Genital Reconstruction and Gender Identity Disorders



Genital Reconstruction and Gender Identity Disorders


Walter O. Bockting PhD

Leo C. T. Fung MD



This chapter introduces plastic surgeons and other health professionals to the psychological aspects of reconstructive surgery to alter primary and secondary sex characteristics. These surgical procedures are most commonly performed to feminize or masculinize the appearance of children born with ambiguous genitalia (referred to as intersex patients) and of adult transgender/transsexual patients. These procedures are also used in rare cases of genital trauma, such as circumcision accidents or genital mutilation (1, 2). The discussion here will be limited to the psychological aspects of genital reconstruction in the context of sex (re)assignment in childhood or adulthood.

Few health providers are knowledgeable about, or have experience with, transgender or intersex patients. After reading this chapter, surgeons and other health professionals should have a better understanding of the psychosocial context in which these patients request surgical treatment and, therefore, be better equipped to coordinate care with an interdisciplinary team of professionals. In many parts of the United States, specialists can be found through the Harry Benjamin International Gender Dysphoria Association. It is an organization of professionals in the fields of psychology, psychiatry, social work, endocrinology, surgery, and the law dedicated to the understanding and treatment of both gender identity disorders and intersexuality.

This chapter is divided into two main parts addressing genital reconstruction in the treatment of (i) intersex conditions and (ii) gender identity disorders. Case vignettes taken from the first author’s clinical practice illustrate the therapeutic issues and interventions. The chapter concludes with a Questionnaire 13-1, a list of questions to ask patients who request genital reconstructive surgery.


GENITAL RECONSTRUCTION IN THE TREATMENT OF INTERSEX CONDITIONS

Intersex is an umbrella term used to refer to individuals born with a discrepancy between their genetic, gonadal, hormonal, or genital sex (3, 4, 5). A variety of intersex conditions exist, including chromosomal/gonadal conditions (e.g., Klinefelter Syndrome, Turner Syndrome), female pseudohermaphroditism (e.g., Congenital Adrenal Hyperplasia [CAH]), and male pseudohermaphroditism (e.g., Androgen Insensitivity Syndrome, 5-alpha-Reductase Deficiency). The incidence of children with ambiguous genitalia is estimated at 1 to 2 per every 2,000 births (4).

Guidelines for genital reconstructive surgery in children born with ambiguous genitalia are highly controversial. Initial guidelines were based on the hypothesis of psychosexual neutrality at birth (6). The implication was that early genital surgery would facilitate unambiguous gender-appropriate rearing and development of the
expected “optimal” gender identity (5). Hence, children with ambiguous genitalia were traditionally assigned the gender that the external genitalia, after reconstruction, could best support. These assignments appeared to have worked for many patients; however, some developed significant gender dysphoria and were reassigned to the other gender later in life (6).

Since the establishment of these initial guidelines, research has pointed to the potential role of sexual differentiation of the brain in the development of gender identity and role. This work suggests that biology plays a larger role in gender identity development than initially assumed (7). Along with this new knowledge came greater awareness of the potential negative consequences of early genital surgery. The concerns raised by adult intersex individuals centered on the lack of knowledge and information provided to patients and their families and the performance of sometimes unnecessary surgery that later proved to be detrimental to sexual functioning (8). Recently, proposed guidelines for the management of intersexuality reflect this awareness and illustrate a shift toward greater caution in surgical intervention (9). We agree with Cohen-Kettenis and Pfaefflin (3) that decisions regarding sex assignment and genital surgery are best made on a case-by-case basis after careful review of the available empirical evidence, the specific condition, and psychosocial context of the patient and his or her family.

Surgical procedures performed as part of the management of congenital intersex conditions should be directed toward establishing the necessary anatomy for sexual function and cosmesis, in keeping with the gender identity desired by the patient. Ideally, the patient would first reach a decision regarding the desired gender identity. Then, the necessary surgical procedures would be performed. This ideal is often not possible or, at best, is only partly feasible. On the one hand, early surgery is preferable, because it takes advantage of the superior healing and tissue remodeling potential of children as compared to adults. On the other hand, allowing the patient to reach a sufficiently mature age to determine the desired gender identity could circumvent future gender identity disorder. Health care providers take both factors into account when counseling intersex patients and families about surgical treatment.

It is beyond the scope of this chapter to cover, in depth, the wide range of surgical procedures available to intersex patients. We will explore the principles behind some of these procedures in the context of optimizing the integration of surgery into the psychological development and overall well-being of the patient, while optimizing surgical outcome.


