Caroli's disease: Systematic review
DOI:
https://doi.org/10.22516/25007440.612Keywords:
Caroli’s disease, Cystic liver disease, Intrahepatic dilatation, Portal hypertension, Hepatomegaly, Cystic dilation, Newborn diseasesAbstract
Introduction: Caroli's disease (CD) is a rare congenital disease, which presents with multifocal segmental intrahepatic bile duct dilatation. It was first described by Jacques Caroli in 1958 as a saccular or fusiform dilatation of the intrahepatic bile ducts.
Objective: To characterize the population that has been diagnosed with Caroli’s disease.
Materials and methods: Systematic review.
Results: 66 articles were analyzed. The age group most affected was children under 10 years old, where a higher prevalence was evidenced in males. This condition was associated with other comorbidities such as polycystic kidney in 20%. The most frequent manifestation was hepatomegaly (44.7%), followed by fever (42.4%), and abdominal pain in the right upper quadrant (41.2%). The most used diagnostic method was magnetic resonance imaging in 73.8% of the sample. The findings showed predominance of intra-hepatic dilatation in 76.5%. The most widely used treatment was antibiotic therapy to treat recurrences due to cholangitis.
Conclusion: Caroli's disease has an extremely low incidence and occurs more frequently in the American continent, affecting mainly patients in the first decade of life, with a predilection for the male sex. It is characterized by a dilatation of the intrahepatic ducts that can affect other organs such as the kidneys, causing renal cysts.
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Cabral Correia P, Morgado B. Caroli's Disease as a Cause of Chronic Epigastric Abdominal Pain: Two Case Reports and a Brief Review of the Literature. Cureus. 2017;9(9):e1701. https://doi.org/10.7759/cureus.1701
Fahrner R, Dennler SGC, Dondorf F, Ardelt M, Rauchfuss F, Settmacher U. Liver resection and transplantation in Caroli disease and syndrome. J Visc Surg. 2019;156(2):91-95. https://doi.org/10.1016/j.jviscsurg.2018.06.001
Uribarrena Amezaga R, Raventós N, Fuentes J, Elías J, Tejedo V, Uribarrena Echebarría R. Diagnóstico y tratamiento de los quistes de colédoco. Presentación de 10 nuevos casos. Rev Esp Enferm Dig. 2008;100(2):71-5. https://doi.org/10.4321/s1130-01082008000200002
Tello de Meneses Salazar A, Riggen Martínez L, Orozco Chávez EG. Quiste de colédoco tipo I: Diagnóstico por imagen y anastomosis hepático-duodenal como medida terapéutica. Bol. Med. Hosp. Infant. Mex. 2013;70(6):482-7.
Moslim MA, Gunasekaran G, Vogt D, Cruise M, Morris-Stiff G. Surgical Management of Caroli's Disease: Single Center Experience and Review of the Literature. J Gastrointest Surg. 2015;19(11):2019-27. https://doi.org/10.1007/s11605-015-2918-9
Burt MJ, Chambers ST, Chapman BA, Strack MF, Troughton WD. Two cases of Caroli's disease: diagnosis and management. J Gastroenterol Hepatol. 1994;9(2):194-7. https://doi.org/10.1111/j.1440-1746.1994.tb01242.x
Ciambotti GF, Ravi J, Abrol RP, Arya V. Right-sided monolobar Caroli's disease with intrahepatic stones: nonsurgical management with ERCP. Gastrointest Endosc. 1994;40(6):761-4. https://doi.org/10.1016/S0016-5107(94)70125-3
Bakhotmah MA. Successful control of external biliary fistula by using SMS 201-995 in a child. HPB Surg. 1996;9(3):183-4. https://doi.org/10.1155/1996/80945
