PRIMARY AMENORRHOEA

In this post today we will talk about Amenorrhoea(AMENO), this can be Primary or Secondary.
Primary Amenorrhoea a women has never had a period, but in secondary Amenorrhoea a period did start and then stopped.
(The word AMENORRHOEA is derived from the Greek A meaning NO and Men means month and Rhoea means flow.)

Today we will only discuss about Primary Amenorrhoea as the two topics together make a very long post.
To have normal periods you need a normal brain hypothalamus pituitary function as described in the menstrual cycle, and in addition to this you need a normal functioning ovary and a normal uterus, cervix and vagina. In addition to this there hormones should be co-ordinated and beside the ovary some other hormones also participate , such as the thyroid and general health also plays a role.
The abnormalities often start during foetal development due to chromosome and genetic defects.
The management of this problem should start at birth especially if the sex of the new born is ambiguous. These problems can be classified in various groups but I will just talk about random abnormalities. The domain of the treatment of these complicated abnormalities is in the hands of paediatric endocrinologists.

One of the interesting and not so rare conditions is called Androgen Insensitivity Syndrome is when a foetus who is genetically male(who has one X and Y chromosome) is resistant to male hormones(called Androgens). As a result the foetus develops the features of a female baby, and when this child grows up it looks like a very attractive female. She is tall, slim, attractive with normal breast development, flawless skin, however the female organs are not there except for a small depressed area for the vagina. These are called complete Androgen Insensitivity. Some of these may be incomplete and then they have features of both sexes. The testes often lie in the lower abdominal area , if that is the case they should be removed as they are prone to cancer. This individual always grows as a female and the diagnosis is made when they have no period(Primary Amenorrhoea). The diagnosis is confirmed by blood tests, and genetic testing which will be XY and ultrasound which will show no female organs. these women are treated by hormone replacement therapy , psychological counselling and removing of small testicles as already mentioned.

Most of these individuals suffering from AIS live as women and become involved in sports, modelling and working in the hospitality industry. In fact some of these women in these trades became very famous, and in fact until very recently if there was any doubt about there femineity there chromosomes were tested and if found to be XY they were removed from these sports, but since then it has been worked out that these androgens in these women do not work like normal androgens hence the name AIS, so this rule of ruling out AIS women with XY chromosomes from the sports has been removed.
In this day and age where we have the access and knowledge to all these tests that can be done, we should test a child when it is born should the genitalia be inconclusive.

One of the other situations that you may come across is a young girl who is between 14 and 16 years of age, has normal secondary sexual features but has not had a period, she should be investigated, they can often have pre menstrual symptoms, regular menstrual period pain but no period.
They often have an obstructive abnormality of the genital tract, meaning thereby their cervix, vagina and the hymen. They have a period but the blood cannot flow. If not released it gathers within the vagina, the uterus and the tubes whereby destroying them. The hymen abnormality is easily fixed by a cross incision on the area. If there is a vaginal septum it can be removed, the cervical stenosis is difficult to correct. If the uterus is absent and the ovaries are present the women do suffer pre menstrual symptoms but generally they do not have any complications, these days these women can have a surrogate pregnancy by using their egg and their partners sperm. If there were any problems with the vagina causing difficulty during sex it can be constructed.

We will continue this conversation in the next post.