Congenital multiple arterioportal fistula: a case report treated with portocaval shunt

A case of 48-year-old man, with diffuse arterioportal fistula (APF) considered congenital in origin, is described. The patient presented with recurring intractable variceal hemorrhage which was treated by endoscopic ligation or sclerotherapy. In this case, gastroduodenal artery and branches of mesenterial artery were diffusely involved, so these lesions could not be resected. Portocaval shunt between the superior mesenteric vein and the right iliac vein was successfully performed. Follow-up endoscopic examination one year later revealed no evidence of esophageal and gastric varices. To our knowledge, this is the second case in which a patient underwent portocaval shunt successfully for congenital APF.