儿童烟雾病及烟雾综合征的治疗策略

Therapeutic strategy of moyamoya disease and moyamoya syndrome

烟雾病是一种少见的病因不明的慢性进行性脑血管闭塞性疾病,影响双侧颈内动脉及其分支,当仅单侧发病或虽双侧发病但存在其他系统疾病时称为"烟雾综合征"。因颈内动脉逐渐狭窄导致脑缺血,短暂性脑缺血发作或脑卒中成为儿童患者的主要表现。虽然烟雾病及烟雾综合征发病率低,却是儿童脑血管事件的一个重要原因。诊断是基于脑血管造影所定义的特征性表现,包括狭窄的颈内动脉及其分支和特殊的烟雾状异常血管网。外科治疗疗效明显,主要通过脑血管重建术增加脑血流量,减少脑卒中的发生。本文就儿童烟雾病及烟雾综合征的诊断学研究、手术适应证、围手术期管理和外科治疗以及随访作一综述。

Moyamoya disease is a rare chronic cerebral vascular disease which leads to progressive occlution with unknown etiology,and it affects bilateral internal carotid artery and its branches.When occured unilateral or bilateral but with other systemic diseases,it will be called＂moyamoya syndrome＂.With the gradually narrowing of the internal carotid artery leading to cerebral ischemia,transient ischemic attack or stroke becomes the main performance of the peadiatic patients.Although moyamoya disease and moyamoya syndrome have lower incidence,they are an important reason for peadiatric cerebrovascular events.Diagnosis is based on cerebral angiogram,including the stenosed internal carotid artery and its branches and pathognomonic spray of small collateral vessels.Surgical treatment has an obvious efficacy.The cerebral revascularization can increase cerebral blood flow and reduce the occurrence of stroke.The diagnostic research,surgical indications,perioperative management,surgical treatment and follow-up of moyamoya disease and moyamoya syndrome will be summarized in this review.