摘要背景烟雾病(moyamoya disease,MMD)是一种慢性进展性脑血管闭塞性疾病,随着疾病的进展,脑循环前循环脑灌注压逐渐降低,血流逐渐减少;脑血管储备能力减弱,二氧化碳反应性呈单向表现;脑血流自身调节能力受损,脑血流量(cerebral blood flow,CBF)呈压力依赖型.目的综述MMD的围术期麻醉管理特点,为临床工作提供参考.内容分别从MMD的病理生理学、麻醉前准备、麻醉诱导与维持、围术期脑缺血的预防措施等方面进行阐述.趋向随着医疗技术的进步,MMD的检出率显著提高,外科治疗逐渐完善.积极谨慎的麻醉处理是保证MMD患者围术期大脑前循环氧供需平衡、降低脑血管事件的有效措施.

abstractsBackground Moyamoya disease (MMD) is a chronic and progressive cerebrovascular occlusive disease with special pathophysiology features:cerebral perfusion pressurc goes down and cerebral blood flow decreases gradually along with the development of the disease in the anterior circulation; cerebrovascular reserve is abated,the response to the carbon dioxide is unidirectional; the autoregulation of CBF to the ischemic cerebral areas is impaired and the CBF is totally pressure dependent.Objective Summarizing the features of anesthetic management for MMD is to provide reference data for clinical work.Content We have reviewed the aspects that include the pathophysiology of MMD,preanesthetic preparation,anesthetic induction and maintainance and prophylactic measures for cerebral ischemia so on.Trend With the medical technology progress,MMD detection rate is increased significantly,and surgical treatment is improved gradually.Positive and cautious anesthetic managements are the key to keep balance between anterior circulation cerebral oxygen supply and demand,and to prevent cerebrovascular accident.