In this article, I provide a critical, feminist account of the medical management of intersex conditions under the controversial “Disorders of Sex Development” (DSD) treatment model introduced in 2006. I argue that just like the “Optimal Gender of Rearing” (OGR) treatment model that it replaced, DSD aims at securing a specifically cisgendered future for the intersex patient, and is thus, practically speaking, a suite of medical interventions aimed at the clinical production of cisgendered lives. By cisgendered future I mean something far more expansive than one in which individuals identify with the sex they were assigned at birth. Rather, I use this term to refer to a normalized trajectory of development across the lifespan in which multiple sexed, gendered, and sexual characteristics remain in dynamic but “coherent” alignment. These variables include primary sex characteristics (specifically, external genitalia), secondary sex characteristics (from the development of breasts, to sex‐typical fat distribution, to hair‐growth patterns), gendered characteristics including identity, behavior or role, patterns of cognition, nonsexual gendered desires (such as toy and occupation preference), as well as sexuality. I use the term cisgendered rather than cisgender specifically to: 1) differentiate my critical description from self‐identification as cisgender, transgender, or nonbinary, and so on, and 2) to emphasize the discursive and material constitution of this trajectory of development as cisgendered via the treatment model, insofar as it presumes the normative and descriptive normalcy of cisgendered lives and materially normalizes individuals on the basis of that presumption. In this sense, contemporary intersex management both reifies the normalcy of cisgendered life and materially constitutes it as such.Footnote ¹ Further, in the cases under consideration here, it does all of this without the patient's informed consent.

To make this argument, I critically analyze two ways in which intersex management has changed between OGR and DSD: 1) with regard to sex‐assignment recommendations for three patient populations and 2) with the introduction of prenatal treatments for pregnant individuals identified as at risk of conceiving a 46 XX fetus with congenital adrenal hyperplasia with the steroid hormone dexamethasone (a practice often referred to as “fetal dex”). On the basis of this analysis, I derive a series of conclusions. First, although DSD inherits from OGR both its aim and the empirically unjustified assumption that typical genitals are necessary for “normal” cisgendered development, it is underwritten by a different model of gender development (Creighton et al. Reference Creighton, Michala, Mushtaq and Yaron2014). Specifically, unlike OGR, which was practically dominated by a social‐constructionist model of gender development, DSD is practically dominated by a biologically determinist model known as brain‐organization theory, which holds that brains are “organized” into feminine or masculine patterns by prenatal hormone exposure. Brain‐organization theory has received a good deal of critical attention in the last decade or so, pointing to many ontological and empirical reasons for skepticism (Reference FineFine 2010; Jordan‐Young Reference Jordan‐Young2010).

Thus, I conclude that just like OGR before it, DSD suffers from a complex, theoretically suspect, and empirically inadequate account of gender development as its foundation. As a result, clinicians are just as hamstrung under DSD with regard to providing “evidence‐based recommendations” regarding not only which sex to assign, but also how to assign it in terms of which surgical procedures to use, and their timing (Reference Houk and LeeHouk and Lee 2010, 4506; Creighton et al. Reference Creighton, Chernausek, Romao, Ransley and Salle2012; Lee et al. Reference Lee, Schober, Nordenström, Hoebeke, Houk, Looijenga, Manzoni, Reiner and Woodhouse2012; Creighton et al. Reference Creighton, Michala, Mushtaq and Yaron2014). Further, this means that the primary justifications for intersex management under DSD remains social rather than based on clinical evidence about human biological function. Clinical treatment of intersex patients thus continues to be underwritten by problematically heterosexist and transphobic assumptions regarding the value and normalcy of cisgendered life—without consideration of the ways in which this suite of therapies itself contributes to the reification of cisgendered life as normal and valuable (bolstering transphobia and heterosexism in the process). In this sense, the very same norms cited as demanding interventions like fetal dex and sex assignment are affected through their practical constraint on the range of sexed, gendered, and sexual futures available to those with intersex conditions.

