Young female with pancreaticobiliary maljunction presenting with acute pancreatitis: a case report and review of the literature.

We report a case of pancreaticobiliary maljunction which presented with acute pancreatitis. Pancreaticobiliary maljunction and its complications are mostly observed in the Asian population. There are only few western publications concerning this subject. We reviewed the literature for current knowledge and opinions concerning the pathophysiology and optimal treatment, with special emphasis on the oncologic aspect of this condition. Those patients without a choledochal cyst should at least receive a prophylactic cholecystectomy. Firstly, to prevent further pancreatitis due to biliopancreatic reflux more or less promoted by gallbladder contraction, and secondly, more important, to prevent the occurrence of gallbladder cancer. Patients with choledochal cyst should receive a prophylactic cholecystectomy, and an excision of the extrahepatic bile duct, followed by hepaticojejunostomy.