title: Anaesthesia Management in a Patient with Waardenburg Syndrome and Review of the Literature.
abstract: Waardenburg syndrome is a rare autosomal dominant disease that may cause hearing loss, pigmentary abnormalities, neurocristopathy and partial albinism. Incidence is estimated as 2%-3% among the cases of congenital deafness and 1/42,000 of the general population. Children with Waardenburg syndrome usually require anaesthesia for the cochlear implant operation in early age. The features of the syndrome that may bear importance for anaesthetic management are laryngomalacia, multiple muscle contractures, limited neck movements, cyanotic cardiopathy and electrolyte imbalance. Patients with Waardenburg syndrome stand for difficult airway. We aimed to report anaesthetic management of a child with Waardenburg syndrome who underwent surgery for cochlear implantation.
authors.affiliation: Clinic of Anaesthesiology, Dışkapı Yıldırım Beyazıt Training and Reseacrh Hospital, Ankara, Turkey.
authors.forename: Kevser
authors.initials: K
authors.lastname: Peker
authors.affiliation: Clinic of Anaesthesiology, Dışkapı Yıldırım Beyazıt Training and Reseacrh Hospital, Ankara, Turkey.
authors.forename: Julide
authors.initials: J
authors.lastname: Ergil
authors.affiliation: Clinic of Anaesthesiology, Dışkapı Yıldırım Beyazıt Training and Reseacrh Hospital, Ankara, Turkey.
authors.forename: İbrahim
authors.initials: İ
authors.lastname: Öztürk
keywords: Waardenburg syndrome; airway; anaesthetic management