Case Reports

Use of angio-CT for evaluation of the intrahepatic portal vein in a case of congenital absence of the portal vein（CAPV）

Gen Matsuura, Takeshi Saito, Tomoro Hishiki, Keita Terui, Yoshiharu Sato
Noriyuki Yanagawa¹⁾, Hirotaka Sato¹⁾, Hideo Yoshida
Department of Pediatric Surgery, Graduate School of Medicine, Chiba University
Devision of Radiology, Chiba University Hospital¹⁾

Abstract

　　Congenital absence of the portal vein（CAPV）is a rare abnormality in which the mesenteric venous blood flow drains directly into the systemic circulation. Evaluation of the intrahepatic portal vein is necessary for the correct diagnosis and proper therapy. The treatment can be closure of the shunt vessel or liver transplantation. We report here the case of the one-year-old boy with CAPV. He was referred to our clinic because of worsening hyperammonemia. We performed US, three-dimensional CT, and angiography with occlusion test and concomitant angio-CT. The results showed the shunt vessel communicating between the mesenteric vein and inferior vena cava and no intrahepatic portal vein branch fed from the shunt vessel. He was diagnosed as CAPV Abernethy type 1. Angio-CT was especially useful to evaluate the presence of the intrahepatic portal vein. It could also help to reveal the collateral veins outside the liver which had been misdiagnosed as parts of intrahepatic portal branches by simple angiographic imaging. We recommend to use angio-CT for the evaluation of intrahepatic portal venous flow in CAPV patients.

Keywords：Congenital absence of the portal vein, Angio-CT, Porto-caval shunt,Intrahepatic portal vein, Occlusion test