Avascular necrosis has long been recognized as a complication of femoral head fractures, the usual explanation being traumatic

severance of the blood supply to the femoral head. Segmental osteonecrosis also appears as a distinctive feature in a number of nontraumatic disorders: joint infection, Perthes disease, caisson disease, Gauchers disease, systemic lupus erythematosus (SLE), high-dosage corticosteroid administration and alcohol abuse, to mention only the more common ones. Whatever the cause, the condition, once established, may come to dominate the clinical picture, demanding attention in its own right. Aetiology and pathogenesis Sites which are peculiarly vulnerable to ischaemic necrosis are the femoral head, the femoral condyles, the head of the humerus, the capitulum and the proximal parts of the scaphoid and talus. These subarticular regions lie at the most distant parts of the bones vascular territory, and they are largely enclosed by cartilage, giving restricted access to local blood vessels. The subchondral trabeculae are further compromised in that they are sustained largely by a system of endarterioles with limited collateral connections. Another factor which needs to be taken into account is that the vascular sinusoids which nourish the marrow and bone cells, unlike arterial capillaries, have no adventitial layer and their patency is determined by the volume and pressure of the surrounding marrow tissue, which itself is encased in unyielding bone. The system functions essentially as a closed compartment within which one element can expand Osteonecrosis and related disorders 6 MAIN CONDITIONS ASSOCIATED WITH NON-TRAUMATIC OSTEONECROSIS Infections Osteomyelitis Septic arthritis Haemoglobinopathy Sickle cell disease Storage disorders Gauchers disease Caisson disease Dysbaric osteonecrosis Coagulation disorders Familial thrombophilia

 Hypofibrinolysis Hypolipoproteinaemia Thrombocytopenic purpura Other Perthes disease Cortisone administration Alcohol abuse SLE (? increase in antiphospholipid antibodies) Pregnancy (? decreased fibrinolysis; ? fatty liver) Anaphylactic shock Ionizing radiation SLE, systemic lupus erythematosus. 6.1 Avascular necrosis pathogenesis The medullary cavity of bone is virtually a closed compartment containing myeloid tissue, marrow fat and capillary blood vessels. Any increase in fat cell volume will reduce capillary circulation and may result in bone ischaemia. Louis Solomon only at the expense of the others. Local changes such as decreased blood flow, haemorrhage or marrow swelling can, therefore, rapidly spiral to a vicious cycle of ischaemia, reactive oedema or inflammation, marrow swelling, increased intraosseous pressure and further ischaemia. The process described above can be initiated in at least four different ways: (1) severance of the local blood supply; (2) venous stasis and retrograde arteriolar stoppage; (3) intravascular thrombosis; and (4) compression of capillaries and sinusoids by marrow swelling. Ischaemia, in the majority of cases, is due to a combination of several of these factors. TRAUMATIC OSTEONECROSIS In traumatic osteonecrosis the vascular anatomy is particularly important. In fractures and dislocations of the hip the retinacular vessels supplying the femoral head are easily torn. If, in addition, there is damage to or thrombosis of the ligamentum teres, osteonecrosis is inevitable. Little wonder that displaced fractures of the femoral neck are complicated by osteonecrosis in over 20 per cent of cases. Undisplaced fractures, or lesser injuries, also sometimes result in subchondral necrosis; this may be due to thrombosis of intraosseous capillaries or sinusoidal occlusion due to marrow oedema. Other injuries which are prone to osteonecrosis are fractures of the scaphoid and talus. Significantly, in

