572454 research-article2015
HPQ0010.1177/1359105315572454Journal of Health PsychologyMachado et al.
Review
Follow-up of psychological outcomes of interventions in patients diagnosed with disorders of sexual development: A systematic review
Journal of Health Psychology 1–12 © The Author(s) 2015 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/1359105315572454 hpq.sagepub.com
Paula Sandrine Machado, Angelo Brandelli Costa, Henrique Caetano Nardi, Anna Martha Vaitses Fontanari, Igor Rabuske Araujo and Daniela Riva Knauth
Abstract The objective of this study was to perform a systematic review of articles that followed up the psychological outcomes of interventions in patients with disorders of sexual development. The analysis focused on the methodological quality of the studies and the assumptions on the evaluation of the intervention outcomes. The review signaled the need for better consideration about the complexity of intersexuality and for a shift from the anatomical/functional/aesthetic to the subjective outcomes related to patient’s quality of life. Furthermore, it is suggested that the interventions should be better investigated, given the lack of evidence for the necessity of early procedures.
Keywords disorder of sexual development, follow-up, intersexuality, psychological outcomes, systematic review
There are a considerable number of diagnostic and evaluation criteria coming from genetic, endocrinological, and surgical points of view involved in what medical practitioners label disorders of sexual development (DSDs). The endorsement of this terminology is a product of a gathering of specialists that resulted in what is known as the Chicago Consensus that modify and unify practices, protocols, and terminology that were being used in a nonuniform way to that point (Lee et al., 2006). However, even today, numerous questions remain unanswered in relation to interventions in such conditions, such as which is the most
appropriate period for surgical intervention; who should make the final decision about the person’s sex; are the procedures necessary; moreover, which are the subjective, social, ethical, and political effects of the interventions? (Boyle et al., 2005; Cohen-Kettenis, 2005; Kessler, 1998; Machado, 2009) Universidade Federal do Rio Grande do Sul, Brazil Corresponding author: Paula Sandrine Machado, Department of Social Psychology, Universidade Federal do Rio Grande do Sul, Ramiro Barcelos, 2600, 300h, Porto Alegre, RS, Brazil. Email: [email protected]
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Recently, the World Health Organization (WHO) released an interagency statement (Office of the High Commissioner for Human Rights (OHCHR), United Nations (UN) Women, Joint United Nations Programme on HIV and AIDS (UNAIDS), UN Development Programme (UNDP), UN Population Fund (UNFPA), UN Children’s Fund (UNICEF), and WHO) affirming that “intersex persons may be involuntarily subjected to so-called sexnormalizing or other procedures as infants or during childhood, which, in some cases, may result in the termination of all or some of their reproductive capacity” (WHO, 2014: 7). That statement also refers to the lack of consideration of the views of the children and of their parents, given that those interventions could have “lifelong consequence for their physical and mental health” (p. 7). Anchored in international human rights bodies and national courts, the WHO (2014) uses terms such as “involuntary” and “coercive” to label those practices. Two systematic reviews about the outcomes of interventions in intersexual conditions were located. The first (Santos and Araújo, 2008) concludes that it is necessary to gather greater theoretical background in the discussion of medical interventions outcomes, in addition to the deployment of more longitudinal studies. The second review (Schoenbucher et al., 2010) focused on the sexual life quality of individuals with a 46 XY karyotype, who experienced DSD. The article concludes that there are inconsistent results regarding sexual satisfaction, indicating that future studies should focus more on qualitative aspects of sexuality. Nevertheless, both studies seem to lack in methodological quality for conducting systemic reviews. One of the main limitations on follow-ups to surgical interventions and its assessments is that the majority of the studies in the field are guided by a biomedical perspective (Roen, 2009). The core presumption of this perspective is that an adequate intervention would lead to a positive psychological effect on the subjective wellbeing over one’s body. This point of view does not consider the socio-political aspects related to the interventions, meaning that it ignores
intersex people’s lived experience and social insertion. Moreover, when aspects that are more “psychological” are evaluated, the researchers tend to determine the relevant indicators, mainly over global evaluation judgments regarding the well-being of the populations under study (Karkazis, 2008). There is no doubt that there is still much ground to advance in studies designed to longitudinally evaluate features such as body image, satisfaction with body and genitals, sexuality, overall health, and, in a broader sense, one’s quality of life (Warne, 2008). Aiming to discuss some of the arguments presented here, we propose a systematic review of studies that specifically evaluate the longitudinal psychological outcomes as a result of interventions undergone in patients diagnosed with DSDs.
Methods To identify studies that investigated the longterm psychological outcomes of surgical and hormonal intervention in DSD patients, a search of Scopus and Web of Science databases was performed on October 2013. Aimed at covering the wide range of conditions related to intersexuality, we used the search term “Disorders of Sexual Development” under the Medical subject heading (MeSH). The search strings consisted of each one of the terms listed under DSD AND “Follow up OR Followup OR Follow-up OR Long-term OR Long term.” The search returned 5199 articles. Duplicates, of which there were 1799, were removed. The titles and abstracts of 3400 studies were independently analyzed by three reviewers, who applied the following inclusion criteria: (a) being a long-term follow-up study (1 year minimum); (b) focusing primarily on surgical and/or hormonal interventions in at least one DSD—only interventions upon the sexual characteristicswere taken into account, due to the fact that the main focus of this review is the understanding of the psychological outcomes of sexual assignment; (c) investigating psychological outcomes from the point of view
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Machado et al. of the subject (e.g. quality of life, sexual satisfaction, sexual functionality, aesthetic result of the external genitalia, etc.); (d) primarily focusing on the patient (not the service, physician, family, etc.); (e) being published after the Chicago Consensus (2006); (f) being a full article published in indexed journals; and (g) being written in English. According to these criteria, 106 articles were selected. These were then re-examined and screened by the three independent judges under stricter exclusion conditions, paying particular attention to (a) omitted information we considered essential (age of the first intervention, time of the follow-up) and (b) being a retrospective rather than a prospective follow-up—retrospective studies were excluded because they use indirect data, making it very difficult, and sometimes impossible, to measure certain factors accurately (Lilienfeld and Stolley, 1994); (c) not being focused on a DSD. Even though we were based on MeSH terms, papers reporting unlisted DSDs such as Mayer–Rokitansky–Küster–Hauser Syndrome were not excluded due to the fact that they followed the same clinical management, and thus, procedures other than those DSDs specified under MeSH terms. Additionally, the authors related those syndromes to DSDs. The relevant data from these articles were then documented in a spreadsheet, including year of publication, research team composition and nationalities, journal, DSDs, sample size, median sample age at the time of medical intervention, nature of the intervention, investigated outcome, outcome assessment methodology, follow-up time, relevant study results, and highlights. The final pool contained 13 articles (Figure 1). The analysis focused on the methodological quality of the studies and the assumptions on the evaluation of the outcomes.
