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Caroli Disease
Hee Sun Park, MD, PhD; Aya Kamaya, MD, FSRU, FSAR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Synonyms

                • Communicating cavernous biliary ectasia 
              • Definitions

                • Caroli disease: Congenital, multifocal, segmental, cystic dilatations of intrahepatic bile ducts (IHBD)
                • Caroli syndrome: Caroli disease + congenital hepatic fibrosis

              IMAGING

              • General Features

                • Ultrasonographic Findings

                  • Radiographic Findings

                    • CT Findings

                      • MR Findings

                        • Nuclear Medicine Findings

                          • Imaging Recommendations

                            DIFFERENTIAL DIAGNOSIS

                              PATHOLOGY

                              • General Features

                                • Gross Pathologic & Surgical Features

                                  • Microscopic Features

                                    CLINICAL ISSUES

                                    • Presentation

                                      • Demographics

                                        • Natural History & Prognosis

                                          • Treatment

                                            DIAGNOSTIC CHECKLIST

                                            • Consider

                                              • Image Interpretation Pearls

                                                Selected References

                                                1. Gunay-Aygun M et al: Characteristics of congenital hepatic fibrosis in a large cohort of patients with autosomal recessive polycystic kidney disease. Gastroenterology. 144(1):112-121, 2013
                                                2. 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
                                                3. Venkatanarasimha N et al: Imaging features of ductal plate malformations in adults. Clin Radiol. 66(11):1086-93, 2011
                                                4. Ananthakrishnan AN et al: Caroli's disease: identification and treatment strategy. Curr Gastroenterol Rep. 9(2):151-5, 2007
                                                5. Yonem O et al: Clinical characteristics of Caroli's syndrome. World J Gastroenterol. 13(13):1934-7, 2007
                                                6. Madjov R et al: Caroli's disease. Report of 5 cases and review of literature. Hepatogastroenterology. 52(62):606-9, 2005
                                                7. Sgro M et al: Caroli's disease: prenatal diagnosis, postnatal outcome and genetic analysis. Ultrasound Obstet Gynecol. 23(1):73-6, 2004
                                                8. Levy AD et al: Caroli's disease: radiologic spectrum with pathologic correlation. AJR Am J Roentgenol. 179(4):1053-7, 2002
                                                9. Fulcher AS et al: Case 38: Caroli disease and renal tubular ectasia. Radiology. 220(3):720-3, 2001
                                                10. Mortele KJ et al: Cystic focal liver lesions in the adult: differential CT and MR imaging features. Radiographics. 21(4):895-910, 2001
                                                11. Akin O et al: An unusual sonographic finding in Caroli's disease. AJR Am J Roentgenol. 171(4):1167, 1998
                                                12. 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
                                                13. Miller WJ et al: Imaging findings in Caroli's disease. AJR Am J Roentgenol. 165(2):333-7, 1995
                                                14. Rizzo RJ et al: Congenital abnormalities of the pancreas and biliary tree in adults. Radiographics. 15(1):49-68; quiz 147-8, 1995
                                                15. Choi BI et al: Caroli disease: central dot sign in CT. Radiology. 174(1):161-3, 1990
                                                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

                                                          • Diagnostic Checklist

                                                            TERMINOLOGY

                                                            • Synonyms

                                                              • Communicating cavernous biliary ectasia 
                                                            • Definitions

                                                              • Caroli disease: Congenital, multifocal, segmental, cystic dilatations of intrahepatic bile ducts (IHBD)
                                                              • Caroli syndrome: Caroli disease + congenital hepatic fibrosis

                                                            IMAGING

                                                            • General Features

                                                              • Ultrasonographic Findings

                                                                • Radiographic Findings

                                                                  • CT Findings

                                                                    • MR Findings

                                                                      • Nuclear Medicine Findings

                                                                        • Imaging Recommendations

                                                                          DIFFERENTIAL DIAGNOSIS

                                                                            PATHOLOGY

                                                                            • General Features

                                                                              • Gross Pathologic & Surgical Features

                                                                                • Microscopic Features

                                                                                  CLINICAL ISSUES

                                                                                  • Presentation

                                                                                    • Demographics

                                                                                      • Natural History & Prognosis

                                                                                        • Treatment

                                                                                          DIAGNOSTIC CHECKLIST

                                                                                          • Consider

                                                                                            • Image Interpretation Pearls

                                                                                              Selected References

                                                                                              1. Gunay-Aygun M et al: Characteristics of congenital hepatic fibrosis in a large cohort of patients with autosomal recessive polycystic kidney disease. Gastroenterology. 144(1):112-121, 2013
                                                                                              2. 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
                                                                                              3. Venkatanarasimha N et al: Imaging features of ductal plate malformations in adults. Clin Radiol. 66(11):1086-93, 2011
                                                                                              4. Ananthakrishnan AN et al: Caroli's disease: identification and treatment strategy. Curr Gastroenterol Rep. 9(2):151-5, 2007
                                                                                              5. Yonem O et al: Clinical characteristics of Caroli's syndrome. World J Gastroenterol. 13(13):1934-7, 2007
                                                                                              6. Madjov R et al: Caroli's disease. Report of 5 cases and review of literature. Hepatogastroenterology. 52(62):606-9, 2005
                                                                                              7. Sgro M et al: Caroli's disease: prenatal diagnosis, postnatal outcome and genetic analysis. Ultrasound Obstet Gynecol. 23(1):73-6, 2004
                                                                                              8. Levy AD et al: Caroli's disease: radiologic spectrum with pathologic correlation. AJR Am J Roentgenol. 179(4):1053-7, 2002
                                                                                              9. Fulcher AS et al: Case 38: Caroli disease and renal tubular ectasia. Radiology. 220(3):720-3, 2001
                                                                                              10. Mortele KJ et al: Cystic focal liver lesions in the adult: differential CT and MR imaging features. Radiographics. 21(4):895-910, 2001
                                                                                              11. Akin O et al: An unusual sonographic finding in Caroli's disease. AJR Am J Roentgenol. 171(4):1167, 1998
                                                                                              12. 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
                                                                                              13. Miller WJ et al: Imaging findings in Caroli's disease. AJR Am J Roentgenol. 165(2):333-7, 1995
                                                                                              14. Rizzo RJ et al: Congenital abnormalities of the pancreas and biliary tree in adults. Radiographics. 15(1):49-68; quiz 147-8, 1995
                                                                                              15. Choi BI et al: Caroli disease: central dot sign in CT. Radiology. 174(1):161-3, 1990