link
Bookmarks
Caroli Disease
Alexander J. Towbin, MD
To access 4,300 diagnoses written by the world's leading experts in radiology, please log in or subscribe.Log inSubscribe
0
16
4
11

KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Definitions

              • Congenital hepatic fibrosis (CHF): Presence of fibrotic tissue between portal tracts with persistent ductal plate structure of intralobular ducts
              • Caroli disease (CD): Saccular dilation of large intrahepatic bile ducts (IHBD)
              • Caroli syndrome (CS): CHF + CD
              • Hepatorenal fibrocystic diseases: Renal fibrocystic degeneration + abnormalities of portobiliary system

            IMAGING

            • General Features

              • CT Findings

                • MR Findings

                  • Ultrasonographic 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. 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
                                        2. Rock N et al: Liver involvement in children with ciliopathies. Clin Res Hepatol Gastroenterol. 38(4):407-14, 2014
                                        3. Turkbey B et al: Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis (ARPKD/CHF). Pediatr Radiol. 39(2):100-11, 2009
                                        4. Brancatelli G et al: Fibropolycystic liver disease: CT and MR imaging findings. Radiographics. 25(3):659-70, 2005
                                        5. Levy AD et al: Caroli's disease: radiologic spectrum with pathologic correlation. AJR Am J Roentgenol. 179(4):1053-7, 2002
                                        6. Asselah T et al: Caroli's disease: a magnetic resonance cholangiopancreatography diagnosis. Am J Gastroenterol. 93(1):109-10, 1998
                                        Related Anatomy
                                        Loading...
                                        Related Differential Diagnoses
                                        Loading...
                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Top Differential Diagnoses

                                              • Pathology

                                                • Clinical Issues

                                                  TERMINOLOGY

                                                  • Definitions

                                                    • Congenital hepatic fibrosis (CHF): Presence of fibrotic tissue between portal tracts with persistent ductal plate structure of intralobular ducts
                                                    • Caroli disease (CD): Saccular dilation of large intrahepatic bile ducts (IHBD)
                                                    • Caroli syndrome (CS): CHF + CD
                                                    • Hepatorenal fibrocystic diseases: Renal fibrocystic degeneration + abnormalities of portobiliary system

                                                  IMAGING

                                                  • General Features

                                                    • CT Findings

                                                      • MR Findings

                                                        • Ultrasonographic 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. 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
                                                                              2. Rock N et al: Liver involvement in children with ciliopathies. Clin Res Hepatol Gastroenterol. 38(4):407-14, 2014
                                                                              3. Turkbey B et al: Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis (ARPKD/CHF). Pediatr Radiol. 39(2):100-11, 2009
                                                                              4. Brancatelli G et al: Fibropolycystic liver disease: CT and MR imaging findings. Radiographics. 25(3):659-70, 2005
                                                                              5. Levy AD et al: Caroli's disease: radiologic spectrum with pathologic correlation. AJR Am J Roentgenol. 179(4):1053-7, 2002
                                                                              6. Asselah T et al: Caroli's disease: a magnetic resonance cholangiopancreatography diagnosis. Am J Gastroenterol. 93(1):109-10, 1998