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The overall management strategy for patients with CAH is more clear cut than treatment of other intersex conditions, because research conclusively shows that those born with internal female reproductive structures have the long-term potential to function reasonably as females from a sexual and fertility standpoint.
The first goal of treatment for CAH is to return the baby's androgen levels to normal. The newborn will immediately be put on the proper hormonal replacement, including a cortisone like medication called glucocorticoid, to make-up for cortisone deficiencies. For patients who are salt-wasters, flurdocortisone is also administered. Lifelong hormone replacement is required.
Once hormonal therapies are in place, a plan to correct any significant ambiguity of the genitalia by reconstructive surgery (feminizing genitoplasty) is made in consultation with the baby's parents.
Doctors at Children's Hospital prefer to do the reconstructive surgery between the ages of 3-6 months after the baby has had the chance to stablize medically and the risks of anesthesia are reduced. This waiting period also allows the clitoris to reduce in size through steroid management alone.
The surgery involves the exterorization of the vagina, which can be located at various depths along the genitourinary tract and reduction in the size of the clitoris. (clitoroplasty)
The prognosis for these patients is good, as long as patients remain on hormone replacement therapy. Over the long-term patients are monitored regularly by a pediatric endocrinologist and pediatric urologist.
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