Skip to main content

Advertisement

SpringerLink
Log in

Surgical Management of Caroli’s Disease: Single Center Experience and Review of the Literature

  • Original Article
  • Published:
Journal of Gastrointestinal Surgery Aims and scope

Abstract

Background

Caroli’s disease is a rare congenital condition characterized by non-obstructive dilatation of intrahepatic ducts. In Caroli’s syndrome, there is additionally an associated congenital hepatic fibrosis.

Methods

With institutional review board approval, we identified all patients with Caroli’s disease and syndrome.

Results

Nine patients were identified, seven males and two females, with a median age of 40 years. Final pathological diagnoses included Caroli’s disease (n = 6) and Caroli’s syndrome (n = 3). Patients presented with deranged liver function, cholangitis, cholangiocarcinoma, abdominal pain, cirrhosis, or were diagnosed incidentally. Four patients underwent resection and two underwent liver transplantation. Of the resection group, two patients subsequently underwent transplantation for recurrent cholangitis due to anastomotic stricture in one patient and for end-stage liver disease in the other. All patients with Caroli’s syndrome underwent liver transplantation. Three patients died during follow-up at 26.2, 7.8, and 3 months post-diagnosis with recurrence of cholangiocarcinoma, liver failure, and metastatic cholangiocarcinoma, respectively. Six patients are alive with a median follow-up of 60 months since presentation (range = 10–134 months).

Conclusions

Caroli’s disease and syndrome have a varied presentation. Most individuals with Caroli’s disease may be adequately treated by resection, but transplantation is required for Caroli’s syndrome patients due to the associated hepatic fibrosis.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5

Similar content being viewed by others

References

  1. Caroli J, Soupault R, Kossakowski J, Plocker L, Paradowska. Congenital polycystic dilation of the intrahepatic bile ducts: attempt at classification. Sem Hop 1958;34:488–95.

    CAS  PubMed  Google Scholar 

  2. Yonem O, Bayraktar Y. Clinical characteristics of Caroli’s disease. World J Gastroenterol 2007;13:1934–7.

    Article  PubMed Central  PubMed  Google Scholar 

  3. Tahvanainen E, Tahvanainen P, Kaariainen H, Höckerstedt K. Polycystic liver and kidney diseases. Ann Med 2005;37:546–55.

    Article  CAS  PubMed  Google Scholar 

  4. Kerkar N, Norton K, Suchy FJ. The hepatic fibrocystic diseases. Clin Liver Dis 2006;10:55–71, v-vi.

    Article  PubMed  Google Scholar 

  5. Vasilescu C, Andrei SL, Andronesi DT, Zamfir R, Popescu I. Boli hepatice rare. In: Chirurgia Ficatului, vol. 1, Bucuresti: Ed Univ Carol Davila 2004, pp 526–30.

  6. Dayton MT, Longmire WP Jr, Tompkins RK. Caroli’s Disease: a premalignant condition? Am J Surg 1983;145:41–8.

    Article  CAS  PubMed  Google Scholar 

  7. Guy F, Cognet F, Dranssart M, Cercueil JP, Conciatori L, Krausé D. Caroli’s disease: magnetic resonance imaging features. Eur Radiol 2002;12:2730–6.

    PubMed  Google Scholar 

  8. Dukova B, Ilievski B, Duganovska S, Chadikovski V, Kostovski A. Liver pathology findings in infant with Caroli’s syndrome. International Journal of Hepatobiliary and Pancreatic Diseases 2012;2:4–8.

    Article  Google Scholar 

  9. Mabrut JY, Partensky C, Jaeck D, Oussoultzoglou E, Baulieux J, Boillot O, Lerut J, de Ville de Goyet J, Hubert C, Otte JB, Audet M, Ducerf C, Gigot JF. Congenital intrahepatic bile duct dilatation is a potentially curable disease: Long-term results of a multi-institutional study. Ann Surg 2007;246:236–45.

