Abstract
The patient was a female child aged one year 4 months with a 1-month history of jaundice and loose whitish stools. A physician who examined the patient for cold symptoms detected intra- and extrahepatic bile duct dilatation, and the patient was referred to our department. On CT, the intrahepatic bile duct and common bile duct were found to be markedly dilated (to a diameter of 11 cm) and occupying the entire abdominal cavity, and the patient was diagnosed as a case of Todani type IV-A congenital biliary dilatation. Subsequently, the patient developed cholangitis complicated by sepsis, necessitating external cholangiostomy using a T-tube. After improvement of the general condition of the patient, resection of the dilated bile duct and hepatocholangiojejunostomy were performed. The postoperative course was favorable, and the patient was discharged 15 days after the radical surgery. A large cyst-type congenital biliary dilatation may become serious when it becomes complicated by cholestasis-induced cholangitis. Biliary drainage may be effective for controlling the inflammatory condition and transaction of the dilated bile duct.
