Adult segmental cystic disease of the kidney: a renal-sparing management approach.

Segmental cystic disease of the kidney is a rare entity with the gross and microscopic features of autosomal dominant polycystic kidney disease localized to only a portion of a kidney. We report a renal-sparing management approach to a patient in whom a multifocal cystic process localized to 1 pole of the kidney was recognized preoperatively. Since neither computerized tomography nor ultrasound can exclude an underlying neoplastic process, surgery remains indicated. However, an understanding of the spectrum of diagnostic possibilities can have an impact on planning the most appropriate surgical approach. We conclude that partial nephrectomy, with appropriate intraoperative pathological assessment, may represent a satisfactory renal-sparing therapeutic algorithm for the management of localized cystic disease.