Results and discussion The majority of the selected articles (Table 1) focused on morphological outcomes, that is, the anatomical–functional aspects of interventions and their complications. Those studies were
published in medical journals and written by physicians mainly specialized in urology. The countries of publication were varied—Eastern and Western both included—but no comparative studies were found. Only one of the articles mentions cultural differences (Merali et al., 2009); however, the acknowledgement of cultural differences was trivial. The sample sizes varied from 1, in a case study, to 182, in a prospective follow-up. Both case studies and case-control studies were present, with a sample size of 38 patients lasting for about 14 years, and only four studies with a follow-up design per se. The follow-up time varied from 1 to 24 years. All articles reported surgical procedures, and although the majority also reported the use of hormonal therapy, the effects were discussed either in a superficial manner or not at all; the focus was the effect of the surgical interventions. Most of the studies involved cases of congenital adrenal hyperplasia, which is considered the most frequent DSD. The results of the systematic review show the existence of a diverse range of methodologies when it comes to evaluating the results of the interventions. Some of the studies that proposed to analyze psychological outcomes were limited to external genitalia evaluation— usually from a medical doctor—which neglected the perspective of individuals who underwent the sex assignment procedures. Few papers displayed results about the psychological outcomes or their relation to well-being or even considered participants’ subjective quality of life. Some studies did not even specify how they measure the outcomes. Generally speaking, the assessment of the psychological outcomes did not make use of reliable indicators regarding the lived experience of the participants and/or even validated measures. It is noteworthy that the article with the most robust method of outcome analysis reached a clear conclusion of dissatisfaction in domains such as cosmetic and functional surgical result, overall genital function, and overall sex life (Köhler et al., 2012). Furthermore, psychological outcomes were often treated a synonym for sexual satisfaction.
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Scopus N=1831
Web of Science N=3368
Potentially relevant references N=5199
Search in the abstracts N=3400
Data extraction N=106
Removed Duplicates N= 1799 Removed Did not meet the inclusion criteria N=3506 Removed Met the exclusion criteria N=93
Final pool N=13
Figure 1. Search strategy.
The articles that proposed to measure sexual satisfaction, when referring to it, were predominantly limited to asking how dilated the vagina was; whether there was the presence of orgasms, lubrication, and pain when ejaculating; capacity to penetrate or to be penetrated; having stable relationships; or the evaluation, by an external observer, of the aesthetic of the genital; and, in fewer cases, how he or she feels concerning the surgery. It is also important to draw attention to the assumption that relates good outcomes to marriage and sex, particularly of a heterosexual nature. As already discussed in the literature, traditional values and beliefs presented in some cultures tend to justify fertility, marriage, and heterosexuality as parameters of good outcomes (Warne and Raza, 2008). In all, 10 articles concluded, using heterogeneous criteria, that the surgery was a success. In most cases, success was defined by the opinion of the team, even when it contradicted the presented data. For example, one emblematic article with “positive results” evaluated, in a prospective follow-up consultation, the surgical results and the sexual satisfaction of 47 patients in an average of 12 years after an intestinal vaginoplasty took place. According to the authors, the result was positive because besides the excellent surgical outcome, 38.3 percent of the sample was sexually active and 8.5 percent was
married. Nevertheless, 17 out of 47 patients had complications from the surgery, such as necrosis of part of the genital, abdominal abscess, and vaginal prolapse (Lima et al., 2010). There is a dissonance between the complications resulting from the interventions and the evaluation of those interventions. In other words, there is a tendency to positively evaluate the interventions despite the absence of established criteria and in the face of contradictory evidence. One of the fundamental issues we have found in the reviewed studies is that numerous premises seem to be self-evident. These include the definition of satisfactory sexuality and what makes a body desirable and normal. Sexual intercourse, predominantly pertaining to successful vaginal penetration, is positioned as central to the concept of satisfaction and wellbeing. This heterosexist default, or what Adrianne Rich (2013) has termed compulsory heterosexuality, is problematic, as it assumes that sexuality is oriented around a “normative” heterosexual conception. Therefore, when female genitalia are evaluated, it is in the sense of being able to accommodate a regular-sized penis. In those cases, alternative sexual orientations or practices are not accounted for, even when sexual satisfaction is evaluated. The success of the surgeries is most of the times evaluated according to measures and data
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Sexual adjustment and long-term results among patients who underwent vaginal reconstruction with free vascular jejunal flap
Evaluation of satisfaction with genital surgery and sexual life in adults with XY, DSDs
The use of sigmoid colon in vaginal reconstruction
Köhler et al. (2012)
Nowier et al. (2012)
Doctor selected ~25 years indicators and provided evaluation criteria. Doctor, patients, and parents provided data. Doctor selected 6 years indicators and provided evaluation criteria. Doctor and patients provided data.
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(Continued)
Dissatisfaction with overall treatment was substantial. Dissatisfaction was high regarding cosmetic and functional surgical result, overall genital function, and overall sex life. Long-term follow-up showed introital stenosis in four cases. Mucosal prolapse occurred in one case. Reoperation rate was 9.6 percent. Sexual satisfaction was achieved by nine sexually active cases. The subjective satisfaction score of the surgical outcome was high.
