Abstract
Caroli’s disease (CD) is a rare autosomic recessive congenital pathology that belongs to the spectrum of fibro-polycystic liver disease, a group of entities caused by an abnormal embryologic development of the ductal plate. CD is characterized by non-obstructive dilatations of the large intrahepatic bile ducts that communicate with the biliary system. It can be diffuse, segmental, or multifocal. “Caroli syndrome” defines the presence of CD and hepatic fibrosis. The diagnosis is based upon Magnetic Resonance (MR) findings. Typical symptoms include right upper quadrant abdominal pain and jaundice, related to recurrent bile stasis, with consequent stone formations and cholangitis episodes.
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Similar content being viewed by others
References
Ahmadi T, Itai Y, Minami M. Central dot sign in entities other than Caroli disease. Radiat Med. 1997;15(6):381–4.
Bachler P, Baladron MJ, Menias C, Beddings I, Loch R, Zalaquett E, Vargas M, Connolly S, Bhalla S, Huete A. Multimodality imaging of liver infections: differential diagnosis and potential pitfalls. Radiographics. 2016;36(4):1001–23. https://doi.org/10.1148/rg.2016150196.
Brancatelli G, Federle MP, Vilgrain V, Vullierme MP, Marin D, Lagalla R. Fibropolycystic liver disease: CT and MR imaging findings. Radiographics. 2005;25(3):659–70. https://doi.org/10.1148/rg.253045114.
Caroli J, Couinaud R. Une affection nouvelle, des voies biliares. La dilatation kystique unilobulaire des canaux hepatiques. In: Seminar Hopitales Paris; 1958. p. 496–502.
Chan JH, Tsui EY, Luk SH, Fung AS, Yuen MK, Szeto ML, Cheung YK, Wong KP. Diffusion-weighted MR imaging of the liver: distinguishing hepatic abscess from cystic or necrotic tumor. Abdom Imaging. 2001;26(2):161–5. https://doi.org/10.1007/s002610000122.
Choi BI, Yeon KM, Kim SH, Han MC. Caroli disease: central dot sign in CT. Radiology. 1990;174(1):161–3. https://doi.org/10.1148/radiology.174.1.2294544.
Desmet VJ. Ludwig symposium on biliary disorders--Part I. Pathogenesis of ductal plate abnormalities. Mayo Clin Proc. 1998;73(1):80–9. https://doi.org/10.4065/73.1.80.
Gebel M. Ultrasound in gastroenterology and hepatology. Berlin: Blackwell Science; 2000.
Griffin N, Charles-Edwards G, Grant LA. Magnetic resonance cholangiopancreatography: the ABC of MRCP. Insights Imag. 2012;3(1):11–21. https://doi.org/10.1007/s13244-011-0129-9.
Hintze RE, Adler A, Veltzke W, Abou-Rebyeh H, Hammerstingl R, Vogl T, Felix R. Clinical significance of magnetic resonance cholangiopancreatography (MRCP) compared to endoscopic retrograde cholangiopancreatography (ERCP). Endoscopy. 1997;29(3):182–7. https://doi.org/10.1055/s-2007-1004160.
Joshi G, Crawford KA, Hanna TN, Herr KD, Dahiya N, Menias CO. US of right upper quadrant pain in the emergency department: diagnosing beyond gallbladder and biliary disease. Radiographics. 2018;38(3):766–93. https://doi.org/10.1148/rg.2018170149.
Khalefa AA, Alrasheed M, Saeedan MB. Central dot sign. Abdom Radiol (NY). 2016;41(11):2289–90. https://doi.org/10.1007/s00261-016-0836-2.
Krige J, Beckingham I. ABC of diseases of liver, pancreas, and biliary system: liver abscesses and hydatid disease. BMJ. 2001;322(7285):537.
Lall NU, Hogan MJ. Caroli disease and the central dot sign. Pediatr Radiol. 2009;39(7):754. https://doi.org/10.1007/s00247-009-1147-3.
Levy AD, Rohrmann CA Jr, Murakata LA, Lonergan GJ. Caroli’s disease: radiologic spectrum with pathologic correlation. AJR Am J Roentgenol. 2002;179(4):1053–7. https://doi.org/10.2214/ajr.179.4.1791053.
Leyendecker JR, Brown JJ, Merkle EM. Practical guide to abdominal and pelvic MRI. Philadelphia, PA: Wolters Kluwer Health; 2014.
Maccioni F, Martinelli M, Al Ansari N, Kagarmanova A, De Marco V, Zippi M, Marini M. Magnetic resonance cholangiography: past, present and future: a review. Eur Rev Med Pharmacol Sci. 2010;14(8):721–5.
Martin RF. Biliary cysts: a review and simplified classification scheme. Surg Clin North Am. 2014;94(2):219–32. https://doi.org/10.1016/j.suc.2014.01.011.
Mathieu D, Vasile N, Fagniez PL, Segui S, Grably D, Larde D. Dynamic CT features of hepatic abscesses. Radiology. 1985;154(3):749–52. https://doi.org/10.1148/radiology.154.3.3969480.
Mendez RJ, Schiebler ML, Outwater EK, Kressel HY. Hepatic abscesses: MR imaging findings. Radiology. 1994;190(2):431–6. https://doi.org/10.1148/radiology.190.2.8284394.
Park HJ, Kim SH, Jang KM, Lee SJ, Park MJ, Choi D. Differentiating hepatic abscess from malignant mimickers: value of diffusion-weighted imaging with an emphasis on the periphery of the lesion. J Magn Reson Imaging. 2013;38(6):1333–41.
Riordan R, Khonsari M, Jeffries J, Maskell G, Cook P. Pineapple juice as a negative oral contrast agent in magnetic resonance cholangiopancreatography: a preliminary evaluation. Br J Radiol. 2004;77(924):991–9.
Skucas J. Advanced imaging of the abdomen. London: Springer; 2017.
Taylor AC, Little AF, Hennessy OF, Banting SW, Smith PJ, Desmond PV. Prospective assessment of magnetic resonance cholangiopancreatography for noninvasive imaging of the biliary tree. Gastrointest Endosc. 2002;55(1):17–22. https://doi.org/10.1067/mge.2002.120324.
Visser BC, Suh I, Way LW, Kang SM. Congenital choledochal cysts in adults. Arch Surg. 2004;139(8):855–60. https://doi.org/10.1001/archsurg.139.8.855; discussion 860–2.
Yonem O, Bayraktar Y. Clinical characteristics of Caroli’s disease. World J Gastroenterol. 2007;13(13):1930.
Yu J, Turner MA, Fulcher AS, Halvorsen RA. Congenital anomalies and normal variants of the pancreaticobiliary tract and the pancreas in adults: part 2, Pancreatic duct and pancreas. AJR Am J Roentgenol. 2006;187(6):1544–53. https://doi.org/10.2214/AJR.05.0774.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2021 Springer Nature Switzerland AG
About this chapter
Cite this chapter
Motta, R., Lupi, A., Pirazzini, A., Giraudo, C., Marchesi, P. (2021). Caroli’s Disease. In: Floreani, A. (eds) Diseases of the Liver and Biliary Tree. Springer, Cham. https://doi.org/10.1007/978-3-030-65908-0_5
Download citation
DOI: https://doi.org/10.1007/978-3-030-65908-0_5
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-030-65907-3
Online ISBN: 978-3-030-65908-0
eBook Packages: MedicineMedicine (R0)