surgical management of ambiguous genitalia secondary to congenital adrenal hyperplasia Alexandria experience

ABSTRACT

Congenital adrenal hyperplasia is the most common cause of female pseudohermaphroditism all-over the world. The excessive androgenic intermediates released by the diseased adrenal gland virilize the external female genitalia during the early in-utero life to a variable degree. Medical treatment is mandatory to correct the life-threatening metabolic derangement leading to salt-losing crises and surgical correction of the genitalia is essential for the psychologic and functional integrity of the female patient We operated on 12 girls with CAH to correct their genitalia. The surgical procedure included [1]. after complete degloving of the hypertrophied phallus, mobilization of the roots of the corpora to their attachment at the pubic arch. The neurovascular bundle is dissected and preserved to keep supply of the glans. The corporal bodies are dissected from their attachment to the pubic bone to their termination at the glans. Adequate hemostasis is performed and the glans is fixed with sutures to the roots of the corpora. [2]. A posterior perineal flap is fashioned by drawing an inverted u incision centered on anal verge and reaching to the posterior limit of urogenital sinus anteriorly. [3]. the skin of the phallus, after degloving, is used for replacing the labia minora after division in the midline. The results of this operative procedure proved very effective with excellent cosmetic appearance of the genitalia and good vaginal patency in all the cases followed-up for three years. Mild vaginal stenosis was encountered only in three girls but none had tight stenosis. In conclusion, our data proved that complete correction in one stage is the treatment of choice. Reduction clitoroplasty is the best procedure for the treatment of phallic enlargement. Posterior perineal flap vaginoplasty is highly successful in preventing later vaginal stenosis. However, regular follow up is recommended for calibration and dilatation of the vagina and adjustment of the doses of steroid to assure good suppression of adrenal androgens in these girls