Caroli disease with autosomal recessive polycystic kidney disease (ARPKD)
Diagnosis almost certain
Presentation
Hepatomegaly with bilateral flank masses.
Patient Data
Age: 10 months
Gender: Female
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The CT scan demonstrates:
- enlarged liver
- dilated intrahepatic bile ducts (IHBD) with enhancing dots within the dilated intrahepatic bile ducts "central dot sign", representing portal radicles
- extrahepatic biliary ducts are not dilated
- massively enlarged kidneys with poor cortico-medullary differentiation and numerous bilateral tiny cysts
- left indirect inguinal hernia
Case Discussion
CT features are most consistent with a Caroli disease with associated autosomal recessive polycystic kidney disease (ARPKD).
simple Caroli disease is uncommon; it occurs more frequently with:
- congenital hepatic fibrosis, constituting the Caroli syndrome
- medullary sponge kidney
- autosomal dominant polycystic kidney disease (ADPKD)
- autosomal recessive polycystic kidney disease (ARPKD) as in this case
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