#0299

Gender Assignment of the Virilized Female in Congenital Adrenal Hyperplasia

J. Lin¹, J. Chen²

¹Changhua Christian Children HospitalChanghua Christian Hospital,, Changhua, Institute of Medicine 2, Chung Shan Medical University, Taichung, Taiwan, Section of Pediatric Urology, Department of Urology, Changhua, Taichung, Taiwan
²Changhua Christian Hospital, Department of Urology, Changhua, Taiwan

Introduction:

Congenital adrenal hyperplasia (CAH) is one of the most common inborn endocrine disorders; some patients are not identified, or may even die in an acute salt-losing crisis. Virilized females are 46xx that present at birth with ambiguous external genitalia but normal internal genitalia. The management of children with congenital adrenal hyperplasia (CAH) remains a challenge in both gender assignment and management. To be boy or girl? That is the question. The criteria of sex assignment are according to sex chromosome, the parent’s expectation, psychological manifestation, the external gentalia or internal genitalia.

Material and methods:

Between 1994 and 2014, 12 patients with CAH were counseled for gender assignment. We have been following these patients for 20 years. The factors of consequence for gender assignment are (1) the age at diagnosis and managements, (2) the degree of virilization, (3) sharing decision-making with the parents. The treatments included: one observation, 10 clitoris reduction with feminizing genitoplasty, and one masculinizing reconstructive operations was performed with hypospadias repair, removal of Müllerian structures, correction of bifid scrotum, insertion of both testicular prosthesis, and hysterectomy with oophorectomy. He has been suppling with steroid replacement ( hydrocortisone 10-15mg/m2 per day, 9α-fludrocortsol 30-75 μg per day ) in patient who remained “boy”. Results: There are 12 patients presented after the neonatal period. All patients had already assumed or were advised of a gender for their children, based on the suggestive appearance of the external genitalia and the factors for gender assignment.

Results:

There are 12 patients presented after the neonatal period. All patients had already assumed or were advised of a gender in their children, based on the suggestive appearance of the external genitalia and the factors for gender assignment. All of the patients were followed up for 20 years. They satisfied their genders and enjoyed their life so far.