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

• Published in: Spine (Philadelphia, Pa. 1976) Pa. 1976), 2005-09, Vol.30 (18), p.2051-2055
• Main Authors: MILBRANDT, Todd A, JOHNSTON, Charles E
• Format: Article
• Language: English
• Subjects: Adolescent; Biological and medical sciences; Braces; Cardiac Surgical Procedures; Cerebrospinal fluid. Meninges. Spinal cord; Child; Child, Preschool; Down Syndrome - complications; Equipment Failure; Female; Heart Defects, Congenital - complications; Heart Defects, Congenital - surgery; Humans; Incidence; Internal Fixators - adverse effects; Kyphosis - etiology; Male; Malformations of the nervous system; Medical sciences; Nervous system (semeiology, syndromes); Neurology; Orthopedic Procedures - adverse effects; Pseudarthrosis - etiology; Radiography; Retrospective Studies; Scoliosis - diagnostic imaging; Scoliosis - epidemiology; Scoliosis - etiology; Scoliosis - surgery; Surgical Wound Infection - etiology; Tumors of the nervous system. Phacomatoses
• ISSN: 0362-2436; 1528-1159
• DOI: 10.1097/01.brs.0000179100.54453.72

Retrospective review case series. To evaluate the incidence, patterns, and treatment of scoliosis in a Down syndrome population. 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. 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. 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 degrees (range 0 degrees-44 degrees), 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 junctional kyphosis, and 1 infection, for a 57% complication rate. 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.