link
Bookmarks
Gastrointestinal
Diagnosis
Liver
Congenital
Congenital Hepatic Fibrosis
Congenital Hepatic Fibrosis
Atif Zaheer, MD
To access 4,300 diagnoses written by the world's leading experts in radiology, please log in or subscribe.Subscribe
0
8
2
4

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
                                          Loading...
                                          Related Differential Diagnoses
                                          Loading...
                                          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