Treatment of spinal deformities in patients with diastrophic dysplasia: a long-term, population based, retrospective outcome study.

Study Design: A long-term, population based, retrospective follow-up study.

Objective: To evaluate long-term outcomes of brace and surgical treatment for spinal deformities in patients with diastrophic dysplasia (DD).

Summary Of Background Data: Literature on the brace treatment and surgery of spinal deformities in patients with DD is limited.

Methods: All patients with DD undergoing either brace treatment or surgery for spinal deformity with a minimum of 2 years follow-up were identified in our country. Eight patients had undergone brace treatment and 12 had been treated operatively. Two patients had early progressive and the rest idiopathic-like scoliosis. Five patients underwent posterior only, 1 anterior only, and 6 anteroposterior surgery. Patients' mean age at the beginning of brace treatment was 6.9 (range, 0.9-12.7) years and at the time of surgery 13.4 (range, 6.5-20.1) years. The follow-up time averaged 17 (range, 6.6-44.3) years for the brace and 14.0 (range, 2.1-37.2) years for the surgical treatment group. The radiographic follow-up rate was 100%.

Results: Both thoracic and lumbar curves progressed during brace treatment (mean major curve progression 12%, range, -43%-53%). Before surgery, the mean Cobb angle of the thoracic curve was 68 degrees (range, 42 degrees-100 degrees) and 46 degrees (25 degrees-68 degrees) in the lumbar spine. At final follow-up visit, the mean correction was 23% (-6%-76%) for the thoracic curve and 25% (-68%-82%) for the lumbar curve. The correction of the major curve was higher in patients undergoing anteroposterior versus posterior only (40% vs. 13%, P = 0.017). Five (42%) operated patients had significant complications. The SRS-24 yielded 92 (79-103) points for the brace treatment and 93 (73-114) points for the surgical group, respectively.

Conclusion: Brace treatment does not prevent progression of the spinal deformity in patients with DD. Anteroposterior surgery is indicated in patients with severe spinal deformities. The risk for major complications is high especially in patients with marked kyphosis.