Intrahepatic biliary cysts are formed in the prenatal period. They constitute 3% to 5% of all liver cysts (cysts of extrahepatic bile ducts are more common).
Clinical features: Manifestations often appear in adulthood and usually reflect an infection in the bile ducts (pain, fever, jaundice). Sometimes only fever and intermittent jaundice are present.
Diagnosis is based on imaging findings (ultrasonography, computed tomography [CT]: see Table 7.3-1; also magnetic resonance cholangiopancreatography [MRCP] and endoscopic retrograde cholangiopancreatography [ERCP]).
Treatment: Resection of the liver with careful histologic evaluation of the removed lesion (risk of developing cancer from the biliary epithelium) or transplant in the setting of Caroli syndrome (multiple congenital segmental dilatations of intrahepatic bile ducts).