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Exstrophy of the Bladder and Epispadias
What is 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.
What causes exstrophy of the bladder and epispadias?
Experts don't know what causes exstrophy of the bladder and epispadias.
What are the risks of 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).
What are the symptoms of exstrophy of the bladder and epispadias?
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)
How is exstrophy of the bladder and epispadias diagnosed?
Exstrophy of the bladder can be diagnosed by fetal ultrasound before a baby is born. After the baby is born, the health care provider will give your child a physical exam. Your child may also have imaging tests. These are done to look at the positions oforgans 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.
How is exstrophy of the bladder and epispadias treated?
Treatment is done with surgery. There are often three 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 is an age when a child is ready to stay dry. This final surgery rebuilds the urinary tract, including the bladder and tubes.
Talk with your child’s health care 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