Congenital cystic dilatation of the cystic duct associated with an anomalous pancreaticobiliary ductal junction

We report a case of congenital cystic dilatation of the cystic duct detected in a 40-year-old woman. She had intermittent pain in the right upper quadrant of the abdomen independent of the intake of meals for the last two years. In the ultrasound we found a cystic formation in the vicinity of the enlarged gallbladder, the common hepatic duct/common bile duct and the portal vein. The endoscopic retrograde cholangiography confirmed a cystic duct malformation, which was associated with an anomalous pancreaticobiliary ductal junction (APBDJ). A cholecystectomy with excision of the whole cystic duct and common bile duct and Roux-en-Y hepaticojejunostomy is indicated because there is an increased risk of the development of bile duct cancer and gallbladder cancer in the presence of biliary cystic duct anomalies and APBDJ.