In total, 11 patients were satisfied with their sexual lives after surgery; 27 were married and sexually active.
Follow-up (time Evaluation of the results and between first salient findings intervention and the outcome)
Doctor selected 50 months indicators and provided evaluation criteria. Doctor and patients provided data.
Average age in the Outcome first intervention assessment methodology
34 women: Mayer– 16–31 years Rokitansky–Küster– Hauser syndrome (29 patients), isolated vaginal agenesis (2 patients), androgen insensitivity syndrome (2 patients), and history of gynecologiconcologic surgery (1 patient) 57 individuals with various Phallus reduction XY, DSDs or correction of hypospadias was performed mostly in childhood and vaginoplasty in puberty. 31 patients with DSDs of 14–28 years various causes
Research question Sample size and DSD
Erman Akar et al. (2013)
Reference
Table 1. Reviews study data.
Machado et al. 5
Lima et al. (2010)
Sircili et al. (2010)
Long-term results of anatomical structure and function of the neovagina after transvestibular vaginoplasty Evaluation of long-term morphological and functional outcomes of masculinizing genitoplasty in patients with DSDs Surgical complications and sexual satisfaction after vaginal replacement in pediatric age
Zhou et al. (2010)
6 (1–47) years: 14 patients underwent the intervention before the first year of life.
23 (19–35) years
Non-specified evaluation criteria; doctor selected indicators and provided data.
12 (1–34) years
Doctor selected 14.1 ± 9.2 years indicators and provided evaluation criteria. Doctor and patients provided data.
According to the authors, 38.3 percent of the sample were sexually active, and 8.5 percent were married. In addition, all patients reported excellent aesthetic results without excessive mucus production or the need for expansion. In contrast, 17 of them had complications.
The result was good, but there was significant sample loss during follow-up. All women were provided adequate counseling on the advantages and disadvantages of the techniques. Most of the patients were sexually active; only 3 percent felt dissatisfied with their sexual life mainly due to small penis and were pleased with both the age of the first intervention and its results.
Follow-up (time Evaluation of the results and between first salient findings intervention and the outcome)
Doctor selected 15 years indicators and provided evaluation criteria. Doctor and patients provided data.
Average age in the Outcome first intervention assessment methodology
47 Rokitansky’s Syndrome “Around puberty” and others in the majority and 11 after puberty
38 DSDs in general
182 Rokitansky’s Syndrome
Research question Sample size and DSD
Reference
Table 1. (Continued)
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Cultural diversity 2 (one from Iceland and in the surgical one from Bolivia). outcome and sexual satisfaction after clitoroplasty in two DSD patients. Problems with 1 17β-HSD-3 deficiency mental health, social adjustment, family relationships, socialization, adaptation to gender in patients with 17β-HSD-3 deficiency subjected to genitoplasty
Merali et al. (2009)
Liakopoulou et al. (2009)
Behavior and emotional problems after vaginoplasty
Oner et al. (2009)
28 CAH, 28 age-matched patients with diabetes mellitus and 28 healthy controls
Research question Sample size and DSD
Reference
Table 1. (Continued)
17 years
Around 17 and 32 years
1–5 years 6–18 years: at childhood in every 3 months and then every 6 months.
Doctor and patient More than selected indicators 10 years and provided evaluation criteria and data.
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(Continued)
Patients had adapted well to the chosen gender and demonstrated improvement in social and gender adaptation after surgery and hormone therapy.
Patients with CAH had significantly higher risk of anxiety disorders; patients with higher testosterone levels had higher internalization and externalization problems scores obtained from parents and teachers. The same applied for those who were less satisfied with their genital appearance and whose surgical procedures were rated poor. It is not clear when the evaluation was done. Both patients showed that they were satisfied with the surgery, but differ in opinion about the best time to perform it.
Follow-up (time Evaluation of the results and between first salient findings intervention and the outcome)
Doctor and patients 1 year selected indicators and provided evaluation criteria and data.
Doctor selected indicators and provided evaluation criteria. Doctor, patients, parents, and teachers provided data.
Average age in the Outcome first intervention assessment methodology
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Genetic 62 CAH and 62 controls parameters, psychosocial, cosmetic, and anatomical evaluation in patients with CAH who underwent surgical intervention
The safety of 29 complete androgen keeping gonads insensitivity syndrome in place for spontaneous puberty in a cohort of patients with genetically proved complete androgen insensitivity syndrome
Cheikhelard et al. (2008)
Research question Sample size and DSD
Nordenskjöld et al. (2008)
Reference
Table 1. (Continued)
Pre- or postpuberty depended on age of the diagnosis.
6 months–9 years
Non-specified evaluation criteria; doctor selected indicators and provided data.
±20 years
The surgery affected patients’ sex life, regardless of the aesthetic result causing late onset of sexual life, fewer pregnancies and smaller offspring, and increased rate of homosexuality and bisexuality. In addition, patients say they would like more information about gynecological, surgical, and psychosexual issues related to the procedures. Although the results of hormonal and surgical interventions were morphologically positive, some adult patients were diagnosed with severe psychiatric disorders.
Follow-up (time Evaluation of the results and between first salient findings intervention and the outcome)
Doctor selected 12–54 years indicators and provided evaluation criteria. Doctor and patients provided data.
Average age in the Outcome first intervention assessment methodology
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To improve the 100 (vaginal agenesis) laparoscopic Vecchietti procedure and optimize instrumentation for treatment of congenital vaginal agenesis Sexual intercourse 58 patients (41 CAH and after feminizing 17 MPH) reconstructive surgery
Research question Sample size and DSD
13–17 years
±20 years
Doctor selected indicators. Doctor and patients provided evaluation criteria, and patients provided data.
About 10 years (does not specify); visits two to three times a year
Patients do not highlight severe problems of sexual development. The authors conclude that the desire for sexual activity causes the need for genital correction, since a lesbian patient refused surgery, and refer that the team has not verified any serious problems during the psychosexual development of the patients.
