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Case Reports
. 2021 Jul-Sep;11(3):195-197.
doi: 10.4103/ijabmr.IJABMR_160_20. Epub 2021 Jul 19.

A Rare Case of Caroli's Syndrome

Satish Kumar Prasad  1 , Sameer Kumar Mehta  1 , Ankit Poddar  2
Affiliations
Case Reports

A Rare Case of Caroli's Syndrome

Satish Kumar Prasad et al. Int J Appl Basic Med Res. 2021 Jul-Sep.

Abstract

Caroli's syndrome is a rare autosomal recessive congenital disorder of the biliary tree characterized by intrahepatic bile duct dilation and hepatic fibrosis. Very few cases have been encountered in routine day-to-day practice. The patients usually present with features of cholangitis such as pain abdomen and jaundice. They may also present with features of chronic liver disease and portal hypertension. Very rarely, they may develop cholangiocarcinoma and present with jaundice, weight loss, and abdominal mass or ascites. Here, we report one such case of a young female who presented to us with features of cholangitis with sepsis and encephalopathy, which was finally diagnosed as Caroli's syndrome. The aim of presenting this case is to learn that even patients with common symptoms of pain abdomen and jaundice may be harboring some rare congenital disease like Caroli's syndrome, as in our case.

Keywords: Caroli's disease; Caroli's syndrome; cholangitis; chronic liver disease; intrahepatic bile duct dilatation.

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Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1
Figure 1
USG Abdomen - Showing multiple heterogenous lesions in both the lobes of liver
Figure 2
Figure 2
MRI ABDOMEN - multiple saccular and cystic lesions of varying sizes seen predominantly located peripherally and were continuous with the biliary radicles
Figure 3
Figure 3
MRI abdomen-shows multiple hypoechoic lesions scattered in both lobes of liver. (A)Few lesions show a hyperechoic focus within it -which represents a vessel. These findings are s/o Caroli disease.
See this image and copyright information in PMC

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