Hermaphroditism

Hermaphroditism, also called hermaphroditism or hermaphroditism, refers to individuals who cannot be clearly assigned to one sex genetically, anatomically or hormonally. Today, however, the term intersexuality is more commonly used for this medical phenomenon. Intersexuality belongs to the sexual differentiation disorders. The German Institute of Medical Documentation and Information (DIMDI) (ICD-10-GM-2018) classifies this form in chapter 17 (Congenital malformations, deformities and chromosomal anomalies) also congenital malformations of the genital organs, especially an indeterminate sex and pseudohermaphroditism. Affected individuals usually reject the pathologizing medical term for the disorder.

What is hermaphroditism?

Hermaphrodites are people with ambiguous sex characteristics. In most cases, the genitals of hermaphrodites are abnormally shaped. In psychological hermaphroditism, although there is no dual sex physically, the affected person cannot identify with only one sex. One also speaks of a third gender. In pseudohermaphroditism, the chromosomal sex and internal genitalia do not match the external sex or external genitalia and secondary sex organs. Pseudohermaphroditism is closely related to the concept of androgyny. There is a male and a female pseudohermaphroditism. In the male form, the internal sex is male, but the external sex is female. In female pseudohermaphroditism, it is the other way around.

Causes

An ambiguous body sex can have several causes. For example, chromosomal variation, that is, altered chromosomes, can result in intersexuality. Known chromosomal disorders associated with hermaphroditism are Klinefelter syndrome with a male appearance and Turner syndrome with a female appearance. A gonadal variation is also conceivable. In this case, there is a developmental disorder of the gonads. Gonads are sex glands in which sex hormones and germ cells are produced. In men these are the testicles, in women the ovaries. If the gonads are missing or completely non-functional, this is called agonadism. But also a partial formation can lead to intersexuality. The insufficiently developed strip gonads are not able to produce sufficient sex hormones. If a gonad combines the functions of testis and ovary and produces both eggs and sperm, this is called ovotestis. Other causes of hermaphroditism are hormonal disorders. These can be chromosomal or gonodal. Enzyme defects or kidney disorders can also lead to an imbalance in hormones.

Symptoms, complaints, and signs

As diverse as the causes of intersexuality are, so are its manifestations. In addition to 22 pairs of chromosomes, men usually have one X chromosome and one Y chromosome. Women, on the other hand, have two X chromosomes. If a mistake occurs during sperm production and a sperm without X and Y chromosomes fertilizes an egg, so-called X0 individuals develop. These individuals are therefore missing a sex chromosome. Since an X chromosome is present, the X0 individuals develop into females. However, these females are sterile and cannot father children. This condition is called Turner syndrome. Klinefelter syndrome occurs more frequently. In this case, the sex chromosomes have not separated during sperm maturation and the father has inherited two sex chromosomes to the child. Together with the inherited sex chromosome of the mother, the child now has two X chromosomes and one Y chromosome. Due to the dominant Y chromosome, the children are male but suffer from low testosterone levels due to the second X chromosome. This results in small testes and inability to conceive. In many Klinefelter patients, however, the symptoms are rather mild and often go unnoticed. If the chromosome set is normal and the affected person suffers from so-called insufficient androgen resistance, i.e. a reduced effect of male sex hormones, reduced beard and body hair as well as infertility are the result. In the case of complete androgen resistance, no visible male sex organs are formed. The testes remain inside the body, a vagina is visible externally, but the fallopian tubes and uterus are absent.The affected persons are perceived as girls. The diagnosis is usually made as an incidental finding.

Diagnosis

If there is a suspicion of a disorder of sex differentiation, various examinations of the blood are performed. First, the hormone status is determined, and second, an examination of the chromosome set is performed. In addition, the abdominal and pelvic area is examined by ultrasound. Here, attention is paid to the presence or absence of the uterus and ovaries. With the help of an X-ray, a so-called genitogram can be used to determine whether a vagina is present. In some cases, a biopsy of the gonads is required to determine what tissues are present in the sex glands. This examination is performed under anesthesia in the hospital. Diagnostics can also be used to make a prognosis about the fertility, or fertility, of affected individuals.

Complications

Due to hermaphroditism, in some cases there are various psychological and physical complaints. In most cases, men suffer from a general inability to conceive. This cannot be treated at present, so those affected suffer from this complaint throughout their lives. Furthermore, there are female characteristics in men, so that for example the beard growth is reduced or also the testicles have a small size. It is not uncommon for patients to be mistaken for a girl or a woman. This behavior can have an extremely negative effect on the quality of life and will not infrequently lead to psychological complaints and depression. Everyday life then becomes extremely difficult for the person affected by the hermaphroditism. The treatment of hermaphroditism does not lead to further complications. In most cases, it is possible to compensate for any symptoms of hermaphroditism with the help of hormones. Likewise, parents can choose a gender for their child if it is not clearly evident at birth. Often, unfortunately, children experience bullying and teasing and social exclusion. These complaints can also be examined and treated by a psychologist. Hermaphroditism does not lead to a reduction in life expectancy.

When should you see a doctor?

