title: MR cholangiography in children with autosomal recessive polycystic kidney disease.
abstract: BACKGROUND
  Magnetic resonance cholangiography (MRC) is a relatively new, non-invasive imaging technique of the biliary tree that has shown good correlation with endoscopic retrograde cholangiopancreatography. The liver manifestation of autosomal recessive polycystic kidney disease (ARPKD) is congenital hepatic fibrosis (CHF). CHF may be accompanied by Caroli's disease, which is characterised by a non-obstructive dilation of the intrahepatic bile ducts.
  
  
  OBJECTIVE
  A prospective study was conducted to determine the presence and extent of Caroli's disease in children with ARPKD.
  
  
  METHODS
  Seven children with ARPKD aged from 3.0 to 10. 1 years were examined. CHF was confirmed in all biopsied cases (5 of 7). All children had been followed by repeated abdominal US examinations for many years. The MR examination included a morphological imaging study using a T2-weighted turbo spin-echo sequence and a heavily T2-weighted inversion-recovery turbo spin-echo sequence with three-dimensional maximum intensity projection (MIP) reconstructions for MRC.
  
  
  RESULTS
  The diagnosis of Caroli's disease could be made in one case by US; in two other children Caroli's disease was suspected, but the differentiation from hepatic cysts was not possible. By MRC, Caroli's disease could be diagnosed in three of seven children. Furthermore, MRC with MIP reconstructions demonstrated the extent of the disease by showing the entire biliary tree from different angles.
  
  
  CONCLUSIONS
  MRC is a valuable method to establish the diagnosis and demonstrate the extent of Caroli's disease.
authors.affiliation: Department of Radiology, University of Cologne, Cologne, Germany.
authors.forename: G
authors.initials: G
authors.lastname: Jung
authors.forename: G
authors.initials: G
authors.lastname: Benz-Bohm
authors.forename: H
authors.initials: H
authors.lastname: Kugel
authors.forename: K M
authors.initials: KM
authors.lastname: Keller
authors.forename: U
authors.initials: U
authors.lastname: Querfeld
mesh_terms: D001653:Bile Ducts, Intrahepatic / Q000473:pathology*; D016767:Caroli Disease / Q000175:diagnosis*; D002648:Child; D002675:Child, Preschool; D002758:Cholangiography / Q000379:methods*; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008103:Liver Cirrhosis / Q000151:congenital / Q000175:diagnosis; D008279:Magnetic Resonance Imaging; D008297:Male; D017044:Polycystic Kidney, Autosomal Recessive / Q000150:complications / Q000175:diagnosis*; D011446:Prospective Studies