History of intersex surgery

Main article: Intersex surgery

The History of intersex surgery is intertwined with the development of the specialities of pediatric surgery, pediatric urology, and pediatric endocrinology, with our increasingly refined understanding of sexual differentiation, with the development of political advocacy groups united by a sexual identity, and in the last decade by doubts as to efficacy, and controversy over when and even whether some procedures should be performed.

Ambiguous genitalia has been considered a birth defect throughout recorded history. A cuneiform tablet describing it as a problem has been translated. Single cases were described by doctors sporadically over the centuries. Some of our modern ideas of birth defects can be traced to French anatomist Isidore Geoffroy Saint-Hilaire (1805-1861), who pioneered the field of teratology. Since the 1920s surgeons have attempted to correct an increasing variety of birth defects. Success has often been partial and surgery is often associated with minor or major, transient or permanent complications. Techniques in all fields of surgery are frequently revised in a quest for higher success rates and lower complication rates. Some surgeons, well aware of the immediate limitations and risks of surgery, feel that significant rates of imperfect outcomes are no scandal (especially for the more severe and disabling birth defects). Instead they see these negative outcomes as a challenge to be overcome by improving the techniques.^[1] Genital reconstruction evolved within this tradition, but in the last decade, nearly every aspect of this perspective has been called into question.

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Surgical pioneering and constructed gender

Genital reconstructive surgery was pioneered between 1930 and 1960 by urologist Hugh Hampton Young and other surgeons at Johns Hopkins Hospital in Baltimore and other major university centers. Understanding of intersex conditions was relatively primitive, based on identifying the type of gonad(s) by palpation or by surgery. Since ability to determine even the type of gonads in infancy was limited, sex of assignment and rearing were determined mainly by the appearance of the external genitalia. Most of Young's intersex patients were adults seeking his help with physical problems of genital function.

Demand for surgery increased dramatically with better understanding of the most common intersex condition, congenital adrenal hyperplasia (CAH), and availability of a new treatment (cortisone) by Lawson Wilkins, Frederick Bartter and others around 1950. For the first time, severely virilized infants with this disorder were surviving and could be operated upon. Hormone assays and karyotyping to ascertain sex chromosomes, and the availability of testosterone for treatment led to partial understanding of androgen insensitivity syndrome. Within a decade, most intersex cases could be accurately diagnosed and their future development predicted with some degree of confidence.

As the number of children with intersex conditions referred to Lawson Wilkins' new pediatric endocrinology clinic at Hopkins increased, it was recognized that doctors "couldn't tell by looking" at the external genitalia, and many errors of diagnosis based on outward appearance had led to anomalous sex assignments. Although it seems obvious now that a doctor could not announce to an eight year old boy and his parents that "we have just discovered that you are 'really' a girl, with female chromosomes, and ovaries and uterus inside, and we recommend that you change your sex to match your chromosomes and internal organs," a few such events occurred around the world as doctors and parents tried to make use of new information.

Genital reconstructive surgery at that time was primarily performed on older children and adults. In the early 1950s, it consisted primarily of the ability to remove an unwanted or nonfunctional gonad, to bring a testis into a scrotum, to repair a milder chordee or hypospadias, to widen a vaginal opening, and to remove a clitoris.

John Money, a pediatric clinical psychologist in the new "Psychohormonal Research Unit" at Hopkins and his partners, John and Joan Hampson, analyzed these assignments and reassignments in an attempt to learn the timing and sources of gender identity. In most of these patients, gender identity seemed to follow the sex of assignment and sex of rearing more closely than it did genes or hormones. This apparent primacy of social learning over biology became part of the intellectual underpinning of the feminist movement of the 1960s. In its application to children with intersex conditions, this thesis that sex was a many-faceted social construction changed the management of ambiguous genitalia from determination of the baby's real sex (by checking gonads or chromosomes) to determination of what sex should be assigned.

