A Portal-Rex Shunt Using Patent Proximal Main Portal Vein as Venous Inflow and Internal Jugular Vein as Conduit

Background: Extrahepatic portal vein obstruction (EHPVO) accounts for more than two thirds of pediatric portal hypertension. Rex shunt is the preferred surgical management, as it restores hepatopetal flow and minimizes or reverses liver dysfunction. Case Summary: We report surgical treatment of EHPVO in a 9-year-old girl using portal-Rex shunt with internal jugular vein (IJV) as a conduit and the intact proximal main portal vein instead of the superior mesenteric vein as a venous inlet. Improvement in thrombocytopenia and reduction in splenic size were achieved post-operatively. The portal-Rex shunt remains patent with good hepatopetal flow at one year post-operation. Conclusions: The success of a portal-Rex shunt to selectively bypass EHPVO rests upon careful selection of inlet and outlet veins, as well as a conduit with good patency, such as the IJV.