Bladder Exstrophy and Epispadias

Bladder exstrophy is a congenital condition (identified at or before birth) that is visibly obvious at birth because the bladder is located partially outside the body on the lower abdominal wall. On a boy, the bladder may sit just above the penis and, on a girl, above the vagina.

A normal bladder is like a round balloon that holds liquid inside the body. Children with bladder exstrophy have flat bladders that are outside the body. In boys, the urethra is split and located on top of the penis. In girls, the urethra is also split and the opening sits between the clitoris and the labia minor.

It is a rare congenital condition with no known cause, affecting one to three of every 50,000 live newborns. There is no known cause for bladder exstrophy, and there is nothing mothers can do during pregnancy to cause or prevent it. Researchers believe it happens near the eleventh week of pregnancy when the lower wall of a baby’s abdomen develops. Boys are more likely to be born with this condition than girls.

Bladder exstrophy is sometimes accompanied by two other conditions: epispadias and cloacal exstrophy. Together, they form a triple complex known as bladder exstrophy-epispadias-cloacal exstrophy complex. Much like bladder exstrophy, epispadias exstrophy has the urethra (the tube that carries pee from the bladder) outside the body. Cloacal extrophy is the most severe because the bladder, the urethra (epispadias) and part of the colon or bowel are located outside the body.

Children born with this complex may have a wide spectrum of physical abnormalities that affect their abdominal wall, bladder, genitalia, pelvic bones, anus and rectum. In the United States, treatments begin in the first weeks to months of life. Early treatments are necessary to preserve normal function of the bladder and to restore the genitalia to a more normal appearance and function.

Diagnosis of Bladder Exstrophy

Bladder exstrophy is sometimes diagnosed during pregnancy by prenatal ultrasound and magnetic resonance imaging (MRI), but occasionally it is not noticed until birth. When it is detected prenatally, doctors at the Fetal Center at Riley at IU Health carefully plan a baby’s birth to give each newborn immediate care and evaluation. Children have access to an experienced, multidisciplinary team of pediatric surgeons, urologists and fetal specialists that are prepared to treat this condition and provide lifelong care.

Treatments

If you are a parent of a child born with bladder exstrophy, it is possible that you will go home with your newborn before surgery is done to repair the bladder. You can diaper your baby normally after covering the exstrophy with clear plastic wrap (the type used in your kitchen to keep food fresh). The purpose of the plastic wrap is to keep the bladder lining moist and protect it from exposure to stool and the diaper.

Once treatment begins, it has four goals:

Repair the bladder so it can hold urine, place it inside the pelvis and close the abdominal wall,
Repair the urethra (epispadias),
Reconstruct functional and cosmetically-acceptable genitalia, and
Preserve and facilitate renal function and urinary control.

Some of these goals can be combined in one surgery, but in most cases, children have a series of staged operations over time.

Surgeons have different opinions about the optimal time to perform these procedures, depending on the quality and size of the bladder, the separation of the pelvic bones and the size of the penis (in boys). Some surgeons prefer to repair the urethra and close the exstrophy when a child is 2 or 3 months old. Others believe it is better to do the exstrophy closure early (at 2 or 3 months of age) and delay repair of the urethra until a child is at least 12 months old. Most children with exstrophy will need to have their pelvis broken at the time of bladder closure (osteotomy) in order to bring the pubic bones together in the midline. The child will be placed in a cast with or without traction (weights attached to the legs to prevent movement) for 4 to 6 weeks until the pelvis has completely healed. Pediatric urologists repair the urethra by creating a more functional tube where pee can flow; they also reposition the urethra to a more natural place in the body.

Later in a child’s life, the neck or outlet of the bladder is sometimes repaired to make a better funnel and give children better voiding and urinary continence. These procedures do not always work on the first try. Some children may need additional surgeries to help them urinate better. Some children are not able to urinate normally after a bladder neck repair and will require catheterization to empty their bladders. Most children with bladder exstrophy will require medications to relax the bladder and help it store larger volumes of urine to improve urinary control and provide a safe environment for the kidneys. Some children with bladder exstrophy have such small bladders that they will need to have their bladders enlarged with intestine in order to achieve urinary control and provide a safe environment for the kidneys.

Children born with bladder exstrophy usually resemble their peers in every other way—emotionally, physically and intellectually. Although they may be more prone to urinary tract and bladder infections, their only significant difference is their need for lifelong care by specialists as they develop, especially for kids whose conditions are more severe.

Some kids never need another procedure after their early treatments, but doctors at Riley at IU Health generally recommend follow-up care at least through the teenage years to keep children healthy and observe any changes that warrant further treatment, such as abnormal development of the pelvis. Once they reach adulthood, most children who get early treatment for bladder exstrophy are otherwise healthy. They lead a normal life and need no further treatments.

Research

Physicians and researchers at Riley at IU Health and the Indiana University School of Medicine are part of an alliance that joins experts at four other children’s hospitals in the Midwest to study bladder exstrophy and find better treatments. Because of its rarity and the complexity of the procedures used to treat it, bladder exstrophy benefits from the combined knowledge researchers gain when they collaborate and share discoveries.

When a child is born with bladder exstrophy at one of the five hospitals, physicians from the alliance travel to observe procedures and gain insights about how to successfully manage pediatric care for this condition. This brings patients at Riley at IU Health access to the most advanced expertise for treating bladder exstrophy.

Key Points to Remember

Bladder exstrophy is a congenital condition that occurs while a baby is developing inside his/her mother, usually around the eleventh week when the lower wall of the abdomen forms.
It has no known cause or method of prevention.
Children with bladder exstrophy are born with a flat or abnormal bladder that sits outside the body.
One of the earliest methods of treatment is to reconstruct the bladder and place it back inside the body.
Most children with bladder exstrophy are otherwise healthy, but they do sometimes require more than one surgery to repair the abnormalities in their anatomy.
Occasionally, bladder exstrophy is accompanied by other conditions that place abdominal organs such as the urethra, the colon or bowel outside the body.

Support Services & Resources

Riley at IU Health offers a broad range of supportive services to make life better for families who choose us for their children's care.

Learn More About Riley Support Services

Urinary Care Foundation

This not-for-profit organization is owned by the American Urological Association and provides reliable education for patients and the public about urological conditions such as bladder exstrophy.

MedLine Plus

This U.S. National Library of Medicine website shares health-related content that explains methods for treating bladder exstrophy.

Association of Bladder Exstrophy Community

The Association for the Bladder Exstrophy Community is a support network for patients and families whose lives are affected by bladder exstrophy. They provide literature, referrals, camps, conferences, and networking opportunities.