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Current Medical, Psychological, and Ethical Implications for Treating Differences of Sexual Development (DSD)

By Thomas Chameli, BS; Sarah Heins, BA; Thu Dao, BS; Erica Choe, BS

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Citation

Chameli T, Heins S, Dao T, Choe E. Current medical, psychological, and ethical implications for treating differences in sexual development. HPHR. 2021;46.

Current Medical, Psychological, and Ethical Implications for Treating Differences of Sexual Development (DSD)

Abstract

Purpose of Review

Differences of sexual development (DSD) have significant implications on a child’s health and quality of life. Traditionally, DSD have been treated with early surgical intervention; however, physicians, researchers, and advocates are increasingly working towards balancing medical indications, psychological implications, and necessary ethical questions. Despite the rapid evolution of DSD management, the latest recommendations call for shared decision-making (SDM) but fail to highlight ethical issues or practices that respect the rights of pediatric patients.

Recent Findings

Since the most recent DSD treatment guidelines, published in the 2006 Consensus Statement, research further defines the best SDM for the treatment of DSD as comprising the cooperation of a multidisciplinary team working with patients’ parents. The collaboration of various experts and well-informed parents will expectantly improve the current issues in treatment including excessive rates of unjustified gonadectomies, removal of genital structures, and loss of fertility among patients with DSD. Despite recent research, the psychological impact of early surgical intervention remains inconclusive, but studies highlight the strong need for early and continuous psychological treatment starting with incorporating psychiatrists in the SDM team. Additionally, medical ethics question the legality of performing irreversible medical treatments on patients who cannot consent on their own and the ability of parents and physicians to engage in an effective decision-making process. As a result, many medical and government organizations have called for the elimination of early invasive treatment whereas surgeons and researchers are developing new techniques to preserve genital structures and fertility while correcting medically necessary aspects of DSD.

Summary

This narrative review discusses the progress made since 2006 considering malignancy risks, fertility, urogenital function, psychiatric management, parental consent, patient autonomy, decision-making ability, and communication effectiveness. The authors also highlight areas that require further improvement and propose next steps in improving care for individuals with DSD.

Introduction

Worldwide, approximately 1 in 5,000 babies have differences of sexual development (DSD) (Timmermans et al., 2019). DSD encompass a wide spectrum of diagnoses with varying degrees of “chromosomal, gonadal, or anatomic sex development [atypia]” (Hughes et al., 2006). The diagnosis is substantial enough that 1 in 2,000 babies are recommended for surgical intervention (HRW, 2017). As such, understanding the medical situation of each individual is of the utmost importance. Unfortunately, the wide spectrum of DSD complicates streamlining treatment and prevents a singular protocol from being established. 

 

The decision-making process for the treatment of infants with DSD has evolved alongside the adoption of “shared decision-making” (SDM). Historically, parents of the infant consented to the recommendations of the physician based on the limited information provided (Karkazis et al., 2010). The standard of care was ubiquitously surgical reconstruction in attempts to “fix” the abnormalities (Chawla et al., 2019; Crissman et al., 2011). These surgeries were oftentimes irreversible and of questionable necessity, and were thus increasingly seen as controversial, sparking a change to an approach rooted in the principles of SDM rather than physician opinions (Sandberg et al., 2019; Crissman et al., 2011).

 

In 2006, the U.S.-based Lawson Wilkins Pediatric Endocrine Society and the European Society for Paediatric Endocrinology joined efforts to publish a set of recommendations for the treatment of DSD: the 2006 Consensus Statement (Hughes et al., 2006). In this paper, the authors will discuss the current SDM process, medical interventions, psychological implications, and ethical conversations involving individuals with DSD. Considering these factors and recent advancements, the authors will highlight areas for improvement and propose next steps in improving care for individuals with DSD. 

Shared Decision-Making and the Process for Making Treatment Plans for DSD

SDM involves the healthcare team and the patient cooperating to make an informed decision that reflects optimal clinical management and individualized patient preferences (Sandberg et al., 2019). Studies recommend that once a diagnosis of DSD is suspected, a multidisciplinary team of surgical pediatric subspecialties, psychologists, pediatricians, adult endocrinology and fertility specialists, and social workers should be assembled (Crissman et al., 2011; Karkazis et al., 2010). For effective patient communication, a coordinator and lead physician should also be designated as liaisons on behalf of the team (Karkazis et al., 2010).

