Hermaphroditism in humans: causes, symptoms, treatment. False hermaphroditism - an anomaly of gonadal dysgenesis

False hermaphroditism is a disease accompanied by a violation in the development of the gonad in an individual of either sex. As a result, a person manages to visualize both the development and functioning of the gland, which is characteristic of both sexes.
In medicine, there are two types of such changes. This may be a true mixed structure of the genital organs with the presence of testicles and ovaries. More often, a false condition is revealed, which is expressed by a discrepancy in the structure of the external genitalia to the internal ones.

As the causes that form false hermaphroditism, the effect of adverse factors on a woman during pregnancy is singled out. Gynecology singles out the most critical periods when the laying and formation of the main organs and systems, including the sexual one, takes place. It is the pathogenic impact during these periods that provokes the development of irreversible changes.
These include:

Maternal use during pregnancy medicines, which have an androgenic effect or contain high doses of progesterone.
The presence in the body of girls androgen-secreting tumors that change the hormonal background, standing out in the blood.
The development of genetically determined hyperproduction of androgenic hormones in the body of a child.
Adrenogenital syndrome in the form of a congenital form, manifested in the prenatal period.

False female hermaphroditism

Androgenital syndrome in females is much less common. Its development is characterized by an increased synthesis of male sex hormones, causing disruption of the functioning of various systems. In the internal organs there is typical structure characteristic of women. Hermaphroditism in women is manifested by the fact that the ovaries are fully developed, and all changes relate to the external genitalia. The size of the labia majora decreases, while the clitoris may significantly exceed standard values. In some cases, especially in the first time after birth, it may be difficult to determine the sex. With severe health, fusion of the vulva is possible. The urethra stands out against the background of the modified clitoris.

External signs of hermaphroditism in women

Manifestations are accompanied by:

Strengthening the development of muscles, with the appearance of broad shoulders and a smoothing of the waistline. When playing sports, muscle mass develops in a short time.
Volume increase hairline. Fine vellus hair gradually becomes denser, the color is black, and the growth rate is intense. Patients are characterized by the appearance of hair in the area of the white line of the abdomen, above the lip and chin. In this case, an even contour on the pubis is lost.
Gradually there is a decrease in the timbre of the voice. As the pathology progresses, these anomalies become more pronounced.
Patients change their behavior, more like a man. This disrupts sexual behavior with an attraction to female gender, there is shyness when communicating with others. changing taste preference when choosing clothes and caring for appearance. You can often see these women in trousers and voluminous sweaters that hide their figure. Haircuts are shorter, makeup may be completely absent.

Male pseudohermaphroditism

False male pseudohermaphroditism is serious condition characterized by a violation of the formation of the scrotum and penis. The scrotum has small size, which can be confused with the development of cryptorchidism. Only during palpation and ultrasound scanning is detected normal size testicles with fully developed appendages. The external genitalia resemble female externally. The size of the penis is insignificant and in some cases it does not extend beyond the urethra. The shape of the scrotum resembles folding in the form of large labia.
Secondary sexual characteristics are:

Poor hair development. The hair on the body has a fluffy character with a light shade and a small thickness. They may be slightly on their face, while after puberty a beard and sideburns do not form in a man. On the body, as well as on the genitals, there is no hairline.
The nipples are underdeveloped, the peripapillary fields are not sufficiently pronounced. A small breast is formed.
Muscles are weakly expressed. Even against the background of sports, the formation of muscles similar to the female type occurs.
In men, the waist appears with an expansion of the pelvis. Fat deposits localized mainly on the hips, the shoulder girdle is narrow.
Behavior becomes more feminine, voice more subtle, and facial features become more refined.

Diagnostics

On the early stages It is not always possible to immediately make a diagnosis. Changes in the external genitalia in newborns can be difficult to distinguish from the norm. That is why it is important to conduct a thorough examination of the baby, especially if there have been similar cases in the family or there are risk factors. It is necessary to suspect pathology when identifying symptoms such as a hypertrophied clitoris, the development of the labia, resembling the scrotum with skin fold covering the entrance to the vagina.
The main diagnostic method is currently considered to be an ultrasound scan. During the study of false female hermaphroditism, it is possible to identify the presence of testicles or ovaries with a developed uterus.
An obligatory step is to check the condition of the adrenal glands, as they can be the result of the formation of pathology. In severe diagnostic cases, laparoscopy is performed followed by a biopsy of suspicious elements. Important role karyotyping can play with the study of chromosomes in a patient and the detection of trisomies. Based on the data obtained, the issue of further management tactics by gynecologists, endocrinologists, urologists or geneticists is being decided.

Treatment

Psychotherapy is an important part in the treatment of false male hermaphroditism in women or female hermaphroditism in men. It is indicated for all patients with identified signs of violations in sexual perception.
The choice of method of therapy largely depends on the cause that led to the development given state as well as the severity pathological process. Only on the basis of data on the level of hormones and the state of the genital organs, the question of further treatment. The treatment of false male and female hermaphroditism is based on hormonal therapy.

