Caroli´s disease is first described in 1958, characterized by diffuse cystic dilatation of intrahpatic bile ducts..It is very rare congenital disease and has-been reported only about 20 cases in world literature and one case in this country.
It is manifested by cholelithiasis, cholangitis, fever, chillness, jaundice, upper abdominal and right upper quadrant discomfort and in severe occasions, liver abscess and sepsis may appear, but portal hypertension or liver failure does not present.
Due to recurrent cholelithiasis, repeated laparotomy may be undertaken.
Among diseases to be differentiated are congenital hepatic fibrosis, polycystic liver and obstructive bile duct dilatations.
Chief distinguishing points are: portal hypertension is present in congenital hepatic fibrosis; clear, bile-free cystic contents and no communication of cyst with bile duct in polycystic liver; diffuse proximal dilation of bile duct throughout terminal portion and its reversible change when obstruction is relieved in obstructive bile duct dilatation.
Recently we experienced one case of Caroli´s disease in 13 year old girl who admitted due to jaundice, right upper abdominal discomfort and pain, nausea, vomiting and intermittent fever. She was undertaken Roux-en Y choledochocystoenterostomy at 2 year 8 month old (10 years ago).
Liver scanning revealed three cold lesions at both lobes and operative cholangiogram shows
two cystic dilatations in right lobe and one cystic dilatation in left lobe.
Confirming well communications of these three cystic lesions each other, side-to-side
intrahepatic cystoduodenostomy was done.
With uneventful recovery, she has been in very satisfactory state in the follow up of 23 months postoperatively.
We present this interesting, rare case with the review of literature.
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