years . Accurate reconstruction of the web between the fingers is essential in order to avoid scars and conse quent fixed deformities. �
Ectrodactyly This is the congenital absence of a fin ger(s). �
Cleft hand This congenital absence of one or more of the central (second to fourth) fingers is also called lobster-claw hand. The cosmetic ap pearance is usually worse than the functional impairment. �
Polydactyly This denotes the presence of an extra digit, which may be complete or the rudimentary part of a finger or thumb.
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Congenital trigger thumb This involves congenital stenosis of the flexo tendon sheath ofthe thumb. Surgical in cision of the tendon sheath is indicated at an early age. Congenital anomalies of the hand exhibit great variety and many combinations are ob served. The optimal timing for surgery is around the age of 2-3 years . If several interventions are necessary, these should be completed be fore the first school year.
27.3. Acquired hand disorders 27.3.1. Avascular necrosis of the carpal lunate bone (Kienbock's disease) This d isease comprises necrosis, fragmenta tion and collapse of the l unate bone in adults; it is of unknown etiology. It was first de scribed by KienbOck who presumed a blood supply distu rbance leading to bone necrosis.
The pathomechanism and the outcome dif fer from those in juvenile osteochondritis. In Kienbock' s disease the necrosis of the lunate bone is definitive, while in juvenile osteo chondritis the necrosis is followed by regener ation of the bone.
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The disease occurs most frequently in males who perform heavy manual work, (re peated micro traumas, use of a pneumatic hammer, or activities in a smithy) around the age of 3 0 . Clinical features. Pain o n palpation and a painful swelling of the dorsal aspect of the wrist. The patients usually connect the onset of the symptoms to some trauma in the past. Pain increases upon exertion. Movements of the wrist are painful and limited in all three planes . In a later stage, the range of extension decreases markedly, and a fixed flexion deformity and atrophy of the forearm muscles can be observed.
Fig. 27.S. Avascu lar necrosis of the lu nate bone. The X-ray im age clearly shows the smaller, sclerotic, deformed lunate bone with cystic lesions.
Radiological signs. In the early stages, there are no characteristic radiological alter ations. Later, the bony structure of the lunate changes, and it exhibits an increased density with cystic lesions . In the lateral view, there is a dip in the lunate (Fig. 27.5.). Treatment. Physiotherapy and immobili zation of the wrist (splint or brace) in the early stage. The administration of nonsteroidal anti-inflammatory drugs can relieve the pain considerably. If conservative treatment fails, surgery is indicated.
27.3.2. Cyst and pseudoarthrosis of the scaphoid bone Fracture of the carpal scaphoid bone, the most common fracture of the carpus, can re sult in nonunion in a considerable number of cases.
Fig. 27.6. Pseudo-articulation of the scaphoid bone. The ar row indicates the fracture. In the radiograph, the en largement of the false joint line is clearly visible. The bone has not yet deformed.
This fracture is often not diagnosed at the first examination. The precarious blood sup ply of the scaphoid is at risk and pseudo arthrosis may develop in the fracture line, fol lowed by necrosis of the proximal broken fragment (Fig. 27.6.). Cystic changes may oc cur in the fracture line or even without a frac ture.
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Clinical features. There is usually an old injury in the medical history which is often re vealed only when the patients are requested to recall a possible injury that they suffered in the past. The fovea radialis (fossa tabatiere) is swollen. Dorsiflexion and radial abduction of the wrist provoke pain. There is tenderness over the scaphoid bone. Radiological signs. If a scaphoid fracture is suspected, radiographs of the wrist are taken in four planes (scaphoid views) . Treatment. If nonunion has developed, surgical treatment is usually performed. Sev eral techniques are available, but their princi ples are the same : removal of the dead bone fragments; filling with cancellous bone grafts; and internal fixation (pin, screw, Ender 's plate etc.).
27.3.3. Osteoarthritis (OA) of the wrist and the hand Osteoarthritic changes a re more common in the hand than in the hip or knee joint. They are of great importance because they can compromise the function of the hand.
Etiology. A previous injury, an articular fracture or chronic exertion (activities of a typist or a decorator) may be found in medical history. It may also develop spontaneously in the elderly. Clinical features. There is gradually in creasing pain in the j oints of the hand, usually in the midline of the dorsal aspect of the wrist. The affected j oint becomes swollen and pain ful, and the strength and ability to grip obj ects diminish. The most commonly affected joints are the radiocarpal joint, the saddle j oint and the first metacarpophalangeal joint. Radiological findings. The usual osteo arthritic changes can be detected. In wrist osteoarthritis, the styloid of the radius be-
Fig. 27.7. Osteoarthritis of the saddle-joint. The joint su rfaces of the os trapezium and the first metacarpal that are facing each other are deformed, the bones are sclerotic. The first metacarpal subluxates towards the radial.
comes elongated. The radiocarpal joint space becomes thin first on the radial side. The proximal carpal bones flatten and deform, and degenerative bone cysts may appear. In osteoarthritis of the saddle joint, the base o fthe first metacarpal subluxates or may even dislocate. The radius of the thumb short ens (Fig. 27.7.). Osteoarthritis of the radiocarpal joint (wrist) is treated first conservatively, with im mobilization, pain control and physiotherapy. (Fig. 27.8.). In contrast with the earlier practice, ad ministration of topical steroids is to be avoided. The use of cortisone preparations can result in disintegration of the substance of the joint capsule, ligaments and tendons, lead ing to an impaired mechanical quality.
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(tendon sheath and bursa). Edema and soft tissue distension can lead to a deterioration of the circulation causing necrosis and/or loss of a part of the hand. �
Tendovaginitis crepitans
This is a non-pyogenic inflammation of the tendon sheath. Its mildest form occurs in the dorsum of the wrist due to overexertion of the hand (typist or pianist activities). Synovial effusion develops inside the tendon sheath and fibrin precipitation occurs there.
Clinical features. Pain and swelling in the dorsal aspect of the wrist. Crepitation (a snow- crunching-like sensation) may be expe rienced along the extensor tendons while the wrist is moving. There is tenderness on palpa tion over the swollen j oint. The grip strength of the hand diminishes considerably. Treatment. Immobilization of the wrist and the fingers for 2-3 weeks and administra tion of nonsteroidal anti-inflammatory drugs. Fig. 27.8. Osteoarthritis of the radiocarpal joint. The joint sur faces are thin, and the styloid process is pointed. The scaphoid bone displays pseudo-articu lation (secondary wrist osteoarthritis)
Immobilization can be achieved by the ap plication of plaster of Paris splints or wrist braces. Other types of braces can involve the saddle joint kept in opposition.
27.3.4. Inflammatory processes During work, the hand is exposed to a wide variety of physical effects. Besides overexertion, injuries varying in severity can occur. Injuries in the skin enable pathogenic mi crobes to penetrate the hand, causing various pyogenic infections. Septic conditions of the hand are dangerous because the infection can spread rapidly along the anatomic structures
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Stenosing tenosynovitis
This condition is characterized by stricture of the tendon sheath. Depending on which ten dons are affected, De Quervain's disease and trigger finger (or trigger thumb) are distin guished.
De Quervain ' s disease. Chronic inflam mation and stenosis occur in the common ten don sheath of the abductor pollicis longus and extensor pollicis brevis tendons at the level of the styloid process o fthe radius. The mobility of the thumb is reduced and the grip strength is greatly diminished due to pain (Fig. 27.9.). Clinical features. Besides the pain there is swelling and tenderness on palpation in the radial aspect of the wrist. The pain is severe on palpation in the styloid process. Finkelstein ' s test is usually positive : pas sively abducting the hand ulnarward provokes pain at the site of the tenderness. The inj ection of hydrocortisone results in only short-term pain relief, therefore this type of drug should
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be avoided. Surgical incision of the tendon sheath results in a definitive recovery. Trigger finger and thumb (digitus saltans -
pollex saltans). A nodule is observed on the tendon with relative stenosis of the tendon sheath. As the tendon glides through the sheath, a snapping movement can be seen and palpated usually accompanied by pain. I n more severe cases, there is not enough space for the nodule to glide through the constric tion. The patient is u nable to flex or extend the affected finger. The stenosis is located at the level of the metacarpophalangeal joint.
At the entry of the tendon sheath, there is a strong fibrous annulus, stabilizing the tendon on its base thus providing smooth gliding. Either the tendon sheath becomes thicker due to inflammation or the tendon develops a swelling (intratendinous ganglionic cyst or tu mor) . This can lead to relative stenosis there. The tendon moves through the stenosis only upon forced flexion or extension, accompa nied by a snapping (Fig. 27.1 0.). In adults,
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Fig. 27.9. The abductor pollicis longus tendon (1) and the extensor pollicis brevis tendon (3)in the first dorsal compartment. Close attention should be paid to the radial nerve and its sensory branches. The sur gery comprises the incision of the first dorsal tendon compartment (2).
trigger finger usually occurs on the long fin gers. Trigger thumb may be observed congen itally in children. If the constriction is severe, the thumb remains fixed in flex ion (congeni tal pollex flexus ). Treatment. Conservative treatment usu ally fails or provides only temporary results. Surgical treatments results in an immedi ate and definitive recovery, and thus attempt ing conservative methods for a longer period is meaningless. Surgery comprises longitudi nal incision of the constricted part of the ten don sheath.
27.3.5. Ganglion of the wrist and the hand Gangli onic cysts usually arise from struc tures lined with synovial membrane Uoints, bursae and tendon sheaths). They contain yel lowish synovial fluid. They have an outer fi brous capsule and an inner synovial mem brane. Fig. 27.10.
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Mechanism of digitus saltans: the thickening on the entry of the tendon sheath or on the tendon itself blocks the movement of the ten don. Passed this stenosis the tendon moves freely. 1. metacarpus, 2. flexor tendon, 3. thickening of tendon, 4 and tendon sheath
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Fig. 27.12.
Ganglion of the hand. The cystic lesions arise most frequently from the dorsal capsule of the scapholunate joint.
Dupuytren's contracture. A strong nodular thicken ing affects the palmar fascia above the IV. ray, which decreases significantly the range of movement in the MP and PIP joints.
Pathology. The cause of ganglionic cysts is unclear. They commonly develop on the dorsal (Fig. 27.1 1 .), or occasionally on the palmar surface of the wrist. Ganglionic cysts in the wrist area are usually attached to the capsule of the radiocarpal joint. While the cysts are growing, tissue around the cysts is lifted, i. e. tissue wraps around the cyst. In case they develop on the palmar surface, they may encompass the radial artery.
carpophalangeal j oint is fixed in a 90° flexion (Grade Ill), and then a proximal inter phalangeal flexion contracture occurs (Grade IV, Fig. 27.1 2.) and the patient is unable to move the finger. The other fingers are much more rarely involved. The sensory ability of the fingers is not af fected. Repeated trauma may be a cause but more than half of the patients have never been heavy physical workers .
27.3.6. Dupuytren ' s contracture
Pathology. The nodules initially consist of curly connective tissue bundles rich in cells and poor in fibers . These bundles may extend to the distal phalanges and start to shrink. At this stage mature connective tissue with an in creasing amount of collagen fibers can be de tected. The subcutaneous tissue between the cord and the skin gradually disappears and the thick palmar fascia is covered by only a very thin layer of skin.
This is a benign but aggressive superficial proliferative fibroplasia involving shrinking of the palmar or plantar fascia and resulting in contracture of the finger joints.
Clinical features. The lesion usually be gins with a small nodule (rarely several nod ules) situated in line with the ring or the little finger (Grade I). After a few months or even a year, these nodules develop into a cord (Grade II). In parallel with its thickening, extension of the affected finger becomes limited and a flexion contracture develops. Later the meta-
Treatment. In the early stages, the pa tients usually have no complaints and only ob servation is necessary. When the bundles start to form, surgery can be performed.
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27.3.7. Tumors and tumorous conditions i n the hand Any tumor that may arise in the locomo tion system and the dermal tissue may also oc cur in the hand, but each has its own charac teristic incidence. Malignant tumors are rare. Among the tumorous conditions, seba ceous cysts are often found beneath the skin of the palm or the fingers. These cysts grow slowly, lying just underneath the skin. They are bordered by a white and strong fibrotic capsule filled with a white mass consisting of liquefied dermal elements, sebum and corneous debris. Among benign tumors, benign synovioma, the giant cell tumor of the tendon sheets, is frequent. This tumor is usually
found in the fingers and grows slowly. The X-ray films reveal a lytic lesion with a sharp margin (like an impression) on the bones next to the tumor. When surgically exposed, a grayish- white lobular structure with yellow ish spots in some places can be seen. The tu mor can be easily dissected from the sur rounding tissue, but often involves as much as three-quarters of the circumference of the digit. After excision, recurrence often occurs. The most common benign tumor is the enchondroma, which is a tumor of cartilagi nous origin (See chapter 2 1 , Fig. 2 1 . 1 5 .). Malignant tumors in the hand are ex tremely rare. Among these, synovial sar coma and chondrosarcoma are relatively of ten observed.
28.
Arpad B e l lyei, J6zsef La katos, M i kl6s Sze n d r6i
Hip disorders
28. 1. Pediatric hip disorders 28. 1.1. Normal development of the hip joint �
Development of the acetabulum and pelvis The three bones that together form the acetabulum and the pelvis develop from three ossifying centers . The bony center of the iliac bone, which forms the upper part of the acetabulum appears in the third embryonic month, followed in the fourth month by the ossifying center of the sciatic bone, which forms the posterior part of the acetabulum, and in the sixth month by the ossifying center the of pubic bone, which forms the anterior part of the acetabulum. The ossifying centers rapidly develop, and at the time of birth these three bones are divided by the Y-shaped growth plate located in the depth of the acetabulum, ossification occurring at the end of development, between the ages of 1 4 and 1 6 years . The growth of the edge of the acetabulum is controlled by an extra ossifying center (os acetabuli) . The fully developed acetabulum is a 1 70- 1 75° segment of a sphere that contains loose fat and connective tissue in its base (pulvinar acetabuli). There is a hyaline cover only on that half-moon-shaped part, which is in contact with the femoral head. The fibrous - cartilagi nous rim on the edge of the acetabulum, the acetabular labrum, makes the cup deeper, so that it contains almost three-quarters of the femoral head. The femoral head is held in a central position in the acetabulum by the cap sule, the enforcing ligaments and the muscles bridging over the joint. The posterior bony part of the acetabulum (the sciatic bone) is
better developed and covers the femoral head better than the anterior part (the pubic bone; Fig. 28.1.). The anterior segment of the femo ral head is only partially covered; the further shell is provided by the active anterior acetabulum wall, the tense substance of iliopsoas muscle, which is of maj or clinical significance. Ossification of the proximal and middle parts of the femoral bone is organized by three centers : in the second embryonic month, a bony center appears in the middle of the diaphysis and progresses in both directions of the diaphysis. The epiphyseal bony center of
Fig. 28.1. Right hip frontal view: the bone cover is less anteri orly than posteriorly.
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the femoral head emerges i n the fourth-sixth month of extrauterine life, the ossifying center of the greater trochanter at the age of three years, and that of the lesser trochanter at the age of 8. The epiphysis later forms the j oint; it is covered by hyaline cartilage, while the apophysis serves as the origin and attachment for muscles. �
Progress of collodiaphyseal angle In adults, the angle between the femoral neck and stem is 1 25 - 1 3 5 ° . This angle varies from early embryonic life to puberty. In em bryonic, life the angle first decreases, then gradually increases, and its average value at birth is 1 40°. During the first year it increases to 1 48°, and it then gradually decreases to the adult level. If this angle exceeds the normal value for the given age, the condition is called a valgus deformity, if it is less, it is a varus de formity. Accordingly, mild physiological coxa valga is observed in early infancy rela tive to the adult situation. When the roof of the j oint is steep and dysplastic, and its covering
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pressure effect on the loaded femoral head is therefore insufficient, valgus hip develops. �
Development of antetorsion Under normal circumstances, the projec tion of the femoral head and neck and transcondylar axis in the horizontal plane has an anteriorly open angle; this is the angle of antetorsion (Fig. 28.2.). The origin of this an gle is the forward torsion of the proximal fem oral bone (the head looks forward and the greater trochanter slightly rearward). The av erage value of antetorsion in adults is 1 0- 1 4°. �
Blood supply of the proximal femoral bone The blood supply in extrauterine life must be considered in three phases : - Between the ages of 1 and 3 years, the blood supply of the proximal two-thirds of the femoral head, the epiphysis, is provided via the artery of the liga mentum teres capitis femoris from the arteria obturatoria (Fig. 28.3.). The growth plate is located distal to the epiphysis; it is an avascular area pre senting a vascular blockage between the epiphysis and the metaphysis.
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Fig. 28.2.
Right hip frontal view: the collodiaphyseal (a) and antetorsion (�) ang les; the line d rawn through the distal femur condyles represents the frontal plane.
Fig. 28.3. The ligamentum teres capitis femoris with the sup ply artery.
28. Hip disorders
- Between the ages of 3 and 1 4 years the proximal epiphysis of the femur is subject to relative ischemia, since the capacity of the arteria obturatoria is then greatly decreased. From this period, the blood is supplied to the proximal epi physis by the lateral epiphyseal artery related to the vessel plexus around the metaphysis (a. circumflexa femoris lateralis et medialis). This vessel runs around the growth plate laterally and posteriorly and then enters the j oint space, reaching the ossifying center of the epiphysis and providing the blood supply directly (Fig. 28.4.). This means that a short segment of the supply artery runs unprotected, in an extraosseal and intracapsular position and is exposed to injuries. In this period, any process under the tense hip joint capsule (syno vitis or edema) may occlude the supplying artery, causing necrosis (see Perthes ' disease). - Around the age of 14 years, the growth plate gradually ossifies, and the intra osseal arteries of the metaphysis
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gradually invade the distal part o f the epiphysis and take part in its blood supply. �
Nerve supply of the hip j oint About two-thirds of the hip j oint is sup plied with sensory fibers by the obturator nerve, which originates from segments L2-4 and runs in the medial edge of the psoas maj or muscle, reaching the medial aspect of the thigh through the lateral - upper aspect of the canalis obturatoria. It provides sensory branches to the hip joint, and then runs dis tally between the adductor muscles providing motor branches most of the adductor muscles and a sensory branch for the small skin area at the distal - medial aspect of the thigh and the knee. The essence of these features is, that in adults, but particularly in children, the pain re lated to hip disorders appears not so much in the hip area, but also in the distal thigh or in the knee.
28. 1.2. Congenital dislocation and dysplasia of the hip Synonyms of this condition are hip dysplasia, acetabulum hypoplasia, congenital dislocation of the hip, developmental disloca tion ofthe hip, and dislocation of the hip in in fants. Definition. This is congenital dysplasia of the acetabulum, which may lead to either intra- or extrauterine consecutive hip dislocation.
Fig. 28.4. The lateral epiphyseal artery runs round the growth plate and supplies the epiphysis.
Pathologic forms - The mildest form is hip dysplasia (acetabulum hypoplasia), when the femoral head is located centrally in the acetabulum, but the acetabulum is underdeveloped, and the acetabulum angle is steep (Fig 28.5.) . - Subluxation: In cases of maj or acetabulum hypoplasia, the femoral head may be displaced cranially. The cartilaginous elements of the aceta-
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Pathologic forms of hip d islocation: dysplasia, subluxation and luxation.
bulum and the femoral head are still partially coupled. - Luxation: The femoral head is displaced proximally and dorsally from the acetabulum. Incidence. Hip dysplasia is classified as a frequent developmental disorder; its inci dence in Hungary is estimated to be around 0 . 5 % . This includes both dysplastic and dislo cation cases which require treatment. Its incidence is dependent on the geo graphical regions and on the ethnicity. In Cen tral Europe the incidence is high, but in Negro sub race and in China the incidence is very low. In Japan however the incidence is very high. Hip dislocation is 6 times more frequent in girls than in boys . This entity was already described by Hip pocrates, however, the current nomenclature and approach are associated with the activity of Lorenz ( 1 895). He stated, that children are not born with hip dislocation, but only with a predisposition; the dislocation occurs only as a result of walking - loading, due to the under developed hip j oint. As early as 1 879, Roser explained that hip dislocation, or a predisposi tion to it, can be recognized in newborns, and added, that these children may heal, provided they wear special nappies right after birth, which ensure an abducted position. Hilgenreiner introduced the term hip dys-
plasia, denoting the maldevelopment of the entire hip joint. As proposed by the European Pediatric Orthopedic Society, the present terminology is: developmental dislocation of the hip (DDH) . Etiopathology. DDH is a multifactorial developmental abnormality. The term multi factorial means that both the inheritance fac tors (mainly a predisposition) and the envi ronmental factors acting collectively are re sponsible for the occurrence of luxation. The genetic, hereditary factors (predispo sitions) appear as acetabulum hypoplasia, which is the primary cause of this polygene inheritance. Hereditary factors may also be manifested as the dominantly inherited gen eral joint laxity. Both intra- and extrauterine environmen tal factors may contribute. Intrauterine causes include any compartmental disproportion (such as breech presentation, other position ing disorders, or tight intrauterine situations), and transitional joint laxity is particularly im portant. The essence of this is that in the sec ond and third trimesters of pregnancy, the lev els of estrogen and progesterone increase in both the mother' s and the embryo ' s blood, since their circulation is common. It is well accepted that both estrogen and progesterone cause j oint laxity. The transitional joint laxity endorses the expansion of the vaginal mus cles, and increases the flexibility of the joints of the embryo. The newborn ' s hormone level gradually decreases, and normalizes by the 3 -4th week. Hence, the newborn is in a bor derline state for 3 weeks regarding joint sta bility, when the femoral head may separate from the joint (unstable hip). The two most important extrauterine factors are the inappro priate use of nappies fitting tightly on the lower limbs, and the erect position, i.e. the at tainment of walking itself. From the moment of birth, the newborn assumes the so called re laxed - sleeping or sprawled posture, which is characterized by almost 90° offlexion and ab duction of the hip joint (Fig. 28.6.). In a sprawled position, the femoral head is sunk
28. H i p d isorders
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Fig. 28.6. Relaxed - sleeping posture of the newborn: almost 90° flex ion, abduction and external rotation.
deeply in the acetabulum, enhancing the nor mal development of the acetabular roof, even in dysplastic-hypoplastic cases. The treatment follows the same path. Many clinical findings relate to environmental factors, e.g. 1 6% of babies with hip dislocation exhibit breech pre sentation. Etiologic forms of hip dislocation. 1 . Hip dysplasia or consequential dislocation on the basis of acetabular hypoplasia (polygenic form) can be well managed with conservative treatment; the disloca tion can be easily reduced. 2 Dislocation is already present at the mo ment of birth as a feature of general, inher ited connective tissue laxity (dominant form). Besides the luxation, other signs of general, inherited connective tissue laxity are also detected (knee hyperextension and lax wrist). It reacts well to conserva tive treatment; the dislocation is easily re duced.
Symptomatic hip dislocation is a symptom of identified monogenic (dominant or re cessive) hereditary abnormality (e.g. Ehler-Danlos syndrome, Marfan 's syn drome, Morquio 's syndrome, congenital multiplex arthrogryposis, etc.). It reacts less to conservative treatment, though this may be attempted. It usually requires sur gical intervention. 4 Secondary hip luxation is due to primary neurological or muscular disorders. The dislocation is not present at birth, it ap pears only during childhood (e.g. infantile cerebral palsy, poliomyelitis, etc.). It barely reacts to conservative treatment, and usually requires surgical intervention. 5 Teratological hip luxation is induced by a teratogenic factor in the first third of intrauterine life, it is not inherited. It is of ten seen as a part of the multiple develop mental abnormality of the limb. The series of developmental abnormalities is local ized only to the involved limb, in contrast with symptomatic hip dislocation, where it is part of a general developmental abnor mality involving the entire body. It does not react to conservative treatment, and the outcome of surgical intervention is also generally poor. �
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Pathology of dislocated hip j oint Bony elements - Acetabulum hypoplasia - primary path ologic factor. - Increased antetorsion of the proximal femur (this is a consequence of loosening of the active anterior acetabular wall (iliopsoas muscle). - Coxa valga (this is a consequence of the missing pressure of the body weight transferred to the head and neck by the acetabulum) .
Soft-tissue components The soft tissue at the bottom of the acetabulum proliferates, the limb is inverted, the iliopsoas and adductor muscles are tense, and the gluteus medius and minimus muscles are insufficient.
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Clinical symptoms. The clinical symp toms are classified as follows : � �
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Signs arousing suspicion Signs indicating probability Hip instability Definite signs Late signs X- ray and u ltrasonographic signs (imaging signs)
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Signs arousing suspicion Signs arousing suspicion feature among the history data obtained from the parents : the occurrence of hip dislocation or dysplasia, a pathological pregnancy, an abnormal delivery or the intrauterine posture (breech presenta tion) and other data relating to the behavior of the newborn: an asymmetric movement of a lower limb, or any other factors worth men tioning. �
maius. When the baby is prone, the gluteal folds are also asymmetric and positioned proximally on the involved side (Fig. 28.7.). - Adduction contracture. The hip ab duction of newborns is between 70° and 90° and symmetrical. An abduction of less than 70° bilaterally or asymmetrical abduction is considered a sign indicating probability. - An increased greater trochanter mass. In cases of hip luxation or subluxation, the greater trochanter is displaced proximally and laterally, and on palpation it is felt to be an enlarged mass (Fig. 28.8.). - External rotation of the limb. In hip dysplasia and even more in luxation, the limb is externally rotated. This has no specific diagnostic value, but it may be Impressive.
Signs indicating probability - Fold asymmetry. This is considered only if it is consequently present together with other signs indicating probability. The fold asymmetry is presumed to be consequent when the thigh folds are deeper and proximal, and perhaps more numerous . In girls, the most proximal fold may partially conceal the labium
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Fig. 28.7. In cases of hip dysplasia and luxation the thigh and gluteal folds are asymmetric. I n the involved side, there are more and deeper folds.
Fig. 28.8. The baby is assessed with bent hips and knees. The thigh is shorter on the luxated side, while the greater trochanter is displaced proximally and later ally, and on palpation it gives the feeling of an in creased mass, in the middle of the buttock, there is a fold.
28. Hip d isorders
- Limb shortening (Bettmann 's sign). Limb shortening is associated with hip dislocation, since the femoral head is displaced from the acetabulum proximally, resulting in limb shortening (Fig. 28.8.). - Axial deviation. In Lorenz 's abduction, the femoral axis points above the acetabulum instead of its center. - Atrophy, and flattening of the gluteal muscles. With the hip flexed to 90° the buttocks are asymmetrical and on one side, a dent is visible in the middle of the buttock (Fig. 28.8.). •
Hip instability (Barlow' s sign) The essence of this sign is that the hip is not displaced, but it is dislocatable, luxatable from the acetabulum. The sign is caused by two factors : acetabulum hypoplasia and tran sitional hormonal laxity. Consequently, it is a false-positive sign in 90% of the cases in the first 3 weeks. The mode of assessment is the following: The hips of the supine baby are flexed to 90° with knees fully flexed. The hips are t4en mildly adducted and mild pressure is exerted backward with the examiner' s thumb. In positive cases, a click is palpable in the hip, when the femoral head leaves the acetabulum
Fig. 28.9. Hip instability (Barlow's sign). The pressure of the examiner's thumb dislocates the hip.
