目的探讨全前脑综合征(HPE)的临床病理特点。方法回顾性分析13例HPE病例,以病理解剖、彩色多普勒超声所见为诊断依据。结果我院近3年内共发现HPE病例13例;病理解剖学分型:无叶全前脑10例,半叶全前脑3例;合并畸形:脊柱异常1例,室间隔缺损1例,巨输尿管1例,指畸形1例,小头畸形4例,肾盂积水1例,Dandy-Walker综合症1例。结论 :全前脑合并严重畸形率较高,死亡率高。 Objective To investigate the clinicopathological features of whole forebrain syndrome (HPE). Methods Retrospective analysis of 13 cases of HPE cases, pathological anatomy, color Doppler ultrasound as the basis for the diagnosis. Results A total of 13 cases of HPE were found in our hospital in the past 3 years. Pathological anatomy showed that there were 10 cases of non-leaf anterior frontal brain and 3 cases of half anterior whole forebrain. Combined deformity: 1 case of spinal abnormality, 1 case of ventricular septal defect, 1 case, 1 case of deformity, 4 cases of microcephaly, 1 case of hydronephrosis and 1 case of Dandy-Walker syndrome. Conclusion: The rate of severe deformity in the whole forebrain is high and the mortality rate is high.