Prenatal onset of autosomal dominant polycystic kidney disease

Autosomal dominant polycystic kidney disease presenting in the fetus or newborn is rare, only 22 cases having been reported in the literature. A case is reported of a premature newborn infant with severe renal involvement and extrarenal associated abnormalities. The literature is reviewed, and the importance of considering this entity in infants with polycystic kidney disease is discussed since it may affect both genetic counseling and surgical management when radiographically detected.