Congenital hepatoportal arteriovenous fistula leading to segmental portal hypertension and splenic vein thrombosis.

Congenital hepatoportal arteriovenous fistulas are a rare cause of segmental portal hypertension and splenic vein thrombosis (1–4). These may be intrahepatic or extrahepatic in location and present during infancy or early childhood with portal hypertension and gastrointestinal bleeding secondary to varices, ascites, malabsorption, anemia, and congestion of the bowel (5). In childhood, arterioportal fistulas are usually intrahepatic and regarded as congenital. The congenital fistulas are generally due to cavernous hemangioma, hereditary telangiectasia, and Ehlers-Danlos syndrome. The acquired arterioportal fistulas are usually traumatic, iatrogenic (following liver biopsy), or a result of rupture of a hepatic artery aneurysm (6).