Vanishing Bile Duct Syndrome: A Differential Diagnosis for Painless Jaundice

Abstract

Vanishing bile duct syndrome (VBDS) is a clinicopathological term coined to describe an acquired liver disease characterised by progressive destruction and disappearance of intrahepatic biliary ducts. We report the case of a 69-year-old female who presented with painless jaundice, fatigue, and weight loss. Initial blood workup revealed hyperbilirubinemia, transaminitis, elevated alkaline phosphatase, and a raised international normalised ratio. Para-aortic lymphadenopathy on computed tomography of the abdomen was biopsied to confirm the diagnosis of Hodgkin's lymphoma. Vanishing bile duct syndrome is a paraneoplastic phenomenon of Hodgkin's lymphoma, a recognised cause of cholestatic jaundice, and our patient's liver biopsy was diagnostic of the same. Despite treatment with steroids, ursodeoxycholic acid, and chemotherapy, our patient passed away. This case report aims to highlight VBDS as a differential diagnosis for painless jaundice in the context of cholestatic liver dysfunction. We believe reporting such cases irrespective of their outcome will help raise awareness of VBDS among clinicians, thus bettering the rate at which it is diagnosed and treated, thereby improving patient outcomes.

Keywords: cholestasis; ductopenia; hodgkin’s lymphoma; painless jaundice; vanishing bile duct.

Figure 1. Para-aortic lymphadenopathy on contrast-enhanced computed tomography of the abdomen and pelvis (axial view).

Figure 2. Para-aortic lymphadenopathy on contrast-enhanced computed tomography of the abdomen and pelvis (coronal view).

Figure 3. Lobular inflammation and portal tract lacking small bile ducts (×100 hematoxylin and eosin).

Figure 4. Cytokeratin 7 stain demonstrating a lack of bile ducts and prominent biliary metaplasia of hepatocytes (×40).