AUA Summit - What is Epispadias?

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What is Epispadias?

Epispadias is a rare congenital (present at birth) abnormality that involves the opening of the urethra (the tube from which urine exits the bladder). In boys with epispadias, the urethra opens in the top of the penis rather than the tip. The space between this opening and tip of the penis appears like an open book (gutter). In girls with epispadias, the urethral opening is toward the clitoris or even belly area. This results in the outside genitalia and urethra not forming or working well.

Most boys and girls are born with genitals that look normal and work well. But some children born with a condition called epispadias can have a penis or urethra that don't work well or look normal.

There are many types of surgery that pediatric urologists can use to fix this problem. Here we offer information to help you speak to your child's urologist about options.

How Does Epispadias Affect Boys?

Epispadias is rare. In boys with epispadias, the penis tends to be broad, short and curved upwards (“dorsal chordee”). The pelvic bones are widely separated. Since the penis is attached to these bones, it results in a penis that is pulled back toward the body.

Normally, the opening of the urethra (the “meatus”) is at the tip of the penis. But in boys with epispadias, it's on top of the penis.

From this opening, a groove runs along the top of the penis to the end. The epispadias is classified based on the location of the meatus on the penis.

  • Glanular epispadias: It is found on the head of the penis.
  • Penile epispadias: It is found along the shaft of the penis.
  • Penopubic epispadias: It is found near the pubic bone.

The position of the opening (meatus) can help predict how well the bladder stores urine. If the meatus is close to the base of the penis (and the abdominal wall), the bladder likely won't hold urine because the bladder sphincter (the part of the bladder that stops a bladder from leaking) is likely affected.

In most cases of penopubic epispadias, the bones of the pelvis don't come together in the front. In this case, the bladder sphincter doesn't close all the way since it's shaped more like a horseshoe than a ring. Because of this, urine leaks out. Most boys with penopubic epispadias, and about two out of three with penile epispadias leak urine with stress and sometimes leak urine continuously with things like coughing and strenuous effort. Most will need to have the bladder neck fixed with surgery.

Almost all boys with glanular epispadias have a good bladder neck and therefore a good bladder sphincter. Because of this, they can hold urine and toilet train normally. Still, the bend and abnormal opening of the penis will need to be fixed with surgery.

How Does Epispadias Affect Girls?

Epispadias is much more rare in girls. Girls who are affected have pubic bones that are separated to different degrees. This stops the clitoris from connecting in the middle, resulting in two halves of the clitoris. The bladder neck is also almost always affected. Girls with epispadias likely leak urine with stress or sometimes they will leak continuously with things like coughing and strenuous effort. In most cases, early surgery can fix these problems.

What Happens under Normal Conditions?

The urinary tract is like a plumbing system. It has special "pipes" that allow waste to flow through. The urinary tract is made up of two kidneys, two ureters, the bladder and the urethra.

The kidneys act as a filter system for the blood. They remove toxins and keep useful proteins, sugar, salts and minerals. Urine is the waste product. It is made in the kidneys and flows down two, 10 to 12-inch-long tubes called ureters. The ureters are about a quarter inch wide and have muscled walls. They push urine into the bladder.

The bladder can swell to store the urine until you're ready to drain it. It also closes the path so urine can't flow back into the kidneys.

The tube that carries urine from the bladder out of the body is called the urethra.

In males, the urethra is long. It starts at the bladder and runs through the prostate gland, perineum (the space between the scrotum and the anus) and penis. In females, the urethra is much shorter. It runs from the bladder in front of the vagina and opens outside the body.

Your urethra has muscles called sphincters. The sphincter complex (also called the bladder neck) is a ring-shaped muscle wrapped around the urethra. They help keep the urethra closed so urine doesn't leak before you're ready. These sphincters open up when the bladder contracts so you can release urine.

Symptoms

Most cases of epispadias are diagnosed at birth during physical examination of the newborn. In mild cases of epispadias, the condition can go unnoticed until parents note urine leaks after potty training.

Causes

The way the organs form in a baby during pregnancy is very complicated. Certain steps must occur in a finely timed order. Most often, if there is one defect in an organ, there will also be other defects. Only rarely does epispadias occur alone. There will also often be issues with the bladder. In rare cases, there can also be defects in the large intestine.

How the epispadias affects the genitals varies. In some boys, it can be just a small dimple on the tip of the penis above the normal urethral opening. With girls, it could be a double clitoris. If the urethra or bladder is involved, the epispadias is usually more severe. This large range of issues is called the exstrophy-epispadias complex (see section on Exstrophy).

Diagnosis

Epispadias is most often noticed at birth. If the defect is mild, it might not be noticed at first. In some cases, it might not be noticed until the child has been toilet trained and presents with urine leakage. This is most common with girls.