Most of the patients had satisfactory intercourse 3 months after the surgery. One patient reported regular dyspareunia. A stable relationship was a precondition for treatment in one of the groups.
Follow-up (time Evaluation of the results and between first salient findings intervention and the outcome)
Doctor selected Between 3 and indicators and 37 months provided evaluation criteria. Doctor and patients provided data.
Average age in the Outcome first intervention assessment methodology
DSD: disorder of sexual development; CAH: congenital adrenal hyperplasia; MPH: male pseudohermaphroditism.
Hoepffner et al. (2006)
Brucker et al. (2008)
Reference
Table 1. (Continued)
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provided by doctors, thus neglecting the patient and the family. As an implicit rule, families are not accessed regarding the best time for the procedures, with the exception of Hoepffner et al. (2006) and Merali et al. (2009) where patients differ in opinion about the best time to perform it. This seems to happen for two reasons. The first is that even if the intersex persons or their parents are consulted, the final decision remains a medical matter. The second is idea that the surgery should be done as early as possible. This contrasts with the fact that in most circumstances, there is no medical urgency in the management of DSDs (Fausto-Sterling, 2000). Although not a medical urgency, the management of intersexuality shows itself to be a social urgency (Fausto-Sterling, 2000). Uncertain sex assignment generates discomfort as the person who cannot be categorized inherently breaks gender norms (Butler, 2004). This produces a medical response that seeks to regulate non-normative bodies—to re-order what is perceived as disordered. Roen (2009) has drawn attention to the fact that early performance of surgeries seems to serve to alleviate the parents’ stress. However, this claim is merely a supposition of parents’ feelings and has no empirical basis (Streuli et al., 2013). Nonetheless, it remains a central argument used by doctors to legitimate non-consensual surgery. In her study, Roen (2009) emphasizes that, from a medical standpoint, parents’ desire to have a “normal child” and the “best interest of the child” overlap. Both in Roen’s critique and in our analyses, the studies do not question whether those two assumptions—parents desire and children’s best interests—could be in conflict, creating a sense of relief, presuming that the surgery is everyone’s best interest. The preconceived parental anxiety to early interventions starkly demonstrates sexual binary norms. Butler (1990) argues that our sexual classification is the condition on which we are granted humanity by society. The assignment of a child’s sex at birth (or via sonogram) genders a child. “It’s a girl!” or “It’s a boy!” are performative statements that mark the insertion into social hierarchies, and without them, bodies are neither
intelligible nor recognizable. Early surgeries can be understood as a mechanism of cultural normativity since sex assignment before intersex children have an understanding of themselves, let alone have the ability to give consent, is commonplace (Kessler, 1998). The medical urgency in the studies from our review was not based on evidences. These clinical judgments were based on naught but the opinions and perceptions of medical teams, rooted in gender norms. Justifications, such as “according to our experience …” or “we believe …” cannot support such medical practices. Our analysis of non-evidence-based medical decisions approaches the argument defended by Da Costa Uchôa and De Camargo (2010). The authors assume that the medical support for practices that prioritize the scientific dimension (medicine based on evidences) at the expense of practices that prioritize personal experience does not happen in a linear and cumulative way, following the technical–scientific advances. According to them, “evidence” sometimes serves as construction designed to diminish the uncertainty of medical decisions and to reaffirm the professional autonomy. In the papers we reviewed, medical experience is considered evidence enough, dispensing, eventually, scientific criteria for validation. The conclusions in the majority of the articles we examined extrapolate empirical evidences from anecdotal experience, suggesting that a subjective evaluation could be guiding the decision for the early interventions and the interpretation of their success. Those evaluations seem to be appropriate in the literature, even with ethical issues involved in the reiteration of the gender dichotomy and thus the disrespect of intersex people’s autonomy (Cabral and Benzur, 2005). The treatment protocols in place now, as demonstrated by the articles we reviewed, are still strongly tied to a pathological perspective and are integrated with intervention practices that intend to be corrective. The moment an intersex child is born, different specialists are usually called in to begin three broad and concomitant processes: (a) investigation of the ambiguity etiology, (b) determination of the
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Machado et al. diagnosis, and (c) decisions regarding the types of corrective interventions to be applied (Machado, 2008). Any consideration of abstaining from genital surgery is generally left unconsidered, with the exception of specific professionals. As already pointed, the WHO statement, and the body of research assembled there, calls for the support for the autonomy in the decision-making process and the provision of information to ensure a non-discrimination health practice (WHO, 2014).
Final considerations and clinical implications The review points out to the lack of studies that propose prospective long-term evaluations of the psychological outcomes of sex assignment surgeries. It also points to the questionable quality of the studies, which is related to the use of inconsistent methodology to evaluate the result of the interventions; moreover, the definition of satisfaction and the criteria considered in determining quality of life is not precise. Besides being vague, uncertain, and without consolidated evidences, the evaluation of the success of the surgery in promoting well-being takes into account only in peripheral way the perspective of intersex people and their families. Since there is no consensus on the need of early interventions, we reiterate that the biomedical management of intersexuality should be better investigated, considering its psychological, social, and ethical consequences. Regarding the clinical practice, some urgent questions need to be addressed which affect directly intersex people: (a) the modification of medical protocols centered on the need of an early intervention, (b) the better regulation of the institutional practices related to the handling of intersexuality (medical, juridical, bioethical, educational), and (c) the guarantee of intersex people and their relatives access to a wide range of information, counseling, and comprehensive care. If the sex categorization continues to be based mainly on biomedical parameters, the handling of intersex people will keep on violating their
human rights. Therefore, psychology must play the key role of ensuring the health and rights of this population, and it appears to be going in this direction (Roen and Pasterski, 2014). Funding This research received a grant from the Brazilian Ministry of Science, Technology and Innovation / National Council for Scientific and Technological Development / Secretariat of Policies for Women of the Presidency of the Republic / Ministry of Agrarian Development (32/2012) - Project number: 405304/ 2012-9
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HPQ0010.1177/1359105315572454Journal of Health PsychologyMachado et al.
