The Congenital Intrahepatic Arterioportal Fistula Syndrome

Abstract

Congenital intrahepatic arterioportal fistula is a rare but treatable cause of portal hypertension for which early recognition may lead to successful radiological management. We report an infant presenting with severe failure to thrive, melena and splenomegaly due to a congenital intrahepatic arterioportal fistula, successfully ablated after multiple trials of superselective transarterial embolization. Comprehensive review of congenital cases provides an understanding of the key clinical features defining this syndrome. A classification system is proposed, upon which treatment decisions may be based.

Congenital intrahepatic arterioportal fistula presenting as severe undernutrition and chronic watery diarrhea in a 2-year-old girl

“…Intestinal dysfunction is related to venous congestion and stasis secondary to reversal of flow in the portal and superior mesenteric veins [12,27]. Furthermore, protein-losing enteropathy, steatorrhea, or evidence of fat malabsorption may occur and contribute to the malnutrition [2]. Interestingly, in our case, the development of chronic watery diarrhea occurred much earlier than the variceal bleeding, and the girl had no signs of portal hypertension on initial physical examination.…”

“…Congenital IAPF can be defined as an intrahepatic communication between the hepatic artery and the portal venous system, without any communication with the systemic venous circulation, with no secondary cause or primary hepatic or biliary disease, and presenting before 18 years of age [2]. To date, a total of 33 cases have been reported in the literature, with the first report nearly 40 years ago [12].…”

“…As a result, splenomegaly, hypersplenism, esophageal varices, and ascites commonly develop. Failure to thrive and chronic diarrhea are observed in nearly 50% of the cases of congenital IAPF [2]. Intestinal dysfunction is related to venous congestion and stasis secondary to reversal of flow in the portal and superior mesenteric veins [12,27].…”

“…The interventional radiologic procedure has a high success rate in cases of unilateral lesions with a single feeding artery [2]. Vauthey et al [15] reviewed 88 cases from the literature, with IAPF from various etiologies and at various ages and found that TAE was successful in 42% of all cases.…”

“…Most often it is acquired and develops either after trauma or iatrogenically after transhepatic interventions [1]. Congenital lesions are very rare with few cases described in the literature [2].…”