Familial polythelia without associated anomalies

Of the many forms of supernumerary breast tissue, the most common form is the isolated presence of an accessory nipple, polythelia. While familial polythelia is recognized, it is extremely rare. In the past several years, polythelia has been noted to be associated with nephrourological anomalies. All reports of such a relationship are in random, nonfamilial cases of polythelia. We report three cases of polythelia in a family over two generations who had no urinary tract abnormalities. Discussion includes a comprehensive review of familial polythelia and its association with renal anomalies. From this review, the association of familial polythelia with nephrourological abnormalities will be delineated. Although in this report of a single family with polythelia we did not demonstrate any renal anomalies, we feel that a thorough physical exam, urine analysis, and renal ultrasound should be pursued in any patient with a significant familial history of polythelia.