    Article  PubMed Central  PubMed  Google Scholar 

  10. Vachell HR, Stevens WM. Case of intrahepatic calculi. Br Med J 1906;1:434–6.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  11. Todani T, Watanabe Y, Narusue M, Tabuchi K, Okajima K. Congenital bile duct cyst: classification, operative procedures, and review of 37 cases including cancer arising from choledochal cyst. Am J Surg 1977;134:263–9.

    Article  CAS  PubMed  Google Scholar 

  12. Bayraktar Y, Balkanci F, Kayhan B, Uzunalimoglu B, Ozenc A, Ozdemir A, Dündar S, Arslan S, Sivri B, Telatar H. Congenital hepatic fibrosis associated with cavernous transformation of the portal vein. Hepatogastroenterology 1997;44:1588–94.

    CAS  PubMed  Google Scholar 

  13. Abdullah AM, Nazer H, Atiyeh M, Ali MA. Congenital hepatic fibrosis in Saudi Arabia. J Trop Pediatr 1991;37:240–3.

    Article  CAS  PubMed  Google Scholar 

  14. Dahlstrom JE, Cookman J, Jain S. Joubert syndrome: an affected female with bilateral colobomata. Pathology 2000;32:283–5.

    CAS  PubMed  Google Scholar 

  15. Desmet VJ. Ludwig symposium on biliary disorders-Part I. Pathogenesis of ductal plate abnormalities. Mayo Clin Proc1998;73:80–9.

  16. Ward CJ, Hogan MC, Rossetti S, Walker D, Sneddon T, Wang X, Kubly V, Cunningham JM, Bacallao R, Ishibashi M, Milliner DS, Torres VE, Harris PC. The gene mutated in autosomal recessive polycystic kidney disease encodes a large, receptor-like protein. Nat Genet 2002;30:259–69.

    Article  PubMed  Google Scholar 

  17. Guay-Woodford LM, Desmond RA. Autosomal recessive polycystic kidney disease: The clinical experience in North America. Pediatrics 2003;111:1072–80.

    Article  PubMed  Google Scholar 

  18. Gunay-Aygun M, Avner ED, Bacallao RL, Choyke PL, Flynn JT, Germino GG, Guay-Woodford L, Harris P, Heller T, Ingelfinger J, Kaskel F, Kleta R, LaRusso NF, Mohan P, Pazour GJ, Shneider BL, Torres VE, Wilson P, Zak C, Zhou J, Gahl WA. Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis: Summary statement of a first National Institutes of Health/Office of Rare Diseases conference. J Pediatr 2006;149:159–64.

    Article  PubMed Central  PubMed  Google Scholar 

  19. Nagorney DM. Biliary and liver cysts. In: Blumgart LH, Fong Y, eds. Surgery of the liver and biliary tract. Vol. 2. 3rd ed. London: WB Saunders, 2000:1245–58.

    Google Scholar 

  20. Gupta AK, Gupta A, Bhardwaj VK, Chansoria M. Caroli’s disease. Indian J Pediatr 2006;73:233–5.

    Article  PubMed  Google Scholar 

  21. Zhang DY, Ji ZF, Shen XZ, Liu HY, Pan BJ, Dong L. Caroli’s disease: a report of 14 patients and review of the literature. J Dig Dis 2012;13:491–5.

    Article  PubMed  Google Scholar 

  22. Levy AD, Rohrmann CA Jr, Murakata LA, Lonergan GJ. Caroli’s disease: radiologic spectrum with pathologic correlation. AJR Am J Roentgenol 2002;179:1053–7.

    Article  PubMed  Google Scholar 

  23. Bockhorn M, Malagó M, Lang H, Nadalin S, Paul A, Saner F, Frilling A, Broelsch CE. The role of surgery in Caroli’s disease. J Am Coll Surg 2006;202:928–32.

    Article  PubMed  Google Scholar 

  24. Iancu C, Bodea R, Muresan T, Iancu D, Boruah P, Al Hajjar N. Diffuse form of Caroli’s disease: therapeutical approach in a female patient with recurrent cholangitis. J Gastrointestin Liver Dis 2010;19:457–60.

    PubMed  Google Scholar 

  25. Kassahun WT, Kahn T, Wittekind C, Mössner J, Caca K, Hauss J, Lamesch P. Caroli’s disease: liver resection and liver transplantation. Experience in 33 patients. Surgery 2005;138: 888–98.

