Anatomy of a hermaphrodite. A true hermaphrodite. Above is a diagram of the structure of the dual reproductive system of a hermaphrodite; below - the similarity of the reproductive systems of men and women

In rare cases, children are born with unclearly differentiated primary sexual characteristics. In such a situation, a sensitive medical approach is extremely important, since gender plays a key role in the formation of a sense of self-awareness. Sexual differentiation is the process of physical changes in the embryo, as a result of which it acquires male or female primary sexual characteristics. Usually this process develops in one direction.

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Photo gallery: Hermaphrodite: structure of reproductive organs

However, sometimes sexual differentiation can be disrupted, and at birth it can be difficult to determine the sex of the child. Hermaphrodite, the structure of the reproductive organs - what could happen to the child?

Basic terms

The term hermaphrodite is used to describe an individual who has both female and male characteristics. This is the norm in some animal species. In humans, this pathology refers to disorders of sexual differentiation.

Hermaphroditism

The word “hermaphrodite” comes from the name of the god, the son of Hermes and Aphrodite, who, according to ancient Greek mythology, was united in one body with a female nymph. In medicine, this term is used to refer to certain types of intersex conditions:

True hermaphroditism

It is characterized by the development of both male and female reproductive organs in the fetus. Such an individual has both male and female gonads, and in some cases, a combined gonad (ovotestis), which has tissue elements of the ovaries and testes. True hermaphroditism is very rare. Only about 400 such cases are described in the world medical literature. These patients showed significant variability in the structure of the internal and external genitalia. Most hermaphrodite newborns, however, had more pronounced male characteristics.

Hermaphrodites

Pseudohermaphroditism

It is characterized by the presence of mixed external genitalia, while the gonads are formed correctly according to the male or female type.

Pseudohermabooditism

Pseudohermaphroditism is a group of pathological conditions in which an individual has external signs of both sexes, and the gonads are formed according to the male or female type. Such people have either testes or ovaries, and not both. Pseudohermaphroditism is a fairly rare disease, but it is much more common than true hermaphroditism. There are two main types of pseudohermaphroditism: male and female. In female hermaphroditism, the labia and clitoris are enlarged and resemble the scrotum and penis.

Causes

The sexual duality of male pseudohermaphrodites can be due to several reasons, for example, a violation of the formation of the testes during embryonic development (gonadal dysgenesis), insensitivity of body tissues to testosterone, and deficiency of enzymes that ensure the tissue response to testosterone.

Female pseudohermaphroditism

Female pseudohermaphrodites have a female chromosome set (46, XX), but their external genitalia are formed partially or completely according to the male type. They may have a hypertrophied clitoris that resembles a penis. The vaginal opening is often closed. A masculine appearance is usually caused by excess production of male hormones by the adrenal glands during embryonic development.

Enzyme deficiency

The cause of this unusual disease may be a disruption in the production of enzymes by the adrenal glands, most often 21-hydroxylase. This enzyme is necessary for the production of two important adrenal hormones - cortisol and aldosterone. With 21-hydroxylase deficiency, these hormones are produced in insufficient quantities. The body reacts to hormonal imbalance by increasing the production of adrenocorticotropic hormone, which in turn stimulates the function of the adrenal glands.

Male sex hormones

When the level of adrenocorticotropic hormone increases, the adrenal glands are activated, producing, among other things, male sex hormones - androgens. Under their influence, male characteristics appear in the female body. Children with such abnormalities require replacement therapy with cortisol and aldosterone analogues throughout their lives. However, it is impossible to replace the missing enzyme. If a child is sexually ambiguous, a decision must be made as to whether the child will be male or female. In this case, it is necessary to take into account the interests of the child. Gender duality occurs and is obvious already at birth. Therefore, it is extremely important to take emergency measures. Insensitive handling of a hermaphrodite child can lead to serious problems for both him and his parents. Appropriate treatment is usually prescribed immediately after the baby is born.

Boy or girl?

All mothers and fathers want to know what gender their newborn is. When doubts arise about this, a quick answer should not be given. However, it can be difficult for medical professionals not to answer the main question of parents - they are forced to say whether it is a boy or a girl.

Delaying decisions

Another difficulty lies in the fact that a decision about gender, made immediately after birth, is very difficult to change later. From the very beginning, parents and relatives treat boys and girls differently. This difference is immediately apparent - for example, newborn boys are dressed in blue clothes, and girls are dressed in pink. Moreover, a child’s awareness of his gender is formed before about one and a half years. Therefore, in some cases it is better to postpone the gender announcement than to make a hasty and incorrect decision.

Psychological consequences

Doctors and patients must think about the future and consider that a person's critical attitude towards his appearance - and especially his gender - can lead to serious psychological consequences.

Childhood

Hermaphrodite children may realize that they are different from others when they begin to interact with their peers at school. They may also notice parents' concerns about their gender ambivalence.

Puberty

Puberty is an important developmental stage for every person. Problems at this age related to gender identity or appearance are common knowledge and can be very traumatic. In hermaphrodites, puberty can be even more difficult. Some of them may experience delayed puberty. Others may be concerned about noticeable changes in the body, such as facial hair growth and enlargement of the clitoris in a girl, or breast development in a boy.

Setting the floor

Before deciding on the sex of a hermaphrodite, the doctor should discuss in detail with the parents what is best for the child. The correct tactics of action in relation to a hermaphrodite child involve refraining from hasty statements about his gender.

Consilium

For each patient, a consultation is held with the participation of pediatricians, as well as specialized specialists. Based on genetic studies and ultrasound results, the most appropriate gender for the child is determined. Ultrasound allows you to visualize internal organs, such as the uterus or testicles that have not reached their normal position. Parents often experience a variety of emotions: shock, helplessness, irritation or fear. This difficult situation requires thorough discussion and conversations with parents.

Decision-making

Doctors sometimes need more than one day to decide which gender is most appropriate for the child. At the same time, given the concerns of parents, this should take as little time as possible. Before a final determination is made, a birth certificate cannot be issued. Until a final determination is made, all persons concerned (including friends and relatives) in relation to the child should refrain from using gender-specific words such as “he” or “she”.

Parental Involvement

The final decision regarding the sex of the child is made jointly with the parents. This takes into account the chromosome set, heredity, enzyme activity, as well as the anatomy and physiology of the body. It can be difficult to convey this information to relatives. However, the task of professionals involved in solving this problem is to present the key points as clearly as possible, while at the same time showing empathy and compassion. The final decision should be the choice of gender with which the child can safely live his entire life. More often, experts recommend raising a child as a girl. This is explained by the fact that it is easier for female hermaphrodites to correct the external signs of a male gender using plastic surgery. As a rule, in the future they successfully fit into society as women. A completely different thing is a feminized boy, whose appearance is difficult to change enough to give him masculine characteristics. Therefore, it is preferable to raise such a child as a girl. In the future, he will look like an ordinary girl, and later like a woman (however, she will not be able to have children). In accordance with the law, the child's birth certificate will indicate female gender. If this decision is made, all testicular tissue is removed. Firstly, because testosterone can be produced in the testicles, which can lead to the appearance of some male characteristics (for example, facial hair growth). Secondly, tumor changes can be observed in testicular tissue at a later age. If you decide to raise a hermaphrodite child as a boy and he has a very small penis (micropenis), it is necessary to begin treatment as soon as possible. Thus, it is possible to ensure that as he grows older, he will develop a masculine appearance close to the norm.