Review
Follow-up of psychological outcomes of interventions in patients diagnosed with disorders of sexual development: A systematic review
Journal of Health Psychology 1–12 © The Author(s) 2015 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/1359105315572454 hpq.sagepub.com
Paula Sandrine Machado, Angelo Brandelli Costa, Henrique Caetano Nardi, Anna Martha Vaitses Fontanari, Igor Rabuske Araujo and Daniela Riva Knauth
Abstract The objective of this study was to perform a systematic review of articles that followed up the psychological outcomes of interventions in patients with disorders of sexual development. The analysis focused on the methodological quality of the studies and the assumptions on the evaluation of the intervention outcomes. The review signaled the need for better consideration about the complexity of intersexuality and for a shift from the anatomical/functional/aesthetic to the subjective outcomes related to patient’s quality of life. Furthermore, it is suggested that the interventions should be better investigated, given the lack of evidence for the necessity of early procedures.
Keywords disorder of sexual development, follow-up, intersexuality, psychological outcomes, systematic review
There are a considerable number of diagnostic and evaluation criteria coming from genetic, endocrinological, and surgical points of view involved in what medical practitioners label disorders of sexual development (DSDs). The endorsement of this terminology is a product of a gathering of specialists that resulted in what is known as the Chicago Consensus that modify and unify practices, protocols, and terminology that were being used in a nonuniform way to that point (Lee et al., 2006). However, even today, numerous questions remain unanswered in relation to interventions in such conditions, such as which is the most
appropriate period for surgical intervention; who should make the final decision about the person’s sex; are the procedures necessary; moreover, which are the subjective, social, ethical, and political effects of the interventions? (Boyle et al., 2005; Cohen-Kettenis, 2005; Kessler, 1998; Machado, 2009) Universidade Federal do Rio Grande do Sul, Brazil Corresponding author: Paula Sandrine Machado, Department of Social Psychology, Universidade Federal do Rio Grande do Sul, Ramiro Barcelos, 2600, 300h, Porto Alegre, RS, Brazil. Email: [email protected]
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Recently, the World Health Organization (WHO) released an interagency statement (Office of the High Commissioner for Human Rights (OHCHR), United Nations (UN) Women, Joint United Nations Programme on HIV and AIDS (UNAIDS), UN Development Programme (UNDP), UN Population Fund (UNFPA), UN Children’s Fund (UNICEF), and WHO) affirming that “intersex persons may be involuntarily subjected to so-called sexnormalizing or other procedures as infants or during childhood, which, in some cases, may result in the termination of all or some of their reproductive capacity” (WHO, 2014: 7). That statement also refers to the lack of consideration of the views of the children and of their parents, given that those interventions could have “lifelong consequence for their physical and mental health” (p. 7). Anchored in international human rights bodies and national courts, the WHO (2014) uses terms such as “involuntary” and “coercive” to label those practices. Two systematic reviews about the outcomes of interventions in intersexual conditions were located. The first (Santos and Araújo, 2008) concludes that it is necessary to gather greater theoretical background in the discussion of medical interventions outcomes, in addition to the deployment of more longitudinal studies. The second review (Schoenbucher et al., 2010) focused on the sexual life quality of individuals with a 46 XY karyotype, who experienced DSD. The article concludes that there are inconsistent results regarding sexual satisfaction, indicating that future studies should focus more on qualitative aspects of sexuality. Nevertheless, both studies seem to lack in methodological quality for conducting systemic reviews. One of the main limitations on follow-ups to surgical interventions and its assessments is that the majority of the studies in the field are guided by a biomedical perspective (Roen, 2009). The core presumption of this perspective is that an adequate intervention would lead to a positive psychological effect on the subjective wellbeing over one’s body. This point of view does not consider the socio-political aspects related to the interventions, meaning that it ignores
intersex people’s lived experience and social insertion. Moreover, when aspects that are more “psychological” are evaluated, the researchers tend to determine the relevant indicators, mainly over global evaluation judgments regarding the well-being of the populations under study (Karkazis, 2008). There is no doubt that there is still much ground to advance in studies designed to longitudinally evaluate features such as body image, satisfaction with body and genitals, sexuality, overall health, and, in a broader sense, one’s quality of life (Warne, 2008). Aiming to discuss some of the arguments presented here, we propose a systematic review of studies that specifically evaluate the longitudinal psychological outcomes as a result of interventions undergone in patients diagnosed with DSDs.
Methods To identify studies that investigated the longterm psychological outcomes of surgical and hormonal intervention in DSD patients, a search of Scopus and Web of Science databases was performed on October 2013. Aimed at covering the wide range of conditions related to intersexuality, we used the search term “Disorders of Sexual Development” under the Medical subject heading (MeSH). The search strings consisted of each one of the terms listed under DSD AND “Follow up OR Followup OR Follow-up OR Long-term OR Long term.” The search returned 5199 articles. Duplicates, of which there were 1799, were removed. The titles and abstracts of 3400 studies were independently analyzed by three reviewers, who applied the following inclusion criteria: (a) being a long-term follow-up study (1 year minimum); (b) focusing primarily on surgical and/or hormonal interventions in at least one DSD—only interventions upon the sexual characteristicswere taken into account, due to the fact that the main focus of this review is the understanding of the psychological outcomes of sexual assignment; (c) investigating psychological outcomes from the point of view
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Machado et al. of the subject (e.g. quality of life, sexual satisfaction, sexual functionality, aesthetic result of the external genitalia, etc.); (d) primarily focusing on the patient (not the service, physician, family, etc.); (e) being published after the Chicago Consensus (2006); (f) being a full article published in indexed journals; and (g) being written in English. According to these criteria, 106 articles were selected. These were then re-examined and screened by the three independent judges under stricter exclusion conditions, paying particular attention to (a) omitted information we considered essential (age of the first intervention, time of the follow-up) and (b) being a retrospective rather than a prospective follow-up—retrospective studies were excluded because they use indirect data, making it very difficult, and sometimes impossible, to measure certain factors accurately (Lilienfeld and Stolley, 1994); (c) not being focused on a DSD. Even though we were based on MeSH terms, papers reporting unlisted DSDs such as Mayer–Rokitansky–Küster–Hauser Syndrome were not excluded due to the fact that they followed the same clinical management, and thus, procedures other than those DSDs specified under MeSH terms. Additionally, the authors related those syndromes to DSDs. The relevant data from these articles were then documented in a spreadsheet, including year of publication, research team composition and nationalities, journal, DSDs, sample size, median sample age at the time of medical intervention, nature of the intervention, investigated outcome, outcome assessment methodology, follow-up time, relevant study results, and highlights. The final pool contained 13 articles (Figure 1). The analysis focused on the methodological quality of the studies and the assumptions on the evaluation of the outcomes.
