The Journal of Bone and Joint Surgery, Vol 73, Issue 1 81-92, Copyright © 1991 by Journal of Bone and Joint Surgery, Inc
Deformities of the hip in adults who have sickle-cell disease and had avascular necrosis in childhood. A natural history of fifty-two patients
P Hernigou, F Galacteros, D Bachir and D Goutallier
Department of Orthopaedic Surgery, Hopital Henri Mondor, Creteil, France.
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.
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