Intersex States

Intersex states are conditions in which a newborn's sex organs (genitals) look unusual, making it impossible to identify the sex of the baby from their outward appearance.

Description

All developing babies start out with external sex organs that look female. If the baby is male, the internal sex organs mature and begin to produce the male hormone testosterone. If the hormones reach the tissues correctly, the external genitals change into the scrotum and penis. Sometimes, the genetic sex (as indicated by chromosomes) may not match the appearance of the external sex organs. About one in every 2,000 births results in a baby whose sex organs are ambiguous.

Patients with intersex states can be classified as a true hermaphrodite, a female pseudohermaphrodite, or a male pseudohermaphrodite. This is determined by examining the internal and external structures of the child.

A true hermaphrodite is born with both ovaries and testicles. They also have mixed male and female external genitals. This condition is extremely rare.

A female pseudohermaphrodite is a genetic female. However, the external sex organs have been masculinized and look like a penis. This may occur if the mother takes the hormone progesterone to prevent a miscarriage, but more often it is caused by an overproduction of certain hormones.

This infant was born with female and male genitalia. (Photography by Mike Peres, Custom Medical Stock Photo. Reproduced by permission.)

A male pseudohermaphrodite is a genetic male. However, the external sex organs fail to develop normally. Intersex males may have testes and a female-like vulva, or a very small penis.

Causes and symptoms

Any abnormality in chromosomes or sex hormones, or in the unborn baby's response to the hormones, can lead to an intersex state in a newborn.

Intersex states may also be caused by a condition called congenital adrenal hyperplasia, which occurs in about one out of every 5,000 newborns. This disease blocks the baby's metabolism and can cause a range of symptoms, including abnormal genitals.

Diagnosis

When doctors are uncertain about a newborn's sex, a specialist in infant hormonal problems is consulted as soon as possible. Ultrasound can locate a uterus behind the bladder and can determine if there is a cervix or uterine canal. Blood tests can check the levels of sex hormones in the baby's blood, and chromosome analysis (called karyotyping) can determine sex. Exploratory surgery or a biopsy of reproductive tissue may be necessary. Only after thorough testing can a correct diagnosis and determination of sex be made.

Treatment

Treatment of intersex states is controversial. Traditional treatment assigns sex according to test results; the potential for the child to identify with a sex; and the ease of genital surgery to make the organs look more normal. Treatment may then include reconstructive surgery followed by hormone therapy. Babies born with congenital adrenal hyperplasia can be treated with cortisone-type drugs and sometimes surgery.

Counseling should be given to the entire family of an intersex newborn. Families should explore all available medical and surgical options. Counseling should also be provided to the child when he or she is old enough.

Prognosis

Since the mid-1950s, doctors have typically assigned a sex to an intersex infant based on how easy reconstructive surgery would be. The American Academy of Pediatrics states that children with these types of genitals can be raised successfully as members of either sex, and recommends surgery within the first 15 months of life.

Some people are critical of this approach, including intersex adults who were operated on as children. The remolded genitals do not function sexually and can be the source of lifelong pain. They suggest that surgery be delayed until the patient can make informed choices about surgery and intervention.