Plut Domen,  Zupancic Ziva,  Mali Brajovic Senja,  Kljucevsek Tomaz,  Tomazic Mojca,  Glusic Mojca,  Homan Matjaz

Final Pr. ID: Poster #: CR-006

Congenital portosystemic venous shunts (CPVS) are rare vascular abnormalities in which the portal blood drains into a systemic vein. They are results of embryogenetic alterations during the complex development of the portal system in early gestational period. Anatomically they are classified into two groups; extrahepatic and intrahepatic. Extrahepatic shunts were first described in 1793 by Abernethy and are thus also called Abernethy malformations. Presentation of CPVS can be highly variable and consequences severe.

CASE1:
An 8 year old boy was admitted to our hospital due to severe abdominal pain. Doppler ultrasound (US) revealed an abnormal intrahepatic tubular vascular structure. The computed tomography angiography (CTA) confirmed a direct vascular connection between the left portal vein and the dilated left hepatic vein. The right portal vein was small in size, indicating main portal blood flow through the left side bypassing the liver. The treatment for this intrahepatic CPVS was embolization of the shunt. The boy has been well since the procedure.

CASE2:
A routine medical check-up in a 14 year old girl discovered abnormal liver function tests. Abdominal US showed a huge mass in the right lobe of the liver and a big mass of similar structure in the epigastrium. Portal vein was not identified. CTA showed a short main portal vein connected directly to the inferior vena cava, consistent with the Abernethy malformation. The big mass in the epigastrium was shown to be a tumor in the caudate lobe of the liver. Focal nodular hyperplasia (FNH) was considered for liver lesions due to the imaging findings and known association with the Abernethy malformation. Biopsy confirmed the diagnosis. The girl is currently waiting for liver transplantation, as it is the treatment of choice for this extrahepatic CPVS. Read More

Authors:  Plut Domen , Zupancic Ziva , Mali Brajovic Senja , Kljucevsek Tomaz , Tomazic Mojca , Glusic Mojca , Homan Matjaz

Keywords:  Congenital portosystemic venous shunt, Abernethy malformation, Intrahepatic shunt, Extrahepatic shunt

Guo Chen,  Wang Qian,  Hu Li-wei,  Zhong Yu-min

Final Pr. ID: Poster #: SCI-004

The purpose of this study was to demonstrate manifestations of MSCT in Abernethy malformation and its diagnostic value. Read More

Authors:  Guo Chen , Wang Qian , Hu Li-wei , Zhong Yu-min

Keywords:  Abernethy malformation, Angiograph, Tomography

Vegunta Aishwariya

Final Pr. ID: Poster #: EDU-037

A pictorial review of imaging appearances of anatomic variations of the portal vein.
Classification and pictorial review of various congenital abnormalities of the portal vein.
Common variations and branching patterns include trifurcation of the main PV, right posterior segmental branch arising from the main PV, and right anterior segmental branch arising from the left PV. Other less common branching patterns include left PV arising from the right anterior segmental branch, duplication of PV and absent branching of PV.
Portal venous shunts may be either portosystemic shunts with abnormal communication between the portal and systemic venous systems or arterioportal shunts between portal venous system and hepatic artery.
Portosystemic shunts bypass the liver and shunt directly portal venous blood into systemic venous system.
Congenital intrahepatic portosystemic shunts are classified into four types based on Park et al. Type I, the most common type, a single vessel connects the right portal vein to the IVC. Type II in which peripheral shunt localized to one hepatic segment and single or multiple communications between branches of portal and hepatic veins. Type III with aneurysmal connection between portal and hepatic veins. Type IV with multiple communications between peripheral portal and hepatic veins in several segments.
Abernethy malformations/congenital extrahepatic portosystemic shunts can be divided into 2 major types. Type I with complete absence of intrahepatic portal venous flow and complete shunting. Type Ia with congenital absence of the portal vein and separate drainage of superior mesenteric vein and splenic vein into systemic veins. Type IIb in which superior mesenteric vein and splenic vein join to form a short extrahepatic portal vein which drains into a systemic vein. Type II with some preserved hepatic portal venous flow and partial shunting. Type IIa arising from left or right portal vein including patent ductus venosus. Type IIb arising from main portal vein. Type IIc arising from the other portal vein tributaries. Patients with both these types suffer premature mortality either due to shunting complications or other associated congenital abnormalities.
Congenital arterioportal shunts may present either as a fistula or as vascular malformations in hereditary hemorrhagic telangiectasia.
Aneurysms of the PV may represent approximately 3% of all aneurysms of the venous system. Read More

Authors:  Vegunta Aishwariya

Keywords:  Portal vein, Congenital intrahepatic portosystemic shunts, Abernethy malformations/congenital extrahepatic portosystemic shunts