Asploro Journal of Biomedical and Clinical Case Reports
Article Type: Case Report
Asp Biomed Clin Case Rep. 2023 Apr 14;6(2):64-68
Ennio Duranti1iD*, Diletta Duranti2iD
1Department of Nephrology, Dialysis Hospital of Arezzo, Arezzo, Italy
2Toxicology Laboratory Hospital of Arezzo, Director Nephrology Department Hospital of Arezzo, Arezzo, Italy
Corresponding Author: Ennio Duranti ORCID iD
Address: Department of Nephrology, Dialysis hospital of Arezzo, Via Pietro Ne-nni 20-22, 52100 Arezzo, Italy.
Received date: 13 March 2023; Accepted date: 10 April 2023; Published date: 14 April 2023
Citation: Duranti E, Duranti D. Caroli’s Syndrome in Hemodialysis: When Diagnostic and Therapeutic Difficulties can have Serious Consequences. Asp Biomed Clin Case Rep. 2023 Apr 14;6(2):64-68.
Copyright © 2023 Duranti E, Duranti D. This is an open-access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium provided the original work is properly cited.
Keywords: Caroli’s Syndrome, Polycystic Kidney Disease, Polycystic Liver, Renal Disease, Hemodialysis
What we are going to present is the case of a 65-year-old male patient affected by renal and hepatic disease without checking other cases in his family history. He had been submitted to maintenance hemodialysis for 4 months because of an autosomal recessive polycystic kidney disease. At the start of the hemodialysis session, he reported general malaise, abdominal pain, fever, and, during the last two days, even diarrhea. Laboratory workup showed neutrophilic leukocytosis and an increase in serum amylase and C-reactive protein. Abdominal contrast-enhanced CT scan and MRI cholangiography showed hepatic cysts with marked dilatation of the intra- and extrahepatic bile ducts. The patient underwent cholecystectomy with hepatic-jejunal anastomosis on the loop of Roux and was discharged with oral ciprofloxacin. Histology confirmed marked cystic dilatation of biliary ducts and choledochus. Similar episodes continued, and every time ciprofloxacin was prescribed. After 4 months, a retrograde-endoscopic-pancreatography was undertaken, which led to the diagnosis of Caroli’s syndrome associated with polycystic kidneys.
Given the rarity of the disease and the difficulty of diagnosis, it is our opinion that when patients with polycystic kidneys and liver cysts undergo recurrent episodes of septic fever of unknown origin, Caroli’s disease should be considered, and the appropriate tests should be carried out to confirm the diagnosis.