Please use this identifier to cite or link to this item: http://hdl.handle.net/2440/6970
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Type: Journal article
Title: Late presentation of upper airway obstruction in Pierre Robin sequence
Author: Wilson, A.
Moore, D.
Moore, M.
Martin, A.
Staugas, R.
Kennedy, J.
Citation: Archives of Disease in Childhood, 2000; 83(5):435-438
Publisher: British Med Journal Publ Group
Issue Date: 2000
ISSN: 0003-9888
1468-2044
Statement of
Responsibility: 
A C Wilson, D J Moore, M H Moore, A J Martin, R E M Staugas, J D Kennedy
Abstract: A retrospective review was carried out of 11 consecutive patients with the Pierre Robin sequence referred to a tertiary paediatric referral centre over a five year period from 1993 to 1998. Ten patients were diagnosed with significant upper airway obstruction; seven of these presented late at between 24 and 51 days of age. Failure to thrive occured in six of these seven infants at the time of presentation, and was a strong indicator of the severity of upper airway obstruction. Growth normalised on treatment of the upper airway obstruction with nasopharyngeal tube placement. All children had been reviewed by either an experienced general paediatrician or a neonatologist in the first week of life, suggesting that clinical signs alone are insufficent to alert the physician to the degree of upper airway obstruction or that obstruction developed gradually after discharge home. The use of polysomnography greatly improved the diagnostic accuracy in assesssing the severity of upper airway obstruction and monitoring the response to treatment. This report highlights the prevalence of late presentation of upper airway obstruction in the Pierre Robin sequence and emphasises the need for close prospective respiratory monitoring in this condition. Objective measures such as polysomnography should be used, as clinical signs alone may be an inadequate guide to the degree of upper airway obstruction.
Keywords: Humans; Pierre Robin Syndrome; Airway Obstruction; Failure to Thrive; Polysomnography; Retrospective Studies; Age of Onset; Infant; Infant, Newborn; Infant, Premature; Female; Male
Description: Copyright © 2000 by the BMJ Publishing Group Ltd.
RMID: 0001000849
DOI: 10.1136/adc.83.5.435
Appears in Collections:Paediatrics publications

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