Introduction: Children with neuromuscular scoliosis (NMS) are often indicated for long-segment thoracopelvic posterior spinal fusion, which carries significant risks, particularly in NMS population where patients have multiple medical co-morbidities. Long-term follow-up is needed to understand potential complications and outcomes in these vulnerable patients. We present our findings evaluating long-term outcomes following long-segment fusion in a pediatric NMS cohort.
Methods: At a single, high-volume pediatric hospital, we conducted a retrospective review of patients ( < 18y.o.) with diagnosis of NMS from 2015-2020 who underwent >13 level instrumentation and arthrodesis (CPT 22844/22804) with at least 18 months of follow-up.
Results: One-hundred-nineteen patients (13.53 +/- 2.8 y.o. at surgery) met inclusion criteria. Average length of follow up is 3.86 +/- 1.85 years. Of these, 64 (53.78%) UMN etiology, 12 (10.08%) have LMN, and 45 (37.81%) have other NMS etiology. The average number of spinal levels fused is 17.18 +/- 3.22. Length of hospital stay averaged 10.48 +/- 11.51 days with 64.71% patients requiring the ICU. The mortality at 90-days, 1-year, 2-years, and 5-years was 0.84%, 3.36%, 5.04%, and 9.24%, respectively, with cardiopulmonary failure the cause of death in 91.67%. Reoperation rate was 21.01%. Of these, 32% for wound infection, 32% for hardware failure, 8% for progression of scoliosis, 16% due to intraoperative complications, and 12% planned second operations. The average time to re-operation after index procedure is 1.28 +/- 0.61 years. There was no correlation between NMS etiology and mortality or reoperation rate on multivariate analysis.
Conclusion : Long-segment fusion in patients with NMS is associated with relatively long hospital stays and ICU requirements. While re-operation is not uncommon, surgery-related mortality is low. This research shows that most mortality is likely due to comorbidities related to the natural history of underlying NMS etiologies. Future directions should investigate exact contributors to mortality and complications and define changes in quality-of-life metrics.
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