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Peribiliary Cysts
Atif Zaheer, MD; Michael P. Federle, MD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Synonyms

              • Hepatic hilar cyst, mucinous hamartoma of bile ducts
              • Multiple cysts in hepatic hilum, multiple hilar cysts
              • Hepatic cysts of periductal gland origin 
              • Mucinous hamartoma of biliary system 
              • Hepatic peribiliary cysts, peribiliary cysts
              • Nakanuma disease 
            • Definitions

              • Cystic dilation of obstructed periductal glands of intrahepatic bile ducts

            IMAGING

            • General Features

              • CT Findings

                • MR Findings

                  • Ultrasonographic Findings

                    • Cholangiography

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Gross Pathologic & Surgical Features

                            • Microscopic Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    Selected References

                                    1. Pedica F et al: Peribiliary glands pathology in a large series of end-stage alcohol-related liver disease. Virchows Arch. 477(6):817-23, 2020
                                    2. Bazerbachi F et al: Peribiliary cysts: a systematic review and proposal of a classification framework. BMJ Open Gastroenterol. 5(1):e000204, 2018
                                    3. Matsubara T et al: Alcohol-related injury to peribiliary glands is a cause of peribiliary cysts: based on analysis of clinical and autopsy cases. J Clin Gastroenterol. 48(2):153-9, 2014
                                    4. Da Ines D et al: Peribiliary cysts. Hepatology. 54(6):2271-2, 2011
                                    5. Karcaaltincaba M et al: Multidetector CT and MRI findings in periportal space pathologies. Eur J Radiol. 61(1):3-10, 2007
                                    6. Itai Y et al: Hepatobiliary cysts in patients with autosomal dominant polycystic kidney disease: prevalence and CT findings. AJR Am J Roentgenol. 164(2):339-42, 1995
                                    7. Terayama N et al: Peribiliary cysts in liver cirrhosis: US, CT, and MR findings. J Comput Assist Tomogr. 19(3):419-23, 1995
                                    8. Baron RL et al: Peribiliary cysts associated with severe liver disease: imaging-pathologic correlation. AJR Am J Roentgenol. 162(3):631-6, 1994
                                    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

                                              • Synonyms

                                                • Hepatic hilar cyst, mucinous hamartoma of bile ducts
                                                • Multiple cysts in hepatic hilum, multiple hilar cysts
                                                • Hepatic cysts of periductal gland origin 
                                                • Mucinous hamartoma of biliary system 
                                                • Hepatic peribiliary cysts, peribiliary cysts
                                                • Nakanuma disease 
                                              • Definitions

                                                • Cystic dilation of obstructed periductal glands of intrahepatic bile ducts

                                              IMAGING

                                              • General Features

                                                • CT Findings

                                                  • MR Findings

                                                    • Ultrasonographic Findings

                                                      • Cholangiography

                                                        DIFFERENTIAL DIAGNOSIS

                                                          PATHOLOGY

                                                          • General Features

                                                            • Gross Pathologic & Surgical Features

                                                              • Microscopic Features

                                                                CLINICAL ISSUES

                                                                • Presentation

                                                                  • Demographics

                                                                    • Natural History & Prognosis

                                                                      Selected References

                                                                      1. Pedica F et al: Peribiliary glands pathology in a large series of end-stage alcohol-related liver disease. Virchows Arch. 477(6):817-23, 2020
                                                                      2. Bazerbachi F et al: Peribiliary cysts: a systematic review and proposal of a classification framework. BMJ Open Gastroenterol. 5(1):e000204, 2018
                                                                      3. Matsubara T et al: Alcohol-related injury to peribiliary glands is a cause of peribiliary cysts: based on analysis of clinical and autopsy cases. J Clin Gastroenterol. 48(2):153-9, 2014
                                                                      4. Da Ines D et al: Peribiliary cysts. Hepatology. 54(6):2271-2, 2011
                                                                      5. Karcaaltincaba M et al: Multidetector CT and MRI findings in periportal space pathologies. Eur J Radiol. 61(1):3-10, 2007
                                                                      6. Itai Y et al: Hepatobiliary cysts in patients with autosomal dominant polycystic kidney disease: prevalence and CT findings. AJR Am J Roentgenol. 164(2):339-42, 1995
                                                                      7. Terayama N et al: Peribiliary cysts in liver cirrhosis: US, CT, and MR findings. J Comput Assist Tomogr. 19(3):419-23, 1995
                                                                      8. Baron RL et al: Peribiliary cysts associated with severe liver disease: imaging-pathologic correlation. AJR Am J Roentgenol. 162(3):631-6, 1994