介入封堵治疗小儿Ⅱ型Abernethy畸形合并肺动脉高压1例报告  

Transcatheter closure of rare typeⅡAbernethy malformation with pulmonary hypertension in children:A case report

作　　者：张恒 江斌[2] 方志成[1] 孟忠吉[3] 杜恩辅[4] ZHANG Heng;JIANG Bin;FANG Zhicheng;MENG Zhongji;DU Enfu(Department of Emergency Medicine,Taihe Hospital,Hubei University of Medicine,Hubei Provincial Clinical Research Center for Precision Diagnosis and Treatment of Liver Cancer,Shiyan,Hubei 442000,China;Department of Hepatobiliary and Pancreatic Surgery,Taihe Hospital,Hubei University of Medicine,Hubei Provincial Clinical Research Center for Precision Diagnosis and Treatment of Liver Cancer,Shiyan,Hubei 442000,China;Department of Infectious Diseases,Taihe Hospital,Hubei University of Medicine,Hubei Provincial Clinical Research Center for Precision Diagnosis and Treatment of Liver Cancer,Shiyan,Hubei 442000,China;Department of Interventional Radiology,Taihe Hospital,Hubei University of Medicine,Hubei Provincial Clinical Research Center for Precision Diagnosis and Treatment of Liver Cancer,Shiyan,Hubei 442000,China)

机构地区：[1]湖北医药学院附属太和医院,湖北省肝癌精准诊疗临床医学研究中心急诊医学科,湖北十堰442000 [2]湖北医药学院附属太和医院,湖北省肝癌精准诊疗临床医学研究中心肝胆胰腺外科,湖北十堰442000 [3]湖北医药学院附属太和医院,湖北省肝癌精准诊疗临床医学研究中心感染科,湖北十堰442000 [4]湖北医药学院附属太和医院,湖北省肝癌精准诊疗临床医学研究中心介入放射科,湖北十堰442000

出　　处：《临床肝胆病杂志》2023年第11期2663-2667,共5页Journal of Clinical Hepatology

基　　金：湖北陈孝平科技发展基金会基金项目(CXPJJH11800001-2018246)。

摘　　要：Abernethy又称先天性门体静脉分流畸形,临床罕见,截至2019年全球文献报道不足300例,该病可合并肺动脉高压、肝脏肿瘤、肝衰竭等严重并发症,总体预后极差,早期诊断、积极有效治疗可降低和延缓并发症发生。本文通过门静脉造影,结合球囊阻断,将原本发育不良、纤细未显影的门静脉显影,从而将原本误诊为Ⅰ型Abernethy畸形的患儿明确为Ⅱ型Abernethy畸形,进而经介入封堵治疗成功救治患儿,现报道如下。Abernethy malformation,also known as congenital portosystemic shunts,is rare in clinical practice,with less than 300 cases reported in the global literature up to 2019.The disease can have serious complications such as pulmonary hypertension,liver tumor,and liver failure and tends to have an extremely poor prognosis,and early diagnosis and active and effective treatment can reduce and delay the onset of complications.In this case,portography combined with balloon occlusion helped to display the underdeveloped slender portal vein with dysplasia,so that the child who was formerly misdiagnosed with typeⅠAbernethy malformation was diagnosed with typeⅡAbernethy malformation,and then the child was successfully treated by transcatheter closure.This article gives a detailed report of this case.

关 键 词：ABERNETHY畸形 诊断 介入封堵 治疗 

分 类 号：R726.5[医药卫生—儿科]