Innovation in Pediatric Urology: Treating Bladder Exstrophy at Nationwide Children’s Hospital
MD, Chief of Urology at Nationwide Children’s Hospital
V. Rama Jayanthi
Nationwide Children’s Hospital is one of the largest pediatric hospitals and research institutes in the United States. It is consistently ranked as one of America’s top 10 children’s hospitals by U.S. News & World Report, the recognized authority in United States hospital rankings. V. Rama Jayanthi, MD, leads the Department of Urology, which is also regarded as one of the top programs of its kind in the country. “Hospitals” magazine recently interviewed Dr. Jayanthi about Nationwide Children’s Hospital’s approach to a rare and complicated condition, bladder exstrophy.
What is bladder exstrophy?
Bladder exstrophy is a congenital condition, or birth defect, where a child is born with a bladder that is malformed and is sticking through the abdomen – that is, the bladder is exposed outside of the body. The bladder does not function correctly, so children with exstrophy are incontinent. There may also be future issues with the reproductive and other body systems as well. Exstrophy is very rare. Many surgeons can go years without seeing a single case. It’s important that the urology team treating a child with bladder exstrophy has the appropriate training and experience.
How is bladder exstrophy treated?
The condition is ideally treated with surgery to close the bladder soon after birth, but most children have one or more additional procedures as they grow. Those are often designed to make children continent or have some control over the way urine is removed from the body, because their bladders likely will never be able to function normally. Boys with exstrophy also need surgery to create a urethra that goes to the end of the penis. The outcomes of these surgeries have a huge impact on a child’s everyday life and on the lives of their family members.
What makes your approach different?
The Urology team at Nationwide Children’s Hospital has a crucial focus on our patients’ quality of life. Worldwide, many children undergo a surgical procedure in which the appendix is connected to the bladder and the abdomen. The child then catheterizes him/herself every few hours to remove urine, so their underwear and clothes remain dry.
This is a common option and works well, but means patients have to catheterize every few hours for the rest of their lives.
We have developed a modified procedure allowing urine to be diverted to the rectum so that it can exit the body that way. Those children can then use the bathroom and do not need to catheterize. For those children and their families, this can be life-changing since their day-to-day routine is relatively “normal”. In our experience, urinary tract infections and other complications that can be a risk with other procedures have not been as great with our modified procedure. I should also say because there are so few cases of bladder exstrophy, to begin with, we do not have long-term evidence that would allow us to say this approach is definitely better than any other one. We do feel it has advantages for some people, though, and the families who have chosen this approach are quite pleased with the outcome. The procedure is not necessarily appropriate for every child, but it has been a successful option for our patients in recent years.
How have patients and families responded?
Families who have children with bladder exstrophy have often done a lot of their own research because they want the best possible outcome. They connect with other families online to learn about their experiences. And they are most often going to find that the typical ways of correcting bladder exstrophy are the only options they have. We offer another option, which may be helpful for some. In our most recent experience, a family who lives far away from our hospital sought us out for this different option, because they knew the way it could positively affect their child and their own lives. Their child has had no infections and is able to go to the bathroom like a “normal” child. It has had a big, beneficial impact on them.
Why is Nationwide Children’s Hospital leading in this area?
One of the most important reasons is our desire to collaborate – both inside our institution and outside of it. Inside our hospital, we realize that children with some severe forms of exstrophy will need treatment from multiple surgeons: urologists, colorectal specialists and gynecologists. Through our Center for Colorectal and Pelvic Reconstruction, we bring together those and many other experts so that children can have their medical issues addressed holistically.
We also have helped create a consortium of large hospitals, the Pediatric Urology Midwest Alliance, which allows us to learn from each other. That’s especially helpful in developing expertise with rare conditions like bladder exstrophy and in conducting research on those conditions.