Abstract
The diagnosis, pathogenesis, and management of a particularly rare variety of nonadrenal female pseudohermaphroditism are discussed. The characteristic presenting features are clitoromegaly, duplication of the urethra, and dysplastic vagina. About 20 cases have been reported so far; we describe the details of two new cases. One girl presented at the age of 4 years and the other at the age of 3 1/2 years. Both had a posteriorly placed, single urethrovaginal orifice, an enlarged clitoris with an accessory urethra traversing its entire length, urocolpos, and bladder-outlet obstruction. The operative procedure was staged to achieve a free flow of urine with continence through a single visible opening in the anterior vestibule, a normal-looking clitoris, and a vagina of normal caliber opening at the normal site.
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