Intractable Rare Dis Res. 2016;5(1):58-60. (DOI: 10.5582/irdr.2016.01000)

A large spontaneous intrahepatic portosystemic shunt in a cirrhotic patient.

Qi XS, Ye C, Hou Y, Guo XZ


A spontaneous portosystemic shunt is a rare malformation of the vessels supplying the liver. This condition often leads to the development of hepatic encephalopathy due to excessive shunting of blood from the portal vein to the inferior vena cava. Some studies have suggested that the presence of spontaneous portosystemic shunts is inversely associated with the appearance of large esophageal varices. Spontaneous intrahepatic portosystemic shunts (SIPSS) are far less frequently observed than extrahepatic portosystemic shunts, which include spleno-gastric-renal shunts, mesenteric-caval shunts, and a large patent umbilical vein. Reported here is a case of decompensated liver cirrhosis with a large SIPSS without any incidence of overt hepatic encephalopathy.

KEYWORDS: Spontaneous intrahepatic portosystemic shunt, liver cirrhosis, hepatic encephalopathy, embolization

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