Vanishing bile duct syndrome
Vanishing bile duct syndrome
Vanishing bile duct syndrome (VBDS) is a group of liver diseases that injure the bile ducts in the liver. Over time this can lead to ductopenia, a loss of bile ducts, which disrupts bile flow.
Signs and symptoms
- The symptoms depend on the underlying cause and can progress quickly or slowly
- Common problems include: fatigue, loss of appetite, abdominal pain, weight loss, itching (pruritus), malabsorption, and fat-soluble vitamin deficiencies
- Laboratory clues often include high alkaline phosphatase, high gamma-glutamyltransferase (GGT), and elevated conjugated bilirubin
Causes
- Congenital
- During fetal development, bile ducts may form abnormally. Malformations can be atretic (blocked) or fibrocystic.
- Congenital/Anatomic subtypes
- Intrahepatic bile duct atresia (often linked to Alagille syndrome)
- Extrahepatic bile duct atresia
- Fibrocystic conditions
- Autosomal recessive polycystic kidney disease
- Congenital hepatic fibrosis
- Caroli disease
- Von Meyenburg complex
- Chromosomal associations
- Trisomy 17, 18, or 21
- Genetic associations
- Cystic fibrosis
- Alpha-1 antitrypsin deficiency
- Trihydroxycoprostanic acidemia
- Byler’s disease
- Immunologic associations
- Autoimmune destruction of biliary cells by T cells
- Other causes
- Primary biliary cirrhosis
- Primary sclerosing cholangitis
- Hodgkin’s lymphoma
- Chronic graft-versus-host disease
- Drugs or toxins
- Ischemia
Treatment
- The approach depends on the underlying cause
- In many cases, the treatment is supportive because lost bile ducts do not regrow
- Medical options may include ursodeoxycholic acid
- Immunosuppression has limited and not well-proven benefit
- Organ transplant may be considered in some situations
This page was last edited on 1 February 2026, at 21:01 (CET).