congenital extrahepatic portosystemic shunt

Congenital extrahepatic portosystemic shunt, also known as Abernethy malformation, is a rare malformation in which intestinal and splenic venous blood bypasses the liver and drains into systemic veins.

Aside from the complete or near-complete absence of portal veins, other histologic features of Abernethy malformation have not been evaluated in the literature.

In addition to loss of portal veins, Abernethy malformation is characterized by multiple abnormalities due to remodeling of the hepatic vasculature.

Abernethy malformation may also be associated with hepatocellular carcinoma and focal nodular hyperplasia in some patients.

References

 Congenital extrahepatic portosystemic shunts (abernethy malformation): a histopathologic evaluation. Lisovsky M, Konstas AA, Misdraji J. Am J Surg Pathol. 2011 Sep;35(9):1381-90. PMID: 21836489