Female gender reconstruction surgery for ambiguous genitalia in children and adolescents

Abstract

This review summarizes the principles of diagnosis in patients with ambiguous genitalia and surgical reconstruction for a female gender assignment. Common goals of corrective surgery are to reduce clitoral size, to create or reshape labia majora, to exteriorize or create a vagina, and to remove potentially malignant gonads. A one-stage clitorovaginoplasty was performed on four children with congenital adrenal hyperplasia (Passerini-Glazel et al., J Urol 1989, 142:565-568). The technique preserved the clitoral glans, used the skin of the urogenital sinus to line the introitus and form the distal vagina, and reshaped the labioscrotum. Early follow-up found minimal vaginal stenosis. A postpubertal vaginoplasty for undermasculinized genetic males or for patients with a flat perineum is described. All 15 patients had functional success, and 93% had anatomic success (Rock and Jones, Am J Obstet Gynecol 1989, 160:845-853). Early gender assignment helps stabilize gender identity, and neonatal reconstructive surgery of the external genitalia appears to be safe. Neovaginal construction is often performed postpubertally but should be individualized to the anomaly and the patient.