FUNCTIONAL OUTCOMES IN CHILDREN WITH ARTHROGRYPOSIS

Background Arthrogryposis Multiplex Congenita is a rare congenital disorder associated with multiple musculoskeletal contractures which causes substantial morbidity. Knee involvement is commonly seen among children with arthrogryposis, with flexion contracture being the most frequent. The purpose of this study was to assess the effectiveness of orthopaedic procedures, namely distal femoral supracondylar extension osteotomy and/or Ilizarov external fixator, on the ambulation status of children with knee flexion contracture and whether any functional gains are maintained at the latest follow-up. Methods Fifteen patients were identified and their medical records reviewed. The mean age at their first surgery was 7.6 years (range, 2-16 years). The etiology for all patients was amyoplasia. The mean length of follow-up was 58 months (range, 12-117 months). Contractures were treated with femoral extension osteotomy (n=8), Ilizarov external fixator (n=2), or both (n=5). Results Pre-operatively, 11 patients were non-ambulatory, three patients were household ambulators, and one patient walked with orthoses in the community. There was an average of 1.5 knee surgeries done per patient. At the latest follow-up, nine patients were ambulatory with technical aids, two patients were household ambulators, one patient used a wheelchair but was independent for transfers, and three patients remained non-ambulatory. The mean flexion contracture prior to the first surgery was 62.8 ± 26.7 degrees. Post-operatively, the mean flexion contracture was 13.5 ± 16.4 degrees. At the latest follow-up, the mean flexion contracture was 33.8 ± 23.6 degrees. There were complications in three patients which included infected hardware, transient neurological compromise, and pressure sores, which eventually all resolved. Conclusion early and aggressive orthopaedic management of flexion contractures in children with arthrogryposis is supported by our findings, and may contribute to functional gains.