 

Another crucial step is confirming the diagnosis, as it guides gender assignment and the potential need for surgical intervention. Karyotyping, abdominal ultrasound, urinalysis, and hormone/electrolyte levels are the gold standards for diagnosis (Hughes et al., 2006). With an established diagnosis, the team can discuss gender assignment based on precedents from prior cases. For example, greater than 90% of individuals diagnosed with a 46,XX congenital adrenal hyperplasia (CAH) identify as female, which supports guidelines towards raising the child with CAH as female (Hughes et al., 2006). However, not every diagnosis has a clear recommendation. With partial androgen insensitivity syndrome (PAIS), there is “dissatisfaction with the sex of rearing in 25% of individuals whether raised male or female” (Hughes et al., 2006).

 

Regardless of the diagnosis, the SDM process warrants substantial attention, particularly when considering irreversible surgery. With imminent threats to the infant’s health, such as potential malignancy through dysgenetic gonads, there is a consensus that surgical intervention is required (Timmermans et al., 2019). In all other cases of non-emergent reconstructive surgeries, the conversation has shifted. The traditional approach to cosmetically “fix” the infant faces increased opposition as recent data found that after surgery, both the child and parent continue to face new challenges throughout development, including considerable harm from attitudes sparking embarrassment and silence about their condition, rather than fostering acceptance (Crissman et al., 2011; Karkazis et al., 2010; Reis, 2019).

 

Evidence on Effectiveness and Impact of Current Medical Treatments

Current literature reveals two major potential complications of DSD: the development of malignancy and urogenital symptoms. Regarding malignancy, the 2006 Consensus Statement provided three potential recommendations: immediate gonadectomy, close monitoring with possible biopsies, or no current recommendation (Hughes et al., 2006). In 2020, a more recent and large-scale study in Europe also documented increased malignancy risks but with some disagreement (Table I) (Slowikowska-Hilczer et al., 2020). A key negative implication of gonadectomy is the resulting infertility. Only 10% of patients with DSD reported having at least one biological child; the vast majority were XX females diagnosed with CAH and Klinefelter males (XXY) while less than 1% of all other patients with DSD were able to conceive (Slowikowska-Hilczer et al., 2017). Despite these statistics, there is a documented presence of germ cells in patients with various DSD (Finlayson et al., 2017). The seeming discrepancy may be explained by high gonadectomy rates among all patients with DSD (47.2%), especially androgen biosynthesis defects (97%) (Slowikowska-Hilczer et al., 2020). The rate of gonadectomy in patients with these is especially concerning given that neither the 2006 Consensus Statement nor the incidences published in 2020 indicate immediate surgical intervention.

 

Table 1. Comparison of Gonadal Neoplasia Incidences

2006 Consensus Statementf 

Disorder 

Malignancy Risk (%)

Patient (n)

Recommendation

GDa(+Y)

15-35

>350

Gonadectomy upon Diagnosis

PAIS b

50

24

Gonadectomy upon Diagnosis

Fraser Syndrome

60

15

Gonadectomy upon Diagnosis

Denys-Drash (+Y)

40

5

Gonadectomy upon Diagnosis

Turner (+Y)

12

43

Gonadectomy upon Diagnosis

17-HSDc

28

7

Careful Monitoring

CAISd

2

55

Careful Monitoring

Ovotestis DSD

3

426

Careful Monitoring/Testis Removal

5α-Reductase

0

3

Unresolved

       

2020 dsd-LIFE Study g

Disorder

Malignancy Risk (%)

Patient (n)

 
GDa

26

69

 
 PAISb

0

24

 

Androgen Biosynthesise

0

16

 

CAISd

6.3

32

 

Ovotestis DSD

25

4

 

Hypospadias

20

5

 

Klinefelter (XXY)

