Anesthesia challenges in a case of Crouzon syndrome for corrective rigid external distraction frame insertion

Crouzon syndrome is an autosomal dominant disease occurs in approximately 1 in 25,000 births, due to a mutation in the fibroblast growth factor receptor 2 gene on chromosome 10 characterized by craniosynostosis, mid-face hypoplasia, hypertelorism, high-arched palate, skull base abnormalities, and ex...

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Bibliographic Details
Main Authors: Veena Ganeriwal, Paulomi Dey, Baburao Gore, Tejesh Hujare
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2019-01-01
Series:The Indian Anaesthetists' Forum
Subjects:
Online Access:http://www.theiaforum.org/article.asp?issn=2589-7934;year=2019;volume=20;issue=1;spage=42;epage=45;aulast=Ganeriwal
Description
Summary:Crouzon syndrome is an autosomal dominant disease occurs in approximately 1 in 25,000 births, due to a mutation in the fibroblast growth factor receptor 2 gene on chromosome 10 characterized by craniosynostosis, mid-face hypoplasia, hypertelorism, high-arched palate, skull base abnormalities, and exophthalmos. Patients present in early childhood for cranial reconstructive surgery. We describe here the successful management of a 5-year-old child with Crouzon syndrome with hypertrophic adenoids and Arnold-Chiari malformation type 1 posted for bifrontal craniotomy with fronto-facial advancement and application of a rigid external distraction frame.
ISSN:0973-0311