The most common intersex surgery offered in childhood was amputation of the clitoris and widening of the vaginal opening to make a virilized girl with CAH appear less like a boy. However, by the late 1950s surgical techniques for transforming an adult man into a woman were being developed in response to requests for such surgery from transsexuals.

Rise of infant surgery and "nurture over nature"

By the 1960s, the young specialties of pediatric surgery and pediatric urology at children's hospitals were universally admired for bringing infant birth defect surgery to new levels of success and safety. These specialized surgeons began to repair wider varieties of birth defects at younger ages with better results. Earlier correction reduced the social "differentness" of a child with a cleft lip, or club foot, or skull malformation, or could save the life of an infant with spina bifida.

Genital corrective surgeries in infancy were justified by (1) the belief that genital surgery is less emotionally traumatic if performed before the age of long-term memory, (2) the assumption that a firm gender identity would be best supported by genitalia that "looked the part," (3) the preference of parents for an "early fix," and (4) the observation of many surgeons that connective tissue, skin, and organs of infants heal faster, with less scarring than those of adolescents and adults. However, one of the drawbacks of surgery in infancy was that it would be decades before outcomes in terms of adult sexual function and gender identity could be assessed.

In North American and European societies, the 1960s saw the beginning of the "sexual revolution," characterized by increased public interest and discussion about sexuality, recognition of the value of sexuality in people's lives, the separation of sexuality from reproduction by increasing availability of contraception, the lessening of many social barriers and inhibitions related to sexual behavior, and social acknowledgement of women's sexuality. It was the era when feminism made it politically incorrect to question the social learning theory of gender identity: the main differences between boys and girls resulted from being taught to be different. Genes and hormones were thought not to have a strong influence on any aspect of human psychosexual development, gender identity, or sexual orientation.

The 1970s and 1980s were perhaps the decades when surgery and surgery-supported sex reassignment were most uncritically accepted in academic opinion, in most children's hospitals, and by society at large. In this context, enhancing the ability of people born with abnormalities of the genitalia to engage in "normal" heterosexual intercourse as adults assumed increasing importance as a goal of medical management. Many felt that a child could not become a happy adult if his penis was too small to insert in a vagina, or if her vagina was too small to receive a penis.

By 1970, surgeons still considered it "easier to make a hole than a pole," but had abandoned "barbaric" clitorectomies in favor of "nerve sparing" clitoral recession and promised orgasms when the girls grew up. Pediatric endocrinology, surgery, child psychology, and sexuality textbooks recommended sex reassignment for a male whose penis was irreparably malformed or "too small to stand to urinate or penetrate a vagina," because the surgeons claimed to be able to construct vaginas where none existed. The majority of these genetic males who were reassigned and surgically converted had cloacal exstrophy-type malformations or extreme micropenis (typically less than 1.5 cm). In 1972 John Money published his influential text (Money, 1972) on the development of gender identity, and reported successful reassignment at age 22 months of a boy (David Reimer) who had lost his penis to a surgical accident.

Complications arise

Throughout the 1980s pediatric surgery textbooks recommended female assignment and feminizing reconstructive surgery for XY infants with a severely inadequate phallus. Nevertheless, in the 1980s several factors began to induce a decline in the frequency of certain types of genital surgery. Pediatric endocrinologists had realized that some boys with micropenis had deficiency of growth hormone which could be improved with hormones rather than surgery, and over the next decade a couple of reports suggested adult outcome as males was not as bad as expected for the boys with micropenis who had not had surgery. Although textbooks were slower to reflect the change, few reassignment surgeries for isolated micropenis were carried out by the 1990s.

In the 1980s research in both animals and humans began to provide evidence that sex hormones play an important role in early life in promoting or constraining adult sex-dimorphic sexual behavior and even gender identity. Examples of apparent androgen determination of gender identity in XY people with 5-alpha-reductase deficiency in the Dominican Republic had been published, along with reports of masculinized behavior in girls with congenital adrenal hyperplasia (CAH), and unsatisfactory sexual outcomes in adult women with CAH. Many endocrinologists were becoming skeptical that reassignment of genetic males to females was just a matter of learning and appearance, or that the newer clitoral reductions would be more successful than clitoral recessions.

