摘要： 目的 总结Dandy-Walker综合征患儿腹腔分流术前后临床与影像学特征。方法与结果 选择2016年1月至2023年1月福建三博福能脑科医院收治的共6例Dandy-Walker综合征患儿（包括Dandy-Walker畸形1例、Dandy-Walker变型5例），主要表现为小脑蚓部缺损，小脑幕、窦汇区上移，第四脑室显著扩张，后颅窝囊性病变，幕上脑室扩张、积水（1例）；或小脑蚓部发育不良，小脑半球受压，第四脑室扩张，后颅窝囊性病变，幕上脑室扩张、积水（5例）。分别行右侧侧脑室-腹腔分流术（2例）或后颅窝囊肿-腹腔分流术（4例），手术顺利，无手术相关并发症。随访2.31~7.00年，术后脑积水和后颅窝囊性畸形均逐渐减轻，3例预后良好，大脑半球和小脑发育良好，言语功能、运动功能、智力正常；3例预后一般，遗留轻度小脑平衡障碍（1例）或孤独症、幽闭恐惧症（1例），合并神经皮肤黑变病患儿遗留言语功能、运动功能、智力障碍（1例）。结论 表现为脑积水的Dandy-Walker综合征患儿经单分流术纠正脑积水后，大脑结构可获得良好发育，其中Dandy-Walker变型小脑蚓部良好发育，而Dandy-Walker畸形表现为小脑蚓部缺损时是否发育需更多病例观察。

关键词: 丹迪-沃克综合征, 脑室腹膜分流术, 磁共振成像, 儿童

Abstract: Objective To summarize the clinical and syndrome (DWS) in children before and after peritoneal shunt. Methods and Results All 6 children with DWS admitted to Fujian Sanbo Funeng Brain Hospital from January 2016 to January 2023 were selected as the objects of observation, including one case of Dandy-Walker malformation (DWM) and 5 cases of Dandy- Walker variant (DWV). Before surgery, the main manifestations were cerebellar vermis defect, upward movement of the tentorial and sinusional area, severe dilatation of the fourth ventricle, posterior cranial fossa cystic lesions, supratentorial ventricle dilatation and hydrocephalus (one case); or cerebellar vermis dysplasia, cerebellar hemisphere compression, the fourth ventricle dilatation, posterior cranial fossa cystic lesions, supratentorial ventricle dilatation and hydrocephalus (5 cases). Right lateral ventriculoperitoneal shunt (2 cases) or posterior cranial fossa cyst-peritoneal shunt (4 cases) were performed, and the operations were successful without surgery-related complications. After 2.31- 7.00 years of follow-up, 6 cases of hydrocephalus and posterior cranial fossa cystic malformation were gradually relieved, 3 cases of good prognosis, cerebral hemisphere and cerebellum development, speech, movement and intelligence normal; there were 3 children with general prognosis, including cerebellar disequilibrium symptoms (one case), autism, claustrophobia (one case), and speech, motor and intellectual disorders (one case) complicated with neurocutaneous melanosis. Conclusions The brain structure of the children with DWS presented with hydrocephalus can be well developed after the hydrocephalus is corrected by single shunt. Among them, the cerebellar vermis of the DWV is well developed, while the development of the DWM presented with cerebellar vermis defect needs to be observed in more cases.

Key words: Dandy-Walker syndrome, Ventriculoperitoneal shunt, Magnetic resonance imaging, Child