Hypospadias and Chordee

Penile Torsion (Wandering Raphe) & Webbed Penis (Penoscrotal fusion)

Hypospadias
is a birth defect in boys in which the urethral opening is not located in a normal position. Hypospadias occurs with increased frequency in some families, but in general is not an inherited disorder.

Chordee is a bending of the penis associated with erection, and can occur alone or in combination with hypospadias.

There are different degrees of hypospadias - some minor and others more severe. For most boys, hypospadias is of functional importance and is not just a cosmetic defect. Surgical correction of hypospadias involves straightening the penis and then creating an extension of the urinary tube (urethra) to the tip of the penis with penile skin tissue. The ability to stand and urinate is important for boys. When the urethra opens before it reaches the tip of the penis, a boy may be unable to stand and urinate. The child who has to sit in order to urinate on a toilet is at a significant social disadvantage and may be exposed to teasing from his peers. A penis that is straight when erect 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 unite over the urethral groove. The urethral opening in boys and girls is initially in the same place. In boys, the urethra grows toward the tip of the penis. Formation of a normal circumferential foreskin is the last event after the urethral formation is complete. The exact reasons why hypospadias occurs is unclear and a precise etiology has not been identified.

Hypospadias does not in itself cause infertility. Infertility may be present in the more extreme forms of hypospadias, where there are associated testicular problems. A hypospadic penis is entirely capable of pleasurable sexual sensation and orgasm. Plastic surgery on the genitals normally does not damage erotic sensation.

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 required although many patients desire repair without regard to the severity of the abnormality.

Hypospadias surgery utilizes a tubularized flap of penile shaft skin to form a new segment of urethra. Penile skin is preferred because it will grow with the child and does not develop hair.

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 may be diverted with a stent (a silastic tube through the neourethra). Patients are usually sent home after surgery. Depending on the operation, a tube may be left in the repair for 3 to 10 days.

More severe types of hypospadias with chordee usually require a more extensive operation. In years past, two and three­ stage operations were used. In the first stage, the penis was straightened; this often made hypospadias, in terms of urethral location, worse. Six months to one year later, construction of the neourethra was initiated, using penile skin or skin grafts. Our current preference is to try to correct even these severe abnormalities in one planned operation, if possible. Obviously, the more complex the surgery, the greater the chance for complication. In some patients, a staged approach is still necessary.

Complications

 

  • Bleeding is a risk of any operation. Postoperatively, we use a pressure dressing. Transfusion is almost unheard of in hypospadias operations.

  • Infection is another concern. We use antibiotics in patients with a catheter to minimize the risk.

  • Bladder spasms are usually due to indwelling catheters. It is ironic that these safety measures account for most postoperative discomfort. Medications may help but may not eliminate spasms. If your child has a catheter following surgery, a medicine called Ditropan is usually prescribed to reduce spasms. It may be difficult to tell if your child is having spasms, but if he seems uncomfortable after trying Tylenol (with or without codeine), try a dose of Ditropan and if he seems better, use it as directed. If there is no relief, then do not use it.

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

Stricture or Stenosis
consists of a narrowing where the new urethra joins the native urethra or at the level of the urinary opening. These require dilation (stretching) or internal urethrotomy (a cut using a cystoscope). Such measures may be occasionally repeated as a stricture or stenosis tends to reform.

Recurrent chordee (bending of the penis)
is a difficult and fortunately uncommon problem. When it occurs an operation to straighten the penis may be necessary.

Diverticulum
is an out pouching of the neourethra and may appear as a ballooning of the urethra. These may cause infection and need surgical correction.

Chordee Without Hypospadias
This is a 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 and this is usually readily corrected. 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 second operation. In other instances, the bent part of the erectile portions of the penis must be plicated with permanent sutures in order to straighten the penis.

Other Variations of Chordee
Penile torsion (wandering raphe) consists of a counterclockwise rotation of the penis. It may be referred to as “wandering raphe” because the midline raphe of the penis may wrap around the penis in a counterclockwise fashion. When the raphe approaches 90 degrees there may be penile torsion underneath the foreskin. Penile torsion by itself is not usually corrected unless it approaches 90 degrees. Repair of penile torsion occurs during the circumcision procedure. Penile Torsion may also be seen in association with hypospadias and may be improved to varying degrees in the course of hypospadias repair.

Dorsal preputial hood
may be observed in newborns and should be considered a hypospadias/chordee variant. We usually suggest leaving the foreskin intact in these newborns and re-examination in 3 months. Many times there is a hypoplastic urethra or chordee that needs to be addressed or ruled out before circumcision is performed.

Webbed penis (Penoscrotal fusion) occurs when the scrotum forms the ventral side of the penile shaft. We usually suggest leaving the foreskin intact in these newborns and re-examination in 3 months. Circumcision is performed when the child is older than 6 months in the operating room where we release the penis from the scrotum and determine how much foreskin is needed in order to cover the underside of the penis.

New Born Circumcision
Newborn circumcision should not be performed in any child with hypospadias/chordee. Circumcision in this setting may result in injury to the underlying urethra and/or removal of the skin that is needed for surgical correction. Even in questionable circumstances we recommend waiting until 6 months of age.

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