Division of Pediatric Cardiac Surgery, Department of Cardiology and Cardiothoracic Surgery, Hamad Medical Corporation, Doha, Qatar.
J Clin Anesth. 2013 Sep;25(6):488-90. doi: 10.1016/j.jclinane.2013.02.013. Epub 2013 Aug 17.
The anesthetic implications of Joubert syndrome in an infant who required cardiac surgery using cardiopulmonary bypass (CPB) is presented. Children with Joubert syndrome present with central apnea due to malformations in the midbrain and cerebellum. These patients have a marked sensitivity to opioids. The use of dexmedetomidine along with remifentanil was effective in this case.
本文介绍了一例婴儿在使用体外循环（CPB）进行心脏手术时出现杰伯氏综合征的麻醉相关问题。杰伯氏综合征患儿由于中脑和小脑畸形而出现中枢性呼吸暂停。这些患者对阿片类药物非常敏感。在本例中，使用右美托咪定联合瑞芬太尼是有效的。
