• Lauren E Stone, Michael P Kelly, Madison Alexander, Michael Brandel, Sandi K Lam, and Vijay M Ravindra.
• Department of Neurosurgery, University of California San Diego, San Diego, CA.
• Spine. 2023 Dec 15; 48 (24): E409E416E409-E416.

Study DesignRetrospective database cohort study.ObjectiveTo evaluate U.S. treatment trends and inpatient outcomes for children undergoing posterior spinal fusion (PSF) for Rett syndrome (RTT)-associated scoliosis (RAS).Summary Of Background DataRTT is a rare, sporadic neurodevelopmental disorder presenting in childhood with developmental regression, ataxia, and seizures. RAS occurs in 50% to 80% of cases of RTT, but little is known about the case volume and perioperative experience for children undergoing PSF.Materials And MethodsUsing the International Classification of Diseases Ninth and 10th revision codes in the national Kids' Inpatient Database, we identified children with RTT who underwent PSF from 2000 to 2019. Annual case volumes were analyzed. Clinical characteristics and outcomes were compared with those of a cohort of patients with neuromuscular scoliosis (NMS).ResultsAmong 220 patients with RAS, 216 (98.2%) were females (mean age at surgery: 12.3±3.3 yr). Surgical case incidence steadily increased over 19 years, with more RAS admissions in the South (31.4%). Overall, patients with RAS demonstrated a higher mean Elixhauser Comorbidity Index score (2 vs . 1, P < 0.001) and had more perioperative complications (41.4% vs . 18%, P < 0.001) than patients with NMS. RTT diagnosis independently predicted higher odds of any complications (odds ratio: 1.98, P < 0.001) and increased length of stay (odds ratio: 1.18, P = 0.009) for admissions for PSF.ConclusionsSurgical treatment for RAS is rare but increased over a 19-year period. Cases appear to be clustering by region, with the highest proportion in the South. The higher Elixhauser Comorbidity Index in RAS patients predicted higher costs, longer hospital stays, more complications (particularly respiratory), and more nonroutine discharge disposition than in other patients with NMS. RTT was independently associated with higher odds of complications and longer length of stay. Because RAS cases appear to be increasing in number, future studies should emphasize methods to reduce morbidity and investigate deformity-specific metrics to help better understand this population.Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.

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