Abstract

To identify the epidemiological, clinical and therapeutic aspects of hip-joint disorders in children with sickle cell disease, to point out the diagnostic problems, and to stress the necessity of early diagnosis for optimal outcome. This retrospective study, conducted from January 1987 through December 2006, included children with at least one haemoglobin S gene and hospitalised for osteonecrosis of the femoral head. Ficat staging was used. The study included 14 children (12 boys and 2 girls) with a mean age of 14 years, all hospitalised and treated for osteonecrosis of the femoral head during the study period 8 SS and 6 SC. Osteonecrosis of the femoral head was diagnosed at Ficat stage 3 in 8 cases and at stage 4 in 6. Ten children (8 at stage 3 and 2 at stage 4) had orthopaedic treatment (continuous traction for 30 days and then a Thomas's splint for a mean 14 months). Outcome was good for 6 of the stage-3 cases (mean follow-up period 9 years) and poor for the other patients with purely orthopaedic treatment (mean follow-up period 11 years). Four children at stage 4 underwent surgery (varus osteotomy, immobilization in a cast for 8 weeks, and then Thomas's splint for a mean 12 months) with good results (mean follow-up period 10 years). Early diagnosis, assisted by computed tomography, magnetic resonance imaging or scintigraphy, makes it possible to provide better treatment and preserve hip function.

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