Hepatic Arterioportal Fistula: A Curable Cause of Portal Hypertension in Infancy

Hepatic arterioportal fistulae are a rare cause of portal hypertension. The case is reported of a two-year old girl with a congenital arterioportal fistula, who presented with splenomegaly and ascites. Colour doppler ultrasound showed a large shunt between the left hepatic artery and a branch of the left portal vein, producing a reversal of flow in the main portal vein. She was treated by a formal left hemihepatectomy, which has been successful in eliminating the fistula and its consequent portal hypertension in the long term. The literature regarding arterioportal fistulae and their treatment is reviewed.