The principle of hormone therapy

Hormone therapy is aimed at possible correction changes, when early start it is possible to correct external defects in the development of the organs of the reproductive system within a short time without the use of surgical intervention.

In addition, earlier treatment helps to exclude the development of problems associated with mental sphere. Therapy is long-term and in some cases it continues in women until the expected onset of menopause or for life, while the dynamics of the process should be regularly assessed.
The main objectives of hormone therapy are:

Regulation of the ratio of sex hormones.
Normalization of work thyroid gland and syndromes of its imbalance.
Ensuring a sufficient functional level of the adrenal glands.
Correction of the pituitary cells.

Surgical intervention

This method of treatment is indicated in the presence of severe cosmetic defects. Among the main goals, which carries surgical treatment false hermaphroditism in men, an increase in the size of the penis is distinguished, followed by straightening of its shape. Treatment of female hermaphroditism in women involves plastic surgery of the labia.
In some cases, men require surgery to treat cryptorchidism and correct abnormalities in the tubule or scrotum.
In the presence of a tumor process that has hormonal activity, these tissues are removed with a possible radical excision of surrounding structures, for example, a testicle or ovary.

Complications

Among the most common consequences are:

Violation reproductive function with infertility.
Absence of normal sexual intercourse.
Pathologies in sexual behavior.
Difficulties with urination.
Low adaptation of a person in society.

Early detection of pathology helps to reduce the risk of developing severe complications or their expression.

Video: Hermaphroditism psychosomatics

Nature clearly divided human organisms into male and female. The difference is manifested not only in the structure of the genital organs, but also in external data. Voice, hair growth pattern, mammary glands, fat distribution and muscle mass is closely related to the predominance of hormones of one's sex. But sometimes it is difficult to determine by external data whether it is a man or a woman. So hermaphroditism can manifest itself.

Varieties of violations of differentiation by sex

According to the timing of the appearance, hermaphroditism is divided into:

congenital;
acquired.

The first one depends on prenatal development the child and the correct formation of the pelvic organs. Acquired develops when organs that produce hormones are damaged, followed by the appearance of secondary sexual characteristics (for example, damage to the pituitary gland, hyperplasia of the adrenal cortex).

There are also two main types of hermaphroditism:

true, extremely rare medical practice about 200 cases have been registered;
false, which is male and female.

The genitalia develop from the mesonephric duct in boys and the paramesonephric duct in girls. Already at 3 weeks embryonic development the laying of organs corresponding to the sex begins.

The paramazonephric duct forms the uterus, appendages, and vagina. The mesonephric duct atrophies. If the rudiments of the genital organs of both sexes continue to coexist in the body, then true congenital hermaphroditism develops.

False female hermaphroditism develops somewhat differently. The reason is a defect in the enzyme system, which leads to a violation of the synthesis of cortisol in the fetal adrenal cortex and excessive production of androgens. The internal organs - the uterus, ovaries - develop in the normal way, but the effect of hormones leads to the formation of the external genital organs in the male pattern.

What's the Difference true hermaphroditism from false?

This can be explained based on the structure of the genital organs. If the glands of both sexes are found in an individual, then this condition is called true hermaphroditism.

The location of the gonads can be different:

bilateral - on each side there is an ovary and a testicle;
one-sided - on one side there is a male and female gonad, on the second - only one of them;
lateral - on each side there is a gonad of its own type - one female and one male;
bilateral - the gonad consists partly of the tissue of the ovary and testis.

False hermaphroditism is female and male. At the same time, the corresponding karyotype was preserved in both sexes. For women it is 46XX, for men 46XY. But outwardly, gender signs of the opposite sex are added.

Manifestations of various types of hermaphroditism

True hermaphroditism

True hermaphroditism has various manifestations. Its presence is not always determined at the birth of a child. Sometimes appearance genitals allows you to set the sex, but during the period of growing up, additional features. Patients apply for the first time for medical care when during puberty there is an underdevelopment of the female genital organs, if education and social formation took place as a girl.

Visually, the genitals with true hermaphroditism are formed in four types:

Predominantly female type - the clitoris is slightly enlarged, the opening to the vagina is preserved, there is a separate mouth urethra.
The clitoris is enlarged to a size that makes it similar to the penis, there may be skin folds that mimic foreskin, the mouth of the urethra and the entrance to the vagina are located separately.
The urethra has an exit to the vagina, there is a small penis, sometimes - the prostate gland.
The genitals are differentiated to the male side - the urethra comes out on the head of the penis, there is a scrotum. But in parallel, there is an underdeveloped vagina and uterus.

The testicle can be localized in various places:

in the scrotum;
a fold of skin resembling the labia;
in the inguinal canal;
abdominal cavity.

Often available inguinal hernia. The seminiferous tubules are usually atrophied, but spermatogenesis is preserved in some hermaphrodites. In 25% of cases are registered.

