Introduction: The lower limb deformities in relation to hip dysplasia and genu valgum seen in Hurler’s Syndrome are well recognised. Bone marrow transplantation has improved the survival of patients with Hurler’s Syndrome, reversing many of the clinical features associated with it. This is of increasing importance because the musculoskeletal manifestations do not appear to be affected.

Methods: Between 1990 and 2003, 18 patients have been successfully engrafted and have been followed up for a mean of 6.8 years (range 18 months to 15 years) at Royal Manchester Children’s Hospital. We describe the lower limb problems and their management in these patients. We report on their skeletal development following successful transplant. Radiographic analysis was done using the following measurements where possible – acetabular index, centre-edge angle, migration percentage, femoral neck-shaft angle and tibio-femoral shaft angle.

Results: Of the 18 patients, one has had bilateral staged shelf acetabuloplasty and bilateral staged medial epiphyseal stapling (MES) of the upper tibia. The second patient has had bilateral upper tibial MES.

Discussion: There is very little in the literature on the long-term natural history of the orthopaedic manifestations of Hurler’s Syndrome after bone marrow transplantation. Presently there is no consensus as to the best management of the lower limb problems in this disorder. Well conducted long-term follow up is essential.

The results of the Ferguson medial open reduction of the hip for DDH were reviewed to determine the complications, re-operation rate, clinical and radiological outcome. Notes were reviewed for 75 cases, of which 5 were bilateral. X-rays were available for 69 hips and were analysed for Acetabular index (AI) and Centre Edge (CE) angles of the operated and unaffected hips. The hips were assessed for avascular necrosis by the method of Kalamchi and MacEwan and were graded according to Severin.

The mean age at operation was 11.8 months (range 3-23, SD 4.42, mode 11). The mean clinical follow up was 65.1 months (range 4-148, SD 33.4). The mean radiological follow up was 58.2 months (range 3 – 131, SD 31). No further surgical procedure was required for 60 hips (75%). Of the remainder, a Salter osteotomy was performed for 8 hips, of which 6 had additional procedures. 8 hips required a femoral osteotomy, 2 an Arthrogram and one a triple pelvic osteotomy.

The AI improved following surgery, with a rate of increase double that for the unaffected side. The mean centre edge of the operated side was 6 degrees less than the unaffected side. Tables 2 and 3 show that the majority of hips had no avascular necrosis and a good radiological outcome. The results compare favourably with the literature. The conclusion is that the Ferguson medial open reduction has good long-term results with low rates of avascular necrosis.