Bone disorders in sickle-cell disease

Sickle cell disease involves many organs but musculo-skeletal problems present most often with bone pain, the most common reason for admission to hospital. Two separate pathological abnormalities cause these lesions. Sickling of the red cells produces thromboembolic infarcts in bone leading to pain, crises and sometimes osteomyelitis; increased destruction of sickle red cells produces haemolysis, an increase in erythroblastic activity and expansion of the bone marrow cavity. Dactylitis, avascular necrosis of the head of the femur particularly, osteomyelitis, retardation of growth and leg ulcers are commonly encountered Management is by standard orthopaedic principals. At operation care must be taken in the use of a tourniquet, adequate oxygenation is required and the possibility of acute renal failure must be recognised.