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Hepatic ductopenia and vanishing bile duct syndrome

Nancy Reau, MD
Section Editor
Sanjiv Chopra, MD, MACP
Deputy Editor
Kristen M Robson, MD, MBA, FACG


Vanishing bile duct syndrome refers to a group of acquired disorders resulting in progressive destruction and disappearance of the intrahepatic bile ducts and, ultimately, cholestasis. Ductopenia (a pathologic description) refers to the associated reduction in the number of intrahepatic bile ducts, a process that ultimately leads to cholestasis.

The diagnosis is made pathologically, although it can be suggested on imaging tests such as endoscopic retrograde cholangiopancreatography or magnetic resonance cholangiopancreatography when disease is advanced. There are multiple etiologies that can result in this final common pathologic picture, including autoimmune disorders, medications, genetic abnormalities, infectious diseases, and neoplastic disorders. The prognosis and treatment depend on the etiology and degree of injury. This topic will review the anatomy, classification, pathophysiology, diagnosis, and treatment of this heterogeneous group of disorders.


The biliary tree is the tubular system of ducts that transports bile from the liver to the small intestine. It is divided into intra- and extrahepatic systems. Few disease states involve both systems, thus allowing for a natural taxonomic separation between the intra- and extrahepatic biliary tree [1].

The extrahepatic system includes the right and left hepatic ducts, cystic duct, and common bile duct.

The intrahepatic biliary tree contains ducts proximal to the right and left hepatic ducts [2]. The intrahepatic system is further divided into intrahepatic large bile ducts (segmental and area ducts) that are grossly visible, and the intrahepatic small bile ducts (septal, interlobular, ductules, and canal of Hering).

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Literature review current through: Nov 2017. | This topic last updated: Mar 07, 2016.
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