    Article  PubMed  Google Scholar 

  26. Guntz P, Coppo B, Lorimier G, Cronier P, Guntz M. Single-lobe Caroli’s disease. Anatomoclinical aspects. Diagnostic and therapeutic procedure. Apropos of 3 personal cases and 101 cases in the literature [in French]. J Chir 1991;128:167–81.

    CAS  Google Scholar 

  27. Akhan O, Karaosmanoglu AD, Ergen B. Imaging findings in congenital hepatic fibrosis. Eur J Radiol 2007;61:18–24.

    Article  PubMed  Google Scholar 

  28. Wu KL, Changchien CS, Kuo CM, Chuah SK, Chiu YC, Kuo CH. Caroli’s disease - a report of two siblings. Eur J Gastroenterol Hepatol 2002;14:1397–9.

  29. Barros JL, Polo JR, Sanabia J, Garcia-Sabrido JL, Gomez-Lorenzo FJ. Congenital cystic dilatation of the intrahepatic bile ducts (Caroli’s disease): report of a case and review of the literature. Surgery 1979;85:589–92.

    CAS  PubMed  Google Scholar 

  30. Asselah T, Ernst O, Sergent G, L’herminé C, Paris JC. Caroli’s disease: a magnetic resonance cholangiopancreatography diagnosis. Am J Gastroenterol 1998;93:109–10.

    Article  CAS  PubMed  Google Scholar 

  31. Gunay-Aygun M. Liver and Kidney Disease in Ciliopathies. Am J Med Genet C Semin Med Genet 2009;151C:296–306.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  32. Espinoza R, San Martín S, Court F, Vera E, Ferreira R, Croxatto H. Hepatic resection in localized Caroli disease [in Spanish]. Rev Med Chil 2003;131:183–9.

    PubMed  Google Scholar 

  33. Lipsett PA, Pitt HA. Surgical treatment of choledochal cysts. J Hepatobiliary Pancreat Surg 2003;10:352–9.

    Article  PubMed  Google Scholar 

  34. Ammori BJ, Jenkins BL, Lim PC, Prasad KR, Pollard SG, Lodge JP. Surgical strategy for cystic disease of the liver in a western hepatobiliary center. World J Surg 2002;26:462–9.

    Article  PubMed  Google Scholar 

  35. Millwala F, Segev DL, Thuluvath PJ. Caroli’s disease and outcomes after liver transplantation. Liver Transpl 2008;14:11–7.

    Article  PubMed  Google Scholar 

  36. Habib S, Shakil O, Couto OF, Demetris AJ, Fung JJ, Marcos A, Chopra K. Caroli’s disease and orthotopic liver transplantation. Liver Transpl 2006; 12: 416–21.

    Article  PubMed  Google Scholar 

  37. Harring TR, Nguyen NT, Liu H, Goss JA, O’Mahony CA. Caroli disease patients have excellent survival after liver transplant. J Surg Res 2012;177:365–72.

    Article  PubMed  Google Scholar 

  38. De Kerckhove L, De Meyer M, Verbaandert C, Mourad M, Sokal E, Goffette P, Geubel A, Karam V, Adam R, Lerut J. The place of liver transplantation in Caroli’s disease and syndrome. Transpl Int 2006;19:381–8.

    Article  PubMed  Google Scholar 

  39. Takatsuki M, Uemoto S, Inomata Y, Egawa H, Kiuchi T, Hayashi M, Ueda M, Tanaka K. Living-donor liver transplantation for Caroli’s disease with intrahepatic adenocarcinoma. J Hepatobiliary Pancreatic Surg 2001;8:284–6.

    Article  CAS  Google Scholar 

  40. Iwatsuki S, Todo S, Marsh JW, Madariaga JR, Lee RG, Dvorchik I, Fung JJ, Starzl TE. Treatment of hilar cholangiocarcinoma (Klatskin tumors) with hepatic resection or transplantation. J Am Coll Surg 1998;187:358–64.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  41. Shimoda M, Farmer DG, Colquhoun SD, Rosove M, Ghobrial RM, Yersiz H, Chen P, Busuttil RW. Liver transplantation for cholangiocellular carcinoma: analysis of a single-center experience and review of the literature. Liver Transpl 2001;7:1023–33.

