REFERENCES:
1. Ros E, Navarro S, Bru C, Gilabert R, Bianchi L, Bruguera M. Ursodeoxycholic acid treatment of primary hepatolithiasis in Caroli’s syndrome. The Lancet. 1993;343(8868):404-6. [PubMed|DOI|Full Text]
2. Caroli J, Soupault R, Kossakowski J, Plocker L, Paradowska. Congenital polycystic dilation of the intrahepatic bile ducts; attempt at classification. Sem Hop. 1958 Feb 18;34(8/2):488-95/SP. [PubMed]
3. Arnon R, Rosenberg HK, Suchy FJ. Caroli Disease, Caroli Syndrome, and Congenital Hepatic Fibrosis. Fibrocystic Diseases of the Liver. 2010. p. 331–58. [DOI]
4. Ozlem Yonem YB. Clinical characteristics of Caroli’s syndrome. World J Gastroenterol. 2007 Apr 7;13(13):1934. [PubMed| DOI|Full Text]
5. Caroli J. Diseases of the Intrahepatic Biliary Tree. Clin Gastroenterol. 1973 Jan;2(1):147-61. [PubMed]
6. Kerkar N, Norton K, Suchy FJ. The hepatic fibrocystic diseases. Clin Liver Dis. 2006 Feb;10(1):55–71. [PubMed|DOI]
7. Levy AD, Rohrmann CA Jr, Murakata LA, Lonergan GJ. Caroli’s disease: radiologic spectrum with pathologic correlation. AJR Am J Roentgenol. 2002 Oct;179(4):1053–7. [PubMed|DOI]
8. Kim JT, Hur YJ, Park JM, Kim MJ, Park YN, Lee JS. Caroli’s syndrome with autosomal recessive polycystic kidney disease in a two month old infant. Yonsei Med J. 2006 Feb 28;47(1):131–4. [PubMed|DOI|Full Text]
9. Abdalla EK, Forsmark CE, Lauwers GY, Nicolas Vauthey J. Monolobar Caroli’s Disease and Cholangiocarcinoma. HPB Surgery. 1999;11(4):271–7. [PubMed| DOI|Full Text]
10. Nicolau C, Torra R, Badenas C, Pérez L, Oliver JA, Darnell A, et al. Sonographic pattern of recessive polycystic kidney disease in young adults. Differences from the dominant form. Nephrol Dial Transplant. 2000 Sep;15(9):1373–8. [PubMed| DOI]
11. Arnold HL, Harrison SA. New advances in evaluation and management of patients with polycystic liver disease. Am J Gastroenterol. 2005 Nov;100(11):2569–82. [PubMed|DOI]
12. O’Brien K, Font-Montgomery E, Lukose L, Bryant J, Piwnica-Worms K, Edwards H, et al. Congenital hepatic fibrosis and portal hypertension in autosomal dominant polycystic kidney disease. J Pediatr Gastroenterol Nutr. 2012 Jan;54(1):83–9. [PubMed|DOI]
13. Wambura C, Sharma M, Surani S. Revisiting Caroli Syndrome in a Tanzanian Patient. Cureus. 2020 Jan 15;12(1):e6661. [PubMed| DOI|Full Text]
14. Wang Z-X, Li Y-G, Wang R-L, Li Y-W, Li Z-Y, Wang L-F, et al. Clinical classification of Caroli’s disease: an analysis of 30 patients. HPB (Oxford). 2015;17(3):278-83. [PubMed| DOI|Full Text]
15. Zhong Y, Peng S, Yang Y. Hepatobiliary and Pancreatic: Caroli syndrome: Egg-like sign on CT. J Gastroenterol Hepatol. 2020 Feb;35(2):180. [PubMed| DOI]
16. De Kerckhove L, De Meyer M, Verbaandert C, Mourad M, Sokal E, Goffette P, et al. The place of liver transplantation in Caroli’s disease and syndrome. Transpl Int. 2006 May;19(5):381–8. [PubMed|DOI|Full Text]
17. Ananthakrishnan AN, Saeian K. Caroli’s disease: Identification and treatment strategy. Current Gastroenterology Reports. 2007;9(3):151–5. [PubMed|DOI]
18. Tzoufi M, Rogalidou M, Drimtzia E, Sionti I, Nakou I, Argyropoulou M, et al. Caroli’s disease: Description of a case with a benign clinical course. Annals of Gastroenterology: 2011;24(2):129-33. [PubMed|Full Text]
19. Wu K-L, Changchien C-S, Kuo C-M, Chuah S-K, Chiu Y-C, Kuo C-H. Caroli’s disease - a report of two siblings. Eur J Gastroenterol Hepatol. 2002 Dec;14(12):1397–9. [PubMed|DOI]
20. Perricone G, Vanzulli A. Hepatology: “central dot sign” of Caroli syndrome. J Gastroenterol Hepatol. 2015 Feb;30(2):234. [DOI |Full Text]
21. Guy F, Cognet F, Dranssart M, Cercueil J-P, Conciatori L, Krausé D. Caroli’s disease: magnetic resonance imaging features. Eur Radiol. 2002 Nov;12(11):2730–6. [PubMed|DOI]
22. Sinakos E, Papalavrentios L, Chourmouzi D, Dimopoulou D, Drevelegas A, Akriviadis E. The clinical presentation of Von Meyenburg complexes. Hippokratia. 2011 Apr;15(2):170–3. [PubMed|Full Text]
23. Patil S, Das HS, Desai N, Manjunath SM, Thakur BS, Sawant P. Caroli’s syndrome–a rare cause of portal hypertension. J Assoc Physicians India. 2004 Mar;52:261. [PubMed]
24. Jang MH, Lee YJ, Kim H. Intrahepatic cholangiocarcinoma arising in Caroli’s disease. Clinical and Molecular Hepatology. 2014;20(4):402-5. [PubMed|DOI|Full Text]
25. AbouAlaiwi WA, Ratnam S, Booth RL, Shah JV, Nauli SM. Endothelial cells from humans and mice with polycystic kidney disease are characterized by polyploidy and chromosome segregation defects through survivin down-regulation. Hum Mol Genet. 2011 Jan 15;20(2):354–67. [PubMed| DOI|Full Text]
26. Kassahun WT, Kahn T, Wittekind C, Mössner J, Caca K, Hauss J, et al. Caroli’s disease: liver resection and liver transplantation. Experience in 33 patients. Surgery. 2005 Nov;138(5):888–98. [PubMed|DOI]
27. Abdullah AM, Nazer H, Atiyeh M, Ali MA. Congenital hepatic fibrosis in Saudi Arabia. J Trop Pediatr. 1991 Oct;37(5):240–3. [PubMed|DOI]
28. Lai Q, Lerut J. Proposal for an algorithm for liver transplantation in Caroli’s disease and syndrome: putting an uncommon effort into a common task. Clin Transplant. 2016 Jan;30(1):3–9. [PubMed| DOI]
FIGURES:Figure 1: Multiple cystically dilated intra hepatic biliary ducts in segments V, VI, VII and VIII of the liver. These ducts show no enhancement in post contrast images with central enhancing portal radicle giving central dot sign (arrow). However, common bile duct is normal. Figure 2: Ducts show no enhancement in post contrast images with central enhancing portal radicle giving central dot sign (arrow). Figure 3: Multiple cystically dilated intra hepatic biliary ducts (arrow).
Figure 4: CT Scan (Coronal view) Multiple variable sized cystic lesions are seen (arrows) involving all poles of both kidneys. No calcification, septation or enhancing solid components seen within the cyst.
Consent: Written informed consent was taken from the patient’s parents before writing the manuscript.