Surgery for Congenital Adrenal Hyperplasia

Exposure in the womb to excess amounts of male sex hormones, called androgens, can affect the development of a girl’s external genitals, giving them an ambiguous or masculine appearance. Pediatric surgeons at Hassenfeld Children’s Hospital at NYU Langone can perform procedures to change the appearance of the genitals.

Some girls with congenital adrenal hyperplasia are born with an enlarged clitoris, the erectile tissue at the front of the vulva that resembles a penis. In others, the labia, the tissue folds surrounding the opening of the vagina, are fused and appear more like testes.

In some instances, the vagina and urethra—the tube through which urine is eliminated—open into a common channel, rather than separately. Girls whose vaginas are in this position may have an increased risk of urinary tract infections. Their internal reproductive organs, however, are not affected.

Our doctors can perform surgery for girls with anatomic differences in the external genitals. Altering the appearance of a girl’s genitals can have a positive impact on her self-confidence and psychological wellbeing.

Our doctors may suggest one or more surgical procedures, such as clitoroplasty, labiaplasty, and vaginoplasty, depending on your child’s anatomy. These procedures can be performed at different stages of development, including infancy, early childhood, or preadolescence, or at the same time, depending on the complexity of the condition. Surgery is often begun when an infant is between three and six months old.

Before performing surgery, our doctors may use a procedure called cystoscopy to obtain a better view of the position of the vagina. After your child has been given general anesthesia, the doctor inserts a cystoscope—a thin, hollow tube with a camera and light at its tip—into the urethra. The camera projects images onto a computer monitor, allowing the doctor to determine where the vagina enters the urethra and what’s the best surgical approach.

Surgery does not cure congenital adrenal hyperplasia. Children who have these procedures must continue to take medication to replace missing hormones and salt.

Parents considering surgery are encouraged to discuss all of these options with our team of pediatric endocrinologists, surgeons, urologists, and psychologists so you can decide the best course of treatment for your child.