✦ LIBER ✦

Airway Management in Nager Syndrome

✍ Scribed by Allen S. Ho; Pedram Aleshi; Sheila E. Cohen; Peter J. Koltai; Alan G. Cheng

Publisher: John Wiley and Sons
Year: 2009
Tongue: English
Weight: 368 KB
Volume: 119
Category: Article
ISSN: 0023-852X
DOI: 10.1002/lary.20468

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✦ Synopsis

Nager acrofacial dysostosis is a rare congenital syndrome characterized by malformations of the mandibulofacial structures and pre axial upper limbs. Patients classically present with malar hypoplasia, micro/retrognathia, anti mongoloid slanting, cleft deformities, ear anomalies, and hypoplastic thumbs. Trismus and glossoptosis from mandibular abnormalities predispose infants to life threatening respiratory distress after birth. A case of a Nager Syndrome mother delivering a similarly afflicted fetus is presented, with approaches to maintaining both tenuous airways described. Nager Syndrome pts warrant meticulous airway management upon delivery.

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