There is a lot of variation in how the genitalia develop from person to person in all of us. Nature provides us with a wide spectrum of forms, onto which our society imposes two absolute categories of male and female. In my last post, I described how all people start out with the same genitals in the womb, and how the phalloclitoris differentiates during development. In this post I will discuss the range of natural genital forms, explaining how they develop from the shared embryonic phalloclitoral structures.
I will illustrate this post with simple diagrams. I know that there is a lot of interest in what intersex genitals actually look like—most of the people who find this blog do so by searching for these words. I've discussed elsewhere why I will not post medical photographs of intersex people's genitalia—these often picture children photographed without their consent, and I will not participate in their exploitation. But I do support people's impulse to know more about the range of human forms. I want to help lift the veil of medically-enforced secrecy that makes our bodies invisible, so that interesex bodies can be demystified and accepted. So: diagrams it is.
I will start
by reviewing the structures of our shared original genital form, and showing how they develop in what are deemed “normal” males and females.
The Embryo
We all begin life with genitals that have four basic external elements. At the top is the part numbered 1 and colored pink on this illustration: the sensitive end of the phalloclitoris, which can differentiate into the head of the penis or clitoris. Below it is structure 2, drawn in orange, which is capable of differentiation into either a phallic shaft, or clitoral body and labia minora. In the center is structure 3, drawn in green: an inset membrane that can widen or can seal as the fetus develops. It will form the urethra, and the vagina, if any. And at the outside is the fourth part, colored blue: the labioscrotal swellings, which can develop into labia majora or a scrotum.
“Normal Differentiation”
You can see how the four sectors of the embryonic genitalia differentiate in the diagrams of “typical” male and female genitals pictured here (illustrated without the foreskin or "hood"). Click on any illustration to see it larger. Notice that the pink phalloclitoral head points downward in typical female development and upright in typical male development. The orange body of the phalloclitoris separates and is buried beneath the labia in females, while it closes around the urethra and forms the penile shaft in males.
Sex variance occurs in many forms, but they are not random. Intersex conditions are produced by regular patterns of variation in development of one or more of the four parts of the embryonic genitalia. Let us consider a series of intersex conditions to see how these variations arise, and how they are framed by doctors.
“Aphallia”
Aphallia the term given by doctors to a form of sex variance produced when the first two sections of the embryonic genitala do not develop. While this is equally likely to occur in individuals with ovaries as those with testes, it is only generally commented upon medically when the individual has testicles and XY chromosomes. This illustrates how Western medicine is permeated by a strong gender bias. Having a large, erectile penis is considered a necessity for males, and its absence a tragedy of the highest order, to be addressed by somber medical articles. Having a clitoris capable of sensation and erection, however, is given little attention—so little that its congenital absence is treated as worthy of nothing more than a footnote.
The gendered beliefs that permeate Western medicine are further illustrated by the treatment plan for infants with testes who have aphallia. American doctors typically give these children sex reassignment surgery to remove the testes and create a vagina, it being apparently impossible to tolerate the idea of children being raised as boys without a penis. Without this surgical castration, the children could grow up to be fertile, but their fertility is medically sacrificed without their consent. What is particularly noteworthy is that doctors speak of the sex-reassigned patient with aphallia as growing up to have “normal female sexual function.” “Normal,” for a female, is thus medically defined as being capable of receiving a penis in a vagina, not having sexual pleasure.
“Microphallus”
Some people have large feet and some people have small ones; some have large noses and while others' are petite. When the phalloclitoris is quite small in a person with external testes and a male genital configuration, doctors say the individual has “microphallus.” If the testes are deemed "inadequate," doctors often advise sex assignment to female in infancy as they do in the case of aphallia, because life as a man with tiny sex organs is deemed tragic. Again, the individual's fertility is sacrificed without consent. If the testes are considered normal the child may be treated instead with injections of testosterone, in effect triggering puberty in toddlerhood and leading to moderate enlargement of the clitorophallus (along with other premature pubertal effects such as the development of adult patterns of body hair).
Rarely considered as options by doctors are simply allowing the child to live life as a male with a small penis, or to decide for zirself what course of action to take. Whether the sacrifice of some or all sexual sensation to have genitals that appear female is better than living life as a person with testes and a very small phallus is not a question that science can give a single “correct answer.” It is a subjective and highly personal decision, and will be driven most strongly by the gender identity the child grows to develop. I and other intersex advocates believe that only the intersex person can make such a lifechanging decision, and that for doctors to force their choice upon an unconsenting child is both arrogant and cruel.
