We report the natural course of the hip in fifty-two patients
(ninety-five hips) who had sickle-cell disease and had had avascular
necrosis in childhood. There were twenty-one African, twenty-one West
Indian, and ten Mediterranean patients. At the most recent follow-up
examination (at an average duration of nineteen years after the onset of
the disease), 80 per cent of the hips that had been affected by avascular
necrosis during childhood were painful and had permanent damage with regard
to decreased mobility, limb-length discrepancy, and an abnormal gait. When
the patients were evaluated, at an average age of thirty-one years, fifteen
hips (16 per cent) had had an operation for progressive disability and
sixty (63 per cent) had major problems because of pain. Of the twenty hips
(21 per cent) that were not painful, five were in patients who had an
abnormal gait, with decreased agility. The mean Iowa hip-rating score at
the most recent follow-up examination was 73 points (range, 30 to 100
points). Correlations were found between the hip score and the patient's
age at the onset of the disease and at the latest follow-up, between the
hip score and degenerative changes in the hip, and between degenerative
changes and radiographic evidence of deformity of the hip.