3. What are the goals of endocrine treatment for intersex patients ?

For patients raised as males, the goals of endocrine treatment are to encourage masculine development, and correspondingly suppress feminine development, of sexual characteristics. For example, increased penile size, hair distribution and body mass can be accomplished for some individuals through the use of testosterone treatment.

For patients raised as females, the goals of treatment are to simultaneously encourage feminine development and discourage masculine development of sexual characteristics. For example, breast development and menstruation can occur for some individuals following estrogen treatment.

In addition to sex hormones, patients with Congenital Adrenal Hyperplasia may also take glucocorticoids and salt-retaining hormones. Glucocorticoids can help these patients maintain appropriate reactions to physical stress as well as suppress unwanted masculine sexual development in female patients.

4. How long do patients need to take their hormone treatments?

Sex hormone therapy is usually initiated at puberty and glucocorticoids are administered when appropriate much earlier, usually at the time of diagnosis. Whether patients take male hormones, female hormones or glucocorticoids, it is important to continue with these medications throughout life. For example, male hormones are needed in adulthood to maintain masculine sexual characteristics, female hormones to protect against osteoporosis and cardiovascular disease, and glucocorticoids to protect against hypoglycemia and stress-related illnesses.

Surgical Treatment

1. What is the goal of reconstructive female genital surgery?

The goal of reconstructive female genital surgery is to have external feminine genitalia which look as normal as possible and will be correct for sexual function. The first step is to reduce the size of the markedly enlarged clitoris while preserving the nerve supply to the clitoris, and to place it in the normal female hidden position. The second step is to exteriorize the vagina so that it comes to the outside of the body in the area just below the clitoris.

The first step is usually more appropriate early in life. The second step is probably more successful when the patient is ready to start her sex life.

2. What are the goals of reconstructive male genital surgery?

The major goals are to straighten the penis, and to move the urethra from wherever it lies to the tip of the penis. This can be done in one step. However, in many cases, it will take more than one step particularly if the amount of available skin is limited, the curvature of the penis is marked, and the overall condition is severe.

3. What are the pros and cons of early surgery vs. late surgery in the male sex of rearing?

As far as male sex of rearing is concerned, early surgery can be performed easily between the ages of 6 months and 11/2 years. Generally speaking, it is better to try to obtain full correction of the genitalia before the child is two years of age, when he will be less aware of the problems related to surgery.

Late surgery in males would be defined after two years of age. Most male surgery should be performed early in life and should not be postponed until adolescence.

4. What are the pros and cons of early surgery vs. late surgery in the female sex of rearing?

As far as female sex of rearing is concerned, when the vaginal opening is easily reached and the clitoris is not markedly enlarged, exteriorization of the vagina without clitoral correction can be done early in life. If there is a great deal of masculinization with a markedly enlarged clitoris and an almost closed vagina (or a vagina located high and very posterior), then it is often advised to postpone exteriorization of the vagina until adolescence.

There are two distinct schools of thought in reconstructive surgery today concerning bringing the vagina down to the normal female position. Some people recommend that this all be done in infancy so that the entire reconstruction is complete by two years of age, accepting that mild complications may occur later in life. Others think that the surgery should be postponed until puberty, until the girl is under the influence of estrogen and the vagina can be brought down more easily when the young woman is ready to begin her sex life.

5. What are the complications associated with each type of procedure?

In male reconstructive surgery the complications include failure to get the penis straight, resulting in continued bending of the penis. Another complication would be a fistula or leak in the reconstructed male urethra. Neither one of these are severe complications presently and can be repaired without a great deal of difficulty. However, successful reconstruction does not result in a fully normal penis, as a rebuilt urethra is not surrounded by normal spongious tissue (corpus), nor does surgery correct the size of the penis.

In female reconstructive surgery, complications depend on the location of the vagina. One complication that can occur is that scar tissue forms where the vagina exits the inside of the body and causes stenosis or narrowing of the entrance to the vagina. With a high vagina, which is up near the bladder neck in the urinary control area (sphincter), the urinary control mechanism could be damaged and the child could become incontinent of urine as a result. This is why surgery should be performed by a surgeon who is experienced in dealing with birth defects of this magnitude. On occasion, it is necessary to reconstruct a neo-vagina. In such cases, the neo-vagina is normally functional but it may not look like normal female genitalia.