Intersex problems, such as pseudohermaphroditism, true hermaphroditism or complex intersex disorders, may present at birth with clinical signs of ambiguous genitalia or may develop later as hormonal insufficiencies during puberty. Historically, intersex problems have been treated with surgery, hormonal supplementation and psychological support. However, treating intersex children remains controversial, as there is much debate regarding whether the formation of gender identity is based more on chromosomal (nature) or environmental (nurture) factors.
Categories of Intersex
The most common types of intersex problems include female pseudohermaphrodites (46, XX intersex), who have ovaries with genitalia that appears male; male pseudohermaphrodites (46, XY intersex), who have normal male chromosomal structure and may have normal testes, but have external genitalia that appears female; true hermaphrodites (true gonadal intersex), who have both ovaries and testes; and individuals with complex or undetermined intersex disorders, who display no discrepancy between internal and external sexual organs but may have problems with sexual hormone levels and sexual development.
Causes of Intersex
Female pseudohermaphroditism can be caused by congenital adrenal hyperplasia, a defect in the adrenal glands that cause inadequate amounts of corticosteroids to be produced; maternal exposure to testosterone during pregnancy; maternal androgen-producing tumors during pregnancy; or aromatase deficiency that fails to convert testosterone to estrogen. Male pseudohermaphroditism can be caused by gonadal dysgenesis that results in no testosterone production; enzymatic deficiencies that cause inadequate testosterone formation or inadequate testosterone conversion; and androgen insensitivity syndrome, which results in testosterone receptors that do not function properly. The causes of true hermaphroditism and complex intersex disorders are not known, but disordered chromosomal configurations may play a part.
Treatment
Infants who present with intersex problems are typically treated with surgery before they are 1 year of age. Surgical correction for male sexual assignment includes genitoplasty, insertion of testicular prosthetics and removal of female sexual organs (if present). Female sexual assignment includes clitoroplasty or clitoral reduction, vaginoplasty and removal of male gonads (if present). Children with intersex disorders may also be supplemented with steroids for adrenal suppression to correct congenital adrenal hyperplasia, testosterone for phallic growth or estrogen if a uterus is present.
Psychological Effects on Intersex Girls
In the past, surgeons have considered it easier to create or reconstruct female genitalia, so intersex children were most often assigned female. It was thought that gender roles were most often influenced by how a child was raised. In a Dutch study at Sophia Children's Hospital, clinicians found that 13 percent of girls with intersex problems developed gender identity disorder, displaying sadness and dissatisfaction with their assigned sex. While all of these girls with gender identity disorder had chromosomal or hormonal abnormalities, 86 percent of them also experienced psychological family trauma, such as familial death, divorce or rejection by their parents due to their condition.
Psychological Effects on Intersex Boys
While the Dutch study at Sophia Children's Hospital did not find any instances of gender identity disorder in intersex boys, this could be due to the low number of males enrolled in their study, 5:59. However, the clinicians did note that typical male gender behavior was absent in these five intersex boys, and they were fearful and concerned about the small size of their penises. These boys also exhibited other psychopathological behaviors, such as obsessive compulsive disorder and conduct disorder. In fact, 39 percent of the total intersex children studied (male and female) met the diagnostic criteria for general psychopathology.
References
- Medline Plus: Intersex
- Pediatric Surgery International; Gender Assignment and Hormonal Treatment for Disorders of Sexual Differentiation; Shilpa Sharma, D.K. Gupta;October 2008
- Archives of Sexual Behavior; Long-Term Psychological Evaluation of Intersex Children; Froukje M.E. Slijper, et al; April 1998
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