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Caroli Disease
Alexander J. Towbin, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Definitions

              • Caroli disease (CD): Saccular dilation of large intrahepatic bile ducts (IHBD)
              • Congenital hepatic fibrosis (CHF): Presence of fibrotic tissue between portal tracts with persistent intralobular ducts
              • Caroli syndrome (CS): CD + CHF &/or autosomal recessive polycystic kidney disease (ARPKD)
              • Hepatorenal fibrocystic diseases: Renal fibrocystic degeneration + abnormalities of portobiliary system

            IMAGING

            • General Features

              • Ultrasonographic Findings

                • CT Findings

                  • MR Findings

                    • Other Modality Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Staging, Grading, & Classification

                              • Gross Pathologic & Surgical Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        Selected References

                                        1. Lewin M et al: Diffuse versus localized Caroli disease: a comparativesMRCP Study. AJR Am J Roentgenol. 216(6):1530-8, 2021
                                        2. Lai Q et al: Proposal for an algorithm for liver transplantation in Caroli's disease and syndrome: putting an uncommon effort into a common task. Clin Transplant. 30(1):3-9, 2016
                                        3. Moslim MA et al: Surgical management of Caroli's disease: single center experience and review of the literature. J Gastrointest Surg. 19(11):2019-27, 2015
                                        4. Rock N et al: Liver involvement in children with ciliopathies. Clin Res Hepatol Gastroenterol. 38(4):407-14, 2014
                                        5. Turkbey B et al: Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis (ARPKD/CHF). Pediatr Radiol. 39(2):100-11, 2009
                                        6. Brancatelli G et al: Fibropolycystic liver disease: CT and MR imaging findings. Radiographics. 25(3):659-70, 2005
                                        7. Levy AD et al: Caroli's disease: radiologic spectrum with pathologic correlation. AJR Am J Roentgenol. 179(4):1053-7, 2002
                                        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

                                                  • Definitions

                                                    • Caroli disease (CD): Saccular dilation of large intrahepatic bile ducts (IHBD)
                                                    • Congenital hepatic fibrosis (CHF): Presence of fibrotic tissue between portal tracts with persistent intralobular ducts
                                                    • Caroli syndrome (CS): CD + CHF &/or autosomal recessive polycystic kidney disease (ARPKD)
                                                    • Hepatorenal fibrocystic diseases: Renal fibrocystic degeneration + abnormalities of portobiliary system

                                                  IMAGING

                                                  • General Features

                                                    • Ultrasonographic Findings

                                                      • CT Findings

                                                        • MR Findings

                                                          • Other Modality Findings

                                                            • Imaging Recommendations

                                                              DIFFERENTIAL DIAGNOSIS

                                                                PATHOLOGY

                                                                • General Features

                                                                  • Staging, Grading, & Classification

                                                                    • Gross Pathologic & Surgical Features

                                                                      CLINICAL ISSUES

                                                                      • Presentation

                                                                        • Demographics

                                                                          • Natural History & Prognosis

                                                                            • Treatment

                                                                              Selected References

                                                                              1. Lewin M et al: Diffuse versus localized Caroli disease: a comparativesMRCP Study. AJR Am J Roentgenol. 216(6):1530-8, 2021
                                                                              2. Lai Q et al: Proposal for an algorithm for liver transplantation in Caroli's disease and syndrome: putting an uncommon effort into a common task. Clin Transplant. 30(1):3-9, 2016
                                                                              3. Moslim MA et al: Surgical management of Caroli's disease: single center experience and review of the literature. J Gastrointest Surg. 19(11):2019-27, 2015
                                                                              4. Rock N et al: Liver involvement in children with ciliopathies. Clin Res Hepatol Gastroenterol. 38(4):407-14, 2014
                                                                              5. Turkbey B et al: Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis (ARPKD/CHF). Pediatr Radiol. 39(2):100-11, 2009
                                                                              6. Brancatelli G et al: Fibropolycystic liver disease: CT and MR imaging findings. Radiographics. 25(3):659-70, 2005
                                                                              7. Levy AD et al: Caroli's disease: radiologic spectrum with pathologic correlation. AJR Am J Roentgenol. 179(4):1053-7, 2002