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Hypospadias and Chordee

Hypospadias is a birth defect that affects boys. When boys have hypospadias, the opening of their urethra (the tube that carries urine from the bladder to through the penis) is not located at the tip of the penis as it should be. Although hypospadias is not considered an inherited disorder, it does seem to occur more often than normal in some families.

Chordeeis a bending of the penis. It is noticeable during an erection. It can occur alone, or it can be seen in combination with hypospadias.

Hypospadias ranges from minor to severe. The degree of severity depends upon where the urethral opening is. In minor cases of hypospadias when urethral opening doesn’t cause any functional problems, the condition may not have to be corrected. However, most boys with hypospadias will have difficulty with function and will need treatment.

Surgical treatment for hypospadias

If hypospadias must be treated with surgery, the surgeon will likely straighten the penis (correcting chordee) and create an extension of the urethra, bringing it to the tip of the penis. Surgeons may use penile skin tissue to create the new urethral pathway for the urine.

Why consider surgery for hypospadias?

When someone has hypospadias, it can make normal urination difficult – even if it’s more of a cosmetic defect than a functional one. For example, some boys with hypospadias will not be able to stand to urinate because the opening of the urethra doesn’t reach the tip of the penis. When boys have to sit to urinate, there may be social implications. Boys who can’t stand to urinate may be teased by their peers.

Why consider surgery for chordee?

A straight, erect penis is necessary for proper sexual function. Chordee may not affect boys while they are children, but if chordee is significant enough, it can inhibit sexual function later in adulthood. Because this surgery is much easier during childhood, we recommend correcting it when your child is young rather than waiting until later.

Embryology

The penis begins to form about the fifth week of fetal development. Before boys develop their penises, their urethral openings are in the same place as they are for girls. But as the penis forms, the urethra grows toward the tip of the penis. Then the foreskin grows after the urethra has been completely formed. When boys have hypospadias, this process gets disrupted somewhere along the way. Experts are uncertain why hypospadias occurs.

Can hypospadias affect fertility?

Hypospadias does not directly cause infertility. However, men with severe hypospadias may be infertile due to accompanying problems with the testes.

Can men with hypospadias function sexually?

Hypospadias does not inhibit sexual sensation or orgasm. Some parents ask whether plastic surgery to correct hypospadias and/or chordee will damage erotic sensation. Fortunately, corrective plastic surgery of the penis or testicles will not normally inhibit erotic sensation or the ability to reach orgasm.

Correcting Hypospadias and Chordee with Surgery

Boys with mild cases of hypospadias who do not have significant chordee may not need to have surgery. However, many parents (and patients) elect surgery regardless of the degree of severity because even mild hypospadias is cosmetically different and may make urinating while standing difficult.

During surgery to correct hypospadias, surgeons use a tube-shaped flap of skin from the shaft of the penis to form a new portion of the urethra. Surgeons elect to use penile skin because it will continue to grow as the penis does, and it does not have hair follicles, so it won’t grow hair.

Surgeons often divert urine away from the new urethra (called the neourethra) to protect it while it heals. This is done with a stent, which is a silastic tube placed through the neourethra. Most of the time this is an outpatient procedure, and patients can go home the same day. Depending on the type of surgery necessary, the stent may be left in the neourethra for 3 to 10 days after surgery.

Doctors can evaluate the degree of chordee by creating an artificial erection. This is done by using saline solution to inflate erectile bodies.

When hypospadias is severe and chordee is present, the surgery required may be more extensive than the standard hypospadias corrective surgery. The traditional method was to use two- and three-stage operations. The first stage involved straightening the penis. This had a tendency to increase the severity of the hypospadias. Then, six to twelve months later, surgeons would begin constructing a new urethra using penile skin or skin grafts. Today, our preference for hypospadias and chordee correction is to combine the neourethra creation with the penile straightening. However, some patients will still need to have more than one surgery in order to fully correct their defect.

Complications

All surgeries come with risks of complications. Below is a list of some of the potential risks of hypospadias and/or chordee correction.