“Clitoromegaly”
When a child with typically-male-configured genitals has a large phalloclitoris, the doctors make admiring jokes with the parents. But when the child is female, having a large phalloclitoris is deemed a “birth defect.” Despite the lack of any functional harm from having a large clitoris, doctors perform surgery to “reduce” it to the “acceptable” female range. This often seriously impairs sexual sensation. Although today doctors like to brag that they preserve sexual sensation because they have abandoned the older surgical treatment of “clitoral amputation,” usually some sensation is lost in “clitoral reduction,” and sometimes the phalloclitoris loses all sensation, even though some of the tissue is permitted to remain. It is especially ironic that the removal of part of the clitoris in traditional female circumcision practices is renounced as “female genital mutilation” by Western doctors, yet they perform a similar procedure in cases of “clitoromegaly” without compunction.
“Chordee”
The head of the phalloclitoris bends down in typical “female” configuration. When it does so in a person assigned male, it is termed “simple chordee.” In some individuals, the only atypical characteristic is the folded-down head of the phallus, which is of typical penile size. Doctors present this status as a “malformation of unknown cause,” rather than as a typically-female shape of the phalloclitoris in a male, because they are averse to terming any condition in a child assigned male “intersex.” But chordee is not a random alternative shaping of the penis, as if the penile head might have been equally likely to spontaneously bend in an S-shape. Chordee arises when Sector 1 of the embryonic tissue develops in the “female” configuration, while the rest of the genital development is typically male. Doctors usually suggest surgical “correction” of the phalloclitoral bend, citing locker-room teasing and a purported challenge to fertility. Such surgery presents a serious risk to sexual sensation in the penile head. Furthermore, fertility is not impaired by having a bent or curved penis—the production of sperm is unaltered. Penetrating some partners may be more difficult, but there are many ways to engage in both sexual interaction and fertilization other than via penetrative sex, and only the possessor of the bent penis can decide whether it makes sense to risk the sacrifice of sensation in the phalloclitoral head to make it easier to engage in penetrative sex with partners who prefer a narrow penis. (Some partners may find the phallus with chordee to be more sexually stimulating than a typical penis.)
In other individuals with chordee, the phalloclitoris is of intermediate size. It appears as an intermediary form evenly balanced between the male and female manifestations of the phalloclitoris. Often the individual also has a shallow vagina (discussed below under “hypospadias”).
Whether individuals assigned female at birth may have phalloclitoral heads that do not bend down like a typical clitoris but conform instead to the linear shape typical of males is not discussed in Western medical literature, with its obsession with penises and general disinterest in clitori. I consider it extremely likely that this unnamed counterpart to chordee does occur.
“Diphallia”
Section 2 of the embryonic genitalia is generally expected to fuse into a single penile shaft in male development, or to spread apart to form the two clitoral crura around the labia majora in female development. If the genitals devleop along male lines but the two sides do not fuse, the individual is born with two separate phalloclitori, side by side, each associated with one testis and having only one corpus cavernosum. Doctors remove one of the phalli (the one deemed smaller, no surprise there), though as in clitoromegaly there seems to be no functional danger involved in having two clitorophalli. This gential configuration can be associated with actual functional problems like an imperforate anus, obviously a true surgical emergency, but constructing an anus has nothing to do with removing half of the phalloclitoris. Doctors do not deem diphallia an intersex condition—the off-the-cuff reading is that the child is “doubly male”--but in fact the clitorophallus has developed in a manner intermediate between male and female norms.
A rarer bodily form than diphallia is phalloclitoral duplication, in which the embryo begins to twin in the genital region but ceases there—similar to what happens in the case of conjoined twins or people born with three legs. The individual is born with two penises or clitori, which may be located side-by-side or one above the other.