Healing and Tissue Considerations

Infants and children have superior healing and regenerative capabilities as compared to adults. This difference is especially pronounced in the early postnatal period. Surgeries performed before a child is full-grown usually result in cosmetically superior results. The benefits of these properties are well recognized in hypospadias repair. When surgical intervention is desirable to an undervirilized male, it is standard practice to perform the surgery when the child is roughly between 1 and 2 years of age (10).

The tissue of the young child has a greater degree of elasticity. For genital reconstruction, this can produce a superior cosmetic result and be the critical determinant in whether or not a certain surgical procedure can be used successfully to bridge a gap in tissue. This principle is well understood in the repair of undescended testes. In a young infant, the mobilized intact spermatic cord allows the testis to reach a relatively long distance. This minimizes the degree of surgical dissection necessary to bring the undescended testis into intra-scrotal position. Should the repair be done at an older age in a child with the same degree of testicular undescent, it becomes exponentially more difficult to bring the testis into position.

In conditions where there is minimal deficiency in vaginal tissue, such as in patients with CAH associated with minimal virilization, early versus late surgery probably
makes little difference. It is in the highly virilized 46, XX patients—with a long distance between the internalized vaginal cavity and the perineum—that the timing of surgery can have a critical impact on physical outcome. In an infant or young child, relatively large gaps between the high internalized vagina and the perineum can generally be bridged simply by surgical mobilization and the use of adjacent perineal tissue (11, 12). At an older age, however, these gaps can no longer be bridged using only locally available tissue. Alternative methods, including the use of skin grafts from a separate donor site (13) or a piece of intestine interposing between the internal vaginal tissue and the perineal skin (14, 15) become necessary. Such procedures are more invasive and inferior in anatomical outcome. In the case of the skin graft procedures, they usually require long-term self-dilation of the vagina (13).


Vaginal Dilation

In the adult or adolescent female, vaginal dilation can be performed with little or no difficulties. Although vaginal dilation is not particularly painful, it can be traumatic for a prepubertal child (16). Thus, reconstructive procedures expected to require vaginal dilation should generally be avoided in these children (17). Other procedures that do not require dilation can be used, or the surgery can be delayed until the patient is old enough to participate in postoperative management. Vaginal plasties which do not require long-term dilation during childhood, include primary vaginal reconstruction using vaginal mobilization (12) and local perineal skin flaps (18, 19). The vaginal cavity typically remains patent throughout childhood. Although some degree of outlet stenosis is likely, this can be easily addressed with vaginal self-dilation at, or after, puberty and prior to sexual activity. Neo-vaginas created with an interposed intestinal segment do not require long-term dilation during the prepubertal period (14, 15).

Vaginal plasties which use skin grafts from a distant donor site are prone to significant contractures and, therefore, need to be maintained by long-term dilation (13). This is generally true even for full-thickness skin grafts, though partialthickness skin grafts are especially prone to contracture. In another technique, pressure is applied to a perineal dimple progressively via a dilator or a probe. This stretches the perineal skin internally to become a vagina suitable for intercourse, but requires long-term dilation for maintenance (20). These techniques can produce satisfactory results, but are less desirable for the prepubertal child.

For patients who have a functional uterus, but a deficient or obstructed vagina, vaginal reconstruction is often a matter of necessity to provide suitable menstrual outflow, prevent infections, and optimize potential fertility. If a patient has a female gender identity but lacks a functional uterus and female genitalia, the need for vaginal reconstruction depends on whether or not the patient desires a reconstructed vagina for functional, psychological, or aesthetic reasons.


Hormonal Brain Imprinting and Limitations in Reconstructive Procedures

It was once thought that a newborn could be successfully reared into a well-adjusted male or female, regardless of the infant’s chromosomal sex, if given the appropriate postnatal hormonal, surgical, and environmental interventions. Unfortunately, this is not always the case. There are examples of 46, XY male infants raised as girls who developed into happy and well-adjusted adult women; but there are also examples of patients who later chose to change to a male gender identity. Before birth, the imprinting effects of androgen exposure in utero appears to have a significant and lasting influence on a person’s gender identity.

Adding to the complexity is the reality that genital reconstruction techniques are by no means perfect. A reasonable outcome can generally be achieved for female
external genitalia, even if tissue availability is limited. The same cannot be said for male external genitalia. Unless there is already sufficient usable penile tissue present, penile reconstruction by other means (e.g., forearm pedicled flaps) remains suboptimal both cosmetically and functionally (21, 22).