During puberty, signs of virilization develop, development can proceed according to the bisexual type. The voice remains low, the figure has both female and male features, hair grows in a male pattern, there are mammary glands. menstrual bleeding with the female type of development of the genital organs, they have normal view if the male type predominates, blood appears in the urine for the period of menstruation.

Violation of gender differentiation affects social behavior, leads to bisexuality, homosexuality, transsexuality.

False female hermaphroditism

The development of pathology occurs with hyperplasia of the adrenal cortex and the formation of adrenogenital syndrome.

The disease is associated with a tumor of the adrenal cortex or congenital hyperplasia of the cortical substance. Pathology can be congenital or acquired. The tumor can occur at any age, and the congenital form is inherited. Pathology can have several forms that differ in the clinical picture:

virilous;
salt-losing;
hypertensive;
virilny with periods of fever.

The manifestation of excessive synthesis of androgens is the formation of external sexual characteristics. Such patients have signs of hermaphroditism that make them look like men:

low voice;
developed muscles;
male pattern pubic hair growth;
excess body hair - hypertrichosis;
mustache and beard.

At the same time, the amount of anabolic hormones is increased. Therefore, in children with adrenogenital syndrome, there is fast growth. But due to the early closure of the ossification zones in the epiphyses of the bones, by the age of 9-13, growth completely stops, usually it does not exceed 145 cm.

In younger children school age the body is developed disproportionately: the body is long, the head is large, and the limbs are short. With congenital adrenogenital syndrome, the clitoris may be hypertrophied.

Changes in appearance lead to problems in the psychological sphere - such children can be shy, uncommunicative, with intellectual retardation, reduced or increased sexuality.

The salt-wasting form develops in newborns at 2-3 weeks of development. At the same time, symptoms of dehydration are observed, the child constantly vomits. Fluid loss cannot be eliminated by ingestion. Facial features are sharpened, the skin becomes dry, its turgor decreases, the complexion changes from pink to earthy. In severe cases, peripheral circulatory failure occurs, vascular collapse, there are convulsions, disturbances heart rate. Sodium and chlorine in this form are strongly excreted in the urine.

With hypertensive form clinical picture characterized by seizures arterial hypertension, which is resistant to the action of antihypertensive drugs, but can be corrected with the appointment of Prednisolone. permanent high blood pressure affects the state of target organs: observed characteristic change vessels of the kidneys, fundus, expansion of the left ventricle of the heart.

If adrenogenital syndrome is the result of a tumor in the reproductive age, then the woman develops the following symptoms:

or a menstrual disorder;
clitoral hypertrophy;
hypertrichosis;
atrophy of the mammary glands;

The severity of false hermaphroditism determines further tactics. If a gender discrepancy is found, it is necessary to conduct an examination and the necessary treatment.

Ways to establish the true gender

Even at the birth of a child for 3-4 days in the maternity hospital, a blood test is taken to identify genetic abnormalities - hypothyroidism, cystic fibrosis. These pathologies can affect mental development, but with timely prescribed treatment, clinical manifestations can be corrected or prevented.

If after the birth of a child there are difficulties with determining the sex, an additional examination is carried out.

During an external examination, doubts arise if there is a hypertrophied clitoris, the labia resemble the scrotum, the entrance to the vagina is covered with a skin fold or is absent. Sometimes the ovaries can be located in the labia. The outlet of the urethra may be at the base of the clitoris. ultrasound internal organs will reveal the presence of the uterus, ovaries or their absence. Also held ultrasound procedure adrenal glands.

Counseling is carried out jointly by doctors of several specialties - a gynecologist, urologist, endocrinologist and geneticist.

You can accurately determine the gender and causes of deviations using the following studies:

determines the set of chromosomes;
sex chromatin analysis;
study of the level of hormones: testosterone, estrogen, 17-corticosterone, follicle-stimulating and others.

True hermaphroditism confirmed diagnostic laparoscopy and gonadal biopsy. Histological examination helps to establish the state of the tissue, it corresponds to the ovaries or testicles.

Opportunities to eliminate gender mismatch

Awareness of their gender occurs in children of 2 years. Further mental and social development proceeds according to this knowledge. The discrepancy between the structure of the genitals and the appearance of one's gender leads to serious psychological overstrain. Therefore, it is important to diagnose abnormal sex formation as early as possible and choose a correction technique.

Hermaphroditism is treated with hormonal and surgical methods. With true hermaphroditism, tactics are determined by the severity of the external genitalia. If they are formed according to type 1-3, then the issue is resolved in favor of the female. With type 4 of the formation of the genital organs, the sex is male.

The upbringing of children with false hermaphroditism takes place according to female type. Removal of the hypertrophied clitoris is performed at the age of 1-3 years. The operation may include the separation of the fused labia, the formation of the entrance to the vagina. Sometimes, when the vagina is infected, its plastic surgery may be required, which is made from a flap of the peritoneum.

Hormonal therapy consists in the appointment of corticosteroids, which inhibit the excess synthesis of ACTH. At the same time, the production of androgens by the adrenal glands is inhibited. For the appearance of female signs, they are prescribed. Combinations can be used oral contraceptives, which will set the normal hormonal cycle. With insufficient growth, somatotropic hormone is prescribed.

