Intersex surgery
Encyclopedia
Intersex surgery is one of several terms referring to surgery
performed to correct birth defects or early injuries of the genitalia, primarily for the purposes of making the appearance more normal and to reduce the likelihood of future problems. The recent history of intersex surgery
has been characterized by controversy after publicized reports that surgery failed to achieve the desired outcomes in many cases. Timing of surgery (infancy, adolescence or adult age) has also been controversial.
Intersex surgery is a form of genital reconstructive surgery
, which also includes surgery performed for the purpose of transforming normal adult genitalia of one sex to that of the other (discussed elsewhere as sex reassignment surgery
).
condition. Undervirilized
boys typically have at least one of these two procedures performed in addition to others described below.
Types of undervirization and malformation for which some type of masculinizing surgery has been performed most often in the last 50 years are:
The disorders above comprised over 90% of reported surgical series from North America and Europe. 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 genetic females with CAH
is even rarer.
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
.
Hypospadias
repair is a relatively simple single procedure if the hypospadias is first or second degree (urethra
l opening on glans or shaft respectively) and the penis
is otherwise normal. Repair of a 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. (Glassberg, 1999)
Urogenital closure describes 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
.
Gonadectomy (also referred to as "orchiectomy") refers to removal of the gonads. This is done in three circumstances. (1) If the gonad
s 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 with completely virilizing congenital adrenal hyperplasia
(Prader stage 5) is being raised as a male, the ovaries
must be removed before puberty to prevent breast
development and/or menstruation
. (3) Gonadectomy would be needed for the equally rare instance of a child with true hermaphrodite
virilized enough to raise as male: any ovary or ovotestis
would need to be removed. (Manuel, 1976)
Chordee
release refers to 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.
Cloacal repair is among the most complex of the surgeries described here. Bladder exstrophy
or more severe cloacal exstrophy
refers to 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. (Schober, 2002)
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.
Hysterectomy
is removal of a uterus
. It is rare that a uterus
or mullerian duct
derivatives would need to be removed from a child being raised as a boy. The most common scenario is accidental discovery of persistent mullerian 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.
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.
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 augmenation 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
.
Concealed penis is the term used to describe a normal penis 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. (Casale, 1999)
conditions and birth defects in order to make the genitalia more like those of normal females:
There are rarer causes of virilization of genetic females or undervirililizaton of genetic males (see intersex
for a fuller list), as well as some less easily categorized types of intersex conditions or other malformations of the genitalia. In the last decade, feminizing surgery to support reassignment of genetic males with non-ambiguous micropenis has been largely discontinued, and surgical reassignment of genetic males with exstrophy or other severe malformations or injuries is diminishing. See history of intersex surgery
.
Clitorectomy describes amputation or removal of most of the clitoris
, including glans
, erectile tissue, and nerve
s. 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.
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.
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. (Rangecroft, 2001)
Clitoral reduction was developed in the 1980s to reduce size without reducing function. Lateral wedges of the erectile tissue of the clitoris are removed to reduce the size and protrusion. The neurovascular tissue is carefully spared to preserve function and sensation. Nerve stimulation and sensory responses are now often performed during the surgery to confirm function of the sensory nerves. (Chase, 1996; Rangecroft, 2001))
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 mullerian 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. (Creighton, 2001; Rink, 1998; Schnitzer, 2001)
Gonadectomy refers to removal of the gonads. If the gonad
s 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; abnormal 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. (Manuel, 1976)
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 last decade, many of these infants were assigned and raised as females, with fashioning of a vagina and gonadectomy as part of the perineal reconstruction. (Schober, 2002)
for more detail.
Is functional outcome better when surgery is performed in infancy, in adolescence, or adulthood, for vaginoplasty for markedly virilized females (e.g., from congenital adrenal hyperplasia, mixed gonadal dysgenesis, or partial androgen insensitivity)?
Do any advantages of infant clitoral reduction surgery outweigh the potential disadvantages of reduced or distorted sexual sensation? Clitoral reduction is rarely done except in combination with vaginoplasty when substantial virilization is present.
Should parents have the same ethical and legal right to consent on behalf of their child to genital surgery as to consent to other reconstructive surgery (e.g., cleft lip repair or birth mark removal) for largely psychosocial purposes?
How can we minimize gender identity problems? Is it valid to assume in cases of ambiguous genitalia that the magnitude of the "innate" tendency to develop a specific gender identity
is usually similar to the degree of genital virilization? Should we abandon completely the idea that an unambiguous XY child with an irreparably defective penis might be better off raised as a girl?
Within the last decade, some people have raised the question of whether surgery to correct abnormal genitalia should be done at all, especially for purposes of changing appearance. Opponents of all "corrective surgery" on abnormal genitalia suggest we should be attempting to change social opinion regarding the desirability of having genitalia that look more average, rather than performing surgery to try to make them more like other peoples'.
Historical background, supporting arguments, and changing practice standards are treated in more detail in History of intersex surgery
, and in some of the following references (Creighton, 2001).
Surgery
Surgery is an ancient medical specialty that uses operative manual and instrumental techniques on a patient to investigate and/or treat a pathological condition such as disease or injury, or to help improve bodily function or appearance.An act of performing surgery may be called a surgical...
performed to correct birth defects or early injuries of the genitalia, primarily for the purposes of making the appearance more normal and to reduce the likelihood of future problems. The recent history of intersex surgery
History of intersex surgery
The history of intersex surgery is intertwined with the development of the specialities of pediatric surgery, pediatric urology, and pediatric endocrinology, with our increasingly refined understanding of sexual differentiation, with the development of political advocacy groups united by a sexual...
has been characterized by controversy after publicized reports that surgery failed to achieve the desired outcomes in many cases. Timing of surgery (infancy, adolescence or adult age) has also been controversial.
