Abstract
Purpose: To review the orthopaedic manifestations and document the results of surgical intervention. Material and Methods: A review of all 22 children currently attending a specialist scleroderma clinic was performed. Disease extent was measured in terms of percentage body surface area (BSA) affected and all orthopaedic abnormalities were documented. The outcome of surgical intervention was evaluated.
Results: All children presented by the age of 12 and all but 2 had developed joint contractures of either the lower or upper limbs affecting function within 2yrs of diagnosis. Overall, lower limbs were more commonly affected than upper. Abdominal scleroderma led to a scoliosis in 75% of cases. The mean BSA affected was 35% (range 5-65%) with contractures more related to site of disease rather than extent. Pain was associated with lower limb contractures and loss of function with hand contractures. Limb length discrepancy (LLD) was common with a mean of 3cms (range 2-6.5cms). 8 children have had surgery. 7 developed wound healing problems. 50% of operations failed to correct the deformity and in a further 25% relapse has occurred. In the remaining 2 cases a good result was achieved. In addition, one epiphysiodesis has been performed and 3 are planned.
Conclusions: This is the largest known review of children with linear scleroderma. Joint contractures are common but poorly managed by conservative methods alone. Surgical intervention is difficult but early defini-tive treatment is recommended with subsequent aggressive splinting during growth whilst the disease is active. LLD must be corrected.
Local Host: British Society for Children’s Orthopaedic Surgery. Conference Theme: Congenital Deficiencies of the Lower Limb. These abstracts were prepared by A.Catterall.
