title: Spinal fusion for scoliosis in Rett syndrome with an emphasis on early postoperative complications.
abstract: METHODS
  Retrospective case-control study.
  
  
  OBJECTIVE
  To examine the postoperative complications of posterior spinal fusion in a population of patients with Rett syndrome (RS).
  
  
  BACKGROUND
  Scoliosis is a common feature of RS, a progressive neurologic disorder affecting almost exclusively females. Despite this, there is little published information regarding the surgical treatment of scoliosis in this disorder.
  
  
  METHODS
  Sixteen consecutive female patients with RS treated by posterior spinal fusion and unit rod instrumentation for progressive scoliosis between 1995 and 2003 were evaluated. Only patients with a minimum of 2-year follow-up were included. Preoperative medical conditions and postoperative complications were recorded. As a control group, we randomly selected 32 spastic quadriplegic patients who underwent the identical procedure during the same time period, selected from our database and matched according to age, level of neurologic impairment, and medical complexity.
  
  
  RESULTS
  There was a high rate of early medical complications in the RS patients, with 28 major and 37 minor complications. Only 1 patient did not have a major medical complication, and every patient had at least 1 minor gastrointestinal and/or respiratory complication. Major respiratory complications occurred in 10 patients (63%) and comprised 61% of all major complications. Major gastrointestinal complications occurred in 6 patients (37%) and comprised 21% of all major complications. Other major complications included disseminated intravascular coagulopathy (1 patient), subacute bacterial endocarditis (1 patient), sacral decubiti requiring surgical debridement (2 patients), and extensive bilateral heterotopic ossification of the hips (1 patient). There were no cases of instrumentation failure, pseudarthrosis, deep infection, or need for rod revision. Postoperative complication scores were similar to those in patients with spastic quadriplegic pattern cerebral palsy.
  
  
  CONCLUSIONS
  Spinal fusion for scoliosis in RS can give a satisfactory technical result, but a high rate of early postoperative medical problems should be anticipated.
authors: Division of Spine and Scoliosis Surgery, Alfred I. duPont Hospital for Children, Wilmington, DE 19899, USA. pgabos@nemours.org
authors: Peter G
authors: PG
authors: Gabos
authors: Muharrem
authors: M
authors: Inan
authors: Mihir
authors: M
authors: Thacker
authors: Buttugs
authors: B
authors: Borkhu
mesh_terms: D000293:Adolescent; D016022:Case-Control Studies; D002648:Child; D005260:Female; D006801:Humans; D011183:Postoperative Complications / Q000453:epidemiology* / Q000209:etiology / Q000503:physiopathology; D011782:Quadriplegia / Q000150:complications / Q000503:physiopathology; D012189:Retrospective Studies; D015518:Rett Syndrome / Q000150:complications / Q000503:physiopathology*; D012600:Scoliosis / Q000209:etiology / Q000503:physiopathology* / Q000601:surgery*; D013123:Spinal Fusion / Q000009:adverse effects* / Q000295:instrumentation / Q000379:methods*; D055815:Young Adult