From “Optimal Gender of Rearing” to “Disorders of Sex Development”

In 2006, the American and European pediatric endocrine associations published a special article titled “Consensus Statement on Management of Intersex Disorders” (hereafter Consensus Statement) proposing the first official revision in both the treatment model and the system of nomenclature for these conditions since John Money and his colleagues Joan and John Hampson introduced their “Optimal Gender of Rearing” (OGR) treatment model in the 1950s (Lee et al. Reference Lee, Houk, Ahmed and Hughes2006). Underwriting OGR was Money's complex account of gender development, which held that “variables of sex” such as “chromosomal sex, gonadal sex, internal and external morphologic sex and hormonal sex (prenatal and pubertal)” could be independent of one another (Reference MoneyMoney 1995, 21).Footnote ² For Money, the development of gender was a “multistage process that relied on multiple attributes of biological sex and social variables but that could not be said to derive from these exclusively” (Reference KarkazisKarkazis 2008, 53). However, for a large portion (95%) of patients studied by Money and his colleagues at Johns Hopkins in the early 1950s, gender identity corresponded to sex of rearing—or rather, gender socialization (Reference RubinRubin 2012, 888). This led Money and the Hampsons to identify sex of rearing as being of particular importance to gender development—perhaps even more so than biological variables given the stability of gender once learned (Reference KarkazisKarkazis 2008, 52–54). Further, Money and his colleagues saw normalized genitalia as key to gender development—despite accumulating a good deal of contradictory evidence from the patients they were studying.Footnote ³ Genitals that did not match a child's assigned sex would impede the child's identification with their assigned sex and gender and, further, might cause parents to “waver in their commitment to raising the child in the assigned gender” (57–58). The prioritization of sex of rearing over other variables in sex‐assignment—including those of biological sex—and the importance placed on normalized genitalia for both the child being socialized and those doing the gendered socialization meant that Money's complex account of gender development boiled down to a social‐constructionist model organized around what intersex scholar Morgan Holmes refers to as Money's “genital determinism” (Reference HolmesHolmes 2008, 69; Reference KarkazisKarkazis 2008, 52–54).

By the time DSD was introduced in 2006, the ethics, politics, and science underlying the OGR treatment model had been the subject of almost twenty years of intense, often scathing critique by both feminist academics and intersex activists (Reference KesslerKessler 1990; Fausto‐Sterling Reference Fausto‐Sterling1993; Reference ChaseChase 1999; Fausto‐Sterling Reference Fausto‐Sterling2000; Reference Chase, Stryker and WhittleChase 2013).Footnote ⁴ Critics from these intimately entangled communities began raising concerns in the early 1990s, focusing in particular on the performance of medically unnecessary genital normalizing surgeries that were (and remain) generally accompanied by sterilizing gonadectomies, on infants and children unable to provide informed consent—often at the expense of later sexual function. They questioned the positioning of the medical treatment of intersex conditions as exceptional, legitimating practices that routinely violated bioethical principles of respect for autonomy, beneficence, nonmalfeasance, and justice. They pointed out that these surgeries were (and remain) necessarily experimental given that the kind of clinical trials that might provide quality evidence regarding the efficacy of the techniques involved and their ideal timing—trials that would involve randomly assigning medically unnecessary genital surgeries, performed on differing schedules to infants—are ethically prohibited. Furthermore, they called into question the presumed naturalness of physical sex dimorphism that constituted intersex conditions as pathologies requiring treatment, as well as the heterosexism underlying treatment recommendations and outcome analyses that valued “aggressiveness and sexual potency for boys and passiveness and reproductive/sexual‐receptive potential for girls” and according to which homo or bisexuality and instability of gender identification constituted bad outcomes (Reference Dreger and HerndonDreger and Herndon 2009, 204).

Both feminist academics and activists (particularly those associated with the Intersex Society of North America [ISNA]) often forwarded a type of natural‐variation account regarding intersex bodies, based fundamentally on the notion that “although certain intersex‐related conditions (such as salt‐[wasting] associated with Congenital Adrenal Hyperplasia) can be life threatening and thus may require intervention, intersexuality itself is not pathological and thus does not require medical treatment” (Reference Spurgas and HolmesSpurgas 2009, 99). It was because intersex conditions generally, and ambiguous genitalia in particular, threatened social norms about sex, gender, and sexuality that structure so much of daily existence that they were pathologized, they argued, and this pathologization justified their treatment with unsuccessful, experimental surgeries and secrecy, causing immense physical and psychological harm.

The Consensus Statement cites progress made in “diagnosis, surgical techniques, understanding psychosocial issues, and [in] recognizing and accepting the place of patient advocacy” as motivating the revision in treatment model and nomenclature it lays out (Lee et al. Reference Lee, Houk, Ahmed and Hughes2006, e488). It reclassifies intersex conditions under the umbrella category “disorders of sex development,” and assigns a DSD‐specific diagnosis (to be used in conjunction with one's condition‐specific diagnosis) that references one's total chromosome number, specific “sex chromosome” makeup, and, in some cases, gonadal makeup (for example, an individual with Complete Androgen Insensitivity Syndrome (CAIS) would now also receive the diagnosis 46 XY testicular DSD). Beyond the shifts in sex‐assignment recommendations I explore below, the treatment model outlined within the Consensus Statement is not that different substantively from OGR. Its surgery recommendations, however, are certainly the most conservative ever issued publicly by a group of clinicians, stating that surgery should be limited to “cases of severe virilization,” that functional outcomes be emphasized over aesthetic, and cautioning that although “it is generally felt that surgery that is performed for cosmetic reasons in the first year of life relieves parental distress and improves attachment between the child and the parents; the systematic evidence for this belief is lacking” (Lee et al. Reference Lee, Houk, Ahmed and Hughes2006, e491).Footnote ⁵

