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Gastrointestinal
Diagnosis
Biliary System
Congenital
Caroli Disease
Caroli Disease
Siva P. Raman, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • Communicating cavernous biliary ectasia
            • Congenital saccular dilatation of intrahepatic bile ducts (IHBDs)

          • Definitions

            • Originally divided into 2 variants
              • Caroli disease ("pure" or "simple" form): Congenital multifocal saccular dilation of large intrahepatic bile ducts (IHBDs) in absence of other hepatic abnormalities (type I Caroli disease)
              • Caroli syndrome ("complex" form): More common variant characterized by cystic dilatation of large intrahepatic ducts, hepatic fibrosis, and portal hypertension (type II Caroli disease)

          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. Cannella R et al: Congenital cystic lesions of the bile ducts: imaging-based diagnosis. Curr Probl Diagn Radiol. 49(4):285-93, 2020
                                              2. Zhong Y et al: Hepatobiliary and pancreatic: Caroli syndrome: egg-like sign on CT. J Gastroenterol Hepatol. 35(2):180, 2020
                                              3. Mamone G et al: Hepatic morphology abnormalities: beyond cirrhosis. Abdom Radiol (NY). 43(7):1612-26, 2018
                                              4. Zahmatkeshan M et al: Liver transplantation for caroli disease. Int J Organ Transplant Med. 3(4):189-91, 2012
                                              5. Zhang DY et al: Caroli's disease: a report of 14 patients and review of the literature. J Dig Dis. 13(9):491-5, 2012
                                              6. Lefere M et al: Caroli disease: review of eight cases with emphasis on magnetic resonance imaging features. Eur J Gastroenterol Hepatol. 23(7):578-85, 2011
                                              7. Vachha B et al: Cystic lesions of the liver. AJR Am J Roentgenol. 196(4):W355-66, 2011
                                              8. Venkatanarasimha N et al: Imaging features of ductal plate malformations in adults. Clin Radiol. 66(11):1086-93, 2011
                                              9. Lall NU et al: Caroli disease and the central dot sign. Pediatr Radiol. 39(7):754, 2009
                                              10. Veigel MC et al: Fibropolycystic liver disease in children. Pediatr Radiol. 39(4):317-27, 2009
                                              11. Ananthakrishnan AN et al: Caroli's disease: identification and treatment strategy. Curr Gastroenterol Rep. 9(2):151-5, 2007
                                              12. Brancatelli G et al: Fibropolycystic liver disease: CT and MR imaging findings. Radiographics. 25(3):659-70, 2005
                                              13. Guy F et al: Caroli's disease: magnetic resonance imaging features. Eur Radiol. 12(11):2730-6, 2002
                                              14. Krause D et al: MRI for evaluating congenital bile duct abnormalities. J Comput Assist Tomogr. 26(4):541-52, 2002
                                              15. Levy AD et al: Caroli's disease: radiologic spectrum with pathologic correlation. AJR Am J Roentgenol. 179(4):1053-7, 2002
                                              16. Fulcher AS et al: Case 38: Caroli disease and renal tubular ectasia. Radiology. 220(3):720-3, 2001
                                              17. Mortele KJ et al: Cystic focal liver lesions in the adult: differential CT and MR imaging features. Radiographics. 21(4):895-910, 2001
                                              18. Akin O et al: An unusual sonographic finding in Caroli's disease. AJR Am J Roentgenol. 171(4):1167, 1998
                                              19. Asselah T et al: Caroli's disease: a magnetic resonance cholangiopancreatography diagnosis. Am J Gastroenterol. 93(1):109-10, 1998
                                              20. Gorka W et al: Value of Doppler sonography in the assessment of patients with Caroli's disease. J Clin Ultrasound. 26(6):283-7, 1998
                                              21. Pavone P et al: Caroli's disease: evaluation with MR cholangiography. AJR Am J Roentgenol. 166(1):216-7, 1996
                                              22. Miller WJ et al: Imaging findings in Caroli's disease. AJR Am J Roentgenol. 165(2):333-7, 1995
                                              23. Rizzo RJ et al: Congenital abnormalities of the pancreas and biliary tree in adults. Radiographics. 15(1):49-68; quiz 147-8, 1995
                                              24. Zangger P et al: MRI findings in Caroli's disease and intrahepatic pigmented calculi. Abdom Imaging. 20(4):361-4, 1995
                                              25. Choi BI et al: Caroli disease: central dot sign in CT. Radiology. 174(1):161-3, 1990
                                              26. Cowart VS: Boxing makes more headlines than usual and a lot of the news hasn't been good. JAMA. 261(1):14-6, 1989
                                              27. Murphy BJ et al: The CT appearance of cystic masses of the liver. Radiographics. 9(2):307-22, 1989
                                              28. Marchal GJ et al: Caroli disease: high-frequency US and pathologic findings. Radiology. 158(2):507-11, 1986
                                              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

