CASE 1
ANSWER: Caroli
disease.
.
The oblique ultrasonogram of the liver shows saccular
dilatations of the
intrahepatic bile ducts and
intraluminal portal veins (v).
.
The abdominal CT scan shows saccular dilatation of the intrahepatic bile
ducts.
Note several tiny dots (arrows)
in the dilated bile ducts in the left lobe, and bridge
formation (multiple septa)
across the dilated bile ducts in the right hepatic lobe
..
Because
of persistent cholangitis, the patient underwent hepatectomy of the right
lobe. The pathologic specimen showed saccular dilatation of intrahepatic
bile ducts consistent with Caroli disease, active cholangitis, and congenital
hepatic fibrosis. A microscopic study showed portal radicles surrounded
by dilated bile ducts.
.
Microscopic study (X40) of
the liver shows dilated
bile duct surrounding a portal
radicle.
.
DISCUSSION
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