Liver transplantation for portal cavernoma cholangiopathy

Portal cavernoma cholangiopathy (PCC) is a difficult clinical problem, where the portal cavernoma is both the cause of biliary obstruction and the obstacle to its safe surgical treatment. The available endoscopic and surgical treatment is successful in majority and further intervention is seldom required since the native liver is normal. PCC is not an accepted indication for liver transplantation as only a small proportion of patients will fail both endoscopic and surgical treatment and progressive liver failure is rarely seen. Secondary biliary cirrhosis as a result of long standing biliary obstruction is an accepted indication however establishing a portal inflow in these patients is often difficult and challenging. The deceased donor liver transplantation would always be preferable over living donor liver transplantation as PCC is usually a non-emergency transplant and the graft can have portal blood inflow through a conduit to even a small segment of patent portal venous system or even to a cavernoma vessel.