Evolving indications for surgical intervention in patients with differences/disorders of sex development: Implications of deferred reconstruction.

Rare medical conditions are difficult to study due to the lack of patient volume and limited research resources, and as a result of these challenges, progress in the care of patients with these conditions is slow. Individuals born with differences of sex development (DSD) fall into this category of rare conditions and have additional social barriers due to the intimate nature of the conditions. There is also a lack of general knowledge in the medical community about this group of diverse diagnoses. Despite these limitations, progress has been made in the study of effective ways to care for patients who are born with chromosomal or anatomical differences of their internal reproductive organs or external genitalia. Advocacy groups have placed a spotlight on these topics and asked for a thoughtful approach to educate parents of newborns, medical providers, and the adolescents and young adults themselves as they mature.1 There is growing interest in the approaches to surgical reconstruction of the genitalia and the management of internal gonads, specifically the timing of procedures and the indications for those procedures.2 Advocates suggest deferring surgical procedures until the affected individual can participate in the decision-making process. This approach requires a roadmap for addressing the long-term implications of delayed surgical management. Presented here is a review of the specific issues regarding the complex management of the various categories of DSD.

Ethical issues with early genitoplasty in children with disorders of sex development.

PURPOSE OF REVIEW: Genitoplasty in children with disorders of sex development (DSD) is an ethically complex issue. From a surgical perspective, genitoplasty in early childhood is preferred because it is felt to be associated with improved tissue healing, decreased risk of complications, and reduced psychological impact of genital surgery. However, advocacy groups and recent ethics literature have argued for deferring genitoplasty until a child reaches decisional maturity. This article reviews these arguments using an ethical framework and discusses the application and challenges of recent disorders of sex development research. RECENT FINDINGS: Recent ethics literature and advocacy groups have argued for deferring genitoplasty until a child reaches decisional maturity. As a counterpoint, urological societies have published arguments supporting the practice of early genitoplasty. Data from DSD research lends some guidance but also has a wide range of outcomes, which makes generalizability difficult. A retrospective, multicenter study of 21 individuals with congenital adrenal hyperplasia who underwent feminizing surgery showed no difference between cases and controls in social functioning, parent-child relationships, or sexual fulfillment. Ninety percent of patients thought genitoplasty should occur within the first year of life. In a study of 52 patients with 46,XY and 46,XX DSDs who underwent masculinizing genitoplasty, 57% thought their physical appearance was 'fair' or 'poor,' and problems with sexual function, urinary incontinence, and short penile length were common. SUMMARY: Early genitoplasty in children with DSDs is ethically complex, and discordant results in DSD research makes generalizability difficult. There is unlikely to be a universal solution to the issue of early genitoplasty in children with DSDs; families must be supported while they weigh both parental decision-making and the objective of ensuring an open future for their child.

Intersexual Births: The Epistemology of Sex and Ethics of Sex Assignment.

This article aims to analyse a possible manner of approaching the birth of intersexual children. We start out by summing up what intersexuality is and how it is faced in the dominant clinical practice (the "treatment paradigm"). We then argue against this paradigm, in favour of a postponement of genital surgery. In the second part of this paper, we take into consideration the general question of whether only two existing sexes are to be recognized, arguing in favour of an expansion of sex categories. In the third part, we illustrate the reasons supporting provisional sex attribution: the child's best interest and respect for their developing moral autonomy. This position aims to increase the child's well-being and self-determination, limiting parents' freedom to take decisions on behalf of others, in particular, those decisions concerning basic aspects of their children's personal identity.

Intersex Activists in Israel: Their Achievements and the Obstacles They Face.

This article focuses on the dynamic between the medical policy on intersex bodies and intersex activists in Israel. Recently, in many countries changes have taken place in medical guidelines regarding intersex patients and laws that regulate medical practices and prohibit irreversible surgeries for intersex babies for cosmetic reasons and without the patient's consent. In Israel, intersex activists are limited by several factors. On the one hand, they are influenced by the achievements of intersex activism around the world but on the other, the pathologizing medical discourse and socio-medical practices, which include early diagnosis, early irreversible surgeries, and secrecy surrounding intersexed bodies, present obstacles to achieving bodily autonomy for intersex individuals and social recognition of different sex development. Nevertheless, intersex activists are attempting to find different social and media spaces in which to achieve public acknowledgement and future bodily autonomy for intersexed people and seeking medical professionals' cooperation. Recently, the Israeli Ministry of Health published a new circular for intersex/DSD patients, and while it does not clearly forbid irreversible surgeries, it provides information about the complexities of intersex people and their experience.

Management of intersex newborns: Legal and ethical developments.

Countries worldwide are increasingly expanding male/female binary sex classifications to recognize a third status. Intersex newborns may be included in this third category on birth certification. Parents, families, and communities require counselling and education to accommodate intersex newborns without stigma or discrimination. Whatever its biological or genetic origin, intersex status is a natural if relatively uncommon condition (one in 1500-2000 live births) that distinguishes sex from gender. The tendency of societies to recognize only male and female genders at birth has resulted in intersex children being subjected to invasive surgery and related, sometimes lifelong, medication to confirm them as male or female. On gaining maturity, some are severely distressed and resentful that early gender assignment was mistaken, particularly when excision of testes to enforce femininity or of ovaries to enforce masculinity has denied them procreative capacity. Emerging principles support postponement of such interventions until intersex individuals can make a gender choice for themselves.