Looking ahead

When making any decision, it is necessary to think about the future, assuming what kind of life the child will lead in the future and how he will adapt in society.

A hermaphrodite is a person whose body simultaneously contains male and female sexual characteristics. Therefore, it is impossible to say unambiguously what gender the hermaphrodite is. Many experts classify such patients as middle-sex. A hermaphrodite is a person who cannot have children. Quite often, hermaphrodite girls have a mixture of tissue from the ovaries and testes instead of ovaries. This hybrid tissue is unable to synthesize any hormones because it does not contain follicles or eggs.

Hermaphrodite is a rather rare phenomenon, since this pathology occurs on average in one out of 2 thousand newborns. There are false and true hermaphroditism.

Etiology of the disease

In reality, there are many reasons that provoke hermaphroditism:

Pseudohermaphroditism in women

A false female hermaphrodite is a woman but with male genitalia. As a rule, this pathology develops against the background of certain diseases (arrhenoblastoma, adrosteroma, adrenal hyperplasia). In some cases, the etiology of the disease remains unknown. Hermaphrodite girls have a female set of sex chromosomes (46, XX) and a mixed structure of the external genitalia. In most patients with false female hermaphroditism, the ovaries are formed correctly. In addition, they are able to function effectively throughout life.

Pseudohermaphroditism in men

A false male hermaphrodite is a man, but with female sexual characteristics. This anomaly occurs in one in 300-400 newborn babies. A hermaphrodite is a person who has two anomalies. One of them is the incorrect localization of the testes, and the other is the incorrect development of the male urethra. Those who are interested in what hermaphrodites look like can find photos of people suffering from this pathology in specialized literature.

True hermaphroditism

True intersexuality in people is extremely rare (over the entire history of mankind, about 200 cases have been described in specialized literature). This pathology is understood as a condition in which both testicular and ovarian elements are present in one organism. If you are interested in the topic: “Hermaphrodite girls”, photos will be presented below.

Symptoms

Symptoms of hermaphroditism are varied and depend on the form and causes of the disease. The main signs of pathology include the following:

• underdevelopment of the penis;
• anomaly of the external genitalia;
• curvature of the penis;
• cryptorchidism;
• mixed body type;
• development of the mammary glands in a boy;
• hypertrichosis;
• premature puberty;
• absence of menstruation;
• discrepancy between the physique and timbre of the voice and the passport gender;
• difficulties in sexual life;
• infertility.

Diagnostic methods

To diagnose hermaphroditism, doctors perform a number of examinations and tests:

• analysis of anamnestic data (how the mother’s pregnancy proceeded, the state of health of the newborns, are there any anomalies in the structure of the genitals, how did puberty occur, are there any complaints about sex life, infertility, etc.);
• a physical examination is required to identify abnormalities in the development of secondary sexual characteristics (type of hair and body type, development of muscle tissue and mammary glands), possible symptoms of pathology of internal organs (visual examination of the skin, changes in blood pressure, weight, height, circumference);
• magnetic resonance or computed tomography;
• laparoscopy;
• research on intellectual development;
• examination by an endocrinologist;
• biopsy of the gonads;
• genetic analysis;
• urethroscopy (vaginoscopy);
• gynecography;
• radiography;
• karyotyping (study of mutations in chromosomal composition);
• examination by a urologist (a specialist examines the external genitalia for developmental anomalies, palpates the scrotum area, examines the condition of the prostate);
• ultrasound examination of the adrenal glands and abdominal organs;
• biochemical blood test (determining the concentration of 17-ketosteroids and gonadotropin).

Therapy methods

Treatment of the above pathology is strictly individual. Depending on the etiology of the disease, hormonal therapy is prescribed:

• steroid hormones;
• glucocorticoids (hormones of the adrenal cortex);
• thyroid-stimulating hormones;
• hormonal drugs that affect the regulatory function of the adenohypophysis.

Correction of abnormalities of the genital organs is carried out surgically. Men undergo plastic correction of the external genitalia according to the male type (plastic surgeons shape the scrotum, lower the testes into the scrotum, enlarge and straighten the penis, perform urethroplasty, remove underdeveloped testes, since they very often undergo malignant degeneration).

Consequences and complications

The main complications of the disease include the following:

• infertility;
• lack of sex life;
• social disorientation;
• malignant neoplasms in the testes;
• pathology of urination due to incorrect location of the urethra;
• perverted sexual behavior (homosexuality, bisexuality, transvestism, transsexualism).

A hermaphrodite is a person whose body simultaneously contains male and female sexual characteristics. Therefore, it is impossible to say unambiguously what gender the hermaphrodite is. Many experts classify such patients as middle-sex. A hermaphrodite is a person who cannot have children. Quite often, hermaphrodite girls have a mixture of tissue from the ovaries and testes instead of ovaries. This hybrid tissue is unable to synthesize any hormones because it does not contain follicles or eggs.

Hermaphrodite is a rather rare phenomenon, since this pathology occurs on average in one out of 2 thousand newborns. There are false and true hermaphroditism.

Etiology of the disease

In reality, there are many reasons that provoke hermaphroditism:

Pseudohermaphroditism in women

A false female hermaphrodite is a woman but with male genitalia. As a rule, this pathology develops against the background of certain diseases (arrhenoblastoma, adrosteroma, adrenal hyperplasia). In some cases, the etiology of the disease remains unknown. Hermaphrodite girls have a female set of sex chromosomes (46, XX) and a mixed structure of the external genitalia. In most patients with false female hermaphroditism, the ovaries are formed correctly. In addition, they are able to function effectively throughout life.

Pseudohermaphroditism in men

A false male hermaphrodite is a man, but with female sexual characteristics. This anomaly occurs in one in 300-400 newborn babies. A hermaphrodite is a person who has two anomalies. One of them is the incorrect localization of the testes, and the other is the incorrect development of the male urethra. Those who are interested in what hermaphrodites look like can find photos of people suffering from this pathology in specialized literature.

True hermaphroditism

True intersexuality in people is extremely rare (over the entire history of mankind, about 200 cases have been described in specialized literature). This pathology is understood as a condition in which both testicular and ovarian elements are present in one organism. If you are interested in the topic: “Hermaphrodite girls”, photos will be presented below.