Clinical adoption of the DSD treatment model among pediatric specialists in intersex management internationally was so rapid and so near total that one of the Consensus Statement co‐authors characterized its swift usurping of OGR as “a quiet revolution in medicine” only four years after its introduction (Reference HughesHughes 2010, 160). However, it is now clear that DSD has not brought about a reduction in the frequency with which genital normalizing surgeries are performed on infants unable to provide informed consent, despite the Consensus Statement's call for moderation. Clinical experts in the field, as well as researchers in the humanities drawing on interviews with clinical experts, have found that surgery continues to be positioned as a necessary part of optimal care under DSD, and is performed as frequently as it was before, and may even have increased (see Reference KarkazisKarkazis 2008; Creighton et al. Reference Creighton, Michala, Mushtaq and Yaron2014; Reference FederFeder 2014; Reference DavisDavis 2015; Reference CraryCrary 2017; Reference KirklandKirkland 2017).Footnote ⁶

Despite the Consensus Statement's qualifiers regarding the lack of evidence for surgery, it does nothing to dispute the notion that typical genitalia are in fact essential for normal gender development. It makes clear that genitalia cannot be too atypical, or “severely viriliz[ed]”—however that line is drawn—as surgery is still recommended in these cases, despite the acknowledged lack of evidence. Moreover, it does nothing to disrupt the pursuit of cisgendered life as the primary aim of this suite of interventions.

Thus the primary aim of intersex management and the presumption that typical genitalia are essential to its achievement have not changed between OGR and DSD (as attested to by the stubborn persistence of genital normalizing). However, the model of gender development concretized through and guiding the use of the suite of interventions encapsulated by the treatment model has. Instead of the social‐constructionist model distilled out of Money's more complex account, clinicians now turn to the biologically determinist account known as brain‐organization theory in order to predict gender under DSD. In the following two sections, I critically analyze two ways in which intersex management has changed between OGR and DSD—with regard to sex assignment for three patient populations and with the introduction of fetal dex—and show that both of these changes are justified via appeal to brain‐organization theory. Moreover, in addition to providing further evidence for my account of cisgendered futures, I detail the effects of this shift in model in terms of its reprioritization of physical sex characteristics in predicting gender, and the larger reconstitution of biomedical definitions of normal sex and gender that it achieves.Footnote ⁷

Shifts in Sex Assignment Recommendations

There are clear indications within the clinical literature of shifts in recommendations regarding sex assignment under DSD for three particular patient populations.Footnote ⁸ The first group is those patients with 46 XY cloacal exstrophy, a condition associated with the protrusion of the abdominal organs through the abdominal wall such that infants can be born with their intestines and bladder exposed (Meyer‐Bahlburg Reference Meyer‐Bahlburg2005). The second are those 46 XY patients with micropenis—defined as a penis smaller than 2.5 standard deviations below the mean—that is specifically the result of nonhormonal factors (Hatipoğlu and Kurtoğlu Reference Hatipoğlu and Kurtoğlu2013, 217).Footnote ⁹ Finally, the third group is those 46 XX patients with congenital adrenal hyperplasia (CAH), whose genitalia are considered to be “severely masculinized” (Reference Houk and LeeHouk and Lee 2010). CAH is the most common intersex condition (excluding hypospadias), and is associated with the decrease or complete loss of activity of an enzyme involved in steroidogenesis, the endocrine cascade or multi‐step set of processes via which cholesterol is converted by enzymes into biologically active hormones such as cortisol, androgens, and estrogens (Reference RoughgardenRoughgarden 2004; Reference Miller and AuchusMiller and Auchus 2011). This enzyme deficiency leads to the accumulation of substrates that are then converted to androgens by active enzymes in the environment, leading to an increase in androgen production and the “masculinization” of XX individuals over the lifespan, beginning in utero (Reference Miller and AuchusMiller and Auchus 2011).

