link
Bookmarks
Choledochal Cyst
Sara M. O'Hara, MD, FAAP
To access 4,300 diagnoses written by the world's leading experts in radiology.Try it free - 15 days
0
23
4
7

KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • Choledochal malformations, common bile duct (CBD) cyst or diverticulum, choledochocele, congenital biliary dilation
          • Definitions

            • Choledochal cyst: Spectrum of malformations involving extrahepatic & intrahepatic bile ducts
            • Etiology may be due to pancreaticobiliary maljunction
              • Pancreatic duct joins CBD proximal to sphincter of Oddi → biliary reflux of pancreatic enzymes
            • ↑ cholangiocarcinoma risk in patients with choledochal malformations

          IMAGING

          • General Features

            • Ultrasonographic Findings

              • MR Findings

                • Nuclear Medicine Findings

                  • CT Findings

                    • Other Modality 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

                                          • Image Interpretation Pearls

                                            Selected References

                                            1. Di Serafino M et al: Ultrasound findings in paediatric cholestasis: how to image the patient and what to look for. J Ultrasound. 23(1):1-12, 2020
                                            2. Ono A et al: Imaging of pancreaticobiliary maljunction. Radiographics. 40(2):378-92, 2020
                                            3. Shin HJ et al: Key imaging features for differentiating cystic biliary atresia from choledochal cyst: prenatal ultrasonography and postnatal ultrasonography and MRI. Ultrasonography. 40(2):301-11, 2020
                                            4. Banks JS et al: Choledochal malformations: surgical implications of radiologic findings. AJR Am J Roentgenol. 210(4):748-60, 2018
                                            5. Urushihara N et al: Characteristics, management, and outcomes of congenital biliary dilatation in neonates and early infants: a 20-year, single-institution study. J Hepatobiliary Pancreat Sci. 25(12):544-9, 2018
                                            6. Kamisawa T et al: Pancreaticobiliary maljunction and congenital biliary dilatation. Lancet Gastroenterol Hepatol. 2(8):610-8, 2017
                                            7. Lobeck IN et al: The presentation and management of choledochocele (type III choledochal cyst): a 40-year systematic review of the literature. J Pediatr Surg. 52(4):644-9, 2017
                                            8. Soares KC et al: Pediatric choledochal cysts: diagnosis and current management. Pediatr Surg Int. 33(6):637-50, 2017
                                            9. Urushihara N et al: Classification of pancreaticobiliary maljunction and clinical features in children. J Hepatobiliary Pancreat Sci. 24(8):449-55, 2017
                                            10. Chan KW et al: Laparoscopic management of antenatally detected choledochal cyst: a 10-year review. Surg Endosc. 30(12):5494-9, 2016
                                            11. Hamada Y et al: Diagnostic criteria for congenital biliary dilatation 2015. J Hepatobiliary Pancreat Sci. 23(6):342-6, 2016
                                            12. Le Roy B et al: Pancreaticobiliary maljunction and choledochal cysts: from embryogenesis to therapeutics aspects. Surg Radiol Anat. 38(9):1053-60, 2016
                                            13. Saito T et al: Significance of imaging modalities for preoperative evaluation of the pancreaticobiliary system in surgery for pediatric choledochal cyst. J Hepatobiliary Pancreat Sci. 23(6):347-52, 2016
                                            14. Xia HT et al: Treatment and outcomes of adults with remnant intrapancreatic choledochal cysts. Surgery. 159(2):418-25, 2016
                                            15. Chen J et al: Clinical value of ultrasound in diagnosing pediatric choledochal cyst perforation. AJR Am J Roentgenol. 204(3):630-5, 2015
                                            16. Katabathina VS et al: Adult choledochal cysts: current update on classification, pathogenesis, and cross-sectional imaging findings. Abdom Imaging. 40(6):1971-81, 2015
                                            17. Sastry AV et al: What is the incidence of biliary carcinoma in choledochal cysts, when do they develop, and how should it affect management? World J Surg. 39(2):487-92, 2015
                                            18. Shen HJ et al: Laparoscopic versus open surgery in children with choledochal cysts: a meta-analysis. Pediatr Surg Int. 31(6):529-34, 2015