Hormonal disorders during menopause are eliminated by the appointment of replacement therapy.

The effectiveness of the treatment of hermaphroditism depends on its causes, the severity of the signs and the time of initiation of therapy. The sooner this is done, the higher the chances of success. But not always, even with sex correction up to 6 years, it is possible to achieve complete feminization of girls.

In the treatment of signs of hermaphroditism that have developed in women reproductive age, with correction hormonal background the voice becomes softer, signs of hypertrichosis, hirsutism are eliminated. Gradually recovering menstrual cycle. A hypertrophied clitoris can only be reduced surgically.

For some patients, treatment by a psychologist and psychotherapist may be required to correct behavioral deviations.

This term refers to such a pathology of the development of the gonads, when an anatomically and functionally developed glands of both sexes are found in an individual. There are two types of hermaphroditism: true and false. True hermaphroditism, or bisexuality, is the presence of gonads of both sexes in one individual: an ovary and a testicle or gonads of a mixed structure (ovotestis). False hermaphroditism, or pseudohermaphroditism, is a discrepancy between the structure of the external genital organs and the nature of the gonads. There are false female and male pseudohermaphroditism.

False female hermaphroditism is much less common. The reasons for this form of hermaphroditism are: the pregnant mother of the unborn child taking doses of androgens or progesterone; the presence of an androgen-secreting tumor in the mother; genetically determined hyperproduction of androgens in a child, which began in utero - a congenital form of adrenogenital syndrome. On examination, the internal genital organs (vagina, uterus, the fallopian tubes and ovaries) are developed according to the female type, but at the same time, the rudiments of male sexual elements remain unreduced. The external genital organs are developed in a type approaching the male: the clitoris is enlarged, fused along middle line the labia majora resemble the scrotum, the urethra and vagina in the lower third are not separated and form the urogenital sinus, which opens under the enlarged clitoris.

Male pseudohermaphroditism is characterized by inverse relationships: in the presence of male gonads (testicles), the external genitalia more or less resemble the structure of women. On examination, a small penis is revealed, resembling an enlarged clitoris, hypospadias. Often the scrotum is split into two folds fused along the midline, resembling large genitals.

Diagnostics these vices is often difficult, especially in childhood. Therefore, mistakes are sometimes observed in the upbringing of such children: a female child is brought up as a boy, and vice versa.

Treatment. Defective testicles are surgically removed due to the risk of their malignancy. In the presence of an androgen-secreting tumor, its removal is indicated. The question of surgical treatment is decided individually in each case and depends on the form of hermaphroditism. Plastic surgery is also used to correct the external genitalia, hormone therapy.

Violation of sexual development in girls

The process of sexual development (maturation) is characterized by the occurrence of multiple quantitative and qualitative changes in the girl's body, preparing her for the reproductive function. There are two phases of puberty: the first is prepubertal, characterized by a "jump" in growth, the appearance of secondary sexual characteristics, the further development of the genital organs and ending with the appearance of the first menstruation. The second phase begins with the period of menarche and ends with the completion of sexual and somatic development. The physiological process of sexual development proceeds in a certain genetically encoded sequence, which is mainly facilitated by the neuroendocrine system, especially sex steroid hormones.

Violations of sexual development are not the same in the nature of the pathological process, the clinical picture, the time of occurrence and manifestation of symptoms. Distinguish the following violations of sexual development in girls:

premature sexual development;

delayed sexual development;

disorders of sexual development at puberty;

lack of sexual development (gonadal dysgenesis, ovarian aplasia).

Etiology. An important role in the occurrence of violations of sexual development is played by pathological processes that operate in the extrauterine period, during childbirth and in the neonatal period (preeclampsia, the threat of abortion, anomalies of birth forces, fetal asphyxia, trauma to the central nervous system, prematurity, fetal hypotrophy, infectious diseases and maternal alcoholism, etc.). In a special place are infectious diseases suffered in childhood and puberty. Rare causes of developmental anomalies are neoplasms and cysts of the hypothalamic-pituitary system, ovaries, adrenal glands. The nature of the violations can be organic or functional.

The definition of "hermaphroditism" means "bisexuality", the specificity lies in the presence of signs of both sexes in one organism. In the animal world, the phenomenon of hermaphroditism is considered one of the natural processes of reproduction. In botany, in plants, among which the predominant part is hermaphrodite, the definition of "dioecious" is used, when both male and female are present on the same plant. female flowers. The ability to self-fertilization is common in a significant part of worms, some species of fish. Mollusks, algae, fungi, crustaceans are just a few representatives of natural hermaphroditism on the planet.

What is hermaphroditism in humans

Human hermaphroditism is referred to as an abnormal phenomenon, since it is a defect in the development of the sexual sphere at the genetic and hormonal levels, in which there are signs of both sexes in his body (who are hermaphrodites, see the photo of human organs above).

