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.