Results and discussion The majority of the selected articles (Table 1) focused on morphological outcomes, that is, the anatomical–functional aspects of interventions and their complications. Those studies were
published in medical journals and written by physicians mainly specialized in urology. The countries of publication were varied—Eastern and Western both included—but no comparative studies were found. Only one of the articles mentions cultural differences (Merali et al., 2009); however, the acknowledgement of cultural differences was trivial. The sample sizes varied from 1, in a case study, to 182, in a prospective follow-up. Both case studies and case-control studies were present, with a sample size of 38 patients lasting for about 14 years, and only four studies with a follow-up design per se. The follow-up time varied from 1 to 24 years. All articles reported surgical procedures, and although the majority also reported the use of hormonal therapy, the effects were discussed either in a superficial manner or not at all; the focus was the effect of the surgical interventions. Most of the studies involved cases of congenital adrenal hyperplasia, which is considered the most frequent DSD. The results of the systematic review show the existence of a diverse range of methodologies when it comes to evaluating the results of the interventions. Some of the studies that proposed to analyze psychological outcomes were limited to external genitalia evaluation— usually from a medical doctor—which neglected the perspective of individuals who underwent the sex assignment procedures. Few papers displayed results about the psychological outcomes or their relation to well-being or even considered participants’ subjective quality of life. Some studies did not even specify how they measure the outcomes. Generally speaking, the assessment of the psychological outcomes did not make use of reliable indicators regarding the lived experience of the participants and/or even validated measures. It is noteworthy that the article with the most robust method of outcome analysis reached a clear conclusion of dissatisfaction in domains such as cosmetic and functional surgical result, overall genital function, and overall sex life (Köhler et al., 2012). Furthermore, psychological outcomes were often treated a synonym for sexual satisfaction.
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Scopus N=1831
Web of Science N=3368
Potentially relevant references N=5199
Search in the abstracts N=3400
Data extraction N=106
Removed Duplicates N= 1799 Removed Did not meet the inclusion criteria N=3506 Removed Met the exclusion criteria N=93
Final pool N=13
Figure 1. Search strategy.
The articles that proposed to measure sexual satisfaction, when referring to it, were predominantly limited to asking how dilated the vagina was; whether there was the presence of orgasms, lubrication, and pain when ejaculating; capacity to penetrate or to be penetrated; having stable relationships; or the evaluation, by an external observer, of the aesthetic of the genital; and, in fewer cases, how he or she feels concerning the surgery. It is also important to draw attention to the assumption that relates good outcomes to marriage and sex, particularly of a heterosexual nature. As already discussed in the literature, traditional values and beliefs presented in some cultures tend to justify fertility, marriage, and heterosexuality as parameters of good outcomes (Warne and Raza, 2008). In all, 10 articles concluded, using heterogeneous criteria, that the surgery was a success. In most cases, success was defined by the opinion of the team, even when it contradicted the presented data. For example, one emblematic article with “positive results” evaluated, in a prospective follow-up consultation, the surgical results and the sexual satisfaction of 47 patients in an average of 12 years after an intestinal vaginoplasty took place. According to the authors, the result was positive because besides the excellent surgical outcome, 38.3 percent of the sample was sexually active and 8.5 percent was
married. Nevertheless, 17 out of 47 patients had complications from the surgery, such as necrosis of part of the genital, abdominal abscess, and vaginal prolapse (Lima et al., 2010). There is a dissonance between the complications resulting from the interventions and the evaluation of those interventions. In other words, there is a tendency to positively evaluate the interventions despite the absence of established criteria and in the face of contradictory evidence. One of the fundamental issues we have found in the reviewed studies is that numerous premises seem to be self-evident. These include the definition of satisfactory sexuality and what makes a body desirable and normal. Sexual intercourse, predominantly pertaining to successful vaginal penetration, is positioned as central to the concept of satisfaction and wellbeing. This heterosexist default, or what Adrianne Rich (2013) has termed compulsory heterosexuality, is problematic, as it assumes that sexuality is oriented around a “normative” heterosexual conception. Therefore, when female genitalia are evaluated, it is in the sense of being able to accommodate a regular-sized penis. In those cases, alternative sexual orientations or practices are not accounted for, even when sexual satisfaction is evaluated. The success of the surgeries is most of the times evaluated according to measures and data
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Sexual adjustment and long-term results among patients who underwent vaginal reconstruction with free vascular jejunal flap
Evaluation of satisfaction with genital surgery and sexual life in adults with XY, DSDs
The use of sigmoid colon in vaginal reconstruction
Köhler et al. (2012)
Nowier et al. (2012)
Doctor selected ~25 years indicators and provided evaluation criteria. Doctor, patients, and parents provided data. Doctor selected 6 years indicators and provided evaluation criteria. Doctor and patients provided data.
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(Continued)
Dissatisfaction with overall treatment was substantial. Dissatisfaction was high regarding cosmetic and functional surgical result, overall genital function, and overall sex life. Long-term follow-up showed introital stenosis in four cases. Mucosal prolapse occurred in one case. Reoperation rate was 9.6 percent. Sexual satisfaction was achieved by nine sexually active cases. The subjective satisfaction score of the surgical outcome was high.
