BILE DUCT SYSTEM MALFORMATION - EMBRYOLOGICAL AND PATHOLOGICAL ASSOCIATION. TREATMENT /REVIEW ARTICLE/

Cystic diseases of the liver which are in most cases hereditary, are related to an embryonic disorder know as ductal plate malformation.These diseases correspond to partial or total arrest of remodeling of the ductal plate, leading to more or less complete persistence of the excess of embryonic biliary structures.The ductal plate malformation may concern different segments of the intrahepatic biliary tree (segmental bile ducts, interlobular bile ducts and the smallest bile duct ramifications) leading to various pathoclinical entities Congenital cystic lesions of bile ducts may affect intra or extrahepatic bile ducts.Intrahepatic lesions include five entities: congenital hepatic fibrosis, Caroli's syndrome, von Meyenburg complexes, simple cyst of the liver and polycystic liver disease.Congenital hepatic fibrosis and von Meyenburg complexes are secondary to ductal plate malformation affecting the smallest intrahepatic bile ducts.Choledocal cysts, Caroli's disease and Caroli's syndrome belong to the some family of congenital malformations of the large bile ducts (1).The former affects the extrahepatic bile duct (including occasionally the left and right branch of the hepatic duct) while the latter affects segmental intrahepatic bile ducts.Both are extremely rare (in the order of 1:10.000 or 100.000 and 1:1.000.000births respectively.