Results And Complications Following Surgical Treatment of Scoliosis Associated with Spinal Muscular Atrophy: The Experience at One Institution

Methods: Retrospective review of all patients with SMA having undergone surgical correction of their spinal deformity at one institution. All patients underwent posterior spinal fusion performed by the same surgeon. Thirty-six patients underwent a complementary anterior spinal fusion. Chart review, pre-op and latest greater than 2 year post-op radiographs, pulmonary function tests and anesthesia records were reviewed for all patients.

Results: Complete data was available for 78 of 84 patients. Mean pre-operative and post-operative radiographic measures can be found in table 1. Mean anesthesia time was 481 (range 350-780) and mean operative time was 402 minutes (range 280-660). When anterior surgery was performed, mean operative time was 203 minutes (range 80-420). Blood loss averaged 1498 cc's (range 236-4297). There were 10 (12%) post-operative infections of which 7 (8%) were considered deep infections requiring surgical debridement. Other complications included atelectasis(5), tibia fracture (autograft donor site in 3), pneumonia (3), ARDS (1), tracheal stenosis (1), tension pneumothorax and death (1). A total of 49 complications were found in 35 patients (41%).

Discussion: Scoliosis associated with SMA remains a challenging clinical problem. Although the rate of complication is high, all but one patient survived the surgery with improved coronal, sagittal and pelvic balance while maintaining their vital capacity. Patients undergoing surgery and their relatives should be made aware of the risks associated with the complex nature of the disease.

Conclusion: Patients with SMA should be overseen by an experienced multidisciplinary team with knowledge of the pre-operative and post-operative management.