these cases it is always the proximal fragment which suffers. This is because the principal vessels enter the bones near their distal ends and take an intraosseous course from distal to proximal. Impact injuries and osteoarticular fractures at any of the convex articular surfaces behave in the same way and often develop localized ischaemic changes. These small lesions are usually referred to as osteochondroses and many of them have acquired eponyms which are firmly embedded in orthopaedic history. NON-TRAUMATIC OSTEONECROSIS The mechanisms here are more complex and may involve several pathways to intravascular stasis or thrombosis, as well as extravascular swelling and capillary compression. Intravascular thrombosis Various mechanisms leading to capillary thrombosis have been demonstrated in patients with non-traumatic osteonecrosis. Over 80 per cent of cases are associated with high-dosage corticosteroid medication or alcohol abuse (or both, acting cumulatively). These conditions give rise to hyperlipidaemia and fatty degeneration of the liver. Jones (1994) has favoured the idea that fat embolism plays a part, giving rise to capillary endothelial damage, platelet aggregation and thrombosis. Glueck et al. (1996, 1997a) have suggested that thrombophilia and hypofibrinolysis are important aetiological factors in both adult osteonecrosis and Perthes disease. Other coagulopathies have been implicated, e.g. antiphospholipid deficiency in SLE (Asherson et al., 1993) and enhanced coagulability in sickle-cell disease (Francis, 1991), and it now seems likely that coagulation abnormalities of one sort or another play at least a contributory role in some of the disorders associated with non-traumatic osteonecrosis. Extravascular marrow swelling High-dosage cortico steroid administration and alcohol overuse cause fat cell swelling in the marrow, a feature which is very obvious in bone specimens obtained during joint replacement. There is a demonstrable rise in intraosseous pressure and contrast venography shows slowing of venous blood flow from the bone. Ficat and Arlet (1980) posited that the increase in marrow fat volume in the femoral head caused sinusoidal compression, venous stasis and retrograde ischaemia leading to trabecular bone death; in other words, the

establishment of a compartment syndrome. Whichever of these mechanisms offers the primary pathway to non-traumatic bone ischaemia, it is almost certain that both intravascular and extravascular factors come into play at a fairly early stage and each enhances the effect of the other. 6.2 Avascular necrosis Algorithm showing how various disorders may enter the vicious cycle of capillary stasis and marrow engorgement. Pathology and natural history Bone cells die after 1248 hours of anoxia, yet for days or even weeks the gross appearance of the affected segment remains unaltered. During this time the most striking histological changes are seen in the marrow: loss of fat cell outlines, inflammatory cell infiltration, marrow oedema, the appearance of tissue histiocytes, and eventual replacement of necrotic marrow by undifferentiated mesenchymal tissue. A characteristic feature of ischaemic segmental necrosis is the tendency to bone repair, and within a few weeks one may see new blood vessels and osteoblastic proliferation at the interface between ischaemic and live bone. As the necrotic sector becomes demarcated, vascular granulation tissue advances from the surviving trabeculae and new bone is laid down upon the dead; it is this increase in mineral mass that later produces the radiographic appearance of increased density or sclerosis. Reparative new bone formation proceeds slowly and probably does not advance for more than 8 10 mm into the necrotic zone. With time, structural failure begins to occur in the most heavily stressed part of the necrotic segment. Usually this takes the form of a linear tangential fracture close to the articular surface, possibly due to shearing stress. The crack may break through the articular cartilage and at operation it may be possible to lift the lid off the necrotic segment like the cracked shell of a hard-boiled egg. However, until very late the articular cartilage retains its thickness and viability. In the final stages, fragmentation of the necrotic bone leads to progressive deformity and destruction of the joint surface. In the past, when diagnosis was based entirely on x-ray changes, it was thought that osteonecrosis always progressed to bone collapse. Now that it is possible to detect the earliest signs by MRI, it has

become apparent that this is not the case. The size of the necrotic segment, as defined by the hypo-intense band in the T1 weighted MRI, is usually established at the time of the initiating ischaemic event, and from then on it rarely increases; indeed, there is evidence that non-traumatic lesions sometimes diminish in size and occasionally even disappear (Sakamoto et al., 1997). In persistent lesions, the rate of bone collapse depends largely on the site and extent of the necrotic segment: lesions which lie outside the normal stress trajectories may remain structurally intact while those that involve large segments of the load-bearing surface usually collapse within 3 years (see under Staging). Clinical features The earliest stage of bone death is asymptomatic; by the time the patient presents, the lesion is usually well advanced. Pain is a common complaint. It is felt in or near a joint, and perhaps only with certain movements. Some patients complain of a click in the joint, probably due to snapping or catching of a loose articular fragment. In the later stages the joint becomes Osteonecrosis and related disorders 105 6 (a) (b) (c) (d) (e) (f) 6.3 Osteonecrosis pathology (a,b) Normal femoral head and cut section. The articular cartilage is obviously intact and the subchondral bone is well vascularized. (c,d) In this femoral head with osteonecrosis the articular cartilage is lifted off the bone; the coronal section in (d) shows that this is due to a subarticular fracture through the necrotic segment in the dome of the femoral head. (e) Histological section across the junction between articular cartilage and bone showing living cartilage cells but necrotic subchondral marrow and bone. (f) High power view showing islands of dead bone with empty osteocytic lacunae enfolded by new, living bone. stiff and deformed. Local tenderness may be present and, if a superficial bone is affected, there may be some swelling. Movements or perhaps one particular movement may be restricted; in advanced cases there may be fixed deformities. Imaging X-ray The early signs of ischaemia are confined to the

