The dictionary definition for hermaphrodite is any organism that possess both male and female genitalia. However, this does not happen in humans, and the term is used to label people whose gender is vague. Another term for these people is intersex. Because doctors are under pressure to give a concrete gender assignment to the newborn, they have to use their best judgment on ambiguous genitalia, which may cause issues with gender identity later in life.
There are two ways in which hermaphroditism can arise:
Spontaneous – Happens due to ‘natural’ events
Iatrogenic – Happens due to ‘artificial’ events (ex: medical treatment)
This guide will discuss each method using examples of hermaphroditism in genetically normal males (XY) and females (XX).
Spontaneous
Congenital Adrenal Hyperplasia (aka Adrenogenital Syndrome)
This is caused by overdevelopment of the adrenal glands, causing them to produce excessive amounts of adrenal androgens. Normally, there is a negative feedback between the adrenal gland and the anterior pituitary gland (under the brain). The pituitary secretes adrenocorticotropic hormone (ACTH), which stimulates the adrenal gland to produce hormones. Cortisol, one of the hormones produced, then travels back to the pituitary to inhibit further ACTH release. However, in individuals with CAH, they are unable to produce cortisol, so the adrenal glands keep producing hormones, including androgens.
In females, excessive androgens cause formation of external genitalia with a masculine appearance, including an enlarged clitoris and an empty scrotum-like structure. To treat this, surgery can be performed to reduce masculine features. Glucocorticoid treatments can also be used to compensate for the reduced cortisol levels and bring androgen levels back down.
In males, this causes precocious (early) puberty. Glucocorticoid treatments may have some effect if given very early on, but for the most part, is left untreated. These children are characterized by an early onset of puberty and increased IQ as a child, but as an adult, they are pretty much the same as other adults. The main problem they have as an adult is short stature as excessive androgens before puberty causes stunted height growth.
Maternal Masculinization
If the mother has high levels of circulating androgens while carrying a female child, more masculine genitalia will form, similar to CAH, with an enlarged clitoris and scrotum-like structure. Because the child is XX, the genitalia will only masculinize, not form into male genitals. After birth, there are no hormonal anomalies due to this disorder, so the main treatment would be surgery to reduce the masculine features.
Androgen Insensitivity Syndrome (Testicular Feminization)
This is a disorder found in males, where they lack or have dysfunctional androgen receptors. There can be partial or complete androgen insensitivity. In partial insensitivity, genitalia may be present as an enlarged clitoris, micropenis, and/or undescended testicles. In complete insensitivity, they may have a vagina with no uterus, cervix, or ovaries, and have undescended testicles. Another effect of androgen insensitivity unrelated to sex would be no body hair growth. There is no treatment for this, and many people who have AIS live their lives as a female and might not find out until they have problems conceiving a child.
Pseudohermaphroditism
A major way of manifesting this disorder is inbreeding. Genetic males fail to produce 5-α-reductase to convert testosterone to dihydrotestosterone, which is more effective than testosterone for masculinizing external genitalia. Children can range in looks from almost exactly like a female, to having an enlarged clitoris and a lumpy labia. During puberty, because they are genetic males,testosterone levels increase rapidly, and the body produces a different enzyme to convert testosterone, and the penis and scrotum are able to grow. There is no treatment, and during puberty, the majority of afflicted individuals transitioned from a female to male gender identity.
Iatrogenic
Progestin-Induced Hermaphroditism
This is the main case in iatrogenic hermaphroditism. In the 1950s and 1960s, mothers could opt to have a progestin treatment to prevent miscarriages. It worked, but the synthetic progestin used was also androgenic. Like CAH and Maternal Masculinzation, this increase in androgens caused masculinization of external genitalia. After birth, there are no hormonal anomalies due to this disorder, so the main treatment would be surgery to reduce the masculine features.
How to raise an intersexed child
There is no consensus as to how to raise such a child, but a growing number of specialists follow recommendations put forth by Diamond & Sigmundson (1997).
- Be honest with the intersexed person and the family, and do nothing without their informed consent.
- Identify the child as male or female based on predominant external appearance.
- Raise the child consistent with the identified gender, but be prepared for a change in gender identity as the child grows older.
- Surgery was mentioned as a treatment, but this needs to be left to the discretion of the intersexed person as an adult. Do not perform surgery on the child as it can impair sensations and the child’s sexual orientation could develop either way.
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