Legal, ethical, and human rights considerations for physicians treating children with atypical or ambiguous genitalia.

Some governments, human rights organizations, intersex organizations, and doctors have called for a moratorium on genital and gonadal surgeries for infants born with atypical or ambiguous genitalia. Moratorium supporters believe that the surgeries carry physical and emotional risks, the psychosocial benefits of these procedures have not been proven, and the surgeries violate the patients׳ fundamental human rights if they are performed before these patients can provide informed consent. Given these calls for a moratorium, treatment teams must determine how to treat their patients and how to counsel their patients׳ parents. This article examines the treatment teams׳ ethical and legal responsibilities and provides advice for treatment teams to follow that will protect their patients and their practices.

Sexual Enhancement Treatment for Sexually Violent Predators: Ethical and Legal Implications.

The treatment of sexually violent predators (SVPs) has been controversial since the implementation of sexual predator laws and statutes that seek to balance the civil rights of SVPs and the protection of potential victims. In this paper, we will present the case of an SVP who sought sexual enhancement surgery in order to perform intercourse with his wife. We will also discuss the conflicting legal and ethical issues affecting a decision to implement sexual enhancement therapy for SVPs in an effort to reconcile these divergent positions.

Ethical issues in decision-making for infants with disorders of sex development.

Medical decisions for infants and children should generally be based on the best interests of the child. When there is legitimate controversy over the child's best interests, the right of the child to an open future should generally determine the course of treatment. In the case of infants born with disorders of sex development (DSD), early cosmetic genitoplasty was long believed to be in the child's best interest and was therefore the standard of care. New data suggest that early genitoplasty may be more harmful than helpful, therefore the best interest standard is no longer determinative in such cases. Because children born with DSD have a right to an open future, and because the openness of their future is clearly enhanced by delaying cosmetic genitoplasty until they themselves can participate meaningfully in decision-making, early genitoplasty is ethically supportable only when medically indicated (e.g., when the child is unable to urinate without surgical intervention). Further research is needed to clarify the benefits and burdens of early and delayed genitoplasty. In parallel with further research, efforts should focus on educating society broadly to decrease stigmatization of persons with DSD.

Evidence regarding cosmetic and medically unnecessary surgery on infants.

The Journal of Pediatric Urology has recently published several articles from the Annecy (France) Working Party on DSD. We question several of the presented findings and recommendations. In two key articles summarizing their review, the authors concluded that identified studies are not representative and suffer from methodological weaknesses, such that they "lack the necessary detail to base further recommendations". In a third article, the Working Party reported that the science supporting early surgery is "scanty", and that "no studies" support the belief that gender variant children require early genital surgery. Nevertheless, the Working Party warned that without long-term research, "if no effort is made, we will be left, in the next generation, to continue making the same judgment, based on 'experience' and 'expert opinion'". None of the studies cited in the articles support such assertions as we read them. We maintain that reviewed evidence suggests a moratorium on early surgical intervention is imperative for children with differences in sex development, and that the best ethical and scientific considerations require that gender surgery should be delayed until the child can consent. We further present evidence that UN and case law presently under way in the USA support such a moratorium.

Ethical and legal aspects of management for disorders of sex development.

Intense controversy surrounds the management of disorders of sex development, particularly in relation to the validity of parental consent for genital surgery and the removal of gonadal tissue carried out during infancy or childhood. Past practices have been heavily criticised on ethical grounds by patient advocacy organisations, who have demanded a moratorium on these kinds of operations unless authorised by a court. Some doctors and hospital administrators have been influenced by the controversy and have referred cases to the Family Court of Australia, where a series of judgements have now established legal precedents that apply across Australia, restricting the circumstances in which parents can give consent for surgery. An alternative approach is to use a hospital-based Clinical Ethics Response Group and, if necessary, Clinical Ethics Committee, which has lay and legal representatives as well as health professionals, as a semi-independent committee of review. Finding a solution that protects the human rights and best interests of children is an ongoing challenge.

Lessons from the past: directions for the future. Do new marketed surgical procedures and grafts produce ethical, personal liability, and legal concerns for physicians?

New procedures and materials for incontinence and prolapse are proliferating rapidly. Surgical procedures were developed by physicians and carried their names, but over the last 15 years, these procedures are developed by industry and bear the trade names of the companies selling the kits needed to perform them. The Food and Drug Administration (FDA) approves devices, not procedures, and does not require submission of efficacy or adverse-event data to gain this approval by the 510-K process. Evidence-based medicine is lacking in the performance of these procedures that may be considered experimental by an insurance company or malpractice carrier with denial of payment or coverage. Physicians and hospitals are exposing themselves to financial, legal, and ethical risks when performing or allowing such procedures to be performed. Informed consent from the patient cannot be obtained. We must not confuse medical marketing with evidence-based medicine.

Ethics and the proposed treatment for a 13-year-old with atypical gender identity.

The case of a 13-year-old girl given permission by the Family Court of Australia to begin a sex-change process involves complex issues. Nevertheless, the ethical justification for the decision is not complicated. In this case, it can be argued that the net benefit eclipses concerns about competence, autonomy and the appropriateness of the intervention. The debate this case generated in the media reminds us that one of the essential tasks in ethics debates is to get our facts straight.