“Hypospadias”
Physical statuses in which a child develops external testes while Sections 2 and 3 of the phalloclitoris develop atypically are grouped together under the medical term “hypospadias.” Segment 3 of the embryonic genitalia forms the urethra and vagina, if any. In the normative male configuration, there is a urethral opening at the tip of phalloclitoris, and no vaginal opening. In individuals with hypospadias, the urethral opening is closer to the typically female location, and there may be some vaginal tissue. Individuals born with hypospadias in the U.S. today are almost always assigned male, and doctors rarely call them intersex. This is an ideological choice rather than one driven by anatomical logic. The medical belief seems to be that if a child has external testes and the clitorophallus can be surgically reconstructed along penile lines, then the child should be assigned male and no question ever raised in the parents' minds about the child having an intersex status. Doctors believe being seen as less than “fully male” is untenable for a man.
The degree of difference between typical male morphology and the genital arrangement of individuals with hypospadias varies widely. In many, it is simply a displacement of the urinary meatus from the very tip of the penis, as shown in the first illustration above, so that the urinary orifice is located lower on the phallic head, which is of ordinary penile size. Doctors “correct” this in childhood, claiming that having a “displaced” urinary meatus is unacceptable, as it will lead to teasing, and ostensibly problems with urinating in a standing position and fertility. Loss of sensation in the head of the penis, fistulas, and problems with recurrent bladder infections are deemed a better outcome by doctors than perhaps needing to sit down to pee—though in adulthood, many who have had this surgery complain that the side effects outweigh any benefits in their lives. The idea that fertility is impaired by having semen emitted from a position slightly lower down on the penis is laughable.
Hypospadias is measured by doctors in degrees. The greater the degree, the more the phalloclitoris assumes a vaginal configuration. The urinary outlet takes the shape of a small vaginal slit if located further from the head of the phalloclitoris, becoming larger if located further down the shaft, as in the second illustration above. If the urethral opening is located at the base of the phalloclitoris, the condition is termed “perineal hypospadias.” In people born with this configuration, the genitalia appear intermediate between the female norm and the male, with a vagina located in front of or between the labioscrota. Testes are located in the labioscrotum, with surface skin that can appear more close to typical labia majora or to scrotal skin. The clitorophallus is often intermediate in size and the head may bend down in the typical clitoral configuration called chordee. While children with “perineal hypospadias with chordee” have genitalia that look closer to the female norm than the male, they still may not be classified as “officially” intersex by American doctors, and surgery that closes their vaginas, dissects the clitorophallus from the perineum, and repositions the urethra to the head of the clitorophallus is termed a “repair” rather than sex assignment surgery. Such extensive surgery is painful, life-altering, and usually leads to loss of sensation. Furthermore, a substantial number of people born with this intermediate configuration grow up to identify as female, despite their infant surgical sex assignment to male, and bitterly resent having been given surgery that removed their vaginal tissue while forming their phalloclitori into the sensation-impaired semblance of a penis.
Rarely mentioned by doctors in articles discussing hypospadias is that it can be accompanied by intermediate internal sex structures, particularly a large “prostatic utricle”. (The embryonic structure that typically develops into a uterus in more female bodies forms a small “utricle” in the center of the prostate in bodies that are typically male.) In intersex bodies, this may exist as a small or average sized uterine structure within or aside a prostate—the greater the degree of the hypospadias, the more likely there is a utricle, and the larger it is likely to be. It fascinates me that the fact that people with hypospadias often have a uterine structure, evident in any literature search on the prostatic utricle, is rarely mentioned in medical descriptions of hypospadias, while much rarer associations between intersex conditions and cancer are often mentioned in articles on other intersex conditions. I believe it is not mentioned because discussing a uterine structure would undermine the medical framing of children with hypospadias as “boys with a penile malformation” rather than as intersex children.
"Vaginal Agenesis"
In some individuals, external genitalia are formed which appear close to the female side of the spectrum, but Section 3 only creates a shallow vagina or smooth patch of lubricating skin. Internally, such children may have no gonads, or may have ovaries but no uterus, or may have ovaries and an atypical uterus. Individuals with vaginal agenesis are always called female rather than intersex by doctors, even when they have no gonads and will develop no secondary sexual characteristics (such as breasts or facial hair absence/presence) without taking hormone medications. Again, Western medical ideology seeks to define away intersexuality as much as possible.
There is a lot of attention given by doctors to the creation of a vagina for children with genitals that otherwise appear female to them. This is framed as necessary for “sexual functioning,” presuming that forms of sexual activity other than penetration of a vagina by a penis are “not really sex.” As is the case with many intersex bodies, surgeries are often performed which sacrifice the capacity for sexual sensation out of an ideology that this is necessary for “normal sex.”
