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Choledochal Cyst
Roya Sohaey, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Definitions

              • Choledochal cyst (CC): Congenital cystic dilatation of extrahepatic ± intrahepatic bile ducts 
                • CC is "incomplete" obstruction
              • Cystic biliary atresia (CBA): Extrahepatic bile duct terminates blindly at caudal end
                • Complete biliary obstruction
                • CBA seen in 13-44% with CC 
              • Biliary atresia (BA): Progressive fibroobliterative disease of intrahepatic + extrahepatic bile ducts

            IMAGING

            • General Features

              • Ultrasonographic Findings

                • MR Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Staging, Grading, & Classification

                          CLINICAL ISSUES

                          • Presentation

                            • Demographics

                              • Natural History & Prognosis

                                • Treatment

                                  DIAGNOSTIC CHECKLIST

                                  • Image Interpretation Pearls

                                    Selected References

                                    1. Hattori K et al: Cyst size in fetuses with biliary cystic malformation: an exploration of the etiology of congenital biliary dilatation. Pediatr Gastroenterol Hepatol Nutr. 23(6):531-8, 2020
                                    2. Mahalik SK et al: Cystic biliary atresia or atretic choledochal cyst: a continuum in infantile obstructive cholangiopathy. Fetal Pediatr Pathol. 38(6):477-83, 2019
                                    3. Sanna E et al: Fetal abdominal cysts: antenatal course and postnatal outcomes. J Perinat Med. 47(4):418-21, 2019
                                    4. Tang J et al: Differentiation between cystic biliary atresia and choledochal cyst: a retrospective analysis. J Paediatr Child Health. 54(4):383-9, 2018
                                    5. Weng R et al: Prenatal diagnosis and prognosis assessment of congenital choledochal cyst in 21 cases. J Obstet Gynaecol. 36(3):324-7, 2016
                                    6. Cong X et al: Evaluation and screening ultrasonic signs in the diagnosis of fetal biliary cystic malformation. J Matern Fetal Neonatal Med. 28(17):2100-5, 2015
                                    7. Jensen KK et al: Antenatal sonographic diagnosis of choledochal cyst: case report and imaging review. J Clin Ultrasound. 43(9):581-3, 2015
                                    8. Khalil A et al: Outcome of first-trimester fetal abdominal cysts: cohort study and review of the literature. Ultrasound Obstet Gynecol. 43(4):413-9, 2014
                                    9. Diao M et al: Timing of surgery for prenatally diagnosed asymptomatic choledochal cysts: a prospective randomized study. J Pediatr Surg. 47(3):506-12, 2012
                                    10. Ruiz-Elizalde AR et al: A practical algorithm for accurate diagnosis and treatment of perinatally identified biliary ductal dilation: three cases that underscore the importance of an individualised approach. J Matern Fetal Neonatal Med. 22(7):622-8, 2009
                                    Related Anatomy
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                                    Related Differential Diagnoses
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                                    References
                                    Tables

                                    Tables

                                    KEY FACTS

                                    • Terminology

                                      • Imaging

                                        • Top Differential Diagnoses

                                          • Clinical Issues

                                            • Diagnostic Checklist

                                              TERMINOLOGY

                                              • Definitions

                                                • Choledochal cyst (CC): Congenital cystic dilatation of extrahepatic ± intrahepatic bile ducts 
                                                  • CC is "incomplete" obstruction
                                                • Cystic biliary atresia (CBA): Extrahepatic bile duct terminates blindly at caudal end
                                                  • Complete biliary obstruction
                                                  • CBA seen in 13-44% with CC 
                                                • Biliary atresia (BA): Progressive fibroobliterative disease of intrahepatic + extrahepatic bile ducts

                                              IMAGING

                                              • General Features

                                                • Ultrasonographic Findings

                                                  • MR Findings

                                                    • Imaging Recommendations

                                                      DIFFERENTIAL DIAGNOSIS

                                                        PATHOLOGY

                                                        • General Features

                                                          • Staging, Grading, & Classification

                                                            CLINICAL ISSUES

                                                            • Presentation

                                                              • Demographics

                                                                • Natural History & Prognosis

                                                                  • Treatment

                                                                    DIAGNOSTIC CHECKLIST

                                                                    • Image Interpretation Pearls

                                                                      Selected References

                                                                      1. Hattori K et al: Cyst size in fetuses with biliary cystic malformation: an exploration of the etiology of congenital biliary dilatation. Pediatr Gastroenterol Hepatol Nutr. 23(6):531-8, 2020
                                                                      2. Mahalik SK et al: Cystic biliary atresia or atretic choledochal cyst: a continuum in infantile obstructive cholangiopathy. Fetal Pediatr Pathol. 38(6):477-83, 2019
                                                                      3. Sanna E et al: Fetal abdominal cysts: antenatal course and postnatal outcomes. J Perinat Med. 47(4):418-21, 2019
                                                                      4. Tang J et al: Differentiation between cystic biliary atresia and choledochal cyst: a retrospective analysis. J Paediatr Child Health. 54(4):383-9, 2018
                                                                      5. Weng R et al: Prenatal diagnosis and prognosis assessment of congenital choledochal cyst in 21 cases. J Obstet Gynaecol. 36(3):324-7, 2016
                                                                      6. Cong X et al: Evaluation and screening ultrasonic signs in the diagnosis of fetal biliary cystic malformation. J Matern Fetal Neonatal Med. 28(17):2100-5, 2015
                                                                      7. Jensen KK et al: Antenatal sonographic diagnosis of choledochal cyst: case report and imaging review. J Clin Ultrasound. 43(9):581-3, 2015
                                                                      8. Khalil A et al: Outcome of first-trimester fetal abdominal cysts: cohort study and review of the literature. Ultrasound Obstet Gynecol. 43(4):413-9, 2014
                                                                      9. Diao M et al: Timing of surgery for prenatally diagnosed asymptomatic choledochal cysts: a prospective randomized study. J Pediatr Surg. 47(3):506-12, 2012
                                                                      10. Ruiz-Elizalde AR et al: A practical algorithm for accurate diagnosis and treatment of perinatally identified biliary ductal dilation: three cases that underscore the importance of an individualised approach. J Matern Fetal Neonatal Med. 22(7):622-8, 2009