Imaging of fibropolycystic liver disease

Kedar Sharbidre, Mohd Zahid, Sudhakar Kundapur Venkatesh, Chandra Bhati, Neeraj Lalwani

Research output: Contribution to journalReview articlepeer-review


Fibropolycystic liver diseases (FLDs) make up a rare spectrum of heritable hepatobiliary diseases resulting from congenital ductal plate malformations (DPMs) due to the dysfunction of proteins expressed on the primary cilia of cholangiocytes. The embryonic development of the ductal plate is key to understanding this spectrum of diseases. In particular, DPMs can result in various degrees of intrahepatic duct involvement and a wide spectrum of cholangiopathies, including congenital hepatic fibrosis, Caroli disease, polycystic liver disease, and Von Meyenberg complexes. The most common clinical manifestations of FLDs are portal hypertension, cholestasis, cholangitis, and (in rare cases) cholangiocarcinoma. This article reviews recent updates in the pathophysiology, imaging, and clinical management of FLDs.

Original languageEnglish (US)
Pages (from-to)2356-2370
Number of pages15
JournalAbdominal Radiology
Issue number7
StatePublished - Jul 2022


  • Caroli
  • Congenital hepatic fibrosis
  • Ductal plate
  • Fibrocystic

ASJC Scopus subject areas

  • Radiological and Ultrasound Technology
  • Radiology Nuclear Medicine and imaging
  • Gastroenterology
  • Urology


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