A Case of Arterioportal Fistula Leading to Non-cirrhotic Portal Hypertension

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Introduction: Arterioportal fistula (APF) is a rare cause of non-cirrhotic portal hypertension. This is a case of a hepatic artery-portal vein fistula presenting with recurrent variceal hemorrhage and ascites in a patient with no known liver disease. Case: A 61-year-old woman with aplastic anemia on cyclosporine, remote colon cancer status post sigmoid resection and workup notable for a percutaneous liver biopsy to evaluate a benign liver lesion as a possible metastasis 10 years prior to current presentation, initially presented with hematemesis following 2-3 weeks of increased abdominal girth and new lower extremity edema. EGD showed bleeding esophageal varices that were successfully band ligated. Over the next 6 weeks, the patient required multiple EGDs with banding for recurrent variceal bleeding, and she was then referred to our center for tranjugular intrahepatic portosystemic shunt (TIPS) evaluation. As part of the TIPS evaluation, an MRI identified a right hepatic lobe arteriovenous malformation between the right hepatic artery and portal vein with evidence of portal hypertension. Diagnostic paracentesis demonstrated a high serum ascites albumin gradient (SAAG) of 3.1. Transjugular liver biopsy with pressures was notable for a mean hepatic venous pressure gradient (HVPG) of 7.5 mmHg. The biopsy showed perisinusoidal fibrosis in zone three without cirrhosis, and intimal hyperplasia of the portal vein (arteriolization) consistent with arterioportal fistula (Figure 1). Rather than pursuing TIPS, we elected to perform coil embolization of the two right hepatic arteries supplying the fistula (Figure 2). Despite technical success, a small fistula persisted and repeat embolizations were required. Following these interventions, rebleeding has not occurred during the 1 year follow-up.Figure 1Figure 2Discussion: APFs are a rare cause of pre-hepatic non-cirrhotic portal hypertension, and can be either acquired or congenital. Acquired etiologies include penetrating hepatic trauma, splanchnic artery aneurysms, surgical complications, or they may occur spontaneously in the setting of cirrhosis or liver tumors. Small APFs can occur following percutaneous liver biopsies, but large APFs complicating a remote percutaneous biopsy, as in our case, are rare. APF should be considered in patients with non-cirrhotic portal hypertension and, when identified, treatment is embolization or surgery, not TIPS.Figure 3