积水性无脑畸形22例报道

Hydranencephaly： report of 22 cases

目的增强对积水性无脑畸形的认识，探讨积水性无脑畸形的CT分型。方法回顾性分析22例积水性无脑畸形的临床表现、CT特点和手术结果。结果22例积水性无脑畸形根据CT表现分为：幕上型17例，半球型2例，大脑发育不全型2例和后颅窝型1例。脑脊液分流术后因颅内感染死亡1例。12例幕上型病例在3-24个月内失去随访。随访超过2年的病例有4例，均为非幕上型病例，除后颅窝型病例外，生存质量都很差。结论积水性无脑畸形的CT分型可分为4型，即幕上型、半球型、大脑发育不全型和后颅窝型。脑脊液分流手术不能改善本病的预后。

Objective To improve the diagnostic accuracy ofhydranencephaly and discuss CT classification of hydranencephaly. Methods The clinical manifestations, CT features and surgical results of 22 cases that were diagnosed as hydranencephaly were analyzed retrospectively. Results According to the exhibitions of CT scan, 17 cases were classified into supratentorial type （n=17）, hemispheric type （n=2）, cerebral hypoplasia type （n=2） and posterior fossa type （n=1）. One case died of intracranial infection after by-pass operation of cerebrospinal fluid （CSF）. Follow-up of 12 cases of supratentorial type were discontinued in 3 to 24 months. Only 4 cases survived for more than 2 years and all of them belonged to non-supratentorial type. They led a very poor quality life except the case of posterior fossa type. Conclusion Hydranencephaly can be classified into 4 types： supratentorial type, hemispheric type, cerebral hypoplasia type and posterior fossa type. CSF shunting cannot improve the outcome of hydranencephaly.