Bladder Exstrophy and Epispadias
Bladder exstrophy is a birth defect (also known as a congenital defect) where the skin, muscle and pelvic bones at the lower part of the abdomen do not fully form. The bladder pokes outside the belly. The bladder also forms inside out and is open to the abdominal wall. The condition can affect parts of the lower urinary tract, which is called Epispadias. Epispadias occurs when the urethra does not fully form. Additionally, this causes a shorter than average penis or vagina.
Bladder exstrophy occurs in one out of 30,000 to 50,000 births. It's more common in boys than girls.
Experts don't know what causes bladder exstrophy. The problem usually occurs four to eleven weeks after a woman gets pregnant, when organs, muscles and tissues in the embryo begin to form layers that then separate, divide and fold.
Exstrophy does not occur because of anything the mother did or did not do during pregnancy. Some reports show bladder exstrophy is more common within some families. However, the chance for parents having a second child with bladder exstrophy is still small – one percent or less.
The signs and symptoms of bladder exstrophy vary based on how severe your child's condition is. Possible symptoms include:
- Epispadias. Bladder exstrophy often includes this condition, in which the opening of the urethra – the tube that drains urine from the bladder to the outside of the body – is in an abnormal location. For boys, this may be at the top or side of the penis. For girls, this may be between the clitoris and the labia.
- A triangle-shaped defect in the abdomen where the membrane of the bladder – usually bright pink – is visible.
- Widened pubic bones.
- Outwardly rotated legs and feet.
- Short, small penis in boys and a narrow vaginal opening, wide labia and short urethra in girls.
Sometimes it is diagnosed during pregnancy when the mother has an ultrasound. More often it is diagnosed at birth. During diagnosis, a specialist may be required to confirm whether the baby is a boy or girl. Your baby may also need imaging tests to determine which organs are affected.
Treatment for the condition involves at least two – sometimes more – reconstructive surgeries. The surgeries close the bladder and help join the pelvic bones. This allows the child to develop normal bladder control and avoid future problems with sexual function.
Babies usually have the first surgery in the first couple months of life. They often need further surgeries during early childhood.
It is important to work with a surgeon who has deep experience in treating bladder exstrophy.
Usually, surgical reconstruction to treat bladder exstrophy has good results. Sometimes a child needs multiple operations, but most children will eventually be able to control their urination and function normally.
Children with exstrophy most often grow up and enjoy full happy and active lives with a typical life expectancy.
A book for children with bladder exstrophy
A Story About You ... and Your Special Bladder was written for children with bladder exstrophy to help them understand their physical differences. This book provides a comfortable starting point for the caregiver and child to begin discussing bladder exstrophy. Cook Children's collaborated with the Association for the Bladder Exstrophy Community for the rewriting, illustrating and printing of this book.
If you are interested in having a copy, please contact Emily Haddad, LCSW, Director of Patient and Family Support at Emily.Haddad@cookchildrens.org or 682-303-0388.
Patient and family resources for bladder exstrophy
We're here to help.
If your child has been diagnosed, you probably have lots of questions. We can help. If you would like to schedule an appointment, refer a patient or speak to our staff, please call our offices at 682-303-0376.