Obstructive Sleep Apnoea and the Role of Tongue Reduction Surgery in Children with Beckwith-Wiedemann Syndrome

Overview

Journal Paediatr Respir Rev

Specialties Pediatrics
Pulmonary Medicine

Date 2017 Apr 4

PMID 28366681

Citations 9

Authors

Christopher M Cielo

Kelly A Duffy

Aesha Vyas

Jesse A Taylor

Jennifer M Kalish

Affiliations

Soon will be listed here.

Abstract

Beckwith-Wiedemann syndrome (BWS) is a rare paediatric overgrowth disorder. Associated macroglossia is a feature of many children with BWS and is felt to be a risk factor for obstructive sleep apnoea (OSA). Sleep-disordered breathing is highly variable in this population. The relationship between degree of macroglossia or other genotypic or phenotypic factors and OSA severity has not been established. The natural history of OSA in this population is unknown; a variety of conservative and surgical therapies have been used to treat OSA in children with BWS but none have been studied systematically. Tongue reduction is the mainstay of surgical therapy for macroglossia associated with BWS, but limited data are available regarding its efficacy in treating OSA or its effect on speech and swallowing. More research is needed to better identify which children with BWS are at risk for OSA and the most effective treatment for these patients.

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