Klockgether-Radke A, Henze T, Braun U, Kettler D
Zentrum Anästhesiologie, Rettungs- und Intensivmedizin, Georg-August-Universität Göttingen.
Anaesthesist. 1993 Feb;42(2):111-4.
Two patients with mitochondrial myopathy (Kearns-Sayre syndrome) received general anaesthesia. In the first case propofol-alfentanil anaesthesia was carried out; the second patient received propofol-fentanyl anaesthesia. Muscle relaxation was provided with vecuronium. In both cases we observed a short episode of bradycardia (heart rate < 50.min-1), which was successfully treated with atropine. After the propofol infusion had been stopped, both patients rapidly gained consciousness and were extubated after prompt spontaneous ventilation had returned. Reversal of neuromuscular blockade in the second patient was achieved within 5 min by neostigmine. Special anaesthetic problems in patients with mitochondrial myopathies such as myocardial conduction disturbances, postoperative muscle hypotonia, and possible increased susceptibility to malignant hyperthermia can be overcome by the described anaesthetic management.
两名线粒体肌病（卡恩斯-塞尔综合征）患者接受了全身麻醉。第一例采用丙泊酚-阿芬太尼麻醉；第二例患者接受丙泊酚-芬太尼麻醉。使用维库溴铵提供肌肉松弛。在两例患者中，我们均观察到短暂的心动过缓发作（心率<50次/分钟），经阿托品治疗成功。停止丙泊酚输注后，两名患者均迅速恢复意识，并在自主通气迅速恢复后拔管。第二例患者使用新斯的明在5分钟内实现了神经肌肉阻滞的逆转。所描述的麻醉管理可克服线粒体肌病患者的特殊麻醉问题，如心肌传导障碍、术后肌肉张力减退以及可能增加的恶性高热易感性。