Intersex surgery is a form of genital reconstructive surgery
Genital reconstructive surgery
Genital reconstructive surgery refers to surgery performed on the genitalia of infants, children, or adults for the purpose of correcting birth defects or other anatomic abnormalities, or for the purpose of transforming normal genitalia of one sex into genitalia resembling the other...
, which also includes surgery performed for the purpose of transforming normal adult genitalia of one sex to that of the other (discussed elsewhere as sex reassignment surgery
Sex reassignment surgery
Sex reassignment surgery is a term for the surgical procedures by which a person's physical appearance and function of their existing sexual characteristics are altered to resemble...
).
Purposes of genital reconstructive surgery
The goals of surgery vary with the type of abnormality but usually include one or more of the following:- to improve the potential for fertilityFertilityFertility is the natural capability of producing offsprings. As a measure, "fertility rate" is the number of children born per couple, person or population. Fertility differs from fecundity, which is defined as the potential for reproduction...
- to provide an outlet for menstruationMenstruationMenstruation is the shedding of the uterine lining . It occurs on a regular basis in sexually reproductive-age females of certain mammal species. This article focuses on human menstruation.-Overview:...
- to prevent or reduce urinary tract infectionUrinary tract infectionA urinary tract infection is a bacterial infection that affects any part of the urinary tract. Symptoms include frequent feeling and/or need to urinate, pain during urination, and cloudy urine. The main causal agent is Escherichia coli...
s or obstructionObstructive uropathyObstructive uropathy is a structural or functional hindrance of normal urine flow, sometimes leading torenal dysfunction .It is a very broad term, and does not imply a location or etiology.-Causes:... - to reduce risk of cancerCancerCancer , known medically as a malignant neoplasm, is a large group of different diseases, all involving unregulated cell growth. In cancer, cells divide and grow uncontrollably, forming malignant tumors, and invade nearby parts of the body. The cancer may also spread to more distant parts of the...
in abnormal gonadGonadThe gonad is the organ that makes gametes. The gonads in males are the testes and the gonads in females are the ovaries. The product, gametes, are haploid germ cells. For example, spermatozoon and egg cells are gametes...
s. - to close open wounds or exposed internal organs
- to improve urinaryUrinary incontinenceUrinary incontinence is any involuntary leakage of urine. It is a common and distressing problem, which may have a profound impact on quality of life. Urinary incontinence almost always results from an underlying treatable medical condition but is under-reported to medical practitioners...
or fecal continenceFecal incontinenceFecal incontinence is the loss of regular control of the bowels. Involuntary excretion and leaking are common occurrences for those affected. Subjects relating to defecation are often socially unacceptable, thus those affected may be beset by feelings of shame and humiliation...
. - to alleviate parental distress over the atypical genital appearance.
- to make the appearance more normal for the person's sex of rearing
- to reduce effects of abnormal genitalia on psychosexual development and gender identityGender identityA gender identity is the way in which an individual self-identifies with a gender category, for example, as being either a man or a woman, or in some cases being neither, which can be distinct from biological sex. Basic gender identity is usually formed by age three and is extremely difficult to...
- to improve the potential for adult sexual relationships
Types of surgery
Genital reconstructive surgery can be divided into masculinizing surgical procedures intended to make the genitalia more like those of typical males, and feminizing surgical procedures intended to make the genitalia more like those of typical females.- There are several techniques or approaches for each procedure.
- Some of the variations of procedure are needed for varying degrees of severity of the abnormalities.
- Some of the different techniques have been devised to reduce complications associated with earlier techniques.
- Techniques and procedure have evolved over the last 60 years. Some have been considered obsolete for decades.
- Some children need a combination of procedures.
- For example, a severely undervirilized boy with a pseudovaginal perineoscrotal hypospadiasPseudovaginal perineoscrotal hypospadiasPseudovaginal perineoscrotal hypospadias refers to a configuration of the external genitalia of an infant. In a sense, this configuration is roughly midway between normal male genitalia and normal female genitalia in structure and appearance...
may have midline urogenital closure, third degree hypospadias repair, chordee release and phalloplastyPhalloplastyPhalloplasty refers to the construction of a penis or, sometimes, artificial modification of the penis by surgery, often for cosmetic purposes. It is also occasionally used to refer to penis enlargement....
, and orchiopexyOrchiopexyOrchiopexy is a surgery to move an undescended testicle into the scrotum and permanently fix it there. It is performed by a pediatric urologist or surgeon on boys with cryptorchidism, typically before they reach the age of two...
performed. A severely virilized girl with congenital adrenal hyperplasiaCongenital adrenal hyperplasiaCongenital adrenal hyperplasia refers to any of several autosomal recessive diseases resulting from mutations of genes for enzymes mediating the biochemical steps of production of cortisol from cholesterol by the adrenal glands ....
(CAH) may undergo both a clitoral reduction and a vaginoplastyVaginoplastythumb|right|300px|Vaginoplasty: the pre-operative aspect , and the post-operative aspect of a [[Labiaplasty|labial reduction]].Vaginoplasty is a reconstructive plastic surgery procedure for correcting the defects and deformities of the vaginal canal and its mucous membrane, and of vulvo-vaginal...
.
- For example, a severely undervirilized boy with a pseudovaginal perineoscrotal hypospadias
Masculinizing surgical procedures
Orchiopexy and hypospadias repair are the most common types of genital corrective surgery performed in infant boys, but most of these boys have no other abnormalities and are not considered to have an intersexIntersex
Intersex, in humans and other animals, is the presence of intermediate or atypical combinations of physical features that usually distinguish female from male...
condition. Undervirilized
Virilization
In biology and medicine, virilization refers to the biological development of sex differences, changes that make a male body different from a female body. Most of the changes of virilization are produced by androgens...
boys typically have at least one of these two procedures performed in addition to others described below.
