Living Donor Liver Transplantation with Renoportal Anastomosis for a Patient with Congenital Absence of the Portal Vein

Hajime Uchida,Seisuke Sakamoto,Takanobu Shigeta,Atsuko Nakazawa,Mureo Kasahara,Chiaki Karaki,Ikumi Hamano,Hiroyuki Kanazawa,Akinari Fukuda

doi:10.1155/2012/670289

Abstract

A congenital absence of the portal vein (CAPV) is a rare disorder that may lead to an intrapulmonary shunt. A 14-year-old male with CAPV underwent living donor liver transplantation with a left lobe graft from his father. The portal vein reconstruction was achieved with a renoportal anastomosis using an interpositional graft from the native collateral vein, because portal venous system directly drains to the left renal vein without constructing the confluence of superior mesenteric vein and splenic vein. The patient is doing well with a normal liver function and mild hypoxemia.

Highlights

A congenital absence of the portal vein (CAPV) is a rare disorder that may lead to an intrapulmonary shunt
The complete portosystemic shunt not perfusing the liver via portal vein, defined as type I, is especially rare, but recently liver transplantation has been recognized as a curative operation for symptomatic CAPV type I patients with uncontrollable hepatic encephalopathy, pulmonary hypertension, and intrapulmonary shunt and become increasingly reported
CAPV is a rare disorder that may lead to hepatic encephalopathy and intrapulmonary shunting (IPS)

Summary

Introduction

A congenital absence of the portal vein (CAPV) is a rare disorder that may lead to an intrapulmonary shunt. The complete portosystemic shunt not perfusing the liver via portal vein, defined as type I, is especially rare, but recently liver transplantation has been recognized as a curative operation for symptomatic CAPV type I patients with uncontrollable hepatic encephalopathy, pulmonary hypertension, and intrapulmonary shunt and become increasingly reported

Case Report

Findings

Discussion