Whereas OGR recommended female sex assignment for all three of these patient populations, clinicians increasingly advocate for assigning all of these groups male under DSD, specifically via appeals to brain‐organization theory. The rationale for assigning the first two groups—46 XY patients with cloacal exstrophy and those with micropenis—female under OGR had to do with the limitations of phalloplasty techniques, the social‐constructionist model of gender development underwriting OGR, as well as heteronormative assumptions regarding both masculinity and femininity. It often was—and remains—too difficult to build a penis capable of “voiding in a standing position and achieving straight erections and proper ejaculation that will permit suitable sexual intercourse” (Creighton et al. Reference Creighton, Chernausek, Romao, Ransley and Salle2012, 603). It is comparatively much easier, however, to create an aesthetically typical vagina capable of penetration (if not sensation) (603). Thus, heteronormative assumptions regarding the capacities necessary to normal cisgendered life as either a man or a woman and technological limitations to actualizing those capacities—which, importantly, are rendered limitations only by those heteronormative assumptions—led clinicians to default toward female assignment for these patients. This makes sense when assumptions and technological capacities are read against the social‐constructionist model of gender development that presumes the necessity of typical genitalia for normal cisgendered development, and prioritizes gender socialization over all other contributing variables. With regard to the third patient population—“highly masculinized” 46 XX patients with CAH—their XX chromosomes coupled with the relative ease of vaginoplasty meant that clinicians saw themselves as repairing these latter patients’ bodies rather than changing them in some way; surgery and hormone therapy for these patients restored them to being the girls that they both should have been, and really were, all along (see Reference KarkazisKarkazis 2008, 106–08).

Under DSD, however, there has been a clear shift in clinical practice to assign 46 XY patients male. As 2016's “Global Disorder of Sex Development Update since 2006: Perceptions, Approach and Care” (Global Update hereafter) clearly indicates,

It goes on to explicitly invoke brain‐organization theory in warning clinicians amid this “trend to assign most 46, XY DSD patients as male” that “individualized caution must be taken with male assignment based on evidence of androgen responsiveness and CNS androgen exposure during fetal life [emphasis added]” (169). The implication here is that 46 XY patients with atypical genitalia should receive male assignment only if there is evidence of “typical” androgen exposure (and response) in utero (that is, their underlying etiology is not hormonal in nature). Clinicians are being cautioned not to let themselves get carried away amid this “trend,” and assign as male any 46 XY infants who do not exhibit evidence of being responsive to androgens and having been exposed to it in utero.

Given that the technical limitations plaguing phalloplasty under OGR persist under DSD, one could interpret the move toward male assignment for 46 XY patients with cloacal exstrophy and micropenis as the expansion of those futures constituted as normally cisgendered under the new treatment model (as one reviewer suggested). Indeed, we might read this as an easing of unjustified assumptions regarding the necessity of typical genitalia to being a “normal” boy or man, or of heteronormative assumptions regarding what capacities genitalia must have to be valued, and/or considered typical. Although such an expansion might be an unintended side effect of this shift in clinical practice, it is not the primary intention. Indeed, heteronormative concerns persist under DSD; for example, Global Update advises that “for those with 46, XY and a micropenis, male assignment is preferable for most regardless of penis size except for those with partial [androgen insensitivity syndrome]” (Lee et al. Reference Lee, Nordenström, Houk and Faisal Ahmed2016, 169). Nonetheless, it goes on to note that “the issue of penile length persists” in managing 46 XY patients with micropenis, as many patients will have “insufficient penile length to achieve penetrative intercourse” (169). Further, it emphasizes the importance of considerations regarding penile length and the capacity for penetrative intercourse in deciding how to treat these patients—ostensibly with regard to sex assignment—cautioning that “[i]t is clear that the same care plan cannot be applied to all individuals with a given diagnosis/syndrome and severity,” in light of these issues (169). Beyond this, the fact that the general frequency with which genital normalization is performed has persisted (if not increased) under DSD indicates that the presumption that typical genitalia are essential to the development of normal cisgendered life continues to hold sway in general—it is only members of these populations who seem to avoid the knife.

The apparent relaxing of norms regarding the necessity of typical genitalia for these patients is the result of the discursive and practical ascension of brain‐organization theory as the dominant model of gender development within DSD. Brain‐organization theory presumes that the well‐established sex‐differentiating effects of hormones on genitalia are mirrored within the brain, giving rise to “masculinized” or “feminized” brains. As reflected in my account of cisgendered futures, the masculinizing and feminizing effects of hormones posited by the theory are quite expansive. Indeed, as Rebecca Jordan‐Young writes in Brain Storm: The Flaws in the Science of Sex Differences, brain‐organization theory presumes that hormone exposure generates neurological “permanent masculine or feminine patterns of desire, personality, temperament, and cognition” (Jordan‐Young Reference Jordan‐Young2010, xi). Hormones are thus responsible for not only the development of one's identity as cisgender (or transgender, or nonbinary, and so on), but also “a very wide range of differences related to gender and sexuality—in humans these include everything from spatial relations, verbal ability, or math aptitude, to a tendency to display nurturing behaviors, to sexual orientation” (21–22).

