Histopathology 1992 Jun;20(6):465-77

What is congenital hepatic fibrosis

Desmet VJ
Catholic University of Leuven, University Hospital Sint Rafael, Belgium.

Abstract
The hypothesis presented in this paper suggests that, at birth, 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. The destructive
cholangiopathy may be of variable speed and duration in different patients. The
renal lesions in autosomal recessive polycystic kidney disease, which is most
often associated with congenital hepatic fibrosis, show a comparable pattern and
evolution. The hypothesis that congenital hepatic fibrosis corresponds to a
fetal type of biliary fibrosis would explain a number of disparate observations.
According to this concept, congenital hepatic fibrosis does not correspond to a
single clinical entity but to a broad, merging spectrum of conditions. All have
in common that they represent some stage of biliary fibrosis, usually of the
fetal type, rarely of the adult type, resulting from a slowly progressive,
destructive cholangiopathy