[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.