Laparoscopic Liver Resection in a Case of Asymptomatic Elderly Patient with Caroli Syndrome
Caroli syndrome (CS) is a rare congenital disorder first described in 1958 by Jacques Caroli  and it is characterized by dilatation of the intrahepatic biliary tree with malformation of the small bile ducts and hepatic fibrosis. There are two patterns of Caroli disease: focal or simple Caroli disease consists of abnormally dilated bile ducts affecting an isolated portion of liver. The second form is more diffuse, and when associated with congenital hepatic fibrosis, is referred to as Caroli syndrome . It is generally inherited as an autosomal recessive trait and could be associated with other diseases inherited in this manner, e.g., autosomal polycystic kidney disease (ARPKD). It usually affects the left lobe of the liver even though any part could be affected.
The exact prevalence or incidence of the disease or the syndrome is not known. However, more than 200 cases of caroli disease have been reported in the medical literature . The prevalence of caroli disease...
AOA conceptualized, attended the surgery, and wrote the paper. KMN conceptualized and wrote the paper. JT and GP attended the surgery. NL reviewed the radiological images and all authors read this paper.
Compliance with Ethical Standards
Conflict of Interest
The authors declare that they have no conflict of interest.
Institutional review board approval was exempt from our institution because all data were collected from clinical records and imaging systems for routine preoperative planning and follow-up.
Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.
- 1.J. Caroli, R. Soupault, J. Kossakowski, L. Plocker, Paradowska. [Congenital polycystic dilation of the intrahepatic bile ducts; attempt at classification], Sem Hopitaux Organe Fonde Par Assoc Enseign Med Hopitaux Paris. 1958;34:488–495/SP.Google Scholar
- 8.Calinescu-Tuleasca A-M, Bottani A, Rougemont A-L, Birraux J, Gubler M-C, Le Coultre C, et al. Caroli disease, bilateral diffuse cystic renal dysplasia, situs inversus, postaxial polydactyly, and preauricular fistulas: a ciliopathy caused by a homozygous NPHP3 mutation. Eur J Pediatr. 2013;172:877–81. https://doi.org/10.1007/s00431-011-1552-0.CrossRefPubMedGoogle Scholar