Hypospadias is a congenital disorder wherein the urethral meatus fails to open at the tip of the glans penis and may appear anywhere between the ventral glans and the perineum.

In general, hypospadias repair requires refined surgical and plastic surgical skills. Generally, these patients are best handled by those pediatric urologists experienced in this field of reconstructive surgery.

A boy with any degree of hypospadias should never undergo circumcision, so that the preputial skin is preserved for possible use in the repair.

The incidence of hypospadias is approximately 1 in 300 live male births, and the majority of these defects are in the distal shaft of the penis. Hypospadias results from incomplete development of the embryonic urethral tube to the distal glans.

In most families, hypospadias is an isolated event. However, in some families, males are genetically predisposed to having hypospadias. This condition may be more common in boys who were conceived through in vitro fertilization.

FINDINGS

The degree of hypospadiac severity is best classified by the position of the urethral meatus into distal, middle and proximal. Distal defects usually include glanular, coronal and subcoronal hypospadias. Middle and proximal hypospadiac defects are often associated with significant chordee (bending on erection). Torsion, or twisting of the penis, usually to the left, is common.

INDICATIONS FOR REPAIR

A distal hypospadias with a prominent dorsal preputial hood can be an obvious cosmetic defect, and we have seen older children in the office seeking repair because of "locker room" problem. However, many of these distal hypospadiacs are also affected by downward spraying of the urinary stream because of the anatomy of the native meatus.

Middle and proximal hypospadias and sometimes distal hypospadias, when associated with chordee, obviously need repair to overcome future problems with sexual intromission

TIMING OF REPAIR

The optimal time to perform hypospadias repair is somewhere between 12 and 24 months of age, depending upon the severity of the problem. We have found no evidence to support concerns regarding psychological effects of genital surgery on these children because of current methods of handling children in the hospital, anesthesia, and parental "rooming in." Most distal repairs can be performed at around one year of age, although most of the complex proximal repairs are delayed until the child is closer to two years of age.

PATIENT EVALUATION

Most boys with hypospadias (except possibly for those with proximal disorders) do not have an associated urologic developmental problem, and therefore routine renal ultrasonography is not indicated or cost effective.

Patients with undescended testes, partial penoscrotal transposition, penoscrotal hypospadias or utricular abnormalities should be carefully evaluated to uncover a possible intersex problem. Appropriate investigations with the assistance of pediatric endocrinology would then include a buccal smear and karyotype, serum follicle/stimulating hormone, luteinizing hormone and testosterone, urinary 17 hydroxysteroids, 17 ketosteroids and possibly laparoscopy. A genital skin biopsy to evaluate 5 alpha reductase and androgen receptor content may be valuable in some patients.

PREOPERATIVE SUPPORT

It is important for physicians and parents to be forthright and supportive.

Contrary to popular belief, genital reconstructive surgery, including circumcision, is not inordinately painful, particularly when the children are given a penile anesthetic block immediately at the completion of surgery. Many of these children are up and about within hours of the surgery.

Some discomfort is felt, however, from bladder spasms because of the feeding tube urethral catheter used to stent the repair. This discomfort is usually controlled with Ditropan and/or Belladonna and Opium suppositories.

SURGICAL REPAIR

While multiple techniques have been described and are available for the repair of hypospadias, marked refinements in surgical techniques, the use of microsurgical instrumentation and fine suture material, and optical magnification have allowed the pediatric urologist to perform the vast majority of hypospadias repairs in a single stage.

In boys with hypospadias and chordee, chordee must be corrected before urethroplasty.

After correction of chordee, or in those boys with distal hypospadias that do not have chordee, the neo-urethra is completed to the tip of the penis, either through the use of existing redundant penile skin, usually as a flap, or with skin taken from another part of the body, as a graft. This neo-urethra is usually constructed about a small 5 or 8 French soft silicone stent/urethral catheter. The remainder of the repair is completed after the head of the penis is brought together on the underside over the distal end of the urethroplasty. Residual skin is removed and closed with fine sutures. A semi-occlusive plastic dressing is then placed about the whole penis and repair. The feeding tube catheter is secured by a stitch into the head of the penis to prevent the catheter from being extruded. The catheter simply drains into the diaper.

POSTOPERATIVE CARE

The majority of patients are admitted only for 23 hours and have their parents staying in the room. The patients may ambulate within hours of surgery as long as the repaired penis, with its indwelling stent/ catheter, is well immobilized within a tight-fitting diaper. The penis is taped upwards onto the abdomen.

Because any minimal injury can cause profound penile swelling and compromise healing, the placement of the semi-occlusive transparent penile dressing is important.

The penile anesthetic block, in addition to pain relief and bladder antispasmodics (Ditropan, Belladonna and Opium suppositories) make the child relatively comfortable.

Perioperative and postoperative antibiotics are used.

All the sutures used in the repair dissolve spontaneously. The only things requiring removal are the plastic dressing, which is removed in the office about five days after the procedure, and the feeding tube catheter secured by the glans traction suture, which is removed somewhere between 7 and 14 days after the procedure, depending upon the degree of hypospadias repair. Neither of these manipulations, including the catheter removal, is particularly painful.

During recuperation at home immediately after hospitalization, the parents are reminded to diaper the patient at all times, not to allow the patient on any riding toys, to sponge bathe the patient only (particularly while the dressing is still in place) and to continue a very liberal fluid intake.

At home, the patient may continue on oral antibiotics, Ditropan and oral Tylenol p.r.n.

The parents are also reassured that sometimes the plastic dressing may come off prematurely, that sometimes a little blood staining is seen in the urethral catheter or diaper (usually from bladder spasms) and that rarely some bleeding may be noticed at the tip of the glans about the neo-meatus and catheter. These problems are usually not important and are self-limiting. Rarely, the dressing can slip below the head of the penis, causing profound swelling of the end of the penis; and if this happens, the doctor should be seen as soon as possible. Very rarely, the catheter may be extruded; and this should also be brought to the attention of the doctor.

After removal of the dressing but while the feeding tube catheter is still in place, the patient may bathe. It does not matter that the feeding tube catheter lies underwater. Great care should be taken when drying the newly repaired penis, and it is preferable to let it dry within a diaper. We often have the mother also apply mercurochrome to the underside of the penis to help dry the wounds.

COMPLICATIONS

Complications occur because of several factors: the severity of the hypospadias, whether or not previous repairs have been attempted, periodic erections, and the effects of bladder spasms.

The most common complication is the development of a urethrocutaneous fistula causing urinary leakage through the wound. This may be solitary and pinpoint to large and/or multiple.

Repair of these fistulas is not to be minimized and requires careful timing and many of the principles already outlined.

Other complications include urethral strictures (scars) and meatal stenosis. These complications are most common in those requiring extensive repair because of proximal hypospadias. A stricture should be strongly suspected in any child who develops a urinary tract infection after hypospadias repair and in any patient seen to strain during urination. Some fistulas also develop proximal to a urethral stricture.

Urethral strictures may be repaired through endoscopic incision and catheterization but often require opening through an external urethrostomy with outpatient closure several months later.

Hypospadias repairs are not only of great functional importance to allow normal urination and later intercourse, but also normalize the appearance of the penis and attempt to prevent "locker room problems".