Epispadias

What is epispadias?

Epispadias is a rare birth defect, a condition a baby is born with. It affects the urethra, the tube that carries urine outside of the body from the bladder. In a baby with epispadias, the urethra doesn’t fully develop.

Epispadias usually happens in males, though epispadias in females is rarer. Pediatric urologists, doctors who specialize in problems of the urinary system of babies and children, can fix the problem with surgery.

What is bladder exstrophy?

Most of the time, epispadias occurs with other congenital urological problems, including bladder exstrophy. Children with bladder exstrophy are born with the bladder outside the abdomen instead of inside.

How does epispadias affect males?

A baby boy with epispadias has:

• A penis that’s short, wide and curves up, called dorsal chordee.
• A meatus (me-ATE-us) on top of the penis rather than at the tip. The meatus is the hole or opening of the urethra. With epispadias, a deep groove often runs between the meatus and tip of the penis.

What are the types of epispadias in males?

Providers classify epispadias based on the meatus’ exact location. Its location affects how well the bladder can store urine.

The closer the meatus is to the base of the penis, the more likely the condition also affects the bladder sphincter and urine control. The bladder sphincter opens and closes to release or hold urine.

In boys, types of epispadias include:

• Glanular epispadias: This is the most common type. The meatus is on top of the glans (head of the penis).
• Penile epispadias: The meatus is along the penis shaft.
• Penopubic epispadias: The meatus is near the pubic bone. The pelvic bones don’t come together, and the sphincter can’t close completely.

How does epispadias affect females?

Baby girls may have:

• A urethral opening toward the clitoris or even in the lower abdomen (belly).
• Pubic bones that are separated, so the clitoris can’t connect in the middle.
• Labia (the folds of skin outside of the vagina) that don’t form properly.
• Problems with the bladder neck, resulting in urine control issues.

What’s epispadias vs. hypospadias?

Both epispadias and hypospadias are birth defects involving the urethra. Both describe the position of the urethral opening. Epi- means above, and hypo- means below.

Hypospadias primarily affects boys. In hypospadias, the urethra doesn’t fully develop and reach the penis tip. The urethra opening ends up in a different position along the bottom of the penis.

In epispadias, the tube also doesn’t form correctly. The opening is on the top of the penis. Epispadias is more common in boys but can also affect girls.

How common is epispadias?

Epispadias is a rare disease, affecting about:

• 1 in 117,000 male babies.
• 1 in 484,000 female babies.

What causes epispadias?

Researchers don’t know exactly what causes epispadias, but it occurs when a developing baby has a problem with the cloacal membrane. This membrane covers the cloaca, which normally develops into the gastrointestinal, urinary and reproductive organs.

What are the symptoms of epispadias?

Healthcare providers usually diagnose epispadias at one of the first newborn exams while the baby is still in the hospital. The diagnosis usually occurs before anyone even notices symptoms.

If the case is very mild, providers may not see it in the hospital. Parents may not notice anything until they’re toilet training their child and see some urine leaking. Girls are more likely to go undiagnosed.

How is epispadias diagnosed?

Healthcare providers usually diagnose this condition when a baby is born. Providers notice the unusually shaped penis in a boy or clitoris and labia in a girl. If the pediatrician detects epispadias, they’ll refer you to a pediatric urologist for treatment.

What tests will my baby need for epispadias?

Often, your provider won’t need to do any diagnostic tests. They can confirm the diagnosis just by examining your baby.

Your provider may recommend tests to confirm the extent of the problem. Tests may include:

• Blood tests.
• Imaging tests like ultrasound.
• Urinalysis.

Is epispadias diagnosed during pregnancy?

Epispadias is usually not diagnosed during pregnancy. It’s very difficult to detect on an ultrasound.

How is epispadias treated?

Surgery can correct epispadias to improve the look and function of the genitals. Your pediatric urologist will discuss the surgical options with you.

Healthcare providers recommend doing epispadias repair as early as possible. Early surgery helps achieve better results, especially with bladder control. Your child may need more than one surgery.

