[Atypical Caroli syndrome with kidney failure in cystic renal changes].
In a 61-year-old man in terminal renal failure from bilateral renal polycystic degeneration abdominal sonography chanced to demonstrate in the left lobe of the liver a saccular, pearl string-like dilatation of the, at first no further definable, duct systems. Hepatobiliary scintigraphy revealed them to be bile-draining and thus confirmed Caroli's disease, which had previously been asymptomatic for any hepatic involvement. Since in the majority of cases of Caroli's disease renal changes are symptom-free associated findings, the question arises whether in this case there is the coexistence of polycystic renal disease and Caroli's disease or whether the biliary tract and renal changes constitute a nosological entity in the sense of atypical Caroli's disease.
Habscheid
W
W
Medizinische Universitätsklinik, Universität Würzburg.
Götz
R
R
Becker
W
W
Börner
W
W
D001649:Bile Duct Diseases / Q000151:congenital / Q000175:diagnosis / Q000473:pathology; D001653:Bile Ducts, Intrahepatic / Q000002:abnormalities*; D004108:Dilatation, Pathologic / Q000151:congenital / Q000175:diagnosis / Q000473:pathology; D006801:Humans; D007676:Kidney Failure, Chronic / Q000175:diagnosis* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D007690:Polycystic Kidney Diseases / Q000175:diagnosis* / Q000473:pathology; D013577:Syndrome
Atypisches Caroli-Syndrom mit renaler Insuffizienz bei zystischen Nierenveränderungen.