Symptoms

Symptoms of hermaphroditism are varied and depend on the form and causes of the disease. The main signs of pathology include the following:

• underdevelopment of the penis;
• anomaly of the external genitalia;
• curvature of the penis;
• cryptorchidism;
• mixed body type;
• development of the mammary glands in a boy;
• hypertrichosis;
• premature puberty;
• absence of menstruation;
• discrepancy between the physique and timbre of the voice and the passport gender;
• difficulties in sexual life;
• infertility.

Diagnostic methods

To diagnose hermaphroditism, doctors perform a number of examinations and tests:

• analysis of anamnestic data (how the mother’s pregnancy proceeded, the state of health of the newborns, are there any anomalies in the structure of the genitals, how did puberty occur, are there any complaints about sex life, infertility, etc.);
• a physical examination is required to identify abnormalities in the development of secondary sexual characteristics (type of hair and body type, development of muscle tissue and mammary glands), possible symptoms of pathology of internal organs (visual examination of the skin, changes in blood pressure, weight, height, circumference);
• magnetic resonance or computed tomography;
• laparoscopy;
• research on intellectual development;
• examination by an endocrinologist;
• biopsy of the gonads;
• genetic analysis;
• urethroscopy (vaginoscopy);
• gynecography;
• radiography;
• karyotyping (study of mutations in chromosomal composition);
• examination by a urologist (a specialist examines the external genitalia for developmental anomalies, palpates the scrotum area, examines the condition of the prostate);
• ultrasound examination of the adrenal glands and abdominal organs;
• biochemical blood test (determining the concentration of 17-ketosteroids and gonadotropin).

Therapy methods

Treatment of the above pathology is strictly individual. Depending on the etiology of the disease, hormonal therapy is prescribed:

• steroid hormones;
• glucocorticoids (hormones of the adrenal cortex);
• thyroid-stimulating hormones;
• hormonal drugs that affect the regulatory function of the adenohypophysis.

Correction of abnormalities of the genital organs is carried out surgically. Men undergo plastic correction of the external genitalia according to the male type (plastic surgeons shape the scrotum, lower the testes into the scrotum, enlarge and straighten the penis, perform urethroplasty, remove underdeveloped testes, since they very often undergo malignant degeneration).

Consequences and complications

The main complications of the disease include the following:

• infertility;
• lack of sex life;
• social disorientation;
• malignant neoplasms in the testes;
• pathology of urination due to incorrect location of the urethra;
• perverted sexual behavior (homosexuality, bisexuality, transvestism, transsexualism).

The concept of “hermaphroditism syndrome” refers to a group of disorders of sexual differentiation that accompany many congenital diseases and are manifested by quite diverse symptoms. Patients suffering from this pathology have characteristics of both men and women.

Below we will talk about why hermaphroditism occurs, what clinical manifestations it can be accompanied by, and also introduce the reader to the principles of diagnosis and treatment of this pathology.

False hermaphroditism is distinguished when the structure of the genitals does not correspond to the gender of the gonads (gonads). In this case, the genetic sex is determined by the affiliation of the gonads and is called pseudohermaphroditism, male or female, respectively. If a person has elements of both the testicle and ovary at the same time, this condition is called true hermaphroditism.

In the structure of urological and gynecological pathology, hermaphroditism is recorded in 2-6% of patients. There are no official statistics regarding this pathology today, but it is unofficially believed that hermaphroditism occurs more often than doctors register it. Such patients are often hidden under other diagnoses (“gonadal dysgenesis”, “adrenogenital syndrome” and others), and also receive therapy in psychiatric departments, since their sexual disorders are incorrectly assessed by doctors as diseases of the sexual centers of the brain.

Classification

Depending on the mechanism of development of hermaphroditism, there are 2 main forms of it: impaired differentiation of the genitals (genital organs) and impaired differentiation of the sex glands, or gonads.

There are 2 types of genital differentiation disorders:

1. Female hermaphroditism (partial appearance of male sexual characteristics, the set of chromosomes is 46 XX):
   • congenital dysfunction of the adrenal cortex;
   • intrauterine virilization of the fetus under the influence of external factors (if the mother suffers from any tumor that produces male sex hormones - androgens, or takes medications that have androgenic activity).
2. Male hermaphroditism (inadequate formation of male sexual characteristics; the karyotype looks like this: 46 XY):
   • testicular feminization syndrome (tissues are sharply insensitive to androgens, which is why, despite the male genotype, and therefore the person’s belonging to this sex, he looks like a woman);
   • deficiency of the enzyme 5-alpha reductase;
   • insufficient testosterone synthesis.

Disorders of differentiation of the gonads are represented by the following forms of pathology:

• bisexual gonad syndrome, or true hermaphroditism (the same person combines both male and female gonads);
• Turner syndrome;
• pure agenesis of the gonads (complete absence of the sex glands in the patient, the genitals are female, underdeveloped, secondary sexual characteristics are not determined);
• dysgenesis (disorder of intrauterine development) of the testicles.

Causes of occurrence and mechanism of development of pathology

Both hereditary factors and factors affecting it from the outside can disrupt the normal development of the fetal genital organs.

The causes of disembryogenesis, as a rule, are:

• mutations of genes in autosomes (non-sex chromosomes);
• pathology in the area of ​​sex chromosomes, both quantitative and qualitative;
• external factors affecting the fetus’s body through its mother at a certain stage of development (the critical period in this situation is 8 weeks): tumors in the mother’s body that produce male sex hormones, her taking medications with androgenic activity, exposure to radioactive radiation, various types of intoxication .

Each of these factors can affect any of the stages of sex formation, as a result of which one or another set of disorders characteristic of hermaphroditism develops.

Symptoms

Let's look at each form of hermaphroditism in more detail.

Female pseudohermaphroditism

This pathology is associated with a defect in the enzyme 21- or 11-hydroxylase. It is inherited in an autosomal recessive manner (that is, it is not related to gender). The set of chromosomes in patients is female – 46 XX, the gonads are also female (ovaries), and are formed correctly. The external genitalia have characteristics of both male and female. The severity of these disorders depends on the severity of the mutation and varies from mild hypertrophy (increase in size) of the clitoris to the formation of external genitalia, almost similar to male ones.

The disease is also accompanied by severe disturbances in the level of electrolytes in the blood, which are associated with a deficiency of the hormone aldosterone. In addition, the patient may be diagnosed with diarrhea, which is caused by increased blood volume and high sodium levels in the blood, resulting from a deficiency of the 11-hydroxylase enzyme.

Male pseudohermaphroditism

As a rule, it manifests itself as androgen insensitivity syndrome. The pattern of inheritance is X-linked.

Testicular feminization syndrome may develop due to a mutation in the androgen receptor gene. It is accompanied by insensitivity of the tissues of the male body to male sex hormones (androgens) and, on the contrary, good sensitivity to female hormones (estrogens). This pathology is characterized by the following symptoms:

• chromosome set 46 XY, but looks sick like a woman;
• aplasia (absence) of the vagina;
• insufficient hair growth for a man or complete absence of the latter;
• development of mammary glands characteristic of women;
• primary (although the genitals are developed according to the female type, they are absent);
• absence of a uterus.

