OBJECTIVE: To evaluate the outcome of surgical treatment of scoliosis in patients with spinal muscular atrophy (SMA) type 2. METHODS: A retrospective study with 12 patients with SMA type 2 who underwent arthrodesis and instrumentation for scoliosis correction with more than two years of follow-up. The degree and rate of correction of deformity and pelvic obliquity postoperatively and loss in the last evaluation were evaluated, in addition to the complications and the impact of treatment on respiratory function. RESULTS: Mean follow-up was 77.5 months (6.4 years) ± 58.9 months (4.9 years), Cobb angle before surgery averaged 76.1° ± 31.7° (35° to 144°) and postoperatively was 29.5° ± 23.2° (5° to 90°), with correction average of 46.6° (61.29%). The average pelvic obliquity preoperatively was 15.1° ± 13.3° (range 0° to 37°), and 8.5° ± 9.9° (range 0° to 30°) postoperatively, with an average correction of 6.5° (43.37%). Five patients had complications (41.6%). The forced ventilatory capacity (FVC) preoperative average was 62.9% ± 38.6% (range 23.3% to 89%) and 45.9% ± 25.0% (range 15% to 86.2%) in the last evaluation. The decline was 17% of vital capacity, with a reduction of 2.4% per year of follow-up. CONCLUSIONS: Surgical treatment of scoliosis in patients with SMA allows the correction of pelvic obliquity and restoration of the sagittal and coronal balance freeing the hands for activities of daily living. Lung function was positively affected by treatment.
Muscular atrophy, spinal; Scoliosis; Arthrodesis; Spinal fusion; Respiratory function tests