Types of undervirization and malformation for which some type of masculinizing surgery has been performed most often in the last 50 years are:
- the configuration of ambiguous genitalia referred to as pseudovaginal perineoscrotal hypospadiasPseudovaginal perineoscrotal hypospadiasPseudovaginal perineoscrotal hypospadias refers to a configuration of the external genitalia of an infant. In a sense, this configuration is roughly midway between normal male genitalia and normal female genitalia in structure and appearance...
(PPSH)- the Reifenstein type of partial androgen insensitivity syndromeAndrogen insensitivity syndromeAndrogen insensitivity syndrome is a condition that results in the partial or complete inability of the cell to respond to androgens. The unresponsiveness of the cell to the presence of androgenic hormones can impair or prevent the masculinization of male genitalia in the developing fetus, as...
- gonadal dysgenesisGonadal dysgenesisGonadal dysgenesis is a term used to describe multiple reproductive system development disorders. They are conditions of genetic origin. It is characterized by a progressive loss of primordial germ cells on the developing gonads of an embryo....
disorders such as mixed gonadal dysgenesisMixed gonadal dysgenesisMixed gonadal dysgenesis is a condition of unusual and asymmetrical gonadal development leading to an unassigned sex differentiation. A number of differences have been reported in the karyotype, most commonly a mosaicism 45,X/ 46, XY...
and testicular dysgenesis - idiopathic (specific cause not determined)
- the Reifenstein type of partial androgen insensitivity syndrome
- birth defects of male genitalia
- bladder exstrophyBladder exstrophyBladder exstrophy is a congenital anomality in which part of the urinary bladder is present outside the body. It is rare, occurring once every 10,000 to 50,000 live births with a 2:1 male:female ratio. The diagnosis involves a spectrum of anomalies of the lower abdominal wall, bladder, anterior...
and epispadiasEpispadiasAn epispadias is a rare type of malformation of the penis in which the urethra ends in an opening on the upper aspect of the penis. It can also develop in females when the urethra develops too far anteriorly...
spectrum - chordeeChordeeChordee is a condition in which the head of the penis curves downward or upward, at the junction of the head and shaft of the penis. The curvature is usually most obvious during erection, but resistance to straightening is often apparent in the flaccid state as well. In many cases but not all,...
(simple or as part of undervirilization) - micropenisMicropenisMicropenis is an unusually small penis. A common criterion is a dorsal erect penile length of at least 2.5 standard deviations smaller than the mean human penis size. The condition is usually recognized shortly after birth...
- concealed penis
- bladder exstrophy
The disorders above comprised over 90% of reported surgical series from North America and Europe. In a few parts of the world 5-alpha-reductase deficiency
5-alpha-reductase deficiency
5-Alpha-reductase deficiency is an autosomal recessive intersex condition caused by a mutation of the 5-alpha reductase type 2 gene.-Normal function:...
or defects of testosterone
Testosterone
Testosterone is a steroid hormone from the androgen group and is found in mammals, reptiles, birds, and other vertebrates. In mammals, testosterone is primarily secreted in the testes of males and the ovaries of females, although small amounts are also secreted by the adrenal glands...
synthesis, or even rarer forms of intersex
Intersex
Intersex, in humans and other animals, is the presence of intermediate or atypical combinations of physical features that usually distinguish female from male...
account for a significant portion of cases but these are rare in North America and Europe. Masculinizing surgery for completely virilized genetic females with CAH
Congenital adrenal hyperplasia
Congenital adrenal hyperplasia refers to any of several autosomal recessive diseases resulting from mutations of genes for enzymes mediating the biochemical steps of production of cortisol from cholesterol by the adrenal glands ....
is even rarer.
Orchiopexy
Orchiopexy
Orchiopexy is a surgery to move an undescended testicle into the scrotum and permanently fix it there. It is performed by a pediatric urologist or surgeon on boys with cryptorchidism, typically before they reach the age of two...
for repair of undescended testes (cryptorchidism
Cryptorchidism
Cryptorchidism is the absence of one or both testes from the scrotum. It is the most common birth defect regarding male genitalia. In unique cases, cryptorchidism can develop later in life, often as late as young adulthood. About 3% of full-term and 30% of premature infant boys are born with at...
) is the second most common surgery performed on infant male genitalia (after circumcision
Circumcision
Male circumcision is the surgical removal of some or all of the foreskin from the penis. The word "circumcision" comes from Latin and ....
). The surgeon moves one or both testes, with blood vessels, from an abdominal
Abdomen
In vertebrates such as mammals the abdomen constitutes the part of the body between the thorax and pelvis. The region enclosed by the abdomen is termed the abdominal cavity...
or inguinal
Inguinal canal
The inguinal canal is a passage in the anterior abdominal wall which in men conveys the spermatic cord and in women the round ligament. The inguinal canal is larger and more prominent in men.-Site:...
position to the scrotum
Scrotum
In some male mammals the scrotum is a dual-chambered protuberance of skin and muscle containing the testicles and divided by a septum. It is an extension of the perineum, and is located between the penis and anus. In humans and some other mammals, the base of the scrotum becomes covered with curly...
. If the inguinal canal is open it must be closed to prevent hernia
Hernia
A hernia is the protrusion of an organ or the fascia of an organ through the wall of the cavity that normally contains it. A hiatal hernia occurs when the stomach protrudes into the mediastinum through the esophageal opening in the diaphragm....
.
- Potential problems: Maintaining the blood supply is the major challenge. If vessels cannot be stretched into the scrotum, or are separated and cannot be reconnected, a testis will die and atrophy.
