Bladder exstrophy is a rare congenital abnormality (birth defect) in which the bladder doesn’t develop normally, but instead develops outside of the body through an opening on the abdominal wall. It is a complex defect that varies in severity. It can involve the reproductive and intestinal tracts, along with the urinary tract and muscles and bones in the pelvis.
Bladder exstrophy is more common in males than females. Experts estimate it occurs in about one in every 30,000 to 40,000 live births. There is no known cause.
What are the signs and symptoms of bladder exstrophy?
The signs and symptoms vary depending on the severity of the defect, but they may include:
- a bladder that is turned inside out and exposed on the abdominal wall
- a small bladder with an abnormal bladder neck
- epispadias – a birth defect in which the inner lining of the urethra (the tube that helps urine pass from the bladder out of the body) is malformed and visible either on the top surface of the penis (for boys) or between the labia minora and labia majora (for girls)
- widened pubic bones that don’t join together as they should; outwardly rotated legs and feet
- abnormal genitalia
- In boys, the penis may be short and/or curved upward. The testicles may be positioned abnormally.
- In girls, the clitoris and labia minora may be separated. The vaginal opening may be narrow, and the urethra may be shorter.
- vesicoureteral reflux (VUR)– a condition in which urine can travel back to the kidneys rather than draining down the ureters, into the bladder, and out through the urethra
- displaced belly button (umbilicus) and/or umbilical hernia
How is bladder exstrophy diagnosed?
Some cases of exstrophy of the bladder are diagnosed during a routine fetal ultrasound or MRI during pregnancy. If ultrasounds do not detect the abnormality, it will be diagnosed immediately after birth.
How do you treat bladder exstrophy?
Bladder exstrophy can be repaired with surgery. At Beaumont Children’s, we have a multidisciplinary team of specialists that will work together to treat your child. Our team includes pediatric urologists, reconstructive surgeons and orthopedic surgeons, among others.
Each case will require an individual treatment plan, as each person with bladder exstrophy presents with a unique set of circumstances. The most common surgery is called complete primary repair of exstrophy (CPRE).
This reconstructive surgery involves procedures to correct all abnormalities at once, including:
- closing the bladder
- reconstructing the bladder neck
- repairing epispadias (if necessary)
- closing the pelvic bones (pelvic osteotomy)
After CPRE, additional surgery and/or therapy may be necessary to help your child develop urinary continence both during the day and at night, have normal kidney function, and have genitalia that is as cosmetically normal as possible. One common additional surgery is done to repair vesicoureteral reflux (VUR).
There are other surgical options for bladder exstrophy as well, including modern staged repair of exstrophy (MSRE). MSRE involves three surgeries to help reconstruct your child’s bladder. If this is a good option, your child’s doctor will talk with you about details.
When will you perform surgery?
If your child is going to have complete primary repair of exstrophy (CPRE), otherwise known as primary closure technique, we usually perform the surgery soon after birth. Some surgeons like to wait a couple days before surgery to give you and your child an opportunity to bond outside of the womb. Your child’s doctor will talk with you about options and discuss what he or she thinks is best for your child.
Most of the time, additional surgeries will be performed months later to give your child time to recover and grow stronger.
What happens after surgery?
Your child’s doctor will talk with you about what to expect immediately after surgery and during recovery. The recovery time will vary depending on the severity of your child’s bladder exstrophy.
Once your child leaves the hospital, our team will work with you and your child to come up with a treatment plan. Throughout treatment, we’ll collaborate with your child’s pediatrician to make sure care is coordinated and tailored to your child’s needs. We’ll do regular testing to make sure your child’s bladder and kidneys are functioning and developing as they should, and we’ll make the full spectrum of care available to your child and your family, including psychological services, such as counseling, if necessary. Our care extends from your child to the rest of your family because we know that the stress of having medical issues can affect all of you. If there’s anything you need during and after your child’s treatment, we’ll be here for you.