title: Guidelines for management of scoliosis in Rett syndrome patients based on expert consensus and clinical evidence.
abstract: METHODS
  Modified Delphi technique.
  
  
  OBJECTIVE
  To develop guidelines for the clinical management of scoliosis in Rett syndrome through evidence review and consensus expert panel opinion.
  
  
  BACKGROUND
  Rett syndrome is a rare disorder and clinical expertise is thus with small case series. Scoliosis is a frequent association and the evidence base dealing with scoliosis management in this syndrome is limited. Parents of affected girls and women have expressed needs for more information about scoliosis and Rett syndrome.
  
  
  METHODS
  An initial draft of scoliosis guidelines was created based on literature review and open-ended questions where the literature was lacking. Perspectives of four parents of Rett syndrome patients informed this initial draft. Access to an online and a Microsoft Word formatted version of the draft were then sent to an international, multidisciplinary panel of clinicians via e-mail with input sought using a 2-stage modified Delphi process to reach consensus agreement. Items included clinical monitoring and intervention before the diagnosis of scoliosis; monitoring after the diagnosis of scoliosis; imaging; therapy and conservative management; bracing; and preoperative, surgical, and postoperative considerations.
  
  
  RESULTS
  The first draft contained 71 statements, 65 questions. The second draft comprised 88 items with agreement to strong agreement achieved on 85, to form the final guideline document. A comprehensive, life-span approach to the management of scoliosis in Rett syndrome is recommended that takes into account factors such as physical activity, posture, nutritional and bone health needs. Surgery should be considered when the Cobb angle is approximately 40 degrees to 50 degrees and must be supported by specialist management of anesthesia, pain control, seizures, and early mobilization.
  
  
  CONCLUSIONS
  Evidence- and consensus-based guidelines were successfully created and have the potential to improve care of a complex comorbidity in a rare condition and stimulate research to improve the current limited evidence base.
authors: Centre for Child Health Research, Telethon Institute for Child Health Research, Perth, Australia.
authors: Jenny
authors: J
authors: Downs
authors: Anke
authors: A
authors: Bergman
authors: Philippa
authors: P
authors: Carter
authors: Alison
authors: A
authors: Anderson
authors: Greta M
authors: GM
authors: Palmer
authors: David
authors: D
authors: Roye
authors: Harold
authors: H
authors: van Bosse
authors: Ami
authors: A
authors: Bebbington
authors: Eva Lena
authors: EL
authors: Larsson
authors: Brian G
authors: BG
authors: Smith
authors: Gordon
authors: G
authors: Baikie
authors: Sue
authors: S
authors: Fyfe
authors: Helen
authors: H
authors: Leonard
mesh_terms: D002648:Child; D019317:Evidence-Based Medicine; D005260:Female; D006801:Humans; D007391:International Cooperation; D015518:Rett Syndrome / Q000150:complications*; D012600:Scoliosis / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*