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Gastrointestinal
Diagnosis
Liver
Congenital
Congenital Hepatic Fibrosis
Congenital Hepatic Fibrosis
Atif Zaheer, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Congenital hepatic fibrosis (CHF)

          • Definitions

            • Part of spectrum of congenital abnormalities resulting in variable degrees of fibrosis and cystic anomalies of liver and kidneys (fibropolycystic liver disease)
              • CHF, biliary hamartomas, polycystic liver disease, choledochal cyst, and Caroli syndrome

          IMAGING

          • General Features

            • Radiographic Findings

              • CT Findings

                • MR Findings

                  • Ultrasonographic 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. Alsomali MI et al: Diagnosis of congenital hepatic fibrosis in adulthood. Am J Clin Pathol. 153(1):119-25, 2020
                                          2. Zhu B et al: Congenital hepatic fibrosis in children and adults: clinical manifestations, management, and outcome-case series and literature review. Gastroenterol Res Pract. 2020:8284274, 2020
                                          3. Jiang C et al: Congenital hepatic fibrosis with polycystic kidney disease: two case reports. Medicine (Baltimore). 98(20):e15600, 2019
                                          4. Mamone G et al: Hepatic morphology abnormalities: beyond cirrhosis. Abdom Radiol (NY). 43(7):1612-26, 2018
                                          5. Hartung EA et al: Autosomal recessive polycystic kidney disease: a hepatorenal fibrocystic disorder with pleiotropic effects. Pediatrics. 134(3):e833-45, 2014
                                          6. Vajro P et al: Management of adults with paediatric-onset chronic liver disease: strategic issues for transition care. Dig Liver Dis. 46(4):295-301, 2014
                                          7. O'Brien K et al: Congenital hepatic fibrosis and portal hypertension in autosomal dominant polycystic kidney disease. J Pediatr Gastroenterol Nutr. 54(1):83-9, 2012
                                          8. Venkatanarasimha N et al: Imaging features of ductal plate malformations in adults. Clin Radiol. 66(11):1086-93, 2011
                                          9. Brancatelli G et al: Fibropolycystic liver disease: CT and MR imaging findings. Radiographics. 25(3):659-70, 2005
                                          10. Lonergan GJ et al: Autosomal recessive polycystic kidney disease: radiologic-pathologic correlation. Radiographics. 20(3):837-55, 2000
                                          11. Khan K et al: Morbidity from congenital hepatic fibrosis after renal transplantation for autosomal recessive polycystic kidney disease. Am J Transplant. 2(4):360-5, 2002
                                          12. Desmet VJ: Ludwig symposium on biliary disorders--part I. Pathogenesis of ductal plate abnormalities. Mayo Clin Proc. 73(1):80-9, 1998
                                          13. Ernst O et al: Congenital hepatic fibrosis: findings at MR cholangiopancreatography. AJR Am J Roentgenol. 170(2):409-12, 1998
                                          14. Kaplan BS et al: Variable expression of autosomal recessive polycystic kidney disease and congenital hepatic fibrosis within a family. Am J Med Genet. 29(3):639-47, 1988
                                          15. Premkumar A et al: The emergence of hepatic fibrosis and portal hypertension in infants and children with autosomal recessive polycystic kidney disease. Initial and follow-up sonographic and radiographic findings. Pediatr Radiol. 18(2):123-9, 1988
                                          16. Proesmans W et al: Association of bilateral renal dysplasia and congenital hepatic fibrosis. Int J Pediatr Nephrol. 7(2):113-6, 1986
                                          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

                                                  TERMINOLOGY

                                                  • Abbreviations

                                                    • Congenital hepatic fibrosis (CHF)

                                                  • Definitions

                                                    • Part of spectrum of congenital abnormalities resulting in variable degrees of fibrosis and cystic anomalies of liver and kidneys (fibropolycystic liver disease)
                                                      • CHF, biliary hamartomas, polycystic liver disease, choledochal cyst, and Caroli syndrome

                                                  IMAGING

                                                  • General Features

                                                    • Radiographic Findings

                                                      • CT Findings

                                                        • MR Findings

                                                          • Ultrasonographic 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. Alsomali MI et al: Diagnosis of congenital hepatic fibrosis in adulthood. Am J Clin Pathol. 153(1):119-25, 2020
                                                                                  2. Zhu B et al: Congenital hepatic fibrosis in children and adults: clinical manifestations, management, and outcome-case series and literature review. Gastroenterol Res Pract. 2020:8284274, 2020
                                                                                  3. Jiang C et al: Congenital hepatic fibrosis with polycystic kidney disease: two case reports. Medicine (Baltimore). 98(20):e15600, 2019
                                                                                  4. Mamone G et al: Hepatic morphology abnormalities: beyond cirrhosis. Abdom Radiol (NY). 43(7):1612-26, 2018
                                                                                  5. Hartung EA et al: Autosomal recessive polycystic kidney disease: a hepatorenal fibrocystic disorder with pleiotropic effects. Pediatrics. 134(3):e833-45, 2014
                                                                                  6. Vajro P et al: Management of adults with paediatric-onset chronic liver disease: strategic issues for transition care. Dig Liver Dis. 46(4):295-301, 2014
                                                                                  7. O'Brien K et al: Congenital hepatic fibrosis and portal hypertension in autosomal dominant polycystic kidney disease. J Pediatr Gastroenterol Nutr. 54(1):83-9, 2012
                                                                                  8. Venkatanarasimha N et al: Imaging features of ductal plate malformations in adults. Clin Radiol. 66(11):1086-93, 2011
                                                                                  9. Brancatelli G et al: Fibropolycystic liver disease: CT and MR imaging findings. Radiographics. 25(3):659-70, 2005
                                                                                  10. Lonergan GJ et al: Autosomal recessive polycystic kidney disease: radiologic-pathologic correlation. Radiographics. 20(3):837-55, 2000
                                                                                  11. Khan K et al: Morbidity from congenital hepatic fibrosis after renal transplantation for autosomal recessive polycystic kidney disease. Am J Transplant. 2(4):360-5, 2002
                                                                                  12. Desmet VJ: Ludwig symposium on biliary disorders--part I. Pathogenesis of ductal plate abnormalities. Mayo Clin Proc. 73(1):80-9, 1998
                                                                                  13. Ernst O et al: Congenital hepatic fibrosis: findings at MR cholangiopancreatography. AJR Am J Roentgenol. 170(2):409-12, 1998
                                                                                  14. Kaplan BS et al: Variable expression of autosomal recessive polycystic kidney disease and congenital hepatic fibrosis within a family. Am J Med Genet. 29(3):639-47, 1988
                                                                                  15. Premkumar A et al: The emergence of hepatic fibrosis and portal hypertension in infants and children with autosomal recessive polycystic kidney disease. Initial and follow-up sonographic and radiographic findings. Pediatr Radiol. 18(2):123-9, 1988
                                                                                  16. Proesmans W et al: Association of bilateral renal dysplasia and congenital hepatic fibrosis. Int J Pediatr Nephrol. 7(2):113-6, 1986