Congenital Intrahepatic Arterioportal Fistula: An Unusual Cause of Portal Hypertension Treated by Coil Embolization in an Infant

Introduction

Hepatic arterioportal fistula is an unusual cause of portal hypertension (PH) and massive gastrointestinal bleeding in an infant. Arterioportal fistulas can be located in the liver or extrahepatically. They are commonly acquired and develop secondary to cirrhosis, hepatic neoplasms, blunt or penetrating trauma, percutaneous liver biopsy, transhepatic cholangiography, Kasai portoenterostomy, segmental liver transplantation, Ehlers-Danlos syndrome, Rendu-Osler-Weber syndrome and hereditary hemorrhagic telangiectasia [2], [6], [8].

Congenital arterioportal fistulas are defined as an intrahepatic communication between the systemic, usually hepatic, arterial system and the portal venous system, without any communication to the systemic venous circulation, secondary cause or primary hepatic or biliary disease, presenting before 18 years of age [6]. They are rare and constitute less than 10 % of all hepatic arterioportal fistulas.

We describe an infant with congenital intrahepatic arterioportal fistula (IHAPF) who presented with massive gastrointestinal bleeding episodes and was treated successfully by coil embolization to stress the importance of prompt diagnosis and effective treatment.

References

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