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(positive Barlow 's sign, Fig. 28.9.) . A s soon as the force is released, the head slips back into the acetabulum. •
Definite signs (Ortolani 's sign). This is the only definite sign of hip dislo cation; it can also be evaluated as a reduction sign. The assessment is performed as follows : The hips of the supine baby are flexed to 90° and abducted. During abduction a click is ex perienced, signaling that the femoral head is reduced (Fig. 28.1 0.). In cases involving hips that undergo reduction with difficulty, this maneuver is to be supplemented with axial traction of the femur to facilitate reduction (Lorenz 's reposition). •
Late signs Late signs are detected after the child has started to walk. Fortunately these are seen very rarely nowadays. - The Trendelenburg sign. This is de tected in all other disorders besides hip dislocation, where a gluteus medius and mlll1mus muscle insufficiency 1S present. The standing patient is requested to stand on only one leg, and to lift the other leg up while bending the hip and the knee. In normal individuals,
Fig. 28.10. Reposition sign (Ortolani's sign): during a bd uction, a click is felt and the femoral head is reduced
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28. Hip d isorders
walking, and also during assessment in a prone position. - Increased lumbar lordosis. Mainly in high bilateral dislocations, the lack of proper hip support results in an increase in the pelvic slope, and this is com pensated by increased lumbar lordosis.
the gluteal muscles o n the supporting side (hip abductors) hold the un supported side of the pelvis in a horizontal plane. In case of an in sufficiency of the gluteal muscles, the pelvis on the unsupported side sinks below the horizontal plane and the patient tilts with the trunk toward the supported side, because this is the only way he/she is able to maintain balance (Fig. 28. 1 1 .). In unilateral cases, the trunk tilts toward the supported side with every step during walking, in bilateral cases, the trunk tilts in both directions (duck gait). - Telescope sign. In cases of total dis location, the protruding trochanter (and the femoral head) moves proximally and distally in the axis of the femur during
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Fig. 28.12. Fig. 28. 11. Left side positive Trendelenburg sign; the unsup ported, right side of the pelvis sinks u nder the hori zontal plane
Ultrasonographic assessment of the hip. The draw ing: the angle alpha is not less than 60° and beta is not larger than 55° for in normal hips. The image shows a phase I Il.a dysplastic hip: a: bony acetabular roof; b: cartilage acetabular roof, c: baseline.
28. Hip disorders •
U1trasonographic and X-ray signs (imaging signs) Ultrasonographic signs. Under the age of 4 months X-ray examinations are not per formed on the hips, because the ossifying cen ter is undetectable. Instead of the harmful X-ray radiation, a modem and harmless ultra sonographic assessment is carried out. This reveals dysplasia at an early stage and the de velopment of the hip joint is easily followed up (Fig. 28.1 2.). The examination may be re peated at any time. Ultrasonography is recom mended in case of any existing clinical sign, or when risk factors are recorded (familial oc currence, or breech presentation) . This noninvasive scan helps in the evaluation of the bony and cartilaginous elements of the acetabulum as primary factors. X-ray signs. Hip X-rays may be per formed above the age of 4 months with the following indications : - when ultrasonography and physical assessment lead to a questionable conclusion, - in suspected dysplasia or luxation, - in cases of familial occurrence.
The presence of the ossifying center of the femoral head contributes a great deal to the evaluation of the X-ray film. Before its mani festation, various ancillary lines facilitate ori entation.
Fig. 28.13. 4-month-old baby. Hip X-ray: the acetabular roof is steep on the left side (dysplasia).
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- Acetabular roof angle (Hilgenreiner 's angle) : this is normally symmetrical and less than 3 0°, in cases of dysplasia the value increases (Fig. 28.13.). Assess ment of the acetabular roof angle : a horizontal line is drawn through the identical points of the Y -shaped car tilage, representing the horizontal plane, and the angle is measured between this and the line following the acetabular roof. - Kopits 's rectangle. This is outlined by the lines drawn between the two end-points of the acetabular roof and the two end-points of the femoral neck metaphysis. In normal cases, this is a regular square, and, if the ossifying cen ter is already apparent, it is positioned in the central area (Fig. 28.14.). In cases of dysplasia or luxation, the square is irregular, and the ossifying center is excentric and lateralized. - Menard-Shanton 's line. The cranial arch of the foramen obturatorium is normally the continuation of the lower arch of the femoral neck. In cases of subluxation or dislocation, this arch is interrupted, and the lower arch of the femoral neck is moved proximally (Fig. 28.14) . - Ombredanne 's line. This is a per pendicular line drawn from the lateral edge of the acetabulum distally to the horizontal line connecting the Y -line,
Fig. 28. 14. Kopits's quadrangle (K), Menard-Shanton's line (MS)
and Ombredanne's line (0).
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28. H i p disorders
described above. These two lines divide the hip joint into 4 segments. If the hip is normal, the ossitying center is located in the inner-lower quadrant; in cases of subluxation or dislocation it is located laterally (Fig. 28. 1 4.). - Hilgenreiner 's H-distance. This is the distance between the medial spine of the femoral neck and the sciatic bone, which is normally parallel with the Y-line and it is at most 5 mm. Before the age of 6 months, a larger distance may mean lateralization of the femoral head (Fig. 28.1 5.). Prevention of hip dislocation . The es sence of prevention is to ensure the relaxed resting position for the newborn following birth (Fig. 28.6.). It is not coincidental that hip dislocation is rare in Far-Eastern countries and in Africa, where children are carried on their mother' s back with their legs spread. In contrast, in countries, where babies are wrapped in nappies or swaddled with tightly adducted lower limbs (Eskimos, Canadian In dian tribes, in some places in Ecuador and in Central and Eastern Europe), dislocation is very widespread.
Prevention and recognition are closely re lated. Accordingly, three compulsory infant screenings are performed in Hungary: The first is carried out 3 -4 days after birth, usually in neonatal wards, and is easy to organize. The next is due at the age of 3 -4 weeks, and the third at the age of 3 -4 months. The hips must be checked on all occasions when the child is due to participate at other regular pediatric check-ups or inoculation. Conservative treatment. The methods of conservative treatment depend on the time and severity of the diagnosis. When dysplasia is noticed at the first or second screening, merely exercises and
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Fig. 28.15. Various ancillary lines and ang les for evaluating hip dysplasia or dislocation on the X- ray film. (Y: Y- line; vsz: acetabular roof angle; d l,d2: socket metaphysis d istance; h: Y-line - metaphysis dis tance). On the left, the situation is normal; on the right, dislocation is seen. Vsz and d values are larger, and distance h is lower
a: Right side: normal; left hip: acetabulum hypoplasia is seen in an extended position. The femoral head is pressed against the acetabular roof; no improve ment is expected. b: I n the abducted - flexed position, the femoral head is pressed centrally into the socket, ind ucing normalization of the acetabulum. c: Recovered state.
2 8 . H i p d i s o r d e rs
spreading nappies are recommended (Fig. 28. 1 6. a, c). The method advocated for spreading nappies is the use of "Rugi pants" (Fig. 28.1 7.), which ensures continuous ab duction with the elastic spongious lining. In cases of dysplasia recognized at the third screening and confirmed by ultrasonography or X-ray examination, the application of Pavlik ' s harness is advised (Fig. 28.1 8.). Pavlik 's harness is a functional treatment mo dality popular worldwide. The essence of this functional treatment is that it limits only the extension of the hip; all the other movements can be freely performed. Flexion and abduc tion centralize the femoral head and enhance the development of the acetabular roof. The use of Pavlik 's harness is possible only until the infant sits up (at the age of 6-8 months), since the main effect is eliminated in a sitting position. Instability. As described in the above, at the time of the first screening, instability is a
Fig. 28.17. Sprawling abduction nappies.
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Fig. 28.18. Pavlik's harness. 90° of flexion is secu red in the hip
joint
pseudo-positive symptom induced by transi tional hormonal laxity. At the next screening, it may be decided whether real dysplasia is present: if the instability has ceased, the hip is classified as normal. If the instability persists, severe acetabulum hypoplasia is in the back ground and Pavlik 's harness should be con sidered. The severity of the acetabulum hypo plasia may be defined by an ultrasonographic scan. In cases of instability persisting up to the age of 4 months, Pavlik' s harness is also indi cated. Ortolani 's positive hip (luxation) . If this is detected at the first screening, abduction exer cises and spreading treatment are advised for a short period, knowing that Pavlik 's harness is needed (Fig. 28.1 8.). Pavlik 's harness is not to be applied before the I O- 1 4th day after birth, since it may harm the newborn and the skin adaptation is still underway. Pavlik' s har ness can be used on an outpatient basis. In the maj ority of cases (95%), it results in total re covery. Below the age of 6 months, other modes of conservative treatment may be attempted if Pavlik 's harness fails (5%). One ofthese is the "overhead extension" (Fig. 28.1 9.). In cases
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a ------
Fig. 28.20. Abd uction splint.
Conservative treatment is recommended until the age of 6 months. Above this age, sur gical intervention is advised.
Fig. 28.19. Overhead extension, first vertically (a), and later in the a bd ucted position (b).
of irreducible or barely reducible hip disloca tions, distal traction is applied via cords at tached to the legs by adhesive tapes, which may loosen the hip joints. With gradually in creased abduction, spontaneous reduction may occur. In cases of instability, when the femoral head leaves the acetabulum at 1 0-20° of adduction, a more rigid fixation device, the abduction splint (von Rosen ' s splint) is tem porarily applied (Fig. 28.20.).
Surgical management. In surgical reduc tion, following exposure of the hip joint cap sule, the soft tissue that fills the joint is re moved, together with the inverted limbus. The iliopsoas muscle is separated from the lesser trochanter, and fixed to the capsule at the an terior part of the femoral neck. After removal of the obsolete parts of the capsule, reduction of the femoral head is easily achieved and, by narrowing the capsule, the reduced femoral head can be stabilized. Postoperatively, the hip must be functionally immobilized in the abduction - flexion position with a splint or in Pavlik 's harness. After the age of 2 years, besides the cor rection of soft-tissue components by the open reduction procedure described above, the bony components must also be corrected. In cases of mild acetabular hypoplasia, the cor rection of the proximal femur can be satisfac-
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Fig. 28.21. A 2-year-old girl with a developmental dislocation of the left hip. Open reduction, varus and derotation osteotomy. The steep acetabular roof is normalized, and the head is central. Normal hip at the ages of 6, 8 and 31 years.
tory: varus and derotation osteotomy ensures the development of the acetabular roof after the centralization of the femoral head (Fig. 28.2 1 . a, b) .
Correction of the acetabulum can be sub divided into complete and incomplete pelvic osteotomies (pericapsular pelvic osteotomy and acetabuloplasty).
Fig. 28.22. Pelvic osteotomies for correction of the steep acetabulum: 1: Salter's pelvic osteotomy, 2: Pemberton's in complete pericapsular pelvic osteotomy, 3: Chiari's pelvic osteotomy, 4: triple osteotomy.
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The most widespread and popular tech niques are as follows : - Chiari 's pelvic osteotomy is a complete pelvic osteotomy over the acetabulum, the distal part being displaced medially (Fig. 28.22.). The center of rotation of this motion is the symphysis. The disadvantage is that the femoral head is covered by fibrous cartilage instead of hyaline. It is nowadays used only in exceptional cases, when no 'other procedures are reasonable. - Salter 's pelvic osteotomy is also a complete pelvic osteotomy over the acetabulum, but the distal part is displaced laterally, providing a hyaline cartilage cover for the femoral head (Fig. 28.22.). The center of rotation of this motion is again the symphysis. The correction effect is limited, depending on the mobility of the symphysis, and it can therefore be used under the age of 6. - Pemberton 's incomplete pelvic oste otomy, acetabuloplasty, is a rounded and arched pelvic osteotomy starting above the upper edge of the acetabulum and extending to the Y -shaped cartilage in the depth of the acetabulum (Fig. 28.22.). The steep acetabulum is folded
down over the femoral head, and the correction achieved is ensured by a bone wedge inserted into the gap. The center of rotation of this motion is the Y -shaped cartilage. It may be ideal until the closure of Y -shaped cartilage. The surgical intervention on the hip joint which corrects the bony and soft tissue ele ments in a single session is called one-stage osteotomy (Fig. 28.23.). The outcome is ex cellent if it is done in time and with good tech nical conditions. Later, the child may take part in sports activities and gymnastic exercises.
28. 1.3. Osteochondritis capitis femoris juvenilis (Perthes' disease, Legg-Calve-Perthes' disease) This is necrosis of the proximal femur epiphy sis in childhood, leading to a deformity of the femoral head.
Incidence. It occurs between the ages. of 3 and 1 3 years; it is the second most common hip illness with an incidence of between 0, 1 - 1 %. It is 3 times more common in boys
Fig. 28.23. A 5-year-old girl with subluxation; one-stage osteotomy which corrects the bony and soft tissue elements in a single session.
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than in girls. It is usually unilateral, but in 1 5%, it is a bilateral condition. Etiology. The definite cause of the disease is unknown, there is however both direct and indirect evidence pointing to a disturbance of the blood supply of the femur proximal epiph ysis. The condition is unquestionably related to occlusion of the lateral epiphyseal artery, which supplies the epiphysis and it is apt to occlude in response to a pressure increase in the hip joint, on the short and vulnerable seg ment of the artery, where it runs on its intra-articular and extra-osseal path. It has been proven, that in 5% of the cases, this con dition is preceded by transitory hip arthritis. It occurs in the temperate zone, in both the northern and southern hemisphere. It is not observed in cold and hot zones where the cli mate is usually more balanced. The influenza common in spring and autumn may be the etiologic factor. It is also characteristic, that the incidence of this condition in childhood corresponds to that of transitoric coxitis. Pathology. In consequence of primary or reactive intra-articular irritation, the synovial membrane swells and becomes hyperemic. In the early stages, the histology of the epiphysis ossifying center reveals an enchondral ossifi cation disturbance besides necrosis. The osteocytes expire and the necrotic foci are gradually surrounded by scar tissue, while os teoclasts form from monocytic elements . The necrotic bone substance is gradually degraded and the osteoblasts produce new bone (keep ing substitution). In the regenerative stage, the bone production is increased. In the stage in which the necrotic areas undergo degradation, the bone is not sufficiently solid enough, and the granulation tissue gradually occupying the necrotic bone does not have appropriate me chanical rigidity, and is therefore not suitable for weight-bearing. Accordingly, the femoral head becomes flattened and mush room-shaped, and may protrude laterally. History. A typical feature is an intermit tent, strange gait or limp lasting for weeks or
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months. I n a number of cases however, the child has no complaints. In early stages, the strange gait or limp temporarily ceases after resting. The child locates the pain in the thigh or in the knee instead of the hip (due to the obturator nerve). As a general rule therefore, in case of knee pain in childhood, the hip must be thoroughly assessed. Occasionally, the child presents acute hip pain, and is unable to bear weight on the involved limb. In these cases, primary or secondary synovitis pre dominates. Clinical symptoms. The first obj ective sign is a decrease in the internal rotation of the involved hip compared to the contralateral side. In advanced cases, the limitation of the internal rotation becomes more severe, and an external rotation contracture may develop to gether with the limited extension. This is a se lective limitation of movement, since the pro cess always involves only one plane of the three axial planes of movement of the hip j oint. The child limps protectively; atrophy of the thigh muscles is also observed. In ne glected cases, the flattening of the femoral head and the shortening of the metaphysis cause moderate limb shortening. Imaging diagnostics. When Perthes ' dis ease is suspected, bilateral anteroposterior and Lauenstein X-ray films are to be taken. Lauenstein ' s position is the flexed, abducted and externally rotated hip. On the basis of X-ray picture, 4 stages are defined: Early stage. The early X-ray image does not show bony changes; medially, the j oint space is wider than on the other side. The cause is the synovitis and edema of the pulvinar acetabuli. 2 Sclerotic stage. The structure of the bony center of the proximal epiphysis is cloudy, and the density is increased; it becomes sclerotic, which is the radiological sign of necrosis (Fig. 28.24.). The bone center is moderately flattened. 3 Fragmentation stage. The previously ho mogenous bone center is even more flat-
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Fig. 28.26. Perthes' disease: epiphysis flattened and widened.
Fig. 28.24. Perthes' disease: sclerotic stage.
Fig. 28.27. Perthes' disease: end stage.
Fig. 28.25. Perthes' disease: fragmentation stage.
tened and fragmented (Fig. 28.25.). Radiolucent areas appear in the bone cen ter as the granulation tissue disintegrates the necrotic bone. The entire epiphysis may flatten, it widens and protrudes later ally, and the widening of metaphysis in creases (Fig. 28.26.). 4 Final stage. The femoral head is flattened to some extent, and its height is less than the contralateral height. The structure of the newly built bone center is normalized. In untreated or severe cases an enlarged,
Fig. 28.28. Bone scan: no perfusion of the involved epiphysis
flat, protruding femoral head is formed (coxa magna, Fig. 28.27.). In cases of clinical suspicion, when the X-ray finding is negative, a bone scan may confirm the diagnosis, showing no or only limited perfusion of the involved epiphysis (Fig. 28.28.).
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Laboratory tests are negative. Treatment. The course of Perthes ' dis ease lasts 3-5 years; the treatment is influ enced to a large extent by the known risk fac tors listed below.
I . Catterall 's stages (Fig. 28.29.) . � �
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Stage I: 25% of the epiphysis is involved. Stage 11: 50% of the epiphysis is involved. Stage I l l : 75% of the epiphysis is involved. IV. stage: The entire epiphysis is necrotized.
The more extensive the necrosis, the worse the prognosis. 2. Lateral pillar sign. Even if the necrosis is extensive, in cases when the small lateral segment of the epiphysis remains un harmed and its structure is intact, the prog nosis is expected to be good (Fig. 28.30.).
Fig. 28.30. The small lateral pillar of the epiphysis remains un harmed and its structure is intact.
1
3
Fig. 28.29. CatteraWs stages of Perthes's disease according to the extent of the affliction.
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3 . The age. This is the most important factor regarding therapy. Comparative clinical studies of treated and untreated cases have proved that below the age of 6 years, a good outcome is to be expected, almost re gardless of the method applied. This is probably due to the good regenerative ca pability of the young organism. After the age of9- l 0, the outcome is poor in the ma j ority of cases, regardless of the therapy. 4 . Metaphyseal involvement. If the process involves the metaphysis, the prognosis is poor (Fig. 28.3 1 .). The current treatment strategy relates to two major points: B elow the age of 6 years, the outcome is expected to be favorable even without treatment. Hence active therapy should only be considered in cases of Catterall 's stage IV of complete necrosis. After the age of 1 0 years, the result is poor with or without treatment. Above this age, therefore, only those interventions are indicated that ensure centralization of the femoral head proven by functional X-ray examination; otherwise only observation and palliative treatment should be provided. The centralizing ( containment) treatment has become predominant in both conservative and surgical methodology. Methods of centralizing treatment Conservative techniques. The basis of the treatment is that abduction and internal ro tation of the hip ensure the central location of the femoral head in the acetabulum. This posi tion decreases the load by distributing it evenly on the surface of the biologically softer femoral head and enhances its new modelation to the shape of the acetabulum (re ciprocal remodelation) . To achieve this obj ective (in either uni-, or bilateral cases), various calipers have been de vised to ensure abduction and internal rotation (Fig. 28.32.). Walking in these calipers and their utilization for 3 - 5 years involves a sub stantial restriction of activity for the child. An alternative approach is the use of crutches or bed rest, but in view of the 3-5
Fig. 28.31. Metaphysea l necrosis.
Fig. 28.32. Calipers used in Perthes' disease to ensure abduc tion and interna l rotation.
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years progress, this appears impracticable and maybe even impossible to implement. Operative therapy. Varus derotation osteotomy of the proximal femur. This method is popular in Hungary and in all of Eu rope, since 8 weeks after the surgically achieved centralization and following the healing of the osteotomy full, weight-bearing and activity may commence. The osteotomy close to the pathology increases the decompo sition and rebuilding of the necrotized femo ral head and virtually halves the usual 3-5 -year period of progression (Fig. 28.33.). �
Pelvic osteotomies A full cover of the femoral head and a de crease of the load on the surface can also be achieved by pelvic osteotomy, which is an other mode of centralization. Mainly Salter ' s osteotomy has become widespread. I t has a biomechanical disadvantage since it increases the pressure on the femoral head. Further more, the author recommends that it is to be
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performed before the age of6. After the age o f 1 0, Chiari ' s complete pelvic osteotomy may be performed mainly as a palliative interven tion (especially when a large lateral femoral head protrusion is detected, Fig. 28.22.). Perthes ' disease is one of the causes of hip osteoarthritis in adults . Therefore, after the healing of Perthes ' disease, school gymnastic exercises and sports are advised as follows : 1 . Cases that have healed without deformity: all activities are allowed. 2. A slightly flattened, but round, central femoral head (physiologic incongruence) : all activities (including sports) are al lowed. 3. A considerably flattened, laterally pro truding deformed femoral head (coxa magna) : moderate gymnastic exercises, swimming and cycling are allowed.
28. 1.4. Slipped capital femoral epiphysis (SCFE, epiphyseolysis capitis femoris juvenilis, coxa vara adolescent) The origin of this entity is the slipping of the capital femoral epiphysis backward and medi ally on the femoral neck. The process is often bilateral.
Fig. 28.33. Perthes's disease on the right side, following varus derotation osteotomy: full recovery. The head is round.
The types: - Epiphyseolysis lenta: This is a gradual slipping, with intermittent knee, thigh and hip pain; the limb is in a position similar to that when the neck of the femur is fractured: mild shortening and external rotation are seen with a restricted range of internal rotation. - Acute epiphyseolysis: The slip happens suddenly, following a fall or a faulty movement. It is accompanied by acute hip and knee pain. The limb is in a position similar to that when the neck of the femur is fractured. The limb is shortened and externally rotated, with a severely restricted range of motion.
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I ncidence. This is the third most common hip condition after hip dysplasia and Perthes ' disease. I t occurs i n the age interval 1 0- 1 5 years. It i s twice as common in boys, as in girls. It is often bilateral, and therefore close observation of the other hip is crucial. The lenta form of epiphyseolysis predominates (95%) . Etiopathology. It is currently believed that the background of this condition involves the development of a latent hormonal dys function between the growth hormones (that control the ossification) and the sex hormones in puberty. In response to the effects of the growth hormones, the proliferation of carti laginous elements in the growth plates in creases, and the growth plates widen. At the
Fig. 28.34. Shearing forces acting on the growth plate (ny), since the load (R) is not perpendicular to the growth plate.
end of growth, the secretion of the growth hormone gradually diminishes. The sex hor mones lessen the proliferation of the cartilagi nous elements, and the growth plates become thinner and ultimately disappear. The diverse secretion activity undergoes a crossover in puberty. If the secretion of sex hormones starts later, the growth plates persist and can not resist the biomechanical load pro duced by the considerable body weight and height gain in puberty, and may be displaced. This is common in the hip joint, because this is the only epiphysis in the body that is not perpendicular to the axis of the body weight, but is inclined obliquely and medially. It is therefore subj ected not only to pressing forces, but also to substantial shearing forces (Fig. 28.34.).
Fig. 28.35. Dystrophia adiposogenital constitution of a 14-year-old boy with right-sided epiphyseolysis.
28. H i p d i s o r d e rs
The condition is common in two constitu tions. One exhibits all the features of hypo gonadism: fat, a lack of secondary sexual signs, an a eunuchoid constitution (relative hypogonadism; Fig. 28.35.). The other type is a tall, thin constitution with long powerful arms (relative growth hormone overproduc tion). History. In cases of epiphyseolysis lenta, the child presents chronic complaints. Usually knee and thigh pain, but occasionally hip pain and fatigue are mentioned and the child avoids physical activity. The gait is peculiar, and an occasional limp is observed. In acute cases a stabbing hip, thigh or knee pain occurs following a fall or faulty move ment and the child is unable or hardly able to stand. Clinical symptoms. Walking with a slight limp is usually detected with an externally ro tated lower limb. One of the constitutions de scribed above is observed (eunuchoid relative hypogonadism, or a tall, thin constitution) . When the hip is assessed in a supine posi tion, the internal rotation is observed to be re stricted, and in most of the cases an external rotation contracture is present. The abduction and extension may also lessen, especially in major slips. Drehmann ' s sign is distinctive: on passive flexing of the hip, constrained ab duction and external rotation occur. This sign is a consequence of the dorsal and medial dis placement of the femoral head. Another com mon feature is the crossing sign: the child is asked to kneel, and the legs will cross each other. In acute cases, besides the intense pain the symptoms are similar to those detected when the neck of the femur is fractured: the limb is shortened and externalIy rotated, and any at tempted movement elicits severe pain. X-ray signs. Similarly as in other hip con ditions, bilateral anteroposterior and Lauenstein films are to be taken. On the Lauenstein views, slippage of the epiphysis is noticeable earlier, since the backward slip is
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then detected on this view and this is always larger than the medial displacement. In the anteroposterior views, the very early signs (preceding the significant slipping) is the wid ening and unevenness of the growth plate (Fig. 28.36.) . A moderate slip is indicated if the decrease in height of the bony center of the epiphysis is mild, compared to the other side. Another valuable sign is that when the lateral contour of the femoral neck is extended, nor mally, this line cuts few mm-s off the edge of the epiphysis (Fig. 28-36.). If the line is pe ripheral to the epiphysis, this may be a radio logical evidence of a mild slip. If a line drawn along the lower contour of the femoral neck on a Lauenstein film is prox imalIy elongated, and this line marks off a small segment from the posterior edge of the epiphysis, this is evidence of a mild slip (Fig. 28.37.). The extent of the slip can be defined by an exact angle on the Lauenstein films : the angle between the axis of the femoral neck, and the line drawn through the edges of the epiphysis. Normally, its value is 90° (Fig. 28.37., and 28.38.). Treatment. Both forms of epiphyseolysis require surgical treatment. Until the operation is performed, immediate bed rest is ordered so as to prevent progression, but a further slip may still occur.
Fig. 28.36. A 12-year-old boy with left sided chronic epiphyseolysis: anteroposterior. X-ray.
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Fig. 28.37. Same boy: Lauenstein X-ray.
