Caroli syndrome and autosomal recessive polycystic kidney disease

Case contributed by Frank Gaillard


Fever, jaundice and abdominal pain.

Patient Data

Age: 15 years
Gender: Female

Enlarged kidneys in addition to bilateral multiple small cystic attenuation areas, 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 syndrome.

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

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 syndrome have association with both ADPKD and ARPKD.

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

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