Spot Diagnosis

Caroli`s disease



Question :
This child had chronic renal failure and hepatomegaly.



What is the diagnosis?

Discussion :
Caroli’s disease. It is a rare communicating segmental or diffuse dilatation of the intrahepatic biliary tree. There are two forms of Caroli’s disease, one associated with congenital hepatic fibrosis and a simpler form occurring alone. The former, called Caroli’s syndrome is associated with portal hypertension. The later, known as Caroli’s disease, may be associated with autosomal recessive polycystic kidney disease(6) or rarely with autosomal dominant polycystic kidney disease.

Correct Answers : yes  43%

Last Shown : Oct 2006
 
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