Yuksel A, Has R, Isikoglu M, Suoglu O. Prenatal diagnosis of Caroli's disease. Ultrasound Obstet Gynecol. 2002;19(5):525-6. https://doi.org/10.1046/j.1469-0705.2002.00692.x
Vlachogiannakos J, Potamianos S, Triantos C, Makri I, Imvrios G, Patsiaoura K, et al. Monolobar Caroli's disease complicated by cholangiocarcinoma in a 70-year-old man, previously asymptomatic. Gastrointest Endosc. 2004;60(2):297-300. https://doi.org/10.1016/s0016-5107(04)01572-x
Gold DM, Stark B, Pettei MJ, Levine JJ. Successful use of an internal biliary stent in Caroli's disease. Gastrointest Endosc. 1995;42(6):589-92. https://doi.org/10.1016/s0016-5107(95)70018-8
Marchal GJ, Desmet VJ, Proesmans WC, Moerman PL, Van Roost WW, Van Holsbeeck MT, et al. Caroli disease: high-frequency US and pathologic findings. Radiology. 1986;158(2):507-11. https://doi.org/10.1148/radiology.158.2.3510448
Sood GK, Mahapatra JR, Khurana A, Chaudhry V, Sarin SK, Broor SL. Caroli disease: computed tomographic diagnosis. Gastrointest Radiol. 1991;16(3):243-4. https://doi.org/10.1007/BF01887356
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
Hermansen MC, Starshak RJ, Werlin SL. Caroli disease: the diagnostic approach. J Pediatr. 1979;94(6):879-82. https://doi.org/10.1016/s0022-3476(79)80206-1
Pavone P, Laghi A, Catalano C, Materia A, Basso N, Passariello R. Caroli's disease: evaluation with MR cholangiopancreatography (MRCP). Abdom Imaging. 1996;21(2):117-9. https://doi.org/10.1007/s002619900026
Pinós T, Xiol X, Herranz R, Figueras C, Catalá I. Caroli's disease versus polycystic hepatic disease. Differential diagnosis with Tc-99m DISIDA scintigraphy. Clin Nucl Med. 1993;18(8):664-7. https://doi.org/10.1097/00003072-199308000-00008
Sharma R, Mondal A, Taneja V, Rawat HS. Radionuclide scintigraphy in Caroli's disease. Indian J Pediatr. 1997;64(1):105-7. https://doi.org/10.1007/BF02795788
Kaiser JA, Mall JC, Salmen BJ, Parker JJ. Diagnosis of Caroli disease by computed tomography: report of two cases. Radiology. 1979;132(3):661-4. https://doi.org/10.1148/132.3.661
Herman TE, Siegel MJ. Autosomal recessive polycystic disease with biliary dysgenesis. J Perinatol. 2001;21(5):339-40. https://doi.org/10.1038/sj.jp.7200224
Desroches J, Spahr L, Leduc F, Pomier-Layrargues G, Picard M, Picard D, Chartrand R, Morais J. Noninvasive diagnosis of Caroli syndrome associated with congenital hepatic fibrosis using hepatobiliary scintigraphy. Clin Nucl Med. 1995;20(6):512-4. https://doi.org/10.1097/00003072-199506000-00009
Won JH, Choi SY, Lee HK, Yi BH, Lee MH, Jung MJ. Accessory gallbladder in an intrahepatic location mimicking a cystic tumor of the liver: A case report. Medicine (Baltimore). 2016;95(44):e5293. https://doi.org/10.1097/MD.0000000000005293
Aguilera V, Rayón M, Pérez-Aguilar F, Berenguer J. Caroli's syndrome and imaging: report of a case. Rev Esp Enferm Dig. 2004;96(1):74-6. https://doi.org/10.4321/s1130-01082004000100009
Jarry J, Leblanc F, Saric J. Maladie de Caroli monolobaire. Presse Med. 2010;39(7-8):847-8. French. https://doi.org/10.1016/j.lpm.2009.10.018
Lyons TJ, Benbow EW, Taylor PM, McCloy RF. Inflammatory pseudotumour of the liver: antecedent causes and clinical experience. J Hepatol. 1993;19(2):273-8. https://doi.org/10.1016/s0168-8278(05)80582-3
Evans-Jones G, Cudmore R. Choledochal cyst and congenital hepatic fibrosis. J Pediatr Surg. 1990;25(12):1259-60. https://doi.org/10.1016/0022-3468(90)90524-d