Often this disease is congenital. Less often, deviations towards the opposite affiliation are detected at the stage of premature sexual development. In adulthood, hermaphroditism can occur as a result of hormonal therapy during sex change.

There is no statistics on the number of people with such deviations due to the reluctance to advertise the diagnosis. Hermaphroditism is always accompanied comorbidities, developmental anomalies not only of the genital organs. Often, violations are diagnosed in parallel:

of cardio-vascular system;
skeletal anomalies.

Accompanied by pronounced, to varying degrees:

mental and mental disorders;
violations in the sexual and psycho-emotional sphere;
problems with social adaptation.

Causes of hermaphroditism

The root cause of the defectiveness characteristic of hermaphroditism is the influence hereditary factors. The second place belongs to the damaging factors from the outside. Hereditary include pathology in the number and quality of sex chromosomes, genetic mutations on the non-sex chromosomes.

To factors external influence, on the developing fetus, include:

radioactive radiation;
the use of toxic substances;
alcohol consumption;
reception hormonal drugs showing .

The influence of these factors is most dangerous at the beginning of the third trimester of pregnancy.

Also, female or male hermaphroditism, while still in the body of a pregnant woman, can be triggered by the following factors:

hormonal disorders in the body of a pregnant woman, occurring with diseases of the hypothalamus and pituitary gland, which are responsible for the production of sex hormones;
excessive content in the fetus of hormones of the opposite sex;
intrauterine infection of the fetus;
tumors and diseases of the adrenal glands and ovaries.

Symptoms of hermaphroditism

True hermaphroditism
male pattern hair growth in women
Hermaphroditism in congenital deficiency of 21-hydroxylase

The following is a symptomatology that is common for all types of this pathology (what hermaphroditism looks like in humans, see the photo above):

early puberty;
defect in the formation of the genital organs:
- the penis is underdeveloped, deformed:
- different options for placing the urethra in the perineum, apart from the head of the penis:
  - there are no testicles in the scrotum, they are located either in the abdominal cavity, or in the folds of the skin, copying the labia majora, or in the inguinal canal;
  - hypertrophy, up to the size of the penis, clitoris;
immature mammary glands in individuals with a female set of chromosomes and breast development in individuals with a male set;
body, body hair, larynx standards that do not correspond to the passport gender;
failure of sexual life;
inability to have offspring.

Classification

There are true and false hermaphroditism:

True hermaphroditism the phenomenon is purely episodic. Manifested in education human body glands of both sexes (testis and ovary).

The chromosome component, with true hermaphroditism, is more often female, less often male. There are cases of mosaicism in chromosomes.

The glands are able to develop independently, or, in the case of a mutational fusion, form into a single gland (ovotestis), partially containing the tissues of both glands. A subspecies of true hermaphroditism is distinguished - cross. Manifested by the formation of human body, at the same time, on the one hand, the male (testicle) gland, and on the other, the female (ovary).

In 10% it is possible to identify gender. In 90% of cases, the structure of the genital organs is combined. It comes in a variety of variations:

female characteristics prevail:
- there is a vagina;
- the clitoris is greatly enlarged;
predominant male characteristics:
- there is a small size of the penis, scrotum;
- unicornuate uterus and vagina;
enlarged to the size of the penis, the clitoris, vagina and urethra are placed apart;
urethra goes into vagina, penis small size, sometimes there is a prostate gland;
there are variants with normally formed genitals, but with the presence of a gonad of the opposite sex.

The symptomatology of true hermaphroditism is variable and is determined by the activity of the gonadal tissue. A quarter of patients with this pathology ovulate, and in some cases spermatogenesis is preserved. Individuals close in the structure of the genitals to the female type have menstruation, to the male type - an admixture of blood in the urine during the menstrual period.

Patients are more likely to have difficulties with self-identification and adaptation in society.

False hermaphroditism subdivided into male and female.

The frequency of episodes is higher than that of the true. It is determined by the formation in a person of the gonads opposite, by gender, to the genitals that have formed in him.

Female false hermaphroditism

With this type of pathology, in the body, the ovaries are determined, but the genitals, which are characterized by masculinizing signs:

the clitoris is significantly enlarged, there is a possibility of growth of the labia majora;
mammary glands not developed;
there is hair growth on the face, chest;
low timbre of voice;
the skeleton and muscles are typical for men;
mental self-perception corresponds to the male.

Male false hermaphroditism.

External organs are visually similar to female ones. The testicles are not descended into the scrotum, their probable location is the abdominal cavity. Develop slowly, pathologically. The penis is underdeveloped, curved. The opening of the urethra is strongly displaced, which makes it difficult to urinate. The shape of the mammary glands is enlarged, reminiscent of women. Structure vocal cords, hair growth characterizes the female type. The structure of the figure and the mental self-perception of women.

Diagnostics

Immediately, after the birth of a child with a set of bisexual external genital organs, measures are taken to establish gender: karyotyping - sex determination by chromosomes and ultrasound of the small pelvis, to establish the correspondence of the gonads. After that, the civil gender is officially registered in the documents.

