General Medical Information

Issues at Puberty and The Adolescent Years

Sarah E. Herbert

Certain sexual differentiation disorders do not manifest themselves until puberty or the adolescent years. Girls with complete androgen insensitivity (CAI) generally present in the adolescent years with amenorrhea and a lack of adrenarche. Children with 5a-reductase deficiency who have been raised as girls since birth will begin to experience growth of the phallus, descent of the testes, and production of testosterone leading to increased hirsutism and muscle development. Likewise, true hermaphrodites being reared as girls might experience masculinization at puberty if there is one testis producing testosterone.

A major developmental task of puberty and early adolescence is accepting and adjusting to the multitude of bodily changes that take place at this age. Girls expect to develop breasts and start menses; they do not expect to develop facial and body hair or to become increasingly muscular at puberty. Adolescence is a time of heightened sensitivity to others’ perceptions and a desire to be accepted by one’s peers. A lack of the anticipated pubertal physical changes or growth in ways that are the opposite of what is expected have the potential to lead to confusion, increased anxiety, a negative self-image, and alienation from peers.

Identity issues are also quite significant for adolescents. This is the age at which some intersex individuals begin to articulate questions about their identities as male or female based on physical changes, as well as internal feelings. It also may be the time when some intersex adolescents begin to consider a change of gender.

Adolescence is a time for becoming aware of sexual feelings, developing romantic relationships, becoming aware of one’s sexual orientation, and, for a sizable percentage of teens, becoming sexually active. Sexual orientation for children born with genital ambiguity may not be what they, or their parents, expected, based on the assigned gender and the assumption of heterosexuality. These feelings can be confusing and upsetting for adolescents born without any genital ambiguity, so one can imagine how confusing it might be for adolescents with genital ambiguity. Both adolescents and their parents may need help in understanding and accepting an atypical sexual orientation. Given that over 50% of adolescents in the 15- to 19-year-old age group are sexually active, one would expect that this would be a particularly problematic time for individuals with genital anomalies or conflicts around sexual activity. There are very limited data on the sexual functioning of adults with disorders of sexual differentiation and almost none about teens with these disorders. This is a particularly important time to be available to provide appropriate sex education for the patient and to assist her parents in this endeavor. In addition to making sure the young woman has adequate sex education, discussion of her sexual history in a matter of fact and nonjudgmental way is important at this time.

The adolescent years are the time when cognitive development may reach the level of formal thinking, thus allowing the individual to have more ability to abstract and imagine the future. Therefore, it is a better time for patients to receive more complex explanations about their particular disorder from their physicians, particularly with regard to issues around infertility and reproductive technologies that might potentially help with fertility. Girls born without a vagina become candidates for vaginoplasty at this time. Because compliance with dilatation is such an important issue in keeping the neovagina functional, it has been recommended that this surgical procedure take place when the girl or young woman can understand the importance of the regular dilatation regimen required after vaginoplasty and that she is motivated to comply with it.

Adolescence is a time of increasing desire for autonomy. Data on adolescent decision making suggest that by the age of 15 years, most individuals have a decision-making capacity similar to adults. If further surgical procedures are recommended, this would be a better time to consider them. Adolescence is an appropriate time to allow the girl or young woman opportunities to participate in her medical care and in decisions made about her body.