Атипикалық жыныс мүшелерін түзету шаралары және мәселелері

Performed to modify atypical or ambiguous genitalia

Intersex medical interventions (IMI), sometimes known as intersex genital mutilations (IGM), are surgical, hormonal and other medical interventions performed to modify atypical or ambiguous genitalia and other sex characteristics, primarily for the purposes of making a person's appearance more typical and to reduce the likelihood of future problems. The history of intersex surgery has been characterized by controversy due to reports that surgery can compromise sexual function and sensation, and create lifelong health issues. The medical interventions can be for a variety of reasons, due to the enormous variety of the disorders of sex development. Some disorders, such as salt wasting disorder, can be life threatening if left untreated. Interventions on intersex infants and children are increasingly recognized as human rights issues. Intersex organizations, and human rights institutions increasingly question the basis and necessity of such interventions. and health

Orchiopexy and hypospadias repair are the most common types of genital corrective surgery performed in infant boys. In a few parts of the world 5 alpha reductase deficiency or defects of testosterone synthesis, or even rarer forms of intersex account for a significant portion of cases but these are rare in North America and Europe. Masculinizing surgery for completely virilized individuals with XX sex chromosomes and CAH is even rarer. An early procedure was performed by London surgeon Thomas Brand in 1779. Orchiopexy for repair of undescended testes (cryptorchidism) is the second most common surgery performed on infant male genitalia (after circumcision). The surgeon moves one or both testes, with blood vessels, from an abdominal or inguinal position to the scrotum. If the inguinal canal is open it must be closed to prevent hernia. Potential surgical problems include maintaining the blood supply. If vessels cannot be stretched into the scrotum, or are separated and cannot be reconnected, a testis will die and atrophy. Hypospadias repair may be a single stage procedure if the hypospadias is of the first or second degree (urethral opening on glans or shaft respectively) and the penis is otherwise normal. Surgery for third degree hypospadias (urethral opening on perineum or in urogenital opening) is more challenging, may be done in stages, and has a significant rate of complications and unsatisfactory outcomes. Potential surgical problems: For severe hypospadias (3rd degree, on perineum) constructing a urethral tube the length of the phallus is not always successful, leaving an opening (a "fistula") proximal to the intended urethral opening. Sometimes a second operation is successful, but some boys and men have been left with chronic problems with fistulas, scarring and contractures that make urination or erections uncomfortable, and loss of sensation. It is increasingly recognized that long term outcomes are poor. Epispadias repair may involve comprehensive surgical repair of the genito urinary area, usually during the first seven years of life, including reconstruction of the urethra, closure of the penile shaft and mobilisation of the corpora. Urogenital closure closure of any midline opening at the base of the penis. In severe undervirilization a boy may have a "pseudovaginal pouch" or a single urogenital opening in the midline of the perineum. Potential surgical problems: The most complicated aspect of closure involves moving the urethra to the phallus if it is not already there (i. e., repairing a perineal hypospadias). Fistulas, scarring, and loss of sensation are the main risks. Gonadectomy (also referred to as "orchiectomy") removal of the gonads. This is done in three circumstances. (1) If the gonads are dysgenetic testes or streak gonads and at least some of the boy's cells have a Y chromosome, the gonads or streaks must be removed because they are nonfunctional but have a relatively high risk of developing gonadoblastoma. (2) In rare instances when an XX child has completely virilizing congenital adrenal hyperplasia (Prader stage 5), the ovaries can be removed before puberty to stop breast development and/or menstruation. (3) Gonadectomy can be performed in the equally rare instance of a child with true hermaphroditism virilized enough to raise as male, in which ovaries or ovotestes can be removed. A lifetime of hormone replacement will be required, to avoid osteoporosis and enable sexual functioning. Chordee release is the cutting of ventral penile skin and connective tissue to free and straighten the penis. A mild chordee, manifest as a well formed penis "bent" downward by subcutaneous connective tissue, may be an isolated birth defect easily repaired by releasing some of the inelastic connective tissue on the ventral side of the shaft. In a complete chordee the phallus is "tethered" downward to the perineum by skin. A more severe chordee is often accompanied