Fistulae between the hepatic artery and portal vein, known as hepatoportal
arteriovenous fistula (HPAVF), most commonly occur secondary to trauma or m
alignancy. Congenital HPAVF is an extremely rare anomaly and only 11 pediat
ric cases have been documented to date HPAVF causes portal hypertension wit
h the reversal of flow in the portal circulation, leading to hyperemia and
congestion of the bowel, causing severe ascites, gastrointestinal bleeding,
anemia, and malabsorption. The diagnosis is first made by Doppler sonograp
hy, then confirmed by angiography. HPAVF is most effectively treated by per
forming either ligation of the hepatic artery or percutaneous transcatheter
embolization, We describe herein the case of an 8-month-old buy diagnosed
to have congenital HPAVF by duplex Doppler sonography and confirmed by digi
tal subtraction angiography, who was successfully managed by percutaneous t
ranscatheter embolization of the feeding right hepatic artery. A review of
the previously reported 11 cases of infants with this unusual anomaly, exam
ining presentation, management, and outcome, is also presented.