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Vanishing bile duct syndrome is a loose collection of diseases leading to hepatic bile duct injury and eventual ductopenia.^[1]

The presentation is dependent upon the underlying cause. The course can be rapid or chronic.

• Fatigue
• Anorexia
• Abdominal pain
• Weight loss
• Pruritus
• Hyperlipidemia
• Malabsorption
• Fat-soluble vitamin deficiencies
• Elevated alkaline phosphatase
• Elevated gamma-glutamyltransferase
• Elevated conjugated bilirubin

In fetal and neonatal life the ductal plates are remodeled. The malformations can be atretic or fibrocystic.

• Intrahepatic bile duct atresia (Alagille syndrome) (ALGS2 MIM:610205 and ALGS1 MIM:118450)
• Extrahepatic bile duct atresia

• Autosomal recessive polycystic kidney disease
• Congential hepatic fibrosis
• Caroli's disease
• Von Meyenburg complex

• Trisomy 17, 18 and 21

• Cystic fibrosis
• Alpha 1 antitrypsin deficiency
• Trihydroxycoprostanic acidemia
• Byler's disease

Bile duct injury and loss can result from autoimmune destruction. T cells recognize biliary epithelial cell antigens causing injury and eventual atresia.

• Primary biliary cirrhosis
• Primary sclerosing cholangitis
• Hodgkin's lymphoma^[2]
• Chronic graft-versus-host disease
• Drugs(chlorpromazine)/Toxins
• Ischemia

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Treatment is dependent upon the underlying cause. Treatment is supportive as it is not possible to induce regrowth of lost ducts.

• Ursodeoxycholic acid
• Immunosuppression
  • General consensus is that more studies are needed before this can be considered
• Organ transplant

• Uptodate:Hepatic ductopenia and vanishing bile duct syndrome