Fig. 28.39. Epiphyseolysis with moderate slip: " in situ " epiphyseodesis with 2 spongiosa screws.
a b
In cases involving a slip of less than 30° in epiphyseolysis lenta, surgical fixation in situ is indicated, ifthis is technically possible. One or more K-wires, cancellous screws or cannulated screws (Fig. 28.39.) are intro duced through the neck into the epiphysis. Because of the stabilization of the epiphy sis, the pain and the contractures rapidly sub side postoperatively. The child may get out of bed on crutches the day after the operation, or in the event of pain, a few days later, and al lowed to bear weight and go to school after 3 weeks. When the slip is more than 3 0°, wedge osteotomy of the neck after Dunn can be car ried out, and the reduction is supplemented with corrective shortening of the femoral neck. In cases of acute si ip, gentle open or closed reduction and fixation is indicated in the first week. The attempt at reduction however, may result in a blood supply deficit and necrosis of the head.
28. 1.S. Coxa vara infantum (congenital) Fig. 28.38. A 14-year-old girl with acute epiphyseolysis. antero posterior (a) and Lauenstein radiographs: total slip (b).
This is a congenital ossifying malfunction of the femoral neck: the collodiaphyseal angl
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e gradually decreases and causes limb short ening. The condition is al ready present at birth, but is usually recognized at the age of 3-4 years. It involves both genders and is usu ally bilateral. It is a rare congenital condition.
Etiopathology. The etiopathology is un known. It is not inherited. The pathological process is progressive; the collodiaphyseal angle gradually decreases during childhood, often to under 90°. The growth plate becomes almost vertical, and may even create a state corresponding to acute epiphyseolysis be cause ofbiomechanical factors, since the ver tical growth plate is subj ected almost exclu sively to shearing forces. The limb is gradu ally shortened, and the maj or trochanter moves cranially. The origin and attachment of the gluteus medius and minimus muscles come closer to each other, and they become insufficient, Trendelenburg ' s gait develops. Clinical symptoms. The condition may already be discovered in infants by a skilled orthopedist, or at least the suspicion arises. Fold asymmetry and limited abduction are typical. The child is usually referred to a spe cialist at about 3 -4 years of age, with a pain less peculiar gait and limp. A higher and thicker trochanter mass, limb shortening and Trendelenburg ' s gait are observed. The hip abduction is slightly restricted. X-ray signs. Before the appearance of the ossifying center of the femoral head epiphy sis, early films merely indicate the irregularity of the proximal metaphysis. Later, the de creased collodiaphyseal angle is unequivo cally determined, and it is less than the aver age for the age group . The direction of the growth plate changes, it becomes close to ver tical. A characteristic X-ray sign is the pres ence of a triangle-shaped component between the distal-medial part of the epiphysis and the medial part of the neck (Fig. 28.40.). The growth plate may close earlier, around the age of 1 0- 1 2 years, because of the ossifying error,
Fig. 28.40. Bilateral coxa vara infa ntum.
or permanent pseudo-arthrosis may often de velop in the femoral neck. Treatment. This deformity can only be treated surgically. In progressive cases, val gus osteotomy is to be carried out in the early years. It must be realized however, that a gradual recurrence may follow because of the progression of the condition. In these cases, valgus osteotomy must be repeated at a later age.
28. 1.6. Sym ptomatic coxa vara A number of illnesses and general conditions, such as epiphyseolysis capitis femoris, fibrous dysplasia, Paget's disease, etc., may ind uce secondary varus hip.
Clinical symptoms. The clinical symp toms are similar to those described on coxa vara infantum (congenital) : limited hip ab duction, limb shortening and Trendelenburg' s gait. Treatment. If the deformity is part of a systemic disease, the latter should be treated, which may or may not cure the ossifying dis turbance.
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28. 1.7. Growth disturbances of the proximal fem u r Various extents o f hypoplasia or aplasia o f the proximal femur may occur for u n known rea sons. A primary teratogenic noxa is presum ably in the background during embryo genesis. It is not inherited.
The main point of this disorder is that ossi fication of the proximal femur is delayed. Os sification of the epiphysis is late and hypo plastic, and occasionally involves more than one ossifying center; moreover, the proximal ossification of the diaphysis is retarded (Fig. 28.4 1 .) . The hypoplastic or aplastic proximal femur is often dislocated. Treatment. In infants, no treatment is needed but observation is recommended. Af-
Fig. 28.41. Right congenital femur hypoplasia.
ter the start of walking, conservative treat ment is advised, depending on the extent of limb shortening. If the shortening amounts to 3 -5 cm, length equalization using surgical shoes is necessary. If the shortening is more extensive, equalizing calipers are prescribed.
28. 1.8. Transitory arthritis coxae (transitory hip joint inflammation) This is an acute, painful hip arthritis, which re solves by itself within a few days. It occurs most frequently between the ages of 2 and 14 years, affecting both genders equally.
Etiopathology. In the majority of the cases, the arthritis is preceded within 1 -3 weeks by influenza. It may occur after any other inflammation or trauma. As an immune response to an inflammation, reactive syno vitis may occur in any joint (not purulent). This is usually a condition in the temperate zone (similarly to Perthes ' s disease), and the incidence of the two disorders is also very similar. Clinical symptoms. It usually appears as an acute condition, the child is unable to bear weight on the upper limb in the morning or daytime, indicating hip, thigh or knee pain. The child protects the hip, and this indicates the hip involvement, despite the fact that the pain seems to be distal . The condition often occurs in a less acute form: the parents com plain that the child limps and avoids loading the involved limb, but the pain is not domi nant. Examination reveals a selective restric tion of movement is similar to that in Perthes' disease. The most outstanding sign is de creased internal rotation, and occasionally an external rotation contracture; the abduction (or adduction) and extension may also be lim ited. The laboratory tests are usually negative; a mildly increased sedimentation rate and a left shifted differential blood test may possi bly be observed in consequence of the earlier influenza.
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Imaging diagnostics. On ultrasono graphy, the accumulation of fluid in the joint is detected unmistakably. It is more important to evaluate whether the hip synovitis and fluid accumulation have caused any impairment to the circulation of the femoral head. The bone scan may demonstrate perfusion loss in the proximal femur area, but this is rarely neces sary. In all cases of transitory arthritis, bilat eral anteroposterior and Lauenstein X-ray im ages are advised, and the diagnosis should be confirmed in case of negative X-ray findings. Treatment. The patient must rest and the basic condition (influenza) is to be dealt with. If not contraindicated, nonsteroidal anti inflammatory drugs are advised. The com plaints cease within a few days. Walking and weight-bearing are allowed after the motion of the hip has become unrestricted.
28. 1.9. Juvenile acetabular protrusion Protrusion is the invasion and distension of the medial wall of the acetabulum toward the pelvis. Juvenile acetabular p rotrusion, which is usually unilateral, causes intermittent intense hip joint pain.
Fig. 28.42. Protrusio acetabuli in an elderly person with sec ondary arthrosis. The Wieberg CE angle is greater than the normal 20-40°.
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Three types of acetabular protrusion are differentiated: 1 . Juvenile protrusion : This is a rare condi tion, with intermittent hip complaints. Exonerative rheumatoid arthritis or non-differentiated collagenosis is possible in the background. The condition involves only the hip joint. 2. Acetabular protrusion in the elderly: Sec ondary osteoarthritis of the hip gradually develops on the basis of bilateral acetabular protrusion, usually in subj ects over the age of 5 0. 3 . Secondary acetabular protrusion : The pro trusion is due to some other identified ill ness (osteoporosis, osteomalacia, sys temic bone disorders, etc.). History. There is a gradual onset of inter mittent hip pain, the hip pain sometimes being severe. The pain is localized to the hip and groin and possibly the thigh and knee too. It occurs 4 times more frequently in girls and young females, than in males. Clinical symptoms. The restriction of hip motion and pain in response to passive move ments are typical. The restriction of hip mo tion is not selective, but homogenous. Both the external and internal rotation and also the ab- and adduction are limited to some extent. The flexion - extension usually remains intact. As the condition progresses, the hip gradually becomes uniaxial : flexion - extension is possi ble, but other movements may completely dis appear. The end-stage of the process is osteo arthritis in early adulthood with joint space narrowing and destruction. X-ray signs. The medial wall of the acetabulum is thinner, and in advanced cases it protrudes toward the pelvis. K6hler ' s drop figure disappears or is crossed over. The fem oral head is seated deep in the acetabulum, and the Wieberg ' s CE angle is larger than 40° (Fig 28.42.).
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Treatment. I n case protrusion is diag nosed, consultation with a rheumatologist or clinical immunologist is needed to verify any possible background disease. Most cases are seronegative, and the rheumatologist gives a negative opinion. In these cases, treatment with nonsteroidal anti-inflammatory drugs is advised according to the complaints, supple mented by a few days of rest. If the hip com plaints persist, intra-articular depot cortisone may be administered. The result is usually spectacular: the complaints cease almost im mediately. If the complaints and arthrosis per sist, hip replacement is indicated, even in early adulthood.
28. 1. 10. Snapping hip, external coxa saltans Coxa saltans i s present when t h e tractus iliotibialis, the thick, strong bundle of the fas cia lata, jumps over the tip of the g reater trochanter with a click. It takes place in late childhood or early adulthood and involves both genders. It is relatively rare.
Etiopathology. The occurrence is usually unilateral if it is due to an accident or scar. If its etiology is related to congenital develop ment, or to a connective tissue deficiency, then it is observed bilaterally. Coxa saltans has no relation to the hip joint, though the pa tients dramatically report that their hip "jumps out". The bursa, normally located between the greater trochanter and the iliotibial tract, de creases friction of the fascia lata. In cases of coxa saltans the smooth friction turns into stretched click, resulting in bursitis. History. The patients recount, that their hip "jumps out" upon certain movements first on one side, an then possibly bilaterally with varying intensity. Clinical symptoms. The patients can re produce the click while walking or squatting, and the sign is well palpable when the exam-
iner ' s hand is placed on the trochanter. It is of ten palpated better with the patient in a supine position, while the hip is flexed and extended, with simultaneous adduction and internal ro tation. The greater trochanter may be tender, indicating bursitis. Treatment. When the pain and bursitis is predominate, conservative therapy is advised with some days of rest and nonsteroidal anti-inflammatory drugs. When the click is the main complaint, the tense bundle of the tractus iliotibialis may be incised.
28. 1 . 1 1 . Inward or outward rotation of the legs Child ren may walk rotating their legs inward or less often outward. This is common and is not pathological, but rather a normal variant. Its incidence is 15-20% between the ages of 2 and 6. It affects both genders.
Etiopathology. It may be regarded as a physiological episode, since the gait is stabi lized this way. The intoeing gait terminates or improves in 95% of the cases in consequence of the changes in muscle power. The anatomi cal and clinical factors may be as follows :
- The decrease in the retrotorsion of the acetabulum and in the antetorsion of the femoral neck is delayed. These levels are more pronounced in childhood, gradually decreasing to adulthood. - Overactivity or weakening of the ilio psoas muscle, the active component of the anterior acetabular wall. The former may result in intoeing, and the latter in an externally rotated gait. - An imbalance between the external and internal rotating hip muscles. - A valgus knee deformity (more frequent in girls). The compensatory gait in the valgus knee could be a mild intoeing gait. - A rotation, ossification deformity of the leg, when the leg is internally rotated
28. H i p d i s o r d e r s
while the kneecaps look anteriorly. It is often accompanied by varus leg, proving the mild ossification disturbance of the leg. - Flatfoot, when the chi Id compensates the valgus heel with internally turned feet. - A structural or dynamic form of pes adductus. (In the dynamic form, the deformity is seen only during walking due to the overactivity of the abductor hallucis muscle, it discontinues at rest). Clinical symptoms and treatment. The child is taken to the doctor because of the strange gait, with inward turned legs. The walking pattern frequently shows a familial accumulation (an inherited alternative gait). Other features of underdeveloped muscles are often seen (e.g. flatfoot, valgus knee, sagging belly or a negligent posture). If no obvious etiology is found, spontane ous correction is expected by puberty. In cases of flatfoot or valgus knee, application of a supinating heel wedge to the footwear is ad vised. Sporting activity, swimming and ball games should be encouraged. Exemption from physical exercises at school is inappro priate. In cases of extreme rotation of the legs, with a valgus knee of more than 1 50, a marked metatarsus varus operative correction may be required.
28. 1. 12. Limb shortening Limb shortening is considered t o exist, if the difference in length between the lower limbs exceeds 1 cm.
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Types Functional shortening: This is caused by an adduction and flexion contracture of the hip. It is resolved if the primary source is eliminated (apparent shortening). An abduction contracture of the hip results in a relative elongation, which is also solved when the primary source is eliminated.
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Absolute shortening: The distance between the anterior superior iliac spine and the lateral (or medial) ankle is shorter than on the other side. It may be caused by: - Hip dislocation - Coxa vara - Perthes ' disease - A congenital local ossification malfunction in the growth plates - Inflammation of the growth plates - Trauma of the growth plates - Radiotherapy or the action of an iatrogenic noxa on the growth plates - Congenital limb hypoplasia
Shortening may involve the upper or the lower limbs. A major functional problem is caused in the lower limbs. Due to the asym metric load, lumbar spine changes may occur. Treatment. The treatment is determined by the age of the patient, the cause and the ex tent of the shortening. It is also influenced by the height of the patient relative to the average height of his age group. Limb shortening categorization : - 1 -3 cm: conservative treatment, foot wear correction - 3 - 1 5 cm: surgical intervention is con sidered - over 1 5 cm: amputation at the middle leg is considered, and application of a mo dem light prosthesis. Surgical solutions: Epiphyseodesis, obstruction of growth on the longer limb. Staples bridging the distal femur and prox imal tibia growth plates may achieve tempo rary epiphyseodesis. This procedure may be repeated. �
Limb lengthening or shortening procedures in one session This involves metaphyseal or diaphyseal shortening of the longer limb. Metaphyseal shortening may be carried out at the proximal and distal metaphysis of the femur and at the
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28. H i p d isorders
a
c
Fig. 28.43. Met ho ds of metaphyseal shortening on femur (a, b) and tibia (c) a: Proximal femural metaphysis, b: distal femural metaphysis, c: proximal tibial metaphysis.
b
Fig. 28.44. Methods of diaphyseal shortening of the longer limb in one ses sion. a: femur, b: tibia.
proximal metaphysis of the tibia (Fig. 28.43.). Diaphyseal shortening is performed at the midshaft on both the femur and the tibia (Fig. 28.44.). This is advocated in case of tall chil dren when the length discrepancy is less than 4 cm. Elongation osteotomy at the midshaft of the femur or the tibia in one sessIOn: - This comprises transverse osteo tomy in the middle part of the femur diaphysis. With use of a distractor, an elongation of 2-3 cm can be achieved. - Corrective osteotomy of the femur distal metaphysis with moderate elongation is performed. This is particularly indicated when mild shortening is associated with substantial valgus knee. These procedures are carried out rela tively rarely, since merely 2-3 cm elongation is achieved.
28. Hip d isorders �
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Continuous limb lengthening in multiple sessions: - This procedure is applied most frequently nowadays; the original limb length can be restored. It is indicated for shortenings between 3 and 1 5 cm. The larger the shortening, the longer the procedure. It may be performed either in childhood or in young adults. The collaboration of the child may be expected after 8 years of age, the procedure demands continuous cooperation between child, parents and physician. Devices for continuous limb lengthen ing (Fig. 28.45. a, b) : - Wagner type telescopic external fixateur: Transverse osteotomy is performed in the middle of the femur or tibia diaphysis. The fragments are fixed with strong screws (Schanz screws) driven into the distal and proximal metaphysis, and the screws are held by the external fixateur. This procedure is able to exert either distraction or
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compression. The modem systems have elastic telescopic solutions built in. - When Ilizarov type rings are used, the fragments are stabilized with stretched K-wires. The rings are fixed by 3 axial telescopes parallel to the limb axis. Both distraction as well as compression can be achieved. This device is more useful in fixing metaphyseal osteotomies, where the ossification is better. Both procedures have advantages and dis advantages. Ilizarov rings have recently be come more popular. �
The steps in continuous elongation : 1 Psychological training: This is neces sary to convince the child, together with detailed, repeated discussions of the working process between physi cian, parents and physiotherapist. This is the only way compliance of the child can be assured. 2 Special exercises are implemented be fore the operation in order to elongate the neighboring muscles and tendons .
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b
Fig. 28.45. Devices for continuous limb lengthening. a: Wagner's diaphyseal lengthening, b. llizarov's metaphyseal lengthening.
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This may decrease the necessity of sup plementary operations. 3 The procedure of continuous elonga tion: After the insertion and stabiliza tion of the external fixateur, the trans-
verse osteotomy of the diaphysis or metaphysis is carried out via a small in cision. On the operating table, an im mediate lengthening of 1 cm is accom plished. After a few days rest, further I mm lengthening may be achieved once or twice daily. This is continued until the required length has been attained, or contracture takes place in the neigh boring joints (Fig. 28.46. a, b). In cases of severe shortenings, the continuous elongation can be repeated once or more after an interval of one year. The rate of complications continuous elongation is relatively high (3 0%). Osteo myelitis, or transient or permanent paresis may occur, as may damage and restriction of motion in the neighboring joints .
28. 1. 15. Congenital hemiatrophy and hemihypertrophy This is a condition with unknown etiology and is not uncommon; one side of the body is deficiently grown and hemiatrophic. The limb length discrepancy may already be apparent in the first year and usually progresses slowly. Very minor length and circumferential differ ences are sometimes detected in both the up per and lower limbs; on other occasions, the dissimilarity progresses. Congenital hemi hypertrophy is diagnosed when ill-defined growth is observed on one side. It is often dif ficult to decide whether the length difference is due to hemihypertrophy or hemiatrophy.
28.2. Hip disorders in adu lts 28.2 . 1 . Functional anatomy Fig. 28.46. a: Following a d istal femur metaphysis fractu re: the growth plate is closed on the left, resulting in a 1O-cm limb shortening. b: After the limb lengthening, ossification is seen between the frag ments.
The hip joint is a spherical or limitless joint. The socket is the acetabulum, and the head is the femoral head. The elements of the acetabulum (the hyaline-covered C-shaped facies lunata, the ligamentum transversum acetabuli, bridging the incisura acetabuli, the
28. Hip d isorders
pulvinar, the fat tissue filling the fossa acetabuli, and the circular labrum) surround the femoral head over its equator, and the j oint is therefore classified as an enarthrosis. The capsule is very strong, reinforced by three ligaments, the iliofemoral, pubofemoral and ischiofemoral ligaments. These are at tached to the femoral neck in the same direc tion, turning from the caudal and posterior di rection. This is important from a mechanical point of view. The ligamentum capitis femoris plays a role mainly in the blood supply of the femoral head. Flexion and extension of the hip are ac complished around the transverse axis con necting the centers of the femoral heads. Dur ing flexion, the spiral twisted external liga ments loosen, during extension they tighten. In the hip, 5- 1 5 ° of extension is possible. Ab duction and adduction are achieved around the sagittal axis. The internal and external ro tation are performed around the construction axis. Combination of the above-mentioned motions effects the conical circumduction. The movement of the hip j oint is powered by a number of inner and outer muscles. Since these muscles have a mixed function, it is use ful to discuss them according to their main function. - M. iliopsoas : the only real flexor of the hip, which also has adduction and internal rotation effects. - M . tensor fasciae latae: a flexor of the hip, a knee extensor and a hip internal rotator. - M . gluteus maximus : a hip extensor. - M. gluteus medius and minimus: hip abductors. - M. piriformis : an external rotator and abductor. - M. obturator internus, gemellus superi or, inferior, obturator externus and quadratus femoris : external rotators. - Adductor muscle group : m. adductor longus, adductor brevis, gracilis and adductor magnus : adductors, internal rotators.
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28.2.2. Deformities of the hip prearthrosis Prearthrosis is a condition in which the joint cartilage cover is present, and the complaints a re minimal (fatigue and intermittent pain), but early osteoarthritis is expected, consider ing the deformity, changed biomechanics, or joint incongruence as a resu lt of earlier ill nesses or trauma. Cartilage damage is a consequence of essen tially the fol lowing major mechanisms: � �
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A pressure increase on any given loading surface A decrease of the loading surface Incongruence between the joint su rfaces Direct damage to the cartilage su rface
Etiologic factors. - Pediatric hip diseases that healed with deformity (congenital dislocation of the hip, epiphyseolysis capitis femoris, Perthes ' disease, etc. - Changes in collodiaphyseal angle (coxa vara, valga, or increased anteversion) - Acetabular protrusion - Post-traumatic conditions - Inflammation (bacterial, rheumatic, etc.) - Metabolic diseases with direct cartilage damage (gout or ochronosis) - Tumors destroying the joint surfaces
The most common factor, the effect of the increased collodiaphyseal angle, due to the valgus deformity is analyzed in Pauwels ' biomechanical system a s follows. In certain phases of the gait, the ipsilateral limb is loaded, while the contralateral limb is advanced. At these moments, the j oint is sub j ected to forces, which maintain balance and prevent pelvic tilt on the other side, the pelvis therefore remaining horizontal. This is ac complished by the hip abductor, namely the gluteus medius muscle. Pauwels' demonstrated that at these mo ments the following forces are acting (Fig. 28.47. a-c) .
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2 8 . H i p d i so r d e rs
b
a
A
B
c
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b
a
352
kIVcm 2
./
25,2
kIVcm 2
Fig. 28.41. Loading conditions A: A normal col lodiaphyseal angle (ratio of force / lever arm (b:a) 3:1). B: Coxa valga (b:a 6:1). C: I ncongruence (a) congruence (b) (following cor rection osteotomy). =
=
The system works by the lever principle. In the balanced position: force
x
lever arm
=
load
x
load arm.
The lever arm is the horizontal proj ection of the distance between the hip center and the force vector of the balancing abductors (Fig. 28.47. A). The load arm is the projection of the distance between the weight line and the hip center. Under normal circumstances, the ratio of these two arms is 1 : 3 . The hip abduc tors balance a 50 kg load with a 1 50 kg force. The rotation point of the lever (the hip joint) is loaded by 1 50+50 kg. If the power arm is
shorter, the force in hip abductors increases. In cases of a steep (valgus) femoral neck, the ratio may even increase to 1 : 6 (Fig. 28.47. B) . Thus the load acting on the hip in this case may reach even 3 5 0 kg ! The extent of cartilage wear is dependent on the pressure, friction and congruence of the joint surface. If a small area is subjected to high pressure, or certain areas do not touch the other joint surface, degeneration takes place (Fig 28.47. C). Deviations of the collodia physeal angle (coxa valga, coxa vara, incon gruence or subluxation) may lead to hyaline degeneration, and this condition may there fore be considered as prearthrosis.
28. Hip disorders
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If the coliodiaphyseal angle in adults is g reater than 130- 135°, the condition known as valgus hip is determined (Fig. 28.48. c).
ral head i s only partial because o f acetabular dysplasia, coxa valga subluxans is present (Fig. 28.49.). Depending on the extent of subluxation, a number of groups are differentiated (after Hartofilakidis) :
The collodiaphyseal angle depends on the age: the nonnal range at the age of 9 years is 1 3 5 - 1 38°, at 15 years, it is 1 3 3 ° , decreasing to 1 20° in elderly ages. The values for valgus de fonnities are therefore dependent on the age. Coxa valga is rarely an independent entity, it is often a component of other diseases, such as congenital dislocation and dysplasia, if these persist after treatment, or i f subcapital coxa valga develops. Infantile cerebral paresis and any noxa affecting the lateral part of the proximal femur growth plate also cause valgus defonnity. If the coverage of the femo-
Stage 1 : Dysplastic hip. The head is attached to the original acetabular hyaline (inde pendently of the extent of subluxation) . The X-ray picture shows an upper seg mental defect: the acetabulum is shallow because of the osteophytes padding up the fossa acetabuli. Stage 2: Low dislocation. The head is in a sec ondary socket, partially connected to the primary acetabulum. The X-ray picture shows an anterior/posterior segmental de fect: the acetabulum is shallow, with in creased socket and femur anteversion.
28.2.2. 1. Valgus hip, coxa valga
Fig. 28.48. Collodiaphyseal angle ranges in adults. a: Varus deformity (95°), b: Normal (126°), c: Va lgus deformity (150°)
Fig. 28.49. Right-side subluxed femoral head; 3D a image.
a b
Fig. 28.50. The left hip is in high dislocation, with severe hip osteoarthritis on the right side. a: Anteroposterior X-ray of pelvis. b: 3 dimension a reconstruction.
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��� a
Fig. 28.51.
(_10 ° )
b ( 12 ° )
c
(45 ° )
Different types of torsions of the proximal femur. a: Retrotorsion. b: Normal. c: Antetorsion.
Stage 3: High luxation. The X-ray picture shows a cranial and dorsal secondary socket, which is totally independent of the primary acetabulum. A segmental defect is seen in the entire acetabular edge.
The highly luxated hip (Hartofilakidis Stage 3) is a special form of prearthrosis. The femoral head is located 5- 8 cm proximally, in the substance of the gluteus medius muscle, attached in a pseudoarthrosis to the iliac crest (Fig. 28.50. a, b). The patient overloads the intact, longer limb (especially if the length discrepancy is not equalized), and early osteoarthritis of the uninvolved hip therefore occurs (Fig. 28.50. a) . Constitutional varus or valgus hips are of ten detected in adults with well-developed sockets and congruence. These are not consid ered to be prearthrotic conditions. 28.2.2.2. Va rus hip, coxa vara
- congenital developmental deformities of the femur - fibrous dysplasia, Paget' s disease, osteomalacia or rickets - malunited fractured neck of the femur 28.2.2.3. Antetorsion and retrotorsion
of the hip (coxa antetorta and coxa retrotorta) Rotation of the proximal femur around the longitudinal axis of the bone is torsion. This may take p lace in two directions. In ante torsion, the ventral angle of the line connect ing the centers of the head and the greater trochanter exceeds the normal 15°. In retro version, this angle is less than 15° (Fig. 28.51. a, c).
Antetorsion is much more common. It rarely occurs on its own, usually being de tected in association with infantile cerebral
Coxa vara deformity is present, when the collodiaphyseal angle in adults is less than 120° (Fig. 28.48. a). Coxa vara is a conse quence of pediatric hip afflictions, which leave residual symptoms despite treatment:
- osteochondritis after the treatment of congenital hip dislocation - femoral head deformities after Perthes ' disease - coxa vara deformity after epiphyseolysis capitis femoris - congenital developmental deformities of the femur (coxa vara infantum, or a proximal femoral deficiency)
Fig. 28.52. Infantile cerebral palsy: bilateral coxa valga antetorta deformity.
