Congenital saccular dilatation of intrahepatic bile ducts (IHBDs)
Definitions
Originally divided into 2 variants
Caroli disease ("pure" or "simple" form):Congenital multifocal saccular dilation of large intrahepatic bile ducts (IHBDs) in absence of other hepatic abnormalities (type I Caroli disease)
Caroli syndrome ("complex" form): More common variant characterized by cystic dilatation of large intrahepatic ducts, hepatic fibrosis, and portal hypertension (type II Caroli disease)
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Ultrasonographic Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Cannella R et al: Congenital cystic lesions of the bile ducts: imaging-based diagnosis. Curr Probl Diagn Radiol. 49(4):285-93, 2020
Zhong Y et al: Hepatobiliary and pancreatic: Caroli syndrome: egg-like sign on CT. J Gastroenterol Hepatol. 35(2):180, 2020
Mamone G et al: Hepatic morphology abnormalities: beyond cirrhosis. Abdom Radiol (NY). 43(7):1612-26, 2018
Zahmatkeshan M et al: Liver transplantation for caroli disease. Int J Organ Transplant Med. 3(4):189-91, 2012
Zhang DY et al: Caroli's disease: a report of 14 patients and review of the literature. J Dig Dis. 13(9):491-5, 2012
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
Vachha B et al: Cystic lesions of the liver. AJR Am J Roentgenol. 196(4):W355-66, 2011
Venkatanarasimha N et al: Imaging features of ductal plate malformations in adults. Clin Radiol. 66(11):1086-93, 2011
Lall NU et al: Caroli disease and the central dot sign. Pediatr Radiol. 39(7):754, 2009
Veigel MC et al: Fibropolycystic liver disease in children. Pediatr Radiol. 39(4):317-27, 2009
Ananthakrishnan AN et al: Caroli's disease: identification and treatment strategy. Curr Gastroenterol Rep. 9(2):151-5, 2007
Brancatelli G et al: Fibropolycystic liver disease: CT and MR imaging findings. Radiographics. 25(3):659-70, 2005
Guy F et al: Caroli's disease: magnetic resonance imaging features. Eur Radiol. 12(11):2730-6, 2002
Krause D et al: MRI for evaluating congenital bile duct abnormalities. J Comput Assist Tomogr. 26(4):541-52, 2002
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
Asselah T et al: Caroli's disease: a magnetic resonance cholangiopancreatography diagnosis. Am J Gastroenterol. 93(1):109-10, 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
Pavone P et al: Caroli's disease: evaluation with MR cholangiography. AJR Am J Roentgenol. 166(1):216-7, 1996
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
Zangger P et al: MRI findings in Caroli's disease and intrahepatic pigmented calculi. Abdom Imaging. 20(4):361-4, 1995
Choi BI et al: Caroli disease: central dot sign in CT. Radiology. 174(1):161-3, 1990
Cowart VS: Boxing makes more headlines than usual and a lot of the news hasn't been good. JAMA. 261(1):14-6, 1989
Murphy BJ et al: The CT appearance of cystic masses of the liver. Radiographics. 9(2):307-22, 1989
Marchal GJ et al: Caroli disease: high-frequency US and pathologic findings. Radiology. 158(2):507-11, 1986
Related Anatomy
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Pathology
Clinical Issues
TERMINOLOGY
Synonyms
Communicating cavernous biliary ectasia
Congenital saccular dilatation of intrahepatic bile ducts (IHBDs)
Definitions
Originally divided into 2 variants
Caroli disease ("pure" or "simple" form):Congenital multifocal saccular dilation of large intrahepatic bile ducts (IHBDs) in absence of other hepatic abnormalities (type I Caroli disease)
Caroli syndrome ("complex" form): More common variant characterized by cystic dilatation of large intrahepatic ducts, hepatic fibrosis, and portal hypertension (type II Caroli disease)
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Ultrasonographic Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Cannella R et al: Congenital cystic lesions of the bile ducts: imaging-based diagnosis. Curr Probl Diagn Radiol. 49(4):285-93, 2020
Zhong Y et al: Hepatobiliary and pancreatic: Caroli syndrome: egg-like sign on CT. J Gastroenterol Hepatol. 35(2):180, 2020
Mamone G et al: Hepatic morphology abnormalities: beyond cirrhosis. Abdom Radiol (NY). 43(7):1612-26, 2018
Zahmatkeshan M et al: Liver transplantation for caroli disease. Int J Organ Transplant Med. 3(4):189-91, 2012
Zhang DY et al: Caroli's disease: a report of 14 patients and review of the literature. J Dig Dis. 13(9):491-5, 2012
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
Vachha B et al: Cystic lesions of the liver. AJR Am J Roentgenol. 196(4):W355-66, 2011
Venkatanarasimha N et al: Imaging features of ductal plate malformations in adults. Clin Radiol. 66(11):1086-93, 2011
Lall NU et al: Caroli disease and the central dot sign. Pediatr Radiol. 39(7):754, 2009
Veigel MC et al: Fibropolycystic liver disease in children. Pediatr Radiol. 39(4):317-27, 2009
Ananthakrishnan AN et al: Caroli's disease: identification and treatment strategy. Curr Gastroenterol Rep. 9(2):151-5, 2007
Brancatelli G et al: Fibropolycystic liver disease: CT and MR imaging findings. Radiographics. 25(3):659-70, 2005
Guy F et al: Caroli's disease: magnetic resonance imaging features. Eur Radiol. 12(11):2730-6, 2002
Krause D et al: MRI for evaluating congenital bile duct abnormalities. J Comput Assist Tomogr. 26(4):541-52, 2002
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
Asselah T et al: Caroli's disease: a magnetic resonance cholangiopancreatography diagnosis. Am J Gastroenterol. 93(1):109-10, 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
Pavone P et al: Caroli's disease: evaluation with MR cholangiography. AJR Am J Roentgenol. 166(1):216-7, 1996
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
Zangger P et al: MRI findings in Caroli's disease and intrahepatic pigmented calculi. Abdom Imaging. 20(4):361-4, 1995
Choi BI et al: Caroli disease: central dot sign in CT. Radiology. 174(1):161-3, 1990
Cowart VS: Boxing makes more headlines than usual and a lot of the news hasn't been good. JAMA. 261(1):14-6, 1989
Murphy BJ et al: The CT appearance of cystic masses of the liver. Radiographics. 9(2):307-22, 1989
Marchal GJ et al: Caroli disease: high-frequency US and pathologic findings. Radiology. 158(2):507-11, 1986
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