Gastrointestinal dysmotility in Rett syndrome

Gordon Baikie, Madhur Ravikumara, Jennepher Downs, Nusrat Naseem, Kingsley Wong, Alan Percy, Jane Lane, Batia Weiss, Carolyn Ellaway, Katherine Bathgate, Helen Leonard

Research output: Contribution to journalArticle

26 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)237-244
Number of pages8
JournalJournal of Pediatric Gastroenterology and Nutrition
Volume58
Issue number2
DOIs
Publication statusPublished - Feb 2014

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