In total, 11 patients were satisfied with their sexual lives after surgery; 27 were married and sexually active.
Follow-up (time Evaluation of the results and between first salient findings intervention and the outcome)
Doctor selected 50 months indicators and provided evaluation criteria. Doctor and patients provided data.
Average age in the Outcome first intervention assessment methodology
34 women: Mayer– 16–31 years Rokitansky–Küster– Hauser syndrome (29 patients), isolated vaginal agenesis (2 patients), androgen insensitivity syndrome (2 patients), and history of gynecologiconcologic surgery (1 patient) 57 individuals with various Phallus reduction XY, DSDs or correction of hypospadias was performed mostly in childhood and vaginoplasty in puberty. 31 patients with DSDs of 14–28 years various causes
Research question Sample size and DSD
Erman Akar et al. (2013)
Reference
Table 1. Reviews study data.
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Lima et al. (2010)
Sircili et al. (2010)
Long-term results of anatomical structure and function of the neovagina after transvestibular vaginoplasty Evaluation of long-term morphological and functional outcomes of masculinizing genitoplasty in patients with DSDs Surgical complications and sexual satisfaction after vaginal replacement in pediatric age
Zhou et al. (2010)
6 (1–47) years: 14 patients underwent the intervention before the first year of life.
23 (19–35) years
Non-specified evaluation criteria; doctor selected indicators and provided data.
12 (1–34) years
Doctor selected 14.1 ± 9.2 years indicators and provided evaluation criteria. Doctor and patients provided data.
According to the authors, 38.3 percent of the sample were sexually active, and 8.5 percent were married. In addition, all patients reported excellent aesthetic results without excessive mucus production or the need for expansion. In contrast, 17 of them had complications.
The result was good, but there was significant sample loss during follow-up. All women were provided adequate counseling on the advantages and disadvantages of the techniques. Most of the patients were sexually active; only 3 percent felt dissatisfied with their sexual life mainly due to small penis and were pleased with both the age of the first intervention and its results.
Follow-up (time Evaluation of the results and between first salient findings intervention and the outcome)
Doctor selected 15 years indicators and provided evaluation criteria. Doctor and patients provided data.
Average age in the Outcome first intervention assessment methodology
47 Rokitansky’s Syndrome “Around puberty” and others in the majority and 11 after puberty
38 DSDs in general
182 Rokitansky’s Syndrome
Research question Sample size and DSD
Reference
Table 1. (Continued)
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Cultural diversity 2 (one from Iceland and in the surgical one from Bolivia). outcome and sexual satisfaction after clitoroplasty in two DSD patients. Problems with 1 17β-HSD-3 deficiency mental health, social adjustment, family relationships, socialization, adaptation to gender in patients with 17β-HSD-3 deficiency subjected to genitoplasty
Merali et al. (2009)
Liakopoulou et al. (2009)
Behavior and emotional problems after vaginoplasty
Oner et al. (2009)
28 CAH, 28 age-matched patients with diabetes mellitus and 28 healthy controls
Research question Sample size and DSD
Reference
Table 1. (Continued)
17 years
Around 17 and 32 years
1–5 years 6–18 years: at childhood in every 3 months and then every 6 months.
Doctor and patient More than selected indicators 10 years and provided evaluation criteria and data.
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(Continued)
Patients had adapted well to the chosen gender and demonstrated improvement in social and gender adaptation after surgery and hormone therapy.
Patients with CAH had significantly higher risk of anxiety disorders; patients with higher testosterone levels had higher internalization and externalization problems scores obtained from parents and teachers. The same applied for those who were less satisfied with their genital appearance and whose surgical procedures were rated poor. It is not clear when the evaluation was done. Both patients showed that they were satisfied with the surgery, but differ in opinion about the best time to perform it.
Follow-up (time Evaluation of the results and between first salient findings intervention and the outcome)
Doctor and patients 1 year selected indicators and provided evaluation criteria and data.
Doctor selected indicators and provided evaluation criteria. Doctor, patients, parents, and teachers provided data.
Average age in the Outcome first intervention assessment methodology
Machado et al. 7
Genetic 62 CAH and 62 controls parameters, psychosocial, cosmetic, and anatomical evaluation in patients with CAH who underwent surgical intervention
The safety of 29 complete androgen keeping gonads insensitivity syndrome in place for spontaneous puberty in a cohort of patients with genetically proved complete androgen insensitivity syndrome
Cheikhelard et al. (2008)
Research question Sample size and DSD
Nordenskjöld et al. (2008)
Reference
Table 1. (Continued)
Pre- or postpuberty depended on age of the diagnosis.
6 months–9 years
Non-specified evaluation criteria; doctor selected indicators and provided data.
±20 years
The surgery affected patients’ sex life, regardless of the aesthetic result causing late onset of sexual life, fewer pregnancies and smaller offspring, and increased rate of homosexuality and bisexuality. In addition, patients say they would like more information about gynecological, surgical, and psychosexual issues related to the procedures. Although the results of hormonal and surgical interventions were morphologically positive, some adult patients were diagnosed with severe psychiatric disorders.
Follow-up (time Evaluation of the results and between first salient findings intervention and the outcome)
Doctor selected 12–54 years indicators and provided evaluation criteria. Doctor and patients provided data.
Average age in the Outcome first intervention assessment methodology
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To improve the 100 (vaginal agenesis) laparoscopic Vecchietti procedure and optimize instrumentation for treatment of congenital vaginal agenesis Sexual intercourse 58 patients (41 CAH and after feminizing 17 MPH) reconstructive surgery
Research question Sample size and DSD
13–17 years
±20 years
Doctor selected indicators. Doctor and patients provided evaluation criteria, and patients provided data.
About 10 years (does not specify); visits two to three times a year
Patients do not highlight severe problems of sexual development. The authors conclude that the desire for sexual activity causes the need for genital correction, since a lesbian patient refused surgery, and refer that the team has not verified any serious problems during the psychosexual development of the patients.