2 8 . H i p d i s o r d e rs
palsy (Fig. 28.52.), or hip dysplasia with a valgus deformity. In cases of maj or ante torsion, the patient walks with typical inter nally rotated lower limbs, in order to achieve that their hip j oints act in normal position. 28.2.2.4. Other p rearthroses
Inflammatory illnesses in childhood may also be considered as prearthrotic conditions. The systemic inflammatory illnesses are in cluded here : rheumatoid arthritis, epiphyseal osteomyelitis, or purulent arthritis, which even after healing may end up with osteo arthritis in adulthood. The primary acetabular protrusion occurs in puberty (see Chapter 2 8 . 1 ) . Medialization of the femoral head leads to a bio mechanically disadvantageous state, which may result in severe arthrosis in adulthood with monoaxial (hinge) hip motion. The secondary acetabular protrusion may develop in consequence of an inflammatory hip disease (e.g. rheumatoid arthritis); it de stroys the bottom of the socket, causing medialization of the head. This is the "thin wall" protrusion. It bears significance in rela tion to bone grafting. 28.2.2.5. Surgical treatment of
prearthrotic conditions The objective is to restore the normal ana tomical and biomechanical situation and axis. This way the arthrosis is prevented, or the pro cess may be slowed down. Preventive operations in the hip area: �
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Femur osteotomies a) Intertrochanteric varus osteotomy b) McMurray's medialization osteotomy c) Valgus intertrochanteric osteotomy d) Subtrochanteric osteotomy Pelvic osteotomies
Femur osteotomies Femur osteotomies may be carried out in the intertrochanteric or subtrochanteric seg-
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ment. They normalize the axial deformities and power arms. Each one involves a different way to achieve the same goal, namely to change the morphology or static of the limb, thereby improving the function (sub trochanteric osteotomies). a) Intertrochanteric varus osteotomy This intervention is performed in prearthrosis or early arthrosis, to correct the axis of the steep femoral neck, to improve the loading aspects and j oint congruence (Fig. 28.53. a, c). A medially based triangular wedge is sawn out from the intertrochanteric area of the femur. After removal of the wedge, the bone ends are united and firmly fixed (sta ble osteosynthesis). During the operation, si multaneous derotation is possible to decrease the enlarged antetorsion of the femur so as to further improve the congruence ofthe femoral head to the socket. Precondition ofthis operation is the appro priate abduction of the j oint, since the varus mechanism decreases part of the abduction range of the joint. Another precondition is, that the femoral head must be more centralized on the abduc tion film, with better joint congruence. b) McMurray's medialization osteotomy The femur is cut through in the intertrochanteric area in a plane inclined slightly upwards, and the distal bone end is displaced medially by half the width of the bone and fixed in this position. No wedge is removed. Through the displacement of the lower bone end, the muscles relax, causing an ad vantageous effect. This operation is currently rarely performed because of the availability of arthroplasties. The bone displacement causes a deformity in the medullary canal, which may make it impossible to insert of the stem of the artificial hip. c) Valgus intertrochanteric femur osteotomy The aim of this procedure is to correct a varus femoral neck to enhance the congru-
36 4
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28. H i p d isorders
Fig. 28.53. a: Outline and effect of varus and medializing intertrochanteric osteotomy (1: hip abductor m., 2: m. iliopsoas, 3: hip adductor m.). b: Femur valgus deformity - preoperative X-ray. c: After varus osteotomy. (Because of the shallow socket, Chiari's pelvic osteotomy was performed later.)
a b
c
ence, to decrease the tension of selected mus cles (adductors and iliopsoas) and to increase the tone of the hip abductors (Fig. 28.54. a, b). In the operation, a laterally based wedge is sawn out from the intertrochanteric area of the femur. After removal of the wedge, the bone ends are reduced and firmly fixed with stable osteosynthesis. Due to the valgus, the femoral head is turned laterally in the socket, the con gruence improves and the distance between the origin and attachment of the muscles changes beneficially. A precondition of this operation is the adduction reserve of the joint, since the varus mechanism decreases part of the adduction range of the j oint. Simultaneous derotation is also possible. The intertrochanteric femur osteotomy is preceded by a careful examination of the pa-
tient and geometrical planning based on the X-ray films. The size of the wedge removed is determined by the measured collodiaphyseal angle. The effects of intertrochanteric femur osteotomy may be summarized as follows: - By centralization of the femoral head, the joint congruence improves, the loaded surface increases, and the pressure is better dispersed, and therefore decreases. - The power of the abductors, adductors and iliopsoas is altered advantageously by the changed distance between the origin and the attachment of the muscles. - The alteration of the collodiaphyseal angle changes the length of the power arm (varus osteotomy) in accordance
2 8 . H i p d i s o r d e rs
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3 a b
Fig. 28.54. a: Outline and effect of valgus intertrochanteric osteotomy. b: X-ray of valgus intertrochanteric osteotomy.
with Pauwels' theory, and consequently the pressure generated by the abductors on the joint decreases. - The consolidation of the osteotomy rearranges the circulation of the pro ximal femur, and decreases venous stagnation, giving rise to an advan tageous, not negligible biological effect. �
Pelvic osteotomies Pelvic osteotomies are discussed in the chapter on congenital hip dislocation. Chiari ' s osteotomy i s performed i n cases o f pre arthrosis in early adulthood (Fig. 28.55. a, b). On the same principle, acetabular roof con struction is also considered (see Chapter 28. 1 ) .
Fig. 28.55. Chiari's pelvic osteotomy in hip dysplasia: a: X-ray picture of dysplastic hip. b: After medial displacement of the proximal part of the iliac bone, the coverage of the femoral head and the loading conditions are better.
28.2.3. Idiopathic fem u r head necrosis (aseptic, avascular fem u r head necrosis) This is ischemic necrosis involving the frontal and u pper lateral weight- bearing area of the femoral head, leading to severe joint defor mity.
Symptoms. Pain in the hip, restriction of the range of motion and a limp. The X-ray pic ture shows a wedge-shaped sclerotic area in the upper perimeter of the femoral head,
366
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which may collapse. I n a large proportion o f the cases (30%) the process is bilateral. Incidence. Zizic and Hungerford report that every thousandth individual is exposed to the possibility of developing avascular femur head necrosis. It is common between the ages on 0-60 years. The male - female ratio is 4: 1 . The pathophysiological basis of the condi tion was discovered in 1 948 by Chandler, who compared the circulation disturbance of the femur head with the occlusion of coronary ar teries in the heart and called the avascular fe mur head necrosis "the coronary disease of the hip". Etiopathology. This has not been clarified precisely. Data in the literature point to vari ous factors which predispose avascular femur head necrosis: �
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chronic steroid therapy alcohol a buse cytostatic treatment and X-ray irradiation a n occupation involving a risk of caisson disease sickle cell anemia or other he mo globinopathy (thrombus production) metabolic diseases (lipid metabolic d isorder, hyperg lycemia, hyperuricemia) storage illnesses (Gaucher's disease)
a
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autoimmune diseases (rheumatoid arthritis, systemic lupus erythematodes, etc.) injuries
Chronic steroid therapy and alcohol abuse are among the causes in 65-75% of idiopathic femur head necrosis. The blood supply of the femoral head is ensured by the circumflex ar tery of the femur. The arteria capitis femoris only rarely functions in adults. The anastomo ses ensure the blood supply of the femoral head, but the circumflex branches are re garded as terminal arteries, in the event of oc clusion, the supplied area necrotizes. Clinical symptoms. The onset of the ill ness is uncertain: pain is felt in the hip, the thigh, or possibly in the knee, and becomes stronger on loading. As the affliction pro gresses, the pain intensity fluctuates. Fluid ac cumulation is associated with joint inflamma tion, increasing both the pressure as well as the pain. Resolution of the inflammation may ease the soreness. Thigh muscle atrophy is also seen. The hip internal rotation, abduction and adduction become limited. Flexion - exten sion is maintained for a relatively long time. The limp is caused primarily by the pain, but later by the limited motion.
b
Fig. 28.56. Bilateral femur head necrosis: Stage 3 on the right-side, Stage 1 on the left. a: X-ray. b: MR image (horizontal plane).
28. Hip disorders
X-ray signs. The radiological phases of this condition were determined by Arlet and Ficat. These phases affect the therapeutic ac tivity. Since the changes in the early phase of the condition are difficult to identify on an X-ray picture, not only an anteroposterior film, but also Lauenstein views are taken. Both hips must be observed because of frequent bilat eral involvement. Ficat 's phases: Phase 0: This is the preclinical and pre radiological phase ("silent hip", no clini cal or radiological signs). The intra medullary pressure is increased. In 60% of the cases, the condition later progresses to Phase 1. Phase I. There are early clinical symptoms, but no radiological signs. A bone scan is advised. MRI is invaluable. The leading symptom is a sudden groin pain that radi ates to the thigh, which intensifies during the night. Movements are restricted (Fig. 28.56. a, b).
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Phase /1. Typical X-ray changes are observed: a wedge-shaped area with a sclerotic mar gin; the joint surface is intact (precollaptic state) . The clinical symptoms stagnate or progress. Transient phase If-Ill: S ickle sign: a sickle-shaped line appears under the j oint surface due to a subchondral fracture. Seg mental collapse is seen. Phase Ill: The joint surface has collapsed, and a sequestrum has separated. The j oint space is intact or wider. There is evidence of inflammation and fluid accumulation. The pain is severe and motion is limited (Fig. 28.56. a, b, 28.57). Phase IV: This is the terminal phase, evidence of secondary arthrosis is seen. The j oint space progressively narrows; osteophyto sis; the head is deformed. Limited j oint motion. Differential diagnosis. Having identified the clinical and radiological signs the diagno sis is not problematical, especially if the his tory has been precisely identified. This condi tion is to be differentiated from hip algo dystrophy, inflammation and tumors . Besides the X-ray image, the bone scan helps to verify the bone necrosis. CT defines the contours of the head. MRI and 3D CT tests provide particularly valuable assistance in confirming the diagnosis and in deciding on the treatment. Treatment. In the early phases non weight-bearing, swimming, gymnastic exer cises, nonsteroidal anti-inflammatory drugs, pain killers, physiotherapy and medical spa treatment may be recommended. However, the condition progresses and the conservative treatment does not lead to substantial results. The surgical treatment varies in the differ ent phases.
Fig. 28.57. Left side: Stage 3 femur head necrosis: with im ploded wedge-shaped sequestrum.
- In Ficat phases 1. and 11. , when, despite the bone necrosis, the j oint surface is still intact, drilling of the necrotized area, cancellous grafting and a vas-
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28. Hip d isorders
cularized implant may produce reasonable results . - In Ficat phase Ill. , unloading and transposition of the collapsed area may be attempted. This is done by inter trochanteric flexion osteotomy. A pre condition of this intervention is that the extent of the necrosis must be less than 50%. If it is more (in the maj ority of cases), total prosthesis implantation is the method of choice. - In Ficat phase Ill . , the condition presents as secondary osteoarthritis, and total prosthesis implantation is in dicated.
28.2.4. Coxarthrosis (hip osteoarthritis, arthrosis deformans coxae) This entity is characterized by deformity, pain and limited movement in the hip due to carti laginous and bone degeneration.
Symptoms. Pain, with progressing restric tion of movement, contractures and a limp. The j oint space becomes thinner, the joint contours are uneven, the j oint surfaces are de formed, and osteophytes, and degenerative cysts may develop. Incidence. In the population aged 65-74 years, X-ray signs (Kellgren) of hip osteo arthritis are present in 1 6-25%. Symptoms re quiring treatment are considerably less fre quent: 6- 1 0%. Male - female ratio is 1 : 2 . 5 . The ratio of primary and secondary hip osteoarthritis in the operated population is 65 and 3 5 % respectively. Etiopathology. Hip osteoarthritis has two different forms :
I . Primary hip osteoarthritis: Its cause is un known, however literature data suggest that the condition is determined. In paral-
lel with aging, the function of the cartilage cells changes, their proteoglycan produc tion decreases, which leads to a decrease in the water content of the intercellular matrix, and to damage of the integrity of the collagen network. Behind the genetic predisposition, gene mutations producing faulty collagen, or ill-defined antigen as sociations may be part of the cause. 2 . Secondary hip osteoarthritis : This devel ops on the bases of other hip disorders. These may originate from childhood or adulthood. The roles of congenital dislo cation, Perthes ' disease, epiphyseolysis capitis femoris, primary acetabular protru sion and inflammatory diseases were dis cussed in the chapter on prearthrosis. In adults, inflammatory diseases (septic arthritis, rheumatoid arthritis and spondyl arthrosis ankylopoetica), idiopathic aseptic necrosis of the femoral head, fractures involv ing the joints, post-traumatic conditions near joints, metabolic diseases, bone dysplasia, and arthropathy may all result in secondary hip osteoarthritis. The pathology of osteoarthritis was dis cussed earlier in connection with diseases of the j oints (Chapter 1 9). Macroscopically, the cartilage is no longer gleaming, its mother-of-pearl white color has turned to yellowish brown. The hyaline is fiberized and softened in places. The areas subj ected to the greatest surface load have no cartilage cover, and ebumated (condensed) bone is visible. The subchondral cavities and degenerative cysts often communicate with the j oint cavity. The femoral head gradually loses its ball shape, and widens due to the marginal osteo phytes. The appositions that grow around the facies lunata in the center of the acetabulum may block the fossa, and a double acetabular bottom may be formed. These central osteoarthritic products (socket bottom osteo phytes) literally displace the femoral head and secondary subluxation is observed. B esides the changes in the cartilaginous bony structures, thickening, inflammation or
2 8 . H i p d i s o r d e rs
shrinking of the capsule may also play a role in the complaints and symptoms. In consequence of the changes listed above, the condition known as hip osteoarth ritis or arthrosis deformans coxae develops. Clinical symptoms. The main complaints are pain, limited movement and a limp. Ini tially, the pain presents only after getting out of bed in the morning, or upon standing up from a chair. It may subside during moving. Later on, the pain increases and becomes con tinuous, both in the daytime and at night. The pain may be located deep in the hip, in the groin, around the greater trochanter or the sacrum, or in the front of the thigh, radiating to the knee. The patient' s quality of life depends on the extent of the pain. As the condition pro gresses, the patient' s working and walking ability diminishes, and he / she may ulti mately become unable to take care of him selflherself. The limiting of their movements is recog nized while they attempt to carry out everyday tasks. In the beginning it may be difficult to put on a pair of socks, or to get on a bus . The limits of movements of the joint gradually narrow. First the extension becomes limited, i.e. a flexion contracture arises. Later the in ternal rotation decreases, which is followed by abduction and adduction. Monoaxial movements may succeed when the hip joint has only flexion and extension, but ultimately the flexion also becomes limited, and the hip is in such a state that only very limited move ment remains . Flexion, adduction and an ex ternal rotation contracture usually prevail. The limp is due to the clinical symptoms listed above, the pain resulting in a protective limp. Because of the flexion contracture, the hyperextension phase of the step is missing. Adduction flexion contractures cause an ap parent limb length difference, which increases the limp. X-ray signs. In the evaluation ofthe radio logical picture of the primary and secondary forms of hip osteoarthritis are differentiated.
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Primary forms (Fig. 28.58) : The j oint space is narrow. - The j oint contours are uneven. - Subchondral sclerosis is observed (on loaded areas) . - Degenerative cysts develop. - Osteophytes are seen at the socket margin and on the head - neck border (collar osteophytes). - A double socket bottom may be present. -
The primary form may be associated with protrusion. The head may be lateralized, when the thickened double socket bottom sublux ates the head from the central position.
Fig. 28.58. X-ray image of bilateral primary hip osteoarthritis.
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Secondary forms (Fig. 28.59) : - When the head is not central and the incongruence is severe, the hip osteoarthritis is usually secondary. - The same X-ray signs are present as in the primary form. - The X-ray signs depicting former disorders, which have not fully recovered anatomically may be identified (e.g. congenital dislocation (Fig. 28.60.), epiphyseolysis capitis femoris, Perthes disease or other types of head necrosis, j oint infection, etc. ) . '
Differential diagnosis. This condition is identified without any difficulty thanks to the
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28. Hip disorders
(arthritis and inclplent spondylarthritis ankylopoetica), or knee diseases (meniscal tear, arthrosis, etc.). On the other hand, pain radiating from the hip to the knee may suggest the presence of a knee disorder.
Fig. 28.60. Secondary hip osteoarthritis on basis of bilateral de velopmental dislocation of the hips with typical short neck and high trochanter.
clinical and radiological characteristics. Symptoms of diseases of the neighboring re gions may occasionally hinder establishment of the clinical diagnosis. These can include degenerative involvement of the lumbar spine accompanied by root irritation (e.g. lumbar disc hernia), diseases of the sacroiliac joint
Treatment. Before an account of the treatment methods, mention should be made of the means of prevention. In the prevention of both types of hip osteoarthritis, a healthy lifestyle, based on healthy nourishment and regular exercise, is essential . Body weight must be kept at the optimal level or weight loss should be considered if necessary. Even in the presence of a prearthrotic state, regular swimming, gymnastics and sports are those factors, which are able to protect the compen sated state of the hip musculature for a long period and to slow down the progress of the disease, which eventually requires either con servative or surgical attention. Apart from changes in lifestyle, it may also be necessary to change schools or working conditions. To prevent secondary hip osteoarthritis, the best possible treatment of the primary dis ease is indispensable. The incidence of con genital dislocation of the hip has decreased significantly in recent decades due to the early screening and successful treatment. The ef fective management of epiphyseolysis capitis femoris and Perthes ' disease has also led to a decrease in the incidence of hip osteoarthritis. Septic arthritis and hip tubercolosis, which earlier resulted in ankylosis (full stiffness of the joint) have now become conditions that rather induce hip osteoarthritis. Early diagnosis and early and appropriate treatment may delay severe hip osteoarthritis. The results are modest in cases when the hip osteoarthritis develops after femoral head ne crosis and fractures involving the joint. Conservative treatment: is applied in hip osteoarthritis to decrease the pain, to ease the contractures and the limp, and to increase the range of motion, and muscle power. Medica tion, physiotherapy and spa treatment may be employed. The following medications or their combi nations may be effective :
28. Hip disorders �
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Pain killers Nonsteroidal anti-inflammatory d rugs Steroid d rugs Muscle relaxants.
The most important goal is to relieve the pain. Instead of general pain killers, non steroidal anti-inflammatory drugs are pre ferred, since the osteoarthritis-induced in flammation is a significant source of the j oint pain. Muscle spasm and contracture may be alleviated by a combination of these drugs and muscle relaxants. Steroid therapy is a very effective element of the anti inflammation treatment. It does have harmful local and general side effects, however, caused by the chronic steroid ther apy: gastrointestinal ulcer, osteoporosis, ste roid necrosis, steroid diabetes and Cushing 's syndrome. Local steroid use (intra- or para-articular) may be indicated for a certain time period and in a limited dose. A traction device is often applied to unload the hip joint, and to decrease the spasm and contractures and the pain. It can be used even at home, combined with other methods. A walking stick is also useful. Surgical treatment. In prearthrosis or moderate hip osteoarthritis, femoral and pel vis osteotomies are performed to improve congruence and containment (see Chapters 2 8 . 1 . and 2 8 .2 . 2 . 5. ). I n advanced cases, the obj ective of surgi cal treatment is to decrease pain and restore movement and stability. Primarily a hip endo prosthesis is implanted; occasionally, stiffen ing of the hip or (after prosthesis removal) pseudoarthrosis formation is carried out. - Hip arthroplasty - Hip arthrodesis - Resection, or excision arthroplasty
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The system of hip endoprostheses i s the fol lowing: �
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surface replacement endoprostheses cervicocapital endoprostheses total hip endoprostheses cemented uncemented hybrid
Surface replacement endoprostheses. The key procedure of this method is double cup arthroplasty. After the removal of the dam aged joint surfaces, a plastic socket is ce mented into the acetabulum, and a matching metal cup is cemented onto the femoral head. Earlier, the results of this operation were modest, but it has nowadays been resurrected in a form involving a metal cup - metal head. Cervicocapital endoprostheses. Follow ing removal of the head and the neck, a cervicocapital endoprosthesis can be inserted into the medullary cavity of the femur. The metal head, moving in the cartilage-covered bony acetabulum, ensures the joint function. This procedure is mainly employed in opera tions involving the elderly, following frac tures of the femoral neck. This endoprosthesis is also manufactured in a bipolar form: a large metal ball is positioned in the cartilage cov ered bony acetabulum, inside this ball is a plastic bushing containing a small metal ball. The bipolar design increases the range of mo tion and the lifetime of the implant.
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Hip arthroplasty The total hip endoprosthesis embodies the most noteworthy achievement in orthopedics. A patient with a painful restriction of move ment can by this means be freed from all the complaints, and the mobility and quality of life restored to normal.
total hip endoprosthesis. Augmentation (:;,���
of the acetabular roof with bone block a nd screws.
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28. Hip d isorders
Total hip endoprosthesis / cemented (Fig. 28.61). After exposure of the hip j oint, the head and neck are resected. The acetabular cartilage is removed, the socket is deepened and the artificial cup is cemented home. After the preparation of the femur medullary cavity, a corresponding prosthesis stem is fixed. A metal head is assembled onto the cone of the metal neck, which is reduced to the polyethyl ene artificial socket. Bone cement (consisting of polymethyl metacrylate) is made from two components (fluid + powder). After mixing of the compo nents, they polymerize, and the cement sets through an exothermic reaction (ca. 80 QC) the cement is setting. This substance fixes the prosthesis firmly in the bone cavity surface. Total h ip endoprosthesis / cementless (Fig. 28.62.). The cementless implants are different in shape and surface from the ce mented ones. No bonding material is utilized in these cases. The cup consists of two com ponents : an external metal ring and a plastic lining. The surface of the external metal ring is threaded, and can be screwed into the bony acetabulum, but may also be designed to be pressed firmly into the bony bed (press fit). The plastic lining covers the head of the pros thesis. The stem of the femoral components matches the anatomical configuration of the medullary cavity (anatomical prostheses). The medullary cavity is prepared with ream-
ers matching the shape of the prosthesis, which will therefore wedge into the cavity (press fit). After the operation, the wedge ef fect and the press fit will ensure primary sta bility; later, especially in cases of a porous surface, bony ingrowth takes place, and sec ondary, biological fixation evolves. The po rous surface ensures attachment to the bony bed over a large surface. Total hip endoprosthesis / Hybridfixation. One component is fixed with, while the other without cement. The expected survival of both cemented and cementless endoprostheses is similar: 90% at 1 0 years . After the setting of the cement, the ce mented prosthesis is totally stable and ready for weight bearing. This prosthesis has the negative feature, that if the implant becomes loose, removal of cement may cause bone loss. The biological fixation of cementless im plants may take some time, but in the event of its loosening, the exchange does not create bone deficiency. The indications of a cementless implant are young age and a good bone structure ensuring the proper primary stability. In cases of poor bone quality (osteo porosis or necrosis), defective anatomical sit uation (previous operations, a dysplastic acetabulum, etc.) the cemented procedure is recommended. Minimally invasive endoprosthesis tech nique. This is the newest enterprise of pros thetic surgery. In a selected patient cohort (young, active patients with no obesity, etc.), the surgical incision is short (under 10 cm), and the joint capsule is preserved as much as possible, possible using minimal trauma tization. The postoperative pain and blood loss are expected to decrease, mobilization is swift, with a better cosmetic outcome. There are also some risks to be considered, e.g. mis placed components due to the limited view, unexpected tissue damage and bleeding. �
Fig. 28.62. Cementless total hip endoprosthesis.
Fusion of the hip j oint, arthrodesis This has almost totally been abandoned. The procedure, which ensures pain-free sta bility, was used in young patients who ac-
28. Hip disorders
quired a severe unilateral hip condition, while performing standing or physically demanding jobs. A good function of the lumbar spine and the knee are preconditions of this arthrodesis, since these structures will take over the lost motion of the hip and their load will increase. The increased load will sooner or later result in the degenerative changes in the mentioned joints, and the ipsilateral knee and contra lateral hip osteoarthritis will also develop. These are the drawbacks of the arthrodesis. �
Resection (excision) arthroplasty Resection (excision) arthroplasty is the fi nal solution in septic/aseptic failures of hip endoprosthesis procedures. The stem,
Fig. 28.63. Girdlestone situation following removal of total hip endoprosthesis.
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acetabulum (and possibly the cement) are all removed ( Girdlestone operation; Fig. 28.63 . ) . This state was earlier considered t o be final. Today however, the patients demand a good quality of life, therefore if the eradication of the infection is safe and the anatomical cir cumstances allow, prosthesis replantation may be attempted. �
Sterile loosening of a hip endoprosthesis and revision arthroplasty According to wide-range statistics, 90% of cemented and cementless endoprostheses re main stable at 1 0 years . This proportion is lower for young adults and in certain cases of secondary hip osteoarthritis (75-80%). The cause of the sterile loosening is that the bone, the cement and the material of the implants all have different moduli of elastic ity. The wear on the substances of the im plants is also a contributing factor. Granules of polyethylene, metal and cement can accu mulate in the joint, together with hystiocytes and foreign body giant cells, which may gen erate tissue reaction. This persevering granu lation tissue invades between the implant and the bone and induces osteolysis, leading to loosening of the implant. In these cases revi sion arthroplasty is advocated, which ac counts for 20-25% out of all endoprosthesis interventions. The success of the revision is dependent on the anatomical circumstances perceived during operation. If no bone loss is revealed, revision is not problematical. Some years ago, in the event of maj or bone loss, only the Girdlestone state was reasonable : the patient walked on the pseudo-arthrosis with a sup porting walking aid, with a pronounced limp but without much pain. Today there are de vices to replace bony defects of both the acetabulum as well as of the femur. The new bone bed receives the new implant with a sta bility matching that ofa primary intervention. The 1 0-year survival rate of revision arthroplasties is similar (about 80%) to that of the primary interventions .
29.
Laszl6 Hangody, M i kl6s Szen d r6i
Disorders of the knee
29. 1. Structure and functional anatomy of the knee The knee is the largest joint of the body, with one of the most complicated structures; it is classified by its motions as a trocho ginglymus. The longitudinal axes of the femur and the tibia in the frontal plane (anatomical axis) enclose a lateral angle (the physiological valgus angle of the knee, which is approxi mately 1 73°). The knee is slightly eccentric: the lateral condyle is somewhat flatter, while the medial one is larger. The loading axis, however, runs along the line drawn from the femoral head through the center of the knee to the ankle joint, and the loads on the medial
1 --��------��.� ______*H�_ 2 � 3
15
14 13
12 --+'���
6
1. 2. 3. 4. 5. 6. 7.
7
8.