                                            19. Soares KC et al: Presentation and clinical outcomes of choledochal cysts in children and adults: a multi-institutional analysis. JAMA Surg. 150(6):577-84, 2015
                                            20. Hukkinen M et al: Increasing occurrence of choledochal malformations in children: a single-center 37-year experience from Finland. Scand J Gastroenterol. 49(10):1255-60, 2014
                                            21. Tirumani SH et al: Imaging of the porta hepatis: spectrum of disease. Radiographics. 34(1):73-92, 2014
                                            22. Acker SN et al: Preoperative imaging does not predict intrahepatic involvement in choledochal cysts. J Pediatr Surg. 48(12):2378-82, 2013
                                            23. Narayanan SK et al: Hepaticoduodenostomy versus hepaticojejunostomy after resection of choledochal cyst: a systematic review and meta-analysis. J Pediatr Surg. 48(11):2336-42, 2013
                                            24. Sacher VY et al: Role of magnetic resonance cholangiopancreatography in diagnosing choledochal cysts: case series and review. World J Radiol. 5(8):304-12, 2013
                                            25. Salles A et al: Complete resection of a rare intrahepatic variant of a choledochal cyst. J Pediatr Surg. 48(3):652-4, 2013
                                            26. Zheng X et al: Surgical treatment of type IV-A choledochal cyst in a single institution: children vs. adults. J Pediatr Surg. 48(10):2061-6, 2013
                                            27. Cakmakci E et al: Sonographic diagnosis of choledochocele. J Clin Ultrasound. 40(7):448-50, 2012
                                            28. Guo WL et al: Imaging findings in 75 pediatric patients with pancreaticobiliary maljunction: a retrospective case study. Pediatr Surg Int. 28(10):983-8, 2012
                                            29. Santiago I et al: Congenital cystic lesions of the biliary tree. AJR Am J Roentgenol. 198(4):825-35, 2012
                                            30. Chiang L et al: Perforation: a rare complication of choledochal cysts in children. Pediatr Surg Int. 27(8):823-7, 2011
                                            31. Fujishiro J et al: Clinical characteristics of liver fibrosis in patients with choledochal cysts. J Pediatr Surg. 46(12):2296-300, 2011
                                            32. Fumino S et al: Diagnostic impact of computed tomography cholangiography and magnetic resonance cholangiopancreatography on pancreaticobiliary maljunction. J Pediatr Surg. 46(7):1373-8, 2011
                                            33. Hung MH et al: Choledochal cysts in infants and children: experiences over a 20-year period at a single institution. Eur J Pediatr. 170(9):1179-85, 2011
                                            34. Ono S et al: Diagnosis and treatment of pancreaticobiliary maljunction in children. Surg Today. 41(5):601-5, 2011
                                            35. Rozel C et al: Imaging of biliary disorders in children. Pediatr Radiol. 41(2):208-20, 2011
                                            36. Vachha B et al: Cystic lesions of the liver. AJR Am J Roentgenol. 196(4):W355-66, 2011
                                            37. She WH et al: Management of choledochal cyst: 30 years of experience and results in a single center. J Pediatr Surg. 44(12):2307-11, 2009
                                            38. Chavhan GB et al: Pediatric MR cholangiopancreatography: principles, technique, and clinical applications. Radiographics. 28(7):1951-62, 2008
                                            39. Hamada Y et al: Magnetic resonance cholangiopancreatography on postoperative work-up in children with choledochal cysts. Pediatr Surg Int. 20(1):43-6, 2004
                                            40. Benya EC: Pancreas and biliary system: imaging of developmental anomalies and diseases unique to children. Radiol Clin North Am. 40(6):1355-62, 2002
                                            41. de Vries JS et al: Choledochal cysts: age of presentation, symptoms, and late complications related to Todani's classification. J Pediatr Surg. 37(11):1568-73, 2002
                                            42. 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
                                            Loading...
                                            Related Differential Diagnoses
                                            Loading...
                                            References
                                            Tables