14

7

 

a Gonadal Dysgenesis

b Partial Androgen Insensitivity

c Hydroxysteroid Dehydrogenase Deficiency

d Complete Androgen Insensitivity Syndromee

e Includes Hydroxysteroid Dehydrogenase Deficiency and 5α-Reductase

f Hughes et al., 2006

g Slowikowska-Hilczer et al., 2020

The 2006 Consensus Statement also addressed concerns over lower urogenital symptoms. Feminizing surgery should only be considered in cases of severe virilization (Prader III-V) and those that present with confluence establishing fistulas for urine to the vagina/perineum and associated increased complication risks (Hughes et al., 2006). Similarly, reconstruction of hypospadias should progress to establish proper urine and seminal flow, however no firm recommendation for the removal nor preservation of a possible utriculus or Mullerian remnant was made (Hughes et al., 2006). Results of postoperative urinary continence and related secondary complications have been positive for females but not for males (Table II). The sexual function and cosmetic satisfaction for one-stage feminizing genitoplasty and hypospadias repairs have both seen positive outcomes (Nidal et al., 2020; Vandendriessche et al., 2009).

Table 2. Summary of Post-Operative Urogenital Complications

Over virilized Females a
Surgical TechniquePrader ScoreContinenceContinuous IncontinenceOther Urinary SymptomatologyNumber of Patients
Total Urogenital MobilizationIV/V42410
Pull-throughIV/V7029
Pull-through with Partial Urogenital MobilizationIV/V1012
Partial Urogenital MobilizationIII/IV3003

Male Hypospadias Repair b

Total Initial Patients: n=115                                   

Long-Term Patients: n=26c

         Initial Complication Rate: 57.2%                               

      Ongoing Fistula: 11%

         Fistulas: 55.7%                                                

      Urethral Stricture: 10%

        Other Major: 2.6%

      Persistent Chordee: 29%

 

      Unable to Void While Standing: 7%

 

      Difficulty Starting Stream: 52%

 

      Pain During Intercourse: 3%

a Table synthesized from Stites et al., 2016

b Table synthesized from Hoag et al., 2013

c Long-Term Patients could self-report more than one issue

 

Psychological Outcomes of Early Surgical Intervention for DSD

Besides the medical reasons above, early surgery has been the consensus due to the assumed negative psychological effects of growing up with phenotypically ambiguous genitalia (Hughes et al., 2006). However, there is growing concern among clinicians that such surgical intervention can contribute to psychological harm to the individual later in life. Many studies on the experiences of people with DSD show that the prevalence of psychiatric disorders remains high. One U.S. adult study reports that 53.6% of people with DSD (n=198) described their mental health status as fair or poor compared to 17.7% in the general population (Rosenwohl-Mack et al., 2020). Furthermore, 40.9% of people with DSD reported experiencing PTSD and 31.8% reported a prior suicide attempt (Rosenwohl-Mack et al., 2020). Likewise, a meta-analysis showed that 16 of the 18 existing studies on psychiatry/psychology related to DSD found increased psychiatric disorders among persons with DSD, with the most common disorder being anxiety, especially among people with CAH (Bohet et al., 2019). The disproportionate incidence of psychiatric disorders among people with DSD suggests a strong need for continuous psychiatric treatment, regardless of whether the individual has had prior surgery.

 

The literature on the psychological impact of early surgery remains sparse with limited statistical evidence or longitudinal study to claim adverse or beneficial psychological effects. People with CAH (n=16) or AIS (n=8) who had early feminizing genitoplasty had better mental health and comparable quality of life to the control group, with the non-significant exception of some people with DSD reporting lower sexual function (Fagerholm et al., 2012). Additionally, 90% of people with CAH believed genitoplasty should be done within the first year of life compared to only 52% of the control group, as a majority (85.7%) of those who underwent surgery (n=21) had gender identity concordant with their sex assignment (Binet et al., 2016). Conversely, other studies among people who had early surgery (n=59) found that adverse psychiatric disorders developed in 39% including gender dysphoria in 13% of the sample (Slijper et al., 1998), compared to 5.2% and 0.002-0.014% in the general U.S. population respectively (Kyriakou et al., 2020; NIMH, 2021). Many people who undergo surgery also described experiencing “shame, sexual dysfunction, gender dysphoria, the feeling of betrayal, and devaluation” which can be compounded with anger and symptoms of trauma if the parents and/or physician withheld information or espoused a feeling of silence and secrecy around their sexuality (Lev, 2006). These psychological issues were twice as likely in those without psychotherapy at the time of diagnosis (Slijper et al., 1998). Lastly, some studies have found that while people with DSD (n=91) experienced significant sexuality-related distress (40%) and sexual dysfunction (66%), sex assignment surgery during infancy or lack thereof did not seem to affect psychosexual development (Callens et al., 2012). Thus, the literature remains widely inconclusive on the psychological effects of early surgery among people with DSD but does indicate potential risks and highlights the need for psychological management.

