Guidelines for management of scoliosis in rett syndrome patients based on expert consensus and clinical evidence

Downs, Jenny, Bergman, Anke, Carter, Philippa, Anderson, Alison, Palmer, Greta M., Roye, David, Van Bosse, Harold, Bebbington, Ami, Larsson, Eva Lena, Smith, Brian G., Baikie, Gordon, Fyfe, Sue and Leonard, Helen (2009) Guidelines for management of scoliosis in rett syndrome patients based on expert consensus and clinical evidence. Spine, 34 17: E607-E617. doi:10.1097/BRS.0b013e3181a95ca4

Author Downs, Jenny
Bergman, Anke
Carter, Philippa
Anderson, Alison
Palmer, Greta M.
Roye, David
Van Bosse, Harold
Bebbington, Ami
Larsson, Eva Lena
Smith, Brian G.
Baikie, Gordon
Fyfe, Sue
Leonard, Helen
Title Guidelines for management of scoliosis in rett syndrome patients based on expert consensus and clinical evidence
Journal name Spine   Check publisher's open access policy
ISSN 0362-2436
Publication date 2009-01-01
Year available 2009
Sub-type Article (original research)
DOI 10.1097/BRS.0b013e3181a95ca4
Open Access Status Not Open Access
Volume 34
Issue 17
Start page E607
End page E617
Total pages 11
Place of publication Philadelphia, PA United States
Publisher Lippincott Williams and Wilkins
Language eng
Subject 2728 Clinical Neurology
2732 Orthopedics and Sports Medicine
Abstract Study Design.: Modified Delphi technique. Objective.: To develop guidelines for the clinical management of scoliosis in Rett syndrome through evidence review and consensus expert panel opinion. Summary of Background Data.: 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° to 50° and must be supported by specialist management of anesthesia, pain control, seizures, and early mobilization. Conclusion.: 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.
Keyword Delphi technique
Neuromuscular diseases
Practice guideline
Rett syndrome
Q-Index Code C1
Q-Index Status Provisional Code
Institutional Status Non-UQ

Document type: Journal Article
Sub-type: Article (original research)
Collection: Institute for Molecular Bioscience - Publications
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