Exstrophy of the Bladder and Epispadias in Children

Exstrophy of the bladder is when a baby’s bladder has grown inside out. It sticks through the wall of the belly (abdomen). The bladder can be seen in the front of the child’s lower belly. It's a rare condition. It includes problems with how the urinary tract, muscles, bones, and the digestive system grew in the womb.

Epispadias is a problem often seen with exstrophy of the bladder. It's when the opening of the tube that carries urine out of the body (the urethra) is in the wrong place. In a baby boy, the opening is often on the top of the penis. But with this condition, the opening may be the whole length of the penis. In a girl, the opening may also be longer and higher in the urethra. It may open right next to the bladder.

Experts don't know what causes these problems. There is some evidence that environmental factors and genetics play a role.

The disorder varies from mild to severe. A child with exstrophy of the bladder may have a triangle-shaped opening in the belly where the bladder can be seen. The bladder will look bright pink. A boy may also have a short, small penis. It will have a urethral opening along the top of the penis (epispadias). A girl may also have a narrow vaginal opening, wide labia, and a short urethra.

In many cases, a child with exstrophy of the bladder may also have these problems:

• Widened pubic bones
• Legs and feet that are turned out
• Abnormally-shaped abdominal muscles
• Belly button that is not in a normal place. It may be above the bladder.
• Bulging of intestine through a weak spot in the abdominal muscles (umbilical hernia)

Symptoms of epispadias may include an abnormal urinary stream and urinary tract infections.

Exstrophy of the bladder can be diagnosed by fetal ultrasound before a baby is born. After the baby is born, the healthcare provider will give your child a physical exam. Your child may also have imaging tests. These are done to look at the positions of organs and other tissues.

Treatment is done with surgery. There are often 3 stages of surgery to fix the problems. They are:

• Stage 1. This surgery may be done when your baby is as young as 48 hours old. The bladder is put inside the body, and the belly is closed.
• Stage 2. This surgery may be done as early as 6 months old. It includes fixing the epispadias and other genital problems.
• Stage 3. This surgery is done at around age 4 or 5. This is when the bladder is large enough. And it’s an age when a child is ready to stay dry. This final surgery rebuilds the urinary tract. This includes the bladder and tubes.

Talk with your child’s healthcare providers about the risks, benefits, and possible side effects of all treatments.

Even with surgery, a child may not be able to control their urine. This is known as urinary incontinence. Newer surgery methods make it possible for most children to control their urine over time. But a child may have long-term damage to the urinary tract and kidneys. In some cases, the problem may also cause loss of fertility in the future. This means that when the child is an adult, it may be hard to have children.

Call the healthcare provider if your child has:

• Symptoms that don’t get better or get worse
• New symptoms

• Exstrophy of the bladder is when a baby’s bladder has grown inside out. It sticks out through the belly wall.
• Epispadias is a problem often seen with exstrophy of the bladder. It is when the opening of the tube that carries urine out of the body (the urethra) is in the wrong place.
• A child with exstrophy of the bladder may have a triangle-shaped opening in the belly where the bladder can be seen. The bladder will look bright pink.
• Treatment is done with surgery. There are often 3 stages of surgery to fix the problems.
• Even with surgery, a child may not be able to control their urine. This is known as urinary incontinence. There may also be long-term damage to the urinary tract and kidneys.

Tips to help you get the most from a visit to your child’s healthcare provider:

• Know the reason for the visit and what you want to happen.
• Before your visit, write down questions you want answered.
• At the visit, write down the name of a new diagnosis and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you for your child.
• Know why a new medicine or treatment is prescribed and how it will help your child. Also know what the side effects are.
• Ask if your child’s condition can be treated in other ways.
• Know why a test or procedure is recommended and what the results could mean.
• Know what to expect if your child does not take the medicine or have the test or procedure.
• If your child has a follow-up appointment, write down the date, time, and purpose for that visit.
• Know how you can contact your child’s healthcare provider after office hours. This is important if your child becomes ill and you have questions or need advice.