Intersex

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Female reproductive anatomy

Intersex is a group of conditions where there is a discrepancy between the external genitals and the internal genitals (the testes and ovaries).

The older term for this condition, hermaphroditism, came from joining the names of a Greek god and goddess, Hermes and Aphrodite. Hermes was a god of male sexuality (among other things) and Aphrodite a goddess of female sexuality, love, and beauty.

Although the older terms are still included in this article for reference, they have been replaced by most experts (and patients and families) because they are misleading, confusing, and insensitive. Increasingly this group of conditions is being called disorders of sex development (DSDs).

Disorders of sex development; DSDs; Pseudohermaphroditism; Hermaphroditism; Hermaphrodite

Intersex can be divided into four categories:

• 46, XX Intersex
• 46, XY Intersex
• True Gonadal Intersex
• Complex or Undetermined Intersex

Each one is discussed in more detail below. NOTE: In many kids the cause of intersex may remain undetermined, even with modern diagnostic techniques.

46, XX Intersex . The person has the chromosomes of a woman, the ovaries of a woman, but external (outside) genitals that appear male. This usually is the result of a female fetus having been exposed to excess male hormones before birth. The labia ("lips" or folds of skin of the external female genitals) fuse, and the clitoris enlarges to appear like a penis. Usually this person has a normal uterus and Fallopian tubes. This condition is also called 46, XX with virilization . It used to be called female pseudohermaphroditism. There are several possible causes:

• Congenital adrenal hyperplasia (the most common cause).
• Male hormones (such as testosterone) taken or encountered by the mother during pregnancy.
• Male hormone-producing tumors in the mother. These are most often ovarian tumors. Mothers who have children with 46, XX intersex should be checked unless there is another clear cause.
• Aromatase deficiency. This one may not be noticeable until puberty. Aromatase is an enzyme that normally converts male hormones to female hormones. Too much aromatase activity can lead to excess estrogen (female hormone); too little to 46, XX intersex. At puberty these XX children, who had been raised as girls, may begin to take on male characteristics.