Case Report

Caroli Syndrome in a Child: A Case Report


Introduction: Caroli syndrome is a congenital disorder characterized by multiple segmental or saccular dilatations of the intrahepatic bile ducts associated with congenital hepatic fibrosis.
Case Presentation: A 3-year-old boy with abdominal distention was referred to gastroentrology ward of Amiralmomenin hospital (Semnan, Iran) in summer 2018. In his abdominal sonography, multiple cysts were detected in the liver with hepatomegaly, and the portal vein pressure was 10 mm. Also, in liver biopsy, dilated portal bile ducts (trichrome stain) with inspissated bile and congenital hepatic fibrosis were reported. He was discharged after conservative therapy and followed up.
Conclusion: Definitive treatment, i.e surgery, should be offered to prevent future complications.

[1] Kadakia N, Lobrito SJ, Ovchinsky N, Remot HE, Yamashiro DJ, Emond JC, et al. A challenging case of hepatoblastoma concomitant with autosomal recessive polycystc kidney disease and caroli syndrome-review of the literature. Fronters in Pediatrics 2017; 5:114. [DOI:10.3389/fped.2017.00114] [PMID] [PMCID]
[2] Yang XY, Zhu LP, Liu XQ, Zhang CY, Yao Y, Wu Y. [Genetc diagnosis of Caroli syndrome with autosomal recessive polycystc kidney disease: A case report and literature review (Chinese)]. Beijing da xue xue bao Yi xue ban=Journal of Peking University (Health Sciences). 2018; 50(2):335-9. [PMID]
[3] Lendoire J, Barros Scheloto P, Alvarez Rodríguez J, Duek F, Quarin C, Garay V, et al. Bile duct cyst type V (Caroli’s disease): Surgical strategy and results. HPB Journal. 2007; 9(4):281-4. [DOI:10.1080/13651820701329258] [PMID] [PMCID]
[4] Fahrner R, Dennler SGC, Dondorf F, Ardelt M, Rauchfuss F, Setmacher U. Liver resecton and transplantaton in Caroli disease and syndrome. Journal of Visceral Surgery. 2018; 156(2):91-5. [DOI:10.1016/j.jviscsurg.2018.06.001] [PMID]
[5] Perricone G, Vanzulli A. Educaton and imaging. Hepatology: “Central dot sign” of Caroli syndrome. 2015; 30(2):234. [DOI:10.1111/ jgh.12828] [PMID]
[6] Kim JT, Hur YJ, Park JM, Kim MJ, Park YN, Lee JS. Caroli’s syndrome with autosomal recessive polycystc kidney disease in a two month old infant. Yonsei Medical Journal. 2006; 47(1):131-4. [DOI:10.3349/ ymj.2006.47.1.131] [PMID] [PMCID]
[7] Zidan A, Bauschke A, Scheuerlein H, Setmacher U, Rauchfuss F. Re-resecton of remnant Caroli syndrome six years afer the frst resecton (case report). Internatonal Journal of Surgery Open. 2016; 3:8-10. [DOI:10.1016/j.ijso.2016.04.004]
[8] Gu DH, Park MS, Jung CH, Yoo YJ, Cho JY, Lee YH, et al. Caroli’s disease misdiagnosed as intraductal papillary neoplasm of the bile duct. Clinical and Molecular Hepatology. 2015; 21(2):175-9. [DOI:10.3350/cmh.2015.21.2.175] [PMID] [PMCID]
[9] Ananthakrishnan AN, Saeian K. Caroli’s disease: Identfcaton and treatment strategy. Current Gastroenterology Reports. 2007; 9(2):151-5. [DOI:10.1007/s11894-007-0010-7] [PMID]
[10] Srivastava G, Raju U, Sherwani P, Kumar M, Gupta D. Caroli’s disease: An unusually early presentaton. Internatonal Journal of Medical Paediatrics and Oncology. 2016; 2:173-6.
IssueVol 4 No 3 (2019): Summer QRcode
SectionCase Report(s)
Caroli syndrome; Child; Case report

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How to Cite
Tajik P, Goudarzian AH. Caroli Syndrome in a Child: A Case Report. CRCP. 4(3):89-93.