Bardet–Biedl syndrome: expect the unexpected, suspect the unsuspected

Southern African Journal of Anaesthesia and Analgesia

 
 
Field Value
 
Title Bardet–Biedl syndrome: expect the unexpected, suspect the unsuspected
 
Creator Cronjé, Larissa
 
Subject — anterior laryngeal web; Bardet–Biedl syndrome; bifid epiglottis; laryngeal anomalies; paediatric anaesthesia; paediatric obesity; stridor; craniofacial syndromes; upper airway obstruction
Description This is the first reported description of Bardet–Biedl syndrome (BBS) with the combination of a malacic bifid epiglottis and anterior laryngeal web. Anaesthesia for BBS has numerous concerns and these are reviewed, focusing on features that manifest not only in BBS but across a spectrum of syndromes that impact airway management. Congenital laryngeal anomalies (CLA) are rare and usually present preoperatively with upper airway obstruction and stridor. In asymptomatic infants, CLA may cause unexpected airway problems under anaesthesia which can be mistaken for more common occurrences, such as laryngospasm. Preoperative dysphonia may be the only clue to suspecting the presence of a CLA. The combination of obesity, polysyndactyly/brachydactyly and even subtle craniofacial abnormalities should always alert the anaesthetist to the possibility of a CLA and difficult airway.
 
Publisher AOSIS
 
Contributor
Date 2016-10-30
 
Type info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion Peer-reviewed Article —
Format application/pdf
Identifier 10.1080/22201181.2017.1379719
 
Source Southern African Journal of Anaesthesia and Analgesia; Vol 23, No 5 (2017); 129-138 2220-1173 2220-1181
 
Language eng
 
Relation
The following web links (URLs) may trigger a file download or direct you to an alternative webpage to gain access to a publication file format of the published article:

https://sajaa.co.za/index.php/sajaa/article/view/1120/1111
 
Coverage — — —
Rights Copyright (c) 2017 Larissa Cronjé http://creativecommons.org/licenses/by-nc/3.0
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