Congenital Intrahepatic Portosystemic Shunt in a Newbom Successfully Treated with Coil Embolization Via Umbilical Vein

Congenital intrahepatic portosystemic shunt is a rare entity and is mostly reported in adults. In embryonic period, the vitelline vein is broken up into the vitelline sinusoids, which become the intrahepatic portal vein branches and the hepatic veins. The portosystemic venous shunts may develop from embryonic vascular remnants, including the vitelline vein and ductus venosus. We report for the first time in Korea a case of congenital intrahepatic portosystemic shunt in a newbom infant presenting with congestive heart failure and hepatomegaly, successfully treated by coil embolization via umbilical vein.