[Pancreatobiliary maljunction. Not only an Eastern disease]

Pancreatobiliary maljunction, an extramural junction of the pancreatic and biliary ducts in the duodenum, apparently beyond the scope of intramural sphincter function, is thought to be a preferential disease of Eastern people. However, this diagnosis is increasingly being made in other populations. We present a 41-year-old woman who presented with intermittent epigastric pain and mild raise in amylase levels. MRCP did not offer a definitive diagnosis, perhaps because of lack of experience in this disease. Finally, ERCP showed a pancreatobiliary maljunction, type acute angle, but with a complex connection between the choledocus and the pancreatic duct. Fusiform dilation of the common bile duct and of the left main hepatic duct were also present. The patient was referred for extrahepatic biliary resection and a diversion procedure. But also because of lack of experience in this disease, a wait-and-see approach was undertaken. In conclusion, pancreatobiliary maljunction will be increasingly diagnosed in western communities and more diagnostic and therapeutic experience will be needed.