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Gastrointestinal
Diagnosis
Liver
Congenital
AD Polycystic Liver Disease
AD Polycystic Liver Disease
Atif Zaheer, MD; 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
              • 2 forms of PLD: Isolated PLD and PLD in association with polycystic kidney disease (PKD)
              • Part of fibropolycystic liver disease spectrum
                • Constitutes group of related lesions of liver and biliary tract caused by abnormal development of embryologic ductal plate

            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. Zhang ZY et al: Polycystic liver disease: classification, diagnosis, treatment process, and clinical management. World J Hepatol. 12(3):72-83, 2020
                                                2. Aussilhou B et al: Treatment of polycystic liver disease. Update on the management. J Visc Surg. 155(6):471-81, 2018
                                                3. Lanktree MB et al: Prevalence estimates of polycystic kidney and liver disease by population sequencing. J Am Soc Nephrol. 29(10):2593-600, 2018
                                                4. Mamone G et al: Hepatic morphology abnormalities: beyond cirrhosis. Abdom Radiol (NY). 43(7):1612-26, 2018
                                                5. van Aerts RMM et al: Clinical management of polycystic liver disease. J Hepatol. 68(4):827-37, 2018
                                                6. Kim JA et al: Pancreatic cysts in autosomal dominant polycystic kidney disease: prevalence and association with PKD2 gene mutations. Radiology. 280(3):762-70, 2016
                                                7. 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
                                                8. Abu-Wasel B et al: Pathophysiology, epidemiology, classification and treatment options for polycystic liver diseases. World J Gastroenterol. 19(35):5775-86, 2013
                                                9. 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
                                                10. Gevers TJ et al: Somatostatin analogues for treatment of polycystic liver disease. Curr Opin Gastroenterol. 27(3):294-300, 2011
                                                11. Venkatanarasimha N et al: Imaging features of ductal plate malformations in adults. Clin Radiol. 66(11):1086-93, 2011
                                                12. Everson GT et al: Advances in management of polycystic liver disease. Expert Rev Gastroenterol Hepatol. 2(4):563-76, 2008
                                                13. Morgan DE et al: Polycystic liver disease: multimodality imaging for complications and transplant evaluation. Radiographics. 26(6):1655-68; quiz 1655, 2006
                                                14. Brancatelli G et al: Fibropolycystic liver disease: CT and MR imaging findings. Radiographics. 25(3):659-70, 2005
                                                15. Qian Q et al: Clinical profile of autosomal dominant polycystic liver disease. Hepatology. 37(1):164-71, 2003
                                                16. Mortele KJ et al: Cystic focal liver lesions in the adult: differential CT and MR imaging features. Radiographics. 21(4):895-910, 2001
                                                17. Steinberg ML et al: MRI and CT features of polycystic liver disease. N J Med. 90(5):398-400, 1993
                                                18. Wan SK et al: Sonographic and computed tomographic features of polycystic disease of the liver. Gastrointest Radiol. 15(4):310-2, 1990
                                                19. 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
                                                20. 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
                                                            • 2 forms of PLD: Isolated PLD and PLD in association with polycystic kidney disease (PKD)
                                                            • Part of fibropolycystic liver disease spectrum
                                                              • Constitutes group of related lesions of liver and biliary tract caused by abnormal development of embryologic ductal plate

                                                          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. Zhang ZY et al: Polycystic liver disease: classification, diagnosis, treatment process, and clinical management. World J Hepatol. 12(3):72-83, 2020
                                                                                              2. Aussilhou B et al: Treatment of polycystic liver disease. Update on the management. J Visc Surg. 155(6):471-81, 2018
                                                                                              3. Lanktree MB et al: Prevalence estimates of polycystic kidney and liver disease by population sequencing. J Am Soc Nephrol. 29(10):2593-600, 2018
                                                                                              4. Mamone G et al: Hepatic morphology abnormalities: beyond cirrhosis. Abdom Radiol (NY). 43(7):1612-26, 2018
                                                                                              5. van Aerts RMM et al: Clinical management of polycystic liver disease. J Hepatol. 68(4):827-37, 2018
                                                                                              6. Kim JA et al: Pancreatic cysts in autosomal dominant polycystic kidney disease: prevalence and association with PKD2 gene mutations. Radiology. 280(3):762-70, 2016
                                                                                              7. 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
                                                                                              8. Abu-Wasel B et al: Pathophysiology, epidemiology, classification and treatment options for polycystic liver diseases. World J Gastroenterol. 19(35):5775-86, 2013
                                                                                              9. 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
                                                                                              10. Gevers TJ et al: Somatostatin analogues for treatment of polycystic liver disease. Curr Opin Gastroenterol. 27(3):294-300, 2011
                                                                                              11. Venkatanarasimha N et al: Imaging features of ductal plate malformations in adults. Clin Radiol. 66(11):1086-93, 2011
                                                                                              12. Everson GT et al: Advances in management of polycystic liver disease. Expert Rev Gastroenterol Hepatol. 2(4):563-76, 2008
                                                                                              13. Morgan DE et al: Polycystic liver disease: multimodality imaging for complications and transplant evaluation. Radiographics. 26(6):1655-68; quiz 1655, 2006
                                                                                              14. Brancatelli G et al: Fibropolycystic liver disease: CT and MR imaging findings. Radiographics. 25(3):659-70, 2005
                                                                                              15. Qian Q et al: Clinical profile of autosomal dominant polycystic liver disease. Hepatology. 37(1):164-71, 2003
                                                                                              16. Mortele KJ et al: Cystic focal liver lesions in the adult: differential CT and MR imaging features. Radiographics. 21(4):895-910, 2001
                                                                                              17. Steinberg ML et al: MRI and CT features of polycystic liver disease. N J Med. 90(5):398-400, 1993
                                                                                              18. Wan SK et al: Sonographic and computed tomographic features of polycystic disease of the liver. Gastrointest Radiol. 15(4):310-2, 1990
                                                                                              19. 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
                                                                                              20. Segal AJ et al: Computed tomography of adult polycystic disease. J Comput Assist Tomogr. 6(4):777-80, 1982