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Mehta et al. Hepatoma Research 2018;4:7 Hepatoma Research
DOI: 10.20517/2394-5079.2017.35

Case Report Open Access

Congenital absence of the portal vein complicated
by hepatocellular carcinoma in the liver of an
adult woman: review of imaging, literature and
management

2
1
2
Ankit Mehta , Shree R. Venkat , Lorraine Portelance , Lynn G. Feun 2
1 Truman Medical Center, Kansas City, MO 64108, USA.
2 University of Miami Miller School of Medicine, Miami, FL 33136, USA.
Correspondence to: Dr. Shree R. Venkat, University of Miami Miller School of Medicine, 1475 NW 12th Ave, Suite C080, Miami, FL
33136, USA. E-mail: vshree@miami.edu
How to cite this article: Mehta A, Venkat SR, Portelance L, Feun LG. Congenital absence of the portal vein complicated by hepatocellular
carcinoma in the liver of an adult woman: review of imaging, literature and management. Hepatoma Res 2018;4:7.
http://dx.doi.org/10.20517/2394-5079.2017.35

Received: 4 Aug 2017 First Decision: 14 Sep 2017 Revised: 13 Jan 2018 Accepted: 18 Jan 2018 Published: 27 Feb 2018
Science Editor: Guang-Wen Cao Copy Editor: Jun-Yao Li Production Editor: Huan-Liang Wu

Abstract
We present a case of absence of the portal vein and Laennec’s cirrhosis in a 51-year-old female who was
diagnosed with hepatocellular carcinoma (HCC). Only 101 cases of this malformation of the splanchnic vasculature
have been reported of which 4 were reported to have HCC. Patient had disease progression while waiting for a
liver transplant. Patient was treated with 3 separate conventional transarterial chemoembolization procedures at
an outside hospital. At our institution, radioembolization of the right hepatic lobe was performed. She succumbed
to liver insufficiency 8 years after being diagnosed with HCC. The features of this patient’s clinical course are
reviewed.

Keywords: Hepatocellular cancer, radioembolization, abernathy malformation

INTRODUCTION
The adult liver has a complex vascular architecture composed of two distinct circulatory systems. The
liver is supplied by blood mostly from the portal vein (PV) and its intrahepatic branches, as well as the
hepatic artery and its intrahepatic branches. The PV is responsible for carrying blood from the organs
of the abdominal cavity such as the gastrointestinal tract, the spleen, pancreas, and biliary apparatus. In
conventional anatomy, the splenic vein (SV) and the superior mesenteric vein (SMV) join to form the PV.
The PV is then subdivided into right and left branches, which form small vessels throughout the liver that
[1]
eventually drain into the sinus venosus .
© The Author(s) 2018. Open Access This article is licensed under a Creative Commons Attribution 4.0
International License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted use,
sharing, adaptation, distribution and reproduction in any medium or format, for any purpose, even commercially, as long
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and indicate if changes were made.

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