In patients with this pathology, the male sex glands (testicles) are formed correctly, but are located not in the scrotum (it is missing after all), but in the inguinal canals, the area of ​​the labia majora, and in the abdominal cavity.

Depending on how insensitive the patient’s body tissues are to androgens, complete and incomplete forms of testicular feminization are distinguished. There is a variety of this pathology in which the patient’s external genitalia look almost normal, close in appearance to those of healthy men. This condition is called Reifenstein syndrome.

Also, false male hermaphroditism may be a manifestation of disorders of testosterone synthesis caused by a deficiency of certain enzymes.

Disorders of gonadal differentiation

Pure gonadal agenesis syndrome

This pathology occurs due to point mutations on the X or Y chromosome. Patients are of normal height, their secondary sexual characteristics are underdeveloped, there is sexual infantilism and primary amenorrhea (initially no menstruation).

The external genitalia, as a rule, have the appearance of a woman. In men, they sometimes develop according to the male pattern.

Turner syndrome

It is caused by a genetic mutation - monosomy (full or partial) on the X chromosome. There are also anomalies in the structure of this chromosome or mosaic variants of the mutation.

As a result of this anomaly, the processes of differentiation of the gonads and the function of the ovaries are disrupted. On both sides there is dysgenesis of the gonads, which are represented by striae.

Genes on non-sex chromosomes are also affected. The growth processes of somatic cells and their differentiation are disrupted. Such patients are always short and have many different other anomalies (for example, a short neck, pterygoid folds of the neck, high palate, heart defects, kidney defects, and others).

Testicular dysgenesis

There are 2 forms of it:

• bilateral (two-sided) – the testicles are underdeveloped on both sides and do not produce normal sperm; karyotype – 46 XY, however, abnormalities in the structure of the X chromosome are detected; the internal genital organs are developed according to the female type, the external ones can have characteristics of both male and female; the testicles do not produce testosterone, so the level of sex hormones in the patient’s blood is sharply reduced;
• mixed - the gonads are developed asymmetrically; on the one hand they are represented by a normal testicle with preserved reproductive function, on the other - by a testicle; in adolescence, some patients develop secondary sexual characteristics of the male type; When studying the chromosome set, as a rule, anomalies in the form of mosaicism are revealed.

True hermaphroditism

This pathology is also called bisexual gonad syndrome. This is a rare disease characterized by the presence of structural elements of both the testicle and ovary in the same person. They can be formed separately from each other, but in some cases, patients have so-called ovotestis - tissue of both sex glands in one organ.

The set of chromosomes in true hermaphroditism is usually normal female, but in some cases it is male. Sex chromosome mosaicism also occurs.

The symptoms of this pathology are quite varied and depend on the activity of the testicular or ovarian tissue. The external genitalia are represented by both female and male elements.

Diagnostic principles

The diagnosis process, as in other clinical situations, includes 4 stages:

• collection of complaints, anamnesis (history) of life and current illness;
• objective examination;
• laboratory diagnostics;
• instrumental diagnostics.

Let's look at each of them in more detail.

Complaints and anamnesis

Among other data, in case of suspected hermaphroditism, the following points are of particular importance:

• whether the patient’s immediate family suffers from similar disorders;
• the fact of removal surgery in childhood (this and the previous points will lead the doctor to think about testicular feminization syndrome);
• characteristics and growth rates in childhood and adolescence (if the growth rate in the first years of a child’s life was ahead of that of peers, and at 9-10 years old it stopped or slowed down sharply, the doctor should think about the diagnosis of “dysfunction of the adrenal cortex”, which arose against the background of increased levels of androgens in blood; this pathology may also be suspected in a child with).

Objective examination

The most important point here is to assess the patient's sexual development and body type. In addition to sexual infantilism, the detection of growth disorders and minor anomalies in the development of other organs and systems allows us to make a diagnosis of “Turner syndrome” even before karyotyping.

If, upon palpation of a man’s testicles, they are detected in the inguinal canal or in the thickness of the labia majora, male pseudohermaphroditism can be suspected. The discovery of the absence of a uterus will further convince the doctor of this diagnosis.

Laboratory diagnostics

The most informative method for diagnosing this pathology is karyotyping - a cytogenetic study of chromosomes - their number and structure.

Also, in patients with suspected hermaphroditism, the concentration in the blood of luteinizing and follicle-stimulating hormones, testosterone and estradiol, and, less often, mineralo- and glucocorticoids is determined.

In difficult diagnostic situations, a hCG test is performed.

Instrumental diagnostic methods

To assess the condition of the genital organs, the patient is prescribed an ultrasound of the pelvic organs, and in some cases, computed tomography of this area.

The most informative is an endoscopic examination of the internal genital organs and their biopsy.

Principles of treatment

The main direction of treatment for hermaphroditism is surgical intervention to correct the patient’s gender. The latter chooses his gender, and in accordance with this decision, surgeons reconstruct the external genitalia.

Also, in many clinical situations, such patients are recommended to undergo a bilateral gonadectomy - completely remove the gonads (testes or ovaries).

Female patients, if they have hypogonadism, are prescribed hormonal therapy. It is also indicated for patients whose gonads have been removed. In the latter case, the purpose of taking hormones is to prevent the development of post-castration syndrome (sex hormone deficiency).

So, patients can be prescribed the following drugs:

• estradiol (one of its trade names is Proginova, there are others);
• COCs (combined oral contraceptives) - Mercilon, Logest, Novinet, Yarina, Zhanin and others;
• drugs for hormone replacement therapy for disorders that arise after the onset (climodien, femoston, and so on);
• synthetic analogues of glucocorticoids and mineralocorticoids (depending on which hormone deficiency occurs in a particular patient); they are prescribed for adrenal dysfunction, which results in sexual disorders;
• to stimulate the growth of the patient, people suffering from Turner syndrome are prescribed somatotropic hormone preparations (Norditropin and others);
• testosterone (omnadren, sustanon) – it is recommended to use it for the purpose of hormonal therapy for males.

Patients suffering from hermaphroditism, even after surgery, should be under the supervision of an endocrinologist. Also, many of them are advised to consult a psychotherapist, sexologist or psychologist.

Hermaphroditism translated from Greek means bisexuality. There are two types of hermaphroditism - true and false (pseudohermaphroditism).

Hermaphroditism is caused by congenital anomalies of both the gonads and the external genitalia of a person and is formed in the period before the eighteenth week of embryonic development.