According to indications, on the 3-4th day of life, a blood test is taken from a newborn to identify genetic abnormalities, including.

To confirm the diagnosis, patients are examined by a geneticist, endocrinologist, gynecologist, urologist.

Older patients, with pathology in development, begin the examination:

from a general inspection;
collecting an anamnesis of life, complaints;
the condition of the genital organs is assessed, as well as the presence and degree of development of secondary sexual characteristics (hair and physique, development of muscles and mammary glands);
draws attention to general state health, as Great chance concomitant diseases;
a vaginal examination and palpation of the scrotum are performed.

Also held:

karyotyping is one of the methods early diagnosis pathologies, even in the period of intrauterine development of the fetus. Studying the composition and quality of chromosomes. Determines the gender of the fetus;
sex chromatin analysis;
ultrasound examination of the pelvic organs, adrenal glands and abdominal cavity. Allows you to determine the state of internal organs, shortcomings in their development, the presence of sex glands;
magnetic resonance imaging (MRI) reports high-precision visual information about the state and location of internal organs;
Laboratory tests of blood and urine. Conducted for the purpose determining the level of hormonal levels. The affiliation of the tests is specified by the attending physician. Usually this:
- testosterone;
- luteinizing and follicle-stimulating hormones;
- estradiol;
- glucocorticoids;
endoscopic examination internal genital organs;
diagnostic laparotomy. It is carried out in order to take gonadal tissue for histological analysis.

Treatment of hermaphroditism

The main goal is the choice by the patient or his legal representatives civil sex, on the basis of which complex treatment: surgical reconstruction and replacement therapy hormonal drugs.

Drug treatment with sex hormones lasts the entire reproductive period of life:

estradiol dipropionate is used to enhance feminization (" Proginova"), microfollin; combined oral contraceptives - Mercilon, Logest, Novinet, Yarina, Janine other;
means for hormone replacement therapy for disorders that have arisen after the onset of menopause ( Klimodien, Femoston);
persons whose disease is caused by a congenital disorder in the production of steroids in the adrenal cortex are prescribed synthetic analogues Glucocorticoid and mineralocorticoid hormones.
for masculinization - Testosterone propionate, Testenat, Sustanon-250, Omnadren.
to stimulate the growth of the patient, people with Turner syndrome are prescribed drugs growth hormone ("Norditropin" or analogues);

Surgery

One of critical factors in the treatment of hermaphroditism, is to carry out surgical measures as early as possible, preferably before the age of two, before the end of the period of sexual consciousness. As a last resort, before the onset of puberty, in order to bring up a full-fledged body corresponding to the established sex.

In all cases of female false hermaphroditism, it is necessary to choose the female version of education.

Successful surgical (feminizing) plastic surgery and hormonal therapy give every chance for successful adaptation to female identity. If there are two rudimentary testicles in the body, their removal is indicated, feminizing plastic surgery and education according to the female type, with the support of hormonal therapy.

The issue of false hermaphroditism is more difficult to resolve. The case when at least one testicle functions in the body and the structure of the genitals is similar to that of men makes it possible to educate the patient according to male gender. Given that plastic correction and hormone therapy.

In older patients, sex correction is carried out taking into account the psychosexual direction and the anatomical and functionality sexual sphere.

If the penis is poorly developed, then they resort to its removal. The testicles are also removed, as there is a possibility of their malignant degeneration. With a successful operation and complex hormone therapy, the chance for the formation female body high enough.

With an average size of the penis, plastic correction is performed:

straightening the penis;
the output of the urethra into it;
the scrotum is formed from the tissue of one's own body;
drop testicles into it;
remove, if present, female organs.

For women, a hypertrophied clitoris is removed, and plastic surgery of the labia is performed. A vagina is formed from the tissue of the peritoneum. All organs containing testicular tissue are removed. Assign glucocorticoids, adrenal hormones that inhibit the production of androgens and estrogens, to form female characteristics.

Patients after surgery are observed by an endocrinologist. When indicated, consultations of a psychotherapist, sexologist or psychologist are necessary.

Possible Complications

Inability to bear children.
Unconventional sexual behavior:
- Transvestism, homosexuality, transsexualism, bisexuality.
Disadaptation in society.
Pathology of urination with the wrong location of the urethra.
Malignant tumors of the testicles and ovaries.

Unfortunately, it is not possible to completely cure hermaphroditism, but with adequate correction, the prognosis for life is favorable. An integral part of therapy is psychological and psychotherapeutic assistance to the patient. Constant monitoring by an endocrinologist. Malignancy, that is, degeneration into a malignant, abnormally developed gonads, can worsen the prognosis.

True hermaphroditism

The syndrome of bisexual gonads is the presence in an individual of functional ovaries and testicles at the same time. This is called bisexuality. In true hermaphroditism, the glands produce both male and female sex hormones in equal amounts.

Note! 25% of hermaphrodites are characterized by ovulation with menstruation and sperm production at the same time.