                                            Tables

                                            KEY FACTS

                                            • Terminology

                                              • Imaging

                                                • Top Differential Diagnoses

                                                  • Clinical Issues

                                                    TERMINOLOGY

                                                    • Synonyms

                                                      • Choledochal malformations, common bile duct (CBD) cyst or diverticulum, choledochocele, congenital biliary dilation
                                                    • Definitions

                                                      • Choledochal cyst: Spectrum of malformations involving extrahepatic & intrahepatic bile ducts
                                                      • Etiology may be due to pancreaticobiliary maljunction
                                                        • Pancreatic duct joins CBD proximal to sphincter of Oddi → biliary reflux of pancreatic enzymes
                                                      • ↑ cholangiocarcinoma risk in patients with choledochal malformations

                                                    IMAGING

                                                    • General Features

                                                      • Ultrasonographic Findings

                                                        • MR Findings

                                                          • Nuclear Medicine Findings

                                                            • CT Findings

                                                              • Other Modality 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

                                                                                    • Image Interpretation Pearls

                                                                                      Selected References

                                                                                      1. Di Serafino M et al: Ultrasound findings in paediatric cholestasis: how to image the patient and what to look for. J Ultrasound. 23(1):1-12, 2020
                                                                                      2. Ono A et al: Imaging of pancreaticobiliary maljunction. Radiographics. 40(2):378-92, 2020
                                                                                      3. Shin HJ et al: Key imaging features for differentiating cystic biliary atresia from choledochal cyst: prenatal ultrasonography and postnatal ultrasonography and MRI. Ultrasonography. 40(2):301-11, 2020
                                                                                      4. Banks JS et al: Choledochal malformations: surgical implications of radiologic findings. AJR Am J Roentgenol. 210(4):748-60, 2018
                                                                                      5. Urushihara N et al: Characteristics, management, and outcomes of congenital biliary dilatation in neonates and early infants: a 20-year, single-institution study. J Hepatobiliary Pancreat Sci. 25(12):544-9, 2018
                                                                                      6. Kamisawa T et al: Pancreaticobiliary maljunction and congenital biliary dilatation. Lancet Gastroenterol Hepatol. 2(8):610-8, 2017
                                                                                      7. Lobeck IN et al: The presentation and management of choledochocele (type III choledochal cyst): a 40-year systematic review of the literature. J Pediatr Surg. 52(4):644-9, 2017
                                                                                      8. Soares KC et al: Pediatric choledochal cysts: diagnosis and current management. Pediatr Surg Int. 33(6):637-50, 2017
                                                                                      9. Urushihara N et al: Classification of pancreaticobiliary maljunction and clinical features in children. J Hepatobiliary Pancreat Sci. 24(8):449-55, 2017
                                                                                      10. Chan KW et al: Laparoscopic management of antenatally detected choledochal cyst: a 10-year review. Surg Endosc. 30(12):5494-9, 2016
                                                                                      11. Hamada Y et al: Diagnostic criteria for congenital biliary dilatation 2015. J Hepatobiliary Pancreat Sci. 23(6):342-6, 2016
                                                                                      12. Le Roy B et al: Pancreaticobiliary maljunction and choledochal cysts: from embryogenesis to therapeutics aspects. Surg Radiol Anat. 38(9):1053-60, 2016
                                                                                      13. Saito T et al: Significance of imaging modalities for preoperative evaluation of the pancreaticobiliary system in surgery for pediatric choledochal cyst. J Hepatobiliary Pancreat Sci. 23(6):347-52, 2016
                                                                                      14. Xia HT et al: Treatment and outcomes of adults with remnant intrapancreatic choledochal cysts. Surgery. 159(2):418-25, 2016
                                                                                      15. Chen J et al: Clinical value of ultrasound in diagnosing pediatric choledochal cyst perforation. AJR Am J Roentgenol. 204(3):630-5, 2015
                                                                                      16. Katabathina VS et al: Adult choledochal cysts: current update on classification, pathogenesis, and cross-sectional imaging findings. Abdom Imaging. 40(6):1971-81, 2015
