Moyamoya是一种起源不明的进行性动脉病，影响颈内动脉（ICA）的分支。动脉病变可以作为一种孤立的医疗状况出现，影响大脑的两侧（“烟雾病”）或可以是单侧的或与系统性疾病有关（“烟雾综合症”）。由腔狭窄引起的缺血使儿童容易发生短暂性脑缺血发作和中风，这是受影响患者的主要表现。尽管这种情况很少见-影响美国Moyamoya的100万儿童中的1人，但占所有儿童中风的6％。诊断是通过动脉造影的特征性发现来定义的，包括ICA分支的狭窄和该区域小侧支血管的病理喷雾，描述性地比喻为“烟雾”(日语中的“moyamoya”)。治疗的前提是通过手术血运重建恢复脑血流。这种疾病的罕见限制了研究和循证临床管理的发展。在承认这些限制的同时，在这篇文章中，作者旨在总结目前儿童烟雾的研究，目的是为构建循证治疗指南提供框架。这些指南中当前数据的汇编应作为一种资源，以帮助小儿神经外科医生发挥其作用，为受影响的儿童提供适当的护理。
Moyamoya is a progressive arteriopathy of unknown origin affecting the branches of the internal carotid artery (ICA). The arteriopathy can present as an isolated medical condition, affecting both sides of the brain (\"moyamoya disease\") or can be unilateral or found in association with systemic disorders (\"moyamoya syndrome\"). The ischemia resulting from luminal narrowing predisposes children to transient ischemic attacks and stroke-the primary presentations of affected patients. Although it is rare-affecting 1 in 1 million children in the US-moyamoya is implicated in 6% of all childhood strokes. Diagnosis is defined by characteristic findings on arteriograms, including stenosis of the branches of the ICA and a pathognomonic spray of small collateral vessels in this region, descriptively likened to a \"puff of smoke\" (\"moyamoya\" in Japanese). Treatment is predicated on restoration of cerebral blood flow by surgical revascularization. The rarity of this disorder has limited research and the development of evidence-based clinical management. While acknowledging these limitations, in this article the authors aim to summarize current studies of pediatric moyamoya, with the objective of providing a framework for construction of evidence-based
guidelines for treatment. The compilation of current data in these
guidelines should serve as a resource to aid pediatric neurosurgeons in their role as advocates for providing appropriate care to affected children.
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