Abstract

Vanishing bile duct syndrome (or "ductopenia") is a loose collection of diseases which leads to the injury to hepatic bile ducts and eventual ductopenia.

Signs and symptoms

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

Cause | Congenital

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

Cause | Immunologic associations

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

Treatment

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