2022 年第 1 期 第 17 卷

先天性儿童肝动脉-门静脉-肝静脉瘘1例

A case of congenital hepatic artery-portal vein-hepatic vein fistula in children

作者：成小蓉李岑姜军吴大琴莫连芹刘洪栋岳韵石娟黄栋

英文作者：Cheng Xiaorong Li Cen Jiang Jun Wu Daqin Mo Lianqin Liu Hongdong Yue Yun Shi Juan Huang Dong

单位：贵州省人民医院儿童重症医学科，贵阳550002

英文单位：Department of Pediatric Critical Medicine Guizhou Provincial People′s Hospital Guiyang 550002 China

关键词：先天性肝内门体静脉分流；肝动脉-门静脉-肝静脉瘘；影像学检查

英文关键词：Congenitalintrahepaticportosystemicvenousshunt；Hepaticartery-portalvein-hepaticveinfistula；Imagingexamination 

• 摘要：

• 1例4个月男性患儿因“咳嗽1周，加重伴喘息5 d”收住院治疗，查体心前区可闻及Ⅲ级连续性杂音，行腹部血管数字减影血管造影检测，明确诊断肝左动脉-门静脉左支-肝左静脉瘘，行左半肝切除术后各方面恢复可。本例患儿的特征性表现是出生后反复肺炎，因瘘口较小或者检查者经验有限，导致患儿诊断滞后，先天性肝内型门静脉-肝静脉瘘属门体静脉分流中罕见的血管畸形，而在此基础上合并肝动脉瘘更为罕见，临床诊疗中需多加注意。

• A 4-month-old boy was hospitalized because of "cough for 1 week and aggravation with wheezing for 5 d". Grade Ⅲ continuous murmur could be heard in the precordial area. Digital subtraction angiography was performed to diagnose left hepatic artery-left portal vein-left hepatic vein fistula. Left hemihepatectomy was performed and all aspects recovered. The characteristic manifestation of this case was recurrent pneumonia after birth. Because the fistula was small or the examiner′s experience was limited, the diagnosis lagged behind. Congenital intrahepatic portal vein-hepatic vein fistula was a rare vascular malformation in portosystemic shunt. On this basis, hepatic artery fistula was more rare, so more attention should be paid to clinical diagnosis and treatment.