Airway obstruction in the Crouzon syndrome: case report and review of the literature.

Publication/Presentation Date

3-1-1995

Abstract

Craniofacial dysostosis (Crouzon syndrome) is a syndrome characterized by premature closing of calvarial and cranial base sutures as well as those of the orbit and maxillary complex. Orofacial manifestations of this disease include maxillary hypoplasia, external nasal deformity, and prognathism. Airway distress is a well described feature of this syndrome, and both upper and lower airway obstruction may be present in the Crouzon syndrome. Obstructions in the upper airway have been reported secondary to septal deviation, midnasal and choanal abnormalities, and nasopharyngeal narrowing. This study presents a child with the Crouzon syndrome who required tracheotomy during the first 2 years of life for airway obstruction caused in the newborn period (first 6 months) by midnasal stenosis and laser (6 months to 2 years) compounded by soft palate obstruction at the level of the oro/hypopharynx. A review of the English language literature revealed one similar patient presentation but no comprehensive review of this subject. Previously reported airway anomalies are reviewed and airway management is discussed in patients with Crouzon syndrome.

Volume

31

Issue

2-3

First Page

235

Last Page

246

ISSN

0165-5876

Disciplines

Medicine and Health Sciences | Otolaryngology

PubMedID

7782181

Department(s)

Department of Surgery, Division of Otolaryngology

Document Type

Article

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