Only very rarely do multiple parapapillapy choledochoduodenal fistulas occu
r concurrently with ampullary carcinoma. The following presents just such a
case, which occurred in a 51 year-old Japanese female hospitalized for epi
gastralgia. Gastrointestinal fiberscopy (GIF) showed abnormal swelling of V
ater's papilla. She was diagnosed as having ampullary carcinoma and choledo
choduodenal fistulas, as determined by hypotonic duodenography (HDG), endos
copic retrograde cholangiopancreatography (ERCP) and from the histopatholog
y 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 throughou
t the entire clinical course. We report on the mechanism of fistula formati
on, with a review of the recent literature.