                                                        • Communicating cavernous biliary ectasia
                                                        • Congenital saccular dilatation of intrahepatic bile ducts (IHBDs)

                                                      • Definitions

                                                        • Originally divided into 2 variants
                                                          • Caroli disease ("pure" or "simple" form): Congenital multifocal saccular dilation of large intrahepatic bile ducts (IHBDs) in absence of other hepatic abnormalities (type I Caroli disease)
                                                          • Caroli syndrome ("complex" form): More common variant characterized by cystic dilatation of large intrahepatic ducts, hepatic fibrosis, and portal hypertension (type II Caroli disease)

                                                      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. Cannella R et al: Congenital cystic lesions of the bile ducts: imaging-based diagnosis. Curr Probl Diagn Radiol. 49(4):285-93, 2020
                                                                                          2. Zhong Y et al: Hepatobiliary and pancreatic: Caroli syndrome: egg-like sign on CT. J Gastroenterol Hepatol. 35(2):180, 2020
                                                                                          3. Mamone G et al: Hepatic morphology abnormalities: beyond cirrhosis. Abdom Radiol (NY). 43(7):1612-26, 2018
                                                                                          4. Zahmatkeshan M et al: Liver transplantation for caroli disease. Int J Organ Transplant Med. 3(4):189-91, 2012
                                                                                          5. Zhang DY et al: Caroli's disease: a report of 14 patients and review of the literature. J Dig Dis. 13(9):491-5, 2012
                                                                                          6. Lefere M et al: Caroli disease: review of eight cases with emphasis on magnetic resonance imaging features. Eur J Gastroenterol Hepatol. 23(7):578-85, 2011
                                                                                          7. Vachha B et al: Cystic lesions of the liver. AJR Am J Roentgenol. 196(4):W355-66, 2011
                                                                                          8. Venkatanarasimha N et al: Imaging features of ductal plate malformations in adults. Clin Radiol. 66(11):1086-93, 2011
                                                                                          9. Lall NU et al: Caroli disease and the central dot sign. Pediatr Radiol. 39(7):754, 2009
                                                                                          10. Veigel MC et al: Fibropolycystic liver disease in children. Pediatr Radiol. 39(4):317-27, 2009
                                                                                          11. Ananthakrishnan AN et al: Caroli's disease: identification and treatment strategy. Curr Gastroenterol Rep. 9(2):151-5, 2007
                                                                                          12. Brancatelli G et al: Fibropolycystic liver disease: CT and MR imaging findings. Radiographics. 25(3):659-70, 2005
                                                                                          13. Guy F et al: Caroli's disease: magnetic resonance imaging features. Eur Radiol. 12(11):2730-6, 2002
                                                                                          14. Krause D et al: MRI for evaluating congenital bile duct abnormalities. J Comput Assist Tomogr. 26(4):541-52, 2002
                                                                                          15. Levy AD et al: Caroli's disease: radiologic spectrum with pathologic correlation. AJR Am J Roentgenol. 179(4):1053-7, 2002
                                                                                          16. Fulcher AS et al: Case 38: Caroli disease and renal tubular ectasia. Radiology. 220(3):720-3, 2001
                                                                                          17. Mortele KJ et al: Cystic focal liver lesions in the adult: differential CT and MR imaging features. Radiographics. 21(4):895-910, 2001
                                                                                          18. Akin O et al: An unusual sonographic finding in Caroli's disease. AJR Am J Roentgenol. 171(4):1167, 1998
                                                                                          19. Asselah T et al: Caroli's disease: a magnetic resonance cholangiopancreatography diagnosis. Am J Gastroenterol. 93(1):109-10, 1998
                                                                                          20. Gorka W et al: Value of Doppler sonography in the assessment of patients with Caroli's disease. J Clin Ultrasound. 26(6):283-7, 1998
                                                                                          21. Pavone P et al: Caroli's disease: evaluation with MR cholangiography. AJR Am J Roentgenol. 166(1):216-7, 1996
                                                                                          22. Miller WJ et al: Imaging findings in Caroli's disease. AJR Am J Roentgenol. 165(2):333-7, 1995
                                                                                          23. Rizzo RJ et al: Congenital abnormalities of the pancreas and biliary tree in adults. Radiographics. 15(1):49-68; quiz 147-8, 1995
                                                                                          24. Zangger P et al: MRI findings in Caroli's disease and intrahepatic pigmented calculi. Abdom Imaging. 20(4):361-4, 1995
                                                                                          25. Choi BI et al: Caroli disease: central dot sign in CT. Radiology. 174(1):161-3, 1990
                                                                                          26. Cowart VS: Boxing makes more headlines than usual and a lot of the news hasn't been good. JAMA. 261(1):14-6, 1989
                                                                                          27. Murphy BJ et al: The CT appearance of cystic masses of the liver. Radiographics. 9(2):307-22, 1989
                                                                                          28. Marchal GJ et al: Caroli disease: high-frequency US and pathologic findings. Radiology. 158(2):507-11, 1986