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AD Polycystic Liver Disease
Michael P. Federle, MD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Autosomal dominant polycystic liver disease (ADPLD) or adult PLD
            • Definitions

              • Uncommon inherited disorder
              • Part of fibropolycystic liver disease spectrum
                • Constitutes group of related lesions of liver and biliary tract caused by abnormal development of embryological ductal plate

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • MR Findings

                    • Ultrasonographic 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. Benzimra J et al: Hepatic cysts treated with percutaneous ethanol sclerotherapy: time to extend the indications to haemorrhagic cysts and polycystic liver disease. Eur Radiol. 24(5):1030-8, 2014
                                              2. Abu-Wasel B et al: Pathophysiology, epidemiology, classification and treatment options for polycystic liver diseases. World J Gastroenterol. 19(35):5775-86, 2013
                                              3. Fedeles SV et al: A genetic interaction network of five genes for human polycystic kidney and liver diseases defines polycystin-1 as the central determinant of cyst formation. Nat Genet. 43(7):639-47, 2011
                                              4. Gevers TJ et al: Somatostatin analogues for treatment of polycystic liver disease. Curr Opin Gastroenterol. 27(3):294-300, 2011
                                              5. Venkatanarasimha N et al: Imaging features of ductal plate malformations in adults. Clin Radiol. 66(11):1086-93, 2011
                                              6. Everson GT et al: Advances in management of polycystic liver disease. Expert Rev Gastroenterol Hepatol. 2(4):563-76, 2008
                                              7. Morgan DE et al: Polycystic liver disease: multimodality imaging for complications and transplant evaluation. Radiographics. 26(6):1655-68; quiz 1655, 2006
                                              8. Brancatelli G et al: Fibropolycystic liver disease: CT and MR imaging findings. Radiographics. 25(3):659-70, 2005
                                              9. Qian Q et al: Clinical profile of autosomal dominant polycystic liver disease. Hepatology. 37(1):164-71, 2003
                                              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. Steinberg ML et al: MRI and CT features of polycystic liver disease. N J Med. 90(5):398-400, 1993
                                              12. Wan SK et al: Sonographic and computed tomographic features of polycystic disease of the liver. Gastrointest Radiol. 15(4):310-2, 1990
                                              13. Wilcox DM et al: MR imaging of a hemorrhagic hepatic cyst in a patient with polycystic liver disease. J Comput Assist Tomogr. 9(1):183-5, 1985
                                              14. Segal AJ et al: Computed tomography of adult polycystic disease. J Comput Assist Tomogr. 6(4):777-80, 1982
                                              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

                                                        • Abbreviations

                                                          • Autosomal dominant polycystic liver disease (ADPLD) or adult PLD
                                                        • Definitions

                                                          • Uncommon inherited disorder
                                                          • Part of fibropolycystic liver disease spectrum
                                                            • Constitutes group of related lesions of liver and biliary tract caused by abnormal development of embryological ductal plate

                                                        IMAGING

                                                        • General Features

                                                          • Radiographic Findings

                                                            • CT Findings

                                                              • MR Findings

                                                                • Ultrasonographic 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. Benzimra J et al: Hepatic cysts treated with percutaneous ethanol sclerotherapy: time to extend the indications to haemorrhagic cysts and polycystic liver disease. Eur Radiol. 24(5):1030-8, 2014
                                                                                          2. Abu-Wasel B et al: Pathophysiology, epidemiology, classification and treatment options for polycystic liver diseases. World J Gastroenterol. 19(35):5775-86, 2013
                                                                                          3. Fedeles SV et al: A genetic interaction network of five genes for human polycystic kidney and liver diseases defines polycystin-1 as the central determinant of cyst formation. Nat Genet. 43(7):639-47, 2011
                                                                                          4. Gevers TJ et al: Somatostatin analogues for treatment of polycystic liver disease. Curr Opin Gastroenterol. 27(3):294-300, 2011
                                                                                          5. Venkatanarasimha N et al: Imaging features of ductal plate malformations in adults. Clin Radiol. 66(11):1086-93, 2011
                                                                                          6. Everson GT et al: Advances in management of polycystic liver disease. Expert Rev Gastroenterol Hepatol. 2(4):563-76, 2008
                                                                                          7. Morgan DE et al: Polycystic liver disease: multimodality imaging for complications and transplant evaluation. Radiographics. 26(6):1655-68; quiz 1655, 2006
                                                                                          8. Brancatelli G et al: Fibropolycystic liver disease: CT and MR imaging findings. Radiographics. 25(3):659-70, 2005
                                                                                          9. Qian Q et al: Clinical profile of autosomal dominant polycystic liver disease. Hepatology. 37(1):164-71, 2003
                                                                                          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. Steinberg ML et al: MRI and CT features of polycystic liver disease. N J Med. 90(5):398-400, 1993
                                                                                          12. Wan SK et al: Sonographic and computed tomographic features of polycystic disease of the liver. Gastrointest Radiol. 15(4):310-2, 1990
                                                                                          13. Wilcox DM et al: MR imaging of a hemorrhagic hepatic cyst in a patient with polycystic liver disease. J Comput Assist Tomogr. 9(1):183-5, 1985
                                                                                          14. Segal AJ et al: Computed tomography of adult polycystic disease. J Comput Assist Tomogr. 6(4):777-80, 1982