Spinal deformities in patients with arthrogryposis. A review of 16 patients.

Abstract

Sixteen patients with arthrogryposis having spinal deformities were reviewed. The age of diagnosis of scoliosis was from birth to 15 years. Many different curve patterns were seen. Five patients had decreased anterior-posterior thoracic diameter associated with decreasing vital capacity. One of them died at age 6 months of bronchopneumonia and one other at 16 years from cardiac arrest during surgery elsewhere. Eight patients had nonoperative treatment with an orthosis, and of these, three are still under orthotic care. For the remaining five, one had improved at the end of the treatment, and four had increased curvatures in the orthosis. Six patients underwent surgery, four from the group treated by bracing, and two having surgery as the primary treatment at this Center. One patient had posterior fusion without instrumentation, four had posterior fusion with instrumentation, and one had posterior instrumentation without fusion. Spinal problems can occur in arthrogryposis patients, are poorly controlled nonoperatively, and frequently require surgical treatment, especially if there is thoracic lordosis.