WOMEN: FEMINISATION OF MALE GENITALS. IMPERFORATE HYMEN
Feminization of male genitals
‘Testicular feminization’ sounds like a contradiction, and in a way it is. It is a rare inherited enzyme deficiency in males that prevents body tissues from responding to male hormones. Those affected have normal male chromosomes (XY) and -because the Y determines testicular development – normal testes (though often within the abdomen or in other abnormal sites) producing normal amounts of androgens and MIH. But because tissues can’t be influenced by androgens, the external genitals develop in the female pattern. However, because Müllerian tissue can respond to MIH, there are no tubes, uterus or upper vagina. When the genitals are inspected at birth, female sex is generally wrongly assigned. What’s more, because the testis produces oestrogen as well as androgens, bale secondary sexual characteristics (breast enlargement, widening of hips, female body hair pattern and female voice) develop at puberty. People with testicular feminization are usually raised as females and the problem may not be suspected until there is failure to menstruate during puberty. The diagnosis is confirmed by the buccal smear test and finding the testes (which are usually removed because they can become malignant if left within the abdomen). Because no treatment is possible, they usually continue their adult lives as females. After the testes are removed, oestrogen replacement therapy must be continued until at least 50 years of age. A vagina can be created to allow sexual intercourse.
I’ve described these unusual congenital conditions as examples of how sex may be wrongly assigned at birth. Errors in sex identification in infancy can have devastating consequences in later life. Unfortunately, most errors aren’t discovered until puberty or later, when gender identification and attitudes to sex are usually established beyond correction. It is always extremely disturbing for a young person to learn that they have been brought up as the wrong sex. Expert initial and follow-up counselling are always needed.
Imperforate hymen
This is an unusual condition in which the hymen has no opening to allow the escape of menstrual blood, which becomes dammed up in the vagina and uterus. Most young women with this problem will have developed normally and may have had regular symptoms of menstruation but no bleeding. Sometimes the distended uterus can be felt above the pubic bone. Imperforate hymen is diagnosed by examining the introitus (entrance to the vagina), and simply treated by making an opening in the hymen. The retained menstrual fluid drains and future menstruation proceeds normally.
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March 12, 2009 - 8:32 AM