However, feminizing reconstructive surgery continued to be recommended and performed throughout the 1990s on most virilized infant girls with CAH, as well as infants with ambiguity due to androgen insensitivity syndrome, gonadal dysgenesis, and some XY infants with severe genital birth defects such as cloacal exstrophy. Masculinizing reconstructive surgery continued on boys with severe hypospadias and the other conditions outlined above, with continued modifications and refinements intended to reduce unsatisfactory outcomes.

Infant surgery falls from favor

By 1990, biological factors were being reported for a wide variety of human behaviors and personality characteristics. The idea that culture accounted for all the differences between men and women seemed as obsolete as psychotherapy for homosexuality.

A more abrupt and sweeping re-evaluation of reconstructive genital surgery began about 1997, triggered by a combination of factors. One of the major factors was the rise of patient advocacy groups that expressed dissatisfaction with several aspects of their own past treatments. The Intersex Society of North America was the most influential and persistent, and has advocated postponing genital surgery until a child is old enough to display a clear gender identity and consent to the surgery. Recommendations from these voices ranged from the unexceptionable (ending shame and secrecy, and providing more accurate information and counseling) to the radical (assigning a third sex or no sex at all to intersex infants). The idea that possession of abnormal genitalia in and of itself does not constitute a medical crisis was stressed.

However, physicians involved in intersex care had embarrassingly little long-term outcome data with which to refute them. In 1997 a patient account was published which could not be ignored. David Reimer's tragic story, told in both popular and medical publications, was widely interpreted by the public and many physicians as a cautionary tale of medical hubris, of the folly of attempting to foil nature with nurture, of the importance of early hormones on brain development, and the risks and limitations of surgery.^[2] Some clinicians proposed a moratorium on pediatric sex reassignment, particularly of undervirilized males as females, due to a lack of data that rearing or appearance of genitalia play a major part in gender identity development. Those clinicians encouraged delaying surgery until elected by adolescents in order to preserve sexual sensitivity.^[3]

Similar controversy occurred in Europe and Latin America. In 1999 Colombia's constitutional court limited the ability of parents to consent to genital surgery for infants with intersex conditions. A number of advocacy groups argue against many forms of genital surgery in childhood.^[4] In 2001, British surgeons argued for deferring vaginoplasty until adulthood on grounds of poor outcomes for women who were operated on as infants.^[5]

Outcomes and statistics

Regardless of the representativeness of the advocacy groups, the doctors began to listen. Within a short time re-evaluation of intersex management was a major topic in the journals and the meetings of the pediatric endocrinologists and urologists. There was no international "registry" of genital reconstructive surgery. Most cases of this type of surgery were being carried out in a relatively small number of centers. Several of the larger institutions embarked on individual and collaborative retrospective outcome studies, attempting to contact all individuals treated as children in their institutions since the 1960s. A large, collaborative, retrospective survey, the North American Task Force on Intersexuality, was organized, with input from patient advocates.

Johns Hopkins Hospital, the institution that was arguably most responsible for the intersex management paradigm of the previous 4 decades, and the principal target of activist criticism in the late 1990s, was also the first to publish a series of outcome studies. Between 2000 and 2004, the pediatric psychologists, endocrinologists, urologists, and gynecologists associated with Hopkins reviewed all cases of complete androgen insensitivity (Wisniewski, 2000 and 2002), micropenis (Wisniewsk1, 2001 and 2002), XY ambiguity (Migeon, 2002a and 2002b), XY cloacal exstrophy (Reiner, 2004), and congenital adrenal hyperplasia (Wisniewski, 2004) seen as children and now grown, whether operated upon or not. They attempted to locate and contact all of the individuals to ascertain outcomes by questionnaire, interview, and examination. With a subsequent questionnaire, they asked these people their opinion of their own management and some of the recommendations of recent years (Meyer-Bahlburg, 2004).

