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Down Syndrome Abstract
of the Month: Dec 2005

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

Milbrandt TA, Johnston CE
Spine. 2005 Sep 15;30(18):2051-5

Department of Orthopaedics, University of Maryland, Baltimore, MD, USA

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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 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. 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.

My comments:

For the purpose of this study, scoliosis was defined as the presence of a curve more than 10° on X-rays.

These kind of studies are useful in determining how often a problem occurs and to what severity within the DS population. The difficulty is when looking at treatment modes and outcomes, as surgical procedures have changed over the last 50 years. In the discussion, the authors note that out of 8 patients, bracing only prevented one patient from having progression of the scoliosis, which produces little hope with avoiding surgery. The other important finding here is that the most frequent complication is the pullout of the distal fixation (where the rod is fixed to the lowest vertebra). The authors speculate that this could be due to patients with DS being unccoperative with postsurgical restrictions on activity, such as bending over and lifting. The authors go on to mention that "pedicle screw fixation" may be a better way to keep the bottom part of the hardware attached to the spine.
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