7. Congenital hepatic fibrosis

Overview

Staining: HE

Organ: Liver

Description:

In the slide we can se normal parenchyme.

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

Diagnosis: Congenital liver fibrosis

Hypercellular eosinophilic area showing fibrosis and abnormal bile ducts

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