bone marrow and cannot be detected by plain x-ray examination. X-ray changes, when they appear (seldom before 3 months after the onset of ischaemia), are due to (a) reactive new bone formation at the boundary of the ischaemic area and (b) trabecular failure in the necrotic segment. An area of increased radiographic density appears in the subchondral bone; soon afterwards, suitable views may show a thin tangential fracture line just below the articular surface the crescent sign. In the late stages there is distortion of the articular surface and more intense sclerosis, now partly due to bone compression in a collapsed segment. Occasionally the necrotic portion separates from the parent bone as a discrete fragment. However, it is now recognized that in the case of the femoral head and the medial femoral condyle such necrotic fragments may have resulted from small osteo-articular fractures which only later failed to unite and lost their blood supply. With all the changes described here (and this is the cardinal feature distinguishing primary avascular necrosis from the sclerotic and destructive forms of osteoarthritis) the joint space retains its normal width because the articular cartilage is not destroyed until very late. Radioscintigraphy Radionuclide scanning with 99mTcsulphur colloid, which is taken up in myeloid tissue, GENERAL ORTHOPAEDICS 106 6 (a) (b) (c) (d) 6.4 Avascular necrosis of bone pathology (a) This is a diagramatic guide to the fine-detail xrays of necrotic femoral heads (bd) which show the progress of osteonecrosis. The articular cartilage (A) remains intact for a long time. The necrotic segment (B) has a texture similar to that of normal bone, but it may develop fine cracks. New bone surrounds the dead trabeculae and causes marked sclerosis (C). Beyond this the bone remains unchanged (D). In the later stages the necrotic bone breaks up and finally the joint surface is destroyed. (a) (b) (c) 6.5 Avascular necrosis x-ray (a) The earliest x-ray sign is a thin radiolucent crescent just below the convex articular surface where load bearing is at its greatest. This represents an undisplaced subarticular fracture in the early necrotic

segment. (b) At a later stage the avascular segment is defined by a band of increased density due to vital new bone formation. At this stage the femoral head may still be spherical and (unlike osteoarthritis) the articular space is still well-defined. (c) In late cases there is obvious collapse and distortion of the articular surface. may reveal an avascular segment. This is most likely in traumatic avascular necrosis, where a large segment of bone is involved, or in sickle-cell disease where a cold area contrasts significantly with the generally high nuclide uptake due to increased erythroblastic activity. 99mTc-HDP scans (in the bone phase) may also show a cold area, particularly if a large segment of bone is avascular (e.g. after fracture of the femoral neck). More often, however, the picture is dominated by increased activity, reflecting hyperaemia and new bone formation in the area around the infarct. Magnetic resonance imaging MRI is the most reliable way of diagnosing marrow changes and bone ischaemia at a comparatively early stage. The first sign is a band-like low-intensity signal on the T1 weighted spin echo (SE) image (and a similar but high-intensity signal on the short-tau inversion recovery (STIR) image), corresponding to the interface between ischaemic and normal bone. The site and size of the demarcated necrotic zone have been used to predict the progress of the lesions (see Chapter 19). Computed tomography CT involves considerable radiation exposure and it is not very useful for diagnosing osteonecrosis. However, it does show the area of bone destruction very clearly and it may be useful in planning surgery. Tests for haemodynamic function During the early stage of ischaemic necrosis the intramedullary pressure is often markedly raised. This phenomenon is most easily demonstrated in the femoral head. A cannula introduced into the metaphysis enables measurements to be taken (1) at rest and (2) after rapid injection of saline. The normal resting pressure is 1020 mmHg, rising by about 15 mm after saline injection; in early osteonecrosis both the intramedullary pressure and the response to saline injection may be increased three- or four-fold. Venous stasis can also be demonstrated by venography after injection of radio-opaque medium into the bone. Similar findings have been recorded in osteoarthritis, but the change is not nearly as marked as in

