An extremely rare combination of choledochocele and bile duct duplication escalating severe acute pancreatitis and cholangitis: A case report.

Background: Combination of choledochocele and extra-hepatic duct duplication is an extremely rare congenital abnormality.

Case Presentation: The patient was an 81-year-old Japanese man. He visited the emergency room for severe abdominal colic pain. He was diagnosed with severe pancreatitis with cholelithiasis and treated conservatively by percutaneous trans-hepatic gallbladder drainage (PTGBD) for 4 months. Thereafter, he was transferred to our institute and cholangiography was performed via the PTGBD tube, revealing cholecysto- and choledocho-lithiasis. The cystic-duct joined the right hepatic duct with extra-hepatic bile duct duplication and the terminal bile duct flowed into the cystic papilla of Vater. The main pancreatic duct also joined into the cystic papilla. These observations confirmed choledochocele with extra-hepatic bile duct duplication. Surgical exploration was performed, and hepatico-jejunostomy with hepatic-ductplasty and cholecystectomy with choledocholithotomy were carried out. He was discharged and his course was uneventful.

Conclusion: A very rare combined case of choledochocele with bile duct duplication, which would escalate the pancreatitis and cholangitis, was successfully treated. Their pathogeneses in relation to pancreaticobiliary maljunction is discussed.