Purpose or Case Report: Congenital portosystemic shunt is a rare vascular malformation that leads to severe complications. Two types are described: extrahepatic and intrahepatic. Extrahepatic shunt is represented by a direct communication of the portal trunk, or one of its branches of origin, to the inferior vena cava, or one of its branches.

We present two cases of extrahepatic shunt treated with stent-graft placement.

Case 1: Two y.o. male with cutaneous telangiectasias and liver calcifications on abdominal ultrasound. An extrahepatic portosystemic shunt was detected on color Doppler examination.

MR and catheter angiography revealed a large fistula between the main portal vein and the suprarenal inferior vena cava. The fistula was closed by deploying a balloon expandable stent-graft in the retrohepatic inferior vena cava. Five years later, the liver function was normalized and no complication was observed.

Case 2: Thirteen y.o. male referred for three hepatic lesions and an extrahepatic portosystemic shunt.

MR and angiography revealed a large fistula between the left portal vein and the inferior vena cava. It was closed by deploying a custom self-expandable stent-graft (Zenith, Cook Medical, Bloomington, IN) in the inferior vena cava. At five years follow-up, the patient is asymptomatic with a resolution of the liver lesions.
Methods & Materials:
Results:
Conclusions: Stent-graft placement in inferior vena cava to close large extrahepatic portosystemic shunt is an alternative to open surgery, especially when the fistula cannot be occluded by coil or amplatzer plugs.