Evaluation of long-term results of Caroli's disease: 21 years' observation of a family with autosomal "dominant" inheritance, and review of the literature.

Abstract

A 5-year-old girl underwent laparotomy in 1972 because of hepatomegaly and mottled radiopacities shown by cholangiography. Polycystic segmental dilatation of the intrahepatic bile ducts, typical of Caroli's disease, was found. Thereafter she remained in good health for over 21 years with careful medical management. In 1972 mottled radiopacities of the… (More)

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