Hip dysplasia in Hurler's syndrome: orthopaedic management after bone marrow transplantation

Abstract

Hurler's syndrome is an autosomal recessive metabolic storage disease with distinct skeletal manifestations, which include progressive hip dislocation. Enzyme-replacement therapy by bone marrow transplantation improves life expectancy but does not prevent hip dislocation. We describe the features of hip dysplasia in a series of eight successfully engrafted patients with Hurler's syndrome. The primary pathologic condition appears to be a failure of ossification of the cartilaginous acetabulum. Five patients underwent bilateral hip-containment surgery, and all operated-on hips were reduced at a mean follow-up of 17 months. Innominate osteotomy would appear to be an essential part of the surgical procedure.