Portal biliopathy: multidisciplinary management and outcomes of treatment

Portal biliopathy (PB) is a rare condition in which portal hypertension because of extrahepatic portal vein obstruction can lead to biliary abnormalities, with some patients developing obstructive jaundice. At present, there is no international consensus on the management of PB. We present the experience of an Australian tertiary referral hospital with the diagnosis and management of PB, and compare this with reported international experience.

The records of nine patients presenting with PB between June 2003 and March 2012 were reviewed and analysed.

All patients had portal hypertension because of portal vein thrombosis, with seven patients showing cavernous transformation of the portal vein. Biliary abnormality presented with jaundice (3/9), abdominal pain (2/9) or without symptoms (3/9). All patients developed a cholestatic pattern of liver function tests (LFTs). First-line endoscopic management was employed in 7 of 8 symptomatic patients. Four patients required endoscopic management alone (sphincterotomy alone (1/9), single stent (2/9), repeated stent changes (1/9) ), while four required second-line surgical intervention (portosystemic shunt (1/9), bilioenteric anastomosis (3/9) ). All patients were well, with stable LFTs, at median 18-month follow-up, with two patients undergoing regular stent changes, and the remainder requiring no further intervention.

PB can be managed successfully with endoscopic therapy as the first-line option, but a multidisciplinary approach is necessary, with second-line surgical intervention often required. We recommend a management algorithm similar to that presented in the UK PB literature, and confirm that bilioenteric anastomosis can be performed successfully without prior portal decompression.