Caroli's disease

Caroli Disease

Saccular intrahepatic duct dilatation with normal sized choledochal duct in Caroli disease

Caroli disease is an autosomal recessive disease secondary to the ductal plate malformation. It is associated with polycystic kidney disease, medullary sponge kidney and medullary cystic disease. So looking at the kidneys can sometimes help you make this diagnosis. On the left we see images of a patient with Caroli disease. Notice the intrahepatic duct dilatation and the normal caliber of the choledochal duct (extrahepatic bile duct).