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Case Reports
. 2006 Feb 28;47(1):131-4.
doi: 10.3349/ymj.2006.47.1.131.

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

Jeong Tae Kim  1 , Yoon Jeong Hur, Jee Min Park, Myung Joon Kim, Young Nyun Park, Jae Seung Lee
Affiliations
Case Reports

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

Jeong Tae Kim et al. Yonsei Med J. .

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.

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Figures

Fig. 1
Fig. 1
Renal ultrasonography showing enlarged, cystic left and right kidneys.
Fig. 2
Fig. 2
MRI showing fusiform dilatation of the common bile ducts with round and tubular dilatation of the intrahepatic bile ducts.
Fig. 3
Fig. 3
Ductal plate showing irregularity of the ducts lined by low cuboidal epithelium and polypoid projections into a dilated lumen (Magnified by × 100).
See this image and copyright information in PMC

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