Senyüz OF, Yesildag E, Kuruoglu S, Yeker Y, Emir H. Caroli's disease in children: is it commonly misdiagnosed? Acta Paediatr. 2005;94(1):117-20. https://doi.org/10.1111/j.1651-2227.2005.tb01798.x
Ninan VT, Nampoory MR, Johny KV, Gupta RK, Schmidt I, Nair PM, et al. Caroli's disease of the liver in a renal transplant recipient. Nephrol Dial Transplant. 2002;17(6):1113-5. https://doi.org/10.1093/ndt/17.6.1113
Fulcher AS, Turner MA, Sanyal AJ. Case 38: Caroli disease and renal tubular ectasia. Radiology. 2001;220(3):720-3. https://doi.org/10.1148/radiol.2203000825
Esmer C, Alvarez-Mendoza A, Lieberman E, Del Castillo V, Ridaura-Sanz C. Liver fibrocystic disease and polydactyly: proposal of a new syndrome. Am J Med Genet. 2001;101(1):12-6. https://doi.org/10.1002/ajmg.1314
Jenss H, Krause FJ. Caroli-Syndrom-Zufallsbefund bei der postoperativen T-Drain-Cholangiographie. Rofo. 1983;139(5):576-8. https://doi.org/10.1055/s-2008-1055953
Treacy PJ, Worthley CS, Harley HA. Computed tomography infusion choledochography in the management of Caroli's syndrome. Aust N Z J Surg. 1997;67(4):221-2. https://doi.org/10.1111/j.1445-2197.1997.tb01947.x
Zangger P, Grossholz M, Mentha G, Lemoine R, Graf JD, Terrier F. MRI findings in Caroli's disease and intrahepatic pigmented calculi. Abdom Imaging. 1995;20(4):361-4. https://doi.org/10.1007/BF00203372
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(12):1397-9. https://doi.org/10.1097/00042737-200212000-00019
Hernández-Ortiz J, Corona R, Méndez-Sánchez N. Caroli's disease and choledochal cyst. Ann Hepatol. 2002;1(4):196. https://doi.org/10.1016/S1665-2681(19)32166-0
Chiba T, Shinozaki M, Kato S, Goto N, Fujimoto H, Kondo F. Caroli's disease: central dot sign re-examined by CT arteriography and CT during arterial portography. Eur Radiol. 2002;12(3):701-2. https://doi.org/10.1007/s003300101048
Cheung VT, Joshi D, Amin Z, Webster GJ. Fever and right upper quadrant pain in a 24-year-old male. Caroli disease and splenomegaly suggesting portal hypertension. Gut. 2014;63(10):1626, 1625. https://doi.org/10.1136/gutjnl-2014-306987
Makino I, Tani T, Shimizu K, Takamura H, Fujimura T, Nishimura G, et al. [A case of monolobar Caroli's disease]. Nihon Shokakibyo Gakkai Zasshi. 2006;103(3):316-21. (Nota: confirmar si esta fue la referencia que usó el autor)
Badura J, Król R, Kurek A, Hartleb M, Cierpka L. Left-hemihepatectomy as a method of treatment of locally limited Caroli disease. Pol Przegl Chir. 2013;85(11):663-5. https://doi.org/10.2478/pjs-2013-0100
Cha BH, Lee SH, Hwang JH, Kim SY, Kim HY. [A case of caroli disease with biliary stones]. Korean J Gastroenterol. 2009;54(4):201-4. https://doi.org/10.4166/kjg.2009.54.4.201
Deimel A, Sturm J, Vielfort T, Zöpf T. Oberbauchschmerzen und Fieberschübe bei einer 44‑jährigen Philippinerin. Internist (Berl). 2018;59(3):276-281. https://doi.org/10.1007/s00108-017-0320-2
Bodas A, Maluenda C, Rivilla F, Ortega L, Pérez O. Caroli's syndrome and horseshoe kidney: a case report. Acta Paediatr. 2001;90(4):469-71. (Nota: confirmar si esta fue la referencia que usó el autor)
Dhanjal NS, Sharif AW, Rosenfelder NA, Lim AK, Taylor-Robinson SD. Cystic duct dilatation after cholecystectomy in fibropolycystic liver disease. J Hepatol. 2005;43(1):192. https://doi.org/10.1016/j.jhep.2004.08.027
Navascués R, Quiñones L, Guinea O, Guerediaga F. Enfermedad de Caroli en paciente portador de trasplante renal por poliquistosis. Nefrología. 2005;25(3):336-337.
