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

Abstract

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%). Surgical portosystemic shunts remain a key option for a subgroup of patients, with about 65% achieving symptom resolution after decompression alone, whereas liver transplantation is rarely indicated. Given the rarity of this condition and the limited quality of existing studies, we conducted this systematic review to provide a comprehensive overview of the current literature and to promote a more standardized approach to managing PCC. This review aims to fill knowledge gaps and enhance consistency in treatment strategies for this complex condition.

Keywords: TIPS; portal biliopathy; portal hypertension; portal vein thrombosis; shunt.

FIGURE 1

Flowchart of literature search. Abbreviation: PCC, portal cavernoma cholangiopathy.

FIGURE 2

Timeline showing the evolution of PCC nomenclature and definitions over time. Abbreviations: PB, portal biliopathy; PCC, portal cavernoma cholangiopathy.

FIGURE 3

Venous drainage of the common bile duct. Created with BioRender.com. Last accessed: July 18th, 2025.

FIGURE 4

Radiological findings in patients with PCC. Created with BioRender.com. Last accessed: July 18th, 2025. Abbreviation: CBD, common bile duct.

FIGURE 5

Prevalence of bile duct stenosis, common bile duct stones, and varices has been reported in studies with outcomes not focused on treatment. Abbreviation: CBD, common bile duct.

FIGURE 6

Clinical stages of PCC. Created with Biorender.com. Last accessed: July 18th, 2025. Abbreviation: PCC, portal cavernoma cholangiopathy.

FIGURE 7

Proposed algorithm for PCC management. Created with Biorender.com. Last accessed: July 18th, 2025. Abbreviations. ES, endoscopic sphincterotomy; LT, liver transplantation; PCC, portal cavernoma cholangiopathy; PH, portal hypertension; PSS, portosystemic shunts; PTBD, percutaneous transhepatic biliary drainage; SE, stone extraction; SEMS, self-expanding metal stent; TIPS, transjugular intrahepatic portosystemic shunt; UDCA, ursodeoxycholic acid.