Treatment

The main goal of treatment is to make the genitals look and work as normal as possible. This is ideally done with the fewest number of surgeries. There are different surgery options available for boys and girls.

Surgery Options for Boys

The main goals of epispadias treatment for boys are to:

  • Make sure the penis works as it should
  • Fix bends in the penis (dorsal bend and chordee)

If the bladder and bladder neck are affected, surgery will be needed to make sure the child can control his urine. Surgery might also be needed to preserve fertility.

There are two popular types of surgery. Both have the goal of providing a penis with straight erections and the ability to pass urine. The surgery is often done while the child is a baby. The type chosen depends on:

  • The type of epispadias
  • Surgeon experience
  • Surgeon preference

Sometimes, boys with the exstrophy-epispadias complex are born with a very underdeveloped penis. In these cases, surgery requires more skill and experience. You may ask your urologist for the name of a specialist.

The Modified Cantwell Technique

The modified Cantwell technique involves "rebuilding" the penis. It takes some of the penis apart to move the urethra to a more normal position.

The Mitchell Technique

The Mitchell technique involves taking the penis apart fully, then putting it back together. This is done so the urethra is in the most functional and normal position, and dorsal bend (chordee) is fixed.

Surgery Options for Girls

Reconstruction in girls is less complex than in boys. The urethra and vagina may be short and near the front of the body. The clitoris may be in two parts. The internal female structures – uterus, fallopian tubes and ovaries – are usually correct.

If diagnosed at birth, the two parts of the clitoris can be brought together and the urethra can be placed in the correct position. If repaired early enough, lack of urinary control (incontinence) may not be a problem.

If the problem isn't diagnosed early or early repair isn't done, then incontinence can be fixed with surgery when it's found. If the vaginal opening is narrow in older girls or younger women, reconstruction can be done after puberty.

Further Treatment

With the newer methods of surgical repair at birth, almost one in three children achieve urinary control without other procedures. Children with the exstrophy-epispadias complex may need additional surgery to repair the bladder neck. This is done to improve the strength of the urethra and sphincter. Or, if a child can't toilet train normally, there are more options.

One way is to inject a bulking material around the bladder neck. This makes the bladder neck thicker so urine can't leak out.

There are also more complex procedures. One surgery creates a longer urethral tube. Another involves wrapping the bladder neck with a sling.

After Treatment

Sometimes a hole ("fistula") can open from the urethra to the skin after a major repair. This isn’t very common with today’s techniques. If this does occur, additional surgery is needed to close the hole.

Persistent dorsal chordee (the penis bending) is fairly typical with older techniques. It’s now less common. Epispadias repair does little harm to erectile function.

More Information

Babies with epispadias are most often healthy. Other than the problems with the genitourinary system and the pelvic bones, other organ systems tend to work well. Because of this, these children don't need a lot of diagnostic tests (such as X-rays and CT scans). If a baby is born with the more severe forms, they are at a higher risk for other defects, like enlarged ureters or vesicoureteral reflux.

Boys with penopubic epispadias or the exstrophy-epispadias complex may be infertile. In normal young men, the bladder neck is closed when sperm moves from the testicles to the urethra during ejaculation. In some men with this birth defect, the bladder neck may not close fully during ejaculation. This allows the sperm to move backwards into the bladder (retrograde ejaculation). This may cause problems when a couple is trying to have children. Some of these men may have poor sperm quality. Also, bending and a short, stubby penis can make it hard to have intercourse. Often, surgical repair can reduce these problems.

Females with epispadias usually aren't at risk for infertility, since their internal organs are normal.

Frequently Asked Questions

Are children with epispadias more likely to get urinary tract infections?

At this time, no studies show that epispadias leads to urinary tract infections (UTIs). But in cases of complete exstrophy-epispadias, the child will be at increased risk. This is due to a condition called vesicoureteral reflux. These children are most often treated with a small dose of prophylactic antibiotics until the reflux is fixed.

Can epispadias be prevented?

There is no known prevention.

What is the best time for surgical treatment?

Many leading reconstructive surgeons recommend epispadias repair as early as possible. This is especially true for boys with penopubic or penile epispadias and all girls with the condition. When treated early, bladder capacity can often be improved. A well-developed bladder is of great value for good urinary control. Early bladder filling and emptying (cycling) can help strengthen the growing bladder. If surgery is done within the first few months of life, the child may have a better chance of having a normal bladder.

Until recently, epispadias reconstruction was delayed until the child was one year old. But some surgeons complete an earlier repair in hopes to achieve better bladder development. Talk to your child’s doctor to find the best treatment plan for your child.

Updated May 2024. 


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