“Femal
e Pseudohermaphroditism”/ Congenital Adrenal Hyperplasia
In some intersex conditions, the four zones of the external genitalia develop so that they look typically male (with urethral opening at the tip of the penis, scrotum, and no vaginal opening) but the individual possesses a uterus and ovaries, and the scrotum is empty. The most common diagnosis in people with such a bodily form is congenital adrenal hyperplasia or CAH in XX individuals. While most any person on the street would say that having both a penis and a uterus is an intersex bodily form, doctors hem and haw, and say instead that the child, while intersex, is a “pseudohermaphrodite,” somehow really female. This is based on the move by doctors almost a century ago to define intersexuality out of existence by saying that only individuals with the very rare condition of having one ovary and one testis, or having intermediate ovotestes, are “true hermpahrodites.” An intersex person with testes was deemed “really male” and anyone with ovaries “really female” by the creation of the term “pseudohermaphrodite.”
At the time doctors came up with the idea of the “pseudohermaphrodite,” sex assignment surgery had not yet been developed. Today, however, doctors insist that babies with CAH should be surgically assigned female in infancy. The language of “female pseudohermphroditism” is used to sooth parents who are shocked at the idea of a doctor cutting off their baby's penis. Doctors tell them that it is not “really” a penis, but is “really a clitoris” that is malformed. The fact, of course, is that all babies have phalloclitori—and that their baby's is exactly like any other typical boy's penis. If doctors were consistent, they'd have to call all men's phalli “malformed clitori.”
In any case, doctors in the U.S. routinely perform what they term “clitoral reduction” on children with CAH—that is, removal of almost all of the phallus--and cut apart the scrotum to give it the form of labia majora. In pressing this surgical sex assignment plan, doctors present parents with an odd assessment of the risks and benefits of such a course of intervention. They gloss over the fact that cutting off most of the phallus seriously impacts adult sexual sensation. They tell parents that this must be done to avoid the catastrophe of adult menstruation through the phallus. (Note that they do not inform parents of children with perineal hypospadias that menstruation is a “danger,” or suggest that children with hypospadias be assigned female to avoid penile menstruation.) Doctors do not inform parents that an alternative would be hormone treatment to suppress menstruation, or that their children could grow up to identify as men and function sexually as males, albeit without semen production. (Some ejaculation could be possible, but it would not contain sperm.) Rather than warn parents that many children with CAH grow up not to identify as female and to despair over having been effectively castrated, they warn that the children “have a heightened risk of lesbianism,” which is an eye-goggling assertion that is both homophobic and ignores the issue of gender identity.
“Male Pseudohermaphroditism”/ Androgen Insensitivity Syndrome
Children with complete androgen insensitivity syndrome or CAIS are the counterparts to XX CAH children. Their external genitalia take the typical female form, but internally they lack a uterus, and in the place where one would ordinarily find ovaries, they have internal testes. Because their bodies do not respond to testosterone, they grow up to develop very feminine secondary sexual characteristics at puberty, though they will never have menstrual periods. Despite their typically-female appearance, doctors call these individuals “male pseudohermaphrodites” because they have testes. However, in contrast to the treatment of children with CAH, doctors do not go on to say that they CAIS children have “malformed penises” that must be surgically altered to fit their “true sex.” Instead of urging genital reconstruction, they tell parents to raise their CAIS children as girls, warn parents that their internal testes could possibly present a risk of cancer, and tell them to have the testes removed.
Unlike children with CAH, who often regret their sex assignment surgeries, individuals with CAIS seem to usually accept having been assigned female at birth. This is probably because of the contrast in the intersex individuals' experiences. Children with CAH are assigned female at birth via traumatic, scarring surgeries that impair sexual sensation, and then must take testosterone-suppressing drugs for life, while those with CAIS may not find out about their condition until puberty, retain uninjured and unaltered genitalia, and take no hormone-suppressant drugs. Nevertheless, despite typically identifying as female, these individuals are termed “male pseudohermaphrodites” on all of their medical records, and must live with the consequences of being deemed medically male throughout their romantic and sexual lives.
