Portal hypertension induced by congenital hepatic arterioportal fistula: report of four clinical cases and review of the literature

Abstract

Intrahepatic arterioportal fistula (IAPF) can be caused by many secondary factors. We report four cases of portal hypertension that were eventually determined to be caused by congenital hepatic arterioportal fistula. The clinical manifestations included ascites, variceal hemorrhage and hepatic encephalopathy. Computed tomography scans from all of the patients revealed the early enhancement of the portal branches in the hepatic arterial phase. All patients were diagnosed using digital subtraction angiography (DSA). DSA before embolization revealed an arteriovenous fistula with immediate filling of the portal venous radicles. All four patients were treated with interventional embolization. The four patients remained in good condition throughout follow-up and at the time of publication. IAPF is frequently misdiagnosed due to its rarity; therefore, clinicians should consider IAPF as a potential cause of non-cirrhotic portal hypertension.

Keywords: Ascites; Intrahepatic arterioportal fistula; Portal hypertension.

Figure 1

Contrast-enhanced computed tomography revealed an earlier enhancement of the portal vein compared with the superior mesenteric vein during the arterial phase. Digital subtraction angiography indicated that there was rapid filling through the fistula into the portal vein. A: Early enhancement of the portal branches in hepatic arterial-phase; B, C: The hepatic argiography; D: The fistula reduction after embolization.

Figure 2

Digital subtraction angiography before embolization revealed the arteriovenous fistula with the immediate filling of the portal venous radicles.

Figure 3

Early enhancement of the portal branches.

Figure 4

Digital subtraction angiography revealed a diffuse arterioportal fistula with flow reversal in the portal venous system.