Congenital absence of the portal vein: Define the portosystemic shunt, avoid liver transplantation

被引:3
作者
Laverdure, Noemie [1 ,4 ]
Lallier, Michel [2 ]
Dubois, Josee [3 ]
Paganelli, Massimiliano [1 ,5 ]
机构
[1] Univ Montreal, CHU St Justine, Pediat Gastroenterol Hepatol & Nutr, Montreal, PQ, Canada
[2] Univ Montreal, CHU St Justine, Pediat Surg, Montreal, PQ, Canada
[3] Univ Montreal, Pediat Radiol, CHU St Justine, Montreal, PQ, Canada
[4] Hop Femme Mere Enfant, Serv Hepatogastroenterol & Nutr Pediat, Lyon, France
[5] CHU St Justine Res Ctr, Liver Tissue Engn & Cell Therapy Lab, Montreal, PQ, Canada
来源
CANADIAN LIVER JOURNAL | 2021年 / 4卷 / 03期
关键词
Abernethy malformation; portal vein; portosystemic shunts; vascular malformations; EXTRAHEPATIC PORTACAVAL-SHUNT; COMPLICATIONS;
D O I
10.3138/canlivj-2020-0011
中图分类号
R57 [消化系及腹部疾病];
学科分类号
摘要
Liver transplantation has been historically recommended for patients with congenital absence of the portal vein associated with extrahepatic congenital portosystemic shunts. Here, based on a case report of a 2-year-old girl and a thorough review of all published cases from 1974 to 2020, we show that such a diagnosis most often conceals a hypoplastic portal vein, which can be successfully re-permeabilized through the closure of the shunt in order to re-establish a physiological vascular anatomy. This highlights the importance of achieving a detailed anatomical description of extrahepatic congenital portosystemic shunts with a balloon occlusion test in order to plan the best surgical approach and avoid unnecessary liver transplantation.
引用
收藏
页码:322 / 327
页数:6
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