Vanishing Bile Duct Syndrome in an Adult Patient: Case Report and Review of the Literature

Abstract

Vanishing bile duct syndrome (VBDS) is a rare condition characterized by progressive loss, destruction, and disappearance of the intra-hepatic bile ducts, leading to cholestasis and ductopenia. The exact mechanism of development of VDBS has not been established yet. Diagnosis of VBDS mainly relies on clinical and disease related presentations, but liver biopsy is compulsory for diagnosis. Due to the low incidence reported in the literature, a standardized treatment of VDBS has not been established; hence, this rare condition must be managed at a tertiary liver referral center. Here, we report the management and treatment of VBDS of an 81-year-old woman without any history of exposure to antibiotics, neoplasms, etc.

Keywords: cholestasis; ductopenia; prednisolone; ursodeoxycholic acid; vanishing bile duct syndrome.

Figure 1

Combination or double image CT scan of the liver, without (blue arrow) and with (double blue arrow) contrast medium, which does not show significant alterations of the intra-hepatic vessels nor dilatations of the biliary ducts.

Figure 2

T2 weighted image (TR 2258 ms TE 100 ms) that does not show any alteration of caliber of the intrahepatic ducts (blue arrow).

Figure 3

Magnetic resonance cholangiopancreatography showing poor uptake of the contrast medium, especially on the left lobe of the liver (blue arrow).