Potter's Syndrome with Adult Polycystic Renal Disease: An autopsy case report

Potter's syndrome including bilateral renal agenesis or polycystic renal disease, bilateral pulmonary hypoplasia and characteristic face was first described in 1946. Although a great number of cases of Potter's syndrome was reported, Potter's syndrome with adult polycystic kidney disease(Potter type III) was very rarely found. In this report, we described an autopsy case of Potter's syndrome having adult polycystic kidneys disease, bilateral pulmonary hypoplasia and characteristic face in conjunction with multiple hepatic cysts, features of congenital hepatic fibrosis and a pancreatic cyst. Microscopically, all cysts were lined by cuboidal epithelial cells, showing positive for epithelial membrane antigen and cytokeratins.