Ibáñez C, Fernández-González I
Hospital Universitario Central de Asturias, Centro General, Oviedo.
Rev Esp Anestesiol Reanim. 2011 Nov;58(9):585-7. doi: 10.1016/s0034-9356(11)70144-x.
Mitochondrial neurogastrointestinal encephalopathy (MNGIE) is characterized by leukoencephalopathy, peripheral neuropathy, ptosis, ophthalmoplegia, and gastrointestinal dysmotility. Mitochondrial myopathies are rare diseases and little is known of how to manage them when the patient requires anesthesia. We describe the anesthetic procedure used during emergency surgery for megacolon in a 26-year-old woman with MNGIE. Variables monitored were electrocardiogram, invasive arterial pressure, oxygen saturation by pulse oximetry, end-tidal carbon dioxide pressure, neuromuscular block, and depth of anesthesia (entropy). Rapid sequence induction was accomplished with midazolam, fentanyl, propofol, and rocuronium as an alternative to succinylcholine. Anesthesia was maintained with intravenous propofol; a second dose of the neuromuscular blocker was not required. No intraoperative problems developed and extubation was possible 2 hours after arrival in the postoperative critical care unit, once we had checked the level of block to confirm that reversion was not required.
线粒体神经胃肠性脑病（MNGIE）的特征为白质脑病、周围神经病变、上睑下垂、眼肌麻痹和胃肠动力障碍。线粒体肌病是罕见疾病，对于患者需要麻醉时如何处理知之甚少。我们描述了一名26岁患有MNGIE的女性因巨结肠接受急诊手术时所采用的麻醉过程。监测的变量包括心电图、有创动脉压、脉搏血氧饱和度、呼气末二氧化碳分压、神经肌肉阻滞和麻醉深度（熵）。采用咪达唑仑、芬太尼、丙泊酚和罗库溴铵进行快速顺序诱导，替代琥珀酰胆碱。麻醉维持采用静脉注射丙泊酚；无需追加第二剂神经肌肉阻滞剂。术中未出现问题，术后进入重症监护病房2小时后，在检查了阻滞水平并确认无需恢复后，即可进行拔管。
