INTRODUCTION
Congenital saccular dilatation of intrahepatic bile ducts, also known as communicating cavernous ectasia or Caroli’s Disease was first described in 1958 by J. Caroli for whom the name of the disease is attributed to.1 Type I, characterized by pure cystic dilatations of the intrahepatic bile ducts is known as Caroli’s disease (CD). Type II or complex CD, known as Caroli syndrome is characterised by multiple or saccular dilatation of intrahepatic biliary duct (IHBD) in association with congenital Hepatic Fibrosis.2
The incidence of Caroli syndrome is extremely low and estimated to be one case per 10,00,000 people, with Caroli disease being even rarer.3
Symptoms of Caroli’s syndrome include complications of Caroli’s disease (bouts of cholangitis, hepatolithiasis, and gallbladder stones) and those of Congenital Hepatic Fibrosis (CHF) and Portal hypertension (PH). The main and often the only symptom is unexplained fever without pain or jaundice.4Portal hypertension is not a common and early presentation of the disease.
Here, We present a case of Caroli’s Syndrome in a Five-year-old girl with dilatation of IHBD in the right lobe of the liver, bilateral polycystic kidney, and portal hypertension.