To estimate how many major genital reconstructions were performed over approximately the first 25 years of traditional management at North America's first and largest program for intersex surgery:

• 134 XX infants with virilizing congenital adrenal hyperplasia raised as girls. About 90% had corrective surgery in childhood.
• 20 XY infants with completely female genitalia due to complete androgen insensitivity syndrome, raised as girls. All had gonadectomy; some had vaginoplasties.
• 6 XY infants with completely female genitalia due to Swyer syndrome, raised as girls, generally diagnosed in adolescence. Most had surgery, usually exploration and gonadectomy.
• 114 XY infants with severely ambiguous genitalia due to a variety of conditions; 64 were raised as males and 50 were raised as females. All had reconstructive surgeries.
• 43 XY infants with micropenis; 31 were raised as males, 12 were reassigned as female and underwent reconstructive surgery.
• 14 XY infants with cloacal exstrophy raised as females; all had surgical reconstruction.

Perhaps the most striking findings from these reports are:

1. the relatively small numbers of patients who actually underwent the more controversial procedures even in one of the largest centers in the world
2. the relatively high proportion of former patients who were located and willing to participate
3. the small differences in terms of outcomes, problems, and satisfaction between groups of people with the same conditions treated very differently.

Exact numbers of patients at other large centers have not been published, but few could have rivaled these numbers. In the early 1980s there were less than 20 children's hospitals in North America with similarly-sized pediatric endocrine clinics, and most had smaller referral areas.

Controversy

See also: Intersex surgery#Controversies and unsettled questions

The goals of surgery, outlined at the beginning of this article have not changed, but some types of surgery have been discontinued. The primary ongoing controversy surrounds timing of feminizing surgery for virilized girls with CAH.

Alhough surgical sex reassignments (to female) of XY males with unambiguous micropenis or penile injury had been dwindling in the 1990s, few if any have been done since. Accounts of spontaneous self-reassignment since then have also reduced the reassignment to female of several other forms of XY ambiguity and malformation (especially the more intermediate forms of androgen insensitivity syndrome and idiopathic undervirilization, and non-hormonal birth defects such as cloacal exstrophy).

References

Melton L. New perspectives on the management of intersex. Lancet 357:2110, 2001. A journalist’s summary of the controversies. Responding letters illustrate both sides of the arguments and complexity of some of the issues. E.g., in response to argument against clitoral reduction surgery, a girl who will not swim at school because her clitoris is visibly prominent is described.

Meyer-Bahlburg HFL, Migeon CJ, Berkovitz GD, Gearhart JP, Dolzal C, Wisniewski AB. Attitudes of adult 46,XY intersex persons to clinical management policies. J Urol 171:1615-9, 2004. This was a questionnaire survey of all adults who could be contacted who had been treated at Hopkins from the 1950s until the early 1980s, to solicit their opinions on the standard intersex management practices of those years and on some proposed changes. Of 96 patients from the above listing of complete androgen insensitivity, XY ambiguity, and micropenis, 72 responded. 85% were satisfied with their assigned genders, though 32% remembered a period of uncertainty. 15% endorsed the idea of assigning a child a third sex (neither male nor female). 68% of the men felt their penises were too small, but only 18% were dissatisfied with sexual function. About half of the women (none of the AIS women) thought their genitalia looked unusual but nearly half were dissatisfied with sexual function. Most disagreed with the idea of postponing surgical correction until adolescence or later. The authors admit that their study cannot be assumed to be representative of all former patients. These data demonstrate at a minimum that some of the policy changes advocated by critics may be unrepresentatively pessimistic about outcomes and policy preferences of intersexed people.