As a result, although typical genitalia—and physicians’ capacity to produce them given an infant's genital tissue and the surgical techniques available—are still presumed to be an important component to a cisgendered future, prenatal hormone exposure and receptivity come to take priority as the most important characteristics to consider in sex assignment under DSD. This is why the Global Update's recommendations make a distinction between those who have hormonal etiologies and with “nonhormonal genital malformations such as cloacal exstrophy of the bladder” and specifies that physicians are only more likely to suggest male assignment for 46 XY patients if they have a nonhormonal etiology that indicates “normal‐male prenatal androgen levels” (Lee et al. Reference Lee, Nordenström, Houk and Faisal Ahmed2016, 169). Cloacal exstrophy is an excellent example of a nonhormonal intersex condition and is included under the umbrella category of intersex conditions only because the condition frequently involves atypical development of the urogenitals (often in a “bifid” manner where they are split into two equal parts) (Meyer‐Bahlburg Reference Meyer‐Bahlburg2005). The Consensus Statement equivocates on the issue of treating these patients, simply noting that those “reared female show variability in gender identity outcome, but >65% seem to live as female”—a statistic that does not imply practice would or should move away from (already normative) female assignment (Lee et al. Reference Lee, Nordenström, Houk and Faisal Ahmed2016, e491). However, Valerie Arboleda, David Sandberg, and Eric Vilain point to a 2004 report of “self‐initiated gender‐change in a cohort of patients with 46 XY cloacal exstrophy” as initiating a shift in pediatric urologists’ recommendations regarding sex assignment, citing a 2011 survey of American pediatric urologists in which 79% of respondents recommended male assignments for 46 XY cloacal exstrophy, “with 97% identifying ‘brain imprinting’ by prenatal androgens [emphasis added] as an important factor in their decision” (Reference Arboleda, Sandberg and VilainArboleda, Sandberg, and Vilain 2014, 611). This is also why the Global Update specifically excludes those with partial androgen insensitivity syndrome from its recommendation that all 46 XY patients with micropenis be assigned male, “regardless of penis size,” and cautions that physicians assign such patients male based only on “evidence of androgen responsiveness and CNS androgen exposure during fetal life” (Lee et al. Reference Lee, Nordenström, Houk and Faisal Ahmed2016, 169). Those with hormonal etiologies (like partial androgen insensitivity syndrome) cannot be presumed to have had typical androgen exposure or responsiveness during fetal life, raising questions about the masculinization of their brains, and thus clinicians’ ability to successfully secure a masculinely cisgendered future for them in my more expansive sense.

In this way, a reconstitution of the range of gendered life normalized as cisgendered has been achieved, and formally codified through DSD. The dominance of brain‐organization theory within the model and the wide range of physical and behavioral effects it attributes to hormones proliferates the number of characteristics that must cohere in order for one to “count” as cisgendered beyond a mere alignment between the sex one is assigned at birth and one's gender identity—if it ever really was merely that.Footnote ¹⁰ Assigning 46 XY patients with “nonhormonal genital malformations” male under DSD does mean potentially assigning them to trajectories of development that deviate from that normalized as cisgendered—insofar as they may not ever have aesthetically or functionally typical genitalia (and hence, could be read as an expansion of the range of life constituted as normally cisgendered, just for these populations). However, genitalia are only one variable of many that makes up gendered life under DSD—and the correlation presumed by brain‐organization theory between multiple neurologically based characteristics of the gendered self (for example, identity, behavior, nonsexual desires, and sexuality) justifies prioritizing them over genitalia. Indeed, if a more global coherence among these variables is the aim, then it is better to have a typically cisgendered boy/man with slightly atypical genitalia than an atypically gendered girl/woman with aesthetically typical genitals. Who knows what surgical advances in phalloplasty might be made? Further, these patients’ presumed capacity to produce and respond to typical levels of androgens ensures their future development of “coherent” secondary sex characteristics—development that could always be supplemented with hormone replacement therapy if needed. In this sense, although the range of life constituted as normally gendered might have expanded somewhat for those 46 XY patients with nonhormonal etiologies under DSD, it has formally narrowed in general under DSD, insofar as it normalizes a high level of coherence across a wide range of sex, gendered, and sexual variables. Additionally, the treatment model places greater trust in advances in knowledge‐production, and the technical capacity to normalize the body, than in sociopolitical capacity to accept a range of sexed and gendered life—something noted by Ellen Feder (Reference FederFeder 2014).

