Down syndrome and scoliosis: A review of a 50-year experience at one institution

Study Design. Retrospective review case series. Objective. To evaluate the incidence, patterns, and treatment of scoliosis in a Down syndrome population. Summary of Background Data. Despite a preponderance of literature concerning cervical abnormalities in Down syndrome, there is little information concerning scoliosis in this patient group. We examined the 50-year history of treating scoliosis at our institution in patients with Down syndrome. Methods. Following institutional review board approval, chart review identified patients with Down syndrome with scoliosis. We performed a radiographic review of curve pattern, and determined results of brace and operative treatment. Results. A total of 379 patients were identified as having Down syndrome. There were 33 patients diagnosed with scoliosis, for an incidence of 8.7%. Mean follow-up was 4.15 years (range 0-12). The double major curve predominated with 18 (55%). Of 33 patients, 16 (49.5%) had previously undergone thoracotomy for congenital heart defects. There were 8 (24%) patients who were braced for an average of 26.5 months (range 12-63), with an average progression in brace of 10° (range 00-44°), 3 of whom went on to spinal fusion. There were 7 (21.2%) patients who underwent spinal fusion, including 6 posterior spinal fusions and 1 anterior spinal fusion. Four patients had complications, including 3 pseudarthroses, 4 implant failures, 3 superior junctionai kyphosis, and 1 infection, for a 57% complication rate. Conclusions. Scoliosis developed in 8.7% of patients with Down syndrome. There was a high rate of cardiac surgery within this population. Bracing was ineffective for the majority of the patients treated. Although surgery has a high rate of complications, there was only one patient who underwent reoperation.