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Gastrointestinal
Diagnosis
Biliary System
Congenital
Choledochal Cyst
Choledochal Cyst
Siva P. Raman, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • Biliary cyst

          • Definitions

            • Choledochal cyst: Congenital, segmental, cystic dilatation of intrahepatic or extrahepatic bile ducts, most commonly affecting main portion of extrahepatic duct (ED)
            • Anomalous pancreaticobiliary junction (APBJ): Long common channel (> 15 mm) between common bile duct (CBD) and pancreatic duct outside of duodenum

          IMAGING

          • General Features

            • Radiographic Findings

              • CT Findings

                • MR Findings

                  • Ultrasonographic Findings

                    • Nuclear Medicine Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Staging, Grading, & Classification

                              • Gross Pathologic & Surgical Features

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Consider

                                            • Image Interpretation Pearls

                                              Selected References

                                              1. Aboughalia H et al: Pediatric biliary disorders: multimodality imaging evaluation with clinicopathologic correlation. Clin Imaging. 75:34-45, 2021
                                              2. Welle CL et al: Advances in MR imaging of the biliary tract. Magn Reson Imaging Clin N Am. 28(3):341-52, 2020
                                              3. Baison GN et al: Choledochal cysts: similarities and differences between Asian and Western countries. World J Gastroenterol. 25(26):3334-43, 2019
                                              4. Soares KC et al: Pediatric choledochal cysts: diagnosis and current management. Pediatr Surg Int. 33(6):637-50, 2017
                                              5. Le Roy B et al: Pancreaticobiliary maljunction and choledochal cysts: from embryogenesis to therapeutics aspects. Surg Radiol Anat. 38(9):1053-60, 2016
                                              6. Lewis VA et al: Imaging of choledochal cysts. Abdom Imaging. 40(6):1567-80, 2015
                                              7. Law R et al: Diagnosis and treatment of choledochoceles. Clin Gastroenterol Hepatol. 12(2):196-203, 2014
                                              8. Martin RF: Biliary cysts: a review and simplified classification scheme. Surg Clin North Am. 94(2):219-32, 2014
                                              9. Soares KC et al: Choledochal cysts: presentation, clinical differentiation, and management. J Am Coll Surg. 219(6):1167-80, 2014
                                              10. Tirumani SH et al: Imaging of the porta hepatis: spectrum of disease. Radiographics. 34(1):73-92, 2014
                                              11. Liu QY et al: MRI manifestations of adult choledochal cysts associated with biliary malignancy: a report of ten cases. Abdom Imaging. 38(5):1061-70, 2013
                                              12. Saluja SS et al: Management of choledochal cysts and their complications. Am Surg. 78(3):284-90, 2012
                                              13. Ulas M et al: Management of choledochal cysts in adults: a retrospective analysis of 23 patients. Hepatogastroenterology. 59(116):1155-9, 2012
                                              14. Vachha B et al: Cystic lesions of the liver. AJR Am J Roentgenol. 196(4):W355-66, 2011
                                              15. Venkatanarasimha N et al: Imaging features of ductal plate malformations in adults. Clin Radiol. 66(11):1086-93, 2011
                                              16. Lee HK et al: Imaging features of adult choledochal cysts: a pictorial review. Korean J Radiol. 10(1):71-80, 2009
                                              17. Chavhan GB et al: Pediatric MR cholangiopancreatography: principles, technique, and clinical applications. Radiographics. 28(7):1951-62, 2008
                                              18. Mortelé KJ et al: Multimodality imaging of pancreatic and biliary congenital anomalies. Radiographics. 26(3):715-31, 2006
                                              19. Brancatelli G et al: Fibropolycystic liver disease: CT and MR imaging findings. Radiographics. 25(3):659-70, 2005
                                              20. Dohke M et al: Anomalies and anatomic variants of the biliary tree revealed by MR cholangiopancreatography. AJR Am J Roentgenol. 173(5):1251-4, 1999
                                              21. Govil S et al: Choledochal cysts: evaluation with MR cholangiography. Abdom Imaging. 23(6):616-9, 1998
                                              22. Irie H et al: Value of MR cholangiopancreatography in evaluating choledochal cysts. AJR Am J Roentgenol. 171(5):1381-5, 1998
                                              23. Matos C et al: Choledochal cysts: comparison of findings at MR cholangiopancreatography and endoscopic retrograde cholangiopancreatography in eight patients. Radiology. 209(2):443-8, 1998
                                              24. Kim OH et al: Imaging of the choledochal cyst. Radiographics. 15(1):69-88, 1995
                                              25. Rizzo RJ et al: Congenital abnormalities of the pancreas and biliary tree in adults. Radiographics. 15(1):49-68; quiz 147-8, 1995
                                              26. Ebel KD et al: Choledochal cysts: classification and cholangiographic appearance. AJR Am J Roentgenol. 159(3):674-5, 1992
                                              27. Todani T et al: Congenital bile duct cysts: Classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst. Am J Surg. 134(2):263-9, 1977
                                              Related Anatomy
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                                              Related Differential Diagnoses
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                                              References
                                              Tables

                                              Tables

                                              KEY FACTS

                                              • Terminology

                                                • Imaging

                                                  • Pathology

                                                    • Clinical Issues

                                                      TERMINOLOGY

                                                      • Synonyms

                                                        • Biliary cyst

                                                      • Definitions

                                                        • Choledochal cyst: Congenital, segmental, cystic dilatation of intrahepatic or extrahepatic bile ducts, most commonly affecting main portion of extrahepatic duct (ED)
                                                        • Anomalous pancreaticobiliary junction (APBJ): Long common channel (> 15 mm) between common bile duct (CBD) and pancreatic duct outside of duodenum