The ovaries and testicles in bisexual syndrome can be localized separately, or they can form a single gonad: it is called ovotestis. Individuals with the pathology of bisexuality can look like anything:

secondary gender signs of masculinity are pronounced;
secondary gender signs of femininity are pronounced;
mixed bisexual type - a combination of gender characteristics;
transsexuals - a combination of male genital organs with female glands.

Causes of deviations in sexual development of this type not yet determined by science. According to statistics, 60% of the female karyotype and 10% of the male karyotype prevail among hermaphrodites, 30% belong to the mixed type.

At birth, almost all babies with bisexual gonadal syndrome have a mixed genitalia. Approximately 10% of infants revealed differentiated sexual characteristics. Other anomalies in the structure of body organs in hermaphrodites are not observed.

Note! With this disease, self-identification is impaired. The patient may show an addiction to transsexualism, homosexuality, transvestism, or bisexuality.

False hermaphroditism

However, more often you can find hermaphroditism of a false type. This disease is defined as follows: a person has an anatomically expressed affiliation of one gender type, but his gonads (sex glands) correspond to another type. The disease affects both sexes.

Symptoms:

anatomical pathology of the reproductive organs;
developed mammary glands in men;
mustache and beard in women;
the presence of a male penis in women;
the presence of testicles in a woman in the labia;
inconsistency of physique with gender type;
change in the sound of the voice;
inability to conceive;
delayed puberty.

In men outward manifestation deviations can serve as underdevelopment or curvature of the penis, the absence of testicles in the scrotum (they are located inside the peritoneum).

Causes of pathology

The deviation can be both congenital and acquired. Congenital depends on the characteristics of the development of the fetus during the formation of the pelvic organs. Acquired hermaphroditism is formed in the pathology of the pituitary gland, the adrenal cortex - organs that produce hormones.

Congenital malformation is determined in the third week of fetal life, when it begins to form reproductive system child. The genitalia of boys and girls are formed from the paramesonephric and mesonephric ducts. If a failure occurs at this stage, male and female genitalia begin to appear in the fetus. This pathology is caused by a change in the composition of chromosomes (karyotype) or a change in the genome (as part of chromosomes).

Also cause improper development the reproductive system of the fetus can become a mutation due to irradiation of the embryo or poisoning with poisons (alcohol, drugs, illegal drugs taken by the mother). A significant contribution to the mutation of the genome of the embryo is also made by infection of the mother with Toxoplasma or other viruses.

Another reason pathological development reproductive system fetus is hormonal disorders mother or embryo. This leads to overproduction of male sex hormones in girls and female sex hormones in boys. Violation of the pituitary gland, responsible for the production of hormones, provokes a pathology of the structure of the reproductive organs of the fetus.

The abnormal structure of the reproductive system of the embryo can be facilitated by the localization of the tumor in the mother, due to which the production of androgens becomes uncontrolled, or by taking hormonal drugs during pregnancy. congenital pathology the adrenal cortex in the fetus also disrupts the process of hormone production.

False female hermaphroditism

The female half of the population is less susceptible to the disease than the male. False hermaphroditism is considered a pathology (disease) in which the body produces excess amount androgens. As a result, a woman's internal reproductive organs develop according to sex (ovaries), and the appearance resembles male reproductive organs.

In addition to pathology, many women have a pronounced body structure according to the masculine type:

developed muscles;
abundant hairline;
low timbre of voice;
behavioral features.

Often women with this deviation perceive themselves as men and behave in accordance with this perception.

False male hermaphroditism

With this pathology, the individual has well-developed masculine glands (testicles) with weakly expressed external sexual characteristics - they are more reminiscent of the constitution of women. The shape of the body in this disease resembles the female type:

hairline is poorly developed;
the timbre of the voice is high;
muscles are weakly expressed;
type of female behavior.

A man with this deviation identifies himself as a woman.

The visual severity of the anomaly depends on the degree of manifestation of the chromosome mutation - from a slight increase in the clitoris to its transformation into a male penis. There are cases of erroneous determination of the sex of a newborn due to the abnormal structure of the reproductive system. Later it turned out that the boy had female ovaries inside. Often this type of men identify themselves as homosexuals (passive).

There is another form of pathology: a man has external reproductive organs of the feminine type and masculine sex glands (gonads). This is facilitated by abnormal development of the urinary tubule or cryptorchidism (testicles in the abdominal cavity).

A man may have a feminine appearance, however, he will lack internal female reproductive organs. At the same time, the patient has pronounced secondary feminine sexual characteristics - the absence of masculine-type hair growth and the presence of developed mammary glands. The degree of feminization of a man depends on the level of production of female hormones.

Doctors distinguish three degrees of feminization of a man:

the patient has a feminine body type;
the appearance of the patient is fully consistent with the masculine type;
type of eunuch - a high timbre of voice with undeveloped mammary glands and the absence of hair on the chest and legs.

