Syndromes of Abnormal Sex Differentiaton
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.
6. On average, how many surgeries are needed to obtain a desirable
cosmetic and functional result?
In males, this depends on the location of the urethra, the amount of
available skin and the degree of bending of the penis. In favorable cases, the
maximum number of operations can be two or three.
In females with a low vagina and a slightly enlarged clitoris, usually one
operation is performed in infancy, followed often by a "touch up"
operation in adolescence. In females with a high vagina, surgery in infancy
feminizes the external genitalia, with subsequent surgery to bring down the
vagina in late childhood or early adolescence, depending on the preference of
the patient.
7. What is required for post-surgical maintenance in females?
We usually do not advise vaginal dilation in our young patients because we
think this is stressful, both on parents and children. However, dilation may be
needed in post-pubertal women. We do accept the fact that some patients may
need touch up surgery when they are older.
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