title: Anaesthetic management of children with Joubert syndrome.
abstract: We report the anaesthetic management of two children with Joubert syndrome. Children with this syndrome have abnormalities of respiratory control due to changes in the brainstem and cerebellum. They are extremely sensitive to the respiratory depressant effects of anaesthetic agents, including nitrous oxide. Anaesthesia using inhalational induction, controlled ventilation, avoidance of opioids, and close postoperative monitoring is recommended.
authors.affiliation: Department of Anaesthesia, Princess Margaret Hospital For Children, Perth, Western Australia.
authors.forename: W
authors.initials: W
authors.lastname: Habre
authors.forename: C
authors.initials: C
authors.lastname: Sims
authors.forename: M
authors.initials: M
authors.lastname: D'Souza
mesh_terms: D000768:Anesthesia, General / Q000009:adverse effects* / Q000379:methods*; D001049:Apnea / Q000209:etiology*; D002526:Cerebellar Diseases / Q000150:complications*; D002531:Cerebellum / Q000002:abnormalities*; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007430:Intraoperative Care; D007431:Intraoperative Complications / Q000209:etiology / Q000628:therapy; D008991:Monitoring, Physiologic; D011182:Postoperative Care; D011183:Postoperative Complications / Q000209:etiology / Q000628:therapy; D013577:Syndrome