                                                      IMAGING

                                                      • General Features

                                                        • Radiographic Findings

                                                          • CT Findings

                                                            • MR Findings

                                                              • Ultrasonographic Findings

                                                                • Nuclear Medicine Findings

                                                                  • Imaging Recommendations

                                                                    DIFFERENTIAL DIAGNOSIS

                                                                      PATHOLOGY

                                                                      • General Features

                                                                        • Staging, Grading, & Classification

                                                                          • Gross Pathologic & Surgical Features

                                                                            • Microscopic Features

                                                                              CLINICAL ISSUES

                                                                              • Presentation

                                                                                • Demographics

                                                                                  • Natural History & Prognosis

                                                                                    • Treatment

                                                                                      DIAGNOSTIC CHECKLIST

                                                                                      • Consider

                                                                                        • Image Interpretation Pearls

                                                                                          Selected References

                                                                                          1. Aboughalia H et al: Pediatric biliary disorders: multimodality imaging evaluation with clinicopathologic correlation. Clin Imaging. 75:34-45, 2021
                                                                                          2. Welle CL et al: Advances in MR imaging of the biliary tract. Magn Reson Imaging Clin N Am. 28(3):341-52, 2020
                                                                                          3. Baison GN et al: Choledochal cysts: similarities and differences between Asian and Western countries. World J Gastroenterol. 25(26):3334-43, 2019
                                                                                          4. Soares KC et al: Pediatric choledochal cysts: diagnosis and current management. Pediatr Surg Int. 33(6):637-50, 2017
                                                                                          5. Le Roy B et al: Pancreaticobiliary maljunction and choledochal cysts: from embryogenesis to therapeutics aspects. Surg Radiol Anat. 38(9):1053-60, 2016
                                                                                          6. Lewis VA et al: Imaging of choledochal cysts. Abdom Imaging. 40(6):1567-80, 2015
                                                                                          7. Law R et al: Diagnosis and treatment of choledochoceles. Clin Gastroenterol Hepatol. 12(2):196-203, 2014
                                                                                          8. Martin RF: Biliary cysts: a review and simplified classification scheme. Surg Clin North Am. 94(2):219-32, 2014
                                                                                          9. Soares KC et al: Choledochal cysts: presentation, clinical differentiation, and management. J Am Coll Surg. 219(6):1167-80, 2014
                                                                                          10. Tirumani SH et al: Imaging of the porta hepatis: spectrum of disease. Radiographics. 34(1):73-92, 2014
                                                                                          11. Liu QY et al: MRI manifestations of adult choledochal cysts associated with biliary malignancy: a report of ten cases. Abdom Imaging. 38(5):1061-70, 2013
                                                                                          12. Saluja SS et al: Management of choledochal cysts and their complications. Am Surg. 78(3):284-90, 2012
                                                                                          13. Ulas M et al: Management of choledochal cysts in adults: a retrospective analysis of 23 patients. Hepatogastroenterology. 59(116):1155-9, 2012
                                                                                          14. Vachha B et al: Cystic lesions of the liver. AJR Am J Roentgenol. 196(4):W355-66, 2011
                                                                                          15. Venkatanarasimha N et al: Imaging features of ductal plate malformations in adults. Clin Radiol. 66(11):1086-93, 2011
                                                                                          16. Lee HK et al: Imaging features of adult choledochal cysts: a pictorial review. Korean J Radiol. 10(1):71-80, 2009
                                                                                          17. Chavhan GB et al: Pediatric MR cholangiopancreatography: principles, technique, and clinical applications. Radiographics. 28(7):1951-62, 2008
                                                                                          18. Mortelé KJ et al: Multimodality imaging of pancreatic and biliary congenital anomalies. Radiographics. 26(3):715-31, 2006
                                                                                          19. Brancatelli G et al: Fibropolycystic liver disease: CT and MR imaging findings. Radiographics. 25(3):659-70, 2005
                                                                                          20. Dohke M et al: Anomalies and anatomic variants of the biliary tree revealed by MR cholangiopancreatography. AJR Am J Roentgenol. 173(5):1251-4, 1999
                                                                                          21. Govil S et al: Choledochal cysts: evaluation with MR cholangiography. Abdom Imaging. 23(6):616-9, 1998
                                                                                          22. Irie H et al: Value of MR cholangiopancreatography in evaluating choledochal cysts. AJR Am J Roentgenol. 171(5):1381-5, 1998
                                                                                          23. Matos C et al: Choledochal cysts: comparison of findings at MR cholangiopancreatography and endoscopic retrograde cholangiopancreatography in eight patients. Radiology. 209(2):443-8, 1998
                                                                                          24. Kim OH et al: Imaging of the choledochal cyst. Radiographics. 15(1):69-88, 1995
                                                                                          25. Rizzo RJ et al: Congenital abnormalities of the pancreas and biliary tree in adults. Radiographics. 15(1):49-68; quiz 147-8, 1995
                                                                                          26. Ebel KD et al: Choledochal cysts: classification and cholangiographic appearance. AJR Am J Roentgenol. 159(3):674-5, 1992
                                                                                          27. Todani T et al: Congenital bile duct cysts: Classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst. Am J Surg. 134(2):263-9, 1977