Double vulva. A case report

Double vulva is an extremely rare congenital malformation; there are only 17 previously reported cases in the world literature. The syndrome usually consists of complete or partial duplication of the vulva, vagina, uterus, urethra, bladder and colon, with normal ovaries and kidneys and associated congenital malformations, especially of the lumbar spine. The etiology is uncertain. Mortality in the 18 cases reviewed was 35%. Diagnostic evaluation should include a careful newborn physical examination for associated congenital malformations, especially congenital heart disease, along with a radiologic survey of the lumbar spine, gastrointestinal tract and urinary system. No gynecologic surgical treatment is needed except for cosmetic reasons.