ICD-10 code

Q56.0 Hermaphroditism, not elsewhere classified

Q96 Turner syndrome

Q97 Other sex chromosome abnormalities, female phenotype, not elsewhere classified

Q98 Other sex chromosome abnormalities, male phenotype, not elsewhere classified

Q99 Other chromosomal abnormalities not elsewhere classified

Epidemiology

In the structure of gynecological and urological diseases, hermaphroditism occurs in 2-6% of cases. It is possible that the frequency of hermaphroditism is much higher. There are currently no official geographical statistics for this disease. Patients suffering from true or false hermaphroditism undergo examination and treatment in family planning and reproduction centers, gynecological and urological hospitals and are “hidden” under the diagnoses of “adrenogenital syndrome”, “testicular feminization”, “gonadal dysgenesis”, “ovotestis”, “scrotal -perineal hypospadias with inguinal or “abdominal cryptorchidism.”

Moreover, patients suffering from hermaphroditism are often treated in psychiatric clinics, since the symptoms of true and false hermaphroditism in the form of transsexualism, homosexuality and bisexuality (alternating sex) are mistakenly considered diseases of the “sexual centers” of the brain. Therefore, the problem of diagnosing and treating true and false hermaphroditism is social in nature and is of particular relevance in modern society.

Forms

False male hermaphroditism

False hermaphroditism (pseudohermaphroditism) is a disease in which a person has gonads of one sex, but the external genitalia, as a result of a developmental defect, resemble those of the opposite sex. It is necessary to distinguish between male and female pseudohermaphroditism. A false male hermaphrodite is a man, but with female external genitalia and a female passport. A false female hermaphrodite is a woman, but with male external genitalia and a male passport.

False male hermaphroditism is a condition when a man has abnormalities of the external genitalia. The external genitalia of a man are similar to the external genitalia of a woman. These anomalies have long been known and well studied, as they occur in one in 300-400 newborn boys. This is usually a combination of two anomalies. One of them is the incorrect development of the male urethra, and the other is the incorrect location of the testicles.

The laying of the testicles in the embryo occurs in the lumbar region, and then descend down, pass through the inguinal canals and descend into the scrotum. As a result of impaired embryogenesis, the testicles may remain in the abdominal cavity or in the inguinal canals and do not descend into the scrotum. This anomaly is called cryptorchidism. There are two forms of cryptorchidism: abdominal and inguinal. With this anomaly, the scrotum is empty or there is complete aplasia.

Another anomaly in false male hermaphroditism is hypospadias. It is an underdevelopment of the peripheral parts of the male urethra with the replacement of the missing part of this canal by a dense scar cord and deformation of the penis. There are several forms of this anomaly depending on the location of the external opening of the urethra.

Hypospadias of the glans penis

The external opening of the urethra opens at the base of the head of the penis. Patients themselves do not notice this form of anomaly and believe that in all people the external opening of the urethra is not at the pole of the head of the penis, but at the base. This form of anomaly does not interfere with either urination or sexual intercourse and does not require treatment.

The trunk form of hypospadias is that the external opening of the urethra opens on the posterior surface of the shaft of the penis. From this opening to the head of the penis there is a short scar cord, which pulls the head to the external opening of the urethra and thereby bends the penis in the form of a hook. This form of the disease causes trouble for patients. When a child urinates, a stream of urine sprays out. In adults, sexual intercourse is impossible because the curved and downwardly fixed penis cannot be inserted into the vagina.

Scrotal form of hypospadias

The external opening of the urethra opens at the root of the penis, where the scrotum begins. Hypoplasia of the penis is noted, and it is also curved with a hook. The act of urination is carried out according to the female type, while squatting. Sexual intercourse is impossible.

Scrotal hypospadias

With this anomaly, the scrotum is split into two halves, which look like the labia majora in women. The external opening of the urethra opens between the halves of the split scrotum. The penis is underdeveloped and looks like a woman's clitoris. The act of urination according to the female type.

Perineal hypospadias

The urethra is short, like a woman's, and it opens at the perineum. The scrotum is split or missing. The penis is pulled up to the external opening of the urethra and looks like a clitoris. As a rule, with scrotal and perineal hypospadias, cryptorchidism is also observed, that is, the testicles are located either in the abdominal cavity or in the inguinal canals.

Newborn boys suffering from scrotal and perineal hypospadias are often registered in maternity hospitals as girls. In such a girl, the external genitalia look female-type. There are labia (split scrotum), there is a clitoris (poorly developed and curved penis). Often in such patients the so-called genitourinary sinus is preserved. This is the cavity into which the urethra flows into the embryo (during embryonic development) and the vagina opens. The opening of this urogenital sinus looks like the entrance to the vagina. Sometimes in such patients the genitourinary sinus becomes a rudiment of the vagina. Such a girl urinates like a woman, squatting, and is raised as a girl.

When puberty occurs, a conflict arises between biological and social sex.

Female pseudohermaphroditism

Female pseudohermaphroditism is that in a woman who has a female genetic (chromosomal) sex and a normal structure of the internal genital organs (uterus with tubes and ovaries), the external genitalia resemble male genitalia. At birth, such patients are often mistakenly assigned male gender. Upon reaching puberty, such a “man” behaves like a woman and becomes a passive homosexual.

There are five degrees of virilization (masculinization, i.e. male appearance) of the external genitalia in false female hermaphrodites.

• I degree - isolated enlargement of the clitoris.
• II degree - the labia minora are underdeveloped. There is a narrowing of the vaginal opening and an enlargement of the clitoris.
• III degree - enlargement of the clitoris. The labia minora are absent. The labia majora are underdeveloped. The urogenital sinus is preserved. The opening of the preserved urogenital sinus is located at the base of the enlarged clitoris.
• IV degree - the clitoris is large and looks like a hypospasmodic penis. It has a head and a foreskin. At the base of such a clitoris, the urogenital sinus opens, into which both the urethra and the vagina flow. The labia majora take on the appearance of a split scrotum. The labia minora are absent. Thus, the external genitalia with IV degree of false female hermaphroditism look exactly the same as the external genitalia of a false male hermaphrodite suffering from scrotal-perineal hypospadias. But the false female hermaphrodite has a uterus and ovaries, and the false male hermaphrodite has a prostate and testicles.
• The fifth degree of masculinization of the external genitalia with false female hermaphroditism is expressed by the fact that there is a normally developed male penis with a male urethra. The vagina either opens at the root of the penis or opens into the posterior portion of the male urethra. The male urethra is formed from the urogenital sinus. This explains the fact that the vagina can open in the posterior part of the urethra. The scrotum may be split, or it may be normally developed, but without testicles. The act of urination in such patients is carried out standing, according to the male type. In case of V degree of false female hermaphroditism, male sex is always mistakenly assigned at birth, and patients come to the attention of urologists.

Both female and male hermaphrodites have a “penis-clitoris” in which there are two cavernous bodies. This penis is always curved downwards and, during erection, takes on an arched shape due to scar remains of the underdeveloped urethra, which pull the head of the penis to the external opening of the urethra (in men) or to the opening of the urogenital sinus (in women).