by a hypospadias and sometimes by severe undervirilization: a perineal "pseudovaginal pouch" and bifid ("split") scrotum with an undersized penis. This combination, referred to as pseudovaginal perineoscrotal hypospadias, is in the spectrum of ambiguous genitalia due to a number of conditions. Scarring and contracture are occasional complications, but most unsatisfactory outcomes occur when a severe hypospadias needs to be repaired as well. Long term complications can include fistulas between colon or upper rectum and skin or other cavities, or between urethra and perineum. Loss of sensation. Cloacal repair is among the most complex of the surgeries described here. Bladder exstrophy or more severe cloacal exstrophy is a major birth defect involving inadequate closure and incomplete midline fusion of multiple pelvic and perineal organs as well as the front of the pelvis and lower abdominal wall. The penis and scrotum are often widely bifid (the two embryonic parts unjoined). The penis often cannot be salvaged, although the testes can be retained. Repair may involve closure of the bladder, closure of the anterior abdominal wall, colostomy (temporary or permanent) with reconstruction of the rectum. If the halves of the phallus cannot be joined, they may be removed. The smallest defect in this spectrum is an epispadias. Surgical repair for this is primarily a phalloplasty. Potential surgical problems: Surgery for the more severe degrees of cloacal exstrophy is extensive and usually multistage. A variety of potential problems and complications can occur, including need for long term colostomy or vesicostomy. In many cases a functional penis cannot be created. Scarring is often extensive and the lower torso severely disfigured even with fairly good outcomes. Phalloplasty is a general term for any reconstruction of the penis itself, especially for more unusual types of injuries, deformities, or birth defects. The principal difficulty is that erectile tissue is not easily constructed and this limits the surgeon's ability to make more than minor size changes. Construction of a narrow tube lined with mucosa (a urethra) is a similar challenge. Minor revisions of the skin are rarely followed by problems. More complicated reconstruction may result in scarring and contracture, which can distort the shape or curvature of the penis, or interfere with erections or make them painful. Hysterectomy is removal of a uterus. It is rare that a uterus or Müllerian duct derivatives would need to be removed from a child being raised as a boy: see persistent Müllerian duct syndrome. The most common scenario is accidental discovery of persistent Müllerian derivatives or a small uterus during abdominal surgery of a normal boy for cryptorchidism, appendectomy, or bowel disease. Removal would not involve genital surgery. A rarer indication would be that of a completely virilized XX child with congenital adrenal hyperplasia (Prader stage 5) being raised as a male; ovaries and uterus must be removed to prevent breast development and menstruation by early adolescence. Risks are simply those of abdominal surgery. Testicular prostheses are saline filled plastic ovoids implanted in the scrotum. They have no function except to provide the appearance and feel of testes. Several sizes are available, but most are implanted in adolescence to avoid repeated procedures to implant larger sizes at puberty. Prostheses made of silastic are no longer available due to safety and perception of safety concerns. Potential surgical problems: Foreign body reactions, rarely with infection or erosion of scrotal skin, are minimal but constitute the most significant complication. Penile augmentation surgery is surgery intended to enlarge a small penis. Early attempts in the 1950s and 1960s involved constructing a tube of non erectile flesh extending a small penis but the penis did not function. In recent years a small number of urologists have been offering an augmentation procedure that involves moving outward some of the buried components of the corpora so that the penis protrudes more. The girth is augmented with transplantation of the patient's fat. This procedure is designed to preserve erectile and sexual function without surgically altering the urethra. This type of surgery is not performed on children and primarily produces a small increase in the size of a normal penis, but would be less likely to produce a major functional change in a severe micropenis. Potential surgical problems include reabsorption of the fat, scarring resulting in interference with erectile function, and issues with physical sensation. Concealed penis where a normal penis is buried in suprapubic fat. In most cases, when the fat is depressed with the fingers, the penis is seen to be of normal size. This is common in overweight boys before the penile growth of puberty. Surgical techniques have been devised to improve it. The most common problems post surgery are recurrence with continued weight gain and scarring.

