Incomplete pancreas divisum with anomalous choledochopancreatic duct junction with choledochal cyst

The coexistence of incomplete pancreas divisum, an anomalous choledochopanc reatic duct junction, and a choledochal cyst is an extremely rare condition , described in only 3 patients in the available medical literature. The sym ptoms may be similar to any of these 3 distinct pathological conditions. Ma gnetic resonance cholangiopancreatography or endoscopic retrograde cholangi opancreatography is usually required for diagnosis. Management of symptomat ic pancreas divisum may be accomplished with open accessory duct sphinctero plasty or endoscopic papillotomy with or without stenting. Treatment of cho ledochal cyst is by complete excision of the cyst whenever possible, with h epaticodochoenterostomy being the treatment of choice. Here, we describe a patient with this complex disorder who was successfully managed with endosc opic minor duct papillotomy with accessory pancreatic duct stenting and res ection of the choledochal cyst with hepaticodochojejunostomy.