| Summary: | Congenital intrahepatic shunts are uncommon anomalies consisting of abnormal connections between the hepatic arteries, portal veins, hepatic veins or systemic venous system. It can be found incidentally or in symptomatic infants.
Intrahepatic arterioportal fistula is caused by an abnormal connection with hepatic artery and portal vein. It is a rare entity and an uncommon cause of portal hypertension in children.
We reported a 10 month-old child with Down syndrome who presented with growth failure, hepatosplenomegaly and ascites. Imaging findings and treatment of the patient are discussed.
Keywords: Portal hypertension, arterio-portal fistula, hepato-portal fistula, congenital hepatic shunts
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