CONGENITAL HEPATOPORTAL ARTERIOVENOUS-FISTULA

Background. Two children with congenital hepatoportal arteriovenous fi stulas have been investigated and treated surgically. These cases have been reviewed with five cases previously reported. Methods. Two child ren, 5 months and 14 months of age, presenting with failure to thrive, hepatosplenomegaly, ascites, and recurrent gastrointestinal bleeding with evidence of portal hypertension, were found to have congenital he patoportal arteriovenous fistulas. Results. Doppler ultrasonographic e xamination was important in identifying abnormal portal venous flow. A ngiogram identified the fistulas, confirming the diagnosis. Both patie nts had significant portal hypertension (pressure more than 30 mm Hg). Surgical resection in one child was unsuccessful, but surgical ligati on of the hepatic artery controlled the symptoms in both patients. Con clusions. Rapid collateralization of the hepatic arterial blood supply made embolization a short-term therapeutic measure, and surgical liga tion of the hepatic artery is the treatment of choice pr congenital he patoportal arteriovenous fistulas.