4 5
8 9 11 ----t--_+_
Fig. 29.1. Anatomy of the knee joint
and lateral half of the knee are therefore equal under normal circumstances. The knee joint may be divided into 3 com partments : patellofemoral, medial and lateral tibiofemoral j oints. The practicality of this di vision is that certain disorders (e.g. osteo arthritis) have different affinities for different compartments. During motion and walking, the joint sur face is loaded by extremely large forces. The stability of the joint in each phase is provided not by the bony structure and shape, but by strong ligaments with complicated paths (pas sive stabilizers) and muscles (active stabiliz ers) (Fig. 29. 1 .) . The medial stabilizers that prevent valgus displacement are the following structures : the dorsomedial capsule, the su-
10
9. 10. 11. 12. 13. 14. 15.
anterior cruciate ligament, posterior cruciate ligament, medial collateral ligament, semimembranosus muscle, medial superficial collateral ligament, menisci, patellar tendon, gracilis muscle, semitendinosus muscle, sartorius muscle, membrana interossea, tractus iliotibialis, tendon biceps femoris, lateral collateral ligament, popliteus tendon
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2 9 . D i s o r d e rs of t h e k n e e
perficial layer of the medial collateral liga ment, the medial capsule ligament, the poste rior oblique ligament, the muscles of the pes anserinus and the semimembranous muscle. The latter muscles are also internal rotators; they produce 20 an internal rotation of 20° at the knee bent to 90°. The lateral stabilizers are : the dorsolateral capsule, the lateral collat eral ligament, the m. popliteus, the iliotibial tract and the m. biceps femoris. (The popliteus muscle has an important function in addition to stabilization: it rotates the tibia inward at the start of flexion.) The other muscles ensure the approximately 40° of external rotation of the leg with the knee bent. The active rotation around the longitudinal axis of the leg is pos sible only with the knee bent and relaxed col lateral ligaments. To achieve this, the flexors attached to both sides of the tibia like reins ro tate the knee in and out. The internal rotation is limited to 20° by the tense cruciate liga ments, and the external rotation to 40° by the collateral ligaments. The central stabilizing system of the knee involves the menisci and the cruciate liga ments. The anterior cruciate ligament origi nates from the mediodorsal part of the lateral femoral condyle and is attached widely in the intercondylar eminence, between the anterior horns of the two menisci. It contains 3 bun dles, which are partially twisted around them selves. In each stage of flexion, a different bundle comes under tension. The posterior cruciate ligament originates from the ventro lateral part of the medial femoral condyle and runs backward and down to the tibial inter condylar fossa; it contains 2 bundles. Functions: � �
Both cruciate ligaments participate in protecting the lateral stability. The anterior cruciate ligament prevents a nterior subluxation of the tibia, while the posterior cruciate ligament prevents the posterior subluxation of the tibia and the a nterior skidding of the femur over the fi xed tibia d u ring walking.
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I n every phase of the knee flexion, when the collateral ligaments are somewhat loosened, they ensure the stability of femur on the tibia, and the axis of the control led rolling-slide movement d u ring knee flexion.
The foremost motion of the knee is exten sion - flexion, which is effected around the transverse axis connecting the epicondyles. The extent varies individually; it is generally around 1 3 0°, depending on the tension of the extensor apparatus, and the soft tissue-mass in the popliteal fossa. In extension, the collateral ligaments are stretched, for the radius of the sagittal curvature of the femoral condyles is greater frontally. The end-point of extension is secured by a number of factors, at the same time inhibiting hyperextension. These com ponents are the collateral ligaments, the ex tremely strong posterior capsule, and the tense cruciate ligaments. In the normal knee, about 1 0° of end-rotation occurs externally at the end of extension. In this situation, the passive stabilizers are tense, and the active ones (mus cles) may relax; thus standing with extended knees does not require much muscle activity to stabilize the joint. In this position, the joint is stable. Apart from flexion, all other move ments are pathological and relate mostly to damage to the passive stabilizers . Physical examination of the knee: the follow ing factors are to be assessed: � �
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The axis (anteroposterior. and sagittal plane) Fluid accumu lation Stability (anteroposterior and lateral, rotational) Range of movement Muscle power Contractures (soft tissue, skin, muscle, capsular or bony) The patel la reflex Meniscal tear Chondropathy and arthrosis signs
The technique of assessment is discussed in the section on each disorder.
29. D i s o r d e r s of the k n e e
29.2. Congenital developmental disorders 29.2. 1. Congenital knee dislocation This rare disorder, which is frequently associ ated with other developmental anomalies, may occur bilateral ly. The tibia is subluxed o r fu lly dislocated anteriorly in t he sagittal plane. The tibia plateau is attached to the ventral surface of the femu r condyle.
Clinical symptoms. This disorder is usu ally detected at birth or in babyhood; the se verity varies. In cases of subluxation, the proximal tibia is positioned anteriorly on the femoral condyles to different extents, the fe mur and the tibia of the patient enclose a fron tally open angle. In mild cases, the knee flexion is generally full, but in more severe forms it may be badly limited. When luxation is total knee flexion is possible, the flexor muscles are displaced in front of the joint and they act as extensors enhancing the hyper extension. In severe bilateral cases, the patient is unable to walk, or occasionally stands and walks on the posterior surface of the leg and popliteal fossa. X-ray signs. The lateral views clearly re veal the ventral subluxation of the tibial condyles. The confronting joint surfaces may be flattened. Therapy. The hips and the entire lower limbs must also be assessed, since the knee disorder may be associated with multiple de velopmental disorders . In minor cases, early conservative treatment may be successful. Gentle reduction may be attempted even in the first week after birth, followed by a circu lar plaster cast up to the groin. With a frequent change of plaster and pas sive exercises, the knee flexion can gradually be increased. In cases of subluxation or full dislocation, surgery is indicated. Elongation of the quadriceps tendon, and incision of the shrunk ventral capsule are followed by appli-
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cation of a plaster cast for 4-6 weeks. After that, intensive rehabilitation is essential.
29.2.2. Genu recurvatum The range extension o f the knee surpasses the normal 5- 0°.
Causes : - Congenital: the tibial plateau is inclined forward - Idiopathic: abnormal load, standing with a loose posterior capsule - Post-traumatic: the ventral part of the tibia growth plate is blocked - Compensatory: in neuromuscular disorders (quadriceps palsy caused by poliomyelitis, and forced knee hyper extension in cases of equinus foot) . Therapy. Depending on the etiology osteotomy or orthosis may be selected.
29.2.3. Developmental disorders of the patel la Complete absence of the patella (aplasia patellae) or hypoplasia is a rare congenital de velopmental disorder. The knee extension strength may be weakened, but usually does not cause a major functional deficit; the pas sive range of movement is full. It is more common that the patella devel ops from two or more ossifying centers, and they do not unite at the end of growth (bipar tite or tripartite patella). The hyaline surfaces of the patella are even, and the disorder is not a source of complaints, or only in the event of overload. A common finding is the high or deep po sition of the patella (patella alta or patella baj a) . Together with the factors discussed above, a high patella increases the chances of habitual dislocation; in the baj a position, the patella is compressed to the femur on flexion, leading to chondropathy and chondromalacia.
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2 9 . D i s o r d e r s of t h e k n e e
29.3. Repeated patella dislocation and subluxation 29.3.1. Habitual dislocation of the patella The patella dislocates laterally on every occa sion, when the knee is flexed. It is clearly seen and palpated. This occurs between the ages of 5 and 9 years, i n older i nd ividuals it is ob served only occasional ly.
Etiopathology. The explanation is the contracture of the quadriceps femoris muscle. The contracture may be congenital or the ef fect of palsy, but the most common reason is an inj ection (mainly antibiotics) into the quadriceps femoris muscle in infancy or early childhood. The inj ections lead to fibrosis, which is the cause of the extension knee contracture in the maj ority of cases; patella dislocation also occurs in around 20% of the patients. C linical symptoms. At every flex ion of the knee, the patella slides down from the lat eral femoral condyle to the lateral side. Ifknee flexion is attempted while the examiner man ually holds the patella in place to prevent the lateral luxation, only 3 0-40° of flexion can be achieved and the muscle is very tense. Treatment. The patella slides over the prominent edge of the lateral condyle at every luxation, and its hyaline surface may therefore be damaged after a time. Surgery is recom mended to elongate the quadriceps tendon, to incise the lateral capsule and to tighten the medial capsule.
29.3.2. Recurrent dislocation and subluxation of the patella The patella occasionally undergoes dislocation or subluxation lateral. Fol lowing the acute epi sode, swelling and pain are observed. This
disorder occurs in pU berty. It is 3 times more common among girls than among boys.
Etiopathology. Similarly to habitual dis location, this is lateral and presents first as a traumatic luxation. The history may indicate and injury, but this is usually not a direct ef fect on the patella. The dislocation is induced by a critical knee movement with distortion during some activity, jumping or sport. In re current cases, the patient knows exactly what movement causes luxation and tries to avoid it. The dislocation recurs again with various incidence, but usually increasingly more com mon. It may initially present once or twice a year, but without a surgical solution it may be come a daily event. An important factor in this disorder is the loosening of the medial capsule and reti naculum. Contributions may also be made by the following factors : a high patella, a hypo plastic lateral femoral condyle or lateral part of the patella, valgus knee or a lateral position of the tibial tubercle due to torsion of the leg. Clinical symptoms. It is not easy to estab lish from the history whether the patient origi nally suffered a patella dislocation or a meniscal tear. If the situation recurs the diag nosis is easier. Following an acute incident, the knee is swollen, and an accumulation of fluid or blood may be present. After a time, patella chondromalacia may develop. The medial edge of the patella is tender, and pressing the patella in a lateral direction elicits sudden and severe pam. X-ray signs. Serial films taken with dif ferent flexions of the knee of the prone patient demonstrate hypoplasia and a tendency to dis location. Treatment. The patient usually arrives following reduction of the dislocated patella (or it may have reduced spontaneously). If blood has accumulated it is evacuated and a plaster cast is applied. Active quadriceps ex-
2 9 . D i s o r d e r s of t h e k n e e
ercises are recommended later. Even after multiple luxation, a conservative method can be attempted, especially active quadriceps training. A special orthosis can be prescribed, enabling the patient to play sports . If the com plaints persist, surgical methods follow. Many procedures have been published, the es sence being incision of the lateral retina culum, tightening the medial and correcting the path of the ligamentum patellae by trans posing the tibial tubercle medially.
29.3.3. Luxatio patellae congenita This disorder is well differentiated from the above form, since it is present at birth. The patella lies on the lateral part of the femoral condyle (Fig. 29.2.), and is not reduced during motion; in fact, it is often irreducible even manually. The patella is hypoplastic, and the ligament is shortened. The knee is in valgus and externally ro tated. Swift operation is warranted, but it is not easy to reduce and fix the patella even surgi cally.
Fig. 29.2. Congenital patella luxation with characteristic lateralized patella, and shallow patellar groove on the femoral surface.
29.4. Developmental disorders of the menisci �
Discoid meniscus
The embryonic meniscus has a discoid shape. If this persists after birth, the condition is called discoid meniscus: instead of the normal
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sickle shape, the meniscus resembles a disc. In 95% of the cases, the lateral is involved.
Clinical symptoms. Discoid meniscus is detected among toddlers. At the beginning of knee flexion and at the end of extension, a typ ical clicking sound is heard, with a few de grees of valgus - varus or external - internal rotation movement. This condition is more vulnerable than with the normal meniscus, and it is symptomatic if a central tear appears after minor trauma or distortion. Treatment. The weak, thin, tom central part is excised via arthroscopy, leaving the pe ripheral part intact.
29.5. Developmental disorders of the tibia and fibula 29.5. 1. Tibia, fibula aplasia, hypoplasia Tibia hypoplasia results in a shorter leg. Partial or total aplasia often presents together with other developmental disorders (see Chapter 1 5) . The leg is usually bent to varus, while the foot is in equinovarus because of the missing medial ankle.
29.5.2. Congenital tibia pseudarthrosis At birth, the leg may be bent in an anteriorly convex shape. Fradure o r pseudoarthrosis de velops later, usual ly in childhood. The typical findings are the angulation between the mid d le and distal third of the leg and occasional pathological movements.
Clinical symptoms. Curvature with fron tal and lateral angulation is seen between the middle and distal third of the leg. If fracture has occurred, acute angulation and irregular movements are detected. The X-ray picture shows the described bend of the tibia with sclerosis or cystic zones,
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2 9 . D i s o r d e r s of t h e k n e e
o r interruption of the bone integrity. The fib ula may be intact, curved or also pseudo arthrotic. The etiology is unclear. It often accompa nies neurofibromatosis (see Chapter 1 3) . Cafe au lait spots are then found on the skin, and neurofibromatosis nodules. It may also be as sociated with fibrous dysplasia. Spontaneous fracture often occurs at the location of the curve detected after birth. The tendency of pseudoarthrosis to heal is ex tremely poor. Surgery (possibly repeated) succeeds. Otherwise, a caliper splint is pre scribed.
29.6. Axial deformities 29.6. 1. Genu varum - crus varum This deformity i s characterized b y the medially convex angle enclosed in the frontal plane between the axes of the femur and the leg (Fig. 29.3.).
Symptoms. The distance between the me dial surfaces of the knees, which is normally a few centimeters, may be greatly increased. The condition may be present unilaterally, but more often it is bilateral, giving rise to bowleg. Incidence. This situation is normal in in fants and toddlers, and gradually turns to a mild valgus position (knock knee) by the age of 8- 1 0 years. Varus deformities that persist after this age and progress are considered to need correction. The disorder appears in both genders, but is more common among boys. Etiopathology. Varus deformities may be idiopathic or of known origin. A number of conditions may lead to varus knee : - Metabolic bone disease (the weakened, occasionally soft bone bends under a load), e.g. rickets, fibrous dysplasia, Paget ' s disease or osteomalacia. - Congenital connective tissue weakness, or loose ligaments - Tibia vara epiphysarea
Fig. 29.3. Genu varum; the loading axis of the lower limb is displaced from the medial part of the knee in the direction of the medial line of the body; the medial knee compartment is overloaded.
- Medial damage to the tibial proximal or femoral distal growth plate - Malunited femur or tibia fractures with axial deformity - Compensation (as a consequence of an abduction hip contracture) - Medial osteoarthritis Clinical symptoms. The deformity is usu ally bilateral. It is symptomless in childhood, and the parents take the child to the doctor merely because of the deformity. Later the leading symptoms are quickly developing tiredness, pain after loading, and an uneven gait. Common further signs are internal rota tion of the leg and flatfoot. In untreated cases, overloading of the me dial part of the knee joint (Fig. 29.3.) results in early arthrosis and instability. In severe cases, the lateral ligaments may become distended, and the knee will wobble. X-ray signs. The extent and the punctum maximum of the axial deformity are best visu alized on a film of the entire lower limb. In
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later stages the X-ray signs of osteoarthritis are seen in the medial joint surfaces (narrow ing of the joint space, marginal osteophytes, sclerosis, etc.) (Fig. 29.3.). Treatment. The etiology should be identi fied and the deformity should be prevented (e.g. recognition and treatment of rickets, osteomalacia and osteoporosis). A mild defor-
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mity detected in a toddler does not require treatment. At a later age, the marked varus is presumed to be a prearthrotic factor, warrant ing a surgical solution. Corrective osteotomy is performed at the metaphysis near the punctum maximum of the deformity, most of ten on the tibia.
29.6.2. Tibia vara epiphysarea (Blount's disease) This is a relatively uncommon disease, which involves dysostosis of the proximal epiphysis metaphysis of the tibia, resulting in a varus knee deformity. The rapidly progress ing varus deformity usually starts in child hood, occasionally around the age of 1 0 years (Fig. 29.4. a, b). The treatment in tall children is temporary closure of the distal growth plate with staples (epiphyseodesis), or usually valgus tibia osteotomy. Because of the pro gression of the condition the operation must sometimes be repeated.
29.6.3. Genu valgum - crus valgum
a b
Fig. 29.4. Bilateral severe Blount disease with varus deformity. a: Clinical picture. b: X- ray image
I n cases o f valgus knee, the axis o f the thigh and the axis of the leg enclose a lateral con vex angle i n the frontal plane (Fig. 29.S.). The distance between the medial ankles with the knees closed together is considerably in creased. In childhood, this deformity is often physiological, and at the age of 8- 10 sponta neous correction is expected.
Etiopathoiogy. The condition may be of idiopathic or known origin. The causes can be similar to those of varus knee, but resulting in the opposite deformity: - Metabolic bone disease e.g. rickets (but this usually causes a varus deformity). - Dyschondroplasia, and multiple exo stosis. - Connective tissue weakness, and loose ligaments. - Damage to the lateral part of the distal femoral growth plate (osteomyelitis, tu mor, trauma or injuries).
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- Malunited femur o r tibia fractures with an axial deformity. - Muscle paralysis. - Loosening following medial collateral ligament injuries. - Compensation (as a consequence of an adduction hip contracture) . Clinical symptoms. When the knees are closed together, the medial ankles can not come into contact, and the patella is turned upward. This is recorded in centimeters. The valgus is the most common deformity of the knee joint; it is often associated with recurvatum. In toddlers, this situation is not considered abnormal if the distance between the medial ankles is not more than 5 cm; it corrects spon taneously by the age of 8- 1 0 years. Valgus knee is usually associated with mild valgus heel, and the parents take the child to the doc tor not because of any complaints, but be cause of the ugly gait. In these cases, only a supination heel elevation and foot exercises are needed. In adults, a pronounced valgus deformity is a prearthrotic factor (Fig. 29.5.). Early
chondropathy and osteoarthritis can develop, with knee instability, leading to serious com plaints. X-ray signs. The extent of the deformity is well visible on weight-bearing films. In a later stage, evidence of osteoarthritis is seen in the lateral compartment. Treatment. It is important to determine the cause and to treat this condition early, be fore the deformity develops. In more serious cases, surgery is advised: If the growth plate persists, temporary epiphyseodesis with sta ples in the medial side (Blount ' s procedure) is possible. Corrective varus osteotomy can be carried out on either the femur or the tibia, as described in the Chapter 29.6. 1 .
29.6.4. Torsion of the tibia Varus and inward torsion of the tibia is a common condition under the age of 1 year, and later it can improve spontaneously (see Chapter 1 2) .
29.7. Aseptic osteochondroses around the knee 29.7. 1. Osteochondritis dissecans genus
-' UIII�....lI..Q9&
Fig. 29.5. I n valgus deformity, the loading axis is lateralized, and the lateral compartment of the joint is there fore overloaded.
This entity i nvolves aseptic bone necrosis which occurs in the joints. In the majority of cases it affects the lateral part of the medial tibial condyle. The necrotized cartilage - bone piece may be detached, forming a loose body and leaving a crater-like hollow 1-2 cm in di ameter in its place. It is observed in young adults, i n half of the cases bilateral ly.
Etiopathology. The cause is not clear: roles may be played by constitutional factors, repeated micro injuries (athletes), circulation anomalies, and the closure of end-arteries. Clinical symptoms. Initially mild pain and swelling are experienced, the flex ion may
29. D i s o r d e r s of the k n e e
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be restricted, and the limit may be painful. If the necrotized osteochondral piece is partially detached, the condition may mimic a meniscal tear; if it is fully detached, forming a loose body, it may cause a joint block or become palpable. X-ray signs. The anteroposterior views of the knee, or rather the special views, show a bean-shaped bony body measuring about 3 cm on the lateral side of the femoral condyle. Treatment. If the disorder is in an early stage, the extent is limited and the joint con gruence is intact (MRl scan), the cessation of overloading (sport) may result in spontaneous healing, especially in adolescents. If partial detachment occurs, the surface of the crater is refreshed, the necrotized parts are removed and the fragment is fixed with absorbable "pins" in an arthroscopic procedure. If the crater is too large and predominantly involves the weight-bearing surface, autologous or ho mologous grafting may be indicated.
29.7.2. Osteochondritis of the tibial tubercle (Schlatter-Osgood's disease) Schlatter-Osgood's disease is juvenile osteochondrosis of the tibial tubercle. It is most common in the age range 1 1- 16 years and affects both boys and girls.
Etiopathology. This is aseptic bone ne crosis of the tibial tubercle. Clinical symptoms. The complaints often start following heavy loading or after direct pressure on the tibial tubercle. Forced knee extension against resistance may elicit pain. A swelling is seen over the tibial tubercle, but other signs of inflammation are missing. X-ray signs. The lateral view of the knee the apophysis of the tibia is fragmented, scle rotic and protruding (Fig. 29.6. a, b).
Schlatter-Osgood disease.
a. The lateral X-ray picture shows the tibial tubercle as an independent bony fragment. b. Characteristic prominent tu bercle when the knee is bent.
Treatment. The ossification disorder usu ally heals in 2 years. It responds well to con servative treatment, several weeks or months of rest, avoidance of physical exercises, occa sionally cold compress, creams, and non steroidal anti-inflammatory drug administra tion. In stubborn cases, a plaster cast up to the groin may be applied for 4 weeks. The com plaint may recur up to the end of growth at around 1 8-2 1 years, when the tibial tubercle
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unites with the tibia. If the tibial tubercle pro trudes to a maj or extent· and disturbs the pa tient during kneeling, the persisting bone frag ment is excised.
29.7.3. Adu lt aseptic femur condyle necrosis (Ahlbiick's disease) Spontaneous aseptic osteonecrosis usually starts after the age of 60 years on the load bearing area of the medial condyle of the fe mur, less frequently of the tibia . .
a b
Fig. 29.7. A 71-year-old female with necrosis of the right medial femoral condyle (M. Ahlbiick disease). a: Anteroposterior X-ray picture; the joint su rface of the medial femoral condyle is flattened and cracked (arrow). b: MR shows the intraosseal extent of the necrosis. c: I ntraoperative picture: the almost separated femoral su rface is visible; the cartilage of the knee is intact else where. d: Unicondylar prosthesis inserted (immediate postopera tive film).
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Etiopathogenesis. The reason is unclear. It is usually unilateral, and the extent is al ways larger than in juvenile osteochondritis. It often results in varus knee. Symptoms. The knee becomes progres sively more painful, and the range of move ment is extremely restricted. The diagnosis is established by X-ray examination. The surface of the condyle is uneven, later becoming flat. The MRI scan illustrates the extent of the defect (Fig. 29.7. a-d) . Treatment. Condyle necrosis in the el derly warrants sledge prosthesis insertion.
29.8. Knee contractures Flexion may be restricted (extension contracture), or extension may be restricted (flexion contractu re), or both may be limited, for many reasons. The contracture may be due to a muscu lar disorder, an innervation anomaly or shrinkage of the capsule, the liga ments or the skin. A special form is postoper ative arthrofibrosis (fibrous a nkylosis).
Etiopathology: - Inflammation and swelling - Trauma and distortion - Neuromuscular diseases (Heine-Medin disease, or infantile cerebral paresis) - Congenital anomalies (knee dislocation) - Bums or scars after operations or InJunes - Arthrosis - Tumors adj acent to j oints - Iatrogenic or repeated knee operations
Flexion contractures and spasms of the knee muscles are most common in cases of in fantile cerebral palsy Extension contractures of the knee are dis cussed separately. Etiopathology. The cause of its congeni tal form is unknown; it often appears with other developmental abnormalities. The
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flexion is limited t o 1 0-20°. The quadriceps femoris muscle is fully or partially shortened. The most common cause of the acquired form is the scarring that follows antibiotic inj ec tions into the thigh, or the m. vastus lateralis or intermedius in infancy. Clinical symptoms. The child walks with a stiff knee, and the hip is therefore abducted and externally rotated. The child is unable to squat, and keeps the knee extended while sit ting. The thigh circumference is less than on the other side, and the quadriceps muscle is atrophic, and scarred. One special form of this extension contracture is isolated shortening of the m. rectus femoris, which can be congenital or a consequence of inj ections. The symptoms are similar to those mentioned above. This mus cle bridges over two joints, and the physical findings are therefore different when the hip flexed or extended. If the child is supine, the knee flexion seems to be complete with the hip flexed. In the prone position with the hip extended the knee flexion may be only 5 - 1 0°, and further flexion is possible only with flexion of the hip. Treatment is surgical, the tendinous part of the hip flexor/knee extensor muscles being lengthened above the knee.
29.9. Fluid accumulation (effusion) i n the knee This condition causes swelling of the suprapatellar pouch, patella ballottement and restriction of the range of movement. The fluid may be serous (hydrops), bloody (hemarthros) or inflammatory (serous, sero fibrinous or purulent) . Genicular synovitis may be due to: - bacterial infection (via direct or hematogenous spread) - viral infection - specific conditions (e.g. tuberculosis)
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Genicular abacterial synovitis may b e due to:
- postbacterial allergic processes (tran sitory arthritis) (rheumatoid diseases - rheumatic arthritis, etc. ) - metabolic diseases (gout, ochronosis, pseudo-gout, etc.) - osteoarthritis - post-traumatic joint deformities - neural diseases (syringomyelia, neurosyphilis, etc. ) - tumors adj acent t o j oints Hemarthros may be due to: - hemophilia (spontaneous bleeding or microtrauma) - hemorrhagic diathesis - trauma (fractures involving the j oint or cruciate ligament and meniscus rup tures) - a tumor or tumor-like condition in vading the j oint (e.g. villonodular synovitis ) . Clinical symptoms. In acute synovitis, the knee is swollen and hot, and the skin is red. The suprapatellar pouch is swollen, and patella ballottement is detected. Diffuse ten derness and strong pain are present. The range of movement is severely restricted; the joint may be blocked in mid-position. In chronic synovitis, the severe pulsating pain and the hot skin may be missing. The joint may be "dry", when patella ballottement is absent, but the knee is swollen due to the thickened synovial membrane, and the parapatellar fossa is smoothed. In the form where considerable amounts of synovial fluid are secreted, the patella ballottement is pres ent, the amount of fluid may reach 1 50-200 ml. The range of movement is moderately re stricted. The X-ray signs are not characteristic; the j oint space may be widened, but there is no di rect evidence relating to the basic disease. MRI and ultrasonography may give more in formation (loose body, tumor, etc .) .
Treatment. The exact diagnosis and the underlying condition must be established. In cases of post-traumatic hemarthros (if this is proven by joint puncture), acute knee arthroscopy may be performed. Diagnostic joint puncture is usually considered neces sary. The aspirated fluid is sent for laboratory tests (to detect crystals, proteins, leucocytes, fungi, LE cytes, bacteria, etc.). The local treatment depends on the source illness, e.g. conservative treatment is preferred in primary osteoarthritis, whereas in rheumatoid arthri tis, if conservative treatment fails, syno vectomy is advocated. If pus is evacuated via the puncture, the subsequent therapy is in ac cordance with the treatment of acute or chronic bacterial synovitis outlined in Chap ter 1 9. 3 . 5 . 1 .
29.10. Cl'.sts in the knee region Cysts are common in the knee region. They may communicate with the joint, have a synovial lining and contain thickened synovial fluid.
29. 10. 1. Ganglion This type of cyst is common in the knee re gion, and also in the wrist, ankle and foot. It originates from the tendinous parts of the extensor muscles attached to the fibula head, with a size between 4 and 7 cm, and is easy to palpate. Exposure reveals a membranous cyst with a yellow, gelatinous content. Surgical re moval is advised, but recurrence is common.