Given these two considerations, intersex patients who would ultimately desire female genitalia can generally be expected to achieve a satisfactory surgical outcome. The situation is more challenging when a patient has been subjected to significant imprinting of the brain under the exposure of androgen and when there is insufficient penile tissue for satisfactory male external genitalia. Such a situation is often encountered in 46, XY patients with cloacal exstrophy. Rearing this patient as a male will result in external genitalia that will continue to appear ambiguous, yet rearing as a female could result in serious future dissatisfaction and a change in gender identity to male. There is no obvious solution to this dilemma.


The Timing of Gonadal Surgery

The timing of gonadal surgery is critical and can significantly impact both the physical and psychological outcomes. Consider the case of a 46, XY patient with testes and male-typical androgen function being considered for a female sex due to not having sufficient penile tissue to allow for effective construction of male genitalia. If the child is to be reared as female, it would be desirable to remove any further androgen influence as soon as possible, necessitating the removal of all functional testicular tissue. In contrast, the patient may ultimately wish to have a male gender identity, and the irreversible nature of removing the testes would not only alter the developmental process as a male, but also destroy any potential for fertility. Once again, a decision regarding the management of the gonads must be made long before the child is mature enough to participate in the decision-making process. Unfortunately, there is no perfect solution.


Psychological Impact of Growing Up with Ambiguous Genitalia

In some situations, intersexuality can be managed by waiting until the patient becomes sufficiently mature to decide upon the desired gender identity. However, foregoing early surgery requires the patient to go through a significant portion of childhood with ambiguous genitalia. Some parents are more likely to accept their child’s ambiguous genitalia than others. Once the child becomes aware of having genitalia that are quite different from his or her peers, the child may develop a sense of shame. If other children become aware of this difference, the child may be teased or ridiculed. Nevertheless, the impact of growing up with ambiguous genitalia may be less traumatic than previously assumed, especially when adequate social support is available. In the absence of empirical research, however, we cannot conclude that foregoing surgery does not result in psychological harm.


Considerations Specific to Different Groups of Intersex Conditions

Intersex conditions often are classified either by the genetic makeup or anatomical characteristics of the patient. Neither approach serves the present discussion well. In considering surgical procedures for intersex conditions, the major categories are more appropriately divided into three groups (see Table 13-1).


Group I

Group I conditions do not require genital reconstruction; the external sexual characteristics are in keeping with the expected gender identity. These conditions are predominantly genetic in nature and may affect reproductive potential. Examples
include 46, XY patients with CAH (precocious puberty with a male phenotype), and females with gonadal dysgenesis (XO karyotype, Turner’s Syndrome, female phenotype). They also include disorders that may require surgery for other, nonreconstructive concerns such as gonadal malignancy. Although the exact risk of dysgenetic gonads undergoing malignant transformation is unknown, dysgenetic gonads containing all or part of a Y chromosome are considered at risk for gonadoblastoma (23) and are generally recommended to be excised (24).








TABLE 13-1 Grouping of Intersex Conditions by Need and Possibilities of Genital Reconstruction















































Desired Sexual Identity


Physical Anatomy


Genital Surgical Reconstruction


I


a


Male


Typical male


None needed



b


Female


Typical female


None needed


II


a


Male


Ambiguous


Anatomically functional; reconstruction possible



b


Female


Ambiguous


Anatomically functional; reconstruction possible


III


a


Male


Ambiguous


Anatomically functional; reconstruction not possible



b


Female


Ambiguous


Anatomically functional; reconstruction not possible



Group II

Group II conditions are characterized by two features: (i) ambiguity of the external genitalia and (ii) physical anatomy that can be expected to yield satisfactory reconstruction of the external genitalia. With successful reconstruction, these patients can be expected to have a good cosmetic result and achieve satisfactory sexual function. However, many of these conditions are associated with infertility. Conditions in Group II include most 46, XX patients with CAH and male pseudohermaphrodites where the hypospadiac penis and/or undescended testes are amenable to surgical reconstruction. The group also includes true hermaphrodites, where an appropriate sex of rearing can be determined with relative certainty and where the corresponding external genitalia can be achieved surgically.

For these patients, early surgery can be performed in view of the superior healing potential of young children. This approach seems reasonable in male reconstructions. Even if the patient later develops a female gender identity, a subsequent surgical reversal from male to female would be feasible. The best management approach is not as clear in treating the severely virilized female. In most instances, feminizing surgery yields satisfactory long-term results both physically and psychologically. But what would happen if the patient has had such a degree of androgen brain imprinting that a male gender identity develops? The feminizing surgery would have removed the possibility that the severely androgenized external genitalia be modified into satisfactory male genitalia. This situation argues that at least some 46, XX patients with CAH may not fit the Group II categorization, but fall into Group III.