Some children have Partial Androgen Insensitivity Syndrome or PAIS. They are born with a wide range of phalloclitoral forms, from looking quite close to the male iconic form, to forms like that illustrated under “perineal hypospadias with chordee,” to looking typically female. Most have an intermediate form and are given childhood sex assignment surgery to one dyadic norm or the other. As usual, such surgery is traumatic, scarring, does not result in genitalia of fully “normal” appearance, and puts sexual sensation at serious risk. This probably explains why a third to half of individuals with PAIS grow up not to live as the sex they were assigned, while 80% of individuals with CAIS identify as “fully female.”
Those Not Pictured
Many bodies vary from sex-dyadic assumptions in ways that are not visible externally, so that they are rarely diagnosed at birth, such as variations in the sex chromosomes. We are told that “men are XY and women are XX,” but there are XX men and XY women who are not visibly distinct in their bodily forms from those with typical chromosomes. There are many individuals with XXY chromosomes, termed Kleinfelter's syndrome, with a typical male genital configuration but small testes—about 1 in 500 of people raised male turn out to have this intersex karyotype. People often only discover they are XXY when undergoing tests due to infertility, or sometimes in cases where they develop substantial breasts (“gynecomastia”). Another fairly common genetic variation is to just have a single X chromosome with no second sex chromosome at all, which doctors term Turner Syndrome. Having only 45 chromosomes instead of the usual 46 is associated with a host of physical problems, and the fact that the individual's gonads never develop is treated as secondary to the many physical and mental challenges the individual faces.
Other intersex conditions exist on a more macro level than tiny chromosomes, but are internal and so may go undiagnosed for years or for an individual's entire life. Included among these, ironically, are the only conditions deemed to constitute “true hermaphroditism” under medical taxonomies: the presence of an intermediate ovotestis, or even more rarely, of an ovary and a testis in the same person. I'll write more about “true hermaphroditism” in a later post.
Also not pictured are the bodies of people with an atypical sex steroid balance between the feminizing hormones (estrogens, progesterone, etc.) and masculinizing hormones (testosterone and its byproducts). Everyone produces all of these hormones, and requires both types for fertility and physical health, but those with bodies on the female size of the spectrum typically produce more feminizing hormones, and those with bodies on the male side typically produce more masculinizing hormones. Variations in this balance lead people with typically-female genitals to have higher levels of body and facial hair, muscle mass, likelihood of balding, and libido, and people with typically-male genitals to develop breast tissue, more curvaceous hips, etc. These variations are not termed intersex by doctors, but there is no logical reason why they should not be. Their intersex character is denied because most adults with such conditions have normative gender identities that match their genitals but are challenged by their contrasting secondary sexual characteristics. They and doctors together strongly assert that their variations do not make them any less male or female. While I agree that no one's gender identity should be deemed undermined by their physical appearance, I believe it would help all sex and gender variant individuals if society and medicine would acknowledge the prevalence of physical sex variance while supporting individuals in their gender identity assertions. Some intersex activists disagree, wishing to limit the conditions that will make a person “count” as intersex, and patrolling the boundaries of the community to exclude others as “wannabes.” Personally, I find this cruel and counterproductive. A woman with a beard lives a life in which her sexvariance is very visible, and saying she can't be included in a community of those with sexvariant bodies because she has typically-female genitalia does not make sense to me.
Another category of hormone-related variance includes individuals who produce low levels of sex steroids and whose bodies do not change much at the usual age of puberty. Such individuals are almost always treated with sex steroid therapy, without presenting them with the option of living in their androgynous bodies medically unaltered.
Finally, let me note that this catalog of intermediate bodily forms is not exhaustive. In my understanding, anyone whose body varies from the iconic male or female dyadic norms is sex variant, cannot be wished out of the intersex rubric by tricks of medical terminology, and should not be excluded from intersex community by gatekeepers.
We need society, the medical field, and intersex communities themselves to acknowledge that nature provides humanity with a wide range of forms, so that all of our bodies can be recognized as valid. Unless there is an actual rare functional problem, our bodies should not be altered in infancy, and only those functional problems should be addressed. Our genitals should be altered only if we ourselves request it, to make ourselves comfortable in our own skin, not to make society comfortable by our medical erasure. Society must come again to embrace the diversity that is nature's gift to us.
