Portal biliopathy.

Portal biliopathy (PB) is a rare disorder, which mostly presents as sub-clinically. It occurs most commonly due to idiopathic extrahepatic portal vein obstruction. We present three cases having features of portal biliopathy secondary to portal hypertension. Our first case did not have a prior history of chronic liver disease while next two patients had previous history of chronic liver disease resulting in portal hypertension. Cavernous transformation of the portal vein due to extrahepatic portal vein obstruction is not infrequent but biliary obstruction in association with this disorder is distinctly uncommon. Proper case management is very important as prolonged biliary duct obstruction can lead to the development of ascending cholangitis or later on secondary biliary cirrhosis.