Gaucher Disease (GD) is the commonest of the lysosomal storage disorders. Orthopaedic manifestations occur in 90% and include osteonecrosis of the femoral head, often producing severe disability at a young age. Historically, arthroplasty has been avoided in GD due to high reported rates of haemorrhage and decreased implant survival. The advent of enzyme replacement therapy (ERT) has revolutionised GD treatment with correction of haematological parameters within five months. However there is little data regarding the effect of ERT on the outcomes of hip arthroplasty. All patients on the Cambridge Gaucher register with a coding for hip replacement were included in the study. Demographic and operative data were recorded from the patient notes and radiographical analysis was conducted. Hip scores were obtained via telephone interview.Introduction
Materials and Method