CAROLI’S DISEASE IN ADULTHOOD – A CASE REPORT
Abstract
Caroli’s disease (CD) is a rare congenital disorder defined by multifocal segmental dilatation of intrahepatic bile ducts. It may cause severe, life-threatening cholangitis hepatobiliary degeneration or even cancer. A 63-years-old male with monolobar CD managed with conservative treatment is presented with revision of literature. The patient presented in the emergency department with a six-hour history of cholic epigastric pain with dorsal irradiation and a normal physical exam. Past medical history included colonic polyposis, Chronic Obstructive Pulmonary Disease (COPD) and Benign Prostatic Hyperplasia. Investigation revealed infracentimetric left cystic dilatation of intrahepatic bile ducts suggestive of Caroli’s disease. Despite its rare incidence, CD should be considered in patients with recurrent cholangitis. The diagnosis of CD relies mostly on imaging studies. Treatment should be customized and multi-disciplinary.
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References
1. Wang ZX, Li YG, Wang RL, Li YW, Li ZY, Wang LF, et al. Clinical classification of Caroli's disease: an analysis of 30 patients. HPB : the official journal of the International Hepato Pancreato Biliary Association. 2015;17(3):278-83. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4333791/
2. Caroli J, Soupault R, Kossakowski J, Plocker L, Paradowska. [Congenital polycystic dilation of the intrahepatic bile ducts; attempt at classification]. La semaine des hopitaux : organe fonde par l'Association d'enseignement medical des hopitaux de Paris. 1958;34(8/2):488-95/sp.
3. 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. Journal of gastrointestinal surgery : official journal of the Society for Surgery of the Alimentary Tract. 2015;19(11):2019-27. https://link.springer.com/article/10.1007/s11605-015-2918-9
4. Todani T, Watanabe Y, Narusue M, Tabuchi K, Okajima K. Congenital bile duct cysts: Classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst. American journal of surgery. 1977;134(2):263-9. https://www.americanjournalofsurgery.com/article/0002-9610(77)90359-2/pdf
5. 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. Journal of digestive diseases. 2012;13(9):491-5. https://onlinelibrary.wiley.com/doi/abs/10.1111/j.1751-2980.2012.00619.x
6. Hassan Seddik HB, Brahim Aitbihi, Tarik Adioui, Hanane Massit, Ahmed Benkirane. Monolobar caroli’s disease: report of three cases. International Journal of Gastroenterology, Hepatology, Transplant & Nutrition. 2017(2(i)):23-5. http://journal.pghtn.com/wp-content/uploads/2017/04/06-Monolobar-Carolis-disease.pdf
7. Bockhorn M, Malagó M, Lang H, Nadalin S, Paul A, Saner F, et al. The role of surgery in Caroli's disease. Journal of the American College of Surgeons. 2006;202(6):928-32. https://www.journalacs.org/article/S1072-7515(06)00142-6/fulltext
8. 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. Clinical transplantation. 2016;30(1):3-9. https://onlinelibrary.wiley.com/doi/abs/10.1111/ctr.12640
9. Duvnjak M, Supanc V, Virović L, Tomasić V, Dojcinović B. [Caroli's disease]. Acta medica Croatica : casopis Hravatske akademije medicinskih znanosti. 2003;57(3):249-52.
10. Boyle MJ, Doyle GD, McNulty JG. Monolobar Caroli's disease. The American journal of gastroenterology. 1989;84(11):1437-44.
11. Dicasillati R, Cantaluppi G, Mezzetti M. [Caroli's disease. A case report]. Minerva chirurgica. 2002;57(1):81-5.
12. Gillet M, Favre S, Fontolliet C, Halkic N, Mantion G, Heyd B. [Monolobar Caroli's disease. Apropos of 12 cases]. Chirurgie; memoires de l'Academie de chirurgie. 1999;124(1):13-8; discussion 8-9.
