[Reconstruction of external genitalia of the girl with adrenogenital syndrome]

Abstract

Congenital adrenal hyperplasia (CAH) is the most common cause for female pseudohermaphroditism. In these patients uterus and Fallopian tubes are present and also in severe cases the clitoris cannot function as penis later in life. Therefore, a neonate with ambiguous genitalia due to CAH should be raised as female. To assess the degree of masculinization, a modification of Prader's classification was used. In 23 girls with ambiguous genitalia reduction clitoroplasty preserving the neurovascular bundle and creating a dorsal preputial skin flap and vaginoplasty with a perineal skin flap were performed. The phallic skin was used to create the labia minora and the anterior vaginal wall. The patients were reevaluated 1 to 14 years after the operation. The cosmetic and functional results have been excellent.