Hepatic ductopenia and vanishing bile duct syndrome
- Nancy Reau, MD
Nancy Reau, MD
- Associate Director of Solid Organ Transplantation
- Section Chief, Hepatology Rush University Medical Center
- Section Editor
- Sanjiv Chopra, MD, MACP
Sanjiv Chopra, MD, MACP
- Editor-in-Chief — Gastroenterology/Hepatology
- Section Editor — General Hepatology; Gallbladder and Biliary Tract Disease
- Professor of Medicine
- Harvard Medical School
- Senior Consultant in Hepatology
- James Tullis Firm Chief
- Beth Israel Deaconess Medical Center
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 .
●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 . 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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- Embryology and development
- PATHOPHYSIOLOGY OF INTRAHEPATIC BILE DUCT INJURY
- CONGENITAL DISEASES
- IDIOPATHIC ADULTHOOD DUCTOPENIA
- NEOPLASTIC DISORDERS
- Hodgkin lymphoma
- Histiocytosis X
- Macrophage activation syndrome (MAS)
- IMMUNOLOGIC DISORDERS
- Primary biliary cholangitis
- Immune cholangitis
- Primary sclerosing cholangitis
- Chronic graft versus host disease
- Acute hepatic cellular rejection
- Chronic hepatic cellular rejection
- Enteric organisms
- Viral infections
- TOXINS AND DRUGS
- ISCHEMIC CAUSES
- CLINICAL MANIFESTATIONS
- Laboratory evaluation
- Liver biopsy
- Ursodeoxycholic acid
- SUMMARY AND RECOMMENDATIONS