  • Bleeding. Any time you have an operation, even a minor one, there is a risk of bleeding. After hypospadias and/or chordee surgery, we use a pressure dressing on the affected area to help prevent bleeding. Severe bleeding and blood transfusions are extremely rare with hypospadias surgeries.
  • Infection. All surgeries carry the risk of infection. We take many precautions to reduce this risk, including using antibiotics in patients who have a catheter.
  • Bladder spasms. When people have indwelling catheters, they may experience bladder spasms. These catheters are used as a safety measure, but they can cause discomfort after surgery. In the event of bladder spasms, medications may help, but they may not eliminate the discomfort. If your child has a catheter after surgery, he may be given a medication called Ditropan to reduce spasms. If your child seems uncomfortable after surgery, first try using Tylenol (either with or without codeine). If that doesn’t work, try a single dose of Ditropan. If it seems to work, continue using it per the package directions. If the Ditropan doesn’t seem to help, stop using it.
  • Fistula. After hypospadias and/or chordee surgery, your child may experience a leak of urine from somewhere in the new urethra. This is called a fistula. If your child’s repair was simple, the risk of fistula is low; however, more complex repairs carry a higher risk of fistula. In the event of fistula, your child will have to have a surgical repair. This repair is usually easy and is done as an outpatient procedure, but it cannot be done for at least six months after the initial surgery.
  • Stricture or Stenosis. Stricture and stenosis involve a narrowing of the urethra, either where the neourethra joins the pre-existing urethra or at the urinary opening. This requires correction by dilation/stretching or by performing an internal urethrotomy. A urethrotomy is a procedure that cuts the narrowing using a tool called a cytoscope. Because stricture and stenosis tend to reform even after correction, the repair procedures may need to be done more than once,
  • Recurrent chordee. Recurrent chordee (bending of the penis) is an uncommon problem after chordee repair surgery. If chordee returns after surgery, another surgery to straighten the penis may be necessary.
  • Diverticulum. Diverticulum is an out pouching of a newly formed urethra. It may look like a ballooning of the urethra. Diverticulum can cause infection and will have to be corrected with surgery.

Chordee Without Hypospadias

Chordee can be present without hypospadias. It is often found when parents bring their children to the doctor due to a bend in the penis. Often, chordee alone is caused by skin tethering. Most of the time, this can be easily corrected.

However, in some boys with chordee, the distal urethra is very thin (called hypoplastic), despite being intact. In this case, surgeons may have to actually create hypospadias in order to straighten the penis. Then the surgeon will have to correct the man-made hypospadias. The hypospadias correction can occur with the chordee repair or in a separate surgery. Some boys will need a surgery that plicates the bent part of the penis with permanent sutures to straighten the penis.

Other Variations of Chordee

Penile torsion

Penile torsion isalso known as wandering raphe, is characterized by a counterclockwise rotation of the penis. It is sometimes called wandering raphe because the midline raphe of the penis wraps counterclockwise around the penis. When the raphe approaches 90 degrees, there may be penile torsion underneath the foreskin. Surgeons do not usually correct penile torsion alone unless it approaches 90 degrees. Usually, doctors repair penile torsion at the same time as performing circumcision.

Sometimes penile torsion occurs along with hypospadias, and it may be improved, at least to some extent, during hypospadias repair.

Dorsal preputial hood

Dorsal preputial hood is a condition that can be found in newborn boys. It is a variant of hypospadias/chordee. When newborns have a dorsal preputial hood, we typically suggest that parents leave the foreskin intact and have their child re-examined in three months. This is because there may be a hypoplastic urethra or chordee that will have to be repaired before circumcision.

Webbed penis (penoscrotal fusion)

Webbed penis, also called penoscrotal fusion, occurs when a boy’s scrotum forms on the underside of the shaft of the penis. When boys have this condition, we usually recommend leaving the foreskin intact and re-examining the condition in 3 months. We correct penoscrotal fusion and perform circumcision in the operating room when boys are older than 6 months of age. The repair involves releasing the penis from the scrotum and determining how much foreskin is needed to cover the underside of the penis where the scrotum had been attached.

Newborn Circumcision

We do not recommend circumcising any newborn who has hypospadias and/or chordee. This is because surgeons may need the foreskin during the surgical correction of hypospadias and/or chordee. Even in questionable circumstances we recommend delaying circumcision until 6 months of age.

When do you perform surgery?

Your son’s doctor will talk with you about the best time to perform surgery. Most of the time, we perform surgery between 6and 12 months of age.