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Year : 2019  |  Volume : 20  |  Issue : 1  |  Page : 42-45

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

Department of Anesthesiology, Grant Government Medical College, Mumbai, Maharashtra, India

Correspondence Address:
Dr. Paulomi Dey
Flat No. 13, LXN3, Sai Krupa CHS, Kashish Park, LBS Marg, Thane West - 400 604, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/TheIAForum.TheIAForum_57_18

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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.

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