Congenital Extrahepatic Portosystemic Shunt

A 3-year-old male child with h/o of abdominal pain and USG shows hyperechoic area in liver.

Findings:

1. Portal vein: There is direct drainage of the non-branching portal vein into the retro hepatic IVC (both splenic vein and superior mesenteric vein forming acommon portal vein trunk draining into IVC) – Direct shunting blood fromthe portal venous system to the systemic venous system - Congenital portosystemicshunts (Extrahepatic)
2. An ill-defined hypodense lesion in left lobe of liver ( ultrasound revealed few (3-4) hyperechoic lesions in the liver) - nodular liver lesions, usually regenerative nodular hyperplasia

Diagnosis:

• Congenital extra-hepatic porto-systemic shunt (Type IB).
• Nodular liver lesions – likely regenerative nodular hyperplasia

Differentials:

For nodular liver lesion

• Focal nodular hyperplasia.
• Hepatocellular adenoma

Discussion:

Congenital extrahepatic portosystemic shunt (CEPS) is a condition in which the portomesenteric blood drains into a systemic vein, bypassing the liver through a complete or partial shunt.

In type 1 CEPS, there is complete diversion of portal blood into the systemic circulation (end-to-side shunt), with absent intrahepatic portal branches. Type 1 shunts are further classified into those in which the splenic vein (SV) and superior mesenteric vein (SMV) drain separately into a systemic vein (type 1a) and those in which the SV and SMV drain together after joining to form a common trunk (type 1b). In type 2 CEPS, the intrahepatic PV is intact, but some of the portal flow is diverted into a systemic vein through a side-to-side shunt
Nearly one-half of cases of CEPS are associated with nodular liver lesions, usually regenerative
nodular hyperplasia, focal nodular hyperplasia, or hepatocellular adenoma.

Details of Student:

Name:Dr. SANEESH PS

Email:[email protected]

Contact no. :7012072828

Institute/Hospital:ASTER MIMS ,Kannur

References:

•  Alonso Gamarra, Eduardo & Parrón-Pajares, Manuel & Pérez, Ana & Prieto,Consuelo & Hierro, Loreto & López-Santamaría, Manuel. (2011). Clinical andRadiologic Manifestations of Congenital Extrahepatic Portosystemic Shunts: AComprehensive Review. Radiographics : a review publication of the Radiological Society of North America, Inc. 31. 707-22. 10.1148/rg.313105070.
• Gupta P, Sinha A, Sodhi KS, Lal A, Debi U, Thapa BR, Khandelwal N. Congenital Extrahepatic Portosystemic Shunts: Spectrum of Findings on Ultrasound,Computed Tomography, and Magnetic Resonance Imaging. Radiol Res Pract.2015;2015:181958. doi: 10.1155/2015/181958. Epub 2015 Dec 13. PMID:26858845; PMCID:PMC4691495.