Bone complications

The chronic haemolytic process results in expansion of the medullary space, although the resultant bony changes are less pronounced than in thalassaemia. Bone infarction due to vaso-occlusion produces tenderness, warmth and swelling, which can be difficult to distinguish from osteomyelitis. In such cases, cultures from blood and direct aspiration are negative and radiographs later show patchy sclerosis and cortical thickening. Collapse of vertebral end plates due to infarction produces the codfish appearance. Patients are managed with analgesia and hydration until resolution of symptoms.

Avascular necrosis of the femoral head is a serious complication that is difficult to treat and leads to chronic disability and pain (Figure 7.4d). Patients with coexisting a-thalassaemia have a higher incidence of osteonecrosis at a younger age. The condition also affects the humeral head but with less functional consequences. The outcome is better in young patients with immature capital epiphysis, who should be treated with analgesics and avoidance of weight bearing for 3-6 months. In older adolescents and adults the condition is more likely to progress to degenerative arthritis with conservative management. Core decompression and osteotomy have been tried, but hip arthroplasty is required for patients with severe symptoms.

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