Congenital dilatation of intrahepatic bile ducts associated with hepatic fibrosis and medullary sponge kidney--reconstruction and microdissection study.

An autopsy case of a four-year-old female with congenital dilatation of intrahepatic bile ducts with bilateral medullary sponge kidney is presented. The liver and kidney were investigated by reconstruction with serial sections and the kidney was also studied by microdissection. In this case the dilatations of collecting tubules and intra- and extrahepatic bile ducts were observed. We regard these lesions as developmental anomalies since these sites of both organs are excretory ducts derived from the endoderm. The liver cirrhosis observed at autopsy had developed in the course of the disease because the liver architecture was well preserved in the biopsy material. The patient had fatal cholangitis without cholelithiasis.