Mirizzi syndrome with a fistula at the confluence of the hepatic ducts.

Background: A rare case of Mirizzi syndrome with atypical presentation is reported.

Methods: An 81-year-old woman with a known history of cholelithiasis presented with epigastric discomfort and indigestion. Imaging investigations revealed Mirizzi syndrome, while a cholecystobiliary fistula at the junction of the hepatic ducts was recognized intraoperatively and treated successfully with cholecystectomy and Roux-en-Y hepaticojejunostomy. During two years' follow-up the patient remains free of biliary symptoms.

Conclusions: Diagnosis of Mirizzi syndrome requires a high degree of clinical suspicion, especially in cases without obstructive jaundice. A fistula at the level of the confluence of the hepatic ducts is a rare topographic variant of the syndrome that may need a totally different surgical approach.