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.