On the issue of the treatment of hermaphroditism, they turn not only in case of the appearance of diseases (tumors of the testicles in the peritoneum and the inability to have a normal sexual intercourse), but also to achieve a sense of inner harmony. Most patients experience discomfort from inconsistency inner world appearance.

In the treatment of the disease, several methods are used:

surgical operation;
surgical correction - with minor deviations from the norm.

In addition to surgery, the patient is prescribed a course of hormone therapy and drugs to stimulate the pituitary gland. In any case, the methods of treatment depend on individual characteristics the course of the disease. Methods of psychotherapeutic influence play an important role in overcoming the social isolation of the patient.

Diagnostics

To identify the cause of an intrauterine anomaly in the development of the child's reproductive system, the doctor asks the woman in detail about the course of pregnancy and collects data:

were there any complications in the course of pregnancy;
whether the woman took medicines dangerous for the fetus;
what infectious diseases the woman suffered during the period of gestation;
how did the anomaly of the structure of the genital organs in the newborn appear after birth;
characteristics of a child's puberty.

In an adult with a disease of hermaphroditism, the features of sexual behavior are ascertained:

is there a libido;
are there any problems with intimate contacts;
whether infertility has been identified.

Next, the doctor conducts a visual examination - the development of muscles, hair growth, the structure of the external reproductive organs, the degree of swelling of the mammary glands. The doctor also writes down in the medical history indicators of growth, body weight, blood pressure patient and examines the skin.

To laboratory diagnostics includes the study of chromosomes - informants of heredity. This is necessary to establish the genetic sex of the patient. Also, the patient's blood and urine are subject to study for the presence of hormones of one type or another.

Important! The diagnosis of true hermaphroditism is confirmed by diagnostic laparotomy.

Men are examined by a urologist. At the reception, the physician records data on the condition of the external and internal reproductive organs. The scrotum, prostate (palpation) is subject to research.

On ultrasound diagnostics, the patient is examined the small pelvis and abdominal cavity. On the diagnosis, you can see the localization of the ovaries in a man, notice undescended testicles, determine the pathology internal systems. This is all necessary to collect data on the causes of the disease. However, the most detailed picture of internal pathology can be obtained by MRI and laparoscopy.

Therapy

Is it possible to treat false hermaphroditism? Patients are offered hormone therapy, since the main cause of the pathology is hormonal disorders. After receiving the results of laboratory and hardware diagnostics, therapy is prescribed. In accordance with the violation of the production of hormones by a certain gland, medicines are prescribed for:

correction of sex hormones;
thyroid function;
functionality of the adrenal glands;
pituitary correction.

To correct the abnormal shape of the external reproductive organs, surgery is used - penis straightening/enlargement, correction of the anatomical pathology of the female reproductive organs. Men can undergo surgery to lower the testicles into the scrotum, correct the anomaly of the urinary canal, and form the missing scrotum from the biomaterial of the body.

If the testicles of a man have been subjected to a malignant change, they are removed. Also, a course of mental correction is carried out with patients to normalize the perception of sexual behavior corresponding to the gender type.

True hermaphroditism is treated by eliminating the gonads of one type - muscular or feminine. Before the operation, the psychosexual orientation of the patient is established. Next, plastic surgery of the external reproductive organs is performed. To maintain the hormonal status, the individual takes drugs during the reproductive period of life: women take estrogen-progestin drugs, men - androgens. Apart from drug therapy, patients must be constantly under the supervision of a psychologist and a sexologist - for successful socialization.

Complications

If a patient with hermaphroditism does not agree to treatment, what can such a decision turn into? The consequences of this pathology:

inability to conceive a child;
impossibility of normal sexual contact;
perversions and pathologies of sexual behavior;
antisocial behavior, difficulty of socialization;
tumors of the testicles located in the peritoneum;
the impossibility of normal urination with an abnormal structure or location of the urinary canal.

Outcome

Modern medicine has necessary equipment and methods of correction of hermaphroditism. Most often with false form This disease is treated when it is impossible to have a normal sexual intercourse or to conceive a child. Another reason to visit a doctor is pain in the peritoneum due to the development of a tumor of the testicles located inside the body. However, the disease may be accompanied by severe mental or mental underdevelopment, perverted sexual behavior or increased libido. Timely appeal to specialists (urologist, psychologist) will help to identify pathology and restore reproductive function.

Hermaphroditism in humans: causes, symptoms, treatment. False hermaphroditism - an anomaly of gonadal dysgenesis

False female hermaphroditism

External signs of hermaphroditism in women

Male pseudohermaphroditism

Diagnostics

Treatment

The principle of hormone therapy

Surgical intervention

Complications

Varieties of violations of differentiation by sex

Manifestations of various types of hermaphroditism

True hermaphroditism

False female hermaphroditism

Ways to establish the true gender

Opportunities to eliminate gender mismatch

Violation of sexual development in girls

What is hermaphroditism in humans

Causes of hermaphroditism

Symptoms of hermaphroditism

Classification

Female false hermaphroditism

Male false hermaphroditism.

Diagnostics

Treatment of hermaphroditism

Surgery

Possible Complications

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