DOI: 10.1097/brs.0000000000004802 ISSN:

Rett Syndrome–Associated Scoliosis

Lauren E. Stone, Michael P. Kelly, Madison Alexander, Michael Brandel, Sandi K. Lam, Vijay M. Ravindra
  • Neurology (clinical)
  • Orthopedics and Sports Medicine

Study Design.

Retrospective database cohort study.


To 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 Data.

RTT is a rare, sporadic neurodevelopmental disorder presenting in childhood with developmental regression, ataxia, and seizures. RAS occurs in 50-80% of cases of RTT, but little is known about the case volume and perioperative experience for children undergoing PSF.


Using International Classification of Diseases (ICD)-9 and ICD-10 codes in the national Kids’ Inpatient Database, we identified children with RTT who underwent PSF in 2000-2019. Annual case volumes were analyzed. Clinical characteristics and outcomes were compared with those of a cohort of patients with neuromuscular scoliosis (NMS).


Among 220 patients with RAS, 216 (98.2%) were female (mean age at surgery 12.3±3.3 y). 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 (OR 1.98, P<0.001) and increased length of stay (OR 1.18, P=0.009) for admissions for PSF.


Surgical 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 cost, longer hospital stay, more complications (particularly respiratory), and more nonroutine discharge disposition than in other NMS patients. RTT was independently associated with higher odds of complications and longer length of stay. Because RAS cases appear to be increasing in number, future study should emphasize methods to reduce morbidity and investigate deformity-specific metrics to help better understand this population.

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