osteonecrosis. Staging the lesion Ficat and Arlet (1980) introduced the concept of radiographic staging for osteonecrosis of the hip to distinguish between early (pre-symptomatic) signs and later features of progressive demarcation and collapse of the necrotic segment in the femoral head. Stage 1 showed no Osteonecrosis and related disorders 107 6 (a) (b) (c) (a) (b) 6.6 Osteonecrosis MRI (a) Before any change is discernible on the plain x-ray, MRI will show a typical hypointense band in the T1 weighted image, outlining the ischaemic segment beneath the articular surface. (b) In this case the size of the ischaemic segment is much larger and the likelihood of bone crumbling much greater. 6.7 Osteonecrosis distribution The most common sites for osteonecrosis are the head of the femur, the head of the humerus and, as shown here, the medial condyle of the femur, the talus and the capitulum. All these areas are located beneath convex articular surfaces; osteonecrosis is seldom seen beneath a concave articular surface. x-ray change and the diagnosis was based on measurement of intraosseous pressure and histological features of bone biopsy (or nowadays on MRI). In Stage 2 the femoral head contour was still normal but there were early signs of reactive change in the subchondral area. Stage 3 was defined by clearcut x-ray signs of osteonecrosis with evidence of structural damage and distortion of the bone outline. In Stage 4 there were collapse of the articular surface and signs of secondary OA. Later modifications involving assessment of both the extent and the location of the early changes on plain x-ray and MRI have proved to be more reliable as predictors of outcome, at least in relation to femoral head necrosis (Shimizu et al., 1994; Steinberg et al., 1995). The location and size of the necrotic segment in Ficat stages 13 are defined by the hypo-intense band on the T1 weighted MRI. Two general observations can be made: (1) the size of the ischaemic segment is determined at a very early stage and it rarely increases after that; (2) small lesions which do not involve the maximally loaded zone of the articular surface tend not to collapse, whereas large lesions extending under

the maximally loaded articular surface break down in over 60 per cent of cases. Shimizus classification is particularly useful in planning treatment; this is discussed in Chapter 19. The most widely used system, which permits comparison between series from different participating centres, is the one promoted by the International Association of Bone Circulation and Bone Necrosis (Association Research Circulation Osseous ARCO) which applies mainly to femoral head necrosis (Table 6.1). Diagnosis of the underlying disorder In many cases of osteonecrosis an underlying disorder will be obvious from the history: a known episode of trauma, an occupation such as deep-sea diving or working under compressed air, a family background of Gauchers disease or sickle-cell disease. There may be a record of high-dosage corticosteroid administration; for example, after renal transplantation where the drug is used for immunosuppression. However, smaller doses (e.g. as short-term treatment for asthma or as an adjunct in neurosurgical emergencies) and even topical steroid preparations can also be dangerous in patients with other risk factors (Solomon and Pearse, 1994). Combinations of drugs (e.g. cortico steroids and azathioprine, or corticosteroids after a period of alcohol abuse) also can be potent causes of osteonecrosis; occasionally corticosteroids have been given without the patients knowledge. Alcohol abuse is often difficult to determine because patients tend to hide the information. There is no biochemical marker that is specific for high alcohol intake but elevation of three or four of the following is suggestive: aspartate transaminase, -glutamyl transpeptidase, serum urate, serum triglyceride and mean red cell volume (Whitehead et al., 1978). Ideally patients with very early non-traumatic osteonecrosis, and children with early Perthes disease, should undergo laboratory tests for coagulopathies; this is justified by reports of cases in which the condition has been halted or reversed by treatment with antithrombotic preparations such as warfarin and stanozolol (Glueck et al., 1997b). Unfortunately the tests are very expensive and there is understandable resistance to adopting this approach in routine management.