What is the goal of epispadias surgery for boys?

Epispadias surgery for boys aims to improve:

• Penis function, size and look.
• Good urine control, if the bladder was affected.
• Future fertility, which is more of a concern if the bladder develops outside of the fetus (exstrophy) along with the epispadias.

What are the types of epispadias surgery for boys?

There are a few epispadias surgery options. Your provider’s recommendation will depend on the type of epispadias. A child with exstrophy-epispadias complex may need a more intricate surgery.

Surgery types include:

• Modified Cantwell-Ransley technique rebuilds the penis. The surgeon may take partially reconstruct the penis and move the urethra to a better location.
• Mitchell technique completely reconstructs the penis. This ensures the urethra is in the best position and corrects chordee.

What are epispadias surgery options for girls?

Girls typically need less complex epispadias surgery than boys do. Usually, fertility isn’t an issue as epispadias doesn’t commonly affect the female reproductive system. The type of surgery may depend on how early a provider diagnosed epispadias.

If epispadias is diagnosed at birth, urinary control most likely won’t be a problem. Healthcare providers can:

• Connect the two parts of the clitoris.
• Place the urethra in the correct location.

If the problem isn’t diagnosed or treated until later, girls may need surgery to:

• Correct incontinence (lack of urine control).
• Reconstruct a narrow vaginal opening.

How long will it take to recover from epispadias surgery?

The recovery period depends on the complexity of the repair. Children who had a minor procedure will recover quickly. For children with bladder exstrophy, epispadias repair may be one procedure of many that they need. Recovery will take longer.

Can I prevent epispadias?

There is no known way to prevent epispadias.

What is the outlook for children with epispadias?

When surgeons perform repairs at birth, children achieve excellent results. Among these children, 1 in 3 will have urinary control without needing any other surgeries.

A child born with other conditions, such as bladder exstrophy, will probably need additional procedures to repair the bladder. With surgical repair, most children can achieve urinary control.

Are there complications from epispadias surgery?

Sometimes, after major surgery, a hole called a fistula can open from the urethra to the skin. However, this isn’t very common today with advanced surgical techniques. If it does happen, the fistula may close on its own or your child may need another procedure to close the fistula.

How can I best take care of my baby?

Your provider will explain how to care for the surgical site after the procedure. Follow your provider’s instructions to relieve pain and keep the area clean.

When should I see my healthcare provider?

After surgery, your provider will discuss when to return for follow-up appointments. Seek immediate medical help if your child has:

• High fever.
• Signs of infection at the surgical site (red, swollen or leaking fluid).
• Other worrying symptoms.

Does epispadias surgery affect erectile function?

No, epispadias repair should not affect erectile function.

Does epispadias affect fertility?

Epispadias doesn’t usually affect women’s fertility. However, men with penopubic epispadias or exstrophy-epispadias complex may experience infertility. Surgery may reduce these problems. Talk to your child’s surgeon about this issue.

If you had epispadias as a child, talk to your healthcare provider about how it might affect you.

If my child had epispadias, are they at higher risk for urinary tract infections?

No, studies haven’t shown any connection between epispadias and more UTIs.

Can a baby boy with epispadias have a circumcision?

Your provider will recommend delaying circumcision until after surgery. The surgeon may need to use the foreskin tissue during the surgical repair to rebuild the penis. After the epispadias repair, you can discuss circumcision with your provider.

A note from Cleveland Clinic

Epispadias is a rare birth defect affecting a baby’s urethra, the tube that carries urine from the body. Usually, providers diagnose the problem shortly after birth. A baby boy might have an unusually curved penis. A baby girl’s clitoris or labia might not have a typical appearance. Epispadias can lead to urinary incontinence, so treatment is essential. A pediatric urologist often performs an epispadias repair procedure shortly after birth. Depending on how complex the problem is, your child may need more than one procedure. Surgery usually provides good results, including well-formed genitalia and bladder control. If you notice urine leaking or anything concerning about your child’s genitals, see your provider.