Abernethy malformation

Case contributed by Dr Vincent Tatco

Presentation

Lower gastrointestinal bleeding secondary to colonic varices

Patient Data

Age: 30
Gender: Female
Modality: CT

The portal venous system is absent.  There is a large caliber, vertically oriented vein along the left hemiabdomen (giant inferior mesenteric vein) with superior mesenteric and splenic venous tributaries superiorly and draining inferiorly into both internal iliac veins.  The tributaries of the mesenteric veins are engorged.  Mesenteric phleboliths are also seen.  These findings are compatible with Abernethy malformation.  

Probable hepatic segment V hemangioma and right simple renal cyst are also demonstrated.

Modality: DSA (angiography)

Catheter angiography confirms the absence of the portal vein.  The superior mesenteric and splenic veins drain into a markedly dilated inferior mesenteric vein, which drains inferiorly into both internal iliac veins then into the common iliac veins and inferior vena cava.

Case Discussion

Abernethy malformation, also known as congenital extrahepatic portosystemic shunt, is a rare malformation in which the intestinal and splenic venous blood bypasses the liver and drains into systemic veins through a complete or partial shunt.  This congenital anomaly may present in children or in adults and are often found following abdominal imaging that was ordered for other reasons.  Clinical presentation may include neurodevelopmental, hepatic, and cardiopulmonary manifestations.  Portosystemic encephalopathy and gastrointestinal bleeding occur only occasionally.  The decision to provide an intervention to close the shunt should be based on the presence of deleterious clinical manifestations.  Current therapeutic options include interventional radiology embolization or closure devices, surgical ligation and liver transplantation. 

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Case Information

rID: 42006
Case created: 31st Dec 2015
Last edited: 14th Nov 2016
Inclusion in quiz mode: Included

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