Localized cystic disease of the kidney: case report and review of the literature.

Localized cystic disease of the kidney (LCDK) is a benign and non-progressive renal anomaly that may initially be confused with autosomal dominant polycystic kidney disease (as in the index case) or cystic neoplasms. It is best diagnosed with contrast-enhanced CT scan demonstrating the characteristic features of an unencapsulated mass of smooth-walled cysts with enhancing renal parenchyma between them. It is most often an incidental finding, but may present with flank pain or haematuria. Hypertension and renal impairment are uncommonly associated with LCDK. A complete physical examination and comprehensive family history are important to exclude other acquired or inherited causes of cystic kidney disease and to avoid unnecessary diagnostic procedures or nephrectomy. We describe a case manifesting many of the typical features of the condition and review previously published cases to describe the natural history and presentation of this rare entity.