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Choledochal Cyst
Sara M. O'Hara, MD, FAAP
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Synonyms

              • Choledochal malformations, common bile duct (CBD) cyst or diverticulum, choledochocele
            • 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. Chan KW et al: Laparoscopic management of antenatally detected choledochal cyst: a 10-year review. Surg Endosc. ePub, 2016
                                              2. Le Roy B et al: Pancreaticobiliary maljunction and choledochal cysts: from embryogenesis to therapeutics aspects. Surg Radiol Anat. ePub, 2016
                                              3. Xia HT et al: Treatment and outcomes of adults with remnant intrapancreatic choledochal cysts. Surgery. 159(2):418-25, 2016
                                              4. Chen J et al: Clinical value of ultrasound in diagnosing pediatric choledochal cyst perforation. AJR Am J Roentgenol. 204(3):630-5, 2015
                                              5. Katabathina VS et al: Adult choledochal cysts: current update on classification, pathogenesis, and cross-sectional imaging findings. Abdom Imaging. 40(6):1971-81, 2015
                                              6. 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
                                              7. Shen HJ et al: Laparoscopic versus open surgery in children with choledochal cysts: a meta-analysis. Pediatr Surg Int. 31(6):529-34, 2015
                                              8. 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
                                              9. 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
                                              10. Tirumani SH et al: Imaging of the porta hepatis: spectrum of disease. Radiographics. 34(1):73-92, 2014
                                              11. Acker SN et al: Preoperative imaging does not predict intrahepatic involvement in choledochal cysts. J Pediatr Surg. 48(12):2378-82, 2013
                                              12. 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
                                              13. 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
                                              14. Salles A et al: Complete resection of a rare intrahepatic variant of a choledochal cyst. J Pediatr Surg. 48(3):652-4, 2013
                                              15. 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
                                              16. Cakmakci E et al: Sonographic diagnosis of choledochocele. J Clin Ultrasound. 40(7):448-50, 2012
                                              17. 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
                                              18. Santiago I et al: Congenital cystic lesions of the biliary tree. AJR Am J Roentgenol. 198(4):825-35, 2012
                                              19. Chiang L et al: Perforation: a rare complication of choledochal cysts in children. Pediatr Surg Int. 27(8):823-7, 2011
                                              20. Fujishiro J et al: Clinical characteristics of liver fibrosis in patients with choledochal cysts. J Pediatr Surg. 46(12):2296-300, 2011
                                              21. 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
                                              22. 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
                                              23. Ono S et al: Diagnosis and treatment of pancreaticobiliary maljunction in children. Surg Today. 41(5):601-5, 2011
                                              24. Rozel C et al: Imaging of biliary disorders in children. Pediatr Radiol. 41(2):208-20, 2011
                                              25. Vachha B et al: Cystic lesions of the liver. AJR Am J Roentgenol. 196(4):W355-66, 2011
                                              26. 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
                                              27. Chavhan GB et al: Pediatric MR cholangiopancreatography: principles, technique, and clinical applications. Radiographics. 28(7):1951-62, 2008
                                              28. Hamada Y et al: Magnetic resonance cholangiopancreatography on postoperative work-up in children with choledochal cysts. Pediatr Surg Int. 20(1):43-6, 2004
                                              29. Benya EC: Pancreas and biliary system: imaging of developmental anomalies and diseases unique to children. Radiol Clin North Am. 40(6):1355-62, 2002
                                              30. 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
                                              31. 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

                                                  • Top Differential Diagnoses

                                                    • Pathology

                                                      • Clinical Issues

                                                        TERMINOLOGY

                                                        • Synonyms

                                                          • Choledochal malformations, common bile duct (CBD) cyst or diverticulum, choledochocele
                                                        • 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. Chan KW et al: Laparoscopic management of antenatally detected choledochal cyst: a 10-year review. Surg Endosc. ePub, 2016
                                                                                          2. Le Roy B et al: Pancreaticobiliary maljunction and choledochal cysts: from embryogenesis to therapeutics aspects. Surg Radiol Anat. ePub, 2016
                                                                                          3. Xia HT et al: Treatment and outcomes of adults with remnant intrapancreatic choledochal cysts. Surgery. 159(2):418-25, 2016
                                                                                          4. Chen J et al: Clinical value of ultrasound in diagnosing pediatric choledochal cyst perforation. AJR Am J Roentgenol. 204(3):630-5, 2015
                                                                                          5. Katabathina VS et al: Adult choledochal cysts: current update on classification, pathogenesis, and cross-sectional imaging findings. Abdom Imaging. 40(6):1971-81, 2015
                                                                                          6. 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
                                                                                          7. Shen HJ et al: Laparoscopic versus open surgery in children with choledochal cysts: a meta-analysis. Pediatr Surg Int. 31(6):529-34, 2015
                                                                                          8. 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
                                                                                          9. 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
                                                                                          10. Tirumani SH et al: Imaging of the porta hepatis: spectrum of disease. Radiographics. 34(1):73-92, 2014
                                                                                          11. Acker SN et al: Preoperative imaging does not predict intrahepatic involvement in choledochal cysts. J Pediatr Surg. 48(12):2378-82, 2013
                                                                                          12. 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
                                                                                          13. 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
                                                                                          14. Salles A et al: Complete resection of a rare intrahepatic variant of a choledochal cyst. J Pediatr Surg. 48(3):652-4, 2013
                                                                                          15. 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
                                                                                          16. Cakmakci E et al: Sonographic diagnosis of choledochocele. J Clin Ultrasound. 40(7):448-50, 2012
                                                                                          17. 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
                                                                                          18. Santiago I et al: Congenital cystic lesions of the biliary tree. AJR Am J Roentgenol. 198(4):825-35, 2012
                                                                                          19. Chiang L et al: Perforation: a rare complication of choledochal cysts in children. Pediatr Surg Int. 27(8):823-7, 2011
                                                                                          20. Fujishiro J et al: Clinical characteristics of liver fibrosis in patients with choledochal cysts. J Pediatr Surg. 46(12):2296-300, 2011
                                                                                          21. 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
                                                                                          22. 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
                                                                                          23. Ono S et al: Diagnosis and treatment of pancreaticobiliary maljunction in children. Surg Today. 41(5):601-5, 2011
                                                                                          24. Rozel C et al: Imaging of biliary disorders in children. Pediatr Radiol. 41(2):208-20, 2011
                                                                                          25. Vachha B et al: Cystic lesions of the liver. AJR Am J Roentgenol. 196(4):W355-66, 2011
                                                                                          26. 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
                                                                                          27. Chavhan GB et al: Pediatric MR cholangiopancreatography: principles, technique, and clinical applications. Radiographics. 28(7):1951-62, 2008
                                                                                          28. Hamada Y et al: Magnetic resonance cholangiopancreatography on postoperative work-up in children with choledochal cysts. Pediatr Surg Int. 20(1):43-6, 2004
                                                                                          29. Benya EC: Pancreas and biliary system: imaging of developmental anomalies and diseases unique to children. Radiol Clin North Am. 40(6):1355-62, 2002
                                                                                          30. 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
                                                                                          31. 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