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
From a global point of view, chronic haematogenous osteomyelitis in children remains a major cause of musculoskeletal morbidity. We have reviewed the literature with the aim of estimating the scale of the problem and summarising the existing research, including that from our institution. We have highlighted areas where well-conducted research might improve our understanding of this condition and its treatment.
We have observed that some patients perceive their LLD to be much greater than the true LLD. A large LLD is sometimes reported by therapists, despite only a small true LLD. We have found that abduction tightness is a potent cause of apparent LLD, and report our investigations into this phenomenon.
Clinical photographs and videos have been produced to demonstrate this phenomenon. A 2-dimensional model has been made to demonstrate how the degree of abduction, offset and over-lengthening affect this phenomenon. A computer model has been used to quantify these effects.
Even with only minor abductor tightness, increasing the true length will disproportionately increase the apparent LLD. In the presence of tight abductors, increasing the offset will cause apparent shortening in the contra-lateral limb. Patients are who have adequate adduction are frequently unaware of true lengthening.