29.10.2. Meniscus cyst This is a degenerative cyst, ongmating from the meniscus; it is 7 times more common laterally. Clinical symptoms. In young adults a thick nodule may be palpated at the lateral j oint line, possible be accompanied by im pingement, a click, and a blocking sign, char-
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acterized by a meniscal tear. It may be ob served at the edge of the meniscus or within the meniscus, when it is not palpable, then ne cessitating arthroscopy or an MRI scan. Treatment. Surgical removal of the me niscus cyst is recommended, in some cases with the entire meniscus. The residual menis cus may result in recurrence.
29.10.3. Popliteal cyst (Baker's cyst) This cyst, containing thickened synovial fluid, appears in the popliteal fossa on t he basis of processes causing chronic synovitis in the knee joints of adu lts. It is usually connected with the joint. Both genders are affected. It is also frequent in chi ldren. The cause is un known.
Etiopathology. This is not clear. Weak ness of the posterior capsule is assumed, for the synovial membrane may protrude by this route. On the other hand, chronic synovitis is considered to play a role, and based on an intra-articular cause (osteoarthritis, loose body, tom meniscus, etc.). It is often seen in rheumatoid arthritis. Clinical symptoms. At the outset the complaints are vague. Squatting is difficult, a
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and some radiating pain may appear. The cyst "expands" in osteoarthritis, following an overload, longer weight-bearing, and be comes palpable in the popliteal fossa (Fig. 29-8. a, b); during rest it decreases in size. The cyst is connected with the knee joint and an increase in knee j oint pressure pumps synovial fluid into the cyst. Later, the connec tion may become unidirectional and work as a vent, when no fluid may return from the cyst to the joint space. The size of the cyst may be constant or increasing. It must be differenti ated from aneurysms and tumors by ultra sonography, which is easy to perform and has 95% surety. In the event of doubt, an MR scan is performed. Treatment. The cause of chronic syno vitis of the knee must be eliminated first. If there are complaints, surgical removal is ad vised. In adults, recurrence is common, espe cially if the chronic synovitis of the knee per sists.
29. 10.4. Bursitis praepatellaris This is chronic inflammation of the prepatellar bursa, usually due to excessive mechanical loading (kneeling occupations, mason, or parquet layers). The inflammation may be acute, chronic, serous or rarely puru-
b
a: b: Intraoperative picture of the cyst, with a membranous wall and a transparent content.
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lent. The skin over the kneecap is hot, red, ten der and hyperkeratotic; the size of the swell ing varies between 7 and 1 0 cm. Treatment. In acute cases, the limb is put at rest, and a cold compress is applied, which together with aspiration, may heal the pro cess. In purulent bursitis, incision, drainage and surgical excision may be the solution.
29. 1 1. 1 . Col lateral ligament injuries Etiopathology. These injuries may be classified in terms of the extent of the force and the damage: overstretching of the liga ment which does not result in instability (sprain or distortion); or partial or total rup ture. The latter may involve the ligament itself or the bony attachment can be broken out. In ruptures, the damage usually disrupts differ ent layers of the ligament at different heights . A purely lateral force may result in a contralateral collateral ligament rupture to gether with tearing of the adj acent capsule, causing simple or uniplanar moderate knee in stability. More common is the complex rota tional knee instability that arises when the force acts in several planes . Flexion - valgus external rotation injuries lead to anteromedial instability, usually with simultaneous tearing of the medial collateral ligament, anterior cruciate and medial meniscus ("unhappy triad ''), sometimes associated with tearing of the capsule and the posterior oblique liga ment. Less frequent are the flexion - varus internal rotation injuries, when the lateral cap sule, lateral collateral and anterior cruciate ligament are damaged, with consequent anterolateral knee instability. Hyperextensive forces or injuries forcing the head of the tibia dorsally cause posterior instabilities, which may likewise be combined with medial, lateral and rotation components, as above. In extremely high-energy injuries (e.g. a traumatic knee dislocation), the complexity is even greater: the anterior and posterior com ponents are combined with lateral elements,
possibly aggravated by bone, vascular, neural and tendon injuries. Clinical symptoms. In fresh injuries pain, swelling, hemorrhage and direct tenderness to palpation are detected along the path and over the attachment of the collateral ligament. Iso lated collateral ligament rupture can not be identified with the knee fully extended, since the strong posterior capsule partially takes over the function of the collateral ligament. Accordingly, the test for the integrity of the collateral ligaments is performed at 1 0-200 of flexion, with valgus stress for the medial, and the varus test for the lateral collateral liga ment. In the event of damage, mild ' opening' is detected in response to the above stresses, denoted by , +, ++ or +++, depending on the extent of the ill-defined movement. In cases of isolated collateral ligament tears the intact cruciate limits the mild ' opening' to + or ++ ; in cases of +++ ' opening ' , therefore, cruciate and oblique posterior ligament ruptures are also present. To substantiate these findings, X-ray examinations can be performed, the joint being hold in a stressed position (the pain-induced muscle spasm may hide the in stability) . In cases of doubt, ultrasonography and MRI can be added to confirm the diagno sis. -
Treatment. Isolated tears of the collateral ligament can be treated conservatively be cause of the excellent blood supply due to the location of this ligament. Bone disruptions are treated surgically.
29. 1 1.2. Chronic collateral ligament insufficiency Etiopathology. This is exceptional as iso lated damage; it usually occurs following the partial healing of a complex ligament rupture. In the majority of cases, it is an element of an anteromedial or posterolateral instability. The following additional factors may lead to a chronic knee collateral ligament insuffi ciency:
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- a marked varus or valgus deformity - a marked hip abduction or adduction contracture as a compensatory symptom - rheumatologic diseases - arthrosis Clinical symptoms. An isolated collateral ligament insufficiency elicits a moderate varus or valgus instability feeling, which pres ents during a physical load, sports activity or walking on uneven ground as an occasional knee distortion. The main complaint is the feeling of a lack of stability. Stress X-ray ex amination, ultrasonography or MRI may con firm the diagnosis. Treatment. The treatment depends on the degree of instability and naturally the associ ated injuries. Ifthe lateral instability is signifi cant, surgical treatment of the associated inj u ries (anterior or posterior cruciate ligament re placement, and replacement of the collateral ligament with autologous tendon fixed via bone holes) can be considered. The latter is a very delicate task, how with regard to how to position the graft isometrically. The best re sults are achieved laterally by cutting the bi ceps tendon in half, and medially by using one of the hamstrings.
Outline of the anterior cruciate ligament rupture.
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29. 1 1.3. Cruciate ligament inju ries These lesions are o bserved following a trauma; they lead mainly to various extents of sagittal i nstability of the knee.
Etiopathology. Cruciate injuries occur from the action of complex forces exerted in the sagittal plane; they may be isolated or as sociated with injuries of other components (Fig. 29.9.) . Injury to the anterior cruciate lig ament (ACL) is 1 0 times more common than one to the posterior cruciate ligament (PCL). The ACL usually undergoes tearing in the middle third of the ligament, and the ends are fringed, resembling a broom. Bony abruption is distal and more frequent in children. Clinical symptoms. These include pain, swelling, blood accumulation and instability, which may cause an inability to bear weight. In fresh cases, locking of the joint or an exten sion deficit may also occur, caused by the im pinged ligament parts. In chronic ACL or PCL inj uries, the patient often describe an in stability feeling, even on horizontal ground (the knee occasionally gives way), but it is more pronounced on uneven ground when the direction is changed.
\
Fig. 29.9.
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Fig. 29. 10. Drawer sign.
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In chronic cases, there are only slight signs of instability. The sagittal stability of the knee is characterized by the drawer sign, which is limited in fresh cases in muscular patients be cause of the pain due to the fixing ability of the active stabilizers. In cases of an ACL tear, the head of the tibia can be moved anteriorly on the femoral condyles to various extents with the knee flexed at 90°. This is the anterior drawer sign, and its extent is denoted simi larly to the lateral instability, on a +, ++ and +++ scale (Fig. 29.1 0.). In PCL injuries in the same flexed position the head of the tibia may be displaced posteriorly because of the rup ture. From this position, it may be reduced to normal and redisplaced posteriorly (posterior drawer sign). This may be pseudo-positive due to the general joint laxity: a comparative assessment with the other knee is advised. Ultrasonography may suggest cruciate lig ament injuries; the MRI almost certainly proves them. Treatment. Acute reconstruction of the ligament is not advised, even in fresh cases, because of the frequent failures. The only ex ception is distal bony abruption of the liga ments, which is currently treated with anchor ing of the abrupted bone and the attached liga ment. In the other cases, the first procedure is arthroscopy (treatment of other intra-articular injuries, meniscal tears and hyaline surface damage, and careful limited resection of the tom, impinging fibers of the cruciate) ; then conservative therapy is advised. Satisfactory stability may be achieved by training of the thigh muscles. When long-term conservative treatment does not terminate the patient ' s complaints of instability and a limited ability to bear weight, if the patient is younger than 50, ACL or PCL replacement may be per formed. The improving results of arthroscopic lig ament reconstruction have led to these proce dures becoming increasingly popular all over the world. This is supported by the observa tion that injured patients who have no instabil ity feeling may exhibit a certain "micro instability", and ACL or PCL-injured knees
therefore degenerate faster and severe carti lage wear may take place within a few years. The main role is definitely played by autologous grafts. Occasionally (especially in revisions), allografts may be used, but the ear lier popular plastic ligaments are no longer recommended. Ten years ago, the bone patellar tendon - bone graft was applied al most exclusively, whereas the positive experi ence has resulted in the replacement being carried out nowadays with four times folded semitendinous tendon, twice folded se mintendinous + gracilis tendon, or rarely quadriceps tendon grafts (Fig. 29. 1 1 .). During this procedure, which is usually performed
us grafts: the semitendinosus tendon is seen above, and the bone - patella tendon - bone
, - -..,- - � - ---,
Arthroscopic picture 1 year after insertion of a four times folded semitendinosus tendon graft.
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arthroscopically, the most important goal is the isometric position and stable fixation of the graft. To achieve this, interference screws are utilized in bone - patellar tendon - bone grafts, and (semintendinous + gracilis tendon) plates, staples, anchors and other special fix ing elements are used in cases involving ten don use (Fig. 29.1 2.).
29. 1 1.5. Meniscal tear These are very common i nj u ries of the knee. The meniscus becomes trapped between the femoral and tibial condyles and sustains rup tures varying in extent and loca lization.
Etiopathology. The mechanism of the in jury is usually a rotational force : while the leg
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is fixed, the femur is rotated inward or out ward. The femoral condyles push the menisci in front of them, and the meniscal edges may become trapped between the contacting joint surfaces. Predisposing factors are earlier or concomitant ligament injuries (most often the ACL), and axial deformities of the knee (genu varum, valgum or recurvatum), and the exten sively loose joints. In cases of osteoarthritis or rheumatologic conditions and in the elderly, degenerative changes of the menisci may result in frequent tears as a result of the repeated microtraurnas . I n the majority of cases (90%) the medial me niscus is involved, which is the less mobile, as it is anchored to the medial capsule and collat eral ligament. The tear takes place most often at the posterior horn and in the middle part of the meniscus (Fig. 29. 13. and 29.1 4.). Regardless of whether if the tear is lobular or bucket-handle-shaped, its interposition be tween the loading surfaces causes gradual hyaline cartilage damage. Clinical symptoms. In a typical case, the patient has a history of sports injury or trauma. Impingement during physiological motion relates to previous degeneration. The injury causes strong pain and is accompanied by the accumulation of serous j oint fluid. Blood accumulates when the meniscus is tom
Normal menisci (a). Longitudinal fissu re (b) and bucket-handle tear in the medial meniscus (c).
Arthr(),rnnir picture of a bucket-handle tear and
impingement of the medial meniscus.
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a t the base. One of the most important symp toms, which is often missing, is the joint lock due to the impingement of the tom part of the meniscus. In chronic cases, very little or no fluid may be accumulated, and the patient ex periences repeated locking and painful clicks in response to certain movements . The joint space is tender to direct pressure. In cases of suspected meniscal injury, the ligaments of the knee must also be assessed. X-ray signs. The ultrasonographic and MRI images reveal the location of the meniscal tear with relatively high accuracy. Treatment. Resection and particularly full excision of the menisci predisposes to osteoarthritis . The basic principle of the surgi cal treatment is to remove only the irreparably tom parts. Unfortunately, partial resection of the meniscus is a necessary intervention in most cases. At present the meniscus operation is almost exclusively performed arthro scopically. In young individuals, fresh and not too long tears may be reconstructed if they are lo calized near the base (this meniscus substance has a blood supply); this involves arthroscop ic resuturing of the meniscus. Reinsertion of the meniscus is possible only in a small pro portion of the cases and resection is usually considered necessary. The practice in these cases is removal of the tom, impinging parts of the meniscus and smoothing of their base with a shaver. If there are extensive ruptures near to the base in youngsters, and the j oint is otherwise intact, the expected osteoarthritis may be prevented by implantation of a fresh meniscus allograft. �
Cartilage damage The sliding surfaces of the knee j oint are covered with hyaline cartilage, which has ex cellent mechanical and weight distribution qualities, but a very poor disposition to regen erate (Chapter 1 9.8). There are zones of this surface which are heavily loaded, and others which bear less weight. The maximal load affects the central
areas of the femoral and tibial condyles; the peripheral areas which are covered by the menisci and the parts next to the intercondylar eminences are loaded with somewhat less weight. The 4-5 -mm-thick hyaline cartilage is un changed structure and is an excellent buffer: it ensures movement with almost no friction for many decades. On the other hand, if this struc ture is damaged, it has a very poor disposition to heal spontaneously.
29.13. Chondromalacia patellae This d isorder (involving early degenerative changes) has long been known, its name char acteristica l ly meaning softening of the carti lage su rface of the kneeca p.
Etiopathology. This clinical picture is only the introductory stage to a progressive degenerative process: the same clinical diag nosis applies to the more advanced stage of patella cartilage damage. For grading of the cartilage damage, the Outerbridge classifica tion is the most popular. In stage I, the cartilage softens, but its surface remains smooth; its color may differ from that of the adjacent. In stage 11, the surface becomes "hairy", i.e. covered with small villi, and the involved area loses its shine. Small superficial fis sures may be present. In stage 111 the degeneration progresses, and the fissures become numerous and spread through the entire thickness to the bone. Stage IV is characterized by large defects ex tending deep to the bone, resulting from the action of shearing forces on the dam aged, cracked cartilage surface.
The latter two stages are regarded as the initial stages of osteoarthritis (see Chapter 1 9 . 8 .). Biomechanical factors often play a role in the development of patella chondromalacia. In a large proportion of the cases, formal de-
2 9 . D i s o rd e r s of t h e k n e e
Fig. 29.15. Patellofemoral arthrosis with lateral hyperpression syndrome.
fects are present: incongruence of the patellotrochleal junction, anomalies of the traction route of the quadriceps (increased Q angle), and a high or low position of the knee cap (patella alta or patella baj a), with repeated luxation or subluxation as a consequence of the former factors, or patellofemoral hyper pressure (Fig. 29.1 5.). Exposure of the knee cap to a direct or indirect trauma leads to hyaline contusion, contusion and hematoma of the subchondral bone or an occasional pa tella fracture. In a large number of cases, none of these factors can be identified; these cases are classified as idiopathic. This is often the situation with young schoolgirls who do not play any sports, when severe hypotrophy of the vastus medialis muscle is observed. Clinical symptoms. Walking up or down stairs, or standing up from a squatposition may produce severe retropatellar pain, and later prolonged flexion without loading may also cause complaints. A long period of sitting with the knees flexed can produce complaints that are known as ' drive-in movie sign ' . Fluid accumulation is not common. Direct pressure on the kneecap, tapping or attempted sub luxation, either laterally or medially, pro vokes pain (the Frond and Zohlen signs). It is important to assess the angle between the di rection of traction of the quadriceps apparatus and the axis of the patellar ligament (Q-angle). The mobility and possible ten dency of the kneecap to undergo dislocation must also be assessed. X-ray signs. X-ray tests play a decisive role in the identification of etiology. In the lat-
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era I view, the high o r low position of the knee cap, or the relative length of the j oint surface (the Blackburne or Insall-Salvati indices) may preconditioning factors. Patella lateralization too may be visualized in accurate antero posterior X-ray images, and the patello femoral views taken at 3 0-60 and 90° of flexion may demonstrate the pathologic match in the different phases of motion. The congruency disturbances relating to the shape anomalies of the patella or the trochlea are of great importance. Treatment. Apart from those cases where there is a severe biomechanical background, the therapeutic approach is basically conser vative. Rest, nonsteroidal anti-inflammatory drugs, various physiotherapeutic processes (iontophoresis, ultrasonographic treatment, selective current stimulation of the vastus medialis, etc.) and well-selected exercises play a deciding role. The most important fea tures are the strengthening of the vastus medialis and of the entire quadriceps muscle, and proprioceptive exercises. Underwater ex ercises and bicycling are useful supplements. If the conservative attempts fail, or if a well-defined biomechanical cause is proven, operative treatment may be considered. The most common procedure is minimally inva sive arthroscopy, when the surface changes exceeding stage 1 1 are smoothed with a shaver, and the patellofemoral pressure condi tions in cases of hyperpression are advanta geously affected by incision of the lateral retinaculum. In stubborn or severe cases, the tibial tubercle may be ventralized to correct the direction of patellofemoral traction.
29. 14. Chondropathies, osteoarthritis (arthrosis) of the knee A lengthy series (many yea rs) of pathologic events usua l ly occur from the mild cartilage damage (chondro pathy) to the end-stage (osteoarthritis o r a rthrosis of the knee).
394
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I n itially, macroscopic changes take place i n t h e carti lage, followed b y deformation a n d destruction o f t h e joint, involving t h e majority of the adjacent structu res, resulting in a re stricted range of movement and pain.
Etiopathology. Primary osteoarthritis of the knee is the most common process destroy ing the large j oints, followed by hip and shoulder degeneration. In the age group over 65 years, the radiological prevalence is 20-34%, but only 7- 1 1 % require treatment of their complaints . The diagnosis is secondary osteoarthritis of the knee, when the destruction is due to a previous illness, trauma or biomechanical anomalies . The most common factors are: - instability (rupture of the collateral or cruciate ligaments, meniscectomy, capsule tear, or a change in muscular balance in neuromuscular disorders) - overweight - axial anomalies - meniscal injuries, or loss - rheumatologic diseases - inflammatory or metabolic diseases - overloading - a post-traumatic state
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- osteochondritis dissecans, or loose joint bodies One of the most common causes is knee j oint instability, when abnormal, pathologic joint movements occur leading in a vicious circle to destruction of the cartilage (Fig. 29.1 6.). The destruction of the cartilage alters the biomechanical conditions and causes inflam matory and reactive changes; the hyaline sub stance may disappear, and subchondral scle rosis and cysts may develop accompanied by osteophyte formation in the non-loaded areas and contractures of the joint capsule. Clinical symptoms. When the changes in volve only the cartilage and a limited area, the symptoms are said to be moderate. The most characteristic symptoms are a decrease in load-bearing, intermittent fluid accumulation, pain, tenderness of the involved compart ment, click and crepitation. Later, the swell ing and fluid accumulation become more common, pain is present even in the resting period, the range of movement decreases and the joint gradually becomes deformed. In the maj ority of cases, the axial anomalies of the knee joint give rise to a severe varus defor mity with flexion contracture.
i n sta bi lity of the knee
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2 9 . D i s o r d e rs of t h e knee
X-ray signs. Anteroposterior X-ray films in the loaded position provide indirect data on the thickness of the cartilage of the weight-bearing surfaces in extension. The Rosenberg views at 30° of flexion furnish in formation on the quality of the posterior parts of the joint surface. As the condition pro gresses, the joint space diminishes or disap pears, the subchondral bone undergoes sclero sis and peripheral osteophytes are formed. Treatment. The process of primary osteoarthritis of the knee usually lasts for many years from the first complaints to the end-stage of the joint destruction. During this period, the symptoms are present with various intensity, the painful stages of inflammation alternating with long symptomless periods. Initially, conservative treatment is advised. The aim is to decrease the symptoms and signs and the contractures. Nonsteroidal anti-inflammatory drugs, muscle relaxants and painkillers are prescribed, together with oral chondroprotective medication (glucose amine sulfate and chondroitin sui fate ) and a
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Fig. 29.17. Closing-type valgus high tibial osteotomy. a: Preoperative loaded anteroposterior view: mild varus deformity. b: Six months following the closing-type valgus high tibial osteotomy (lateral based wedge removal and staple fixation).
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intra-articular hyaluronic acid. Adjuvant ther apy may include cooling, electrotherapy, vari ous iontophoreses, the use of ultrasound and medical spa treatment. The most significant therapy is movement of the j oint without a load, swimming and underwater exercises, the aim of which is to improve the knee func tion and resolve the contractures. The surgical treatment in prearthrotic chondropathies can be divided into two cate gories. One category involves treatment of the biomechanical factors presumed as prea rthrotic factors, while the other deals with the existing cartilage damage. In the former group, the correction of mainly varus and rarely valgus axial deformities is of great im portance. The varus deformity occurs chiefly on the tibia. The correction is most often a clos ing-type high valgus tibia osteotomy with re moval of the laterally based wedge proximal to the tibial tubercle (Fig. 29.17. a, b); more rarely, a varus osteotomy is carried out in the same area with the opening of a medial wedge (Fig. 29. 1 8. a, b). Apart from these techa
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Fig. 29.19. "Microfracture" technique: arthroscopic picture.
niques, dome-shaped or sphere osteotomies are also performed, allowing minimal rota tional correction. For the treatment of carti lage damage, arthroscopic debridement and lavage may be of value, but only for short pe riods. The sliding surface reconstruction of limited, local cartilage defects (hyaline and osteochondral defects) is attempted by one of the surface reconstruction techniques, such as Pridie ' s procedure, abrasive arthroplasty or mosaic plasty. Via small holes made with a sharp surgical awl, radial fissures are created in the bone, forming an ideal attachment sur face for the regeneratum. The most important elements of the postsurgical aftercare are 4-8 weeks of movements without weight-bearing and a further 2-4 weeks of partial weight bearing. The popular procedure of ' micro fracture' is performed arthroscopically, even in cases of multiple degenerative cartilage de fects (Fig. 29.1 9.). In optimal cases, a satis factory gliding surface can be created, rich in collagen fibers of types I, IX and X. The other direction of cartilage surface creation is the treatment of the circumscribed small and medium-sized full-thickness hyaline defects. In these cases, the aim is to produce a hyaline-type and quality sliding surface instead of the above-mentioned fi-
Fig. 29.20. Open mosaic plasty on the lateral femoral condyle. 2 a: 4 cm bony defect caused by osteochondritis b: the defect is filled with grafts 4.5 mm in diameter.
Fig. 29.21. Arthroscopic view of mosaic plasty performed 5 years earlier on the medial femoral condyle.
2 9 . D i s o r d e r s of t h e k n e e
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hyaline cartilage and the intermediate fibrous cartilage takes place (Fig. 29.2 1 .) . This is clearly shown by MRI (Fig. 29.22. a, b). The most common indications of mosaic plasty are osteochondritis dissecans, and traumatic or degenerative defects. Because of the limits of transplantation (limited donor sites and technical difficulties), the future solution may be cartilage formation via cell proliferation (autologous chondrocyte transplantation). So far, however, the struc tural organization of the resulting surface is significantly different form that of the joint hyaline cartilage, and a number of technical and financial problems limit the application of this method. The trend of the present technical development is the integrated use of holding matrices (scaffolds) and biologically active hyaline-inducing materials. The best outcome is expected at present from the transplantation of fresh osteochondral grafts. �
Fig. 29.22. MR view of the mosaic plasty. (29-27 a b) a: Preoperative sagittal MR view of osteochondritis dissecans on the lateral femoral condyle. b: 20 weeks following mosaic plasty.
brous cartilage surface. The ' mosaic plasty' procedure (introduced by L. Hangody) has been performed in a large number of cases since 1 992. Small cylindrical osteochondral grafts ( 1 -4 cm2 ) replace the damaged surface of the loading area (usually arthroscopically) to ensure congruent surface reconstruction and to decrease the damage to the donor site (Fig. 29.20. a, b). During the postoperative period, fibrous cartilage produced between the transplanted cylinders in the transplanted area, generating a composite gliding surface, where the integration of the transplanted
Surgical solutions in osteoarthritis If the degenerative processes are severe and extensive, it is too late for the procedures listed above and a good result cannot be ex pected. Arthrodesis, the stiffening of the de stroyed j oint, is not well tolerated nowadays, even if this procedure eliminates the pain. Re moval of the cartilage and lasting fixation of the joint can fix the bony elements and stop the pain, but the joint function is lost. The stiff
Fig. 29.23. I ntraoperative picture of a total bicondylaer knee endoprosthesis.
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29. D i s o r d e rs of t h e k n e e
Fig. 29.24. Secondary arthrosis due to rheumatoid arthritis. Pre- and postoperative x-ray pictures of total knee prosthesis.
j oint changes the basic dynamics of the gait and exerts an extreme overload on the neigh boring j oints, the contralateral hip and knee j oints, and the lumbar spine, which may lead to early secondary osteoarthritis. Implantation of an artificial joint (endoprosthesis) can lead to a much better quality of life. Depending on the extent of the damage, a part or the entire j oint is replaced. When only the surface of one of the femorotibial compartments is damaged (usually the medial one), a unicondylar (sledge) prosthesis may be indicated (Fig. 29.7.). A precondition of this procedure is the intact state of the ligaments and the other compartments and normal axial circum stances . In cases of severe cartilage damage in volving a number of compartments, the proper solution is implantation of a total j oint replacement (Figs. 29.23. and 29.24.). An im portant precondition of this procedure is the intact function of both collateral ligaments. During surgery, the anterior cruciate ligament
is sacrificed and stability is ensured by the changed geometry of the replaced surfaces. The above-mentioned severe destruction is treated by means of constrained (hinged) prostheses, which permit flexion and limited rotation. These can give rise to complications, as they loosen more often than ordinary pros theses. In a consideration of the indication of an endoprosthesis, it must be borne in mind that the implantation will presumably not be the fi nal solution, and there may well be complica tions. The forces acting at the prosthesis bone junction may eventually loosen the im plant long run (according to present statistics, in 1 0- 1 6 years). This will require revision sur gery, with even higher risks. The chances of sterile loosening are higher for younger pa tients, who generally demand increased phys ical activity. The prostheses mentioned above are designed both with and without bone ce ment fixation. When all the factors are taken into account, prostheses are implanted as late as possible, usually after the age of 60 years.
30.