Hypospadias
Hypospadias
Hypospadias is a birth defect of the urethra in the male that involves an abnormally placed urinary meatus...
repair is a relatively simple single procedure if the hypospadias is first or second degree (urethra
Urethra
In anatomy, the urethra is a tube that connects the urinary bladder to the genitals for the removal of fluids out of the body. In males, the urethra travels through the penis, and carries semen as well as urine...
l opening on glans or shaft respectively) and the penis
Penis
The penis is a biological feature of male animals including both vertebrates and invertebrates...
is otherwise normal. Repair of a third degree hypospadias (urethral opening on perineum
Perineum
In human anatomy, the perineum is a region of the body including the perineal body and surrounding structures...
or in urogenital opening) is more challenging, may be done in stages, and has a significant rate of complications and unsatisfactory outcomes. (Glassberg, 1999)
- Potential 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.
Urogenital closure describes closure of any midline opening at the base of the penis. In severe undervirilization
Virilization
In biology and medicine, virilization refers to the biological development of sex differences, changes that make a male body different from a female body. Most of the changes of virilization are produced by androgens...
a boy may have a "pseudovaginal pouch" or a single urogenital opening in the midline of the perineum
Perineum
In human anatomy, the perineum is a region of the body including the perineal body and surrounding structures...
.
- Potential 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 and scarring are the main risks.
Gonadectomy (also referred to as "orchiectomy") refers to removal of the gonads. This is done in three circumstances. (1) If the gonad
Gonad
The gonad is the organ that makes gametes. The gonads in males are the testes and the gonads in females are the ovaries. The product, gametes, are haploid germ cells. For example, spermatozoon and egg cells are gametes...
s are dysgenetic
Gonadal dysgenesis
Gonadal dysgenesis is a term used to describe multiple reproductive system development disorders. They are conditions of genetic origin. It is characterized by a progressive loss of primordial germ cells on the developing gonads of an embryo....
testes or streak gonads and at least some of the boy's cells have a Y chromosome
Y chromosome
The Y chromosome is one of the two sex-determining chromosomes in most mammals, including humans. In mammals, it contains the gene SRY, which triggers testis development if present. The human Y chromosome is composed of about 60 million base pairs...
, the gonads or streaks must be removed because they are nonfunctional but have a relatively high risk of developing gonadoblastoma
Gonadoblastoma
A gonadoblastoma is a complex neoplasm composed of a mixture of gonadal elements, such as large primordial germ cells, immature Sertoli cells or granulosa cells of the sex cord, and gonadal stromal cells.-Associations:...
. (2) In rare instances when an XX child with completely virilizing congenital adrenal hyperplasia
Congenital adrenal hyperplasia due to 21-hydroxylase deficiency
Congenital adrenal hyperplasia due to 21-hydroxylase deficiency , in all its forms, accounts for over 95% of diagnosed cases of congenital adrenal hyperplasia, and "CAH" in most contexts refers to 21-hydroxylase deficiency...
(Prader stage 5) is being raised as a male, the ovaries
Ovary
The ovary is an ovum-producing reproductive organ, often found in pairs as part of the vertebrate female reproductive system. Ovaries in anatomically female individuals are analogous to testes in anatomically male individuals, in that they are both gonads and endocrine glands.-Human anatomy:Ovaries...
must be removed before puberty to prevent breast
Breast
The breast is the upper ventral region of the torso of a primate, in left and right sides, which in a female contains the mammary gland that secretes milk used to feed infants.Both men and women develop breasts from the same embryological tissues...
development and/or menstruation
Menstruation
Menstruation is the shedding of the uterine lining . It occurs on a regular basis in sexually reproductive-age females of certain mammal species. This article focuses on human menstruation.-Overview:...
. (3) Gonadectomy would be needed for the equally rare instance of a child with true hermaphrodite
True hermaphroditism
True hermaphroditism is a medical term for an intersex condition in which an individual is born with ovarian and testicular tissue.There may be an ovary on one side and a testis on the other, but more commonly one or both gonads is an ovotestis containing both types of tissue.It is rare—so...
virilized enough to raise as male: any ovary or ovotestis
Ovotestis
An ovotestis is a gonad with both testicular and ovarian aspects. In humans, ovotestes are an anatomical abnormality associated with gonadal dysgenesis.- In gastropods :...
would need to be removed. (Manuel, 1976)
- Potential problems: Gonadectomy involves little risk beyond that of any abdominal surgery.
Chordee
Chordee
Chordee is a condition in which the head of the penis curves downward or upward, at the junction of the head and shaft of the penis. The curvature is usually most obvious during erection, but resistance to straightening is often apparent in the flaccid state as well. In many cases but not all,...
release refers to 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
Pseudovaginal perineoscrotal hypospadias
Pseudovaginal perineoscrotal hypospadias refers to a configuration of the external genitalia of an infant. In a sense, this configuration is roughly midway between normal male genitalia and normal female genitalia in structure and appearance...
, is in the spectrum of ambiguous genitalia due to a number of conditions.
- Potential problems: 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 colonColon (anatomy)The colon is the last part of the digestive system in most vertebrates; it extracts water and salt from solid wastes before they are eliminated from the body, and is the site in which flora-aided fermentation of unabsorbed material occurs. Unlike the small intestine, the colon does not play a...
or upper rectumRectumThe rectum is the final straight portion of the large intestine in some mammals, and the gut in others, terminating in the anus. The human rectum is about 12 cm long...
and skin or other cavities, or between urethra and perineum.
Cloacal repair is among the most complex of the surgeries described here. Bladder exstrophy
Bladder exstrophy
Bladder exstrophy is a congenital anomality in which part of the urinary bladder is present outside the body. It is rare, occurring once every 10,000 to 50,000 live births with a 2:1 male:female ratio. The diagnosis involves a spectrum of anomalies of the lower abdominal wall, bladder, anterior...
or more severe cloacal exstrophy
Cloacal exstrophy
Cloacal exstrophy is a severe birth defect wherein much of the abdominal organs are exposed. It often causes the splitting of both male and female genitalia , and the anus is occasionally sealed.Cloacal exstrophy is an extremely rare birth defect, present in only one in 200,000 pregnancies - one...
refers to 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
Bifid penis
A bifid penis is a rare congenital defect where two genital tubercles develop.Historically, males born with a bifid penis often underwent sex reassignment surgery, due to the difficulty of penile reconstruction. They were raised as girls, and often had reconstructive surgery to make them...
