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Hip dysplasia in Hurler's syndrome: orthopaedic management after bone marrow transplantation
E L Masterson1, P G Murphy, A O'Meara
1Our Lady's Hospital for Sick Children, Dublin, Ireland.
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.
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.