The urogenital sinus in false and true hermaphrodites is a cavity that opens at the root of the clitoris or penis, into which the urethra and vagina or rudiment of the vagina flow. Sometimes the depth of the urogenital sinus reaches 10-14 cm. Thus, we emphasize once again that with false female and false male hermaphroditism, the external genitalia can look exactly the same. Patients suffering from pseudohermaphroditism need to undergo surgical correction of the genitals and change their legal gender if it was determined incorrectly at birth. False hermaphroditism is clinically manifested by homosexuality and transvestism.

True hermaphroditism and true hermaphrodites

True hermaphroditism seems to be an amazing biological phenomenon. But all life on earth came from hermaphroditism. Viruses, bacteria and protozoa do not have sexes. One individual is enough for reproduction. Hermaphroditism is observed not only in primitive animal organisms. It also occurs as a norm in highly organized animals. For example, worms have a full set of both female and male reproductive organs, and one individual is enough to reproduce.

Hermaphroditism is widespread among insects and the plant world. Hermaphroditism exists as a norm in fish, leeches, shrimp and even lizards. Since a person in the process of embryonic, intrauterine development, in about 1-2 months, repeats the entire process of evolutionary development of the animal world on planet Earth, people have always had, have and will have hermaphroditism in one form or another as an anomaly (deformity).

True hermaphrodites are people who have both male and female gonads in their bodies, and therefore have both female and male sex hormones in their blood. True hermaphroditism is an abnormality of the gonads, either in the form of the presence of separate male and female gonads, or in the form of ovotestis.

It is necessary to distinguish between two variants of true hermaphroditism.

• True hermaphrodites with anomalies of the external genitalia.
• True hermaphrodites without anomalies of the external genitalia.

If there are abnormalities in the development of the external genitalia, true hermaphroditism can be diagnosed in childhood. If there are no abnormalities of the external genitalia, true hermaphroditism can only be diagnosed after puberty. In true hermaphrodites, such clinical symptoms as transvestism and homosexuality are also often observed when the legal sex does not correspond to the hormonal sex, in cases where sex hormones opposite to the legal sex prevail in the blood.

In cases where in true hermaphrodites the external genitalia are correctly developed according to the male or female type and the secondary sexual characteristics correspond to the structure of the external genital organs, diagnosis of true hermaphroditism is possible only after puberty by the presence of two symptoms that are observed only with true hermaphroditism. These are transsexualism and bisexuality (alternating gender). Transsexualism is that a normally developed man considers himself a woman and turns to doctors with a request to change his sex; he cannot live in a male body.

Or a normally developed woman (sometimes even having children) considers herself a man, and insists on legal and surgical gender reassignment. As a rule, transsexualism develops gradually, in the sense that self-identification of the gender opposite to the physical status of a person becomes deeper and deeper, and can even lead to suicide. This is explained by the fact that in the gonad opposite to the phenotypic status of the patient, a tumor (usually an adenoma) very often develops, which produces a huge amount of sex hormones opposite to the somatic status of this person.

Such a hermaphrodite in the guise of a man can have children, but then he develops transvestism, then homosexuality and, in the end, he becomes transsexual. Or a true hermaphrodite in the form of a woman can also have children, but then she develops transvestism, homosexuality, and it comes to transsexualism. The second symptom, which is observed only in true hermaphroditism, is bisexuality or alternating sex. A person at different periods of life has either male or female sexual behavior. Sexual behavior depends on the predominance of androgens or estrogens in the blood.

Thus, there is usually no clear distinction between transvestism, homosexuality, transsexualism and bisexuality, and all four of these symptoms can be combined with true hermaphroditism. Currently, attempts are being made to treat these sexual conditions by affecting the brain, including brain surgery. Exposure to the brain cannot cure the clinical symptoms of hermaphroditism. It is necessary to ensure that hormones of only one sex (male or female) enter the brain through the blood.

From a clinical point of view, it is advisable to divide true hermaphroditism into two categories:

• true hermaphroditism in combination with anomalies of the external genitalia;
• true hermaphroditism without anomalies of the external genitalia and without secondary sexual characteristics of the contralateral sex.

True hermaphroditism in combination with anomalies of the external genitalia. Such anomalies usually include penoscrotal, scrotal or perineal hypospadias. Often, preservation of the genitourinary sinus in combination with cryptorchidism is observed. Sometimes there are secondary sexual characteristics of the gender opposite to the passport one. There is a sharply enlarged clitoris in the form of a hypospasmodic penis.

Such people turn to urologists, andrologists and gynecologists for help, and sometimes they are diagnosed with true hermaphroditism. But usually the external genitalia are surgically reconstructed and adjusted to the legal gender, and (true) hermaphroditism is not diagnosed.

True hermaphrodites, who have no abnormalities of the external genitalia and no inversions of secondary sexual characteristics, typically have a complete set of all the genital organs, male or female, and have a gonad or gonadal tissue of the contralateral sex. In such patients, hermaphroditism is almost never diagnosed, although they have clear symptoms of hermaphroditism in the form of transvestism, homosexuality, transsexualism and bisexuality. If such people turn to a urologist, andrologist, gynecologist or endocrinologist, these doctors note the correct development of the external genitalia and send them for psychotherapy to a sexologist. Sexologists can neither diagnose nor cure hermaphroditism.

Transvestites, homosexuals and bisexuals usually accept their gender anomaly. And transsexuals, by hook or by crook, are trying to change their legal gender. Transsexualism syndrome occurs in those true hermaphrodites who have a full set of all genital organs of one sex, which allows them to be the mother or father of the child. But they have a gonad of the opposite sex. Transsexualism begins to increase as a hormone-producing tumor of the opposite sex appears in the gonad of the opposite sex in the form of an adenoma. And the radical treatment of transsexualism consists in finding and removing this gonad of the opposite sex with a tumor. There are three types of gonadal anomalies with true hermaphroditism:

• a person has one or two gonads, testicles, and he also has one or two ovaries;
• in humans, one or two gonads are built according to the ovotestis type;
• In humans, one or two gonads are built in a mosaic pattern. In the gonad, the testicular and ovarian tissues are intertwined in a mosaic pattern.

Sexologists believe that if the external genitalia (male or female) are developed normally and do not have any anomalies, then there can be no hermaphroditism. With true hermaphroditism, the external genitalia can be developed absolutely normally. True hermaphroditism is not anomaly of the external genitalia, but anomaly of the gonads.

There are three main morphological variants of true hermaphroditism:

• The first option: a person has a full set of genital organs of one sex (male or female) and also has one or two gonads of the opposite sex in the body without any anomalies of the external genitalia.
• The second option: a person has a full set of genital organs of one sex (male or female), one or two gonads of the opposite sex, as well as other (except gonads) genital organs of the opposite sex. In “men” (according to the full set of genital organs) - the vagina or uterus, or mammary glands. In “women” (according to the full set of genital organs) - scrotum, penis, prostate.
• The third option for true hermaphroditism: a person has gonads of both sexes and incomplete sets of other genital organs in various combinations. This option is characterized by various anomalies of the external genitalia and therefore urologists, andrologists and gynecologists diagnose it most often.

