Spinal Fusion for Scoliosis in Rett Syndrome With an Emphasis on Respiratory Failure and Opioid Usage

J Child Neurol. 2016 Feb;31(2):153-8. doi: 10.1177/0883073815585352. Epub 2015 May 19.

Abstract

Our objective was to characterize our experience with 8 patients with Rett syndrome undergoing scoliosis surgery in regard to rates of respiratory failure and rates of ventilator-acquired pneumonia in comparison to patients with neurologic scoliosis and adolescent idiopathic scoliosis. This study was a retrospective chart review of patients undergoing scoliosis surgery at a tertiary children's hospital. Patients were divided into 3 groups: (1) adolescent idiopathic scoliosis, (2) neurologic scoliosis, and (3) Rett syndrome. There were 133 patients with adolescent idiopathic scoliosis, 48 patients with neurologic scoliosis, and 8 patients with Rett syndrome. We found that patients with Rett syndrome undergoing scoliosis surgery have higher rates of respiratory failure and longer ventilation times in the postoperative period when compared with both adolescent idiopathic scoliosis and neurologic scoliosis patients. There is insufficient evidence to suggest a difference in the incidence of ventilator-acquired pneumonia between the Rett syndrome and the neurologic scoliosis group. We believe our findings are the first in the literature to show a statistically significant difference between these 3 groups in regard to incidence of respiratory failure.

Keywords: Rett syndrome; comparative study; pediatric intensive care unit; scoliosis; ventilator-acquired pneumonia.

Publication types

  • Comparative Study

MeSH terms

  • Adolescent
  • Analgesics, Opioid / therapeutic use*
  • Child
  • Female
  • Humans
  • Male
  • Methyl-CpG-Binding Protein 2 / genetics
  • Postoperative Complications*
  • Respiration, Artificial / adverse effects
  • Respiratory Insufficiency / complications*
  • Respiratory Insufficiency / epidemiology
  • Respiratory Insufficiency / therapy
  • Retrospective Studies
  • Rett Syndrome / complications*
  • Rett Syndrome / epidemiology
  • Rett Syndrome / genetics
  • Scoliosis / complications
  • Scoliosis / drug therapy
  • Scoliosis / epidemiology
  • Scoliosis / surgery*
  • Spinal Fusion* / adverse effects
  • Spinal Fusion* / methods
  • Young Adult

Substances

  • Analgesics, Opioid
  • MECP2 protein, human
  • Methyl-CpG-Binding Protein 2