Intersex Surgery - Feminizing Surgical Procedures

Feminizing Surgical Procedures

In the last 50 years, the following procedures were most commonly performed for the following intersex conditions and birth defects in order to make the genitalia more like those of normal females:

• virilization due to congenital adrenal hyperplasia
• malformations of genitalia in genetic females
  • urogenital sinus malformation
  • cloacal exstrophy
• conditions involving severe undervirilization or malformations of genetic males, or infants with mixed genetic sex, to be assigned and raised as girls
  • gonadal dysgenesis (various forms)
  • partial and complete androgen insensitivity syndrome
  • micropenis
  • cloacal and bladder exstrophy

There are rarer causes of virilization of genetic females or undervirililizaton of genetic males (see intersex for a fuller list), as well as some less easily categorized types of intersex conditions or other malformations of the genitalia. In the last decade, feminizing surgery to support reassignment of genetic males with non-ambiguous micropenis has been largely discontinued, and surgical reassignment of genetic males with exstrophy or other severe malformations or injuries is diminishing. See history of intersex surgery.

Clitorectomy amputation or removal of most of the clitoris, including glans, erectile tissue, and nerves. This procedure was the most common clitoral surgery performed prior to 1970, but was largely abandoned by 1980 because it usually resulted in loss of clitoral sensation.

Potential problems: The primary effect of this surgery, not surprisingly, is a drastic reduction in ability to experience orgasm. The appearance is not very normal. Regrowth of unwanted erectile tissue has sometimes presented problems.

Clitoroplasty, like phalloplasty, is a term that encompasses any surgical reconstruction of the clitoris, such as removal of the corpora. Clitoral recession and reduction can both be referred to as clitoroplasty.

Potential problems: Major complications can include scarring, contractures, loss of sensation, loss of capacity for orgasm, and unsatisfactory appearance.

Clitoral recession involves the repositioning of the erectile body and glans of the clitoris farther back under the symphysis pubis and/or skin of the preputium and mons. This was commonly done from the 1970s through the 1980s to reduce protrusion without sacrificing sensation. Outcomes were often unsatisfactory, and it fell into disfavor in the last 15 years. (Rangecroft, 2001)

Potential problems:Unfortunately the subsequent sensations were not always pleasant, and erection could be painful. Adults who had a clitoral recession in early childhood often report reduced capacity for enjoyment of sexual intercourse, though similar women who had not had surgery also report a high rate of sexual dysfunction. (Minto, 2003)

Clitoral reduction was developed in the 1980s to reduce size without reducing function. Lateral wedges of the erectile tissue of the clitoris are removed to reduce the size and protrusion. The neurovascular tissue is carefully spared to preserve function and sensation. Nerve stimulation and sensory responses are now often performed during the surgery to confirm function of the sensory nerves. (Chase, 1996; Rangecroft, 2001))

Potential problems: The degree to which the goal of preserving sexual sensations is attained is a subject of controversy. Many of the children who have had the newer versions of this procedure have not yet become adults.

Vaginoplasty, the construction or reconstruction of a vagina, can be fairly simple or quite complex, depending on the initial anatomy. If a normal internal uterus, cervix and upper vagina (the mullerian derivatives) exist, and the outer virilization is modest, surgery involves separating the fused labia and widening the vaginal introitus. With greater degrees of virilization, the major challenge of the procedure is to provide a passage connecting the outer vaginal opening to the cervix which will stay wide enough to allow coitus. XY girls or women with partial androgen insensitivity syndrome will have a blind vaginal pouch of varying degrees of depth. Sometimes this can be dilated to a usable depth. Sometimes surgery is performed to deepen it.

The most challenging surgery with the highest complication rate is construction of an entirely new vagina (a "neovagina"). The most common instance of this is when a child will be assigned and raised as a female despite complete virilization, as with Prader 5 CAH, or (in the past) when a genetic male infant with a severely defective penis was reassigned as a female. One method is to use a segment of colon, which provides a lubricated mucosal surface as a substitute for the vaginal mucosa. Another is to line the new vagina with a skin graft. (Creighton, 2001; Rink, 1998; Schnitzer, 2001)

Potential problems: Stenosis (narrowing) of the constructed vagina is the most common long-term complication and the chief reason that a revision may be required when a girl is older. When a neovagina is made from a segment of bowel, it tends to leak mucus; when made with a skin graft, lubrication is necessary. Less common complications include fistulas, uncomfortable scarring, and problems with urinary continence. (Alizai, 1999; Lobe, 1987; Minto, 2003)

Gonadectomy is removal of the gonads. If the gonads are dysgenetic testes or streak gonads and at least some of the cells have a Y chromosome, the gonads or streaks must be removed because they are nonfunctional but have a relatively high risk of developing gonadoblastoma. If the gonads are relatively "normal" testes, but the child is to be assigned and raised as female, (e.g., for intersex conditions with severe undervirilization, or major malformations involving an absent or unsalvageable penis) they must be removed before puberty to prevent virilization from rising testosterone. Testes in androgen insensitivity are a special case: if there is any degree of responsiveness to testosterone, they should be removed before puberty. On the other hand, if androgen insensitivity is complete, the testes may be left to produce estradiol (via testosterone) to induce breast development, but there is a slowly increasing risk of cancer in adult life. Streak gonads without a Y chromosome cell line need not be removed but will not function. Finally, the gonads in true hermaphroditism must be directly examined; abnormal gonads with Y line or potential testicular function should be removed but in rare instances a surgeon may try to preserve the ovarian part of an ovotestis. (Manuel, 1976)

Potential problems: Gonadectomy involves little risk beyond that of any abdominal surgery.

Cloacal exstrophy and bladder exstrophy repair is needed regardless of the sex of assignment or rearing. Simple bladder exstrophy in a genetic female does not usually involve the vagina. Cloacal exstrophy in a genetic female usually requires major surgical reconstruction of the entire perineum, including bladder, clitoris, symphysis pubis, and both the vaginal introitus and urethra. However, the uterus and ovaries are normally formed. Severe bladder exstrophy or cloacal exstrophy in genetic males often renders the phallus widely split, small, and unsalvageable. The scrotum is also widely split, though testes themselves are usually normal. From the 1960s until the last decade, many of these infants were assigned and raised as females, with fashioning of a vagina and gonadectomy as part of the perineal reconstruction. (Schober, 2002)

Potential problems:Surgery for the more severe degrees of cloacal exstrophy is extensive and usually multistage. A variety of potential problems and complications can occur, including need for long-term colostomy or vesicostomy. Creating a functional urethra is difficult and poor healing, with scarring, stricture, or fistula can require a vesicostomy to prevent urinary incontinence. Construction of a functional anal sphincter can be equally difficult when this has been disrupted as well. Functional problems can warrant a temporary or long-term colostomy. The added challenge for the most severely affected genetic females, and for genetic males who are being raised as females, is construction of a neovagina. Scarring is extensive and the lower torso disfigured even with the best outcomes. Finally, it has become apparent in recent years that some genetic males (without intersex conditions) who are reassigned and raised as females have not developed a female gender identity and have sought reassignment back to male. (Reiner, 2004)