Hypospadias

What is hypospadias?

Hypospadias is a congenital condition in which the urinary opening is not at the normal position at the tip of the head (glans) of the penis.

Usually the problem is recognized at birth. The urinary opening may be located anywhere on the underside of the penis from the edge of the glans to behind the scrotum. In addition, the foreskin usually is not completely developed, but rather appears as a hood on the top of the penis leaving the glans partially exposed. The penis may also be curved downwards in some cases, especially when the urinary opening is a distance away from the glans.

The tissues that should have become the urinary channel remain on the penis, extending from the abnormal opening to the tip of the glans. This strip of skin is referred to as the "urethral plate."

How common is hypospadias?

This is one of the most common birth defects, occurring in 1 of every 150 or so boys.

What causes hypospadias?

The penis forms during the ninth to about the 20th week of pregnancy, when male hormones stimulate development of the urinary channel and the foreskin. Although the exact causes for hypospadias are not fully known, problems during this critical time period may result in the condition. Under the direction of Dr. Linda Baker, federal government-funded research in our program's John Duckett Lab at The University of Texas Southwestern Medical Center, seeks to understand how normal development stops before the penis is completely formed.

Hypospadias is the only birth defect in most boys with the condition, and so additional X-ray or lab testing is not necessary. In about 8 percent of patients, a testicle may not have fully descended into the scrotum, and in these cases a blood test (karyotype) is recommended to be certain there are no chromosome abnormalities.

Hypospadias may run in families. About 7 percent of patients have a father who also has the condition. If one boy has hypospadias, the chance a second will be born with it increases to approximately 12 percent. If both the father and a brother have hypospadias, the risk for another boy to be born with the condition is about 21 percent.

Who is affected by hypospadias?

Hypospadias is a disorder that primarily affects male newborns.
It occurs in about one in every 150 to 300 boys.
Hypospadias also has a genetic component. Eight percent of fathers of males with hypospadias also had the condition. Some studies show an increased risk for siblings to be born with hypospadias after the birth of one child with the problem.
Some newborn boys who have other congenital abnormalities such as undescended testes or inguinal hernias may also have hypospadias.

What are the symptoms of hypospadias?

The following are the most common symptoms of hypospadias, however, each baby may experience symptoms differently. Symptoms may include:

abnormal appearance of foreskin and penis on exam
abnormal direction of urine stream
the end of the penis may be curved downward

The symptoms of a hypospadias may resemble other conditions or medical problems. Always consult your baby's physician for a diagnosis.

How is hypospadias diagnosed?

A physician or healthcare professional usually diagnoses hypospadias at birth. The malformation can be detected by a physical examination.

How is hypospadias treated?

The only treatment is surgery to move the urinary opening to the correct position, straighten any bending of the penis that may be present, and either perform circumcision or fix the foreskin so that is covers the entire glans.

This type of surgery began in the late 1800s, and has been continuously refined to the present time. Traditionally, operations have been described to correct different degrees of hypospadias. When the urinary opening is on or near the glans it is called "distal," while openings further down on the penis, within the scrotum, or behind the scrotum are referred to as "proximal." Over the years, it is estimated more that 200 surgical techniques have been developed, although only a few are in common use today.

The goal of modern surgery is to create a functional penis that is cosmetically normal, that appears either to have never been operated upon or only circumcised. Most repairs are completed in 1 to 3 hours, and a single operation is usually all that is needed. Sometimes in proximal hypospadias there is such severe bending of the penis downwards that a staged procedure may be chosen, the first operation to straighten the penis and the second to reconstruct the urinary channel.

At what age is surgery done?

Although hypospadias repair can be done at any time in childhood, or even in adults, it is preferable to do surgery in healthy boys between 3 and 18 months. During this time, surgery can be performed as an outpatient, and completed before the child becomes aware of the genitals.

Who should do the operation?

We recommend a fellowship-trained pediatric urologist do the repair. This is a delicate surgery usually performed wearing magnifying glasses and using very fine sutures. Urologists who specialize in children work with these tools and sutures daily, and perform more hypospadias operations each year than do general adult and pediatric urologists or other surgical specialists. It is reasonable to ask the urologist if he or she is fellowship trained in pediatric urology, how many operations he or she performs each year, and what is their complication rate.

If there are several operations that can be done to correct hypospadias, how is it decided which is best for my boy?

Different surgeons may choose to use different techniques for hypospadias repair. Generally, specialists in hypospadias rely upon several possible operations they are most familiar with and believe give the best results, depending upon the specific situation. In the past this decision-making process was more variable than it is today, especially for distal hypospadias. Since 1994, there has been increasing use of the urethral plate to tubularize into the urinary channel, thereby moving the opening to the tip of the glans. This is the TIP (tubularized incised plate) repair, also widely known as the "Snodgrass" operation since it was developed by Dr. Warren Snodgrass.

How do I care for my son's wounds after surgery?

Studies have proven no special bandages or wound care are needed after hypospadias repair. We prefer a clear plastic bandage that almost always falls off by itself within a few days, rather than various wrappings that have to be removed in the office. We also use a urinary catheter for about a week after surgery so that the boy is not urinating through the fresh repair. This catheter simply drains into a diaper in younger patients, although it can be connected to a bag in older boys or adults. An antibiotic is prescribed while the catheter is in place, and, in older boys and adults, an antispasmodic medication is also provided to reduce bladder irritation from the catheter.

The healing process begins immediately after surgery and continues for several months. In the first few weeks, there may be swelling and bruising that gradually subside. We assess the final cosmetic appearance at 6 months to allow adequate time for healing, and during this period most complications that can occur are detected.

What complications can develop?

Several problems can occur after this reconstructive surgery. The most common is "fistulas," which are abnormal openings under the penis that leak urine during voiding. Sometimes part, or all, of the repair comes open (dehiscence), returning the urinary opening back towards its original location. Scar tissue at the urinary opening (meatal stenosis) or along the new urinary channel (urethral stricture) may cause blockage to urination. If the new urinary channel enlarges, a "diverticulum" results, which looks like a swelling under the shin during urination from which urine dribbles after voiding. Other complications can also occur, but these account for most problems after hypospadias surgery.

The frequency of these complications depends upon several factors. One is the severity of the condition, as distal hypospadias operations have fewer problems after surgery than do more severe proximal ones. Various techniques used to correct the defect are known to have different rates and types of complications. Finally, experience of the surgeon may also affect the likelihood of problems after the repair. It is reasonable to ask about the personal experience of the surgeon when deciding who is going to operate upon your son. Nevertheless, even the most experienced pediatric urologist occasionally has complications after hypospadias repair.