Catheter Embolization of Type II Congenital Extrahepatic
Portosystemic Shunt with Hematochezia: A Case Series
and Review of the Literature
Received: 28 November 2017 / Accepted: 20 April 2018 / Published online: 24 April 2018
Ó Springer Science+Business Media, LLC, part of Springer Nature and the Cardiovascular and Interventional Radiological Society of Europe
Abstract Hematochezia is a rare clinical presentation of
congenital extrahepatic portosystemic shunt (CEPS). We
describe a series of three patients with type II CEPS pre-
senting as hematochezia that were treated by catheter
embolization, followed by a brief review of published
articles. Hematochezia of the patients was due to the giant
inferior mesenteric vein, superior rectal vein and colonic
varices. The catheter embolization was successfully
accomplished in all of the patients. After a mean follow-up
of 27 months, no serious adverse effects were observed.
For unexplained massive hematochezia, CEPS needs to be
considered as a differential diagnosis. Based on our present
results and the review of the literature, transcatheter per-
manent embolization of the giant inferior mesenteric vein
might be an effective and safe treatment for type II CEPS.
Level of Evidence Level 4, case series.
Keywords Congenital extrahepatic portosystemic
shunt Á Catheter embolization Á Inferior mesenteric
vein Á Hematochezia
Congenital extrahepatic portosystemic shunt (CEPS), also
known as Abernethy malformation, is a rare disorder
characterized by partial or complete diversion of por-
tomesenteric blood into systemic veins via congenital
shunts [1, 2]. Based on the presence or the lack of intra-
hepatic portal venous ﬂow, Morgan and Superina classiﬁed
CEPS into two types [3, 4]. In type I CEPS, intrahepatic
portal venous branches are not developed, leading to a
complete shunting of portal venous blood into the inferior
vena cava (IVC) via a ﬁstulous communication. Type II
CEPS is characterized by partial diversion of the portal
blood ﬂow into the IVC with the presence of the intra-
hepatic portal vein branches supplying the liver.
The clinical manifestation of CEPS is highly variable
and nonspeciﬁc, including hepatopulmonary syndrome,
hepatic encephalopathy, liver cirrhosis, focal nodular
hyperplasia, hepatic adenoma or cancer, and heart failure
[1–7]. Though there were a few case reports, hematochezia
is still a rare clinical manifestation of CEPS [8–12].
We report our experience of three patients with catheter
embolization in the management of the type II CEPS
characterized by hematochezia and made a brief review of
published articles on this type of patients.
A 20-year-old male presented with chronic intermittent
hematochezia every 2–3 days from 6 months after birth.
However, hematochezia appeared to be more serious in the
past 3 months. There was not any evidence of liver disease,
such as liver function, vascular structure, alcohol abuse and
& Bo Zhang
Department of Radiology, The Sixth Afﬁliated Hospital, Sun
Yat-sen University, Guangzhou 510655, China
Cardiovasc Intervent Radiol (2018) 41:1121–1127