Perineal reconstruction in ambiguous genitalia infants raised as females.
Sixty-six patients with ambiguous genitalia, representing a combined experience, underwent reconstruction of the perineum to achieve a feminine phenotype. These patients represent four major etiologic groups, adrenogenital syndrome, male pseudohermaphroditism, mixed gonadal dysgenesis, and true hermaphroditism. If the patient is to be raised as a female, the perineum is reconstructed early in the neonatal period by doing a clitoral recession, labial reduction, and vaginal exteriorization. The latter is delayed if the vagina enters the urogenital sinus high, until 2 years. The factors affecting the choice of gender and the details and the timing of the surgical techniques are described.
D000312:Adrenal Hyperplasia, Congenital / Q000601:surgery; D002987:Clitoris / Q000601:surgery; D012734:Disorders of Sex Development / Q000601:surgery*; D005260:Female; D006060:Gonadal Dysgenesis, Mixed / Q000601:surgery; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D010502:Perineum / Q000601:surgery*; D013518:Surgery, Plastic; D014621:Vagina / Q000601:surgery