Severe portal hypertension due to congenital hepatoportal arteriovenous fistula associated with intrahepatic portal vein aneurysm.

A 13-year-old girl was referred for assessment of severe gastrointestinal tract bleeding. Her liver function tests were normal, and she had no evidence of chronic liver disease or history of significant trauma. Clinical and sonographic findings suggested the presence of a portal vein aneurysm associated with a hepatoportal arteriovenous fistula. Abdominal angiography confirmed the diagnosis. The arteriovenous fistula was congenital, and the associated portal vein aneurysm was either congenital or secondary to hemodynamic changes in the portal venous system.