The dominance of brain‐organization theory under DSD means that those 46 XY patients with atypical genitalia, but presumably typical prenatal androgen exposure/receptivity, now receive male rather than female assignment on the basis of their (presumably) masculinized brains. It also means that the third patient population—those 46 XX CAH patients with “severely masculinized” genitals—now receive male rather than female assignment, also on the basis of their (presumably) masculinized brains, as indicated by their physically masculinized genitalia. This is why, despite the Consensus Statement's replication of OGR's recommendation that “markedly masculinized 46 XX CAH infants be assigned female,” there is a good deal of evidence to suggest that clinical practice leans toward male assignment (Lee et al. Reference Lee, Nordenström, Houk and Faisal Ahmed2016, e491). In “Approach to Assigning Gender in 46 XX Congenital Adrenal Hyperplasia with Male External Genitalia: Replacing Dogmatism with Pragmatism,” Consensus Statement co‐authors Christopher Houk and Peter Lee note that “gender assignment in contemporary DSD management encourages consideration of multiple factors … recognizing that some degree of gender predetermination may have taken place in some types of patients,” before going on to argue specifically for male assignment for these patients (Reference Houk and LeeHouk and Lee 2010, 4502). Though they acknowledge that outcome evidence for their proposal “is incomplete,” they argue there are nonetheless “several lines of evidence which support it” (4503). In addition to citing studies showing that there appears “to be a high risk of gender dysphoria” in markedly masculinized patients assigned female “regardless of karyotype,” they also note “additional, albeit indirect support” from a single study revealing gender transition (if not dysphoria) to be relatively rare among the few (33) markedly masculinized XX individuals assigned male who have been studied (4503–04). Finally, they identify the avoidance of genital surgery and “the irrevocable loss of sensitive genital tissue” as an additional benefit of male assignment in these cases, pointing out that it will “offer more options for the adult DSD patient who would logically have a better outcome with surgical reassignment from male to female than vice versa” (4504).

Equivocating evidence, avoidance of surgery (and maintaining options via this), as well as avoiding dysphoria seem like prima facie good reasons for male assignment in these cases—assuming that male assignment here does not involve surgical interventions of any kind (such as phalloplasty). These are good reasons for avoiding surgery for all intersex patients. However, Houk and Lee invoke brain‐organization theory both implicitly and explicitly in their arguments for assigning severely masculinized 46 XX patients male. Their reference to a high risk of gender dysphoria for markedly masculinized patients assigned female “regardless of karyotype” (that is, “sex chromosomes”), for example, is a thinly veiled appeal to brain‐organization theory and its assumption that the developmental effects of hormones on the genitals is mirrored in the brain. The prenatal androgen exposure responsible for the “marked masculin[ization]” of these patients is presumed to have similarly masculinized their brains, such that they are at a high risk of gender dysphoria—despite their XX chromosomes. Thus, the hormonal masculinization of the gendered self can be in a sense read through the severity of the masculinization of the genitalia—hence their specification that only “markedly masculinized” 46 XX CAH patients be assigned male. More explicitly, Houk and Lee identify “androgen‐induced masculinization of the fetal brain” as appearing to be “important in the DSD patient exposed to male typical levels of testosterone during fetal life, including masculinized 46, XX DSD patients in whom such exposure produced male genitalia” (Reference Houk and LeeHouk and Lee 2010, 4506). Furthermore, they cite “high levels of fetal androgen exposure” as a key justification for the move to male assignment for 46 XY cloacal exstrophy patients under DSD, and argue that “similar consideration be given to the 46, XX CAH child with functional male genitalia whose existence implies a high degree of masculinization of the brain” (4506; emphasis added).

The Global Update's recommendations echo those of Houk and Lee (Lee et al. Reference Lee, Nordenström, Houk and Faisal Ahmed2016, 169, citing Reference Lee, Houk and HusmannLee, Houk, and Husmann 2010, which also argues for male assignment for these patients). Further, both Reference Houk and LeeHouk and Lee 2010 and the Global Update underscore the multiplicity of gendered and sexual variables that must align for a future to be considered properly cisgendered (beyond a mere alignment between one's sexed body and one's gender identity). For example, one study finds that highly “masculinized” CAH women were more likely than the control group to be nonheterosexual, and to have “male‐dominant occupations, a greater interest in rough sports and motor vehicles,” before concluding that “46, XX newborns with marked genital masculinization are more likely to show marked masculinization of behavior” (Lee et al. Reference Lee, Nordenström, Houk and Faisal Ahmed2016, 169).