In the last 50 years, the following procedures were most commonly performed to make the genitalia more typically female: virilization due to congenital adrenal hyperplasia; genital variations due, for example, to cloacal exstrophy; genital variations in infants with XY or mixed chromosomes to be raised as girls, such as gonadal dysgenesis, partial and complete androgen insensitivity syndrome, micropenis, cloacal and bladder exstrophy. In the 21st century, feminizing surgery to support reassignment of XY infants with non ambiguous micropenis has been largely discontinued, and surgical reassignment of XY infants with exstrophy or other significant variations or injuries is diminishing. See history of intersex surgery. Clitorectomy amputation or removal of most of the clitoris, including glans, erectile tissue, and nerves. This procedure was the most common clitoral surgery performed prior to 1970, but was largely abandoned by 1980 because it usually resulted in loss of clitoral sensation. Potential surgical problems: The primary effect of this surgery is a drastic reduction in ability to experience orgasm. The appearance is not very normal. Regrowth of unwanted erectile tissue has sometimes presented problems. Clitoroplasty, like phalloplasty, is a term that encompasses any surgical reconstruction of the clitoris, such as removal of the corpora. Clitoral recession and reduction can both be referred to as clitoroplasty. Potential surgical problems: Major complications can include scarring, contractures, loss of sensation, loss of capacity for orgasm, and unsatisfactory appearance. Clitoral recession involves the repositioning of the erectile body and glans of the clitoris farther back under the symphysis pubis and/or skin of the preputium and mons. This was commonly done from the 1970s through the 1980s to reduce protrusion without sacrificing sensation. Outcomes were often unsatisfactory, and it fell into disfavor in the last 15 years. Potential surgical problems: Unfortunately the subsequent sensations were not always pleasant, and erection could be painful. Adults who had a clitoral recession in early childhood often report reduced capacity for enjoyment of sexual intercourse, though similar women who had not had surgery also report a high rate of sexual dysfunction. Clitoral reduction is rarely done except in combination with vaginoplasty when substantial virilization is present. Potential problems: The degree to which the goal of preserving sexual sensations is attained is a subject of controversy regarding the necessity of such treatments, and lack of firm evidence of good outcomes. Vaginoplasty, the construction or reconstruction of a vagina, can be fairly simple or quite complex, depending on the initial anatomy. If a normal internal uterus, cervix and upper vagina (the Müllerian derivatives) exist, and the outer virilization is modest, surgery involves separating the fused labia and widening the vaginal introitus. With greater degrees of virilization, the major challenge of the procedure is to provide a passage connecting the outer vaginal opening to the cervix which will stay wide enough to allow coitus. XY girls or women with partial androgen insensitivity syndrome will have a blind vaginal pouch of varying degrees of depth. Sometimes this can be dilated to a usable depth. Sometimes surgery is performed to deepen it. The most challenging surgery with the highest complication rate is construction of an entirely new vagina (a "neovagina"). The most common instance of this is when a child will be assigned and raised as a female despite complete virilization, as with Prader 5 CAH, or (in the past) when a genetic male infant with a severely defective penis was reassigned as a female. One method is to use a segment of colon, which provides a lubricated mucosal surface as a substitute for the vaginal mucosa. Another is to line the new vagina with a skin graft. Potential surgical problems: Stenosis (narrowing) of the constructed vagina is the most common long term complication and the chief reason that a revision may be required when a girl is older. When a neovagina is made from a segment of bowel, it tends to leak mucus; when made with a skin graft, lubrication is necessary. Less common complications include fistulas, uncomfortable scarring, and problems with urinary continence. Gonadectomy is removal of the gonads. If the gonads are dysgenetic testes or streak gonads and at least some of the cells have a Y chromosome, the gonads or streaks must be removed because they are nonfunctional but have a relatively high risk of developing gonadoblastoma. If the gonads are relatively "normal" testes, but the child is to be assigned and raised as female, (e. g., for intersex conditions with severe undervirilization, or major malformations involving an absent or unsalvageable penis) they must be removed before puberty to prevent virilization from rising testosterone. Testes in androgen insensitivity are a special case: if there is any degree of responsiveness to testosterone, they should be removed before puberty. On the other hand, if androgen insensitivity is complete, the testes may be left to produce estradiol (via testosterone) to induce breast development, but there is a slowly increasing risk of cancer in adult life. Streak gonads without a Y chromosome cell line need not be removed but will not function. Finally, the gonads in true hermaphroditism must be directly examined; atypical gonads with Y line or potential testicular function should be removed but in rare instances a surgeon may try to preserve the ovarian part of an ovotestis. Potential surgical problems: A lifetime of hormone replacement will be required, to avoid osteoporosis and enable sexual functioning. Cloacal exstrophy and bladder exstrophy repair is needed regardless of the sex of assignment or rearing. Simple bladder exstrophy in a genetic female does not usually involve the vagina. Cloacal exstrophy in a genetic female usually requires major surgical reconstruction of the entire perineum, including bladder, clitoris, symphysis pubis, and both the vaginal introitus and urethra. However, the uterus and ovaries are normally formed. Severe bladder exstrophy or cloacal exstrophy in genetic males often renders the phallus widely split, small, and unsalvageable. The scrotum is also widely split, though testes themselves are usually normal. From the 1960s until the 1990s, many of these infants were assigned and raised as females, with fashioning of a vagina and gonadectomy as part of the perineal reconstruction.

