Portal cavernoma cholangiopathy: A systematic review of current understanding, clinical significance, and management

作者：Elisa Pinto, Laure Elkrief, Virginia Hernández–Gea, A. Busatto, Kymentie Ferdinande, Alberto Zanetto, Akash Shukla, Marco Senzolo · 发表于：Hepatology Communications · 年份：2025 · DOI：10.1097/hc9.0000000000000833 · 被引用次数：4 · 研究领域：Liver Disease and Transplantation、Abdominal vascular conditions and treatments、Liver Disease Diagnosis and Treatment

Portal cavernoma cholangiopathy (PCC) is a rare condition characterized by abnormalities of the intrahepatic and extrahepatic bile ducts and the gallbladder, resulting from long-standing chronic portal vein thrombosis (cPVT). These vascular changes often occur in the setting of non-cirrhotic, non-neoplastic portal hypertension. Its prevalence among cPVT patients ranges from 4% to 92%, with an unweighted average of 45.2%, increasing to 67.0% in studies using magnetic resonance cholangiopancreatography (MRCP) or endoscopic retrograde cholangiopancreatography (ERCP). Although most patients remain asymptomatic, about 25% develop symptoms. Diagnosis mainly depends on imaging, with MRCP being the most accurate choice. The classification proposed by Llop and colleagues provides a useful framework by integrating anatomical involvement with the severity of biliary disease. However, combining this classification with the pathophysiological one could support a more personalized treatment approach. Management depends on symptom presentation. Asymptomatic patients are managed conservatively. Symptomatic cases may require endoscopic interventions; plastic stents are most commonly used, although they have around a 30% risk of hemobilia and a high re-intervention rate of ~50%. Interventional radiology techniques, particularly portal vein recanalization with transjugular intrahepatic portosystemic shunt (TIPS), exhibit high technical success rates (median 91%) and patency rates (median 81.8%)...