Portal cavernoma associated cholangiopathy (PCC) is an uncommon disease in western countries. We describe our experience in seven patients with PCC, in particular the endoscopic management. We describe the mode of presentation, frequent symptoms and the outcome of different treatment modalities of patients with symptomatic PCC.

Prospectively maintained database was reviewed at a large tertiary referral unit in London, UK. Data included therapeutic interventions, outcomes and complications.

Seven patients with PCC were followed for a median of 87 months [interquartile range (IQR), 62–107.5]. Causes of EHPVO included (hypercoagulable status, n=2, peritoneal tuberculosis n=1, neonatal sepsis, n=1, idiopathic, n=3). Acute cholangitis constituted the most recurring complications in all patients during the disease course. Endoscopic intervention was deemed required in all patients for biliary decompression, with 5 out 7 patients managed with repeat endoscopic sessions, (total=23 ERCPs). Surgical portal decompression (meso-caval shunt) was successfully performed in one patient and another patient underwent liver transplantation for decompensated liver cirrhosis. When endoscopic intervention was indicated, a fully covered self expanding metal stent (FcSEMS) provided a longer “symptoms free” period when compared to plastic stent, 7.5 (IQR, 4.75–18.25) and 4 (IQR, 3.5–7) months respectively, P=0.03. Bile duct bleeding occurred in two patients during ERCP procedure, however none of the patients had spontaneous haemobilia. Both patients were successfully treated by FcSEMS.

Acute cholangitis is a common presentation and recurrent complication during the disease course. Spontaneous haemobilia seems to be uncommon, however it is a significant potential hazard during endoscopic intervention. Insertion of FcSEMS may remodel choledochal varices and provide a longer “symptoms free” period compared to plastic stents.