Pediatric Exstrophy of the Bladder and Epispadias

Exstrophy of the bladder is when a baby’s bladder has grown inside out and is sticking through the wall of the belly (abdomen). The bladder can be seen in the front of the child’s lower belly. It is 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 is 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 exstrophy of the bladder and epispadias.

One in every 30,000 children is born with bladder exstrophy, according to Hans Pohl, M.D., Division Chief of Urology at Children’s National Hospital. Dr. Pohl adds: "There are few facilities in the country truly equipped to address these cases. Bladder exstrophy repair is a long, complex surgery that requires patience, precision and experience to manage correctly."

Dr. Pohl underwent specialized training in bladder exstrophy repair at Boston Children’s Hospital before coming to Children’s National.

Although some reports show a clustering of exstrophy of the bladder in families, suggesting an inherited factor, the chance for parents to have another child with exstrophy of the bladder is small (1 percent or less).

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 with a urethral opening along the top of the penis (epispadias). A girl may also have a narrow vaginal opening, a wide labia and a short urethra.

The disorder may occur in varying degrees from mild to severe. 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)

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.

Children’s National offers a new option for children born with bladder exstrophy. Instead of performing surgery immediately after birth, Hans Pohl, M.D., now sends families home with explicit instructions on caring for their infants’ special needs and performs surgery at a later date before the infants are 6 months old. This delayed surgical process also allows more time to arrange the surgery.

There are usually three stages to the surgical repair that start when your child is as young as 48 hours old. The first stage involves internalization of the bladder and closing the abdomen. The second stage may be done as early as 6 months of age and involves repairing the epispadias and other genital abnormalities. A final surgery involves reconstruction of the urinary tract including the bladder, and other structures of the urinary tract.

Treatment is done with surgery and your urologist will discuss the several stages of surgery that might be needed to reconstruct the urinary tract. Surgery may be done in a single operation or in several stages depending on the details of your child’s case. 

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

“Bladder exstrophy is one of the most complex conditions that we deal with in the field of pediatric urology. It takes a full team of healthcare professionals to prepare patients and their families for the procedure, ensure patient safety throughout the process, and provide the long-term follow-up care and support needed to help these children thrive. That’s what we’re dedicated to providing here at Children’s National.” – H. Gil Rushton, M.D., Emeritus Chair and Faculty, Children's Division of Urology Department

Even with surgery, a child may not be able to control his or her urine (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 health care provider if your child has:

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