MR cholangiography in children with autosomal recessive polycystic kidney disease

Background. Magnetic resonance cholangiography (MRC) is a relatively new, n on-invasive imaging technique of the biliary tree that has shown good corre lation with endoscopic retrograde cholangiopancreatography. The liver manif estation of autosomal recessive polycystic kidney disease (ARPKD) is congen ital hepatic fibrosis (CHF). CHF may be accompanied by Caroli's disease, wh ich 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. Materials and methods. Seven children with ARPKD aged from 3.0 to 10.1 year s were examined. CHF was confirmed in all biopsied cases (5 of 7). All chil dren had been followed by repeated abdominal US examinations for many years . The MR examination included a morphological imaging study using a T2-weig hted turbo spin-echo sequence and a heavily T2-weighted inversion-recovery turbo spin-echo sequence with three-dimensional maximum intensity projectio n (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 differentiat ion from hepatic cysts was not possible. By MRC, Caroli's disease could be diagnosed in three of seven children. Furthermore, MRC with MIP reconstruct ions demonstrated the extent of the disease by showing the entire biliary t ree from different angles. Conclusions. MRC is a valuable method to establish the diagnosis and demons trate the extent of Caroli's disease.