(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
Colostomy
A colostomy is a surgical procedure in which a stoma is formed by drawing the healthy end of the large intestine or colon through an incision in the anterior abdominal wall and suturing it into place. This opening, in conjunction with the attached stoma appliance, provides an alternative channel...
(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
Epispadias
An epispadias is a rare type of malformation of the penis in which the urethra ends in an opening on the upper aspect of the penis. It can also develop in females when the urethra develops too far anteriorly...
. Surgical repair for this is primarily a phalloplasty. (Schober, 2002)
- Potential 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 colostomyColostomyA colostomy is a surgical procedure in which a stoma is formed by drawing the healthy end of the large intestine or colon through an incision in the anterior abdominal wall and suturing it into place. This opening, in conjunction with the attached stoma appliance, provides an alternative channel...
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.
- Potential problems: 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
Hysterectomy
A hysterectomy is the surgical removal of the uterus, usually performed by a gynecologist. Hysterectomy may be total or partial...
is removal of a uterus
Uterus
The uterus or womb is a major female hormone-responsive reproductive sex organ of most mammals including humans. One end, the cervix, opens into the vagina, while the other is connected to one or both fallopian tubes, depending on the species...
. It is rare that a uterus
Uterus
The uterus or womb is a major female hormone-responsive reproductive sex organ of most mammals including humans. One end, the cervix, opens into the vagina, while the other is connected to one or both fallopian tubes, depending on the species...
or mullerian duct
Müllerian duct
Müllerian ducts are paired ducts of the embryo that run down the lateral sides of the urogenital ridge and terminate at the Müllerian eminence in the primitive urogenital sinus. In the female, they will develop to form the Fallopian tubes, uterus, cervix, and the upper two-third of the vagina; in...
derivatives would need to be removed from a child being raised as a boy. The most common scenario is accidental discovery of persistent mullerian derivatives or a small uterus during abdominal surgery of a normal boy for cryptorchidism
Cryptorchidism
Cryptorchidism is the absence of one or both testes from the scrotum. It is the most common birth defect regarding male genitalia. In unique cases, cryptorchidism can develop later in life, often as late as young adulthood. About 3% of full-term and 30% of premature infant boys are born with at...
, 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
Congenital adrenal hyperplasia due to 21-hydroxylase deficiency
Congenital adrenal hyperplasia due to 21-hydroxylase deficiency , in all its forms, accounts for over 95% of diagnosed cases of congenital adrenal hyperplasia, and "CAH" in most contexts refers to 21-hydroxylase deficiency...
(Prader stage 5) being raised as a male; ovaries
Ovary
The ovary is an ovum-producing reproductive organ, often found in pairs as part of the vertebrate female reproductive system. Ovaries in anatomically female individuals are analogous to testes in anatomically male individuals, in that they are both gonads and endocrine glands.-Human anatomy:Ovaries...
and uterus must be removed to prevent breast
Breast
The breast is the upper ventral region of the torso of a primate, in left and right sides, which in a female contains the mammary gland that secretes milk used to feed infants.Both men and women develop breasts from the same embryological tissues...
development and menstruation
Menstruation
Menstruation is the shedding of the uterine lining . It occurs on a regular basis in sexually reproductive-age females of certain mammal species. This article focuses on human menstruation.-Overview:...
by early adolescence.
- Potential problems: 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
Silastic
Silastic is a trademark registered in 1948 by Dow Corning Corporation for flexible, inert silicone elastomer...
are no longer available due to safety and perception-of-safety concerns.
- Potential 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 augmenation 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
Micropenis
Micropenis is an unusually small penis. A common criterion is a dorsal erect penile length of at least 2.5 standard deviations smaller than the mean human penis size. The condition is usually recognized shortly after birth...
.
- Potential problems: Reabsorption of the fat is common. Scarring resulting in interference with erectile function is less likely but more damaging.
Concealed penis is the term used to describe a normal penis 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. (Casale, 1999)
- Potential problems: The most common difficulty is recurrence with further weight gain. Scarring can occur.
Feminizing surgical procedures
In the last 50 years, the following procedures were most commonly performed for the following intersexIntersex
Intersex, in humans and other animals, is the presence of intermediate or atypical combinations of physical features that usually distinguish female from male...
conditions and birth defects in order to make the genitalia more like those of normal females:
- virilization due to congenital adrenal hyperplasiaCongenital adrenal hyperplasiaCongenital adrenal hyperplasia refers to any of several autosomal recessive diseases resulting from mutations of genes for enzymes mediating the biochemical steps of production of cortisol from cholesterol by the adrenal glands ....
- malformations of genitalia in genetic females
- urogenital sinus malformation
- cloacal exstrophyCloacal exstrophyCloacal exstrophy is a severe birth defect wherein much of the abdominal organs are exposed. It often causes the splitting of both male and female genitalia , and the anus is occasionally sealed.Cloacal exstrophy is an extremely rare birth defect, present in only one in 200,000 pregnancies - one...
- conditions involving severe undervirilization or malformations of genetic males, or infants with mixed genetic sex, to be assigned and raised as girls
- gonadal dysgenesisGonadal dysgenesisGonadal dysgenesis is a term used to describe multiple reproductive system development disorders. They are conditions of genetic origin. It is characterized by a progressive loss of primordial germ cells on the developing gonads of an embryo....
(various forms) - partial and complete androgen insensitivity syndromeAndrogen insensitivity syndromeAndrogen insensitivity syndrome is a condition that results in the partial or complete inability of the cell to respond to androgens. The unresponsiveness of the cell to the presence of androgenic hormones can impair or prevent the masculinization of male genitalia in the developing fetus, as...
