Gastrointestinal dysmotility in rett syndrome

Academic Article

Abstract

  • OBJECTIVES: Through evidence review and the consensus of an expert panel, we developed recommendations for the clinical management of gastroesophageal reflux disease, constipation, and abdominal bloating in Rett syndrome. METHODS: Based on review of the literature and family concerns expressed on RettNet, initial draft recommendations were created. Wherein the literature was lacking, 25 open-ended questions were included. Input from an international, multidisciplinary panel of clinicians was sought using a 2-stage modified Delphi process to reach consensus agreement. Items related to the clinical assessment and management of gastroesophageal reflux disease, constipation, and abdominal bloating. RESULTS: Consensus was achieved on 78 of 85 statements. A comprehensive approach to the assessment of gastroesophageal reflux and reflux disease, constipation, and abdominal bloating was recommended, taking into account impairment of communication skills in Rett syndrome. A stepwise approach to the management was identified with initial use of conservative strategies, escalating to pharmacological measures and surgery, if necessary. CONCLUSIONS: Gastrointestinal dysmotility occurs commonly in Rett syndrome. These evidence-and consensus-based recommendations have the potential to improve care of dysmotility issues in a rare condition and stimulate research to improve the present limited evidence base. Copyright © 2014 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.
  • Authors

    Digital Object Identifier (doi)

    Author List

  • Baikie G; Ravikumara M; Downs J; Naseem N; Wong K; Percy A; Lane J; Weiss B; Ellaway C; Bathgate K
  • Start Page

  • 237
  • End Page

  • 244
  • Volume

  • 58
  • Issue

  • 2