Congenital Diseases of Intrahepatic Bile Ducts: Variations on the Theme “Ductal Plate Malformation”
Congenital diseases of intrahepatic bile ducts (IHBDs) can be divided into two main groups: diseases characterized by necroinflammatory destruction of IHBDs and diseases characterized by a variable degree of ectasia of IHBDs and associated with a variable degree of fibrosis. This review will emphasize that in mostif not allof these conditions, persistence or lack of remodeling of the embryonic ductal plate (so-called "ductal plate malformation" [DPM]) is an essential precursor of the lesions (1).