The Silent Liver Function Mayhem: Unrevealing The Mystery Of Vanishing Bile Duct Syndrome

Vanishing Bile Duct Syndrome (VBDS) is a rare form of cholestatic liver disease that is characterized by the destruction of intrahepatic biliary tree segments. For this narrative review to explore the clinical spectrum, pathophysiology, and outcomes of VBDS, a comprehensive literature search was performed using PubMed, google scholar and other academic databases from year 1995-2025. It was identified that hepatic conditions that can lead to VBDS are primary biliary cholangitis (PBC) and primary sclerosing cholangitis. Our main focus was on non-hepatic aetiologies that have been linked to VBDS such as neoplastic illnesses (especially lymphoma) or drug-induced liver damage (DILI). The diagnosis of VBDS is confirmed by ductopenia, which is a 50% decrease of the interlobular bile ducts on liver biopsy. Significant hepatic inflammation and damage were also observed in chronic active hepatitis. Management centres on withdrawal of the offending agent with added symptomatic relieve with Rifampin, Cholestyramine and ursodeoxycholic acid. High doses of ursodeoxycholic are also beneficial to relieve symptoms of pruritis and improving biochemical markers. In severe cases liver transplant is the only choice.