In cases of suspected SLE, antiphospholipid antibodies may be measured. Prevention Where risk factors for osteonecrosis are recognized, preventive steps can be taken especially in the management of corticosteroid medication and alcohol abuse. Corticosteroids should be used only when essential and in minimal effective dosage. It is important also to be aware of the cumulative effect of even moderate doses of corticosteroids in patients with a history of alcohol abuse. Anoxia must be prevented in GENERAL ORTHOPAEDICS 108 6 Table 6.1 ARCO staging of osteonecrosis Stage 0 Patient asymptomatic and all clinical investigations normal Biopsy shows osteonecrosis Stage 1 X-rays normal. MRI or radionuclide scan shows osteonecrosis Stage 2 X-rays and/or MRI show early signs of osteonecrosis but no distortion of bone shape or subchondral crescent sign Subclassification by area of articular surface involved: A = less than 15 per cent B = 1530 per cent C = more than 30 per cent Stage 3 X-ray shows crescent sign but femoral head still spherical Subclassification by length of crescent/articular surface: A = less than 15 per cent B = 1530 per cent C = more than 30 per cent Stage 4 Signs of flattening or collapse of femoral head A = less than 15 per cent of articular surface B = 1530 per cent of articular surface C = more than 30 per cent of articular surface Stage 5 Changes as above plus loss of joint space (secondary OA) Stage 6 Changes as above plus marked destruction of articular surfaces patients with haemoglobinopathies. Decompression procedures for divers and compressed-air workers should be rigorously applied. Treatment In planning treatment, all the factors that influence

the natural course of the condition must be taken into account: the general medical background, the type of ischaemic necrosis, the site and extent of the necrotic segment, its stage of development, the patients age and capacity for bone repair, the persistence or otherwise of the aetiological agent and its effect on bone turnover. Only general principles will be discussed here; the treatment of osteonecrosis in specific sites is dealt with in the appropriate chapters on regional orthopaedics. EARLY OSTEONECROSIS While the bone contour is intact there is always the hope that structural failure can be prevented. Some lesions heal spontaneously and with minimal deformity; this is seen especially in areas which are not severely stressed: the non-weightbearing joints, the superomedial part of the femoral head and the nonweightbearing surfaces of the femoral condyles and talus. Here one can afford to pursue a waiting policy. In the past, various types of medication failed to show convincing evidence of preventing collapse of the subchondral bone in cases of early osteonecrosis. Recently, however, there have been promising reports of the effect of bisphosphonates in these cases. In a controlled study of the patients (54 femoral heads) with ARCO stage 2 or 3 osteonecrosis, those treated with oral alendronate for 25 weeks were found after 2 years to show a significantly lower rate of femoral head collapse than untreated controls (Lai et al., 2005). Other studies have shown similar results (Nishii et al., 2006). However, it is still too early to comment on the long-term success of this treatment. Lesions in heavily loaded joints have a poor prognosis and will probably end in structural failure if left untreated. Simple measures to reduce loading of weight-bearing joints may help, though their value has not been proven. If the bone contour is still intact, an unloading osteotomy will help to preserve the anatomy while remodelling proceeds. This approach is applicable especially to the hip and knee. Medullary decompression and bone grafting may have a place in ARCO stage 1 and 2 osteonecrosis of the femoral head (Chapter 19). INTERMEDIATE STAGE OSTEONECROSIS Once there is structural damage and distortion of the articular surface, conservative operations are inappropriate.

