What is the name of following sign seen in Caroli’s disease?
Correct Answer: Central dot sign
Description: Ans. D. Central dot signa. Caroli's disease is a rare, congenital cystic dilatation involving intrahepatic bile ducts. The term Caroli's syndrome is used if the above anomaly is associated with congenital hepatic fibrosis.b. These entities belong to the group of hepatic fibro polycystic diseases, which are usually associated with renal polycystic disease. We report a case of Caroli's syndrome presenting as portal hypertension with medullary sponge kidneys.c. The classical "central dot sign" described on CT is well demonstrated easily on ultrasound. Awareness of this finding is highlightedd. Caroli's disease is characterized by congenital, segmental, nonobstructive, saccular dilatation of intrahepatic bile ducts without other hepatic abnormalities.e. It was presumed to be autosomal recessive in character. Two forms of the disease have been described.f. The rare, so called pure form described by Jacqui Caroli is characterized by segmental saccular communicating intrahepatic bile duct dilatation, frequently present with stone formation, recurrent cholangitis and hepatic abscess.g. The liver involvement can be diffuse, lobar or segmental. It usually presents in childhood and about 75% of affected patients are boys.h. Cholangiocarcinoma can develop in 7% of patients. Caroli's syndrome is another form that is more common and is associated with congenital hepatic fibrosis.i. The dilatation of intrahepatic biliary ducts in usually less prominent. Both the conditions result from malformation of the embryonic ductal plate at different levels of the biliary tree.
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Radiology
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