Objective: To study clinical diseases associated with pancreaticobiliary maljunction with or without bile-duct dilatation.

Design: A retrospective study over 16 years.

Setting: A single university surgical service in Japan.

Patients: Thirty-three patients with pancreaticobiliary maljunction.

Main Outcome Measures: Gallstones and cancer in patients with and without bile-duct dilatation.

Results: Twenty-five patients had dilatation of the bile duct, 8 did not. Seven (28%) of the 25 patients with bile-duct dilatation had gallstones compared with 1 (12%) of the 8 patients with no dilatation. Seven (88%) of the 8 patients with no dilatation had carcinoma (all of the gallbladder), but only 3 (12%) of the 25 patients with bile-duct dilatation had carcinoma (1 of the gallbladder, 2 of the bile duct).

Conclusion: Prophylactic cholecystectomy is recommended for patients with pancreaticobiliary maljunction and no dilatation of the bile duct because of the high incidence of gallbladder cancer.

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