Congenital Biliary Atresia (I cyst type) Complicated Wiht Hepa-tolithiasis After An Interval of 18 Years.

Abstract

We experienced a rare case of increasing jaundice appeared at an interval of long term after a surgery for Biliary Atresia (BA). The patient was a female who developed jaundice after an interval of 18 years in good condition. She underwent cyst-jejunostomy under the diagnosis of type I cyst of BA on the 95th day after birth. Jaundice disappeared after operation and she had good post operative course. However, she suffered from intermittent abdominal pain, fever, and chillness since June 1988. From July she developed jaundice which increased progressively. Total bilirubin reached 20.3mg/dl in August. Cystic lesion was detected by computed tomography. Percutaneous transhepatic cholangio-drainage (PTCD) of the cyst was performed and jaundice gradually disappeared. Cholangiography showed cystic formation of the left dilated intrahepatic duct and defects in the abnormal intrahepaticduct. Choledochal-cystectomy lateral segmentectomy of the liver and hepatico-jejunostomy were performed. Although histological examination of the liver showed cirrhosis and severe cholangitis,laboratory data of the liver function was relatively stable. She is now in good health with neither jaundice nor portal hypertension.