Anesthetic management of craniosynostosis repair in patient with Apert syndrome

Apert syndrome is an autosomal dominant disease characterized by craniosynostosis, midface hypoplasia and syndactyly. In general, patients present in early childhood for craniofacial reconstruction surgery. Anesthetic implications include difficult airway, airway hyper-reactivity; however, possibility of raised intracranial pressure especially when operating for craniosynostosis and associated congenital heart disease should not be ignored. Most of the cases described in literature talk of management of syndactyly. We describe the successful anesthetic management of a patient of Aperts syndrome with craniosynostosis posted for bicornual strip craniotomy and fronto-orbital advancement in a 5-year-old child.