Intersex Surgery - Masculinizing Surgical Procedures

Masculinizing Surgical Procedures

Orchiopexy and hypospadias repair are the most common types of genital corrective surgery performed in infant boys, but most of these boys have no other abnormalities and are not considered to have an intersex condition. Undervirilized boys typically have at least one of these two procedures performed in addition to others described below.

Types of undervirization and malformation for which some type of masculinizing surgery has been performed most often in the last 50 years are:

• the configuration of ambiguous genitalia referred to as pseudovaginal perineoscrotal hypospadias (PPSH)
  • the Reifenstein type of partial androgen insensitivity syndrome
  • gonadal dysgenesis disorders such as mixed gonadal dysgenesis and testicular dysgenesis
  • idiopathic (specific cause not determined)
• birth defects of male genitalia
  • bladder exstrophy and epispadias spectrum
  • chordee (simple or as part of undervirilization)
  • micropenis
  • concealed penis

The disorders above comprised over 90% of reported surgical series from North America and Europe. In a few parts of the world 5-alpha-reductase deficiency or defects of testosterone synthesis, or even rarer forms of intersex account for a significant portion of cases but these are rare in North America and Europe. Masculinizing surgery for completely virilized genetic females with CAH is even rarer.

Orchiopexy for repair of undescended testes (cryptorchidism) is the second most common surgery performed on infant male genitalia (after circumcision). The surgeon moves one or both testes, with blood vessels, from an abdominal or inguinal position to the scrotum. If the inguinal canal is open it must be closed to prevent hernia.

Potential problems: Maintaining the blood supply is the major challenge. If vessels cannot be stretched into the scrotum, or are separated and cannot be reconnected, a testis will die and atrophy.

Hypospadias repair is a relatively simple single procedure if the hypospadias is first or second degree (urethral opening on glans or shaft respectively) and the penis is otherwise normal. Repair of a third degree hypospadias (urethral opening on perineum or in urogenital opening) is more challenging, may be done in stages, and has a significant rate of complications and unsatisfactory outcomes. (Glassberg, 1999)

Potential problems: For severe hypospadias (3rd degree, on perineum) constructing a urethral tube the length of the phallus is not always successful, leaving an opening (a "fistula") proximal to the intended urethral opening. Sometimes a second operation is successful, but some boys and men have been left with chronic problems with fistulas, scarring and contractures that make urination or erections uncomfortable.

Urogenital closure closure of any midline opening at the base of the penis. In severe undervirilization a boy may have a "pseudovaginal pouch" or a single urogenital opening in the midline of the perineum.

Potential problems: The most complicated aspect of closure involves moving the urethra to the phallus if it is not already there (i.e., repairing a perineal hypospadias). Fistulas and scarring are the main risks.

Gonadectomy (also referred to as "orchiectomy") removal of the gonads. This is done in three circumstances. (1) If the gonads are dysgenetic testes or streak gonads and at least some of the boy's 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. (2) In rare instances when an XX child has completely virilizing congenital adrenal hyperplasia (Prader stage 5), the ovaries can be removed before puberty to stop breast development and/or menstruation. (3) Gonadectomy can be performed in the equally rare instance of a child with true hermaphrodite virilized enough to raise as male, in which ovaries or ovotestes can be removed.

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

Chordee release is the cutting of ventral penile skin and connective tissue to free and straighten the penis. A mild chordee, manifest as a well-formed penis "bent" downward by subcutaneous connective tissue, may be an isolated birth defect easily repaired by releasing some of the inelastic connective tissue on the ventral side of the shaft. In a complete chordee the phallus is "tethered" downward to the perineum by skin. A more severe chordee is often accompanied by a hypospadias and sometimes by severe undervirilization: a perineal "pseudovaginal pouch" and bifid ("split") scrotum with an undersized penis. This combination, referred to as pseudovaginal perineoscrotal hypospadias, is in the spectrum of ambiguous genitalia due to a number of conditions.

Potential problems: Scarring and contracture are occasional complications, but most unsatisfactory outcomes occur when a severe hypospadias needs to be repaired as well. Long-term complications can include fistulas between colon or upper rectum and skin or other cavities, or between urethra and perineum.

Cloacal repair is among the most complex of the surgeries described here. Bladder exstrophy or more severe cloacal exstrophy is a major birth defect involving inadequate closure and incomplete midline fusion of multiple pelvic and perineal organs as well as the front of the pelvis and lower abdominal wall. The penis and scrotum are often widely bifid (the two embryonic parts unjoined). The penis often cannot be salvaged, although the testes can be retained. Repair may involve closure of the bladder, closure of the anterior abdominal wall, colostomy (temporary or permanent) with reconstruction of the rectum. If the halves of the phallus cannot be joined, they may be removed. The smallest defect in this spectrum is an epispadias. Surgical repair for this is primarily a phalloplasty. (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. In many cases a functional penis cannot be created. Scarring is often extensive and the lower torso severely disfigured even with fairly good outcomes.

Phalloplasty is a general term for any reconstruction of the penis itself, especially for more unusual types of injuries, deformities, or birth defects. The principal difficulty is that erectile tissue is not easily constructed and this limits the surgeon's ability to make more than minor size changes. Construction of a narrow tube lined with mucosa (a urethra) is a similar challenge.

Potential problems: Minor revisions of the skin are rarely followed by problems. More complicated reconstruction may result in scarring and contracture, which can distort the shape or curvature of the penis, or interfere with erections or make them painful.

Hysterectomy is removal of a uterus. It is rare that a uterus or mullerian duct derivatives would need to be removed from a child being raised as a boy. The most common scenario is accidental discovery of persistent mullerian derivatives or a small uterus during abdominal surgery of a normal boy for cryptorchidism, appendectomy, or bowel disease. Removal would not involve genital surgery. A rarer indication would be that of a completely virilized XX child with congenital adrenal hyperplasia (Prader stage 5) being raised as a male; ovaries and uterus must be removed to prevent breast development and menstruation by early adolescence.

Potential problems: Risks are simply those of abdominal surgery.

Testicular prostheses are saline-filled plastic ovoids implanted in the scrotum. They have no function except to provide the appearance and feel of testes. Several sizes are available, but most are implanted in adolescence to avoid repeated procedures to implant larger sizes at puberty. Prostheses made of silastic are no longer available due to safety and perception-of-safety concerns.

Potential problems: Foreign body reactions, rarely with infection or erosion of scrotal skin, are minimal but constitute the most significant complication.

Penile augmentation surgery is surgery intended to enlarge a small penis. Early attempts in the 1950s and 1960s involved constructing a tube of non-erectile flesh extending a small penis but the penis did not function. In recent years a small number of urologists have been offering an augmenation procedure that involves moving outward some of the buried components of the corpora so that the penis protrudes more. The girth is augmented with transplantation of the patient's fat. This procedure is designed to preserve erectile and sexual function without surgically altering the urethra. This type of surgery is not performed on children and primarily produces a small increase in the size of a normal penis, but would be less likely to produce a major functional change in a severe micropenis.

Potential problems: Reabsorption of the fat is common. Scarring resulting in interference with erectile function is less likely but more damaging.

Concealed penis a normal penis buried in suprapubic fat. In most cases, when the fat is depressed with the fingers, the penis is seen to be of normal size. This is common in overweight boys before the penile growth of puberty. Surgical techniques have been devised to improve it. (Casale, 1999)

Potential problems: The most common difficulty is recurrence with further weight gain. Scarring can occur.