However, the joint may still be salvageable and in this situation realignment osteotomy either alone or combined with curettage and bone grafting of the necrotic segment has a useful role. If mobility can be sacrificed without severe loss of function (e.g. in the ankle or wrist), arthrodesis will relieve pain and restore stability. LATE STAGE OSTEONECROSIS Destruction of the articular surface may be give rise to pain and severe loss of function. Three options are available: (1) non-operative management, concentrating on pain control, modification of daily activities and, where appropriate, splintage of the joint; (2) arthrodesis of the joint, e.g. the ankle or wrist; or (3) partial or total joint replacement, the preferred option for the shoulder, hip and knee. Osteonecrosis and related disorders 109 6 (a) (b) 6.8 Osteonecrosis treatment (a) Alcohol abuse has led to bilateral femoral head necrosis, advanced on the left but detectable only by MRI on the right. (b) The left hip had to be replaced; at the same time the right side was treated by drilling of the femoral neck (medullary decompression). This x-ray was taken 8 years later. SYSTEMIC DISORDERS ASSOCIATED WITH OSTEONECROSIS DRUG-INDUCED NECROSIS Alcohol, corticosteroids, immunosuppressives and cytotoxic drugs, either singly or in combination, are the commonest causes of non-traumatic osteonecrosis. At risk doses for these drugs have not been established; the threshold depends not only on the total intake but also on the time over which the intake is spread and the presence or absence of associated disorders which themselves may predispose to osteonecrosis. A cumulative dose of 2000 mg of prednisone equivalent administered over several years (for example in the treatment of rheumatoid arthritis) is less likely to cause osteonecrosis than the same dose given over a period of a few months (e.g. after organ

transplantation). It is important to bear in mind that multiple causative agents have an additive effect; thus, osteonecrosis has been encountered after comparatively short courses and low doses of corticosteroids (totals of 800 mg or less), but in these cases an additive factor can almost always be identified (Solomon and Pearse, 1994). The threshold dose for alcohol is equally vague. However, based on the known dose relationship of alcohol-induced fatty degeneration of the liver, we would set it at around 150 mg of ethanol per day (for men) the equivalent of 300 mL of spirits, 1.2 litres of table wine or 3 litres of beer continuing for over 2 years. The dose for women is considerably less. Asking patients How much do you drink? is unlikely to elicit an accurate response. However, the presence of raised serum triglyceride and g-GT levels, together with an increased mean corpuscular volume (MCV), is suggestive of excessive alcohol intake. SICKLE-CELL DISEASE Sickle-cell disease is a genetic disorder in which the red cells contain abnormal haemoglobin (HbS). In deoxygenated blood there is increased aggregation of the haemoglobin molecules and distortion of the red cells, which become somewhat sickle-shaped. At first this is reversible and the cells reacquire their normal shape when the blood is oxygenated. Eventually, however, the red cell membrane becomes damaged and the cells are permanently deformed. The sickle-cell trait, which originated in West and Central Africa centuries ago, is an example of natural selection for survival in areas where malaria was endemic. From there the gene was carried to countries along the Mediterranean, the Persian Gulf, parts of India and across the Atlantic where it appears in people of Afro-American descent. In recent years it has spread more widely in Europe but it is rarely encountered south of the equator. Sickle-cell disease is most likely in homozygous offspring (those with HbS genes from both mother and father), but it may also occur in heterozygous children with HbS/C haemoglobinopathy and HbS/thalassaemia. Inheritance of one HbS gene and one normal b-globin gene confers the (heterozygous) sickle-cell trait; HbS concentration is low and sickling occurs

only under conditions of hypoxia (e.g. under inefficient anaesthesia, in extreme cold, at very high altitudes and when flying in unpressurized aircraft). In the established disorder, the main clinical features are due to a combination of chronic haemolytic anaemia and a tendency to clumping of the sickleshaped cells which results in diminished capillary flow and recurrent episodes of intracapillary thrombosis. Secondary changes such as trabecular coarsening, infarctions of the marrow, periostitis and osteonecrosis are common. Complications include hyperuricaemia (due to increased red cell turnover) and an increased susceptibility to bacterial infection. Clinical features Children during the first two years of life may present with swelling of the hands and feet. X-rays at first seem normal, but later there may be suggestive features such as marrow densities and periosteal new bone formation (dactylitis). These changes are usually transient, but treatment is required for pain. In older children a typical feature is recurrent episodes of severe pain, sometimes associated with fever. These crises, which may affect almost any part of the body, are thought to be due to infarcts. Osteonecrosis of the femoral head is common, both in children (when it is sometimes mistaken for Perthes disease) and in young adults, in whom other causes of non-traumatic osteonecrosis have to be excluded (Iwegbu and Fleming, 1985). Males and females are affected with almost equal frequency. The child develops a painful limp and movements are restricted. X-rays may show no more than a diffuse increase in density of the epiphysis; however, in most cases the changes are very similar to those of Perthes disease, usually going on to flattening of the epiphysis. In young adults there are both destructive lesions and diffuse sclerosis of the femoral head. The head of the humerus and the femoral condyles may be similarly affected. Other bone changes are due to a combination of marrow hyperplasia and medullary infarctions. Trabecular coarsening and thickening of the cortices may be mistaken for signs of infection. GENERAL ORTHOPAEDICS 110 6