There is widespread evidence of prenatal testing and hormone treatment to prevent intersex traits. In 1990, a paper by Heino Meyer Bahlburg titled Will Prenatal Hormone Treatment Prevent Homosexuality? was published in the Journal of Child and Adolescent Psychopharmacology. It examined the use of "prenatal hormone screening or treatment for the prevention of homosexuality" using research conducted on foetuses with congenital adrenal hyperplasia (CAH). Dreger, Feder, and Tamar Mattis describe how later research constructs "low interest in babies and men – and even interest in what they consider to be men's occupations and games – as "abnormal", and potentially preventable with prenatal dex [amethasone]". There is widespread evidence of pregnancy terminations arising from prenatal testing, as well prenatal hormone treatment to prevent intersex traits. In April 2014, Organisation Intersex International Australia made a submission on genetic selection via preimplantation genetic diagnosis to the National Health and Medical Research Council recommending that deselection of embryos and foetuses on grounds of intersex status should not be permitted. It quoted research by Professors Morgan Holmes, Jeff Nisker, associate professor Georgiann Davis, and by Jason Behrmann and Vardit Ravitsky. It quotes research showing pregnancy termination rates of up to 88% in 47,XXY even while the World Health Organization describes the trait as "compatible with normal life expectancy", and "often undiagnosed". Behrmann and Ravitsky find social concepts of sex, gender and sexual orientation to be "intertwined on many levels. Parental choice against intersex may thus conceal biases against same sex attractedness and gender nonconformity."

The DSM 5 included a change from using gender identity disorder to gender dysphoria. This revised code now specifically includes intersex people who do not identify with their sex assigned at birth and experience clinically significant distress or impairment, using the language of disorders of sex development. This move was criticised by intersex advocacy groups in Australia and New Zealand.

A 2006 clinician "Consensus Statement on Intersex Disorders and Their Management" attempted to prioritise psychosocial support for children and families, but it also supports surgical intervention with psychosocial rationales such as "minimizing family concern and distress" and "mitigating the risks of stigmatization and gender identity confusion". A 2004 paper by Heino Meyer Bahlburg and others examined outcomes from early surgeries in individuals with XY variations, at one patient centre. The study has been used to support claims that "the majority of women have clearly favored genital surgery at an earlier age" but the study was criticized by Baratz and Feder in a 2015 paper for neglecting to inform respondents that:
(1) not having surgery at all might be an option; (2) they might have had lower rates of reoperation for stenosis if surgery were performed later, or (3) that significant technical improvements that were expected to improve outcomes had occurred in the 13 or 14 years between when they underwent early childhood surgery and when it might have been deferred until after puberty.

In 2006, an invited group of clinicians met in Chicago and reviewed clinical evidence and protocols, and adopted a new term for intersex conditions: Disorders of sex development (DSD) in the journal article Consensus Statement on Intersex Disorders and their Management. The term has been controversial and not widely adopted outside clinical settings: the World Health Organization and many medical journals still refer to intersex traits or conditions. Academics like Georgiann Davis and Morgan Holmes, and clinical psychologists like Tiger Devore argue that the term DSD was designed to "reinstitutionalise" medical authority over intersex bodies. On surgical rationales and outcomes, the article stated that:
blockquote|It is generally felt that surgery that is carried out for cosmetic reasons in the first year of life relieves parental distress and improves attachment between the child and the parents. The systematic evidence for this belief is lacking. information across a range of assessments is insufficient outcomes from clitoroplasty identify problems related to decreased sexual sensitivity, loss of clitoral tissue, and cosmetic issues Feminising as opposed to masculinising genitoplasty requires less surgery to achieve an acceptable outcome and results in fewer urological difficulties Long term data on sexual function and quality of life among those assigned female as well as male show great variability. There are no controlled clinical trials of the efficacy of early (less than 12 months of age) versus late surgery (in adolescence and adulthood), or of the efficacy of different techniques" Creighton and others in the UK have found that there have been few audits of the implementation of the 2006 statement, clitoral surgeries on under 14s have increased since 2006, and "recent publications in the medical literature tend to focus on surgical techniques with no reports on patient experiences".

