Hypospadias

Hypospadias is a birth defect found in boys in which the urinary tract opening is not at the tip of the penis. Bending of the penis on erection may be associated with this and is known as chordee. Hypospadias occurs in about 8 of 1000 male births. There is some family risk of hypospadias. When we see a boy with hypospadias there is a 20% chance of finding it in another family member.

There are different degrees of hypospadias -- some minor and others more severe. We name the types of hypospadias according to their anatomic location, but one must always determine whether or not there is associated chordee.

Reasons For Repair:

Surgical correction of hypospadias involves straightening of any chordee and then extension of the urinary tube (urethra) out to the tip of the penis (glans).

The ability to stand and urinate is important for boys. When the urethra opens before it reaches the glans a boy may be unable to stand and urinate with a direct stream. The youngster who has to sit down to urinate on a toilet is at a painful social disadvantage.

A straight penis is necessary for satisfactory sexual function. Although this may not seem to be an important matter in childhood, this is a crucial concern later in life - early childhood is generally the best time for correction.

Embryology:

The penis begins to form around the fifth fetal week. The urethral folds start to unite over the urethral groove and by the fourteenth week the process is complete. A short ingrowth from the tip of the glans progresses inward to meet the urethral tube at the fossa navicularis. Formation of a normal circumferential foreskin (prepuce) certifies that the urethra has probably formed normally.

Surgical Correction Of Hypospadias And Chordee:

When the urethral opening is just slightly out of position and when there is no chordee, surgical correction may not be necessary, although in occasional patients there is a significant cosmetic reason for repair.

Mild to moderate grades of hypospadias with minimal chordee may be corrected by simple outpatient procedures such as Meatal Advancement and Glanduloplasty (MAGPI) or simple Flip-Flap procedures.

Moderate hypospadias with some chordee may require a more extensive operation such as a Mustarde procedure which utilizes a tubularized flap of penile shaft skin. Chordee is evaluated with an artificial erection in which the erectile bodies are inflated with a saline solution. To protect the newly constructed urethra (neourethra) the urine is usually diverted with a stent (a silastic tube through the neourethra). Patients may be sent home after surgery, but occasionally are hospitalized for a day or two. Depending on the operation, a tube may be left in the repair for 1 to 10 days.

More extensive hypospadias or chordee requires a bigger operation. In the past, two and three-stage operations were used. In the first stage the penis was straightened; this often made the hypospadias, in terms of urethral location, worse. Six months to a year later construction of the neourethra was initiated, using penile skin or free skin grafts. Our current preference is to try to do most of this in one stage with the Transverse Island Pedicle Tube Graft. This utilizes the inner portion of the foreskin, retaining its own blood supply. The remaining outer portion of foreskin resurfaces the front of the penis.

In some situations (such as perineal hypospadias, genital ambiguity, or significant hypospadias with previous circumcision) more extensive operations are necessary. The older multi-stage operations may be of occasional use. In some situations we will make a neourethra out of a graft of bladder lining or buccal mucosa.

Complications:

Bleeding is a risk of any operation. We keep this under control during hypospadias surgery with epinephrine and cauterization. Postoperatively, in the bigger repairs, we use a pressure dressing. Transfusion is almost unheard of in hypospadias operations.

Infection is another concern. We use antibiotics to minimize this risk.

Bladder spasms are usually due to indwelling catheters. It is ironic that these safety measures account for most postoperative discomfort. Medications help but do not eliminate spasms. Other catheter problems including kinking, which causes the bladder to fill and then leak urine around the stent or SP tube.

Fistula is another risk. This is a leak of urine from somewhere along the neourethra. This risk is minimal in the simple repairs, but significant in more extensive operations. Fistulas are usually easily fixed with an outpatient surgical procedure, although this is done no sooner than 6 months after the original operation.

When extensive use is made of the foreskin for a neourethra (as in the Transverse Island pedicle repairs) remaining foreskin may have marginal blood supply and will slough. This will look like a scab or eschar on the repaired penis and may result in fistula or skin tethering. Corrective skin adjustment may be desirable. This is usually an outpatient procedure and is also delayed at least six months. In some other patients there may be cosmetic reasons for minor late skin adjustments.

Stricture or stenosis consists of narrowing where the neourethra joins native urethra or at the level of the glans. These require dilation (stretching) or internal urethrotomy (a cut through a cystoscopy). Such measures may be repeatedly necessary as a stricture or stenosis tries to reform.

Recurrent chordee is a difficult, and fortunately uncommon, problem. Overall, the complications seen with the Transverse Island procedures involve less surgery and trouble than the former two and three-stage operations.

Chordee Without Hypospadias:

This is an unusual problem in which there is chordee (parents often bring the child in to see us because "the penis is bent") without apparent hypospadias. Skin tethering may be the main factor in some patients, and this is usually readily fixed. In some boys the distal urethra, even though intact, is paper thin (hypoplastic). Correction of significant chordee in this setting may involve creation of hypospadias to straighten the penis. The hypospadias must then be repaired in the same or a stage operation. In other instances the bent part of the erectile portions of the penis must be plicated.

Other Variations:

Penile torsion consists of a counterclockwise rotation of the glans penis. This is often seen in association with hypospadias and may be improved to varying degrees in the course of hypospadias repair. Penile torsion by itself is rarely of functional significance.

Dorsal preputial hood may be observed in newborns in whom hypospadias cannot be evaluated without forcible separation of foreskin from glans. We usually suggest leaving foreskin intact in these newborns and re-examination in 6 months. By this time there is generally enough separation that hypospadias can be evaluated, and the family may have some idea by then if there is chordee.

Webbed penis occurs when scrotum forms the ventral side of penile shaft.

A buried penis may be hidden by generous suprapubic fat pad.

A concealed penis occurs when preputitial scarring covers the glans.