Two-dimensional ultrasonography and CEUS of a case with primary biliary cholangitis and vanishing bile duct syndrome

J Clin Ultrasound. 2022 Nov;50(9):1368-1372. doi: 10.1002/jcu.23350. Epub 2022 Sep 23.

Abstract

Vanishing bile duct syndrome (VBDS) is a pathological concept that refers to the gradual reduction, destruction and disappearance of intrahepatic bile ducts caused by drugs, immunity, malignancy, and infections (including HIV and tuberculosis). Its clinical manifestation is cholestasis. The pathological diagnostic criteria for VBDS are the occurrence of intralobular vanishing bile ducts in more than 50% of 10 or more portal areas. At present, the diagnosis of VBDS still relies on liver biopsy. Contrast-enhanced ultrasound has been widely used in the diagnosis of liver-related diseases. The intravenous injection of microbubbles could enhance the observation of tissue microcirculation and significantly expand the possibility of ultrasound hemodynamic research. VBDS is a rare disease, and there are few reports on the early ultrasound and CEUS manifestations. The purpose of this report is to explore the unique performance of ultrasound and CEUS in the diagnosis of VBDS.

Keywords: contrast-enhanced ultrasound; ultrasound; vanishing bile duct syndrome.

Publication types

  • Case Reports

MeSH terms

  • Bile Ducts
  • Bile Ducts, Intrahepatic / pathology
  • Cholestasis* / diagnosis
  • Cholestasis* / etiology
  • Cholestasis* / pathology
  • Humans
  • Liver Cirrhosis, Biliary* / complications
  • Liver Cirrhosis, Biliary* / pathology
  • Ultrasonography / adverse effects