- micropenisMicropenisMicropenis is an unusually small penis. A common criterion is a dorsal erect penile length of at least 2.5 standard deviations smaller than the mean human penis size. The condition is usually recognized shortly after birth...
- cloacalCloacal exstrophyCloacal exstrophy is a severe birth defect wherein much of the abdominal organs are exposed. It often causes the splitting of both male and female genitalia , and the anus is occasionally sealed.Cloacal exstrophy is an extremely rare birth defect, present in only one in 200,000 pregnancies - one...
and bladder exstrophyBladder exstrophyBladder exstrophy is a congenital anomality in which part of the urinary bladder is present outside the body. It is rare, occurring once every 10,000 to 50,000 live births with a 2:1 male:female ratio. The diagnosis involves a spectrum of anomalies of the lower abdominal wall, bladder, anterior...
- gonadal dysgenesis
There are rarer causes of virilization of genetic females or undervirililizaton of genetic males (see intersex
Intersex
Intersex, in humans and other animals, is the presence of intermediate or atypical combinations of physical features that usually distinguish female from male...
for a fuller list), as well as some less easily categorized types of intersex conditions or other malformations of the genitalia. In the last decade, feminizing surgery to support reassignment of genetic males with non-ambiguous micropenis has been largely discontinued, and surgical reassignment of genetic males with exstrophy or other severe malformations or injuries is diminishing. See history of intersex surgery
History of intersex surgery
The history of intersex surgery is intertwined with the development of the specialities of pediatric surgery, pediatric urology, and pediatric endocrinology, with our increasingly refined understanding of sexual differentiation, with the development of political advocacy groups united by a sexual...
.
Clitorectomy describes amputation or removal of most of the clitoris
Clitoris
The clitoris is a sexual organ that is present only in female mammals. In humans, the visible button-like portion is located near the anterior junction of the labia minora, above the opening of the urethra and vagina. Unlike the penis, which is homologous to the clitoris, the clitoris does not...
, including glans
Clitoral glans
The clitoral glans is an external portion of the clitoris.- Anatomy :It is covered by the clitoral hood, which is also external and attached to the labia minora...
, erectile tissue, and nerve
Nerve
A peripheral nerve, or simply nerve, is an enclosed, cable-like bundle of peripheral axons . A nerve provides a common pathway for the electrochemical nerve impulses that are transmitted along each of the axons. Nerves are found only in the peripheral nervous system...
s. 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 problems: The primary effect of this surgery, not surprisingly, is a drastic reduction in ability to experience orgasmOrgasmOrgasm is the peak of the plateau phase of the sexual response cycle, characterized by an intense sensation of pleasure...
. The appearance is not very normal. Regrowth of unwanted erectile tissue has sometimes presented problems.
Clitoroplasty
Clitoroplasty
Clitoroplasty is a term for the surgical creation of a clitoris , or restoration, in the case of procedures reversing the damage caused by female genital cutting....
, 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 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. (Rangecroft, 2001)
- Potential 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. (Minto, 2003)
Clitoral reduction was developed in the 1980s to reduce size without reducing function. Lateral wedges of the erectile tissue of the clitoris are removed to reduce the size and protrusion. The neurovascular tissue is carefully spared to preserve function and sensation. Nerve stimulation and sensory responses are now often performed during the surgery to confirm function of the sensory nerves. (Chase, 1996; Rangecroft, 2001))
- Potential problems: The degree to which the goal of preserving sexual sensations is attained is a subject of controversy. Many of the children who have had the newer versions of this procedure have not yet become adults.
Vaginoplasty
Vaginoplasty
thumb|right|300px|Vaginoplasty: the pre-operative aspect , and the post-operative aspect of a [[Labiaplasty|labial reduction]].Vaginoplasty is a reconstructive plastic surgery procedure for correcting the defects and deformities of the vaginal canal and its mucous membrane, and of vulvo-vaginal...
, the construction or reconstruction of a vagina
Vagina
The vagina is a fibromuscular tubular tract leading from the uterus to the exterior of the body in female placental mammals and marsupials, or to the cloaca in female birds, monotremes, and some reptiles. Female insects and other invertebrates also have a vagina, which is the terminal part of the...
, can be fairly simple or quite complex, depending on the initial anatomy. If a normal internal uterus, cervix and upper vagina (the mullerian derivatives
Müllerian duct
Müllerian ducts are paired ducts of the embryo that run down the lateral sides of the urogenital ridge and terminate at the Müllerian eminence in the primitive urogenital sinus. In the female, they will develop to form the Fallopian tubes, uterus, cervix, and the upper two-third of the vagina; in...
) 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
Androgen insensitivity syndrome
Androgen insensitivity syndrome is a condition that results in the partial or complete inability of the cell to respond to androgens. The unresponsiveness of the cell to the presence of androgenic hormones can impair or prevent the masculinization of male genitalia in the developing fetus, as...
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
Congenital adrenal hyperplasia
Congenital adrenal hyperplasia refers to any of several autosomal recessive diseases resulting from mutations of genes for enzymes mediating the biochemical steps of production of cortisol from cholesterol by the adrenal glands ....
, or (in the past) when a genetic male infant with a severely defective penis was reassigned
Sex assignment
Sex assignment refers to the assigning of the biological sex at the birth of a baby. In the majority of births, a relative, midwife, or physician inspects the genitalia when the baby is delivered, sees ordinary male or female genitalia, and declares, "it's a girl" or "it's a boy" without the...
