POLYCYSTIC disease of the kidney is relatively uncommon in children. It is generally believed that the cysts are due to a failure of union between the secretory and collecting portions of the kidney unit. Polycystic disease of the kidney is mostly bilateral, but unilateral involvement has been reported. Bell has expressed the opinion that polycystic kidneys are inherited as a Mendelian dominant. Cairns and others list the defects with which it is most frequently associated, such as harelip, spina bifida, atresia ani, and defects of kdneys, ureters, and sex organs. Rall and Odel have found associated congenital anomalies encountered in polycystic disease of the kidneys, as polycystic liver, polycystic pancreas, polycystic spleen, cardiac defects, defects of nervous system, and defects of musculoskeletal system.
Potter classifies the polycystic kidneys found in the fetus or young infant into two groups: (1) those in which the amount of renal connective tissue is normal