先天性肝外门体分流的研究进展  

Research advances in congenital extrahepatic portosystemic shunt

作　　者：王莹[1] 徐声润 余荻 杨晋辉[1] 常江[1] WANG Ying;XU Shengrun;YU Di;YANG Jinhui;CHANG Jiang(Department of Gastroenterology,The Second Affiliated Hospital of Kunming Medical University,Kunming 650101,China)

机构地区：[1]昆明医科大学第二附属医院消化内科,昆明650101

出　　处：《临床肝胆病杂志》2023年第2期450-456,共7页Journal of Clinical Hepatology

摘　　要：先天性肝外门体分流(CEPS)也称Abernethy畸形,表现为先天性门静脉与下腔静脉之间存在异常吻合。该病在临床上极为罕见,加之其症状表现多样,易漏诊误诊。本文就CEPS的发生机制、分型特点、临床表现、诊断及治疗等研究进展作一综述,以期提高对CEPS的认识,并为其诊治流程的进一步规范提供参考。Congenital extrahepatic portosystemic shunt(CEPS) is also called Abernethy malformation,with the manifestation of congenital abnormal anastomosis between the portal vein and the inferior vena cava.CEPS is extremely rare in clinical practice and has diverse clinical symptoms,which often leads to missed diagnosis and misdiagnosis.This article reviews the pathogenesis,classification,clinical manifestation,diagnosis,and treatment of CEPS,so as to improve the awareness of this disease and provide a reference for further standardization of its diagnosis and treatment process in the future.

关 键 词：先天性肝外门体分流 ABERNETHY畸形 诊断 治疗学 

分 类 号：R725.7[医药卫生—儿科]