[1] Migeon CJ, Wisniewski AB, Brown TR, Rock JA, Meyer-Bahlburg HFL, Money J, Berkovitz GD. 46,XY Intersex individuals: phenotypic and etiologic classification, knowledge of condition, and satisfaction With knowledge in adulthood. Pediatrics 110: electronic pages, pp. e32, September 2002. Between 1953 and the 1980s (i.e., all patients who were above 21 in 2000) 183 infants and children had been seen in the Hopkins pediatric endocrine clinic who had an XY karyotype and complete undervirilization, partial undervirilization (ambiguous genitalia), or micropenis. The 26 with complete undervirilization included 20 with complete androgen insensitivity and 6 with Swyer syndrome. All infants with complete undervirilization (i.e., female external genitalia) were raised as girls. Of the 43 with micropenis, 12 were reassigned as female and underwent feminizing surgery; 31 were raised as boys and treated with extra testosterone. Causes of partial undervirilization with ambiguity included defects of testosterone synthesis, partial gonadal dysgenesis, partial androgen insensitivity, Leydig cell hypoplasia, timing defects, true hermaphroditism, and multiple congenital anomalies. Of the 114 patients with ambiguity, 50 were raised as female (most with feminizing surgery) and 64 were raised as boys (some had surgery). They attempted to locate and survey all patients for outcome information. They located 73%, but 12% were developmentally delayed and 9% were deceased (2 from suicide). Of the 96 located, eligible adults, 78% consented to participate (18 women born with complete undervirilization, 18 women born with partial undervirilization, 21 men with partial virilization, 5 women born with micropenis, and 13 men born with micropenis). Roughly half of the patients had a satisfactory understanding of their condition; half wanted more. For more detailed outcome data for each group: see reference [] for complete androgen insensitivity, reference [2] for partial virilization (XY ambiguity), and reference [] for micropenis.

[3] Migeon CJ, Wisniewski AB, Gearhart JP, Meyer-Bahlburg HFL, Rock JA, Brown TR, Casella SJ, Maret A, Ngai KM, Money J, Berkovitz GD. Ambiguous genitalia with perineoscrotal hypospadias in 46,XY individuals: long-term medical, surgical, and psychosexual outcome. Pediatrics 110: electronic pages, pp e31, September 2002. This is a report of the adult outcome data of the 39 adults with partial undervirilization (ambiguity) described in [4]; 18 had been raised female and 21 had been raised male. Those raised as men underwent an average of 5.8 surgical procedures, while those raised as women had 2.1 surgeries. Both men and women rated their body image and genital function in the middle of a scale between satisfied and dissatisfied. Female sex of rearing resulted in fewer genital surgical procedures overall and better cosmetic appearance of the external genitalia as rated by physicians, compared with patients who were reared male. Despite these seeming advantages to female sex of rearing, men and women did not differ according to their degree of satisfaction with their physical appearance, genital function, and sex of rearing. The majority of respondents reported being exclusively heterosexual. The majority of men felt masculine. The majority of women felt feminine. However, 23% (5 men, 4 women) were dissatisfied with the sex of rearing chosen by parents and doctors in infancy.

Money J, Ehrhardt AA. Man & Woman, Boy & Girl. Baltimore: Johns Hopkins University Press, 1972. Classic, influential exposition of Money's theories on gender identity development, including an early report on the Reimer case.

Oesterling JE, Gearhart JP, Jeffs RD. A unified approach to early reconstructive surgery of the child with ambiguous genitalia. J Urol 138:1079-82, 1987. A report from Hopkins from the mid-1980s, describing varied approaches for variations of feminizing surgery, including a case of sex reassignment for micropenis.

Passerini-Glazel G. Editorial: feminizing genitoplasty. J Urol 161:1592-3, 1999. Restatement of the classic surgeon's arguments that (1) complications are being reduced by newer techniques, and (2) that surgery should optimally be performed even earlier (by 2 months of age) to take advantage of the estrogenized state of the tissue in early infancy.

Penny R. Disorders of the testes. In: Kaplan S, ed. Clinical Pediatric and Adolescent Endocrinology. Philadelphia: Saunders, 1982. An example of a now-obsolete recommendation to consider reassigning a boy with severe micropenis as a girl.