Undoubtedly, there is another, fourth, morphological variant of true hermaphroditism, when a person has complete sets of genital organs. both men and women. Such hermaphrodites have a scrotum and testicles in the scrotum, there are epididymis, vas deferens, seminal vesicles, prostate and penis with the male urethra. But in this same person, between the root of the penis and the beginning of the scrotum, there is an entrance to the vagina, vagina, cervix, uterus, fallopian tubes and ovaries. A variant of such true hermaphroditism in humans has not yet been described in the domestic medical literature.

Hermaphroditism can develop against the background of any genetic, chromosomal sex. With the male chromosomal sex 46XY, hermaphroditism is most often accompanied by scroperineal hypospadias with cryptorchidism, and the presence of a genitourinary sinus, or without it. With female chromosomal sex 46XX, hermaphroditism is most often observed in the presence of clitoral hypertrophy and vaginal ectopia of the urethra with or without the presence of a urogenital sinus.

Much less often, hermaphroditism is observed with a mosaic structure of chromosomes: XX/XY; XX/XXYY; XX/XXY. Other options for the set of sex chromosomes are also possible.

Based on clinical observations, a working classification of forms and variants of hermaphroditism has been developed.

Classification of forms and variants of hermaphroditism

• False hermaphroditism
  • Male false hermaphroditism.
  • Female false hermaphroditism.
• True hermaphroditism
  • Presence of gonads of both sexes.
  • Ovotestis.
  • Mosaic structure of the gonads.
• True hermaphroditism without abnormalities of the external genitalia
  • With male secondary sexual characteristics.
  • With female secondary sexual characteristics.
  • With secondary sexual characteristics of both sexes.
  • A complete set of genital organs of one sex (male or female) and a gonad (or gonad tissue) of the opposite sex with a hormone-producing tumor in it, which is manifested by transsexualism.
• True hermaphroditism with the presence of anomalies of the external genital organs
  • A complete set of genital organs of one sex (male or female) and the presence of organs of the opposite sex.
  • Incomplete sets of genital organs of both sexes in various combinations.
  • Complete sets of genital organs of both male and female sexes.

Diagnosis of hermaphroditism

Gender is a very important characteristic of absolutely every person. In all documents that accompany a person from birth to death, the second column after the last name, first name and patronymic is the gender designation. Gender is indicated on the birth certificate, on all forms filled out during life, and on the death certificate.

Diagnosis of sex in hermaphroditism is based on the determination of six main sex criteria. Therefore, sometimes they say and write that each person has not one, but six genders, and they may or may not coincide. Each person has a genetic sex, gonadal sex, hormonal sex, phenotypic sex, psychological (mental) sex and legal (passport) sex.

Genetic sex is determined at the moment of fertilization. It is also called chromosomal sex.

The normal human chromosome set contains 22 pairs of chromosomes. In addition, there are two more sex chromosomes. There are 46 chromosomes in humans. Female sex chromosomes are designated by the sign X. Women have two of them. The chromosomal genotype of a woman is designated 46XX. Sex cells (gametes) contain a half set of chromosomes, which includes only one sex chromosome. Eggs are produced in the female gonad (ovary) and contain 22 chromosomes (autosomes) and one sex X chromosome. Spermatozoa (spermine) are formed in the male gonad (testicle), contain 22 autosomes and one sex chromosome (either the X chromosome or the Y chromosome). Y is the designation of the chromosome that determines male sex. If, as a result of fertilization of an egg with sperm, a set of sex chromosomes is XX, the embryo develops female genital organs. If a set of XY sex chromosomes is obtained, the embryo develops male genital organs. This is how nature “intended”, but there are deviations. The karyotype of a man is designated 46XY. If, as a result of fertilization, a so-called mosaic set of sex chromosomes is obtained (XX/XY; XX/XXYY; XXX/XY; XX/XXY, etc.), then the embryo will form both female and male genital organs, that is, a bisexual organism will develop in the form false or true hermaphrodite. However, hermaphroditism can develop with both a normal female karyotype (46XX) and a normal male karyotype (46XY).

Diagnosis of chromosomal, genetic sex, genotype, karyotype is carried out by studying chromosomes, but this requires special equipment and qualifications. A widely used method for diagnosing the genotype is the method of determining the sex chromatin of cell nuclei. To do this, a skin section or scraping from the oral mucosa, or a blood smear is performed. The drug is subjected to special staining. The location of the staining parts in the cell nuclei is different in men and women. The method of determining genetic sex by sex chromatin is imperfect because the arrangement of sex chromatin typical for the female sex is found in women only in 70-90% of all cells, however, such an arrangement of sex chromatin is found in 5-6% of the cells of the male body.

Chromosomal sex by itself (in isolation) is not an accurate determinant of a person’s gender. With the normal male genotype (46XY), a person cannot be a woman, but he can be a hermaphrodite (bisexual) or a eunuch (asexual). So, with anorchism (gonadal agenesis) aplasia of both testicles, the karyotype can be male (46XY), the genital organs develop either according to the female type or have a rudimentary structure. This is classic eunuchoidism with a male genotype.

The second sex (sexual criterion) that every person has is gonadal. This gender is also called true sex or biological sex. Humans have four gonadal sexes:

• female gender - the body has ovaries:
• male gender - the body has testicles;
• bisexuality (true hermaphroditism) - the body contains both ovarian and testicular tissue:
• asexuality (eunuchoidism) - there are no sex glands (gonads) in the body.

Gonadal sex is determined by biopsy and histological examination. Pieces for histological examination are taken from both glands, since one of them may turn out to be a testicle, and the other an ovary. It is necessary to examine the gonad from one and the other pole, since one half of the gland may be an ovary and the other half a testicle. This gonad is called ovotestis. The gland can also have a mosaic structure (there are sections of testicular tissue in the ovarian tissue, or, conversely, there are sections of ovarian tissue in the testicular tissue). In order to take a piece of the gonad for research, you need to find it and expose it. Human gonads are normally located in the scrotum in men, and in the abdominal cavity on the sides of the uterus in women. With hermaphroditism, the testicle may be in the abdominal cavity, and the ovary may be in the scrotum. By the way, the uterus may also be in the scrotum. Gonads can also be in the labia majora, in the inguinal canals, in the perineum and in inguinal hernias. The gonads are in the scrotum, in the inguinal canals and labia can be palpated. To determine the presence of gonads in the abdominal cavity, ultrasound and laparoscopy are performed, which can be combined with a biopsy. Normally, the genetic sex should match the gonadal sex, but they may not coincide, and then different options are observed gender anomalies.

The third sex (sexual criterion) is hormonal. It is also called biological or true sex. It would seem that the gonadal sex should always coincide with the hormonal sex because sex hormones are produced by the gonads. The ovary always produces estrogens, and the testicle should always produce androgens.

