Abstract

Potter's syndrome was originally described as combination of bilateral renal agenesis and unique progeric facial characteristics in 1946 by Potter. In cases of severe renal maldevelopment such as bilateral polycystic kidney or marked hypoplasia, similar facial features has been also associated. At 1963, Potter and Osathanondth reported classification of polycystic kidney as Type I-IV by microdissection study. Recently authors experienced an autopsy case of polycystic kidney with characteristic potter face, and other combined malformations such as; absence of both ureters and urinary bladder, pulmonary hypoplasia, undescended testes and oligohydramnios, fetal growth retardation and breech presentation. According to the classification of polycystic kidney of Potter and Osathanondth, this presenting case is compatible with type IIB.