Thus, in all three of these patient populations—46 XY patients with cloacal exstrophy, 46 XY patients with nonhormonal micropenis, and those “severely masculinized” 46 XX patients with CAH—changes in treatment are not motivated exclusively or even primarily by a desire to avoid surgery and its attendant complications, or a relaxing of heteronormative assumptions regarding genitalia, but to produce stably cisgendered subjects, and secure a future free of gender ambiguity, dysphoria, and/or transition. Prenatal administration of “fetal dex” attempts to do the same thing by intervening even earlier in the developmental process, by preventing the “masculinization” of 46 XX fetuses with CAH in utero.

Fetal Dex

Prenatal administration of dexamethasome to those identified as at risk of conceiving a fetus with CAH (either through genetic screening or having already had a child with classic CAH) for the specific purpose of preventing the “masculinization” of 46 XX CAH fetuses in utero was first introduced in France in 1978 and in the USA in 1986 by endocrinologist Maria New (Meyer‐Bahlburg et al. Reference Meyer‐Bahlburg, Dolezal, Haggerty, Silverman and New2012, 103). Alice Dreger, Ellen Feder, and Anne Tamar‐Mattis write that the practice is considered by many specialists as constituting the standard of care when treating women who might give birth to a child with CAH (Dreger, Feder, and Tamar‐Mattis Reference Dreger, Feder and Tamar‐Mattis2012, 278). A glucocorticoid steroid twenty‐five times more potent than cortisol, fetal dex will not cure CAH. Rather, the goal is specifically to prevent the overproduction of androgens in only those CAH‐affected fetuses with XX chromosomes, and thus prevent the masculinization of both their genitalia and their brains. That is, it aims at preventing the development of those “highly masculinized” 46 XX CAH infants that clinical practice is now shifting toward assigning male on the basis of that masculinization discussed above.

Because the human genitalia and reproductive structures differentiate very early on in fetal development (between seven and seventeen weeks), the masculinization process this treatment seeks to avoid begins prior to clinicians’ ability to test whether the fetus is affected—that is, before we can test whether the fetus has both XX chromosomes and CAH (Reference Warne, Grover and ZajacWarne, Grover, and Zajac 2005, 23; Reference Yiee and BaskinYiee and Baskin 2010). As a result, fetal dex must be administered to a pregnant woman as soon as she learns she is pregnant (ideally five to six weeks’ gestation) and is then discontinued if the fetus is found to have XY chromosomes or if it is found to have XX chromosomes but tests negative for CAH, which accounts for 7/8 of all cases (Reference Warne, Grover and ZajacWarne, Grover, and Zajac 2005, 23).Footnote ¹¹ Although the atypically low cortisol production associated with CAH results in the production of atypically high levels of androgens in all affected fetuses, it is not considered a problem for those XY fetuses with CAH as such exposure does not carry the risk of genital or gender ambiguity (that is, “incoherent” masculinization of the body and mind characteristic of a noncisgendered future) as it does for those with XX chromosomes. Thus, the futures of such fetuses are conceived of as already securely cisgendered. The administration of fetal dex to prevent masculinization of 46 XX CAH infants has been lauded as “an excellent example of pharmacological therapy during pregnancy” and a “paradigm of prenatal diagnosis and treatment” (Rosner et al. Reference Rosner, Shachar, Yaron and Evans2006, 803; Reference Nimkarn and NewNimkarn and New 2010, 5).

Despite this, in recent years concerns have been raised regarding the ethics of fetal dex. Because the administration of fetal dex to avoid masculinization in 46 XX fetuses with CAH is an off‐label use of the steroid, it has never been clinically trialed. Thus, despite its standardization within intersex management, and its promotion as safe (particularly to those pregnant women who receive it), clinicians have no real idea of the effects of the prenatal administration of dexamethasone—which exposes the developing fetuses to 60–100 times the normal level of glucocorticoids—on either the fetus or the mother (Dreger, Feder, and Tamar‐Mattis Reference Dreger, Feder and Tamar‐Mattis2012, 281). Fetal dex can nonetheless be administered for this use due to its categorization as a category C drug by the FDA, meaning: “[a]nimal reproduction studies have shown an adverse effect on the fetus and there are no adequate and well‐controlled studies in humans, but potential benefits”—in this case, securing a cisgendered future for an infant—“may warrant use of the drug in pregnant women despite potential risks” (FDA Pregnancy Categories—CHEMM n.d.). Indeed, animal studies, as well as retroactive studies of human children treated with fetal dex, have raised serious concerns regarding its use. These studies have reported correlations between prenatal exposure to fetal dex and increased risk for heart disease and diabetes (Kelly et al. Reference Kelly, Lewandowski, Worton, Davis, Lazdam, Francis, Neubauer, Lucas, Singhal and Leeson2012), as well as cognitive delays in both animals and humans (Dreger, Feder, and Tamar‐Mattis Reference Dreger, Feder and Tamar‐Mattis2012, 281; Hirvikoski et al. Reference Hirvikoski, Nordenström, Wedell, Ritzén and Lajic2012; Meyer‐Bahlburg et al. Reference Meyer‐Bahlburg, Dolezal, Haggerty, Silverman and New2012). In fact, there have been so many problems identified with fetal dex that American endocrinologists Walter Miller and Selma Feldman Witchel published a “clinical opinion” piece in the May 2013 issue of the American Journal of Obstetrics and Gynecology urging obstetricians to abstain from treating their eligible pregnant patients with fetal dex, and to prevent their being treated with it by other clinicians involved in their patients’ care. Miller and Witchel argue that the risks of this intervention far outweigh the benefits of “ameliorating genital virilization” for about 80–85% of affected fetuses, citing evidence from human and animal studies that show that:

