Habre W, Sims C, D'Souza M
Department of Anaesthesia, Princess Margaret Hospital For Children, Perth, Western Australia.
Paediatr Anaesth. 1997;7(3):251-3. doi: 10.1046/j.1460-9592.1997.d01-65.x.
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.
我们报告了两例患有乔伯特综合征儿童的麻醉管理情况。患有该综合征的儿童因脑干和小脑的变化而存在呼吸控制异常。他们对包括氧化亚氮在内的麻醉药物的呼吸抑制作用极为敏感。建议采用吸入诱导麻醉、控制通气、避免使用阿片类药物以及术后密切监测。