Total hip replacement in Gaucher’s disease

Abstract

Total hip replacement in patients with Gaucher's disease with symptomatic osteonecrosis of the femoral head is controversial because of the high early failure rates. We describe four patients who had an uncemented total hip replacement following enzyme replacement therapy for a median of two years and one month (1 to 9.8 years) prior to surgery, and who remained on treatment. At operation, the bone had a normal appearance and consistency. Histopathological examination showed that, compared with previous biopsies of untreated Gaucher's disease, the Gaucher cell infiltrate had decreased progressively with therapy, being replaced by normal adipose tissue. The surfaces of viable bone beyond the osteonecrotic areas showed osteoblasts, indicating remodelling. In one case acetabular revision was carried out after 11 years and eight months. The three remaining patients had a mean follow-up of six years and four months (3.3 to 12 years). We recommend initiating enzyme replacement therapy at least one to two years prior to total hip replacement to facilitate bone remodelling and to allow implantation of uncemented components in these young patients.

The Pathogenesis of Nontraumatic Osteonecrosis

“…Some of the well accepted and common associations include corticosteroid use [8, 9], alcohol abuse [10], systemic lupus erythematous [11], hemoglobinopathies including sickle cell anemia [12–14], Legg-Calve Perthes disease [15], and exposure to radiation [16] or cytotoxic agents [17]. Other less common associations include Gaucher's disease [11, 18, 19], dysbarisms [4], HIV [20], hyperlipidemia [21], pancreatitis, and gout [22]. A substantial number of cases have no identifiable etiologic factors and have been referred as idiopathic [23].…”