Ethical Implications and Current Debate on Early Surgical Treatment for DSDs

Discussing the intricacies of treating DSD also requires ethical considerations, which are grounded in autonomy and informed consent. Modern notions of autonomy and consent began with the establishment of the Nuremberg Code in 1947, and informed consent was firmly established as a medical-legal requirement in the US in 1957 (Reis, 2019). However, the application of this law has contained many ambiguities. In Salgo v Leland Stanford Jr. University Board of Trustees, Justice A. Frank Bray made physicians legally obligated to provide their patients with all information necessary to provide consent, but also granted them power and discretion over that information (Reis, 2019) which essentially gave physicians power over their patients. This unequitable power was solidified in the 1960s by Dr. John Money who established the norm of performing surgical genital reconstruction of babies with DSD (HRW, 2017). Despite many subsequent debates and developments in both the scientific and legal sectors, the precedent set by Salgo v. Leland and the standard of care established by doctors like John Money created the ethical scenario at the forefront of modern debate on DSD treatment.

 

As pediatric patients cannot consent to medical treatments for themselves, their parents must do so for them–a scenario that is especially complicated when the decisions carry immense physical and psychological consequences with highly uncertain outcomes. Scholars question whether parents even can legally make decisions about children’s surgeries if they affect a constitutionally protected right, such as the right to privacy or reproductive freedom (Greenberg, 2017). Autonomy, ethics and human rights law mandate protecting “the child against having important life choices determined by others before she has the ability to make them for herself,” which provides a legal framework for delaying treating DSD (Millium, 2014). Furthermore, because many DSD treatments require lifelong therapies, parental decisions to treat DSD infringe on the autonomy of both the baby in front of them and the adult who may face continued medical interventions to maintain that initial treatment. In other complicated cases of highly invasive procedures on pediatric patients, a court-order that authorizes the medical intervention has been required. This legal backstop may be applicable to the surgeries performed on babies with DSD (Greenberg, 2017).

 

Finally, having a child with medical complications can produce emotions that may cloud judgment making parents’ decisions susceptible to stereotypes and social norms (Greenberg, 2017). Notably, many of the social norms that impact early parental decision-making are also likely to become obsolete by the time the child is a teenager or adult.

 

It is also necessary to question the capabilities of medical teams to effectively communicate and bolster autonomy by ascertaining that parents fully understand their child’s condition and the implications of every treatment option. Parents need time to gain knowledge and understanding of their child’s diagnosis before being fully capable of participating in an SDM process–a need that is often incompatible with the timeline of most surgical decisions (Greenberg, 2017). Current evidence suggests that physicians are not capable of these tasks (Reis, 2019). Many parents who recounted their experiences of medically managing their children with DSD explained that physicians “pressured them to undertake irreversible procedures, including surgery” and “made them feel they were being unreasonable when they resisted or asked questions” (HRW, 2017).

 

Many governments, private organizations, and other groups have already taken a stand on surgical treatment for DSD, shaping the modern ethical conversation by asserting that surgical procedures on children with DSD should be delayed until they are fully capable of giving informed consent (Table III). In the US, however, no laws specify treatment guidelines for children born with ambiguous genitalia (Greenberg, 2017). In 2016, the National Institutes of Health did designate the intersex community as a health disparity population, while the United States Bureau of Public Affairs asserted that surgeries performed on children with DSD without their fully, consciously given consent would “jeopardize their physical integrity and ability to live free” (Ejiogu, 2020; Greenberg, 2017). Finally, in 2017, three former US surgeon generals wrote about the lack of evidence that children with atypical genitals face substantial psychological distress and took the stance that surgical treatment is not only unnecessary but causes appreciable harm and should be avoided and replaced by other forms of support such as education and mental health resources (HRW, 2017).

