Caroli's syndrome with autosomal recessive polycystic kidney disease in a two month old infant

Abstract

Caroli's syndrome is a rare congenital disorder that involves intrahepatic bile duct ectasia and congenital hepatic fibrosis, frequently seen with concomitant autosomal recessive polycystic kidney disease (ARPKD). Literature on infants with ARPKD is rare. Here, we present a case of a two month old boy who was diagnosed with Caroli's syndrome and ARPKD.

Fig. 1

Renal ultrasonography showing enlarged, cystic left and right kidneys.

Fig. 2

MRI showing fusiform dilatation of the common bile ducts with round and tubular dilatation of the intrahepatic bile ducts.

Fig. 3

Ductal plate showing irregularity of the ducts lined by low cuboidal epithelium and polypoid projections into a dilated lumen (Magnified by × 100).