Rapidly Progressive Portal Cavernoma Cholangiopathy in a Patient With Infeasible Decompressive Shunt Surgery.

We present a 27-year-old man with a 2-year history of extrahepatic portal vein obstruction and selective immunoglobulin A deficiency, referred for acute cholangitis from portal cavernoma cholangiopathy (PCC). Because recurrent cholangitis rapidly led to liver failure, orthotopic liver transplantation (OLT) was successfully performed. To date, this is one of the few cases of patients with symptomatic PCC who required OLT and the first case who had a successful 6-year follow-up.