title: Anesthetic Management of a Patient With Emanuel Syndrome.
abstract: Emanuel syndrome is associated with supernumerary chromosome, which consists of the extra genetic material from chromosome 11 and 22. The frequency of this syndrome has been reported as 1 in 110,000. It is a rare anomaly associated with multiple systemic malformations such as micrognathia and congenital heart disease. In addition, patients with Emanuel syndrome may have seizure disorders. We experienced anesthetic management of a patient with Emanuel syndrome who underwent palatoplasty. This patient had received tracheotomy due to micrognathia. In addition, he had atrial septal defect, mild pulmonary artery stenosis, and cleft palate. Palatoplasty was performed without any complication during anesthesia. Close attention was directed to cardiac function, seizure, and airway management.
authors.affiliation: Department of Dental Anesthesiology, Kyushu University Hospital, Fukuoka, Japan.
authors.forename: Masanori
authors.initials: M
authors.lastname: Tsukamoto
authors.affiliation: Department of Dental Anesthesiology, Faculty of Dental Science, Kyushu University, Fukuoka, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Hitosugi
authors.affiliation: Department of Dental Anesthesiology, Kyushu University Hospital, Fukuoka, Japan.
authors.forename: Kanako
authors.initials: K
authors.lastname: Esaki
authors.affiliation: Department of Dental Anesthesiology, Faculty of Dental Science, Kyushu University, Fukuoka, Japan.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Yokoyama
mesh_terms: D000758:Anesthesia / Q000379:methods*; D002675:Child, Preschool; D025063:Chromosome Disorders / Q000601:surgery*; D002972:Cleft Palate / Q000601:surgery*; D006330:Heart Defects, Congenital / Q000601:surgery*; D006801:Humans; D008607:Intellectual Disability / Q000601:surgery*; D008297:Male; D009123:Muscle Hypotonia / Q000601:surgery*; D010159:Palate / Q000601:surgery
keywords: Congenital heart disease; Emanuel syndrome; General anesthesia