J a n o s K r a n i cz, Ka l m a n T6t h
Disorders of the foot
30. 1. The functional anatomy and biomechanics of the foot The function of the foot is to transfer body . weIght onto the ground, and at the same time to ensure the mobility necessary for walking : In orde� to meet this twofold requirement, a system IS needed that can withstand large im pacts of pressure, and guarantee good mobil ity and elasticity in response to different dy namic effects . Regarding its anatomic construction ' the foot resembles an arrangement of pillars con nected by a system of arches (Fig. 30. 1 .) . Five longitudinal arches are distinguished from the calcaneus to the metatarsus . The transverse arch is positioned in a medial lateral direction. The arches are maintained by two stabilizing systems : the passive stabiliz ing system, comprising the following ele-
Fig. 30.1. The bony structure of the foot is constructed of arches.
� ents : the joint capsules to connect the joints m the foot, and the collection of minuscule liga�ents to s �pport these j oints; and the dy . . namic stablhzmg system, which is composed of the short muscles in the foot and the set of long muscles originating on the tibia and at tached to the foot. By themselves, neither the passive nor the active stabilizers are sufficient to support the normal arch system, they operate flawlessly only when complementing each other. As mo tion becomes more and more dynamic, the arch system gains increasing significance in each phase of walking. In the heel-strike phase, for example, the arch system acts as a sho � k-absorbe� in order to neutralize a pro portIOn of the mertial forces originating from the contact with the ground. In the push-off phase, the elasticity of the arch increases the strength of the push-off. Computerized pres sure-distribution analyses have unequivocally proven that higher levels of pressure are ex erted under the second, third and fourth metatarsus heads than under the first and fifth metatarsals (Fig. 30.2a), even in the standing phase. The explanation for this is that the t� ick adipose tissue under the sole plays a sig . pressure distribution. This is lllficant role m also indicated by the clinical experience that the atrophy of the adipose tissue of the foot in rheumatoid arthritis patients leads to severe metatarsalgia. During the gait cycle, the pressure factors b�tween �he g�ound and the foot undergo cy clIc modificatIOn (Fig. 30.2b). The force in creases on every area of the sole, but the load inc �eases fr0 t? the rear to the front. The expla . that the middle metatarsals natIOn for thIS IS are longer than the first and fifth. With the lift ing of the heel, the short metatarsals lift off the
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30.2. Foot deformities in childhood
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Identified congenital foot deformities demonstrate an extremely wide range of pre sentations. Depending on the direction of the defor mity, joint contractures occurring in the fron tal or sagittal plane are identified. These may occur in an isolated manner, limited to one j oint, but combined contracture groups are more common. These are well known as uni form diagnoses (e.g. clubfoot or navicular foot).
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Pes equinus (talipes eq u i nus): the foot is in a plantar flexion position at the a nkle joint. Pes calcaneus: the foot is constantly i n an u pwa rd, dorsal flexed position. Pes cavus (excavated foot): the anterior part of the foot is in a cu rved position compared to the tarsus and the longitudinal a rch is higher than average.
Deformities of the foot occurring in the fron tal plane
ground earlier, and thus the transfer of energy takes place via the metatarsals maintaining contact. The more dynamic the movement, the higher the load on the anterior part of the foot. Walking is a harmoniously structured string of movements resulting from actively coordinated operations of both the upper and lower extremities and the axial muscles. An understanding of each phase of walking is necessary in order for the physician to recog nize deviations, detailed descriptions of which are to be found in the chapter on patient examination (Chapter 4).
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Pes varus: the heels i nvert i nward from the median at the subtalar joint. Pes valgus: the heels turn o utward from the median at the subtalar joint. Pes adductus, metatarsus varus (intoe): the forepart of the foot is bent toward the medial at Chopart's joint. Pes supinatus (inverted foot): the forepart of the foot supinates at Chopart's joint. Pes abductus: the forepart of the foot is bent latera l ly at Chopart's joint.
Congenital foot deformities that occur less frequently: - Cleft foot (the transverse arch is missing). - Developmental abnormalities of the toes : polydactyly, oligodactyly, syndac-
30. D i s o r d e r s o f the f o o t
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tyly, digitus varus, valgus, subductus and superductus. - Different amnion stricture disorders.
30.2.2. Pes equinovarus congenitus (congenital clubfoot) Terminology. This is a case of congenital contractu res affecting all joints of the foot, which can best be described by the Lati n term: p e s equ inovarus add uctus et plantiflexus congenitus (Fig. 30.3). �
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Equinus: A flexion contracture is present in the ankle, the talus in the mal leolar fork is set in a flexed position, and the m. triceps surae has shrunk. Varus: The calcaneus in the subtalar joint is tilted toward the medial and the forepart of the foot, and it is a lso twisted, supinating toward the medial. Supination of the forefoot: As regards the d irection of deform ity, this corresponds to the varus position of the heel, but due to the different position, heel varus and supination of the forepart of the foot a re d isti nguished in clinica l practice. The forefoot may take up a pronated position relative to the heel. Adductus: The forepa rt of the foot bends toward the medial at the Chopart's and Lisfranc joints. Plantiflexus: The a nterior part of the foot is in a further cu rved position as compared to the tarsus at Chopart's joint and the sole is excavated.
Occurrence. Congenital club foot, which is present at birth, is more frequent among males population, the male-female ratio being approximately 2: 1 . Most cases are bilateral, but it occurs with Similar frequency on the left or right side. The incidence in Europe is between 0, 1 -0,2 %, in Hungary, it is 0, 1 25 %. Its incidence demonstrates racial charac teristics. Occurrence among the Polynesian Maoris is 0,6- 0,7 %.
Fig. 30.3. Congenital, severe bilateral clu bfoot, the a nterior part of the foot displays 90° adduction and supination.
Etiology. There are only hypothetical the ories relating to the etiology of congenital structural club foot: �
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mecha nical reasons, endogenous reasons, mu ltifactorial reasons (genetic + environmental effects)
The multifactorial origin is most widely ac cepted, i.e. congenital clubfoot tends to de velop as a result of the joint presence of a ge netic predisposition and environmental effects.
Familial aggregation. Congenital club foot demonstrates a familial aggregation, the risk of multiple occurrence depending on the familial incidence. The higher the number of cases of congenital clubfoot in the index fam ily, the higher the familial incidence, and con sequently, the higher the expectancy ofthe re peated occurrence of congenital clubfoot. Thus, if two siblings are affected, the ex pected incidence among subsequent siblings is 3-5%. If one parent has congenital club foot, the risk is 2%, and if one parent and one sibling
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3 0 . D i s o rd e r s of t h e f o o t
are both affected, the risk of further occur rence is approximately 25%. Pathology. Disease-induced lesions affect all structures of the foot. Diagnosed alter ations of the bones are mostly secondary and are consequences of adaptation to a chronic malfunction. - Changes in shape such as a shortened calcaneus, an elongated talus neck or a wedge-shaped deformity of the navicular bone are most typical. - Regarding the altered articulation of the bones, the twisting of the calcaneus under the talus is significant, it is situated high above the horizontal plane, the talus is in a flexed position, or the navicular bone and the os cuboideum have slipped towards the medial at Chopart ' s joint. - The malleolar fork is rotated toward the medial. - The soft tissue shrinks on the posterior, medial surface and the sole. - The attachment of the tendons widens, and is often atypical. - The tibial muscle mass decreases and becomes atrophic.
Fig. 30.4. Untreated severe bilateral clubfoot. The bursa de veloping on the instep impairs walking.
ward. A toddler, who is able to stand or walk, will position the weight on the outer soles, or even stand on the dorsum of the foot (Fig. 30.4.). In the course of a joint mobility examina tion, the degree of contracture and the passive correction are determined. Feet that are short, with an elevated heel position and a transverse fold of skin behind the heel are indicative of a poor prognosis. The contractures are rigid, the first toe is shorter, and there is pronounced muscle atro phy along the tibia.
Clinical symptoms. Congenital club foot is easily diagnosable at birth due to character istic contractures, it can be said that the foot is "bean-shaped" and turned entirely toward the medial, while the inner sole is concave, the outer sole is convex, the heel is upright, and high located, and often only fat tissue is palpa ble behind the ankle. In severe cases, the sole faces backward, with the instep facing for-
Radiological symptoms. Taking an X-ray picture in order to determine the diagnosis is not necessary, but during treatment proce dures, an antero-posterior (a-p) and a lateral X -ray image should be taken in order to evalu ate the results of conservative treatment and establish a surgical plan.
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Fig. 30.5. The ang les of the tarsal bones diverge char acteristically in case of foot deformities. A de creased angle causes clubfoot and an in creased angle resu lts in navicular feet. (30-9) a: Clubfoot, angle: 0° . b: Excavation, angle: 20°. e: Normal, angle: 35°. d: Flat feet, angle: 50°. 80°. e: Navicu lar foot, angle:
3 0 . D i s o r d e r s of t h e f o o t
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The X-ray image depicts the position of the tarsal bones in relation to each other and also the shapes and structure of the bones. In order to describe the position of the tar sal bones, the anteroposterior and the lateral talocalcaneal angles are used, which are smaller than normal in patients with clubfoot (Fig. 30.5.). The relation of the posterior and the ante rior parts of the foot can best be described through the talometatarsal and the naviculo metatarsal angles (Fig. 30.6.). Treatment principles �
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Conservative treatment Treatment is primarily conservative: the methods applied a re early passive and active p hysica l exercise, g rad ual correction with plaster casts, o rthoses and specia l footwear. Surgical treatment Ea rly soft-tissue surgery, which is nowadays usually a conseq uence of unsuccessful conservative treatment. Reoperation i n recu rrent cases. Broader surgical i ntervention on soft tissue and bones at a later age. Nursing Organized care should be offered at specialized treatment centers. Regular physiotherapy is carried out until the p hase of foot g rowth ends, after which orthopedic technical support and continuous o rthopedic specia list fol low- u p is needed.
Detailed description of conservative treatment implementation. Passive physical exercises of the foot (unless contraindicated) and correction of the j oints in the direction op posite to the contraction should be com menced immediately after birth. This may be performed by infant ward nurses, physiother apists and parents alike. Stimulation of the skin of the sole (e.g. with a soft toothbrush) will induce active foot movements. The opti mal instance for commencing gradual correc tion with plaster casts is on day 5-7.
Fig. 30.6. I ncreased talometatarsal angle in clubfoot. a: Normal. b: C1u bfoot.
Gradual correction with plaster casts may be implemented according to the Lorenz prin ciples in the following order: inflexion, adduction, supination of the forefoot, varus of the heel and finally correction ofthe equinus. Correction methods aimed at reducing or eliminating all possible contractures (as long as the foot can endure them) are also acknowl edged. In both cases, the achieved result is to be stabilized in a circular plaster cast with the knee in a 90° position and the cast running from mid- or upper thigh to the end of the toes, with the top of the toes left uncovered. Felt sheets should be attached corresponding to the bones running directly under the skin, and the skin itself should be protected with a thin layer of cotton wool. It is advisable to inspect the plastered foot after 20-30 minutes in order to ensure that no blood circulation disorders have arisen. If any such problems are perceived, loosening or re placement of the cast is necessary. The plaster should initially be changed weekly and, after manual exercising, a new circular plaster cast should be applied.
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3 0 . D i s o r d e r s of the foot
A t later ages, alternating plaster correction should be applied every 2-3 weeks. Special care should be paid not to place the foot in a "rocking chair" position, which would mean over-correction of Chopart 's j oint and initiat ing subluxation in the dorsal direction. It is advisable to verify the situation by means of an antero-posterior and a lateral X-ray picture. When untreated clubfoot is encountered in infants or early childhood, the conservative treatment applied is identical to that for the newborn. In these cases, gradual correction with plaster casts is merely capable of reduc ing the contractures and the establishment of better conditions for surgical treatment. In recurrent cases of clubfoot or after re peated surgery, the application of a long-term plaster cast may be necessary, sometimes with use of the Kite method (following surgical correction and stabilization of the foot in plas ter, a wedge is removed from the cast after which the cast can be further corrected). A plastic orthosis may be applied at any point after removal of the plaster cast. Following the conclusion of conservative or surgical treatment, the foot and the muscles should be exercised and strengthened, primar ily by means of physiotherapy. Standard shoes are generally adequate for walking, but it may be necessary to acquire antivarus shoes with a straight sole-axis and an elongated inner counter reaching to the front of the shoe. In recurrent cases, an orthopedic shoe spe cially constructed for the patient' s foot may be necessary, with a straight sole-axis, a fron tally extended pronated heel, and a stronger inner counter. �
Surgical treatment Surgical indication . It is a general princi ple, that all structures sustaining contractures should, as far as possible, be eliminated in the course of surgery. Surgery is advised in the fol lowi ng cases: �
After 6 months of age, fol lowing conservative therapy, i n o rder to correct
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residual disorders, dorsal, or dorsa l-inner soft-tissue release. In u ntreated, late cases in older children, or as repeated su rgery for recu rrence between the ages of 1 and 3, extensive soft-tissue surgery or peritalar a rthrolysis may be indicated. Between the ages of 3 and 12, the treatment may be as mentioned above, access being ensured laterally for repositioning of the os navicu lare: calca neocuboidal wedge resection according to Evans, resection of the cuboidal su rface of the ca lcaneal joint according to Lichtblau or calcaneus osteotomy. Above the age of 3 years, muscle transplantation is possible in order to ensure recovery of the muscle ba lance. As soon as the g rowth of the foot has ended after pu berty, wedge resection, subtalar or Chopart's joint a rthrodesis may be carried out. Supramal leolar rotational tibial osteo tomy of the i nverted foot has the aim of palliative correction.
Surgical methods. Dorsal release. This involves "Z" shaped Achilles tendon length ening, dorsal capsulotomy of the ankle and hindfoot joints, and posterior incision of the talofibular ligament. Dorsal and medial release. Posterior tenotomy of the m. tibialis is performed, to gether with the previous interventions, medial and plantar capsulotomy of the talonavicular joint, ad capsulotomy of the frontal and dor sal-medial segments of the subtalar joint, capsulotomy of the naviculocuneiform and the metatarsocuneiform joints. Aspects of physical exercise. These in clude alleviation of contractures, passive cor rective exercise, active exercise, and strength ening of the femur and the foot muscles. Electric stimulation of weakened muscle groups may be performed and the patient may be provided with walking instruction, walking
3 0 . D i s o r d e r s of t h e f o o t
correction, implementation of passive sup porting and stabilizing orthoses, and lifestyle and vocational guidance. Orthopedic shoes should be worn. Suggested sports that ensure regular mus cle strengthening for the feet are short- and long-distance running, cycling and ball games.
30.2.3. Pes adductus The forefoot bends medially at Chopart's joint. The situation is similar to clubfoot, a l though it is well distinguishable, since the heel is not in varus or equ i n us. Although to various extents, the heel is i n a valgus position, and it is therefore often referred to as pes adductus planovalgus.
Occurrence. It is easily diagnosed imme diately after birth. Some consider it to be a genuine developmental abnormality, while others assume it to be an abnormality due to the constraint of maintaining the foot in a varus position while in the womb . The condition is in most cases bilateral; it occurs more frequently in the male popula tion, the male-female proportion being 3 : 1 .
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position of the heel, the diagnosis may b e es tablished on the basis of the medial (metatarsus varus) inflexion of the first toe (Fig. 30.7. and 30.8.). The increase of the dis tance between the first and the second toes is clinically also clearly visible. Radiological symptoms. The angles of the talus and the calcaneus are larger than nor mal, the navicular bone at the head of the talus is displaced laterally, directed in a subluxed position, and the metatarsals deviate toward the medial side. Treatment. In mild cases, there is a ten dency to spontaneous correction. Release of the adduction contracture of Chopart ' s joint is necessary, by conservative methods if possible. Manual correction. Axial traction of the forefoot by fixed calcaneus, and exercising the forefoot in abduction and pronation is ad vised with the application ofBebax redressing sandals.
Diagnosis. Besides the adduction contracture of Chopart ' s j oint and the valgus
a
Fig. 30.7. Pes adductus, the forefoot curves medially at Chopart's joint.
b
Fig. 30.S. Graphic illustration of a normal foot (a) and a pes adductus (b).
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Active physical exercise is also recom mended with the concurrent application of skin stimulation. Surgical treatment. Incision of the m. ab ductor hallucis and the medial joint capsules may be carried out after 2 years of age. Metatarsus base osteotomy in order to correct the deformity is possible at a later age.
30.2.4. Pes excavatus The term hol low foot (pes varus o r pes cavovarus) describes such foot defo rmities when t he longitudinal arch is higher than av erage, rega rd less of the cause i n itiating the condition.
Clinical symptoms. This unmistakable foot deformity takes shape by about the age of 1 0- 1 2 ; the foot is relatively short, the longitu dinal arch is higher, the anterior part of the foot is in adduction, the heel in varus position, and the 1 st metatarsus head is situated low down, which appears more explicitly due to the strongly constrained dorsal-flexed posi tion of the big toe and the other toes in the condition known as hammertoe (Fig. 30.9. a, b). The aponeurosis plantaris is stretched like a taut string, which intensifies during the dorsiflexion of the forefoot and raises the skin of the sole. Soreness and callosities may emerge on the plantar side of the forefoot over the meta tarsal heads.
a: Excavated foot, the longitudinal arch is elevated (X-ray). b: In cases of excavated foot, the patient is unable to rest the heel on the ground because of the exten sive inflexus.
Initiating causes. This deformity gener ally develops as a consequence of a mus cle-balance disorder. The lumbrical and interosseus muscles weaken, and therefore the effects of the long flexors and extensors take over. The reason for the muscle-balance disor der is generally myelodysplasia or spina bifida occulta, though other neuromuscular disorders may also occasionally be diag nosed.
Treatment Conservative treatment. The treatment of mild cases of pes excavatus is in conservative. Physical exercise is advised to stretch the shortened muscles, and the wearing of ortho pedic shoes if necessary. Surgical treatment. In childhood, Steindler 's operation is carried out: the aponeurosis plantaris and the short plantar muscles are detached from the heel . This may
Fig. 30.9.
3 0 . D i s o r d e r s of t h e f o o t
be supplemented with incision of the capsules on the plantar surface of Chopart 's joint. Tendon and muscle transplantation may be needed to correct the arched position of the toes, especially the big toe. In the event of a pronounced deformity af ter 1 0- 1 2 years of age, osteotomy of the cunei form bones with removal of a dorsal-based wedge will ensure an improvement in appear ance, due to the shortening of the longitudinal arch. After the foot has finished growing, arthrodesis of Chopart 's joint and the tarsal joint may come into consideration.
30.2.5. Pes calcaneovalgus congenitus This is a congenital developmental va lgus de formity of the foot, with varying extents of severity.
In line with the appearance of the defor mity, the condition is often referred to as rock ing chair foot, navicular foot or congenital flatfoot. With regard to the main pathogenetic fac tor behind these deformities, the disease is of ten referred to as vertical talus . Clinical symptoms. In all cases, the sole is convex, the instep is slightly concave, the forefoot is abducted, pronated and extended at
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Chopart 's joint, furthermore, the tarsus is in equinus and the heel is in a distinct valgus (Fig. 30. 1 0.). Etiology. Foot deformities described as vertical talus do not form a closed etiological entity; similarly to clubfoot, several varieties are defined. - Structural navicular foot: This is a distinct developmental deformity of the foot; its isolated occurrence is very rare. - Symptomatic navicular foot: This deformity is present already at birth, as one symptom of many relating to a complex syndrome (e.g. Freeman Scheldon 's syndrome) . It is often regarded as a result of arthrogryposis multiplex congenita. It is generally bilateral, the contractures are rigid, and respond very poorly to corrective treatment. - Myelodysplasia-related navicular foot. This too is often already present at birth. A muscle-balance disorder plays the main role in its development. It has been reported by Sharrard, that if the lesion is present in or above the 3 rd lumbar vertebra, the contractures are extremely rigid. In case of lesion between L5 and S I , looser contractures are perceived. - Spastic navicular foot. This develops in most cases after the patient has learned
Fig. 30.11. Fig. 30.10. Oinical depiction of bilateral congenital navicular foot.
Talus positioned vertically, demonstrating the pathological relation of the talocalcaneal joint and Chopart's joint.
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to walk and is a consequence of a muscle-balance disorder, with the heel in an equinus position. Radiological symptoms. The steep posi tion of the talus, the pathological correlation of the tibiotalaris and the talocalcaneal is, and the dislocated or dorsolaterally subluxed posi tion of the os naviculare on the talus-neck (Fig. 30. 1 1 .) are characteristic of all above listed foot deformities . Treatment. The obj ective is repositioning of the talus and the os naviculare, normaliza tion of the position of the tarsus and the ante rior part of the foot and preservation of the corrected position. Conservative treatment. If any type of navicular foot is noticed at birth, passive physical exercising should be commenced immediately, followed by gradual correction with plaster casts. In case of navicular foot developing as a result of spastic or flaccid paralysis, gradual correction with plaster casts is rarely imple mented, treatment is primarily surgical. Surgical treatment. From the age of 6 months to 3 years, posterolateral or, if neces sary, medial release is performed.
30.2.6. Accessory ossicles on the foot Generally, no complaints are caused by this condition, although its occurrence is fre quent, most often involving the os tibiale externum ( 1 0%), the os trigonum (8%), and the os peroneum (5%). �
Os tibiale externum This is found on the inner side of the foot, beside the os naviculare. It is associated via either fibrous tissue or cartilage with the navicular bone, together forming a horn shape, which is why it is also referred to as os naviculare cornutum. Its clinical significance is that it protrudes in the medial part of the foot, on the inner side, and consequently, the wearing of shoes will
Fig. 30.12. Accessory bones on the medial side of both navicular bones: os tibiale externum.
eventually cause painful inflammation and hyperkeratosis. On an X-ray, it is easily detectable, espe cially in an antero-posterior position beside the os naviculare as a separate bone center (Fig. 30.1 2.). Treatment. The use of insoles may reduce discomfort, but only surgical removal will re sult in complete elimination of the irritation. Special attention should be paid to maintain ing the posterior attachment of the m. tibialis.
30.2.7. Tarsal coalition This is a pathologic connection(s) between different tarsal bones.
When stiff flat feet are detected in adoles cence, possible pathologic bindings between different tarsal bones should be taken into consideration. Depending on which bones are connected, the following forms are identified: calcaneo navicular coalition, talonavicular coalition and calcaneocuboid coalition. In most cases, calcaneonavicular coalition is diagnosed. The binding tissue may be fi brous, cartilaginous or bony. The possibility of this disorder is often suspected on a lateral
3 0 . D i s o r d e r s of t h e foot
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X-ray picture, but a cartilaginous or bony co alition is weII perceivable on an oblique im age (Fig. 30.13.). Treatment. The disorder is not curable by conservative treatment. In childhood, resection of the bony bar, and in adulthood, subtalar and Chopart ' s j oint arthrodesis is performed in order to terminate the complaints. Fig. 30.13.
30.2.8. Osteochondrosis calcanei (apophysitis calcanei)
The bony fusion between the os naviculare and the calca neus can be observed in an oblique image.
This mostly develops between the ages of 7 and 13 in male patients, often bilatera l ly. It is believed that besides ossification d isorders, an i ncreased functional load and d isorders of the blood supply of the apophysis a lso p lay a part in its development.
Clinical symptoms. Children complain of rapid exhaustion, and of tarsal pain and sensi tivity around the adherence of the Achilles tendon, which escalates when subjected to ex tensive strain. The pain is more intense in re sponse to the dorsiflexion of the foot, it de creases upon plantiflexion. Walking up and down stairs is especiaIIy difficult. The heel is slightly thickened. Radiological symptoms. The apophysis of the calcaneus is indistinct, with an unclear contour and an irregular shape. The ossifying center is fragmented (Fig. 30.1 4.). Treatment. Treatment of the disorder is conservative. Complaints should cease upon relaxation and lifting of shoe-heels.
30.2.9. Osteochondrosis ossis navicu laris pedis (Kohler's I disease) The essence of this d isorder is aseptic necrosis occu rring in the os navicu lare pedis. The d iag nosis was described by Kohler i n 1908. It ge-
Fig. 30.14. The apophysis of the calcaneus is shattered and sclerotic.
nera l ly occurs between the ages of 3 and 10. The d isorder is more freq uent in males and approximately 30% of the cases a re bilateral.
Etiology. The etiology has not yet been clarified; the most probable cause is an ossifi cation disorder. Some associate the iIIness with trauma. Clinical symptoms. The condition often develops without causing any particular com plaints and only an X-ray image taken for some other reason will shed light on its exis tence. The complaints are manifested in rapid exhaustion, limping, and local pain and swell ing in the area of the navicular bone in the in step. The limping intensifies after extensive walking. Passive movement of Chopart ' s j oint is limited, and may even be painful.
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Radiological symptoms. The structure of the navicular bone is irregular, it is porotic. During the progression of the process, the bone decreases in size, gradually forms a disk shape, becomes sclerotic and diminishes. At rophy may be observed on other bones of the foot. In the process of healing, the os naviculare regains its original form and shape (Fig. 30. 1 5). Treatment. Shoe inserts are recom mended, through which the navicular bone can be partially relieved of body weight. There is no need for surgical treatment.
Fig. 30.15. Kohler's I disease: the ossifying center of the os
naviculare is cracked and the bone is flattened.
30.2. 10. Ostechondrosis capitis metatarsi 11. (Kohler's 11 disease) This disease denotes aseptic necrosis occur ring in the 2nd, (rarely the 3rd or 4th) meta tarsa l head. It genera l ly develops between the age of 10 and 18 and is three times as fre q uent i n female popu lation as in males, rarely, it is bi latera l.
Etiology. It generally occurs in cases of pes transversoplanus. Its development is ex plained by the fact that since the arch of the foot is descended, the 2nd metatarsal head is set deeper and is exposed to increased weight pressure. An inadequate blood supply of the epiphysis may also play a role in its develop ment. Clinical symptoms. The symptoms vary in accordance with the 3 stages of the disor der.
a) In the early stage, the dorsal surface of the metatarsal head is sensitive to pressure. Rapid exhaustion occurs, which intensi fies upon exertion, and the patient there fore tends to walk on the edge ofthe sole. b) In the middle or compression stage, swell ing and painful disability arise in the fore foot. The patient tries to evade the regular rolling phase of the feet, since the pain in-
Fig. 30.16.
