Controversy concerning optimal treatment for individuals affected by syndromes of abnormal sex differentiation can best be resolved with knowledge about long-term medical, surgical, and psychosexual outcomes of patients. Follow-up information has recently been gathered on older cohorts of the following patient groups: (1) those affected by complete androgen insensitivity syndrome (CAIS) raised female and (2) those affected by congenital micropenis raised male or female. As a group, women with CAIS were satisfied with their female gender and sexual function. However, a need for better patient education was identified for this specific population. Most patients with congenital micropenis, whether raised male or female, were satisfied with their gender. Regardless of sex of rearing, dissatisfaction with the appearance and function of the genitalia as judged by both physicians and subjects was evident. For patients with congenital micropenis, male sex of rearing was concluded to be optimal because genital reconstructive surgery is not required with this choice.
KEYWORDS
Androgen insensitivity - micropenis - gender - sex - intersex
REFERENCES
1
Birnbacher R, Marberger M, Weissenbacher G, Schober E, Frisch H.
Gender identity reversal in an adolescent with mixed gonadal dysgenesis.
J Pediatr Endocrinol Metab .
1999;
12
687-690
2
Phornphutkul C, Fausto-Sterling A, Gruppuso P A.
Gender self-reassignment in an XY adolescent female born with ambiguous genitalia.
Pediatrics .
2000;
106
135-137
6
Wisniewski A B, Migeon C J, Gearhart J P.
Congenital micropenis: long-term medical, surgical and psychosexual follow-up of individuals raised male or female.
Horm Res .
2001;
56
3-11
7 Lewis V G, Money J. Gender-identity/role: G-I/R Part A: XY (androgen-insensitive) syndrome and XX (Rokitansky) syndrome of vaginal atresia. In: Dennerstein L, Burrows GD, eds. Handbook of Psychosomatic Obstetrics and Gynaecology
New York: Elsevier Biomedical Press 1983: 51-60
8
Masica D N, Money J, Ehrhardt A A.
Fetal feminization and female gender identity in the testicular feminizing syndrome of androgen insensitivity.
Arch Sex Behav .
1971;
1
131-142
9 Migeon C J, Brown T R, Fichman K R. Androgen insensitivity syndrome. In: Josso N, ed. The Intersex Child: Pediatric and Adolescent Endocrinology
Basel, Switzerland: Karger 1981: 171-202
10
Money J, Ehrhardt A A, Masica D N.
Fetal feminization induced by androgen insensitivity in the testicular feminizing syndrome: effect on marriage and maternalism.
Johns Hopkins Med J .
1968;
123
105-114
11
Money J, Schwartz M, Lewis V G.
Adult erotosexual status and fetal hormonal masculinization and demasculinization: 46,XX congenital virilizing adrenal hyperplasia and 46,XY androgen-insensitivity syndrome compared.
Psychoneuroendocrinology .
1984;
9
405-414
13 Migeon C JM, Berkovitz G, Brown T R. Sexual differentiation and ambiguity. In: Kappy MS, Blizzard RM, Migeon CJ, eds. Wilkins, The Diagnosis and Treatment of Endocrine Disorders in Childhood and Adolescence
4th ed. Springfield, IL: Charles C Thomas 1994: 573-715
14
Polani P E.
Hormonal and clinical aspects of hermaphroditism and the testicular feminizing syndrome in man.
Philos Trans R Soc Lond B Biol Sci .
1970;
259
187-204
18
Bin-Abbas B, Conte F, Grumbach M M, Kaplan S.
Congenital hypogonadotropic hypogonadism and micropenis: effect of testosterone treatment on adult penile size-why sex reversal is not indicated. J Pediatr .
1999;
134
579-583