congenital hepatic fibrosis
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See also: ductal plate malformations (DPMs)
The basic lesion of congenital hepatic fibrosis corresponds to ductal plate malformation of interlobular bile ducts, resulting from faulty development, i.e. disturbance in epithelio-mesenchymal inductive interactions. The immature bile ducts are subject to a progressive destructive cholangiopathy, resulting in a pattern of more or less advanced fetal type of biliary fibrosis.
Synopsis
hepatomegaly (hard and enlarged liver)
portal hypertension
secondary infections with systemic sepsis
islands of normal parenchyma surounded by fibrous septa
duct-like structures
- numerous epithelium-lined structures of biliary origin
- elongated and cystic spaces lined by regular biliary epithelium
- central or peripheral localization in the portal space
- luminal inspissated bile
+/- cholangitis
- associated Caroli disease
Etiology
hepatic ARPKD
phosphomannose isomerase deficiency (hypoglycosylation)
Differential diagnosis
cirrhosis
- secondary biliary cirrhosis
See also
ductal plate malformations (DPMs)
autosomal recessive polycyctic kidney disease (ARPKD)
Refreences
Desmet VJ. What is congenital hepatic fibrosis? Histopathology. 1992 Jun;20(6):465-77. PMID: 1607148