7. Congenital hepatic fibrosis

Page created on April 26, 2020. Last updated on April 29, 2020 at 11:02

Staining: HE

Organ: Liver


In the slide we can se normal parenchyme.

In the background we can se a hypercellular eosinophilic area containing fibrosis and abnormal bile ducts.

Hypercellular eosinophilic area showing fibrosis and abnormal bile ducts

Diagnosis: Congenital liver fibrosis

Theory: Fibropolycystic liver disease is a collective term for a group of congenital liver and biliary abnormalities resulting from abnormal development of the ductal plates. Diseases in this group include:

  • Congenital liver fibrosis (recessive polycystic liver disease)
  • Caroli syndrome
  • Von Meyenburg complex
  • dominant polycystic liver disease

    DD between Caroli syndrome and congenital liver fibrosis is done by the presence of bile in the abnormal bile ducts and cholestasis, which can be seen in the former. Congenital liver fibrosis is accociated with polycystic kidney disease.

    Written by G cobra

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