PURPOSE: 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. MATERIAL AND METHODS: 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. RESULTS: 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). CONCLUSION: Early diagnosis, assisted by computed tomography, magnetic resonance imaging or scintigraphy, makes it possible to provide better treatment and preserve hip function.
PURPOSE: 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. MATERIAL AND METHODS: 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. RESULTS: 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). CONCLUSION: Early diagnosis, assisted by computed tomography, magnetic resonance imaging or scintigraphy, makes it possible to provide better treatment and preserve hip function.