General Anesthesia for a Patient With Pelizaeus-Merzbacher Disease.
General Anesthesia for a Patient With Pelizaeus-Merzbacher Disease.
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DOI：
10.2344/15-00022.1
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发表时间：
2016-01-01
影响因子：
--
通讯作者：
Fujisawa, Toshiaki
中科院分区：
文献类型：
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作者：
Kamekura, Nobuhito;Nitta, Yukie;Fujisawa, Toshiaki
我们报告一位患有Pelizaeus-Merzbacher病（PMD）的病人成功地进行了全身麻醉。PMD是一组大脑白色物质的进行性、退行性疾病之一。PMD的典型临床表现包括精神发育迟滞、眼球震颤、肌张力异常、癫痫发作和认知障碍。PMD患者的全身麻醉可能很困难，主要是因为癫痫发作和与咽肌控制不良相关的气道并发症。此外，应考虑痉挛状态恶化的可能性。一位20岁的PMD患者需要在全身麻醉下拔除阻生智齿。用硫代巴比妥、芬太尼和地氟烷诱导全身麻醉。在脑电双频指数和4串监测下，用地氟烷和持续静脉注射瑞芬太尼维持麻醉。麻醉持续1小时20分钟，顺利完成。术后未发生气道并发症、癫痫发作和痉挛加重。术前，我们的病人没有癫痫发作或吸入性肺炎的病史，也没有胃食管反流病的临床症状。因此，痉挛加重是最可能的潜在并发症之一。在术前检查中识别这些相关疾病并评估危险因素对于这些患者的安全麻醉非常重要。
We report the successful management of general anesthesia for a patient with Pelizaeus-Merzbacher disease (PMD). PMD is one of a group of progressive, degenerative disorders of the cerebral white matter. The typical clinical manifestations of PMD include psychomotor retardation, nystagmus, abnormal muscle tone, seizures, and cognitive impairment. General anesthesia for a patient with PMD may be difficult mainly because of seizures and airway complications related to poor pharyngeal muscle control. In addition, the possibility of exacerbation of spasticity should be considered. A 20-year-old man with PMD required removal of impacted wisdom teeth under general anesthesia. General anesthesia was induced with thiamylal, fentanyl, and desflurane. Anesthesia was maintained with desflurane and continuous intravenous remifentanil under bispectral index and train-of-4 monitoring. Anesthesia lasted 1 hour 20 minutes and was completed uneventfully. Airway complications, seizures, and exacerbation of spasticity did not occur postoperatively. Preoperatively, our patient had no history of epilepsy attacks or aspiration pneumonia, and no clinical symptoms of gastroesophageal reflux disease. Therefore, exacerbation of spasticity was one of the most likely potential complications. Identification of these associated conditions and evaluation of risk factors during preoperative examination is important for performing safe anesthesia in these patients.