Caroli's disease and autosomal dominant polycystic kidney disease: a rare association?

Abstract

The visceral abnormalities most frequently associated Endoscopic retrograde cholangiography failed, but with autosomal dominant polycystic kidney disease transparietal cholangiography showed diuse cystic ( ADPKD) are hepatic in origin. Liver cysts are the dilatations of intrahepatic biliary ducts with multiple most common but by no means the sole manifestation: calculi in the right lobe and led to the diagnosis of the extrahepatic biliary tract may also be involved Caroli’s disease. We then concluded that previous E. coli [1,2] and rare cases of congenital hepatic fibrosis may septicaemias were angiocholitis episodes. be observed [3]. In a case of ADPKD associated with Surgery was decided on, at first to explore the left Caroli’s disease, we were confronted with diagnostic lobe biliary system, and secondly to perform right lobe and therapeutic problems raising embryological and hepatectomy if Caroli’s disease was limited. In fact, genetic issues. because of diuse dilatations (Figure 1) with lithiasis in both lobes, only a choledocoduodenal anastomosis was performed, with an enterostomy to permit endoscopic