What is Bladder Exstrophy?

Male Urinary Tract
Male Urinary Tract
Medical Illustration Copyright © 2015 Nucleus Medical Media, All rights reserved

Female Urinary Tract
Female Urinary Tract
Medical Illustration Copyright © 2015 Nucleus Medical Media, All rights reserved

Sometimes problems happen with the way the bladder forms. The bladder muscles don't develop properly for children with bladder exstrophy.

If your newborn is diagnosed with this condition, read on. This information can help you talk with your child's urologist and plan for the future.

How Does the Bladder Work?

The bladder is a balloon-shaped organ that stores urine (waste). It's held in place by pelvic muscles inside the lower part of your belly.

Under normal conditions, the bladder is located in your lower belly. It is relaxed when it's not full. When urine produced by the kidneys fills the bladder, nerve signals let you feel that it's full. You then feel the need to urinate. The brain's nerve signals tell the bladder muscles to squeeze (or contract). The squeezing (contracting) of your bladder forces urine out of your bladder. The urine then goes through your urethra and out of your body.

What is Bladder Exstrophy?

Bladder exstrophy is a birth defect. It's a condition where the bladder and parts around it form inside-out. The skin, muscle, and pelvic (hip) bones at the lower part of the belly or abdomen are not joined. As a result, the inside of the bladder pokes outside the belly. Instead of its normal round shape, the bladder is flat. There are also problems with the abdominal muscles and pelvic bones.

How Often does Bladder Exstrophy Occur?

Bladder exstrophy is rare. On average, it occurs in about 1 out of every 50,000 live births. It is slightly more common in males than females.

Family history plays a role. Children born to a parent with bladder exstrophy have about a 1 in 70 chance of having it. If a family has one child with this condition, they have a 1 in 100 chance of having another child with it.

Fertility treatments may play a role. Recent studies suggest that children born with assisted fertility are 7 times more likely to have bladder exstrophy.



What Causes Bladder Exstrophy?

Bladder exstrophy is not caused by anything a parent did or didn't do during pregnancy. There is no clear cause for this condition.

It is thought to happen during the 11th week of pregnancy, as the organs develop. Some experts believe that the bladder defect occurs at the times the tissues in the lower wall of the belly or abdomen develop. At the same time, the developing muscles and pelvic bones are affected too.

A temporary tissue called the cloacal membrane covers the lower belly wall and is replaced by maturing and developing abdominal muscles. If the cloacal membrane bursts before the abdominal muscles fully form, this may result in an "exstrophied" bladder. Another tissue called the urorectal septum helps to separate the developing bladder from the bowels or intestines.

Whether the child is born with epispadias, classic bladder exstrophy, or cloacal exstrophy depends on when the cloacal membrane bursts and if the bladder and intestines are separated by the urorectal septum.

  • An epispadias is a rare birth defect of the penis, where the urethra ends in an opening on the top side of the penis. For girls, the urethra develops too far up front.
  • Cloacal exstrophy (EC) is a severe birth defect where the abdominal organs (the bladder and intestines) are exposed. The penis or vagina is split, and the anus may be sealed.

Mostly, bladder exstrophy occurs as the only birth defect. Spinal cord problems can also occur but are more common with cloacal exstrophy.



How is Bladder Exstrophy Diagnosed?

Bladder exstrophy can often be found before birth during a routine sonogram. In any case, this condition will be clear at birth. The bladder will clearly be seen outside of the baby’s belly.

If this condition has been noticed before birth, plans are often made for immediate treatment at birth. A pediatrician and surgeon can check the baby right away.

If the condition isn’t found until the time of birth, the baby will be moved to the specialist unit. The doctor will note the bladder size and quality, the shape of the pelvis, and the state of the outer sex organs. A surgeon will be contacted for treatment.



How is Bladder Exstrophy Treated?

Bladder exstrophy is treated with surgery. The type of surgery used depends on how severe the defect is. It is critical to work with a surgeon who is experienced with treating exstrophy experience.

Advances over the last 15 years have led to successful reconstruction. This includes reconstruction of the penis and bladder for a more "normal" and functional lifestyle.

The main goals of treatment are to:

  • Close the bladder, the back of the urethra, and the pelvis
  • Rebuild a penis that looks normal and works in boys/ the outer sex organs in girls
  • Fix the bladder so it can hold urine until it's time to urinate ("urinary continence") without harming kidney function

One form of treatment is "staged reconstruction." This involves parts of the above surgeries done over the early childhood years:

  • The first surgery is to close the bladder and pelvis. This is done just after the baby is born.
  • When the child is about 6 months old, surgery is done to rebuild the epispadiac urethra and penis.
  • When the bladder has grown large enough and the child is ready for potty-training (often around 4 or 5 years old), surgery is done on the bladder neck to achieve continence.

When the bladder's quality is good, and the penis size is good at birth, closing the bladder and penile reconstruction can be done in a "single operation" at an early age.

Both early and staged reconstruction have good results. If the bladder has grown enough and the surgeon is skilled, continence (control over urination) is possible. Often, further operations are needed over time to improve the child's ability to urinate. More surgery may also be needed to rebuild and/or make better the outer sex organs.

In more difficult situations, longer-term management is needed. Modern reconstructive surgery can still allow a baby to reach his/her late teens with successful results.



Frequently Asked Questions

What is the outlook for a baby born with this condition?

Children with exstrophy grow up to be robust people. They have a normal life expectancy. They can often participate in normal activities without restrictions.

Are there other health problems linked to bladder exstrophy?

Yes. These may include:

Patients often walk with a "waddle" gait, where the legs are pointed out. This gets better with age. Bowel function and control are normal.