Feminizing genitoplasty in patients with 46XX congenital adrenal hyperplasia

Congenital adrenal hyperplasia (CAH) is the most common cause of ambiguous genitalia in newborns. This paper is based upon review of the literature and personal experience. We focus upon the surgical anatomy, pre-operative evaluation, including imaging, mainly by transabdominal ultrasound, and upon the goals and the history of surgical reconstruction. The various surgical techniques are mentioned with a detailed description of our technique used in 52 patients. The timing and staging of the operation and the implications of prenatal therapy are discussed. In conclusion, we believe that infants with 46XX CAH can undergo one-stage feminizing genitoplasty very early in life with satisfactory cosmetic and functional results.