Most of the patients had satisfactory intercourse 3 months after the surgery. One patient reported regular dyspareunia. A stable relationship was a precondition for treatment in one of the groups.
Follow-up (time Evaluation of the results and between first salient findings intervention and the outcome)
Doctor selected Between 3 and indicators and 37 months provided evaluation criteria. Doctor and patients provided data.
Average age in the Outcome first intervention assessment methodology
DSD: disorder of sexual development; CAH: congenital adrenal hyperplasia; MPH: male pseudohermaphroditism.
Hoepffner et al. (2006)
Brucker et al. (2008)
Reference
Table 1. (Continued)
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provided by doctors, thus neglecting the patient and the family. As an implicit rule, families are not accessed regarding the best time for the procedures, with the exception of Hoepffner et al. (2006) and Merali et al. (2009) where patients differ in opinion about the best time to perform it. This seems to happen for two reasons. The first is that even if the intersex persons or their parents are consulted, the final decision remains a medical matter. The second is idea that the surgery should be done as early as possible. This contrasts with the fact that in most circumstances, there is no medical urgency in the management of DSDs (Fausto-Sterling, 2000). Although not a medical urgency, the management of intersexuality shows itself to be a social urgency (Fausto-Sterling, 2000). Uncertain sex assignment generates discomfort as the person who cannot be categorized inherently breaks gender norms (Butler, 2004). This produces a medical response that seeks to regulate non-normative bodies—to re-order what is perceived as disordered. Roen (2009) has drawn attention to the fact that early performance of surgeries seems to serve to alleviate the parents’ stress. However, this claim is merely a supposition of parents’ feelings and has no empirical basis (Streuli et al., 2013). Nonetheless, it remains a central argument used by doctors to legitimate non-consensual surgery. In her study, Roen (2009) emphasizes that, from a medical standpoint, parents’ desire to have a “normal child” and the “best interest of the child” overlap. Both in Roen’s critique and in our analyses, the studies do not question whether those two assumptions—parents desire and children’s best interests—could be in conflict, creating a sense of relief, presuming that the surgery is everyone’s best interest. The preconceived parental anxiety to early interventions starkly demonstrates sexual binary norms. Butler (1990) argues that our sexual classification is the condition on which we are granted humanity by society. The assignment of a child’s sex at birth (or via sonogram) genders a child. “It’s a girl!” or “It’s a boy!” are performative statements that mark the insertion into social hierarchies, and without them, bodies are neither
intelligible nor recognizable. Early surgeries can be understood as a mechanism of cultural normativity since sex assignment before intersex children have an understanding of themselves, let alone have the ability to give consent, is commonplace (Kessler, 1998). The medical urgency in the studies from our review was not based on evidences. These clinical judgments were based on naught but the opinions and perceptions of medical teams, rooted in gender norms. Justifications, such as “according to our experience …” or “we believe …” cannot support such medical practices. Our analysis of non-evidence-based medical decisions approaches the argument defended by Da Costa Uchôa and De Camargo (2010). The authors assume that the medical support for practices that prioritize the scientific dimension (medicine based on evidences) at the expense of practices that prioritize personal experience does not happen in a linear and cumulative way, following the technical–scientific advances. According to them, “evidence” sometimes serves as construction designed to diminish the uncertainty of medical decisions and to reaffirm the professional autonomy. In the papers we reviewed, medical experience is considered evidence enough, dispensing, eventually, scientific criteria for validation. The conclusions in the majority of the articles we examined extrapolate empirical evidences from anecdotal experience, suggesting that a subjective evaluation could be guiding the decision for the early interventions and the interpretation of their success. Those evaluations seem to be appropriate in the literature, even with ethical issues involved in the reiteration of the gender dichotomy and thus the disrespect of intersex people’s autonomy (Cabral and Benzur, 2005). The treatment protocols in place now, as demonstrated by the articles we reviewed, are still strongly tied to a pathological perspective and are integrated with intervention practices that intend to be corrective. The moment an intersex child is born, different specialists are usually called in to begin three broad and concomitant processes: (a) investigation of the ambiguity etiology, (b) determination of the
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Machado et al. diagnosis, and (c) decisions regarding the types of corrective interventions to be applied (Machado, 2008). Any consideration of abstaining from genital surgery is generally left unconsidered, with the exception of specific professionals. As already pointed, the WHO statement, and the body of research assembled there, calls for the support for the autonomy in the decision-making process and the provision of information to ensure a non-discrimination health practice (WHO, 2014).
Final considerations and clinical implications The review points out to the lack of studies that propose prospective long-term evaluations of the psychological outcomes of sex assignment surgeries. It also points to the questionable quality of the studies, which is related to the use of inconsistent methodology to evaluate the result of the interventions; moreover, the definition of satisfaction and the criteria considered in determining quality of life is not precise. Besides being vague, uncertain, and without consolidated evidences, the evaluation of the success of the surgery in promoting well-being takes into account only in peripheral way the perspective of intersex people and their families. Since there is no consensus on the need of early interventions, we reiterate that the biomedical management of intersexuality should be better investigated, considering its psychological, social, and ethical consequences. Regarding the clinical practice, some urgent questions need to be addressed which affect directly intersex people: (a) the modification of medical protocols centered on the need of an early intervention, (b) the better regulation of the institutional practices related to the handling of intersexuality (medical, juridical, bioethical, educational), and (c) the guarantee of intersex people and their relatives access to a wide range of information, counseling, and comprehensive care. If the sex categorization continues to be based mainly on biomedical parameters, the handling of intersex people will keep on violating their
human rights. Therefore, psychology must play the key role of ensuring the health and rights of this population, and it appears to be going in this direction (Roen and Pasterski, 2014). Funding This research received a grant from the Brazilian Ministry of Science, Technology and Innovation / National Council for Scientific and Technological Development / Secretariat of Policies for Women of the Presidency of the Republic / Ministry of Agrarian Development (32/2012) - Project number: 405304/ 2012-9
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