title: Spinal Fusion for Scoliosis in Rett Syndrome With an Emphasis on Respiratory Failure and Opioid Usage.
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.
authors.affiliation: Division of Pediatric Critical Care Medicine, Department of Pediatrics, Children's Hospital at Montefiore/Albert Einstein College of Medicine, USA Division of Child and Adolescent Health, Department of Pediatrics, Columbia University Medical Center, New York, NY, USA daniarumbak@gmail.com.
authors.forename: Dania M
authors.initials: DM
authors.lastname: Rumbak
authors.affiliation: Department of Epidemiology and Population Health, Albert Einstein College of Medicine, Bronx, NY, USA.
authors.forename: Wenzhu
authors.initials: W
authors.lastname: Mowrey
authors.affiliation: Department of Epidemiology and Biostatistics, University of South Florida College of Public Health, Tampa, FL, USA.
authors.forename: Skai
authors.initials: S
authors.lastname: W Schwartz
authors.affiliation: Department of Orthopaedic Surgery, Montefiore Medical Center, Bronx, NY, USA.
authors.forename: Vishal
authors.initials: V
authors.lastname: Sarwahi
authors.affiliation: Division of Neurology and Tri-State Rett Syndrome Center, Department of Pediatrics, Children's Hospital at Montefiore/Albert Einstein College of Medicine, Bronx, NY, USA.
authors.forename: Aleksandra
authors.initials: A
authors.lastname: Djukic
authors.affiliation: Division of Pediatric Critical Care Medicine, Department of Pediatrics, Children's Hospital at Montefiore/Albert Einstein College of Medicine, USA Division of Pediatric Critical Care Medicine, Department of Pediatrics, Weill Cornell Medical College, New York Presbyterian Hospital, New York, NY, USA.
authors.forename: James S
authors.initials: JS
authors.lastname: Killinger
authors.affiliation: Division of Pediatric Critical Care Medicine, Department of Pediatrics, Children's Hospital at Montefiore/Albert Einstein College of Medicine, USA.
authors.forename: Chhavi
authors.initials: C
authors.lastname: Katyal
mesh_terms: D000293:Adolescent; D000701:Analgesics, Opioid / Q000627:therapeutic use*; D002648:Child; D005260:Female; D006801:Humans; D008297:Male; D051783:Methyl-CpG-Binding Protein 2 / Q000235:genetics; D011183:Postoperative Complications; D012121:Respiration, Artificial / Q000009:adverse effects; D012131:Respiratory Insufficiency / Q000150:complications* / Q000453:epidemiology / Q000628:therapy; D012189:Retrospective Studies; D015518:Rett Syndrome / Q000150:complications* / Q000453:epidemiology / Q000235:genetics; D012600:Scoliosis / Q000150:complications / Q000188:drug therapy / Q000453:epidemiology / Q000601:surgery*; D013123:Spinal Fusion / Q000009:adverse effects / Q000379:methods; D055815:Young Adult
chemical_list: D000701:Analgesics, Opioid; C497392:MECP2 protein, human; D051783:Methyl-CpG-Binding Protein 2
keywords: Rett syndrome; comparative study; pediatric intensive care unit; scoliosis; ventilator-acquired pneumonia