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.
Autosomal dominant polycystic kidney disease (ADPKD) is a hereditary disorder characterized by multiple expanding cysts in both kidneys, and they ultimately destroy the renal parenchyma and cause renal failure. Intracranial aneurysms are found in approximately 10% to 15% of ADPKD patients.
Subarachnoid hemorrhage (SAH) from a ruptured intracranial aneurysm is a frequent complication in patients with ADPKD and it makes up a considerable proportion of the causes of death in this group of patients. We report here an autopsy case of polycystic kidney disease that was morphologically identical to ADPKD, and the patients had presented after death with SAH due to a ruptured cerebral aneurysm.