Hermaphroditism is a form of disorder of gender development and nowadays it called “intersexuality” or “intersex”. The older term (“hermaphroditism”) came from joining the names of a Greek god and goddess – Hermes and Aphrodite. Hermes was a god of male sexuality and Aphrodite was a goddess of female sexuality, love and beauty. Hermaphroditos is also the hero of a Greek myth who is half-man and half-woman – he is a son of Hermes and Aphrodite.
Although the older term is still used by some professionals but in most cases this term “hermaphroditism” is changed because it could be misleading, confusing, not client-friendly and insensitive. Nowadays more often this group of conditions is being called disorders of sex development (DSDs).
Generally speaking, intersexuality (“hermaphroditism”) is a group of conditions where there is a discrepancy between the external genitals and the internal genitals (the testes and ovaries) – it is a medical term for an intersex condition in which an individual is born with ovarian and testicular tissue.
In some cases there could be an ovary on one side and a testis on the other, but more commonly one or both gonads is so called “ovotestis” which contain both types of tissue.
Hermaphroditism is very rare condition and it is possible only if person has the following karyotypes – 47XXY, 46XX/46XY, or 46XX/47XXY.
Even with modern diagnostic techniques the causes of hermaphroditism are not well known. But some scientists suggest that hermaphroditism could be caused by the division of one ovum, followed by fertilization of each haploid ovum and fusion of the two zygotes early in development OR it could be caused by fertilization of ovum by two spermatozoids OR it could be caused by two ovum fertilized by two spermatozoids (if one male zygote and one female zygote fuse, a hermaphroditic individual may result).
Hermaphroditism could be associated with mutation.
According to medical professional there are four categories of hermaphroditism (intersexuality):
Category 1 – 46, XX Hermaphroditism
Category 2 – 46, XY Hermaphroditism
Category 3 – True Gonadal Hermaphroditism
Category 4 – Complex or Undetermined Hermaphroditism
46, XX hermaphroditism
Hermaphrodit client has the chromosomes of a woman and female ovaries BUT external genitals appear male. In most cases it is the result of a female fetus having been exposed to excess male hormones before birth. Usually this type of clients have normal uterus and Fallopian tubes BUT never have period (AMENORRHEA). This type of hermaphroditism called “female pseudohermaphroditism”. Main causes of “female pseudohermaphroditism” include the following:
- Congenital adrenal hyperplasia (very common cause);
- Male hormones used by pregnant mother (for several reasons or by mistakes);
- Tumors which produce male hormones during pregnancy (often ovarian tumors);
- Enzyme pathology (aromatase deficiency) which can be discovered at puberty.
46, XY hermaphroditism
Hermaphrodit client has the chromosomes of a man, but the external genitals are incompletely formed, ambiguous or clearly female. Internally, testes could be normal or malformed or absent. This condition used to be called “male pseudohermaphroditism”. Main causes of “male pseudohermaphroditism” include the following:
- Problems with the tissue of testes;
- Enzyme deficiency in testosterone formation;
- Enzyme deficiency in testosterone absorption in body tissues (androgen insensitivity);
- Misuse of testosterone and/or wrong prescriptions of testosterone.
True Gonadal hermaphroditism
Clients with true gonadal hermaphroditism have both ovarian and testicular tissue. In some cases could have both tissues in the same gonad (in so called “ovotestis”) and/or some clients could have one ovary and one testis.
Clients could have XX chromosomes or XY chromosomes or both. In these cases the external genitals could be ambiguous or could appear as female or male. This condition used to be called “true hermaphroditism”.
Unfortunately the causes of “true hermaphroditism” are not known.
Complex or undetermined hermaphroditism
If client has chromosome configurations other than simple 46, XX or 46, XY, it could be a reason for sex development diseases. In these cases the chromosomes could be 45, XO (only one X chromosome) or 47, XXY or 47, XXX.
Complex hermaphroditism do not result in discrepancy between internal and external genitalia but there could be problems with sex hormone levels, overall sexual development and/or altered numbers of sex chromosomes.
Symptoms of hermaphrotism could be different depending on main causes but in most cases hermaphroditism causes include the following:
- Absence of periods – AMENORRHEA;
- Delayed or absent or abnormal puberty development;
- Ambiguous and/or Unusual genitalia at birth;
- Clitoromegaly (an enlarged clitoris);
- Partial labial fusion;
- Apparently undescended testes (which may turn out to be ovaries) in boys;
- Labial or inguinal (groin) masses which could turn out to be testes in girls;
- Hypospadias (the opening of the penis is somewhere other than at the tip; in females, the urethra opens into the vagina);
- Electrolyte abnormalities.
Diagnosis of hermaphroditism is not complicated. Simple medical observation of genitalia could be used. Usually doctors could see ambiguous and/or unusual genitalia at birth and later at puberty.
The following medical tests are recommended for clients with hermaphroditism:
- Chromosome analysis;
- Hormonal analysis – mainly sex hormones;
- Hormone stimulation tests;
- A analysis of electrolytes;
- Specific molecular testing;
- Endoscopic examination (to verify the absence or presence of internal sex organs – uterus, ovaries, fallopian tubes);
- Ultrasound or MRI (to verify the absence or presence of internal sex organs – uterus, ovaries, fallopian tubes).
HERMAPHRODITISM – solutions
Parents and medical professionals actually are responsible for choosing the “official gender” of the client with hermaphroditism. Chromosomal, neural, hormonal, psychological and behavioral factors can all influence gender identity. Some medical professionals prefer to make final decision with participation of clients at puberty because some other factors could be more important in gender satisfaction than functioning external genitals.
All decisions could be made according to all specific symptoms, feelings and social factors.
If you notice that your child has unusual genitalia or sexual development, discuss this with your health care provider.
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