Assessment of Efficacy and Safety of Z-Shaped Skin Flap, Posterior Release Combined with External Fixation for Severe Arthrogryposis Multiplex Congenita and Flexion Deformity

Objective: To evaluate the efficacy and safety of Z-shaped skin flap, posterior release combined with external fixation for children diagnosed with severe arthrogryposis multiplex congenita and flexion deformity. Methods: Five children (10 knees) with AMC complicated with severe flexion contracture of >40 degrees were enrolled and subject to posterior Z-shaped flap, posterior release combined with external fixator technique. After the operation, external fixation bracket was used to fix the knee joint. Results: The degree of average knee extension was increased up to 25.5 degrees after the soft tissue release. The knee joint could be completely straightened after using wearing the external fixator for 12.3 d on average. No distal femur osteotomy was performed. The average time of wearing the external fixator was 43.1 d. During an average of 19.2-month follow-up, children could independently walk in the community area and 1 case could walk at home. No child recurred during postoperative follow-up. Conclusion: The combined technique of Z-shaped skin flap, posterior release and external fixation is an efficacious and safe for children diagnosed with severe arthrogryposis multiplex congenita and flexion deformity. During the short-term follow-up, the time of external fixator wear is significantly shortened, the incidence of postoperative complications is lowered and the recurrence rate is equally reduced.