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Page 2 of 9 Mehta et al. Hepatoma Res 2018;4:7 I http://dx.doi.org/10.20517/2394-5079.2017.35

During embryological development, the PV originates from the right and left vitelline veins between
gestational weeks 4 to 10. There is selective involution and persistence of the peri-intestinal vitelline venous
loops. The vitelline veins originally emerge from the yolk sac, cross the septum transversum, and drain into
the sinus venosus. During the 3rd to 8th gestational week, abnormal patterns of involution and persistence
may result in pre-duodenal, pre-biliary, or duplicated PV. Excessive involution can result in the absence of
the PV as seen in type 1 portocaval shunts. Type 2 portocaval shunts may develop due to persistence of the
right vitelline vein, where the shunt drains into the retrohepatic inferior vena cava (IVC), or the left vitelline
[2]
vein, where the shunt drains into the suprahepatic IVC or right atrium .

[3]
A London surgeon by the name of John Abernethy first described congenital absence of the PV in 1793
during a postmortem examination of a 10-month-old girl. Since, there have been 101 reported cases with 66%
in women and most cases being in children. Most patients presented with encephalopathy, hepatopulmonary
syndrome, or hepatorenal syndrome. Almost half of cases have liver masses at presentation such as focal
[4]
nodular hyperplasia (FNH), adenomas, hepatoblastoma, or hepatocellular carcinoma (HCC) .

[5]
In 1994, Morgan and Superina proposed a classification of portosystemic anomalies. Type 1 shunts
are characterized by the absence of intrahepatic PV. Liver is not perfused with portal blood because of a
complete shunt. A type 2 shunt is characterized as a partial shunt. The liver is perfused with portal blood
in the presence of a partial shunt to systemic circulation. The type 1 shunts are subdivided into two further
types, depending on the anatomy of the PV. The SV and the SMV drain separately into the IVC in a type
1a shunt. The SMV either drains into the IVC or the left renal vein. A confluence of SMV and SV is usually
present in a type 1b shunt, but it does not supply the liver. While type 1 shunts are managed with liver
[5]
transplant, type 2 shunts may be surgically ligated .
In this report we will review a case of congenital absence of the portal vein (CAPV) in a 51-year-old
woman who was diagnosed with HCC and had a history of Laennec’s cirrhosis and a type Ib Abernethy
malformation.

CASE REPORT
A 51-year-old female who was diagnosed in 2008 with HCC was referred to the interventional radiology
clinic from the liver transplant service. She had been managed with conventional transarterial
chemoembolization (c-TACE) on three separate occasions and she had signs of disease progression around
the prior treated areas as marked by lipiodol. Imaging revealed PV agenesis (type 1b). Her clinical course was
marked by Laennec’s cirrhosis related to alcohol abuse complicated by occasional hepatic encephalopathy
resulting in hospitalization. Limited pediatric history included only an episode of meningitis of unclear
etiology and struggles with psychiatric illness. Histologic evaluation of liver parenchyma from a biopsy
at presentation to transplant team revealed ballooning hepatocytes, mixed with collapsed hepatocytes,
Mallory-Denk bodies, and glycogenated nuclei, which can be seen in the setting of alcoholic hepatitis. These
were accompanied by bridging and pericellular fibrosis as seen after trichome staining to the extent of stage
3 or severe fibrosis. A trial of sorafenib failed due to development of a rash, fatigue and weight loss. Social
history was positive for prior alcohol abuse but patient stopped drinking 3 years after being diagnosed with
HCC.

Interventional radiology was consulted for another TACE procedure to downstage her disease to allow for
a transplant 6 years after HCC initial diagnosis. At that point her liver profile was: alkaline phosphatase
720 U/L, aspartate aminotransferase 69 U/L, total bilirubin 2.6 mg/dL, ammonia 30 mcmol/L, albumin
3.0 g/dL. Her coagulation profile was normal (international normalized ratio was 1.02). Her alpha fetal
protein (AFP) level was 357.1 ng/mL. Her Eastern Cooperative Oncology Group (ECOG) performance status
[6]
was 0. Child Pugh score was B (8) therefore she had a expected 2-year overall survival of ~57% .