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. (Creighton, 2001; Rink, 1998; Schnitzer, 2001)
- Potential 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. (Alizai, 1999; Lobe, 1987; Minto, 2003)
Gonadectomy refers to removal of the gonads. If the gonad
Gonad
The gonad is the organ that makes gametes. The gonads in males are the testes and the gonads in females are the ovaries. The product, gametes, are haploid germ cells. For example, spermatozoon and egg cells are gametes...
s are dysgenetic
Gonadal dysgenesis
Gonadal dysgenesis is a term used to describe multiple reproductive system development disorders. They are conditions of genetic origin. It is characterized by a progressive loss of primordial germ cells on the developing gonads of an embryo....
testes or streak gonads and at least some of the cells have a Y chromosome
Y chromosome
The Y chromosome is one of the two sex-determining chromosomes in most mammals, including humans. In mammals, it contains the gene SRY, which triggers testis development if present. The human Y chromosome is composed of about 60 million base pairs...
, the gonads or streaks must be removed because they are nonfunctional but have a relatively high risk of developing gonadoblastoma
Gonadoblastoma
A gonadoblastoma is a complex neoplasm composed of a mixture of gonadal elements, such as large primordial germ cells, immature Sertoli cells or granulosa cells of the sex cord, and gonadal stromal cells.-Associations:...
. 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
Androgen insensitivity syndrome
Androgen insensitivity syndrome is a condition that results in the partial or complete inability of the cell to respond to androgens. The unresponsiveness of the cell to the presence of androgenic hormones can impair or prevent the masculinization of male genitalia in the developing fetus, as...
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
True hermaphroditism
True hermaphroditism is a medical term for an intersex condition in which an individual is born with ovarian and testicular tissue.There may be an ovary on one side and a testis on the other, but more commonly one or both gonads is an ovotestis containing both types of tissue.It is rare—so...
must be directly examined; abnormal 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. (Manuel, 1976)
- Potential problems: Gonadectomy involves little risk beyond that of any abdominal surgery.
Cloacal exstrophy
Cloacal exstrophy
Cloacal exstrophy is a severe birth defect wherein much of the abdominal organs are exposed. It often causes the splitting of both male and female genitalia , and the anus is occasionally sealed.Cloacal exstrophy is an extremely rare birth defect, present in only one in 200,000 pregnancies - one...
and bladder exstrophy
Bladder exstrophy
Bladder exstrophy is a congenital anomality in which part of the urinary bladder is present outside the body. It is rare, occurring once every 10,000 to 50,000 live births with a 2:1 male:female ratio. The diagnosis involves a spectrum of anomalies of the lower abdominal wall, bladder, anterior...
repair is needed regardless of the sex of assignment
Sex assignment
Sex assignment refers to the assigning of the biological sex at the birth of a baby. In the majority of births, a relative, midwife, or physician inspects the genitalia when the baby is delivered, sees ordinary male or female genitalia, and declares, "it's a girl" or "it's a boy" without the...
or rearing. Simple bladder exstrophy in a genetic female does not usually involve the vagina
Vagina
The vagina is a fibromuscular tubular tract leading from the uterus to the exterior of the body in female placental mammals and marsupials, or to the cloaca in female birds, monotremes, and some reptiles. Female insects and other invertebrates also have a vagina, which is the terminal part of the...
. Cloacal exstrophy in a genetic female usually requires major surgical reconstruction of the entire perineum
Perineum
In human anatomy, the perineum is a region of the body including the perineal body and surrounding structures...
, including bladder
Urinary bladder
The urinary bladder is the organ that collects urine excreted by the kidneys before disposal by urination. A hollow muscular, and distensible organ, the bladder sits on the pelvic floor...
, clitoris
Clitoris
The clitoris is a sexual organ that is present only in female mammals. In humans, the visible button-like portion is located near the anterior junction of the labia minora, above the opening of the urethra and vagina. Unlike the penis, which is homologous to the clitoris, the clitoris does not...
, symphysis pubis, and both the vaginal introitus and urethra
Urethra
In anatomy, the urethra is a tube that connects the urinary bladder to the genitals for the removal of fluids out of the body. In males, the urethra travels through the penis, and carries semen as well as urine...
. However, the uterus
Uterus
The uterus or womb is a major female hormone-responsive reproductive sex organ of most mammals including humans. One end, the cervix, opens into the vagina, while the other is connected to one or both fallopian tubes, depending on the species...
and ovaries
Ovary
The ovary is an ovum-producing reproductive organ, often found in pairs as part of the vertebrate female reproductive system. Ovaries in anatomically female individuals are analogous to testes in anatomically male individuals, in that they are both gonads and endocrine glands.-Human anatomy: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 last decade, many of these infants were assigned and raised as females, with fashioning of a vagina and gonadectomy as part of the perineal reconstruction. (Schober, 2002)
- Potential 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 colostomyColostomyA colostomy is a surgical procedure in which a stoma is formed by drawing the healthy end of the large intestine or colon through an incision in the anterior abdominal wall and suturing it into place. This opening, in conjunction with the attached stoma appliance, provides an alternative channel...
or vesicostomy. Creating a functional urethra is difficult and poor healing, with scarring, stricture, or fistula can require a vesicostomy to prevent urinary incontinence. Construction of a functional anal sphincter can be equally difficult when this has been disrupted as well. Functional problems can warrant a temporary or long-term colostomyColostomyA colostomy is a surgical procedure in which a stoma is formed by drawing the healthy end of the large intestine or colon through an incision in the anterior abdominal wall and suturing it into place. This opening, in conjunction with the attached stoma appliance, provides an alternative channel...