Kohler's 11 disease: deformation of the metatarsal
head.
tensifies during the roll and when standing on tiptoe (plantarflexion of the foot). c) In the final stage, arthrotic symptoms pre dominate. Exostosis is palpable on the metatarsal head, and the toes sub luxate. Callosity develops in the corresponding spot on the sole. Radiological symptoms. No changes are perceivable in the initial period. In the com pression stage, the first sign is the sub-
3 0 . D i s o r d e r s of t h e f o o t
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chondral, transverse radiolucency in the meta tarsal head; later, the joint surface becomes flattened and deformed, but it remains well defined. Arthrosis and deformed joint sur faces are characteristic in the final stage; the metatarsal head is cylindrically deformed (Fig. 30.1 6.). Treatment. Treatment of this deformity, if possible, is conservative, the chief objective being to relieve the load on the forefoot and the metatarsal head. Suitable insoles and spe cially designed rocking bottom shoes will eliminate the discomfort. During the develop ment of normal bone structure, the deformity will heal in 1 -2 years . Resection of the head will result in total re cuperation. Excochleation of the necrotic seg ment and spongiosa plasty may also be at tempted.
a
30.3. Foot deformities in adu lthood 30.3. 1. Pes planus (pes planovalgus, flat foot, fallen arch) This is the most frequent foot d isorder of static orig in. The background of the condition is the collapse of the a rched system of the foot, since the active and passive stabilizing systems supporting the arch a re insufficient. The d isorder is cal led pes pla novalgus if the predomi nant factor is collapse of the longitu dinal arch together with the va lgus heel. If the transverse arch has fa llen with the calcaneus i n the normal position, the condition is ca l led pes transversoplanus.
It is worthwhile to reflect on the different types concurrently, since they are often asso ciated and the triggering causes are mostly the same. Etiopathology. When the active and pas sive stabilizing systems of the foot are not ca pable of equalizing the pressure of body weight placed on the feet, the valgus position of the calcaneus will develop and the longitu-
c d
Fig. 30.17. Normal longitudinal arch (al and pes planus b: The descent of the lon.gitudinal arch. c: This image of the patient clearly reveals how the medial side of the foot almost touches the g round; calluses are also well observable. d: X-ray picture of a flattened longitudinal arch in a standing patient.
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dinal arch will descend (Fig. 30. 1 7. a-c). This is generally followed by the collapse of the transverse arch as well. The reasons for the disruption of the bal ance: - overloading of the foot (body weight or pregnancy) - the decreased weight-carrying capacity of the foot - insufficient functioning of the passive stabilizers (congenital weakness of connective tissue, Ehlers-Dahnlos syndrome, hormonal effects or preg nancy) - insufficient functioning of the active muscles (paralysis, stabilizers Heine-Medin disease or myelodys plasia) - improper loading of the foot: com pensatory (infantile cerebral paresis) - following trauma or inflammation, or in case a tumor can damage the bony structure, tendons or ligaments of the foot arch
more, medially from the tip of the medial an kle, distally and slightly anterioriy, the con tour of the talus head protrudes. Under normal conditions, body weight is transferred onto the trochlea of the talus, and from here is dis tributed toward the calcaneus and the metatarsus. With weakening of the m. tibialis posterior, the m. extensor hallucis longus, the m. extensor digitorum longus and the pero neus muscle group, the talus head will tilt
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Clinical symptoms. The height of the nor mal longitudinal arch decreases, or in extreme cases it may disappear totally. Posterior ob servation of the patient reveals the position of the calcaneus valgus (Fig. 30.1 8.). Furthera b
Fig. 30. 19.
Fig. 30.18. Pes calcaneovalgus. The axes of the leg and the calca neus are at a n angle of approximately 20°.
a: Graphic image of a normal (1) and a collapsed transverse arch (2) and an expanded forefoot (3). b: Calluses developed on the sole below the 2nd-4th metatarsal heads (as a sign of increased pressure on the sole) in the case of a fallen trans verse arch.
3 0 . D i s o r d e r s of t h e foot
downward, toward the medial. The medial contour of the foot will therefore become con vex and the forefoot will assume a pronated position in relation to the heel. Collapse of the transverse arch is also a static deformity, its triggering causes corre spond to those of pes planovalgus, with which it is often associated, generally in the mid dle-aged. Because of the increased weight af fecting the metatarsal heads, the transverse arch drops (Fig. 30.19. a), and the foot spreads out. Calluses may develop under the 2nd, 3rd and 4th metatarsal heads on the sur face of the sole (Fig. 30.19. b). In cases of rheumatic diseases, the decay of the ligaments and the inflammated bursa on the sole of the foot intensify the deformity and the pain. In case of neonates, the arch system of the foot is not yet developed, and similarly, their physiological curves are not yet perceptible on the spine. These features develop later, in response to the appropriate stimuli . Parents often worry that the transverse and longitudi nal arches have not yet developed on the foot of their infant, who is about to start walking. The development of the arch system is time-consuming, and furthermore, the muscu lar system of the foot has to grow used to the muscle exertion necessary for walking. The final development of the arch system is con sidered normal up to the age of two years . Throughout growth (bearing in mind that growth is not a linear process) there are peri ods when the foot is exposed to increased ex ertion. One of these periods is the commenc ing of school, when the wearing of a backpack and walking to school may trigger an over loading of the feet. Adolescence is similarly a critical stage, owing to the significant increase in body weight and the changing circum stances (intensive sporting activity and per haps the commencing of employment) on the other. In cases of fixed, muscularly affixed flat foot, both the ligamental system and the dy namic stabilizing system are insufficient, but the disorder can still be corrected: in this con dition, the arch may be restored under anes thesia. When ligamental fixation has devel-
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oped, the arches can not be restored, not even under narcosis. This condition is referred to as bony rigidity. Naturally, there are certain circumstances or occupations that may bring about an insuf ficiency of the arch system by straining it ex cessively. Such occupations include those of waiters, hairdressers and surgeons. Physio logical changes too may cause the develop ment of flat feet, as in pregnancy, when the enhanced loosening of tissue results in a de crease in the passive stabilizing effect of the ligamental system. With a normal ligament and muscular system, obesity will lead to the collapse of the arches because of the excess weight load. Treatment. One of the most important ob j ectives is prevention. In childhood, the child should not be forced to walk and should be carried when tired. Strengthening of the foot muscles is also very important. One technique is to encourage walking on uneven surfaces. It is very impor tant, that walking barefoot on artificially flat surfaces such as wooden floors or boarding is explicitly harmful, since in these cases there is no need for the active operation of the foot muscles, and thus the muscles will wither. In contrast, on pebbly, bumpy, grassy or sandy surfaces, the foot is forced to adapt to the given surface owing to its uneven nature, which results in the strengthening of the dy namic stabilizing system. At the same time, it is possible to actively fortify the foot muscles by means of playful activities, e.g. picking up pens and pencils from the floor with the toes. It is possible to encourage the strengthening of the muscles of the sole through such exer clses. Insoles are not prescribed automatically for children aged 6-7 with flatfoot. In a pes calcaneovalgus condition, the valgus position of the heel is compensated by the lifting of the medial part of the shoe heels. A 5 -mm wedge is positioned medially under the heel, sloping toward the lateral. Thus, the elevation corrects the axis of the valgus positioned calcaneus. Physical exercising of the foot is especially
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3 0 . D i s o r d e r s of t h e f o o t
important for these children. Insoles are not recommended, since relieving of the foot muscles and passive support of the arch will lead to further weakening of the muscles. When fixed flatfoot develops in older chil dren, loosening of the rigidity may be achieved conservatively through application of a cold compress, and after loosening, the corrected position should in certain cases be maintained with a plaster cast. If no results can be attained through appli cation of a cold compress, manipulation should be performed under narcosis and a plaster cast should possibly be applied. In adolescence and adulthood, patients with flatfoot are recommended insoles. In soles correct the valgus position of the calcaneus, thereby establishing the longitudi nal arch and they may also support the trans verse arch. In case conservative treatment does not bring the desired results, surgical so lutions may also come into question. Instead of the former practice of positioning a bony wedge into the sinus tarsi in order to achieve an extra-articular arthrodesis, corrections are nowadays executed through the utilization of screws. Another possibility is calcaneus osteotomy, in the course of which the patho logic axis is corrected through wedge resec tion and/or shifting. When marked arthrosis is associated with axis deviation, fusion of the talocalcaneal and Chopart 's j oints may be considered.
part of the 1 st metatarsal in the medial direc tion (Fig. 30.20. a, b). One of the conse quences of this displacement is that the m. ab ductor hallucis slides onto the surface of the sole and pronates the hallux. The flexors and extensors with the sesamoid bones lateralize relative to head of the I st metatarsal, pulling the hallux into adduction (valgus) (Fig. 30.2 1 . a ) . Hyperactivity of the m. adductor hallucis and the position of the flexors results in a vi cious circle that constantly amplifies the de formity.
a b
30.3.2. Hallux valgus, metatarsus I. varus This is a valgus deformity (subluxation in the metatarsop halangeal joint) of the ha llux of static origin with a variable extent of varus position of the 1st metatarsal.
Etiopathology. Congenital, constitutional (loose ligaments or falling of the transversal arch) and external factors (tight, fashionable or pointed shoes) can all play a role in the de velopment of a bunion. The most common cause of this disorder is a shift of the distal
Fig. 30.20. a: Severe hallux valgus. b: X- ray image of the above patient.
3 0 . D i s o r d e r s of t h e f o o t
i i
i
a
b
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Fig. 30.2 1. Hallux valgus. a: The pathological pulling direction of the tendons intensifying the valgus (1: m. extensor hallucis longus, 2: m. flexor hallucis brevis, 3: m. add uctor hallucis, 4: m. flexor hallucis longus, 5: m. abductor hallucis). b: Graphic illustration of an orthotic device to cor rect hallux valg us.
Treatment. Pain killers, insoles, the wear ing of wide or orthopedic shoes and perhaps a hallux valgus-correcting appliance (Fig. 30-2 1 . b) at night may come into consider ation as forms of conservative therapy. A sat isfactorily improved condition may be ex pected only after surgical treatment. Radiological parameters, which play a role in the surgical indication are demon strated in Fig. 30.22. There are more than 1 50 possible surgical interventions for the correction of hallux valgus. These may be divided into three basic groups: operations involving only soft tissue, operations of only bony tissue, and a combi nation of the two. One factor that significantly influences the surgical indication is whether arthrosis is present in the 1 st metatarso phalangeal joint. If it is, correctional osteo tomy can not come into question. Soft-tissue operations are generally carried out before the closure of the growth plates. In adulthood, the indication of combined surgery depends on the following factors :
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3
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Fig. 30.22. Radiological parameters playing a part in the indica tion: 1: interphalangeal angle, 2: hallux valgus angle, 3: the angle formed by the metatarsus axis and the joint su rface, 4: the angle formed by the axes of the 1st and 2nd metatarsi.
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a b
c d
Fig. 30.23. Osteotomy of the 1st metatarsus base and Akin osteotomy of the proximal phalanx. a: Clinica l image before su rgery. b: Antero-posterior X-ray image of the foot before surgery. c: Antero-posterior X-ray image of the foot after surgery. d: Clinical image after su rgery.
Phase 1 : The hallux valgus angle is smaller than 2 5° , the intermetatarsal angle is smaller than 1 2° , the mobility of the 1 st metatarsophalangeal j oint is complete, and the dislocation of the sesamoid bone is smaller than 25%. In this case, Schede ' s operation and/or distal metatarsal and/or distal phalanx osteotomy may come into question concurrently with a soft-tissue correction (which generally involves shortening on the medial side and release on the lateral side), supplemented with tenotomy of the adductor hallucis. Schede 's operation: chiseling away the exostosis that has developed on the medial side of the 1 st metatarsal, together with re-
moval of the irritative bursa formed be tween the exostosis and the skin. Phase 2: The hallux valgus angle is 25-3 5° (possibly even 40°), the intermetatarsal angle is 1 2 - 1 8°, there may be subluxation in the 1 st metatarsophalangeal joint, and the dislocation of the sesamoid bones is 25-50%. The interventions applied in this phase: besides removal of the exostoses and soft-tissue interventions, distal (possi bly diaphyseal) or proximal 1 st metatarsal osteotomy and if necessary, proximal pha lanx osteotomy. Phase 3: The hallux valgus angle is larger than 40°, the intermetatarsal angle is larger than 1 8°, the 1 st metatarsophalangeal joint
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is subluxed and/or the 1 sI ray is hyper mobi le. Surgical interventions imple mented in this phase (at a young age, if no arthrosis is present in the metatarso phalangeal joint) are proximal 1 st metatar sal osteotomy (Fig. 30.23. a-d) or meta tarsocuneiform arthrodesis and if neces sary, proximal phalanx osteotomy.
30.3.3. Hallux rigidus Hallux rigid us is the term used to describe pain-ind ucing, isolated limitation of move ment of the 1st metata rsophalangeal joint, it is genera l ly caused by arthrosis. I n these cases, valgus deformity is minimal or not p resent at all. The ROM may be minimal i n the functional aspect, but enough is left to sustai n the pain (Fig. 30.24.).
The clinical symptoms of osteoarthritis of the 1 st metatarsophalangeal j oint: pain, crepi tation and a significantly decreased ROM. The condition may develop as a result of trauma or inflammation, or a preceding bun ion operation. The patient ' s gait is disturbed by the pain induced and the lift-off phase of the patient' s gait i s perturbed because of the absence o f the 1 st metatarsophalangeal j oint extension. Rocking bottom orthopedic shoes are recom mended as conservative treatment. Besides
Fig. 30.25. Schematic illustration of cheilectomy. 113 oblique dorsal resection of the distal joint surface of the 1st metatarsus, and 113 dorsal resection of the proximal joint su rface of the proximal phalanx.
the radiological and clinical condition, the in dication of surgical treatment is strongly de pendent on the patient' s age and the level of his/her requirements . The surgical treatment of hallux rigidus may be categorized as follows : a) An operation retaining the 1 sI metatarso phalangeal joint, which consists in remov ing with a chisel the bony osteophytes that have developed on the metatarso phalangeal joint area of the metatarsus and the proximal phalanx dorsally, in this way re-enabling dorsiflexion of the j oint (cheilectomy) (Fig. 30.25.). b) Operations sacrificing the 1 st metatarso phalangeal j oint, consisting in either re section of the base of the proximal phalanx of the hallux or arthrodesis of the meta tarsophalangeal joint.
30.3.4. Diseases of the 5th ray Bony irregularities and axia l deformities may develop on the 5th ray on the latera l side of the metata rsus (Fig. 30.26. a, b).
Fig 30.24. X- ray image of hallux rigid us. The joint space of the metatarsophalangeal joint is significantly narrowed, and osteophytes may be observed on the edge of the bones.
These disorders are essentially equivalent to lateral deviation or increase of the 4th-5th intermetatarsal angle. They may cause dis comfort and/or problems even when normal shoes are worn.
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1
2
b
3
4
Fig. 30.26. a: Categorization of deformities of the 5th metatarsus: Type 1: Exostosis on the lateral side of the 5th metatarsus head; no lateral deviation on the metatarsus, the 4th-5th intermetatarsal angle is normal. Type 2: I ntense lateral deviation of the 5th metatarsus. No real hypertrophy can be observed on the lateral side of the metatarsus head; the intermetata rsal angle is within the normal range. Type 3: I ncreased i ntermetatarsal ang le; no hypertrophy and the extent of deviation is normal. Type 4: Combined variation: hypertrophy and/or increased deviation is present with an increased intermetatarsal ang le. b: Schematic drawing of a correctional osteotomy performed on$ a type 4 deformity as described above.
Treatment. In cases of exostosis, if no other disorder is observed (an increased 4th-5th intermetatarsal angle or enlarged curve of the 5th metatarsus), removal of the exostosis is recommended. When deformities of the 5th metatarsus are observed, osteotomy may be considered. If the 5th toe is super posed over the 4th toe (digitus V varus super ductus, which may be present congenitally), the above solutions may be supplemented with plastic surgical interventions carried out on the joint capsule, the tendon or the skin, through the formation of artificial syndactyly.
30.3.5. Diseases of the sesamoid bone The most frequent pathological disorders are fractures, avascular necrosis and arthrosis.
30.3.6. Metatarsalgia Metatarsalgia is the term used to describe the pain ind uced by load ing of the metata rsals. Hyperkeratosis is a lso generally present under the metatarsal heads concerned. The d isorder usua l ly develops as a resu lt of pes transversoplanus.
Conservative treatment may prove to be successful with well-adapted insoles, other wise, surgical treatment is recommended. Metatarsalgia of static origin generally affects the central (2nd, 3rd and 4th) metatarsi; in these cases, it is advisable to carry out surgery on the 2nd-4th rays concurrently (e.g. sub capital osteotomy according to Helal), since osteotomy involving only one ray may lead to metatarsalgia of the other rays.
3 0 . D i s o r d e r s of t h e f o o t
30.3.7. Digitus malleus (hammertoe) The flexor-extensor balance on the forefoot is disrupted as a resu lt of the dropping of the transverse arch, and a characteristic condition develops. The metatarsophalangeal joint takes up a hyperextended position, the proximal interphalangeal (PIP) joint fixes in a flexed po sition and the distal interphalangeal (DIP) joint assumes a n extended position.
A clavus forms on the dorsal side of the toe, corresponding to the proximal inter phalangeal joint as a result of the pressure in duced by wearing shoes. A sensitive, irritable bursa develops between the clavus and the skin (Fig. 30.27. a, b). Treatment. In the early stage, the process may be reversible by the wearing of insoles and regular toe exercises; later, after the de formity becomes rigid, only surgical interven tion may yield a good result. Of the several
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possible surgical solutions, the most common is surgical resection of the proximal phalanx head. In neglected cases, when subluxation or luxation has developed in the metatarso phalangeal joint, arthrodesis of the proximal interphalangeal joint, or possibly capsul ectomy in the metatarsophalangeal joint, re section of the base of the proximal phalanx or temporary wire fixation may be necessary.
30.3.8. Morton's neuroma The essence o f t h i s d isorder is a typica l spin d le-shaped neuroma on t he digita l nerve be tween the 3rd and 4th, less freq uently be tween the 2nd and 3rd metatarsi, located d i rectly p roximally from the partition of the nerve.
Clinically, a sharp, flashing pain occurs in the supply area of the nerve, after which a per sistent pain of lower intensity remains. It is important to distinguish the disorder from metatarsalgia, which may be achieved through a simple physical examination. In metatarsalgia, the metatarsals are sensitive to direct pressure from underneath, while the pain is not induced upon transverse pressure of the anterior part of the foot. In contrast, in Morton' s neuralgia, the foot is not sensitive to pressing of the metatarsals from below, but the pain may be provoked by transverse com pression of the metatarsus heads . Treatment. Lidocaine infiltration admin istered in the area of the neuroma eliminates the pain immediately, this may partially be utilized for diagnostic purposes. A local ste roid inj ection may be attempted once, or pos sibly twice. If no improvement is experi enced, removal of the thickened nerve seg ment may be considered.
Fig. 30.27. a: Schematic illustration of a hammertoe deformity. b: Hammertoe.
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30.3.9. March fracture (stress fracture, fatigue fractures on the metatarsals) The term "ma rch fracture" also implies the es sence of the i l l ness. It was first described among soldiers, as a syndrome occu rring after persistent strain, marching.
The fundamentals of the disorder are stress fractures which develop most commonly on the 2nd and 3rd metatarsi, as a result of per sisting, repeated strain; these fractures can not be spotted immediately in the X-ray picture. A few days later, upon osteolysis of the edges of the fracture, it can be well distinguished, and after a few weeks, a large mass of callus may be observed (See Chapter 3 and Fig. 3 . 5). The significance of this diagnosis is chiefly the differential-diagnostic aspect: if it is not taken into consideration, a turnor may be suspected. Later, metatarsalgic complaints may arise subsequent to march fractures. Treatment. The condition heals spontane ously if the legs are rested.
30.3. 10. Achillodynia (paratenonitis of the Achilles tendon) This d isorder is cha racterized by pain in the Achilles tendon and the surrounding paratenon, possibly accompanied by its thick ening.
The condition is characteristic at a young age, or in male patients who earlier exercised regularly and resume physical exercise after a long intermission. The increased strain on the Achilles tendon causes pain, pressure sensi tivity and a palpable, reel-shaped swelling 1 -2 cm above the point where the tendon is at tached to the calcaneus. In certain cases, crep itation is also observed above the tendon upon movement of the feet. When further physical exercise is forced, the total rupture of the damaged Achilles tendon often occurs.
Differential diagnosis. This disorder must be distinguished from partial or total rupture of the Achilles tendon. If a rupture has occurred, an indent is palpable in the affected area, and the patient can not support him/her self on tiptoe on one foot. The Thomson test may be of assistance in establishing the cor rect diagnosis: with the patient lying in prone position, in normal cases, compression of the flexing muscles of the leg results in plantarflexion of the foot; with a ruptured Achilles tendon, the foot does not move. Treatment. This comprises resting, eleva tion of the leg and the administration of nonsteroidal anti-rheumatic drugs. After the temporary discontinuance of physical exer cise, it is very important to recommence and build up the exercising program gradually, corresponding to the load-bearing capacity of the tendon. Locally administered steroid products may reduce the discomfort, but they are not recommended, for they increase the danger of rupture of the Achilles tendon.
30.3. 1 1. Haglund heel This disorder involves a bony enlargement developing in adolescence, after the end of the growth phase, on the upper part of the dorsal surface of the calcaneus, in the area where the Achilles tendon adheres to the bone. The upper rim of the shoe presses on the bony enlargement, causing severe discomfort. This is not genuine exostosis, the disorder is rather a variation of form, or excessive bone mass. Treatment: by means of periodic change of the shoes and conservative treatment (cold compresses and nonsteroidal anti-inflamma tory cream) the complaints usually cease. Rarely, in stubborn cases, resection of the bone excess may be carried out.
30.3. 12. Calcaneus spur The essence of this disorder is that a small, spur-like protrusion develops at the origin of
3 0 . D i s o r d e r s o f t h e foot
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30.3.13. Arthrosis of the foot joints
Fig. 30.28.
This d isorder develops most frequently in the subtalar joint and Chopart's joint. Primary a rthrosis occurs in elderly patients. The sig nifi cance of secondary a rthrosis is higher. These cases a re genera l ly recognised as end stages of progressive prearthrotic conditions (pes excavatus, clu bfoot, etc.) o r as a conseq uence of some other d isease (inflammation, aseptic bone necrosis, or trauma).
X-ray picture of a calcaneal spur.
the plantar fascia, originating from the tuber of the calcaneus, which is sensitive to strain or direct pressure. Patients generally complain of a stinging pain in the heel area, which they compare to stepping on a sharp pebble. The sensitive area is well palpable, and the pain (usually originating at the medial perim eter of the calcaneus tuber) can be provoked by applying pressure. In a lateral X-ray pic ture, a spike-shaped protuberance can be ob served at the origin of the aponeurosis plantaris in the maj ority of cases (Fig. 30.28). Considering that in many cases, a calcaneus spur is not always seen in spite of the intense pain, and as a spur may develop without caus ing the patient any discomfort, it may be pre sumed, that the pain is not induced by the exostosis, but by the overstraining of the plan tar structures and periostitis. Treatment is generally conservative: in most cases, relaxation, the wearing of insoles to relieve the foot and the administration of anti-inflammatory agents will improve the pa tient's condition. Also, local steroid inj ections often have a good effect. If conservative treat ment does not produce acceptable results in 3-6 months, incision of the aponeurosis plantaris adjacent to the calcaneal origin may be considered. Recently, by means of an ar throscopic intervention, the aponeurosis can be detached through a minimally invasive procedure as an outpatient operation.
Clinical symptoms. Pain localized in the arthrotic area causes discomfort on movement of the affected joint. The area is sensitive to pressure, and osteophyte formation is often palpable upon external examination. Radiologically, narrowing of the joint space is characteristic in osteoarthritis, with subchondral sclerosis, subchondral cysts and osteophytes. Treatment. In mild cases, non steroidal anti-rheumatic agents, symptomatic treatment and therapeutic X-ray irradiation may be highly effective. Orthopedic shoes may be recommended later. In case conservative treatment does not prove successful, surgical treatment may be considered, with arthrodesis of the arthrotic joint. When the entire tarsus is affected, combined arthrodesis (triple arthrodesis) of the talocalcaneal j oint and Chopart ' s j oint is performed. It is important to distinguish arthrosis from diabetes-related arthropathy, which in its early stage may dis play a picture similar to that of conventional osteoarthritis, but demonstrates only a few of the clinical symptoms of the disorder.
30.3. 14. Tarsal tunnel syndrome This involves compression of the tibial nerve on the inside of the medial ankle at the entrance of the tarsal tunnel . The mechanism of its development corresponds to that of the carpal tunnel syndrome. The causes of the dis order may be any of the following : inflamma tion, trauma, a venous circulation disorder, or
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3 0 . D i s o rders of the foot
functional reasons. Patients describe nightly paresthesia on the sole of the foot, around their toes. The symptoms can be provoked by applying direct pressure to the tarsal tunnel. An EMG examination may help establish the diagnosis. Treatment. Conservative treatment, shoe inserts to support the medial side of the sole, and the administration of non steroidal anti-inflammatory drugs lead to an improve ment. Rarely, surgical decompression may be indicated.
30.3. 15. Diabetic foot Diabetic foot is a collective term for foot p roblems relating to diabetes: macro and microangiopathy and foot deformities devel oping as a conseq uence of neuro pathy, ac companied by u lcers o r necrosis in severe cases.
The nutrition and oxygenization of the tis sues is diminished because of the impaired blood circulation, and wounds therefore heal with greater difficulty, than under normal conditions. Because of the sensation disorders developing as an accessory symptom of the neuropathy, minor injuries of the foot do not
Fig. 30.29. Characteristic symptoms of a diabetic foot: the skin is d ry and cracked, the toenails are dry and are mycosis-infected, and a circulation disorder in an early stage may be perceived u nder the 1st metatarsus head.
attract attention, until more severe complaints occur, resulting from the disorder not having been treated (Fig. 30.29.). Clinical experience shows that certain diabetic ulcers develop not on the sole (under pressure), but on the dor sum ofthe foot, this proving that ulcers are not caused merely by pressure. It is most impor tant to diagnose the neuropathy and also to prevent it. The risk factors of ulcers develop ing on the diabetic foot are as follows : - retinopathy, - old age, - previous foot ulcers, - alcohol consumption, - smoking, - known cardiac disease, - foot deformity (hallux valgus, hammertoe, etc.), - limited joint mobility. The following measures may be recom mended as forms of prevention. The diabetic patient should wash hislher feet regularly with warm water. The temperature of the water should be checked before stepping into it. The patient should examine hislher soles every day with the help of a mirror. Drying-out of the skin should be avoided by the application of skin cream. Before putting on shoes, the pa tient should ascertain that there are no wrin kles in the socks and no other pressure-induc ing obj ects are present. The patient should not walk barefoot or wear new shoes for long pe riods. If the patient perceives any problems, he/she should turn to a physician immedi ately, and should not treat the complaint at home. The development of ulcers and rapid progression of the infection is a serious risk in case of diabetic feet and in certain cases may lead to amputation of the foot. It is most im portant, therefore, to control the patient's blood sugar level. From an orthopedic aspect, total contact shoe inserts and the provision of diabetic orthopedic shoes are of great signifi cance as concerns the avoidance of the devel opment of ulcers and other complications .
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