Presentation
Work up for chronic anemia
Patient Data
CT study shows a small stellar dilated vascular lesion at the superior aspect of the right hepatic lobe measures 1.5 cm, appearing to communicate with both peripheral right portal and hepatic veins branches. It elicits low density in the non-enhanced CT study and homogenously enhanced in the Porto-venous phase
Annotated images highlight the findings; the yellow circle highlights the shunt, the red arrow points to the right portal vein, the orange arrow points to the hepatic vein, and the green arrow points to the IVC
Case Discussion
Portosystemic shunts are usually acquired and rare to be congenital. Secondary causes such as portal hypertension, cirrhosis, portal vein thrombosis, previous liver biopsy, or trauma. If there is no history or evidence of any of these conditions, the term "congenital portosystemic shunt" could be applied. Congenital portosystemic shunts may be extrahepatic (Abernethy malformation) or intrahepatic.
congenital intrahepatic portosystemic shunt classification
- type 1: a single large vessel of constant diameter connecting the right portal vein to the inferior vena cava
- type 2: localized, peripheral shunt with one or more communications in a single hepatic segment
- type 3: peripheral portal and hepatic veins connected through an aneurysm
- type 4: multiple communications between the peripheral portal and hepatic veins in several segments
- type 5: persistent ductus venosus
In our case, the lesion is incidentally discovered with no any history or evidence of a secondary cause consistent with congenital intrahepatic portosystemic shunt type 3