title: [Anesthesia for a patient with Cockayne's syndrome].
abstract: Cockayne's syndrome is a rare, inherited, autosomal recessive disorder, characterised by dwarfism and progressive physical and mental retardation. A case is described of a 6-year-old girl (height 75 cm, weight 5.0 kg) with Cockayne's syndrome who was scheduled for liver biopsy under general anesthesia. Anesthesia was induced using 5% sevoflurane combined with 67% nitrous oxide in oxygen. After the end-tidal sevoflurane concentration of 3.5% had been maintained for 10 minutes, laryngoscopy was attempted. The vocal cord was visualised and the trachea was intubated easily without muscle relaxant. Anesthesia was maintained with sevoflurane and nitrous oxide in oxygen. The procedure lasted for 13 minutes. The tracheal tube was removed uneventfully following the return of adequate spontaneous respiration and airway reflexes. There were no postoperative problems. Concerning Cockayne's syndrome, there have been some reports of difficult airway, laryngospasm, aspirations of gastric contents and others. It is important to prevent laryngospasm by keeping the adequate depth of anesthesia for endotracheal intubation.
authors.affiliation: Department of Anesthesia, Tsukuba University Hospital, Tsukuba 305-3857.
authors.forename: Takeru
authors.initials: T
authors.lastname: Shimizu
authors.forename: Shinichi
authors.initials: S
authors.lastname: Inomata
authors.forename: Masayuki
authors.initials: M
authors.lastname: Miyabe
authors.forename: Hidenori
authors.initials: H
authors.lastname: Toyooka
mesh_terms: D000768:Anesthesia, General / Q000379:methods*; D002648:Child; D003057:Cockayne Syndrome / Q000150:complications; D005260:Female; D006801:Humans