先天性肝內膽道擴張症（Caroli氏疾病）併總膽管囊腫

A 9-year-old girl was admitted to the Pediatric Ward of the National Taiwan University Hospital because of acute abdominal pain, fever and jaundice. She was placed on antibiotics arid the symptoms relieved. Alter a series of examinations including abdominal sonography, liver scan1 endoscopic retrograde pancreaticocholangiography and percutaneous transhepatic cholangiography, the diagnosis of congenital dilatation of the intrahepatic bile duct (Caroli's disease) and extrahepatic bile duct was established, Fever and jaundice recurred after the percutaneous transhepatic cholangiography examination. A preliminary decompression cholecystostomy was made, then a total excision of the dilated extrahepatic bile duct with hepatojejunostomy followed. The postoperative course was uneventful and she has been asymptomatic since