Multiple parapapillary choledochoduodenal fistulas with ampullary carcinoma

Only very rarely do multiple parapapillary choledochoduodenal fistulas occur concurrently with ampullary carcinoma. The following presents just such a case, which occurred in a 51 year-old Japanese female hospitalized for epigastralgia. Gastrointestinal fiberscopy (GIF) showed abnormal swelling of Vater's papilla. She was diagnosed as having ampullary carcinoma and choledochoduodenal fistulas, as determined by hypotonic duodenography (HDG), endoscopic retrograde cholangiopancreatography (ERCP) and from the histopathology of the ampullary mucosal biopsy. Pancreatoduodenectomy was performed. We postulated that the multiple fistulas were formed on the longitudinal fold of Vater's papilla by an ampullary carcinoma, and that the fistulas played a major role in bile drainage. As a result, jaundice was not seen throughout the entire clinical course. We report on the mechanism of fistula formation, with a review of the recent literature.