[Congenital hepatorenal polycystic disease with unusual long survival (author's transl)]

The authors report a case of hepatorenal polycystic disease discovered in the course of a routine physical examination. The patient was asymptomatic and the progression of the disease was extremely low. The existence of the disorder, however, was confirmed in the autopsy. Although hepatorenal polycystic disease is a relatively common clinical finding, the rareness of this condition in patients of advanced age and its occasionally asymptomatic form are pointed out. Clinical and prognostic characteristics of the two types of the disease (infantile and adult) are mentioned too. Physical examination and pyelography are the most import exploratory methods; other procedures such as echography, scanning and peritoneoscopy are complementary techniques. Lastly, the need to establish an early diagnosis in order to avoid unappropiated surgical maneuvers is stressed.