Table 3. Timeline of Significant Political and Organizational Statements Against Infant DSD Surgical Treatment

Year

Event

2005

San Francisco Human Rights Commission Report cautions against ‘normalizing’ infant genital reconstructive surgeriesa

2011

The first ever Intersex Forum takes place in Brusselsb

2012

German Ethics Council concludes that “irreversible medical sex assignment measures in persons of ambiguous gender infringe the right to physical integrity, to preservation of sexual and gender identity, to an open future and often also to procreative freedom” and states that they need to be delayed until children are of consenting age.c

2012

Swiss Bioethics Commission concludes that surgeries on nonconsenting infants with DSDs violate patients’ human rights and integrity.c

2014

The American Academy of Pediatrics “Provisional Section on Lesbian, Gay, Bisexual, and Transgender Health and Wellness” publishes a statement that “If it is not medically necessary, any irreversible procedure can be postponed until the child is old enough to agree to the procedure (e.g. genital surgery),” marking their first statement that directly opposes early DSD surgery. b

2013

Australian Senate Community Affairs References Committee emphasizes the need for human rights considerations when treating babies with DSDs, including delaying the procedures until the patient is of consenting age while reducing surgical interventions overall.c

2013

The United Nations special rapporteur on torture classifies nonconsensual genital normalizing surgery on intersex children as a form of ill-treatment, and says that such surgeries “often… arguably meet the criteria for torture, and they are always prohibited by international law.” b

2015

Malta’s parliament passes new legislation that outlaws “avoidable” surgery on babies with DSD and becomes the first country to fully ban such surgeries.a

2016

National Institutes of Health (NIH) formally designates the intersex community as a health disparity population.d

2016

The United States Bureau of Public Affairs declares that performing surgeries on children with DSDs without their consciously consent will “jeopardize their physical integrity and ability to live free.” c

2016

The American Medical Association issues a statement that barring life-threatening circumstances, performing surgeries to treat DSD should be delayed until the child is able to consent.b

2017

Human Rights Watch and InterAct call for complete cessation of any genital or gender-altering surgical treatment on infants with DSD.b

2017

Three former U.S Surgeon Generals wrote about the lack of evidence that children with atypical genital appearances face substantial psychological distress and took the stance that surgical treatment is not only unnecessary, but causes appreciable harm and should be avoided and replaced by other forms of support such as education and mental health resources.b

2021

California State Senator Scott Weiner submits State Bill 225 to provide restrictions on surgical treatments for young children with DSDs to protect their rights and reduce unnecessary treatment.e

a Reis, 2019

b HRW, 2017

c Greenberg, 2017

d Ejiogu, 2020

e Weiner, 2021

Today, progress continues in the conversation about ethical treatment of children with DSD. Earlier this year, Senator Scott Weiner of California introduced Senate Bill 225, which “would prohibit a physician and surgeon from performing certain sex organ modification procedures on an individual born with variations in their physical sex characteristics who is under 6-12 years of age unless the procedure is a surgery required to address an immediate risk of physical harm” (SB-225).

Modern Medical Developments and Implications for the Future

The medical field is becoming increasingly aware of the issues surrounding surgical intervention on children with DSD. Fortunately, this awareness has led to new social and governmental policies and increased innovation in surgical techniques that strive to respect an individual’s rights to their future. The innovations have made great progress in preserving sexual structures and fertility while providing surgical urogenital sinus correction and prophylaxis against malignancy.

