乳児嚢胞性胆管形成異常症

抄録

Congenital malformations of the infantile hepato-biliary system are classified into two major groups of biliary atresia and biliary dilatation. The former is represented by so-called congenital extra-hepatic biliary atresia, and the latter by congenital choledochal cyst or bile duct dilatation. However, there has been a controversy regarding the morphological concept and classification of the hapato-biliary malformation, as indicated by the resemblance of cystic formation at porta-hepatis seen in both biliary atresia and bile duct dilatation. Recently, a male infant who had an unusual complication of extra-hepatic biliary atresia with cystic dilatation of the hepatic duct and of two intra-hepatic biliary solitary cysts, was successfully operated on by hepatic porto-enterostomy at the 56 days, and incision and external drainage of the cysts at 13 months of age. The purpose of this paper concerns presentation of a case and a proposal of new clinical entity of infantile hepato-biliary dysplasia ; infantile cystic cholangiopathy as a name for understanding of the disorders.