The term “portal biliopathy” (PB) refers to abnormalities of the entire biliary tract, including intrahepatic and extrahepatic bile ducts, cystic duct, and gallbladder in patients with portal hypertension .These changes are believed to be caused by the pressure of the venous collaterals on both the intrahepatic and extrahepatic bile ducts and the gallbladder. More recently, PB due to extrahepatic portal venous obstruction (EHPVO) has been renamed “portal cavernoma cholangiopathy (PCC),” by the Indian National Association for Study of the Liver (INASL) task force . PCC in adults is asymptomatic in 81–100%. Despite EHPVO being a non-cirrhotic disease with a favorable outcome, the cholangiopathy is identified only when the patient becomes symptomatic with jaundice or cholangitis (biliary strictures or calculi) as seen in 5–38% of adults . However, once symptoms ensue, the course of illness becomes tenacious, with the progressive worsening necessitating biliary decompression, shunt surgery, or even liver transplantation in terminal stages. PB is also known to occur in cirrhosis (0–33%) and idiopathic portal hypertension (9–40%).
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