Scoliosis associated with arthrogryposis multiplex congenital: clinical characteristics and surgical outcomes.

Abstract

Objective To evaluate the clinical characteristics and surgical outcomes of the scoliosis associated with arthrogryposis multiplex congenital (AMC). Methods This retrospective study included 14 cases (7 males and 7 females; average age, 14.3 years) with scoliosis associated with AMC from November 2001 to August 2006. The curve patterns and Cobb angles in the coronal and sagittal plane, and pelvic obliquity were evaluated before and after spinal surgery respectively. Results A long-C shaped thoracolum-bar curve was seen in 12 cases, and 5 cases were associated with congenital scoliosis. The mean Cobb angle of all the cases in the coronal plane improved from 79.1° to 35.9°. The mean correction rate was 54.6%. The mean Cobb angle of the thoracic lordosis in 10 cases was 43.0° preoperatively, and was corrected to thoracic kyphosis with a mean Cobb angle of 16.4°postoperatively. The mean Cobb angle of the thoracic kyphosis in 2 cases was improved from 91.5° to 54.5°. The mean pelvic obliquity in 3 cases was reduced from 22.1° to 13.3°. During a mean 29-month follow-up, the average loss of the frontal correction was 6.8%. Complications occurred in three cases: massive blood loss (4000 ml) in one case, a transient dyspnea in one case, and com-plete paraplegia after posterior correction with instrumentation in another case (recovered 3 months after op-eration). Conclusion A long-C shaped thoracolumbar curve, which is typical in scoliosis associated with paralytic neuromuscular disorders, is the most common pattern in scoliosis with AMC. And congenital verte-bral anomalies may also be found in AMC. Satisfactory correction can be achieved through three-dimension spinal correction, yet may result in perioperative complications. Key words: Arthrogryposis; Scoliosis; Treatment outcome