Group III

This group constitutes conditions where the management issues are unresolved. This may occur when male genitalia are desired, but unable to be successfully reconstructed. This group also includes the severely virilized 46, XX patient with CAH and true hermaphrodites for whom the optimal sex of rearing is unclear and where significant limitations in the anticipated outcome of genital surgery exist. In these
challenging situations, the difficult task of selecting the sex of rearing and deciding upon additional surgery is relegated to the child’s parents or legal guardian. The health professionals’ role should be limited to providing unbiased information regarding the pros and cons of the full range of treatment options. Because there are no perfect solutions, each decision will necessarily lead to some desired benefits, while foregoing other desirable potentials.


Psychological Support

Psychological support and counseling should be provided for children born with ambiguous genitalia and their families. Parents may experience a range of emotions when learning about their child’s condition, from initial shock and confusion, shame and anxiety, to anger and sadness. Addressing these feelings will facilitate the acceptance necessary to act in the child’s best interest. Parents will have questions about their child’s future, including issues of sexual orientation as well as sexual and reproductive functioning. At birth, parents should be informed completely about their child’s condition and counseled in order to make a fully informed decision regarding sex assignment and the available medical interventions. Such counseling is best provided by a mental health professional who is part of a multidisciplinary team that specializes in the clinical management of intersexuality. These teams are not always readily available and parents are not always amenable to seeing a psychologist or psychiatrist (25). The treating physician can help by explaining that counseling is a regular part of the assessment and treatment process. Peer support organizations are another potential resource.

Affected patients are best served by accurate information about their condition and prognosis. However, information needs to be paced and provided in a sensitive, age-appropriate manner (26) and preferably by the parents (3). This is easier said than done. Many parents have great difficulty talking about gender and sexuality, especially to children. Above all, parents, counselors, and treating physicians should agree on what information to provide and when. Information and emotional support will help most children and adolescents to accept and adapt to their intersexuality. Children and adolescents who are ridiculed by their peers for being different may need additional assistance in the form of psychological and school-based interventions. Teasing by peers appears to be an important contributor to mental health problems of intersex patients (27).

All intersex patients are likely, at one time or another, to struggle with shame as a result of the stigma associated with their condition. In an analysis of the life histories of intersex adults, their adaptation to being different was likened to the “coming out” process for gay and lesbian individuals, in which shame and secrecy transformed into pride (28, 29). Contact with similar others enabled participants to “own” their intersexuality, create greater visibility to combat isolation, and shape their treatment. Intersex patients may encounter challenges associated with their condition at any point in their lives. These may stem from gender identity or body image concerns (30), sexual functioning or fertility concerns (8), academic performance issues (e.g., as a result of cognitive deficits associated with Klinefelter’s Syndrome) (3), or socialization difficulties (e.g., associated with Turner’s Syndrome) (31).


GENITAL RECONSTRUCTION IN THE TREATMENT OF GENDER IDENTITY DISORDERS

Transgender is an umbrella term used to refer to a diverse group of individuals who cross or transcend culturally defined categories of gender (32). Transgender individuals include transsexuals (who desire or have had hormone therapy and/or genital reconstructive surgery), transgenderists (who live full-time in the cross-gender role and may take sex hormones, but do not desire genital reconstructive surgery), cross-dressers
or transvestites (who wear clothing associated with another sex for emotional or sexual gratification), drag queens or drag kings (who adopt a hyperfeminine or hypermasculine presentation), and individuals identifying as bigender or “two-spirit” (both woman and man). A transgender patient might report having an intersex condition, as illustrated in the first vignette at the end of this chapter. Often, however, a physical examination of the transgender patient does not confirm intersexuality. Data on the prevalence and incidence of transsexualism are scarce. Prevalence is estimated at one in 11,900 for male-to-females (MtF) and one in 30,400 for female-to-males (FtM) (33). Incidence estimates range from .15 to 1.58 per 100,000 persons annually (34).

Transgender individuals may meet criteria for gender identity disorders (see Table 13-2) as described in the Diagnostic Statistical Manual of Mental Disorders (DSM) (35) or the International Classification of Diseases (36). A key criterion of the DSM definition is that “the disturbance causes clinically significant distress or impairment in social, occupational, or other important areas of functioning” (p. 581). For individuals who experience such distress, sex reassignment surgery may alleviate significant impairment in interpersonal and/or vocational functioning. For these reasons, the surgery is almost always medically necessary, not elective or cosmetic.

