Intrahepatic bile ducts are patent in the liver of patients with biliary atresia in early infancy. Small bile ducts at the porta hepatis continuing to the intrahepatic ducts usually terminate in fibrous tissue replacing the hepatic radicles, which does not extend to the common hepatic duct. Therefore, the level of transection of the fibrous tissue at the porta hepatis is of prime importance for successful hepatic portoenterostomy. Transection should be done at the level of the posterior surface of the portal vein. The overall cure rate of surgery for biliary atresia was 30%. In our recent series, however, the results have markedly improved and the cure rate has been increased to 55%. There are 47 patients living without jaundice, 22 of them for more than 5 years. The longest survivors are 23 years old. All the longterm survivors are leading a normal life for their age, although 6 of them have some physical or mental handicap. Early operation, adequate surgical technique, and prevention of postoperative cholangitis are requisites for successful hepatic portoenterostomy.
A case of Wilms tumor associated with hydronephrosis is reported. The tumor extended into the dilated renal pelvis but there was no invasion in the mucosa of the ureter or renal pelvis.
Congenital bile duct dilatation in siblings was recently experienced at the Tohoku University Hospital. Anomalous junction of the pancreatico-biliary ductal system was disclosed in both patients by the operative cholangiogram.
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