Many pediatric urological complications can happen before, during, and after childbirth. Some of them are more serious than others, and some are more easily treatable. Bladder exstrophy is one of those conditions that affect the child as they are developing in the womb. Luckily, there are reconstructive procedures that can repair this congenital abnormality so that children can grow up and enjoy most of the same activities as others.
What is Bladder Exstrophy?
Bladder exstrophy is a developmental abnormality that happens when the lower abdominal skin does not properly form, causing the bladder and part of the urethra to be exposed to outside elements. The bladder is essentially turned inside out when the muscle and skin do not correctly close around it. The inner lining of the urethra is exposed on the top of a boy’s penis or between the separated left and right halves of the clitoris in girls.
Due to the bladder and urethra not being closed properly, the bladder cannot hold urine. Urine continuously leaks out and onto the exposed bladder and the surrounding skin. Bladder exstrophy is slightly more common in boys than girls.
How is Bladder Exstrophy Repaired?
A child born with bladder exstrophy requires some form of immediate treatment after birth. First, the bladder and urethra need to be protected from diaper material with a transparent adhesive dressing. Each child will be different when it comes to a specific plan of treatment that the primary care physician, pediatric urologist, and the urology health care team devise.
There are also different options for the repair; immediate or delayed. Immediate reconstructive surgery will be performed within the first two to three days of a child’s life, whereas delayed surgery will be around six to twelve weeks of age. Sometimes the delayed choice is preferred due to the small anatomy of a newborn. Allowing the body to develop more might be a safer repair option.
Bladder exstrophy reconstructive surgery usually involves multiple operations at different times in a child’s life as they mature to obtain the best results.
Initial Closure and Pubic Bone Repair
The first surgery is either done immediately after birth or delayed at approximately six to twelve weeks. This procedure closes the bladder, abdominal wall, and posterior urethra and reconstructs the belly button and pubic bone repair. Because the pubic bone is separated and spread widely apart in children with bladder exstrophy, correcting this abnormality when the bones are soft is more successful. Bringing the pubic bones closer together helps support the soft tissues of the pelvis.
Babies are put into a device, so their lower legs do not move. This device helps prevent the separation of the pelvic bones and aids in recovery.
Repair of the Urethra
If the initial surgery was delayed, this procedure could be combined with the first at approximately six to twelve weeks of age. The bladder should have increased in size at this point, and the urethra is closed and rerouted through the usual passageway. The ureters may be repositioned within the bladder if they are not connected in the right place.
Common Problems Associated with Bladder Exstrophy
Children with bladder exstrophy can experience problems at any stage of life after their surgical repairs are complete. Often, lifelong follow-up care is needed to help ensure the best quality of life.
Since the pelvic bones are spread widely apart in a child with bladder exstrophy, there is inadequate support of the soft tissue of the pelvis. Because of this lack of support, there is increased tension on the pelvic bones, leading to involuntary urinary leakage.
When the ureters are not joining the bladder in the correct place, urine can flow back toward the kidneys. This backflow can lead to kidney damage and recurring urinary tract infections.
A psychologist or licensed therapist may need to be considered part of the child’s health care team. They can help the child and the family deal with complex issues together.
What are the Risks Involved with Bladder Exstrophy Surgery?
Wound Site Problems
There is a risk that the wound site will not heal properly and open up, causing the bladder to move out of position. Another surgery is required to repair this problem.
The other risk is kidney damage from vesicoureteral reflux when urine flows back into the kidneys. If this condition is left untreated, it can lead to kidney failure.
As with any surgical procedure, there are some risks. However, the health care team will monitor both of these conditions throughout the child’s treatment plan.
What is the Long-term Outlook for Babies Born with Exstrophy?
Children born with bladder exstrophy can live a happy and productive life with an average life expectancy. Many people have a normal sexual function and can have children of their own. Some males with bladder exstrophy may experience fertility issues. However, there are many treatment options available for that and can be discussed with a doctor.
Intermittent Catheterization or Urostomy Surgery Later in Life
During a child’s journey with bladder exstrophy, there may come when they are unable to empty their bladder on their own completely. In this case, the health care team can determine if the use of pediatric intermittent catheters might be an alternate approach. If a more long-term drainage solution is needed, a urostomy can be surgically created as an alternate opening for access to the urinary system. The child will then have complete control over urine flow from their body using an ostomy pouching system with a drainage tap.
Most children with bladder exstrophy go about their daily lives without anyone ever even knowing what is different underneath their clothing. They participate in sports and other activities much the same as any other child. The success stories are endless from families that have had a child undergo bladder exstrophy surgeries.
For any questions related to incontinence products, intermittent catheters, ostomy supplies, or any other product that we carry to manage medical conditions, our Product Experts are available to assist. Our compassionate and knowledgeable team will make it easy for you to choose the right products for your needs so you can get back to doing the things you enjoy most.