Hepatic arterioportal fistula after biopsy conditioning portal hypertension: first case in primary biliary cholangitis and a systematic literature review

Hepatic arterioportal fistulas (HAPFs) are aberrant shunts between the portal venous and the hepatic arterial systems leading to portal hypertension (PH). Acquired post-biopsy HAPFs are rare and most of them resolve spontaneously with no clinical manifestations. If symptomatic, they should be treated, and endovascular embolization represents the standard of care. We present the first case of an acquired high-flow post-biopsy HAPF causing clinically significant PH and PH-related bleeding in a 26-year-old female patient affected by early-stage primary biliary cholangitis (PBC). The HAPF was safely and successfully treated with selective arterial embolization through spring steel coils and liquid embolizing agents. In fact, no peri-procedural complications were reported, and final result showed the complete closure of the shunt at control abdominal Doppler ultrasound, with almost complete regression of PH-related endoscopic signs. Despite the possibility of developing PH even in early histologic stages of PBC, we attributed the most significant proportion of PH to the high-flow HAPF and we proved that its embolization was needed to be prioritized in the therapeutic management of the case.