AI Article Synopsis

  • Fibropolycystic liver disease encompasses a range of disorders linked to defects in the ductal plate during development, including Caroli's syndrome, polycystic liver disease, biliary hamartomas, and congenital hepatic fibrosis.
  • These conditions are often linked to kidney issues like polycystic kidney disease and medullary sponge kidney, with choledochal cysts being debated regarding their inclusion in this spectrum due to differing origins.
  • The article aims to review these liver and kidney abnormalities through various imaging techniques while connecting them to their embryological origins, which may aid in accurate diagnosis and management.

Article Abstract

Fibropolycystic liver disease is a continuum of disorders that result from insults to the ductal plate at different stages of development and are often associated with each other. Caroli's syndrome, polycystic liver disease, biliary hamartomas, and congenital hepatic fibrosis are included in this complex spectrum that also shows frequent association with renal anomalies, such as polycystic kidney disease and medullary sponge kidney. Choledochal cysts are a controversial point in this topic since they have long been considered part of this spectrum due to morphological similarities, but studies have shown different pathogenesis. This article's purpose is to review these abnormalities through a multimodality radiological perspective offering correlation with its key embryological aspects. Knowing these numerous anomalies and their possible associations may ease an accurate diagnosis and prompt management.

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Source
http://dx.doi.org/10.1053/j.sult.2022.06.008DOI Listing

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