. The added challenge for the most severely affected genetic females, and for genetic males who are being raised as females, is construction of a neovagina. Scarring is extensive and the lower torso disfigured even with the best outcomes. Finally, it has become apparent in recent years that some genetic males (without intersex conditions) who are reassignedSex assignmentSex assignment refers to the assigning of the biological sex at the birth of a baby. In the majority of births, a relative, midwife, or physician inspects the genitalia when the baby is delivered, sees ordinary male or female genitalia, and declares, "it's a girl" or "it's a boy" without the...
and raised as females have not developed a female gender identity and have sought reassignment back to male. (Reiner, 2004)
Controversies and unsettled questions
Management practices for several types of intersex conditions and other abnormalities and injuries of the genitalia have evolved over the last 50 years. In the last decade several of the surgical practices have become the subject of public and professional controversy. See History of intersex surgeryHistory of intersex surgery
The history of intersex surgery is intertwined with the development of the specialities of pediatric surgery, pediatric urology, and pediatric endocrinology, with our increasingly refined understanding of sexual differentiation, with the development of political advocacy groups united by a sexual...
for more detail.
Is functional outcome better when surgery is performed in infancy, in adolescence, or adulthood, for vaginoplasty for markedly virilized females (e.g., from congenital adrenal hyperplasia, mixed gonadal dysgenesis, or partial androgen insensitivity)?
- Argued or putative advantages of infant surgery
- Tissue is more elastic and heals better according to many surgeons.
- Genital surgery performed before the age of memory is less emotionally traumatic.
- Surgery in infancy avoids asking adolescent to make a decision that is stressful and difficult even for adults.
- Assuming infant surgery is successful, there is no barrier to engaging in normal sexual activities, and less distortion of psychosexual identity.
- Argued or putative advantages of surgery in adolescence or later
- If outcome is less than satisfactory, early surgery leaves a person wondering if she would have been better off without it.
- Any surgery not absolutely necessary for physical health should be postponed until the person is old enough to give informed consent; parents should not be empowered to make medical decisions for their children.
- Genital surgery should be handled differently than other birth defect surgery; this is the one type of surgery that parents should not be empowered to make decisions about because they will be under social pressure to make "bad" decisions.
- By mid-adolescence or later, a woman may decide that her abnormal genitalia do not need to be changed.
- Infant vaginoplasties should not be done because most women who have had them performed report some degree of difficulty with sexual function; even though we have no evidence that adult sexual function will be better if surgery is deferred, the outcomes couldn't be worse than they currently are after infant surgery.
Do any advantages of infant clitoral reduction surgery outweigh the potential disadvantages of reduced or distorted sexual sensation? Clitoral reduction is rarely done except in combination with vaginoplasty when substantial virilization is present.
- How much weight should be given to the cosmetic argument that there is value in making it more normal looking? See for example the letters following Melton, 2001.
Should parents have the same ethical and legal right to consent on behalf of their child to genital surgery as to consent to other reconstructive surgery (e.g., cleft lip repair or birth mark removal) for largely psychosocial purposes?
- The high court in ColombiaColombiaColombia, officially the Republic of Colombia , is a unitary constitutional republic comprising thirty-two departments. The country is located in northwestern South America, bordered to the east by Venezuela and Brazil; to the south by Ecuador and Peru; to the north by the Caribbean Sea; to the...
has ruled no http://www.isna.org/drupal/node/view/181, and some advocacy groups in the US and elsewhere agree (http://www.isna.org, comparing this type of surgery to genital mutilationGenital mutilationGenital mutilation can refer to:*Clitoridectomy*Female genital mutilation, also known as female circumcision*Genital modification and mutilation*Infibulation...
(intactivism).
How can we minimize gender identity problems? Is it valid to assume in cases of ambiguous genitalia that the magnitude of the "innate" tendency to develop a specific gender identity
Gender identity
A gender identity is the way in which an individual self-identifies with a gender category, for example, as being either a man or a woman, or in some cases being neither, which can be distinct from biological sex. Basic gender identity is usually formed by age three and is extremely difficult to...
is usually similar to the degree of genital virilization? Should we abandon completely the idea that an unambiguous XY child with an irreparably defective penis might be better off raised as a girl?
- 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 assignmentSex assignmentSex assignment refers to the assigning of the biological sex at the birth of a baby. In the majority of births, a relative, midwife, or physician inspects the genitalia when the baby is delivered, sees ordinary male or female genitalia, and declares, "it's a girl" or "it's a boy" without the...
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 the last decade, as a result of a small number of spontaneous self-reassignments back to male in a number of genetic males who had been raised as female because of birth defects of the penis which did not involve undervirilization (e.g., exstrophy or traumatic loss). - 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.
- However, support groups tend to identify intense feelings of shame and betrayal as the worst outcomes of a philosophy of management that focuses on normalizing the child's anatomy. Many individuals who have developed a discordant gender identity and rejected the sex assigned during infancy have done quite well after transition (Reiner 2004, Consortium 2006, http://home.vicnet.net.au/~aissg/anthony.htm). Gender identity may not be the most important variable to consider in caring for children with intersex conditions.
Within the last decade, some people have raised the question of whether surgery to correct abnormal genitalia should be done at all, especially for purposes of changing appearance. Opponents of all "corrective surgery" on abnormal genitalia suggest we should be attempting to change social opinion regarding the desirability of having genitalia that look more average, rather than performing surgery to try to make them more like other peoples'.
Historical background, supporting arguments, and changing practice standards are treated in more detail in History of intersex surgery
History of intersex surgery
The history of intersex surgery is intertwined with the development of the specialities of pediatric surgery, pediatric urology, and pediatric endocrinology, with our increasingly refined understanding of sexual differentiation, with the development of political advocacy groups united by a sexual...
, and in some of the following references (Creighton, 2001).
See also
- History of intersex surgeryHistory of intersex surgeryThe history of intersex surgery is intertwined with the development of the specialities of pediatric surgery, pediatric urology, and pediatric endocrinology, with our increasingly refined understanding of sexual differentiation, with the development of political advocacy groups united by a sexual...
- Intersexuality
- Sex reassignment surgerySex reassignment surgerySex reassignment surgery is a term for the surgical procedures by which a person's physical appearance and function of their existing sexual characteristics are altered to resemble...