Since the 1970s, the Benjamin Association has set forth standards of care for the treatment of gender identity disorders (37). The standards delineate eligibility and readiness criteria for cross-gender hormone therapy and sex reassignment surgery. In addition, the standards offer guidelines for competency and practice for surgeons and other professionals (see Table 13-3). To ensure quality of care, the standards require that two mental health professionals independently evaluate and recommend sex reassignment surgery before the surgeon makes the final determination whether or not to operate on a transsexual patient.

Although these standards are widely used, not everyone who takes feminizing or masculinizing hormones or requests sex reassignment surgery, has been prepared in accordance with them. Needs assessment studies in transgender communities across the U.S. have found rates of illicit hormone use ranging from 29% to 71% (38, 39, 40). This is especially common among the more marginalized and impoverished, including those who work in the sex industry (41). In light of the psychological and social issues that applicants for sex reassignment may face (as described below), it is essential for every surgeon working with transgender patients to familiarize themselves with the standards of care and adhere to their guidelines.


Sex Reassignment and Identity: From Gender Dichotomy to Gender Diversity

The meaning and associated clinical approach to sex reassignment has gone through an important paradigm shift since the first surgery in the United States (42). In the 1960s and 1970s, treatment was guided by a dichotomous view of gender (male versus female, man versus woman, masculine versus feminine); the focus of sex reassignment was to assist males to become women and women to become men (43, 44). Indeed, the effectiveness of sex reassignment was evaluated on the basis of how well transsexuals were able to “pass” as members of the “opposite” sex (45). A change in one’s genitalia signified the ultimate change in sex.

This paradigm began to shift in the 1980s when Prince coined the term “transgenderist” to refer to males who live full-time as women without undergoing genital reconstructive surgery (46). A growing number of transgenderists and a generation of postoperative transsexuals began to question the dichotomous understanding of gender. Stone (47), a postoperative MtF transsexual, was one of the first to call for transsexuals to come out and affirm their unique identity and experience. Rather than starting a new life as a member of the other sex, individuals began to claim a transgender or transsexual identity that continues beyond the transition or sex reassignment phase.









TABLE 13-2 Diagnostic Criteria that May Include an Indication for Sex Reassignment Surgery




















































DSM IV-TR (American Psychiatric Association, 2000)


ICD-10 (World Health Organization, 1992)


302.85


Gender Identity Disorder in Adolescents or Adults


F(64.0)


Transsexualism


A.


A strong and persistent cross-gender identification (not merely a desire for any perceived cultural advantages of being the other sex), … manifested by symptoms such as a stated desire to be the other sex, frequent passing as the other sex, desire to live or be treated as the other sex, or the conviction that he or she has the typical feelings and reactions of the other sex


1.


The desire to live and be accepted as a member of the opposite sex, usually accompanied by the wish to make his or her body as congruent as possible with the preferred sex through surgery and hormone treatment


B.


Persistent discomfort with his or her sex or sense of inappropriateness in the gender role of that sex, … manifested by symptoms such as preoccupation with getting rid of primary and secondary sex characteristics (e.g., request for hormones, surgery, or other procedures to physically alter sexual characteristics to simulate the other sex) or belief that he or she was born the wrong sex


2.


The transsexual identity has been present persistently for at least 2 years


C.


The disturbance is not concurrent with a physical intersex condition


3.


The disorder is not a symptom of another mental disorder or a chromosomal abnormality


D.


The disturbance causes clinically significant distress or impairment in social, occupational, or other important areas of functioning


302.6


Gender Identity Disorder Not Otherwise Specified


F(64.9)


Gender Identity Disorder, Unspecified



This category is included for coding disorders in gender identity that are not classifiable as a specific Gender Identity Disorder. Examples include:



No specific criteria, yet could be used for gender dysphoria among patients with an intersex condition


1.


Intersex conditions (e.g., partial androgen insensitivity syndrome or congenital adrenal hyperplasia) and accompanying gender dysphoria


2.


Transient, stress-related cross-dressing behavior


3.


Persistent preoccupation with castration or penectomy without a desire to acquire the sex characteristics of the other sex




Adapted from the World Health Organization. International Statistical Classification of Diseases and Related Health Problems. 10 rev. Geneva, Switzerland: World Health Organization, 1992.

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