usually been diagnosed incidentally. 2 However, the treatment of asymptomatic parapapillary choledochoduodenal fistula remains controversial. We report herein a case of parapapillary choledochoduodenal fistula associated with cholangiocarcinoma and discuss the relationship between this anomalous condition and biliary carcinogenesis.
Case reportAn asymptomatic 61-year-old Japanese woman was admitted to our hospital for further examination of a liver tumor detected by screening abdominal ultrasonography. Her family history was noncontributory. Although she had a history of diabetes mellitus of 10 years' duration, her history revealed no gallstone or gastroduodenal ulcer. She was afebrile on admission, and physical examination demonstrated no abnormalities. Laboratory data showed elevation of serum alkaline phosphatase, at 1188 U/l (normal range, 115-359 U/l) and glutamine-oxaloacetic transaminase, at 38 IU/l (normal range, 13-33 IU/l). Carcinoembryonic antigen was within the normal limit, and carbohydrate antigen 19-9 was elevated, at 2819 U/ml (normal range, Ͻ37 U/ml). Abdominal computed tomography (CT) revealed an irregularly contoured tumor, 4 cm in diameter, in the right hepatic lobe, and a dilated intrahepatic bile duct in the posterior segment of the liver. Pneumobilia was also noted in the intrahepatic bile duct. Enhanced CT showed that the tumor was slightly enhanced and involved the left branch of the portal vein (Fig. 1). Moreover, Swelling of multiple lymph nodes, in the hepatoduodenal ligament, pancreatic head, and abdominal para-aorta, was demonstrated. Duodenal fiberscopy revealed a fistula orifice 1.0 cm proximal to the orifice of the papilla of Vater. The orifice of the papilla of Vater was normal in shape (Fig. 2). Although we could not insert a cannulation tube into the orifice of Abstract Parapapillary choledochoduodenal fistula is a rare disorder. We herein report a case of parapapillary choledochoduodenal fistula associated with cholangiocarcinoma. A 61-year-old woman was admitted to our hospital for further examination of a liver tumor. She had no clinical symptoms, but computed tomography scans showed an irregularly contoured liver tumor which was histologically confirmed to be adenocarcinoma, by a needle biopsy examination. Duodenal fiberscopy revealed a fistula orifice 1.0 cm proximal to the orifice of the papilla of Vater, and endoscopic retrograde cholangiography through the fistula showed a communication to the common bile duct. Hypotonic duodenography demonstrated reflux of contrast material into the choledochoduodenal fistula. The bile sample collected from the common bile duct showed extremely high levels of pancreatic enzymes, including amylase, phospholipase-A2, and elastase-I. Furthermore, Helicobacter DNA was detected in bile by polymerase chain reaction (PCR) analysis. This experience suggests to us that parapapillary choledochoduodenal fistula may be a risk factor for biliary tract carcinoma, and surgical management is the treatment of choice for this rare condition, even when t...