A novel treatment of congenital hepatoportal arteriovenous fistula.

Congenital hepatoportal arteriovenous fistula is a rare cause of portal hypertension in young children. Unlike the acquired form, which is usually isolated and can be cured by hepatic artery (HA) embolization, recurrence of portal hypertension often occurs with congenital hepatoportal arteriovenous fistula after embolization and/or HA ligation because of early, rapid collateralization and the presence of multiple arterioportal fistula. Although long-term outcome after embolization is not known, liver transplantation has been proposed as the only option for this condition. However, portal vein and hepatic arterial anastomoses are made difficult because of the presence of portal vein arterialization and previous HA ligation, with a significantly increased risk of vascular complications. We report a case where resolution of portal hypertension has been achieved by an end-to-side portocaval shunt, to preserve the portal vein and HA for future liver transplantation, should it be required.