Aseptic necrosis of the femoral head in sickle-cell disease

Aseptic necrosis of the femoral head accounted for 75 (28.2%) of the 266 major skeletal complications seen in 207 patients with sickle-cell disease in a 66-month period. Forty-five (60%) of the 75 patients were males. The onset of symptoms occurred between the ages of 10 and 29 years in 60 (80.0%) of the patients, and the mean age at onset was 20.8 (range 8-54) years. There were 37 patients with sickle-cell anaemia (SS) with 46 hips affected by necrosis, and 38 patients with sickle-cell haemoglobin C with 40 affected hips. Perthes-like changes occurred in 40 hips, osteochondritis dissecans-like lesion in one hip and severe hip deformity in 45 hips. Four of the five hips with Perthes-like necrosis which were treated by rotation upper femoral osteotomy had partial reconstitution of the femoral head, and all five were symptom-free. The other hips were treated conservatively with generally poor results.