肝硬変像を呈した先天性胆道拡張症非黄疸例の1例

概要

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A 1 year-old-boy of congenital dilatation of the bile duct with cirrhosis was reported, when he was admitted at the age of 10 months with asthmatic attack, he was pointed out to have hepatomegaly, abnormal liver function tests and cystic dilatation of extrahepatic bile duct shown by imaging studies. But he showed no jaundice or abnormal serum bilirubin level throughout his clinical course. Because liver function become worse, bile drainage was made with T tube. Though serum transaminase improved, alkaline phosphatase was not normalized. Pancreaticocholangiography done through T tube showed abnormal long common channel (15 mm). Twenty seven days later, resection of dilated bile duct and hepaticojejunostomy with Roux-en-Y anastomosis was performed. Serum transaminase, alkaline phosphatase and other liver function tests were all normalized. Histological study of biopsy specimen of the liver showed cirrhosis. Amylase levels in the bile obtained through T tube drainage persisted abnormally high. As regard to the pathogenesis of cirrhosis in congenital dilatation of the bile duct, in addition to biliary obstructive factors, it is speculated that anomalous junction of the pancreaticobiliary ductal system giving rise to reflux of pancreatic juice into the biliary truct, then pancreatic enzymes being activated, activated enzymes could induce the damage of liver.
日本小児外科学会の論文
1991-10-20