INTRAHEPATIC BILE DUCT LESIONS OF INFANTS WITH POLYCYSTIC KIDNEY

Abstract

Among 171autopsy cases of infants there were 11 cases with polycystic kidney, and 6 cases with anomalous intrahepatic bile ducts. All three cases with type I polycystic kidney (Potter) and 2 of 5 cases with type II polycystic kidney showed anomalous intrahepatic bile ducts. Five of 6 infants with anomalous intrahepatic bile ducts showed polycystic kidney. Therefore, it seemed likely that anomalous intrahepatic bile ducts and polycystic kidney were intimately associated in infants.
Three cases with type I polycystic kidney showed proliferation and tortuosity of the dilated intrahepatic bile ducts, while in 2 cases with type II polycystic kidney proliferated and tortuous intrahepatic bile ducts were not dilated and rather transformed into the cordlike structure. This finding and other morphological differences in the liver of two types of polycystic kidneys suggested that morphological and morphogenetical differences might be present in the intrahepatic bile duct lesions between in infants with type I and those with type II polycystic kidney.