Leeds and West Yorkshire Radiology Academy, Leeds General Infirmary, Leeds University Teaching Hospitals, National Health Service Trust, Leeds, United Kingdom.
Pediatr Neurol. 2011 Jun;44(6):401-13. doi: 10.1016/j.pediatrneurol.2011.02.007.
Moyamoya disease is an uncommon chronic cerebrovasculopathy, characterized by progressive stenosis of the terminal portion of the internal carotid artery and its main branches, in association with the development of compensatory collateral vessels at the base of the brain. The etiology is unknown, and was originally considered exclusive to East Asia, with particular prevalence in Japan. Moyamoya disease is increasingly diagnosed throughout the world, and represents an important cause of childhood stroke in Western countries. In some cases, similar angiographic features are evident in children with other medical conditions, such as sickle cell disease and Down syndrome. In these instances, the term "moyamoya syndrome" is used. Diagnosing the vasculopathy, excluding possible associated conditions, and planning treatment and follow-up imaging comprise important aspects of clinical management. We review the key imaging features of childhood moyamoya disease and syndrome, present examples of its associations, and discuss new neuroradiologic methods that may be useful in management.
烟雾病是一种罕见的慢性脑血管病，其特征是颈内动脉末端及其主要分支进行性狭窄，同时在脑底形成代偿性侧支循环。病因不明，最初被认为仅存在于东亚地区，在日本尤为常见。目前，烟雾病在全球范围内的诊断率逐渐升高，也是西方国家儿童脑卒中的一个重要病因。在某些情况下，患有镰状细胞病和唐氏综合征等其他疾病的儿童也会出现类似的血管造影特征。在这些情况下，术语“烟雾病综合征”可适用。诊断血管病变、排除可能的相关疾病，以及制定治疗和随访影像学方案是临床管理的重要方面。我们回顾了儿童烟雾病和综合征的关键影像学特征，介绍了其相关病例，并讨论了可能有助于管理的新神经影像学方法。
