The natural history of osteonecrosis of the femoral head in children and adolescents who have Gaucher disease.

Abstract

We reviewed the cases of eight patients (thirteen hips) with Gaucher disease who had had osteonecrosis of the femoral head and had been managed with bed rest and non-weight-bearing with crutches only in the symptomatic stage of the bone crisis. The mean age of the patients at the onset of the first crisis in each hip was ten years (range, six to fourteen years). The mean age at the most recent follow-up examination for the six living patients (ten hips) was twenty-three years (range, nineteen to thirty-three years). The Mose rating was good for one hip, fair for two, and poor for seven. Despite the over-all unfavorable radiographic ratings, the six patients were asymptomatic and did not need assistance with daily activities. There is no known treatment that effectively prevents the development of deformities of the femoral head. Thus, we recommend symptomatic management for osteonecrosis of the femoral head in Gaucher disease with bed rest and analgesics followed by non-weight-bearing on the involved limb, if it makes the patient more comfortable, during the symptomatic stage of bone crisis.