Lesmana CR, Harris A, Kooshartoro A, Poerniati K, Hukom R, Czeresna HS, et al. Hepatic cirrhosis caused by Caroli disease. Acta Med Indones. 2005;37(1):33-5.
Bonet J, Oller B, Castellote E, Rodriguez N. Caroli's disease and cystic kidney disease in a woman. Nephron. 1996;73(2):310-1. https://doi.org/10.1159/000189058
Takatsuki M, Uemoto S, Inomata Y, Egawa H, Kiuchi T, Hayashi M, et al. Living-donor liver transplantation for Caroli's disease with intrahepatic adenocarcinoma. J Hepatobiliary Pancreat Surg. 2001;8(3):284-6. https://doi.org/10.1007/s005340170030
Loreno M, Bo P, Senzolo M, Cillo U, Naoumov N, Burra P. Successful pregnancy in a liver transplant recipient treated with lamivudine for de novo hepatitis B in the graft. Transpl Int. 2005;17(11):730-4. https://doi.org/10.1007/s00147-004-0785-5
Braga AC, Calheno A, Rocha H, Lourenço-Gomes J. Caroli's disease with congenital hepatic fibrosis and medullary sponge kidney. J Pediatr Gastroenterol Nutr. 1994;19(4):464-7. https://doi.org/10.1097/00005176-199411000-00018
Charny CK, Ling P, Botet J, Blumgart LH. Clinical observation: congenital absence of the left portal vein in a patient undergoing hepatic resection. HPB Surg. 1997;10(5):323-6; discussion 326-7. https://doi.org/10.1155/1997/18965
Ozkurt S, Canaz F, Temiz G, Sahin G, Yalcin AU. Unusual renal manifestation of Caroli disease: AA amyloidosis. Ren Fail. 2012;34(7):930-3. https://doi.org/10.3109/0886022X.2012.690923
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
Lefere M, Thijs M, De Hertogh G, Verslype C, Laleman W, Vanbeckevoort D, et al. Caroli disease: review of eight cases with emphasis on magnetic resonance imaging features. Eur J Gastroenterol Hepatol. 2011;23(7):578-85. https://doi.org/10.1097/MEG.0b013e3283470fcd
Levin G, Sabag L, Khatab AA, Rottenstreich A. An unusual case of pregnancy in a patient with Caroli's syndrome. Eur J Obstet Gynecol Reprod Biol. 2018;228:333-335. https://doi.org/10.1016/j.ejogrb.2018.06.030
Perricone G, Vanzulli A. Education and imaging. Hepatology: "central dot sign" of Caroli syndrome. J Gastroenterol Hepatol. 2015;30(2):234. https://doi.org/10.1111/jgh.12828
Takaki Y, Takahashi M, Ueno S, Fukui K. Radiologic demonstration of Caroli's disease: a case report. J Comput Tomogr. 1986;10(2):153-6. https://doi.org/10.1016/0149-936x(86)90068-8
Watschinger B, Schwaighofer B, Wrba F, Pohanka E, Kovarik J. Secondary hypersplenism due to Caroli syndrome complicating immunosuppression in a renal allograft recipient. Nephron. 1989;51(3):413-5. https://doi.org/10.1159/000185334
Saracíbar E, Martín J, Sedano E. Pericarditis constrictiva y quistes hepáticos unilobulares. Revista clínica española. 2001;201(8):483-484. https://doi.org/10.1016/S0014-2565(01)70887-9
Shenoy P, Zaki SA, Shanbag P, Bhongade S. Caroli's syndrome with autosomal recessive polycystic kidney disease. Saudi J Kidney Dis Transpl. 2014;25(4):840-3. https://doi.org/10.4103/1319-2442.135176
Ahmed Z, Pemira S, Chaoji, Mundle R. Caroli's disease-a case report. Journal of Evolution of Medical and Dental Sciences. 2013;2(41):7936-7939. https://doi.org/10.14260/jemds/1398
Bawany MZ, Alaradi O, Nawras A. Caroli's syndrome in a post renal transplant patient: case report and review of the literature. Saudi J Gastroenterol. 2012;18(1):59-61. https://doi.org/10.4103/1319-3767.91728
Kil H, Choi EY, Jeong JI, Park CS, Park SM, Kim SH, et al. [A case of simple type Caroli's disease confined to right lobe of the liver]. Korean J Gastroenterol. 2007;50(4):271-6.
