Mortality in Neuromuscular Early Onset Scoliosis Following Spinal Deformity Surgery

Background: The purpose of this study was to report mortality and associated risk factors in neuromuscular early onset scoliosis following spinal deformity surgery. Methods: This is a multicenter retrospective cohort study of patients with cerebral palsy (CP), spinal muscular atrophy, myelodysplasia, muscular dystrophy, or myopathy undergoing index spine surgery from 1994 to 2020. Mortality risk was calculated up to 10 years postoperatively. Proportional hazard modeling was utilized to investigate associations between risk factors and mortality rate. Results: A total of 808 patients [mean age 7.7 y; 439 (54.3%) female] were identified. Postoperative 30-day, 90-day, and 120-day mortality was 0%, 0.001%, and 0.01%, respectively. 1-year, 2-year, 5-year, and 10-year mortality was 0.5%, 1.1%, 5.4%, and 17.4%, respectively. Factors associated with increased mortality rate: CP diagnosis [hazard ratio (HR): 3.14, 95% confidence interval (CI): 1.71; 5.79, P<0.001]; nonambulatory status (HR: 3.01, 95% CI: 1.06; 8.5, P=0.04)]; need for respiratory assistance (HR: 2.17, 95% CI: 1.00; 4.69, P=0.05). Conclusions: In neuromuscular patients with early onset scoliosis, mortality risk at 10 years following spine surgery was 17.4%. As mortality was 1.1% at 2 years, premature death was unlikely a direct result of spine surgery. Diagnosis (CP) and markers of disease severity (nonambulatory status, respiratory assistance) were associated with increased mortality rate. Level of Evidence: Prognostic level II.