Case ReportCaroli's Disease
Introduction
Caroli's disease is a rare congenital disorder of the liver characterized by saccular or fusiform dilatation of the intrahepatic biliary ducts. In majority the transmission is autosomal recessive [1] but autosomal dominant inheritance has also been reported [2]. The main clinical features are intermittent abdominal pain, hepatomegaly and recurrent cholangitis [1, 3]. We report an unusual case where in spite of advanced cystic dilations of biliary tree, the patient did not suffer a single episode of cholangitis.
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Case Report
A 10 year old male child presented with the complaints of intermittent right upper quadrant abdominal pain of four years duration. There was no history of fever, jaundice, haematemesis or melena. There was no other significant past history. He was the first child of a third degree consanguineous marriage, the other two siblings being healthy. Physical examination revealed an averagely built and nourished child with no pallor, icterus or significant lymphadenopathy. The liver was enlarged, 13 cm
Discussion
1n 1958 Jacques Caroli first described this rare congenital anomaly of intra hepatic bile ducts of which he described two variants [4]. The first variant called Caroli's syndrome is associated with hepatic fibrosis and portal hypertension. The second variant called Caroli's disease is associated with autosomal recessive polycystic kidney disease, autosomal dominant polycystic kidney disease and choledochal cysts [4, 5]. The cystic dilatations in Caroli's disease may be localized to one lobe of
Conflicts of Interest
None identified
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