目的:提高对积水性无脑畸形的诊断水平。材料与方法:分析临床和CT 诊断证实的7 例积水性无脑畸形。结果:根据CT 和MRI表现并结合病因,积水性无脑畸形可分为:幕上、半球型和脑发育不全3 型。结论:本病是脑部少见的一种先天畸形,较易延误诊断。只要加强对此病的认识,紧密结合临床,大多数能作出及时诊断。 Objective: To improve the diagnosis of hydronephrosis without brain. MATERIALS AND METHODS: Seven cases of hydrocephalus anencephaly confirmed by clinical and CT diagnosis were analyzed. Results: According to CT and MRI manifestations combined with etiology, hydrocephalus without brain deformity can be divided into three types: supratentorial, hemispheric and hypoplasia. Conclusion: The disease is a rare brain congenital malformations, more likely to delay diagnosis. As long as the understanding of the disease is strengthened and the clinical practice is closely linked, most patients can make timely diagnosis.