Maladies fibro-kystiques congénitales du foie : aspects radiologiques

The identification of dilatations of the intrahepatic bile ducts in the absence of bile duct obstruction is rare. Imaging techniques, especially MR cholangio-pancreaticography, generally permit the distinction between fibrocystic liver diseases and polycystic liver diseases. The presence of dilated sacciform or tubular bile ducts on cholangio-pancreaticography associated with a centrally located fibrovascular bundle (central dot sign) suggests Caroli's syndrome. The presence of associated signs of liver dysmorphia including right lobe atrophy and hypertrophy of segment IV suggests associated congenital hepatic fibrosis. The findings on cholangio-pancreaticography, computerized tomography or Doppler ultrasonography correlate well with the pathogenesis of fibrocystic liver diseases, linked to an embryologic malformation of the ductal plate.