Bacterial osteomyelitis and septic arthritis, sometimes involving multiple sites, are serious complications, particularly in children. In over 50 per cent of cases the organism is Salmonella. Treatment A follow-up study of untreated children with femoral head necrosis due to sickle-cell disease showed that 80 per cent of them had permanently damaged hips with severe loss of function (Hernigou et al., 1991). This may be due to recurrent infarction and inflammatory changes in the joint. Hypoxic conditions favouring the occurrence of crises should be avoided. If episodes of bone pain are frequent, transfusions may be necessary to reduce the concentration of HbS. During a crisis the patient should be given adequate analgesia and should be kept fully oxygenated. Infections should be guarded against, or treated promptly with the appropriate antibiotics. Femoral head necrosis in children should be treated in the same way as Perthes disease (see page 511). Adults are treated along the lines described on page 531. The emphasis in all cases should be on conservatism. Anaesthesia carries definite risks; failure to maintain adequate oxygenation may precipitate vascular occlusion in the central nervous system, lungs or kidneys. Prophylactic antibiotics are advisable as the risk of postoperative infection is high. CAISSON DISEASE AND DYSBARIC OSTEONECROSIS Decompression sickness (caisson disease) and osteonecrosis are important causes of disability in deep-sea divers and compressed-air workers building tunnels or underwater structures. Under increased air pressure the blood and other tissues (especially fat) become supersaturated with nitrogen; if decompression is too rapid the gas is released as bubbles, which cause local tissue damage, generalized embolic phenomena and intracapillary coagulation. Prolonged compression may also cause swelling of marrow fat cells and decreased intramedullary blood flow, possibly due to oxygen toxicity (Pooley and Walder, 1984). The symptoms of decompression sickness, which may develop within minutes, are pain near the joints (the bends), breathing difficulty and vertigo (the staggers). In the most acute cases there can be circulatory and respiratory collapse, severe neurological

changes, coma and death. Only 10 per cent of patients with bone necrosis give a history of decompression sickness. Radiological bone lesions have been found in 17 per cent of compressed-air workers in the UK; almost half the lesions are juxta-articular mainly in the humeral head and femoral head but microscopic bone death is much more widespread than x-rays suggest. Clinical and x-ray features The necrosis may cause pain and loss of joint movement, but many lesions remain silent and are found only on routine x-ray examination. Medullary infarcts cause mottled calcification or areas of dense sclerosis. Juxta-articular changes are similar to those in other forms of osteonecrosis. Management The aim is prevention; the incidence of osteonecrosis is proportional to the working pressure, the length of exposure, the rate of decompression and the number of exposures. Strict enforcement of suitable working schedules has reduced the risks considerably. The treatment of established lesions follows the principles already outlined. GAUCHERS DISEASE (see also page 177) In this familial disorder lack of a specific enzyme results in the abnormal storage of glucocerebroside in the macrophages of the reticuloendothelial system. Osteonecrosis and related disorders 111 6 6.9 Sickle-cell disease (a) Typical features of osteonecrosis are seen in the femoral head, often accompanied by patchy areas of bone destruction and endosteal sclerosis in the femoral shaft. (b) The spine also may be involved, producing appearances similar to those of bone infection. (c) In severe cases infarctions of tubular bones may resemble osteomyelitis, with sequestra and a marked periosteal reaction. (a) (b) (c) The effects are seen chiefly in the liver, spleen and bone marrow, where the large polyhedral Gaucher cells accumulate. Bone complications are common and osteonecrosis is among the worst of them. The hip is most frequently affected, but lesions also appear in the distal femur, the talus and the head of the humerus. Bone ischaemia is usually attributed to the increase in medullary cell volume and sinusoidal compression,

