Intrahepatic bile ducts (IHBDs) develop from bipotential liver progenitor cells in contact with the mesenchyme of the portal vein and thus form the "ductal plate". The ductal plate are remodelate into mature tubular ducts. Lack of remodeling results in the persistance of periportal epithelial sleeves or "ductal plate malformation" (DPM). A proposal is that virtually all congenital disease of IHBDs represent examples of DPM. Some early, severe types of extra-hepatic bile duct atresia are characterized by DPM, a suggestion of a prenatal beginning of the disease. Several congenital disease are charcterized by dilatation of segments of IHBDs and variable degrees of fibrosis. Such "fibro tic disease" represent DPM at different levels of biliary tree. Autosomal ressive polykystic kidney disease represents DPM of interlobular bile ducts, associated with tubular dilatation of collecting renal tubules. Cogenital fibrosis may derive from the same type of liver lesion, through a superimposed destructive type  of cholangiopathy associated with scarring fibrosis. Caroli's disease represents DPM of larger IHBDs, whereas Caroli's syndrome combines the lesions of Caroli's disease and congenital hepatic fibrosis. Von Meyenburg complexes represent DPM of smaller interlobular ducts; their dilatation gives rise to the liver cysts in autosomal dominant polycystic kidney disease. Finally, DPM is a coponnent of the tissue abnormalities in so-called mesenchymal hamartoma.
 

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