Treatment of wrist deformities in children with arthrogryposis multiplex congenita

Introduction: Treatment of wrist contractures in children with arthrogryposis multiplex congenita (AMC) is extremely problematic because of the high incidence of recurrence. This study aimed to improve the outcome of wrist contracture treatment in children with AMC.

Materials and Methods: A total of 90 patients (162 wrists) were examined and treated. Patients were assessed using a number of clinical, radiological, and electrophysiological examinations. There are several different clinical variants of wrist contracture, including flexion contracture of the wrist, flexion contracture associated with ulnar deviation, and isolated ulnar deviation of the wrist. Patients were divided into three groups according to the level of spinal cord lesion: С6-С7, С5-С8, and С5-Th1. As the number of damaged spinal cord segments increased, the amplitude of passive and active movements, degree of passive correction, muscle power, and wrist function decreased. Surgical treatment involved the following three approaches: tendon transfers, tendon transfers and carpal wedge osteotomy, and tendon transfers with carpal wedge osteotomy and shortened osteotomy of the forearm.

Results: Analysis of treatment results showed that patients with segmental spinal cord lesions at the  С6-С7 and С5-С8 level were mostly associated with a good outcome, whereas patients with lesions at the  С5-Th1 level achieved satisfactory outcomes.

Conclusions: Patients with segmental lesions of the spinal cord at the С6-С7 and С5-С8 level were associated with restoration of active wrist extension up to the neutral position or more and were expected to achieve significant improvement of hand function. Patients with spinal cord lesions at the C5-Th1 level exhibited significant lesions of the muscles, along with bone deformities. Consequently, surgical treatment could only achieve functional wrist position with minimal improvement of hand function. Using differential approaches in the treatment of wrist contracture that are selected by determining the level of spinal cord lesion will enable physicians to predict the outcome and improve the function and appearance of the wrist.