A 12-month-old boy presented with fever, Klebsiella bacteremia, emesis, and abdominal pain. US demonstrated multiple intrahepatic cysts containing fibrovascular bundles, known as the central dot sign (Fig. 1). Subsequent percutaneous transhepatic cholangiography showed segmental saccular biliary ductal dilation (Fig. 2). Caroli disease is a rare congenital cystic dilatation of the intrahepatic bile ducts. This predisposes to biliary stasis and consequent lithiasis, cholangitis, abscesses, and septicemia. Additionally, children face increased risk of hepatic fibrosis (termed Caroli syndrome), renal disease, cholangiocarcinoma, and amyloidosis [2].
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