Congenital, nonobstructive, saccular, multisegmental dilatation of large intrahepatic bile ducts (IHBDs)
IMAGING
General Features
Ultrasonographic Findings
Radiographic Findings
CT Findings
MR Findings
Nuclear Medicine 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
Karaosmanoglu AD et al: Congenital and hereditary cystic diseases of the abdomen. Insights Imaging. 11(1):90, 2020
Rivas A et al: Prenatal MR imaging features of Caroli syndrome in association with autosomal recessive polycystic kidney disease. Radiol Case Rep. 14(2):265-68, 2019
Hwang MJ et al: Diffuse-type Caroli disease with characteristic central dot sign complicated by multiple intrahepatic and common bile duct stones. Clin Endosc. 50(4):400-3, 2017
Gunay-Aygun M et al: Characteristics of congenital hepatic fibrosis in a large cohort of patients with autosomal recessive polycystic kidney disease. Gastroenterology. 144(1):112-21, 2013
Lefere M et al: Caroli disease: review of eight cases with emphasis on magnetic resonance imaging features. Eur J Gastroenterol Hepatol. 23(7):578-85, 2011
Venkatanarasimha N et al: Imaging features of ductal plate malformations in adults. Clin Radiol. 66(11):1086-93, 2011
Ananthakrishnan AN et al: Caroli's disease: identification and treatment strategy. Curr Gastroenterol Rep. 9(2):151-5, 2007
Yonem O et al: Clinical characteristics of Caroli's syndrome. World J Gastroenterol. 13(13):1934-7, 2007
Madjov R et al: Caroli's disease. Report of 5 cases and review of literature. Hepatogastroenterology. 52(62):606-9, 2005
Sgro M et al: Caroli's disease: prenatal diagnosis, postnatal outcome and genetic analysis. Ultrasound Obstet Gynecol. 23(1):73-6, 2004
Levy AD et al: Caroli's disease: radiologic spectrum with pathologic correlation. AJR Am J Roentgenol. 179(4):1053-7, 2002
Fulcher AS et al: Case 38: Caroli disease and renal tubular ectasia. Radiology. 220(3):720-3, 2001
Mortele KJ et al: Cystic focal liver lesions in the adult: differential CT and MR imaging features. Radiographics. 21(4):895-910, 2001
Akin O et al: An unusual sonographic finding in Caroli's disease. AJR Am J Roentgenol. 171(4):1167, 1998
Gorka W et al: Value of Doppler sonography in the assessment of patients with Caroli's disease. J Clin Ultrasound. 26(6):283-7, 1998
Miller WJ et al: Imaging findings in Caroli's disease. AJR Am J Roentgenol. 165(2):333-7, 1995
Rizzo RJ et al: Congenital abnormalities of the pancreas and biliary tree in adults. Radiographics. 15(1):49-68; quiz 147-8, 1995
Choi BI et al: Caroli disease: central dot sign in CT. Radiology. 174(1):161-3, 1990
Related Anatomy
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References
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Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Synonyms
Communicating cavernous biliary ectasia
Definitions
Congenital, nonobstructive, saccular, multisegmental dilatation of large intrahepatic bile ducts (IHBDs)
IMAGING
General Features
Ultrasonographic Findings
Radiographic Findings
CT Findings
MR Findings
Nuclear Medicine 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
Karaosmanoglu AD et al: Congenital and hereditary cystic diseases of the abdomen. Insights Imaging. 11(1):90, 2020
Rivas A et al: Prenatal MR imaging features of Caroli syndrome in association with autosomal recessive polycystic kidney disease. Radiol Case Rep. 14(2):265-68, 2019
Hwang MJ et al: Diffuse-type Caroli disease with characteristic central dot sign complicated by multiple intrahepatic and common bile duct stones. Clin Endosc. 50(4):400-3, 2017
Gunay-Aygun M et al: Characteristics of congenital hepatic fibrosis in a large cohort of patients with autosomal recessive polycystic kidney disease. Gastroenterology. 144(1):112-21, 2013
Lefere M et al: Caroli disease: review of eight cases with emphasis on magnetic resonance imaging features. Eur J Gastroenterol Hepatol. 23(7):578-85, 2011
Venkatanarasimha N et al: Imaging features of ductal plate malformations in adults. Clin Radiol. 66(11):1086-93, 2011
Ananthakrishnan AN et al: Caroli's disease: identification and treatment strategy. Curr Gastroenterol Rep. 9(2):151-5, 2007
Yonem O et al: Clinical characteristics of Caroli's syndrome. World J Gastroenterol. 13(13):1934-7, 2007
Madjov R et al: Caroli's disease. Report of 5 cases and review of literature. Hepatogastroenterology. 52(62):606-9, 2005
Sgro M et al: Caroli's disease: prenatal diagnosis, postnatal outcome and genetic analysis. Ultrasound Obstet Gynecol. 23(1):73-6, 2004
Levy AD et al: Caroli's disease: radiologic spectrum with pathologic correlation. AJR Am J Roentgenol. 179(4):1053-7, 2002
Fulcher AS et al: Case 38: Caroli disease and renal tubular ectasia. Radiology. 220(3):720-3, 2001
Mortele KJ et al: Cystic focal liver lesions in the adult: differential CT and MR imaging features. Radiographics. 21(4):895-910, 2001
Akin O et al: An unusual sonographic finding in Caroli's disease. AJR Am J Roentgenol. 171(4):1167, 1998
Gorka W et al: Value of Doppler sonography in the assessment of patients with Caroli's disease. J Clin Ultrasound. 26(6):283-7, 1998
Miller WJ et al: Imaging findings in Caroli's disease. AJR Am J Roentgenol. 165(2):333-7, 1995
Rizzo RJ et al: Congenital abnormalities of the pancreas and biliary tree in adults. Radiographics. 15(1):49-68; quiz 147-8, 1995
Choi BI et al: Caroli disease: central dot sign in CT. Radiology. 174(1):161-3, 1990
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