Itaya K, Takahata O, Mamiya K, Saito T, Tamakawa S, Akama Y, Kubota M, Ogawa H
Department of Anesthesiology and Critical Care Medicine, Asahikawa Medical College.
Masui. 1995 May;44(5):710-2.
MELAS is a type of the mitochondrial myopathy characterized by elevation of pyruvate and lactate levels in both the blood and cerebrospinal fluid. This syndrome frequently accompanies cerebral infarction like symptoms. Recently, we experienced two patients for anesthesia with MELAS (both 11-year-old girls). V-P shunt construction and IVH reservoir implantation were conducted, respectively. Anesthesia was induced with fentanyl and midazolam, and vecuronium was used to facilitate tracheal intubation. Volatile anesthetic was avoided, and anesthesia was maintained with fentanyl, midazolam, and nitrous oxide. Arterial blood gases and pH were frequently checked, and acetated electrolyte solution was infused mainly during surgery. No complications occurred during anesthesia in both patients. In the anesthetic management for MELAS, measures to prevent malignant hyperthermia must also be considered.
线粒体脑肌病伴乳酸血症和卒中样发作(MELAS)是一种线粒体肌病,其特征是血液和脑脊液中的丙酮酸和乳酸水平升高。该综合征常伴有脑梗死样症状。最近,我们为两名患有MELAS的患者(均为11岁女孩)实施了麻醉。分别进行了脑室-腹腔分流术和脑室内出血贮液器植入术。用芬太尼和咪达唑仑诱导麻醉,并用维库溴铵辅助气管插管。避免使用挥发性麻醉剂,用芬太尼、咪达唑仑和氧化亚氮维持麻醉。频繁检查动脉血气和pH值,手术期间主要输注醋酸盐电解质溶液。两名患者在麻醉期间均未发生并发症。在MELAS的麻醉管理中,还必须考虑预防恶性高热的措施。