13. Giovanardi RO. Monolobar Caroli's disease in an adult. Case report. Hepato-gastroenterology. 2003;50(54):2185-7.
14. Jarry J, Leblanc F, Saric J. [Monolobar Caroli disease]. Presse medicale (Paris, France : 1983). 2010;39(7-8):847-8. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2727094/
15. Hodgson SV, Coonar AS, Hanson PJ, Cottrell S, Scriven PN, Jones T, et al. Two cases of 5q deletions in patients with familial adenomatous polyposis: possible link with Caroli's disease. Journal of medical genetics. 1993;30(5):369-75. https://jmg.bmj.com/content/30/5/369.long
2. Caroli J, Soupault R, Kossakowski J, Plocker L, Paradowska. [Congenital polycystic dilation of the intrahepatic bile ducts; attempt at classification]. La semaine des hopitaux : organe fonde par l'Association d'enseignement medical des hopitaux de Paris. 1958;34(8/2):488-95/sp.
3. 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. Journal of gastrointestinal surgery : official journal of the Society for Surgery of the Alimentary Tract. 2015;19(11):2019-27. https://link.springer.com/article/10.1007/s11605-015-2918-9
4. Todani T, Watanabe Y, Narusue M, Tabuchi K, Okajima K. Congenital bile duct cysts: Classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst. American journal of surgery. 1977;134(2):263-9. https://www.americanjournalofsurgery.com/article/0002-9610(77)90359-2/pdf
5. 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. Journal of digestive diseases. 2012;13(9):491-5. https://onlinelibrary.wiley.com/doi/abs/10.1111/j.1751-2980.2012.00619.x
6. Hassan Seddik HB, Brahim Aitbihi, Tarik Adioui, Hanane Massit, Ahmed Benkirane. Monolobar caroli’s disease: report of three cases. International Journal of Gastroenterology, Hepatology, Transplant & Nutrition. 2017(2(i)):23-5. http://journal.pghtn.com/wp-content/uploads/2017/04/06-Monolobar-Carolis-disease.pdf
7. Bockhorn M, Malagó M, Lang H, Nadalin S, Paul A, Saner F, et al. The role of surgery in Caroli's disease. Journal of the American College of Surgeons. 2006;202(6):928-32. https://www.journalacs.org/article/S1072-7515(06)00142-6/fulltext
8. 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. Clinical transplantation. 2016;30(1):3-9. https://onlinelibrary.wiley.com/doi/abs/10.1111/ctr.12640
9. Duvnjak M, Supanc V, Virović L, Tomasić V, Dojcinović B. [Caroli's disease]. Acta medica Croatica : casopis Hravatske akademije medicinskih znanosti. 2003;57(3):249-52.
10. Boyle MJ, Doyle GD, McNulty JG. Monolobar Caroli's disease. The American journal of gastroenterology. 1989;84(11):1437-44.
11. Dicasillati R, Cantaluppi G, Mezzetti M. [Caroli's disease. A case report]. Minerva chirurgica. 2002;57(1):81-5.
12. Gillet M, Favre S, Fontolliet C, Halkic N, Mantion G, Heyd B. [Monolobar Caroli's disease. Apropos of 12 cases]. Chirurgie; memoires de l'Academie de chirurgie. 1999;124(1):13-8; discussion 8-9.
13. Giovanardi RO. Monolobar Caroli's disease in an adult. Case report. Hepato-gastroenterology. 2003;50(54):2185-7.
14. Jarry J, Leblanc F, Saric J. [Monolobar Caroli disease]. Presse medicale (Paris, France : 1983). 2010;39(7-8):847-8. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2727094/
15. Hodgson SV, Coonar AS, Hanson PJ, Cottrell S, Scriven PN, Jones T, et al. Two cases of 5q deletions in patients with familial adenomatous polyposis: possible link with Caroli's disease. Journal of medical genetics. 1993;30(5):369-75. https://jmg.bmj.com/content/30/5/369.long
Published
2023-06-23
How to Cite
FERNANDES, Urânia et al.
CAROLI’S DISEASE IN ADULTHOOD – A CASE REPORT.
Revista Portuguesa de Cirurgia, [S.l.], n. 55, p. 28-31, june 2023.
ISSN 2183-1165.
Available at: <https://revista.spcir.com/index.php/spcir/article/view/877>. Date accessed: 27 july 2024.
doi: https://doi.org/10.34635/rpc.877.
Issue
Section
Clinical Case