Reiner WG, Gearhart JP. Discordant sexual identity in some genetic males with cloacal exstrophy assigned to female sex at birth. New Engl J Med 350:333-41, 2004. Eight of 14 genetic males who were born with exstrophy and unsalvageable penis, assigned and raised as females, spontaneously reassigned themselves to male sex as they grew up. This outcome suggests XY infants with exstrophy but normal testes should not be raised as females.

Rink RC, Adams MC. Feminizing genitoplasty: state of the art. World J Urol 16:212-218, 1998. Historical review of evolution of techniques for feminizing reconstruction.

Schnitzer JJ, Donahoe PK. Surgical treatment of congenital adrenal hyperplasia. Endocrinol Metab Clin N Am 30:137-54, 2001. Recent, brief state-of-the-art review of infant intersex surgery without addressing the controversies. Patricia Donahoe is the renowned chief of pediatric surgery at Massachusetts General Hospital.

Walsh PC, Scott WW. Intersex. In: Ravitch MM, et al, eds. Pediatric surgery, 3rd ed. Chicago: Year Book Medical Publishers, 1979. Details of techniques for feminizing surgery from the first two decades, including an explanation of how the size of the phallus is the most important aspect of assignment decisions.

Wisniewski AB, Migeon CJ. Long-term perspectives for 46,XY patients affected by complete androgen insensitivity or congenital micropenis. Sem Reprod Med 20:297-304, 2000. Survey of patients treated with older management, including 5 with micropenis reassigned and raised as women. Gender identity was concordant with sex of assignment in all. Women with CAIS were satisfied with genitalia, men and women who had had micropenis were universally dissatisfied, but sexual function was better in men than women. Outcomes suggest infant reassignment to female and surgery does not produce an improved adult satisfaction with sexual function.

Wisniewski AB, Migeon CJ, Gearhart JP, Rock JA, Berkovitz GD, Plotnick LP, Meyer-Bahlburg HFL, Money J. Congenital micropenis: long-term medical, surgical and psychosexual follow-up of individuals raised male or female. Hormone Res 56:3-11, 2001. This is an informative review of experience at the institution that pioneered the standard management of the last few decades. It was an attempt to survey and determine outcome of all patients with unambiguous micropenis seen prior to 1979 at Hopkins. Of 45 patients, 12 had been reassigned and raised female. Two females were not locatable, 4 were cognitively impaired, and 1 refused to participate; there were 13, 7, and 2 men in those categories. Of the 13 men and 5 women who participated, all were satisfied with sex of rearing and none had changed sex, though most of the women had gender identity doubts as adolescents. Dissatisfaction with appearance or function of genitalia was higher in the women than men. Outcomes suggest that adult penile size and intellectual function are often inaccurately predicted in infancy. Authors conclude that XY infants with unambiguous micropenis should be raised male, without reassignment and surgery. This represents a change of recommendation over the last 2 decades.

Wisniewski AB, Migeon CJ, Malouf MA, Gearhart JP. Psychosexual outcome in women affected by congenital adrenal hyperplasia due to 21-hydroxylase deficiency. J Urol 171:2497-2501, 2004. Of 134 women seen for CAH during childhood before the early 1980s, 61 could be located and 41 participated, including 22 with salt-losing CAH and moderate-to-severe virilization, and 19 with mild-to-moderate virilization ("simple virilizing" CAH). Of the more severely virilized group, 37% had "questioned" their female gender but none regretted female assignment or reassigned themselves, 18% had avoided sexual relations and 74% had "sexual concerns." Percentages were lower in the more mildly virilized group. Most recommended that surgical reconstruction be done in childhood rather than deferred.

Young HH. Genital Abnormalities, Hermaphroditism, and Related Adrenal Diseases. Baltimore: Williams and Wilkins, 1937. This was the standard work on intersex conditions until the middle of the century, and helped establish the reputation of Johns Hopkins as a pioneering center for these conditions.

See also

• Intersex
• Ambiguous genitalia
• Congenital adrenal hyperplasia due to 21-hydroxylase deficiency
• David Reimer
• Pediatric urology
• Hypospadias
• Cloacal exstrophy
• Sex reassignment surgery