                                                                                      17. Sastry AV et al: What is the incidence of biliary carcinoma in choledochal cysts, when do they develop, and how should it affect management? World J Surg. 39(2):487-92, 2015
                                                                                      18. Shen HJ et al: Laparoscopic versus open surgery in children with choledochal cysts: a meta-analysis. Pediatr Surg Int. 31(6):529-34, 2015
                                                                                      19. Soares KC et al: Presentation and clinical outcomes of choledochal cysts in children and adults: a multi-institutional analysis. JAMA Surg. 150(6):577-84, 2015
                                                                                      20. Hukkinen M et al: Increasing occurrence of choledochal malformations in children: a single-center 37-year experience from Finland. Scand J Gastroenterol. 49(10):1255-60, 2014
                                                                                      21. Tirumani SH et al: Imaging of the porta hepatis: spectrum of disease. Radiographics. 34(1):73-92, 2014
                                                                                      22. Acker SN et al: Preoperative imaging does not predict intrahepatic involvement in choledochal cysts. J Pediatr Surg. 48(12):2378-82, 2013
                                                                                      23. Narayanan SK et al: Hepaticoduodenostomy versus hepaticojejunostomy after resection of choledochal cyst: a systematic review and meta-analysis. J Pediatr Surg. 48(11):2336-42, 2013
                                                                                      24. Sacher VY et al: Role of magnetic resonance cholangiopancreatography in diagnosing choledochal cysts: case series and review. World J Radiol. 5(8):304-12, 2013
                                                                                      25. Salles A et al: Complete resection of a rare intrahepatic variant of a choledochal cyst. J Pediatr Surg. 48(3):652-4, 2013
                                                                                      26. Zheng X et al: Surgical treatment of type IV-A choledochal cyst in a single institution: children vs. adults. J Pediatr Surg. 48(10):2061-6, 2013
                                                                                      27. Cakmakci E et al: Sonographic diagnosis of choledochocele. J Clin Ultrasound. 40(7):448-50, 2012
                                                                                      28. Guo WL et al: Imaging findings in 75 pediatric patients with pancreaticobiliary maljunction: a retrospective case study. Pediatr Surg Int. 28(10):983-8, 2012
                                                                                      29. Santiago I et al: Congenital cystic lesions of the biliary tree. AJR Am J Roentgenol. 198(4):825-35, 2012
                                                                                      30. Chiang L et al: Perforation: a rare complication of choledochal cysts in children. Pediatr Surg Int. 27(8):823-7, 2011
                                                                                      31. Fujishiro J et al: Clinical characteristics of liver fibrosis in patients with choledochal cysts. J Pediatr Surg. 46(12):2296-300, 2011
                                                                                      32. Fumino S et al: Diagnostic impact of computed tomography cholangiography and magnetic resonance cholangiopancreatography on pancreaticobiliary maljunction. J Pediatr Surg. 46(7):1373-8, 2011
                                                                                      33. Hung MH et al: Choledochal cysts in infants and children: experiences over a 20-year period at a single institution. Eur J Pediatr. 170(9):1179-85, 2011
                                                                                      34. Ono S et al: Diagnosis and treatment of pancreaticobiliary maljunction in children. Surg Today. 41(5):601-5, 2011
                                                                                      35. Rozel C et al: Imaging of biliary disorders in children. Pediatr Radiol. 41(2):208-20, 2011
                                                                                      36. Vachha B et al: Cystic lesions of the liver. AJR Am J Roentgenol. 196(4):W355-66, 2011
                                                                                      37. She WH et al: Management of choledochal cyst: 30 years of experience and results in a single center. J Pediatr Surg. 44(12):2307-11, 2009
                                                                                      38. Chavhan GB et al: Pediatric MR cholangiopancreatography: principles, technique, and clinical applications. Radiographics. 28(7):1951-62, 2008
                                                                                      39. Hamada Y et al: Magnetic resonance cholangiopancreatography on postoperative work-up in children with choledochal cysts. Pediatr Surg Int. 20(1):43-6, 2004
                                                                                      40. Benya EC: Pancreas and biliary system: imaging of developmental anomalies and diseases unique to children. Radiol Clin North Am. 40(6):1355-62, 2002
                                                                                      41. de Vries JS et al: Choledochal cysts: age of presentation, symptoms, and late complications related to Todani's classification. J Pediatr Surg. 37(11):1568-73, 2002
                                                                                      42. 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