Management practices for intersex conditions have evolved over the last 60 years. In recent decades surgical practices have become the subject of public and professional controversy, and evidence remains lacking.

Parents are frequently considered able to consent to feminizing or masculinizing interventions on their child, and this may be considered standard for the treatment of physical disorders. However this is contested, particularly where interventions seek to address psychosocial concerns. A BMJ editorial in 2015 stated that parents are unduly influenced by medicalized information, may not realize that they are consenting to experimental treatments, and regret may be high. Child rights expert Kirsten Sandberg states that parents have no right to consent to such treatments.

Reports published in the early 1990s state that 20 50% of surgical cases result in a loss of sexual sensation. A 2007 paper by Yang, Felsen and Poppas provided what the authors believe is the first study of clitoral sensitivity after clitoris reduction surgery, but the research was itself the subject of ethical debate. Postoperative patients aged older than five years were "considered candidates" for clitoral sensitivity testing, and 10 of 51 patients were tested, with 9 undergoing extended vibratory sensory testing. The initial tests were performed on the inner thigh, labia majora, labia minora, vaginal introitus and clitoris, with a "cotton tip applicator" and extended tests with a biothesiometer, a medical device used to measure sensitivity thresholds. Values were recorded. The authors note that there are no control data "for assessment of the viability and function of the clitoris in unaffected women." The ethics of these tests have been criticized by bioethicists, and subsequently defended by the Office for Human Research Protections. Loss of sexual function and sensation remains a concern in a submission by the Australasian Paediatric Endocrine Group to the Australian Senate in 2013. In a major Parliamentary report in Australia, published in October 2013, the Senate Community Affairs References committee was "disturbed" by the possible implications of current practices in the treatment of cancer risk. The committee stated: "clinical intervention pathways stated to be based on probabilities of cancer risk may be encapsulating treatment decisions based on other factors, such as the desire to conduct normalising surgery Treating cancer may be regarded as unambiguously therapeutic treatment, while normalising surgery may not. Thus basing a decision on cancer risk might avoid the need for court oversight in a way that a decision based on other factors might not. The committee is disturbed by the possible implications of this " Medical professionals have traditionally considered the worst outcomes after genital reconstruction in infancy to occur when the person develops a gender identity discordant with the sex assigned as an infant. Most of the cases in which a child or adult has voluntarily changed sex and rejected sex of assignment and rearing have occurred in partially or completely virilized genetic males who were reassigned and raised as females. This is the management practice that has been most thoroughly undermined in recent decades, as a result of a small number of spontaneous self reassignments to male. Reducing the likelihood of a gender "mismatch" is also a claimed advantage of deferring reconstructive surgery until the patient is old enough to assess gender identity with confidence. Human rights institutions question such approaches as being "informed by redundant social constructs around gender and biology".

Parents may be advised that without surgery, their child will be stigmatized, but they may make different choices with non medicalized information. However, there is no evidence that surgeries help children grow up psychologically healthy. Unlike other aesthetic surgical procedures performed on infants, such as corrective surgery for a cleft lip (as opposed to a cleft palate), genital surgery may lead to negative consequences for sexual functioning in later life (such as loss of sensation in the genitals, for example, when a clitoris deemed too large or penis is reduced/removed), or feelings of freakishness and unacceptability, which may have been avoided without the surgery. Studies have revealed how surgical intervention has had psychological effects, affecting well being and quality of life. Genital surgeries do not ensure a successful psychological outcome for the patient and might require psychological support when the patient is trying to distinguish a gender identity. The Swiss National Advisory Commission on Biomedical Ethics states that, where "interventions are performed solely with a view to integration of the child into a family and social environment, then they run counter to the child's welfare. In addition, there is no guarantee that the intended purpose (integration) will be achieved." along with their ethics, control and usage.

Additionally, parents are not often consulted on the decision making process when choosing the sex of the child, and they may be advised to conceal information from their child. The Intersex Society of North America stated that "For decades, doctors have thought it necessary to treat intersex with a concealment centered approach, one that features downplaying intersex as much as possible, even to the point of lying to patients about their conditions."

In 2015, an editorial in the BMJ described current surgical interventions as experimental, stating that clinical confidence in constructing "normal" genital anatomies has not been borne out, and that medically credible pathways other than surgery do not yet exist.