Hip dysplasia in Hurler's syndrome: orthopaedic management after bone marrow transplantation.
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.
Our Lady's Hospital for Sick Children, Dublin, Ireland.
E L
EL
Masterson
P G
PG
Murphy
A
A
O'Meara
D P
DP
Moore
F E
FE
Dowling
E E
EE
Fogarty
D001175:Arthrography; D016026:Bone Marrow Transplantation; D002648:Child; D002675:Child, Preschool; D005260:Female; D006617:Hip Dislocation / Q000209:etiology / Q000473:pathology / Q000601:surgery*; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D008059:Mucopolysaccharidosis I / Q000150:complications / Q000601:surgery* / Q000628:therapy*; D010027:Osteotomy / Q000379:methods