Dandy-Walker综合征(Dandy-Walker syndrome,DWS)是一种罕见的先天性中枢神经系统疾病,通常表现为脑积水。DWS的典型解剖学特征是小脑蚓部发育不全、后颅窝前后扩大、窦环和横窦向上移位以及第4脑室囊性扩张。临床症状可表现为癫痫、步态不稳、言语功能障碍、精神症状及眼部症状等一系列颅脑相关症状,上述临床表现可单独或合并存在。目前的治疗方法包括后颅窝囊肿切除术、脑室-腹腔分流术、囊肿-腹腔分流术、脑室镜下第三脑室造瘘术伴或不伴脉络丛灼烧。DWS 的最佳治疗方法一直以来备受争议,并且缺乏大样本数据的对比,因此需要开展大量的随机对照试验进行验证。本文旨在总结DWS 的临床症状及治疗方法,为诊断及治疗方案的选择提供参考。

Dandy-Walker syndrome(DWS) is a rare congenital disorder of the central nervous system,usually manifested by hydrocephalus. The typical anatomical features of DWS are hypoplasia of the cerebellar vermis, anteroposterior enlargement of the posterior fossa upward displacement of the sinus ring and transverse sinus, and cystic dilation of the fourth ventricle. Clinical symptoms can be manifested as a series of brain-related symptoms such as epilepsy, gait instability, speech dysfunction, mental symptoms and eye symptoms, and the clinical manifestations can exist alone or in combination. Current treatments include posterior fossa cyst resection, ventriculoperitoneal shunt, cyst peritoneal shunt, third ventriculotomy with or without choroid plexus burn under ventriculoscopy. The best treatment for DWS has been controversial for a long time, and there is a lack of comparison of large sample data, so a large number of randomized controlled trial are needed, This article aims to summarize the symptoms and treatments of DWS, and to provide reference for diagnosis and treatment options.