Kumar A, Akselrod D, Prikis M. Caroli Disease Revisited: A Case of a Kidney Transplant Patient With Autosomal Polycystic Kidney Disease and Recurrent Episodes of Cholangitis. Transplant Proc. 2019;51(2):541-544. https://doi.org/10.1016/j.transproceed.2018.12.025
Carrera C, Castiella A, Fernandez J, Cosme A, Garcia-Bengoechea M. Caroli's disease diagnosed by magnetic resonance cholangiopancreatography. Eur J Gastroenterol Hepatol. 2002;14(5):577. https://doi.org/10.1097/00042737-200205000-00019
Sans M, Rimola A, Navasa M, Grande L, García-Valdecasas JC, Andreu H, et al. Liver transplantation in patients with Caroli's disease and recurrent cholangitis. Transpl Int. 1997;10(3):241-4. https://doi.org/10.1007/s001470050050
Kim RD, Book L, Haafiz A, Schwartz JJ, Sorensen JB, Gonzalez-Peralta RP. Liver transplantation in a 7-month-old girl with Caroli's disease. J Pediatr Surg. 2011;46(8):1638-41. https://doi.org/10.1016/j.jpedsurg.2011.04.011
Izawa K, Tanaka K, Furui J, Matsumoto T, Segawa T, Tsunoda T, et al. Extended right lobectomy for Caroli's disease: report of a case and review of hepatectomized cases in Japan. Surg Today. 1993;23(7):649-55. https://doi.org/10.1007/BF00311917
Murcia S, Serrano B. Enfermedad y síndrome de Caroli. Presentación de dos casos y revisión de literatura. Rev Fac Med. 2002;50(1):88-91.
Sinha RJ, Sharma A, Singh V, Pandey S. Medullary sponge kidney and Caroli's disease in a patient with stricture urethra: look for the hidden in presence of the apparent. BMJ Case Rep. 2018;11(1):bcr2018226746. https://doi.org/10.1136/bcr-2018-226746
Martínez A, Gorriz J, Bover J, Segura-de la Morena J, Cebollada J, Escalada J, et al. Documento de consenso para la detección y manejo de la enfermedad renal crónica. Nefrología. 2014;34(2):243-62.
Balsells J, Margarit C, Murio E, Lazaro JL, Charco R, Vidal MT, et al. Adenocarcinoma in Caroli's disease treated by liver transplantation. HPB Surg. 1993;7(1):81-6; discussion 86-7. https://doi.org/10.1155/1993/61048
Higuera F, Servín A, Navarro A, Soto-Martínez K, Cruz-Estrada A, Alexanderson-Rosas EG. Identificación de principales factores predictivos relacionados con mortalidad hepática y muerte cardiovascular en pacientes cirróticos con diabetes tipo 2: estudio de cohorte histórica con seguimiento a 10 años. Revista de Gastroenterología de México. 2018;83(supl 2):9-13.
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