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. In 2011, Christiane Völling won the first successful case brought against a surgeon for non-consensual surgical intervention. In 2015, the Council of Europe recognized, for the first time, a right for intersex persons not to undergo sex-assignment treatment and Malta became the first country to prohibit involuntary or coerced modifications to sex characteristics.
Purposes of genital reconstructive surgery.
The goals of surgery vary with the type of intersex condition but usually include one or more of the following:
Physical health rationales:
Psychosocial rationales:
Both sets of rationales may be the subject of debate, particularly as the consequences of surgical interventions are lifelong and irreversible. Questions regarding physical health include accurately assessing risk levels, necessity and timing. Psychosocial rationales are particularly susceptible to questions of necessity as they reflect parental, social, and cultural concerns. There remains no clinical consensus or clear evidence regarding surgical timing, necessity, type of surgical intervention, degree of difference warranting intervention and evaluation method. Such surgeries are the subject of significant contention, including community activism, and multiple reports by international human rights and health institutions and national ethics bodies.
Types of intervention.
Interventions include:
Surgical interventions can broadly be divided into "masculinizing surgical procedures" intended to make genitalia more like those of typical XY-males, and "feminizing surgical procedures" intended to make genitalia more like those of typical XX-females. There are multiple techniques or approaches for each procedure. Some of these are needed for variations in degrees of physical difference. Techniques and procedure have evolved over the last 60 years. Some of the different techniques have been devised to reduce complications associated with earlier techniques. There remains a lack of consensus on surgeries, and some clinicians still regard them as experimental.
Some children receive a combination of procedures. For example, a child regarded as a severely undervirilized boy with a pseudovaginal perineoscrotal hypospadias may have midline urogenital closure, third degree hypospadias repair, chordee release and phalloplasty, and orchiopexy performed. A child regarded as a severely virilized girl with congenital adrenal hyperplasia (CAH) may undergo both a partial clitoral recession and a vaginoplasty.
Masculinizing surgical procedures.
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.
Feminizing surgical procedures.
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