As a rule, it is not necessary to see a doctor for diagnosis in hermaphroditism, because the condition can be detected immediately after birth or even before birth. However, those affected are dependent on therapy and treatment, especially at the beginning of their lives, to alleviate the symptoms. A doctor should generally be consulted if symptoms occur despite therapy. A visit to a doctor is particularly necessary in the case of an inability to conceive. The doctor should also be consulted if hermaphroditism is not detected immediately after birth, but later in life. In this case, the symptoms can also be treated by surgical intervention or with the help of hormones. Furthermore, many patients also suffer from psychological complaints due to hermaphroditism and therefore require psychological treatment. Here, early diagnosis and treatment always has a positive effect on the further course and can prevent various complications.

Treatment and therapy

If a disorder of sex differentiation was diagnosed in children from the year 1960, gender reassignment surgery was often performed shortly after birth. This was usually followed by hormone treatment. This had drastic consequences and often led to infertility later on. Medical information was often inadequate and the operations were not always necessary. Today, surgical interventions to adjust the sex are seen rather critically. In case of gender ambiguity, parents have the right to choose the gender of their child. Since 2009 it is possible to get a birth certificate without a registered gender. This means that parents with a known disorder do not have to decide on a gender immediately after birth and can let their child decide later. Therapies today are much more individualized than they were in the 1960s and 1970s. The focus is not on anatomical assimilation, but rather on the psychological handling of the affected persons with their physical conditions. Many intersexual people fight for the fact that their intersexuality is no longer perceived as a disease but as a variation of the normal gender development.Therapy is not seen by them as help, but as discrimination.

Outlook and prognosis

Hermaphroditism persists for life without treatment. In humans, true hermaphroditism does not exist, but so-called pseudo-hermaphroditism does. Since the rare cases are always very individual, it must be decided on a case-by-case basis whether treatment is necessary and useful at all. In most cases, it is not until the teenage years or young adulthood that it is recognized that a case of pseudo- hermaphroditism exists, so that sexual characteristics have long since formed and the person also feels that he or she belongs to one or the other sex. Treatment can be useful if the patient does not identify with the biological sex. Then it can be considered whether a partial or complete sex reassignment could reduce the suffering. If, on the other hand, the patient feels that he or she belongs to the sex to which he or she has been assigned, it may be useful to use surgical and medicinal measures to regress the characteristics of the other biological sex. If the external or internal sex organs have unusual shapes, this can be corrected surgically, depending on the necessity. Often this alone reduces the hormone levels of the opposite sex, so that hormonal treatment over a short period of time is sufficient. This can help to develop the characteristics of the actual sex more strongly in pseudo-hermaphroditism. However, the later the (pseudo)hermaphroditism is recognized, the more difficult it becomes to still influence the development.

Prevention

Intersexuality cannot be prevented, since in many cases it is a hereditary disorder in sex development.

Aftercare

In hermaphroditism, there are usually no direct options or measures of aftercare available to the affected person. In this case, the patient is first dependent on a comprehensive diagnosis and treatment. However, the symptoms cannot always be completely alleviated. Only in a few cases are surgical interventions possible that assign a clear gender to the affected person. In most cases, comprehensive surgery is performed immediately after birth to alleviate the symptoms. Therefore, the early recognition of the disease by the respective symptoms is also in the foreground. After the operation, the patients are dependent on taking medication, mainly hormones. As a result, the sexual organs are fully developed. Parents in particular must pay attention to the correct and regular intake of the medicines, so that the symptoms are completely alleviated. As a rule, psychological treatment is also necessary in hermaphroditism. This should be initiated at a very early stage. The support of the patient’s family, relatives and friends is also very important. The relatives must understand the disease and deal with the patient in the right way. The life expectancy of the affected person is not reduced by hermaphroditism.

This is what you can do yourself

The relationship of an intersexual to his sexual characteristics, physical and psychological, often depends on his socio-cultural environment, and not infrequently on his medical history. For many decades, irreversible alignments were performed shortly after birth or in infancy, which later led to serious problems in finding identity for some affected persons; the same applies to hormone administration. Thereby the pathologization of hermaphroditism without the presence of a health complaint is to be questioned in principle. Intersexuals, whose bodily functions, if necessary apart from their reproductive ability, are not or hardly limited by their more or less pronounced sexual characteristics, are normal healthy people with regard to their gender identity. (And there are also intersexuals who feel “male” or “female” despite their sexual characteristics and do not belong to a “third sex”, which makes them also “male” or “female”). However, since they might be considered exotic, especially in non-intersexual circles, despite the normality of intersexuality to be attributed to them, many keep their true gender identity – belonging to the “third gender” – secret at work, etc.Special (regional and interregional) self-help groups offer the possibility of exchange with others. In addition, reading about the recognition of hermaphrodites in other times and cultures can be enriching when dealing with one’s own gender; for example, there are three genders in Buddhist myths of origin. From a legal perspective, the emancipation of intersex people continues to be on the move; on November 8, 2017, the Federal Constitutional Court in Karlsruhe ruled that intersex people must be granted a “positive identity” – that is, not just the choice to be neither “male” nor “female.”