ABSTRACT
The magnitude and timing of surgical reconstruction are the subjects of significant controversy. Patients with a low-confluence UGS can be operated on once their metabolic management is well controlled; in most cases, the authors undertake an elective reconstruction between 3 and 6 months of age, but repair can be done in the newborn period if the social situation so warrants. Planning of the surgical reconstruction should incorporate the three components of a feminizing genitoplasty, in which the prepuce is used to create labia minora, if needed the clitoris is reduced, and the labiosacrotal swellings are used to fashion female-appearing labia majora and to enhance the vaginoplasty. The vaginoplasty is incorporated into the surgical reconstruction outlining a wide base inverted "UG"-shaped perineal flap based on the anus and using the ischial tuberosities as a landmark; the apex of this flap is advanced and placed at the estimated final location of the widened vagina.
