Down’s Syndrome and Scoliosis: A Review of a 60-year Experience at One Institution

Purpose: Despite a preponderance of literature concerning cervical abnormalities in Down’s syndrome (Trisomy 21), there is little information concerning the incidence, curve patterns, and treatment of scoliosis in this patient group. We examined the 60-year history of treating scoliosis at our institution in patients with Down’s syndrome.

Methods: Following IRB approval, chart review identified Down’s patients with “idiopathic” scoliosis, nonidiopathic causes being excluded. Demographic data, time of follow-up, brace treatment, operative treatment, and complications were determined. We performed radiographic review of curve pattern and magnitude, and determined results of brace and operative treatment.

Results: 379 patients were identified as having Down’s syndrome. 33 were diagnosed with idiopathic scoliosis for an incidence of 8.7%. There were 9 males and 24 females. Mean age at diagnosis of scoliosis was 11.7 years (4-16 years) and 32 of 33 were ambulatory. Mean follow-up was 3.8 years (0-12 years). Those with less than two-year follow-up were excluded from progression analysis. There were 7 (21.2%) single thoracic curves, 2 (6.1%) double thoracic curves, 18 (55 %) double major curves, 1 (3%) thoracolumbar curve, and 6 (18.2%) lumbar curves. The initial curve magnitude was 29 degrees (10-59 degrees). 16 of 33 (49.5%) patients had previously undergone thoracotomy for congenital heart defects.

Brace treatment: 8 (24%) patients were braced for an average of 26.5 months (12-63 months) with average progression in brace of 10 (0-44) degrees, two of whom went onto spinal fusion. The other 6 patients, 5 of whom were Risser O at prescription, reached maturity with an average progression of 5.6 degrees (20%) in the thoracic spine and 7.3 degrees (23%) in the lumbar at final followup.

Surgical treatment: 7 (21.2%) patients underwent spinal fusion (6 PSF and 1 ASF). The mean number of segments fused was 11.3 with all but 2 extending below L2. Only one patient required time in the intensive care unit post operatively. The immediate post-operative correction was 51% (24-68%) in the thoracic curve and 45% (32-55%) in the lumbar curve, with loss of correction at f/u to 40% (10-61%) thoracic and 29% (- 10-50%) lumbar. Four patients sustained complications (3 pseudoarthrosis, 4 implant failures, and 1 infection) for a 57% complication rate. Only one patient required re-operation for complication management. The others were either non-symptomatic or had become too ill for surgical intervention. The SSEP monitoring was effective in all cases used.

Conclusions: 8.7% of Down’s syndrome patients develop significant idiopathic-like scoliosis perhaps due, in part, to the high rate of cardiac surgery. Bracing was effective for the majority of the patients treated. The 57% complication rate was similar to what has been previously described for cervical fusions in this population.

Significance: Patients with Down’s syndrome are carefully scrutinized for cervical abnormalities, however scoliosis also has a significant incidence. A high rate of surgical complications can be seen in this population.