    Article  CAS  PubMed  Google Scholar 

  42. Darwish Murad S, Kim WR, Harnois DM, Douglas DD, Burton J, Kulik LM, Botha JF, Mezrich JD, Chapman WC, Schwartz JJ, Hong JC, Emond JC, Jeon H, Rosen CB, Gores GJ, Heimbach JK. Efficacy of neoadjuvant chemoradiation, followed by liver transplantation, for perihilar cholangiocarcinoma at 12 US centers. Gastroenterology 2012;143:88–98.

    Article  PubMed  Google Scholar 

  43. Nagasue N. Successful treatment of Caroli’s disease by hepatic resection. Report of six patients. Ann Surg 1984;200:718–23.

    Article  CAS  PubMed  Google Scholar 

  44. Mercadier M, Chigot JP, Clot JP, Langlois P, Lansiaux P. Caroli’s disease. World J Surg. 1984;8:22–9.

    Article  CAS  PubMed  Google Scholar 

  45. Dağli U, Atalay F, Saşmaz N, Bostanoğlu S, Temuçin G, Sahin B. Caroli’s disease: 1977–1995 experiences. Eur J Gastroenterol Hepatol 1998;10:109–12.

    Article  PubMed  Google Scholar 

  46. Gillet M, Favre S, Fontolliet C, Halkic N, Mantion G, Heyd B. Maladie de Caroli monolobaire. A propos de 12 cases [in French]. Chirurgie 1999;124:13–8.

    Article  CAS  PubMed  Google Scholar 

  47. Shi LB, Peng SY, Meng XK, Peng CH, Liu YB, Chen XP, Ji ZL, Yang DT, Chen HR. Diagnosis and treatment of congenital choledochal cyst: 20 years’ experience in China. World J Gastroenterol 2001;7:732–4.

    CAS  PubMed  Google Scholar 

  48. Pimentel FM. Enfermedad de Caroli [in Spanish]. Rev Chilena de Cirugia 2004;56:426–33.

    Google Scholar 

  49. Ulrich F, Pratschke J, Pascher A, Neumann UP, Lopez-Hänninen E, Jonas S, Neuhaus P. Long-term outcome of liver resection and transplantation for Caroli disease and syndrome. Ann Surg 2008;247:357–64.

    Article  PubMed  Google Scholar 

  50. Lendoire JC, Raffin G, Grondona J, Bracco R, Russi R, Ardiles V, Gondolesi G, Defelitto J, de Santibañes E, Imventarza O. Caroli’s disease: report of surgical options and long-term outcome of patients treated in Argentina. Multicenter study. J Gastrointest Surg 2011;15:1814–9.

    Article  PubMed  Google Scholar 

  51. Zahmatkeshan M, Bahador A, Geramizade B, Emadmarvasti V, Malekhosseini SA. Liver transplantation for Caroli disease. Int J Organ Transplant Med 2012;3:189–91.

    PubMed Central  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of HPB Surgery, Digestive Disease Institute, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH, 44195, USA

    Maitham A. Moslim, David Vogt & Gareth Morris-Stiff

  2. Recanati Miller Transplant Institute, Mount Sinai Hospital, 1425 Madison Avenue, New York, NY, 10029, USA

    Ganesh Gunasekaran

  3. Department of Anatomic Pathology, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH, 44195, USA

    Michael Cruise

Authors
  1. Maitham A. Moslim
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Ganesh Gunasekaran
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. David Vogt
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Michael Cruise
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Gareth Morris-Stiff
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Maitham A. Moslim.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Moslim, M.A., Gunasekaran, G., Vogt, D. et al. Surgical Management of Caroli’s Disease: Single Center Experience and Review of the Literature. J Gastrointest Surg 19, 2019–2027 (2015). https://doi.org/10.1007/s11605-015-2918-9

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11605-015-2918-9

Keywords

Search

Navigation