Severe gastrointestinal tract bleeding in a two-month-old infant due to congenital intrahepatic arterioportal fistula

A 2-month-old boy was referred for assessment of severe upper gastrointestinal tract bleeding and melena. On physical examination, a continuous murmur was heard over the right upper quadrant of the abdomen. A splenomegaly and dilated veins were also noted on the abdominal wall. Liver functions were normal. There was no history of trauma or jaundice. Doppler ultrasonography, magnetic resonance arteriography and angiography suggested the presence of an intrahepatic arteriovenous fistula between the phrenic artery and the portal vein.Management consisted of successful embolization by coiling of the phrenic artery.To our knowledge this is the first documented case report of a congenital fistula between the phrenic artery and the portal vein.