Haas A, Wappler F
Kliniken der Stadt Köln, Krankenhaus Merheim, Klinikum der Universität Witten/Herdecke, Ostmerheimer Str. 200, 51109, Köln, Deutschland.
Anaesthesist. 2015 Oct;64(10):747-53. doi: 10.1007/s00101-015-0075-x. Epub 2015 Aug 28.
The mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) syndrome is a disease triggered by a disorder in energy production within mitochondria. The cause of this syndrome is a mutation in the mitochondrial DNA where in 80% of cases an A-to-G mutation is present at nucleotide 3243 and with a prevalence of 18.4/100,000 in the population. Predominantly affected are organ systems with a high energy metabolism, such as the heart, brain and musculature. During the premedication visit a thorough patient history and examination with respect to neurological impairments must be carried out. Epilepsy and the appropriate permanent medication lead to possible alterations in effectiveness of anesthetics and muscle relaxants which are difficult to predict. An extensive patient cardiac history and a preoperative electrocardiogram (ECG) for an appraisal of possible disorders in the cardiac conduction system and when necessary extended cardiac diagnostics, are recommended. The monitoring must be adapted depending on the functional limitations and the forthcoming intervention and when necessary a postoperative surveillance in an intensive care unit should be initiated. Knowledge of the special features of MELAS syndrome in association with a consideration of the characteristics of anesthesia in MELAS patients and an individually adapted intensified perioperative surveillance, can contribute to a reduction in perioperative morbidity in patients suffering from MELAS syndrome.
线粒体肌病、脑病、乳酸酸中毒和卒中样发作(MELAS)综合征是一种由线粒体能量产生紊乱引发的疾病。该综合征的病因是线粒体DNA发生突变,80%的病例在3243位核苷酸处存在A到G的突变,在人群中的患病率为18.4/10万。主要受影响的是能量代谢高的器官系统,如心脏、大脑和肌肉组织。在术前访视时,必须就神经功能损害进行全面的患者病史询问和检查。癫痫及相应的长期用药可能导致麻醉药和肌肉松弛剂的效果发生难以预测的改变。建议详细了解患者的心脏病史,并进行术前心电图检查以评估心脏传导系统可能存在的紊乱,必要时进行扩展的心脏诊断。必须根据功能限制和即将进行的干预调整监测,必要时应在重症监护病房进行术后监测。了解MELAS综合征的特殊特征,并结合考虑MELAS患者的麻醉特点以及进行个体化调整的强化围手术期监测,有助于降低MELAS综合征患者的围手术期发病率。