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congenital hepatic fibrosis

Wednesday 1 February 2006

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. See also: ductal plate malformations (DPMs)

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.

Portal tract fibrosis with biliary ductular enlargement or proliferation occurs in a number of genetic diseases that have cystic or tubulointerstitial renal lesions.

These include some with renal cystic diseases (RCDs) such as autosomal recessive diseases (e.g., infantile polycystic disease ARPKD, juvenile polycystic disease, and Meckel’s syndrome), autosomal dominant diseases (e.g., adult polycystic disease ADPKD) and, rarely, tuberose sclerosis and dominant glomerulocystic disease.

Portal tract fibrosis with biliary enlargement and proliferation occurs also with tubulointerstitial kidney diseases.

These probably include at least three disorders in the category nephronophthisis-congenital hepatic fibrosis (one autosomal recessive disease and two either autosomal or X-linked recessive diseases) plus Jeune syndrome (the tubulointerstitial diseases Fanconi familial nephronophthisis and anti-tubular membrane antibody disease do not regularly cause hepatic fibrosis).


- 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


- hepatic ARPKD
- phosphomannose isomerase deficiency (hypoglycosylation)

Differential diagnosis

- cirrhosis

  • secondary biliary cirrhosis

See also

- ductal plate malformations (DPMs)
- autosomal recessive polycyctic kidney disease (ARPKD)

  • hepatic ARPKD


- Desmet VJ. What is congenital hepatic fibrosis? Histopathology. 1992 Jun;20(6):465-77. PMID: 1607148

- Morphometric studies of cystic and tubulointerstitial kidney diseases with hepatic fibrosis in children. Landing BH, Wells TR, Lipsey AI, Oyemade OA. Pediatr Pathol. 1990;10(6):959-72. PMID: 2082340