Caroli disease and autosomal recessive polycystic kidney disease

Case contributed by A.Prof Frank Gaillard

Presentation

Fever, jaundice and abdominal pain.

Patient Data

Age: 15
Gender: Female

Multiple small cystic attenuation areas are seen in bilateral kidneys, suggesting ARPKD.

Multiple hypodense cystic areas are seen in right lobe of liver, which appear to be of similar attenuation to few linear dilated intrahepatic biliary radicals. Some of the lesions show a central enhancing focus ("central dot sign"), suggesting Caroli's disease.

Associated findings such as atrophied right lobe, left lobe hypertrophy, massive splenomegaly (due to portal hypertension), are also seen.

Large number of varices around the spleen. 

Ectatic cystically dilated peripheral intrahepatic biliary radicals are seen, communicating with biliary tree. Common bile duct and main hepatic duct are relatively prominent measuring 8-9 mm, however appear to have smooth regular outline and does not appear ectatic.

Case Discussion

Caroli disease have association with both ADPKD and ARPKD.

This case shows association of autosomal recessive polycystic kidney disease with Caroli's disease.

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Case Information

rID: 8362
Case created: 25th Jan 2010
Last edited: 12th Mar 2017
Inclusion in quiz mode: Included

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