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
Alsomali MI et al: Diagnosis of congenital hepatic fibrosis in adulthood. Am J Clin Pathol. 153(1):119-25, 2020
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
Jiang C et al: Congenital hepatic fibrosis with polycystic kidney disease: two case reports. Medicine (Baltimore). 98(20):e15600, 2019
Mamone G et al: Hepatic morphology abnormalities: beyond cirrhosis. Abdom Radiol (NY). 43(7):1612-26, 2018
Hartung EA et al: Autosomal recessive polycystic kidney disease: a hepatorenal fibrocystic disorder with pleiotropic effects. Pediatrics. 134(3):e833-45, 2014
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
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
Venkatanarasimha N et al: Imaging features of ductal plate malformations in adults. Clin Radiol. 66(11):1086-93, 2011
Brancatelli G et al: Fibropolycystic liver disease: CT and MR imaging findings. Radiographics. 25(3):659-70, 2005
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
Desmet VJ: Ludwig symposium on biliary disorders--part I. Pathogenesis of ductal plate abnormalities. Mayo Clin Proc. 73(1):80-9, 1998
Ernst O et al: Congenital hepatic fibrosis: findings at MR cholangiopancreatography. AJR Am J Roentgenol. 170(2):409-12, 1998
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
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
Proesmans W et al: Association of bilateral renal dysplasia and congenital hepatic fibrosis. Int J Pediatr Nephrol. 7(2):113-6, 1986
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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
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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
Alsomali MI et al: Diagnosis of congenital hepatic fibrosis in adulthood. Am J Clin Pathol. 153(1):119-25, 2020
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
Jiang C et al: Congenital hepatic fibrosis with polycystic kidney disease: two case reports. Medicine (Baltimore). 98(20):e15600, 2019
Mamone G et al: Hepatic morphology abnormalities: beyond cirrhosis. Abdom Radiol (NY). 43(7):1612-26, 2018
Hartung EA et al: Autosomal recessive polycystic kidney disease: a hepatorenal fibrocystic disorder with pleiotropic effects. Pediatrics. 134(3):e833-45, 2014
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
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
Venkatanarasimha N et al: Imaging features of ductal plate malformations in adults. Clin Radiol. 66(11):1086-93, 2011
Brancatelli G et al: Fibropolycystic liver disease: CT and MR imaging findings. Radiographics. 25(3):659-70, 2005
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
Desmet VJ: Ludwig symposium on biliary disorders--part I. Pathogenesis of ductal plate abnormalities. Mayo Clin Proc. 73(1):80-9, 1998
Ernst O et al: Congenital hepatic fibrosis: findings at MR cholangiopancreatography. AJR Am J Roentgenol. 170(2):409-12, 1998
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
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
Proesmans W et al: Association of bilateral renal dysplasia and congenital hepatic fibrosis. Int J Pediatr Nephrol. 7(2):113-6, 1986
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