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

Abstract

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.