title: [Imaging in congenital fibrocystic diseases of the liver].
abstract: 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.
authors.affiliation: Service d'Imagerie Médicale, Centre hospitalo-universitaire Henri Mondor, Créteil. alain.luciani@hmn.aphp.fr
authors.forename: Alain
authors.initials: A
authors.lastname: Luciani
authors.forename: Hicham
authors.initials: H
authors.lastname: Kobeiter
authors.forename: Benhalima
authors.initials: B
authors.lastname: Zegai
authors.forename: Marie-Christine
authors.initials: MC
authors.lastname: Anglade
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Deux
authors.forename: Caroline
authors.initials: C
authors.lastname: Malhaire
authors.forename: Alain
authors.initials: A
authors.lastname: Rahmouni
mesh_terms: D001659:Biliary Tract / Q000196:embryology / Q000473:pathology; D016767:Caroli Disease / Q000175:diagnosis*; D003937:Diagnosis, Differential; D003952:Diagnostic Imaging / Q000379:methods*; D006222:Hamartoma / Q000175:diagnosis; D006801:Humans; D008103:Liver Cirrhosis / Q000151:congenital / Q000175:diagnosis*