Total hip replacement in patients with Gaucher’s disease with symptomatic osteonecrosis of the femoral head is controversial because of the high early failure rates. We describe four patients who had an uncemented total hip replacement following enzyme replacement therapy for a median of two years and one month (1 to 9.8 years) prior to surgery, and who remained on treatment. At operation, the bone had a normal appearance and consistency. Histopathological examination showed that, compared with previous biopsies of untreated Gaucher’s disease, the Gaucher cell infiltrate had decreased progressively with therapy, being replaced by normal adipose tissue. The surfaces of viable bone beyond the osteonecrotic areas showed osteoblasts, indicating remodelling. In one case acetabular revision was carried out after 11 years and eight months. The three remaining patients had a mean follow-up of six years and four months (3.3 to 12 years). We recommend initiating enzyme replacement therapy at least one to two years prior to total hip replacement to facilitate bone remodelling and to allow implantation of uncemented components in these young patients.
We undertook a prospective, single blinded, randomized, controlled trial of one hundred children treated with either an above or below elbow cast for treatment of closed, distal third forearm fractures requiring reduction. The re-manipulation rate in the below elbow group was 2% (95%CI: 0–11%) compared to 6% (95%CI: 2–15%) in the above elbow group, p=0.62. Above elbow casts do not appear to improve fracture immobilization nor reduce the requirement for re-manipulation in pediatric distal third forearm fractures. Debate exists regarding the benefits of using below elbow casts instead of above elbow casts for maintaining reduction in pediatric distal third forearm fractures. The literature indicates a loss of reduction rate of 14.6% of children treated in an above elbow cast and 2.5% in those treated with a below elbow cast. We undertook a prospective, single blinded, randomized, controlled trial of one hundred children treated with either an above or below elbow cast for treatment of closed, distal third forearm fractures requiring reduction. Outcome measures included re-manipulation rate, fracture displacement during cast wear, and cast complications. One hundred patients were suitably enrolled; fifty-four received an above elbow cast, forty-six received a below elbow cast. The two groups were similar in terms of age and gender. The above elbow group contained a higher proportion of both bone fractures (41/54) than the below elbow group (27/46). There were no significant differences between the two cast groups in initial, post-reduction or cast-off fracture angulation; nor any difference in the amount of fracture displacement during cast wear. The number of cast complications was similar between the two groups. The re-manipulation rate in the below elbow group was 2% (95%CI: 0–11%) compared to 6% (95%CI: 2–15%) in the above elbow group, p=0.62. Above elbow casts do not appear to improve fracture immobilization nor reduce the requirement for re-manipulation in pediatric distal third forearm fractures.