Furthermore, Swedish researchers performing the only controlled and prospective (rather than retrospective) study of fetal dex to have ever occurred announced in 2012 that they stopped recruiting new patients into the study in 2010 after “severe adverse events” were noted in the treatment group on follow‐up, such as developmental delay, hydrocephalus, and severe mood fluctuations (Hirvikoski et al. Reference Hirvikoski, Nordenström, Wedell, Ritzén and Lajic2012, 1882). The Swedish team concluded that the use of fetal dex is unethical not only in daily medical practice, but even in the context of clinical trials. Given that the risks of fetal dex are inadequately understood and the fact that 7/8 of those fetuses exposed “do not benefit from the treatment per se,” they argue that it is “globally” unacceptable that fetuses at risk for CAH “are still treated prenatally with [dex] without follow‐up” (1882). Further, they stress their position that if any clinicians are going to continue to administer fetal dex for this purpose, then “the minimal requirement should be” that it occurs only in the context of clinical trials “including long‐term follow‐up of all treated individuals,” wherein parents are given “thorough information … about the potential risks and uncertainties, in addition to the benefits of this treatment” (1882). However, given the already evidenced risks, and the cessation of their own study, it is clear that even this fails to reach the standards of ethical research for this group.

One of the acknowledged goals of fetal dex is preventing the development of—and thus, avoiding the surgery to treat—anomalies of the urogenital sinus in CAH‐affected XX fetuses. Most commonly, the urethra and vagina are joined, potentially increasing the risk of repeat infections. Dreger, Feder, and Tamar‐Mattis argue, however, that this is a secondary goal, pointing to articles by New—the original and one of the primary proponents of fetal dex—to argue that the primary aim of treatment is to ensure that “CAH‐affected female fetuses … develop in a more female‐typical fashion than they otherwise might” (Dreger, Feder, and Tamar‐Mattis Reference Dreger, Feder and Tamar‐Mattis2012, 280). Fetal dex aims at securing a cisgendered future for the fetus—and at foreclosing potential noncisgendered futures—by preventing the development of both ambiguous genitalia and so‐called “behavioral masculinization” in the form of tomboyishness and lesbianism or bisexuality (280). Further, Dreger, Feder, and Tamar‐Mattis identify brain‐organization theory as playing a key role in the rationale behind the use of fetal dex, identifying its aim as “engineer[ing] the CAH‐affected female fetus's hormonal system to be typically female” in the face of concerns regarding its potential development along “a more masculine pathway neurologically and genitally” (281; emphasis added). Furthermore, they underscore the expansive, developmental nature of the cisgendered future clinicians aim to secure, quoting Saroj Nimkarn and Maria New's rationale that

Dreger, Feder, and Tamar‐Mattis note that Nimkarn and New seem particularly concerned with XX CAH women failing to be heterosexual wives and mothers, having raised the issue in multiple publications and at multiple events (Dreger, Feder, and Tamar‐Mattis Reference Dreger, Feder and Tamar‐Mattis2012, 281).Footnote ¹² Whether one is using fetal dex or surgical sex assignment, one is trying to produce girls who are girls across the lifespan, and then appropriately feminine, heterosexual women, in both body (sex) and mind (gender/sexuality). Codified within and reconstituted through the DSD treatment model is a narrowing of the range of life constituted as normally cisgendered (and an expansion of the abnormally noncisgendered). Sex assignment and fetal dex are part of a suite of interventions aimed at securing heternormatively cisgendered futures for intersex patients under DSD, and thus, simultaneously, the foreclosing of all other potential trajectories of gendered development.