 

Current genital correction surgeries are successfully preserving both female and male structures. Persistent Mullerian Duct Syndrome is characterized by the presence of rudimentary uterine structure, cryptorchidism, and a virilized external genitalia. Usually, supracervical hysterectomy is performed in order to progress with a bilateral orchiopexy; however, a novel procedure utilizes a different approach to achieve orchiopexy without hysterectomy (Bowen et al., 2015). The orchiopexy is medically necessary to discourage the development of malignancy; however, the preservation of uterine tissue maintains the child’s rights to a self-determined future regarding gender identity. A hypospadias repair with conservation of a perineal utricle/blind vaginal pouch also achieves the necessary treatment of urogenital complications while facilitating a delayed future gender assignment surgery that coordinates with the patient’s identity. Long-term follow up in two patients who underwent this surgery was very positive—both self-identified as males, reported normal male voiding, erections, and did not show any signs of gender dysphoria (Wolffenbuttel and Holmdahl, 2019).

 

There has also been increasing progress in the fertility treatment of patients with DSD. While still experimental, the use of cryopreservation of immature tissues demonstrates great promise. Additionally, the potential confirmation and regular implementation of cryopreservation may become another indication for early prophylactic gonadectomy or biopsy, as the presence of germ cells was shown to decrease with age in patients with DSD (Finlayson et al., 2017). This fertility preservation respects the rights and future decisions of patients with DSD.

Conclusion

While the medical studies discussed in this review demonstrate progress in surgical techniques and outcomes, data is still extremely limited and sample sizes have been small. There is also additional concern surrounding the high rate of complications in surgical management of DSD, especially conditions such as hypospadias, and a lack of understanding on the risks of malignancies in untreated DSD. As such, the authors first highlight the need for continued development of surgical techniques and practices to avoid complications in DSD treatment. Regarding research into the psychosocial impacts of surgical DSD treatment, sample sizes have also been small, and the generalizability of such studies is limited by the diverse sociocultural and geographic context in which they were conducted, which is especially significant because social context is a strong predictor of well-being for people with DSD. Selection bias in these studies, which were largely based on voluntary participation and had response rates of 52-75% may have impacted the data, as people with positive treatment experiences with early surgery may have been more likely to respond (Binet et al., 2016; Callens et al., 2012). Finally, most current psychologically based studies interviewed people or parents of those who had undergone early surgery anytime between 1970 and 2014, making it difficult to compare individuals’ experiences and outcomes across time (Binet et al., 2016; Wolffenbuttel and Holmdahl, 2019). The authors thus stress continued, and more robust analyses modelled off the presented studies so that larger samples and a more modern data set can inform future practice.

 

In concert with supporting continued advances in the medical field, it is also crucial to continue advancing our understanding of the psychological impact that various treatments have on children with DSD and combining this with considerations from modern ethical conversations. It is clear that the existing SDM model, which disproportionately gives power to physicians and sways with social norms, is inadequate for fostering effective decision-making. Therefore, the authors call for continued reform of this SDM process to meet modern advances in both ethics and medicine. This may involve drawing on modern ethical principles such as ensuring the child’s autonomy and legal rights when making decisions, as well as steering treatment towards new medical developments that focus on clinically necessary elements of DSD while preserving the child’s potential for self-determination in gender, reproduction, and other areas. Finally, there are many smaller ways that physicians in particular can participate in the efforts to improve treatment of DSD, such as educating co-workers through grand rounds presentations, performing DSD-oriented research projects, and helping connect both parents and children with DSD to other families, thus providing a support network and empowering the patients themselves (Ejiogu, 2020; HRW, 2017).

Disclosure Satement

The author(s) have no relevant financial disclosures or conflicts of interest.

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About the Author

Thomas Chameli, BS

Thomas Chameli is an MD candidate at Georgetown University School of Medicine. His research areas include Gynecological Surgery and Laparoscopy. He received his Bachelor of Science in Chemistry and Psychology at Canisius College.

Sarah Heins, BA

Sarah Heins is a second-year medical student at the Georgetown University School of Medicine. Her research areas include the intersections between Humanities, Literature, Medicine, and Healthcare Ethics. She received her Bachelor of Arts at the College of William and Mary.

Thu Dao, BS

Thu Dao is an MD candidate at Georgetown University School of Medicine. Her research areas include maternal health and social medicine. She received her Bachelor of Science in International Health at Georgetown University.

Erica Choe, BS

Erica Choe is a second-year medical student at Georgetown University School of Medicine. Her research areas include population health and oncology. She received her Bachelor of Science in Biochemistry from Loyola Marymount University. 

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