Female pseudohermaphroditism is a physiological anomaly of a person associated with the presence of external sexual characteristics of a male in a woman and vice versa. This phenomenon is considered a congenital deviation of the reproductive system.
The content of the article:
Reasons for the development of female pseudohermaphroditism
Signs of female pseudohermaphroditism
Diagnosis of pseudohermaphroditism in women
Ways to treat female pseudohermaphroditism
Prognosis for the future with a diagnosis of hermaphroditism
Female pseudohermaphroditism
Female pseudohermaphroditism is the presence of more or less developed male genitalia in a woman based on the characteristics of the genital glands. The main criteria for the diagnosis of this disease, doctors consider the formation of a woman’s external genitalia characteristic of men, that is, the penis, as well as delayed puberty, underdevelopment of female genitalia.
Women who have been diagnosed with such an anomaly are usually distinguished by the presence of a sufficiently developed uterus, fallopian tubes, ovaries, vagina, and a woman’s chromosomal set. And external sexual characteristics, on the contrary, indicate male development: the structure of the body, the timbre of the voice, thick hair, the presence of a more or less pronounced male hollow organ. There are situations when determining the sex of a person causes difficulty even for experienced specialists. This is due to the unhurried development of the disease itself. However, it is very important to determine the correct gender identity of the child in time, creating comfortable conditions for psychological adaptation in society.
Reasons for the development of female pseudohermaphroditism
The reasons that cause such serious sexual deviations in girls are hidden even in the process of its development in the womb. Gynecologists often warn women, urging them to take a responsible approach to the issues of motherhood and conception of the baby, emphasizing that pregnancy, if possible, should be planned. Unexpected conception can lead to all sorts of deviations in the development of the unborn baby, especially if the actual pregnancy was diagnosed at a period of more than eight weeks. The thing is that it is at the stage of such early development that the embryo forms its sexual identity. The embryo at the age of eight to ten weeks undergoes sexual differentiation of the reproductive system. If at this moment a woman takes hormonal drugs or other medications that are not agreed with the doctor, they can distort the processes of sexual determination of the child, which will lead to the development of false or ordinary hermaphroditism.
The reasons for the formation of female pseudohermaphroditism are considered genetic abnormalities, as well as the harmful effects of external factors on embryonic development. These include such phenomena as radiation exposure to a pregnant woman, her chemical, narcotic, alcohol intoxication, the use of synthetic progesterones and androgens in the first trimester of pregnancy, some endocrine diseases of the mother.
Examining the described sexual anomaly, most often doctors find a noticeable lack of the enzyme 21-hydroxylase, which plays a crucial role in the release of such important adrenal hormones as cortisol and aldosterone. It is known that their lack during the period of sexual intrauterine development of the baby leads to increased synthesis of androgens, sex hormones, in girls, which causes virilization. It is noteworthy that in some cases, such an adrenogenital syndrome can lead to acute adrenal insufficiency, less often to a fatal outcome.
Signs of female pseudohermaphroditism
As mentioned above, deviations in the process of intrauterine genital differentiation of the baby most often cause the birth of a child with an indeterminate gender. In most cases, such an anomaly is noticed immediately when diagnosing female pseudohermaphroditism. At the same time, doctors are guided by the explicit heterosexual structure of the girl’s genitals. The main symptoms indicating this disease are called:
a significant increase in the clitoris, emphasizing the similarity with the penis;
partial or complete fusion of the labia majora, blocking of the vaginal opening (in the form of a male scrotum);
manifestation of the urogenital sinus, more or less pronounced virilization of the urethra.
In newborns, adrenogenital syndrome or pseudohermaphroditism in girls may manifest through signs of hypertension or dehydration.
Such sexual abnormalities require immediate treatment, immediately after diagnosis. Otherwise, female pseudohermaphroditism already in the first few years of a child’s life causes the accelerated development of virilization. In addition to this, there is a faster somatic maturation, the epiphyseal slits are tightened much faster, which causes stunting. The puberty period has its surprises: the girl’s voice tone decreases (it becomes similar to a man’s), female sexual development slows down, the menstrual cycle may not occur, acne appears, the growth of excess hair, shoulders may expand and muscle mass may increase.