but it is likely that other effects (abnormal cell emboli and increased blood viscosity) are equally important. Clinical features Bone necrosis may occur at any age and causes pain around one of the larger joints (usually the hip). In longstanding cases movements are restricted. There is a tendency for the Gaucher deposits to become infected and the patient may present with septicaemia. Blood tests reveal anaemia, leucopenia and thrombocytopaenia. A diagnostic, though inconstant, finding is a raised serum acid phosphatase level. X-ray The appearances resemble those in other types of osteonecrosis, and silent lesions may be found in a number of bones. A special feature (due to replacement of myeloid tissue by Gaucher cells) is expansion of the tubular bones, especially the distal femur, producing the Erlenmeyer flask appearance. Cortical thinning and osteoporosis may lead to pathological fracture. Treatment The condition can now be treated by replacement of the missing enzyme and there is evidence that this will reduce the incidence of bone complications. The management of established osteonecrosis follows the principles outlined earlier. However, there is a greater risk of infection following operation and suitable precautions should be taken. For adults, total joint replacement is probably preferable to other procedures. RADIATION NECROSIS Ionizing radiation, if sufficiently intense or prolonged, may cause bone death. This is due to the combined effects of damage to small blood vessels, marrow cells and bone cells. Such changes, which are dose-related, often occurred in the past when low-energy radiation was in use. Nowadays, with megavoltage apparatus and more sophisticated planning techniques, longterm bone damage is much less likely; patients who present with osteonecrosis are usually those who were treated some years ago. Areas affected are mainly the shoulder and ribs (after external irradiation for breast cancer), the sacrum, pelvis and hip (after irradiation of pelvic lesions) and the jaws (after treatment of tumours around the head and neck). GENERAL ORTHOPAEDICS 112

6 (a) (b) (c) (d) 6.10 Gauchers disease (a) Gaucher deposits are seen throughout the femur. The cortices are thin and there is osteonecrosis of the femoral head. (b) Bone infarction is seen in the distal end of the tibia and the talus. (c) The typical Erlenmeyer flask appearance is seen in the x-ray of this teenager. (d) Ten years later the bone changes are much more marked, the cortices are extremely thin and the patient has obviously suffered a pathological fracture. 6.11 Radiation necrosis x-rays This patient received radiation therapy for carcinoma of the bladder. One year later he developed pain in the left hip and x-ray showed (a) a fracture of the acetabulum. Diagnosis of radiation necrosis was confirmed when (b) the fracture failed to heal and the joint crumbled. (a) (b) Pathology Unlike the common forms of ischaemic necrosis, which always involve subchondral bone, radiation necrosis is more diffuse and the effects more variable. Marrow and bone cells die, but for months or even years there may be no structural change in the bone. Gradually, however, stress fractures appear and may result in widespread bone destruction. A striking feature is the absence of repair and remodelling. The surrounding bone is usually osteoporotic; in the jaw, infection may follow tooth extraction. Clinical features The patient usually presents with pain around the shoulder, the hip, the sacrum or the pubic symphysis. There will always be a history of previous treatment by ionizing radiation, though this may not come to light unless appropriate questions are asked. There may be local signs of irradiation, such as skin pigmentation, and the area is usually tender. Movements in the nearby joint are restricted. General examination may reveal scars or other evidence of the original lesion. X-rays show areas of bone destruction and patchy sclerosis; in the hip there may be an unsuspected fracture of the acetabulum or femoral neck, or collapse of the femoral head. Treatment

Treatment depends on the site of osteonecrosis, the quality of the surrounding bone and the life expectancy of the patient. If a large joint is involved (e.g. the hip), replacement arthroplasty may be considered; however, bone quality is often poor and there is a high risk of early implant loosening. Nevertheless, if pain cannot be adequately controlled, and if the patient has a reasonable life expectancy, joint replacement is justified.