Figure 923

Autosomal-dominant polycystic kidney disease (ADPKD): hepato-biliary manifestations. Liver cysts are the most frequent extrarenal manifestation of ADPKD. Their prevalence increases dramatically from the third to the sixth decade of life, reaching a plateau of 80% thereafter [25, 26]. They are observed earlier and are more numerous and extensive in women than in men. Though usually mild and asymptomatic, cystic liver involvement occasionally is massive and symptomatic (see Figure 9-24). Rare cases have been reported of congenital hepatic fibrosis or idiopathic dilatation of the intrahepatic or extrahepatic tract associated with ADPKD [25, 26].

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