Idiopathic hepatic arterio-portal fistula: report of one case

Hepatic arterio-portal fistula is a rare cause of portal hypertension in children; it is an abnormal communication of hepatic artery and portal venous system, the most common causes being trauma or malignancy. There were only 11 cases reported in English literature and were not ever reported in Taiwan. We report a 9-year old boy with idiopathic hepatic arterio-portal fistula presented as intractable hematemesis due to esophageal and gastric varices. He had received sclerotherapy twice, and Sugiura operation (resection of the lower part of esophagus, devasculization of the stomach and splenectomy). Idiopathic hepatic arterio-portal fistula was found in angiography examination and the esophageal and gastric varices disappeared after transarterial embolization (TAE). We conclude that angiography is the golden diagnostic method for portal hypertension when the etiology is hepatic arterio-portal fistula and TAE will provide immediately therapy.