Hormonal sex is diagnosed by determining the level of androgens and estrogens in the blood. There are also four hormonal sexes in humans:

• male - normal level of androgens in the blood;
• female - normal level of estrogen in the blood;
• hermaphroditism in the blood, high levels of both androgens and estrogens (the amount of male and female sex hormones in the blood varies widely);
• asexuality - there are no or almost no female or male sex hormones in the blood.

Normally, the genetic, gonadal and hormonal sexes should match, that is, they should all be male or all should be female. If they do not match, then a sex anomaly occurs.

The fourth sex (sexual criterion) of each person is somatic or phenotypic sex. This is the general appearance of a person, the structure of the external genitalia, secondary sexual characteristics, clothing and shoes, hairstyle and jewelry. All these signs depend on the influence of sex hormones and may vary depending on age and the occurrence of tumors in the gonadal tissue. Changes in the phenotypic picture are always due to changes in the hormonal activity of the gonads. There are four phenotypic sexes in humans:

• male - a person looks like a man;
• female - a person looks like a woman;
• bisexuality in a person’s appearance has both male and female traits;
• eunuchoidism - a person in appearance has neither male nor female features. He is childish and looks like a child.

Phenotypic sex always coincides with gonadal and hormonal sex, but may not coincide with genetic sex. Phenotypic sex can only be diagnosed in sexually mature individuals based on their appearance. Children do not have secondary sexual characteristics, and somatic sex is diagnosed only by the structure of the external genitalia. And when diagnosing it, errors are common, since the structure of the external genitalia may not correspond to the gonadal and hormonal sex. Somatic sex can change under the influence of hormonal treatment of certain diseases.

The fifth gender (sexual criterion) of a person is psychological or mental. This gender is completely determined by the presence or absence of sex hormones in the blood. Children have no sex hormones in their blood and no sexual behavior.

Mental gender in children is determined by the sexual psychoorientation that the child receives from his parents and people around him. In sexually mature people, psychological gender is determined by gender self-identification: whether a person feels like a man or a woman. There are four mental genders:

• male gender - male sexual behavior;
• female gender - female sexual behavior;
• bisexuality is manifested by either male or female sexual behavior (alternating sex; bisexuality);
• asexuality - no sexual behavior.

The sixth gender (sexual criterion) of a person is the legal, passport, metric, civil, social, legal gender. This is the gender that is indicated in a person’s personal documents. There are only two legal genders: male and female. Passport gender may not coincide with genetic sex, gonadal sex, hormonal sex, somatic sex, or psychological sex. Hermaphrodites and eunuchs have either male or female documents. In the first days after the birth of a child, the gender is officially registered as male or female. In this case, the gender may be determined incorrectly. Incorrect sex determination occurs not only in cases of anomalies of the external genitalia. With typically female or male external genitalia, the sex may be determined incorrectly, since the child may turn out to be a false or true hermaphrodite.

Thus, at present, urologists and gynecologists can, using surgical treatment of hermaphroditism, create male and female external genitalia. On the male side: by straightening the deformed body of the penis and creating an artificial male urethra from the patient’s own tissues, as well as surgical or hormonal (using human chorionic gonadotropin) reduction of the testicles into the scrotum. The scrotum, in turn, is created from the skin of the patient’s perineum or “labia”.

Correction of the external genitalia in the female direction consists of surgically reducing the size of the “clitoris-penis”, as well as creating a vagina using tissue and the cavity of the urogenital sinus, or alloplastic materials, or a fragment of the peritoneum. Moreover, the possibility is now opening up to create surgically sex gonads by transplanting into the body a male or female gonad on a vascular pedicle, which will ensure the appropriate gonadal and hormonal sex. Hormonal sex can also be created by injecting cultures of embryonic cells into the body that produce sex hormones. These methods relieve patients from daily administration of synthetic sex hormones.

Children do not have clinical manifestations of true and false hermaphroditism in the form of transvestism, homosexuality, transsexualism and bisexuality, but the prevention of these phenomena is entirely up to pediatricians. They should understand abnormalities of the external genitalia and all such children should be referred to urological, gynecological or pediatric surgical clinics.

True hermaphroditism can be cured if the gonad (or gonad tissue) of the opposite sex is found and removed from the body. You need to look for the gonad in the pouch of Douglas, in the scrotum, in the inguinal canals and in the labia majora. If a separate gonad is not found, it is necessary to do a biopsy of the gonads and, in cases of ovotestis, to perform resection of the gonad, and in cases of mosaic structure of the gonads, it is necessary to raise the question of castration, followed by hormone therapy or transplantation of a donor gonad and correction of the external genitalia.

Treatment for hermaphroditism is very effective and involves removing the gonads of one sex and leaving the gonads of the other sex. The direction of gender correction in the male or female direction depends on the development and anomalies of other genital organs, except the gonads. Sexually mature patients usually choose the direction of gender correction themselves. They feel like they are either men or women. This sexual self-awareness depends only on which sex hormones (male or female) prevail in the body.

Dynamic monitoring of patients with false and true hermaphroditism consists of periodically determining the concentration of pituitary-gonadal hormones in the blood and performing an ultrasound of the genitals and gonads. It is necessary to control the stability of the patient’s sexual self-identification and his phenotypic secondary sexual characteristics.

Prevention

Prevention of hermaphroditism - minimizing the influence of factors causing disembryogenesis, including the correct use of hormonal estrogen-containing contraceptives by women planning pregnancy. It has been proven that when a fetus is conceived, which occurs against the background of prolonged hyperestrogenization of the female body, the risk of the formation of anomalies of the external genitalia and gonads increases, in particular various forms of hypospadias in male fetuses.

Forecast

The prognosis for the life of patients with hermaphroditism is generally favorable, but we must not forget about the possibility of malignancy of the abnormal gonad, including ovotestis. In this case, the prognosis of the disease depends on the histological form of the gonadal neoplasm. For the purpose of prevention and early warning of the disease, it is advisable to conduct an ultrasound of the structure of the gonad tissue approximately once every six months. If a neoplasm is detected, it is imperative to perform a biopsy and histological examination of the formation to decide whether radical surgery is necessary.

Unfortunately, at present, patients with false and true hermaphroditism, especially those who do not have external anomalies of the external genitalia, do not always receive timely, qualified care. This is due to two factors - insufficient coverage of this problem in the medical literature (the problem of hermaphroditism is not included in the curricula of medical universities), and the reluctance of the patients themselves to advertise their condition, taking into account the specific clinical manifestations of this disease in the form of homosexuality, transsexualism, bisexuality and attitudes towards them society.

Therefore, the majority of these patients are in the category of sexual minorities. It sounds absurd, but patients with hermaphroditism, who have external anomalies of the external genitalia, are in a better position. They, as a rule, attract the attention of urologists or gynecologists; they are subjected to a special examination, as a result of which gonadal abnormalities are often detected.