Incomplete pancreas divisum with anomalous choledochopancreatic duct junction with choledochal cyst

The coexistence of incomplete pancreas divisum, an anomalous choledochopancreatic duct junction, and a choledochal cyst is an extremely rare condition, described in only 3 patients in the available medical literature. The symptoms may be similar to any of these 3 distinct pathological conditions. Magnetic resonance cholangiopancreatography or endoscopic retrograde cholangiopancreatography is usually required for diagnosis. Management of symptomatic pancreas divisum may be accomplished with open accessory duct sphincteroplasty or endoscopic papillotomy with or without stenting. Treatment of choledochal cyst is by complete excision of the cyst whenever possible, with hepaticodochoenterostomy being the treatment of choice. Here, we describe a patient with this complex disorder who was successfully managed with endoscopic minor duct papillotomy with accessory pancreatic duct stenting and resection of the choledochal cyst with hepaticodochojejunostomy.