Diagnosis of pseudohermaphroditism in women
Experts say that female pseudohermaphroditism requires detection at an early stage of development. This will facilitate the treatment process and minimize the complications of the disease. The primary diagnosis of abnormalities in the formation of the genitals of a baby by a pediatrician most often occurs immediately after his birth. However, to confirm or refute such a diagnosis, a detailed examination of the genitals by narrowly specialized specialists – a pediatric urologist, endocrinologist and gynecologist is required. By general examination and palpation of the internal genitals, physicians evaluate the sexual difference of the patient, guided by the following criteria:
position, size and structure of the present genitals;
assessment of the degree of their development;
the presence of a vaginal opening, its size;
are the ovaries palpable, if so, what is their size;
the correct position of the urethra;
the presence and level of fusion of masonry – labioscrotal.
At the next stage of the diagnosis of female pseudohermaphrodism, an examination is carried out for the presence or absence of congenital pathology of the adrenal glands, their hyperplasia. In order to avoid mistakes in determining the true sex of the child, the family history is very carefully checked, the history of pregnancy is studied, the use of instrumental diagnostics, laboratory tests.
In parallel with the “archival” studies, cytogenetic studies of the baby’s urine and blood serum are performed for the karyotype of leukocytes, pituitary hormones, electrolyte levels, hormones of the gonads and adrenal glands. To visualize the internal genitalia, uterus, cervix, uterus and ovaries, they resort to ultrasound examination of the small pelvis of the child.
If, as a result of all the examinations carried out, the presence of female pseudohermaphroditism is confirmed, then the newborn is assigned a passport, gonadal and chromosomal female sex.
Ways to treat female pseudohermaphroditism
Treatment of female pseudohermaphroditism begins with its diagnosis immediately after birth. Sometimes the detection of such a sexual anomaly can be delayed, which has a very negative effect on the situation, since effective treatment should begin at the age of no later than two years. It is very important from the first day of a baby’s life with a diagnosis of female pseudohermaphroditism to monitor compliance with its water-electrolyte and salt balance. In parallel with this, hormone replacement therapy should be carried out.
We must not forget about the psychological component of treatment, and not only children, but also their parents need the help of psychologists.
With the help of reconstructive plastic medicine, the patient is undergoing intimate plastic surgery – surgery to correct the genitals. At the age of up to two years, the child should have a reduction clitoroplasty, and while the girl has not yet reached the beginning of puberty, vaginoplasty. Such operations are necessary for a woman to acquire the natural state of her genitals and restore their functionality.
By applying the right treatment, a woman suffering from pseudohermaphroditism can count on a complete transformation of her body – the restoration of the normal appearance of internal and external genitalia, the restoration of the menstrual cycle, the acquisition of smoothness of the skin, a change in voice, etc. It is noteworthy that patients with a similar diagnosis, receiving proper treatment throughout the time, are able to lead a full sexual life and are even capable of conceiving a child.
Prognosis for the future with a diagnosis of hermaphroditism
The condition of a person suffering from such a sexual anomaly as female pseudohermaphroditism fully depends on the timeliness of the start of treatment, the correctness of its management and the quality of plastic surgery performed.
If a child was diagnosed with female pseudohermaphroditism from the moment of his birth and began to provide the necessary care, then most likely his life will differ little from the life of his peers. The cosmetic operations performed will help to establish not only a healthy appearance of the external genitals, but also to restore menstrual and reproductive functions. It is important not to forget to take hormones that block the virilization of the girl’s body.
In the absence of treatment or a high degree of neglect of the disease, a woman faces serious virilization of the body, the inability to conduct a full sexual life, the development of the menstrual cycle and female puberty, the possibility of pregnancy and childbirth is excluded.
