Consortiums Improve Proficiency in Bladder Exstrophy Care in U.S. and Overseas

Image of Study Participants

Watching 2-year-old Gianni Garrow scramble up and down the physician’s chair in the urology exam room, it’s difficult to imagine that he was born with his bladder exposed on the outside of his body. Six surgeries later, he no longer points to his belly as his “boo-boo” place — instead, he shows off his scraped knees, the hallmarks of toddlerhood.

The Garrow family traveled four hours from their New York home to visit the Urology team at Children’s Hospital of Philadelphia who are part of a unique multi-institutional consortium to increase their experience and proficiency in the care of bladder exstrophy, a rare, complex abnormality that occurs early in fetal development. The infant’s hips are splayed, and the abdominal wall doesn’t fuse properly, leaving an open bladder that protrudes through this defect.

Other parts of the infant’s “plumbing system” may not form or function correctly, including the opening of the urethra (epispadias) and the sphincter muscles that work together with the bladder to hold and release urine. In Gianni’s case, he developed inguinal hernias associated with bladder exstrophy that also needed to be repaired.

The Multi-Institutional Bladder Exstrophy Consortium (MIBEC), which formally launched in 2013, includes CHOP, Boston Children’s Hospital, and Children’s Hospital of Wisconsin. When an infant with bladder exstrophy is ready for surgery, the participating urology surgeons visit each other’s operating rooms to observe, provide helpful commentary, and record all of the procedures with high-definition video. The MIBEC program’s research goals are to evaluate both short and long-term outcomes of bladder exstrophy patients, including quality of life, continence, and body image.

The MIBEC team published study results in the June issue of the Journal of Pediatric Urology that describe how this real-time, collaborative teaching approach benefited a cohort of 27 patients who underwent complete primary repair of exstrophy (CPRE) from 2013 to 2015. With CPRE, the bladder closure, epispadias repair, and bladder neck reconstruction occur during the same surgery.

“This represents the most complex surgical correction that we get challenged to do in pediatric urology,” said Douglas Canning, MD, chief of the division of Urology at CHOP. “We all get better when we have more experience tackling such a complicated problem.”

Experience Needed to Enhance Surgeons’ Knowledge of Rare Condition

Only about 100 babies a year in the U.S. are born with bladder exstrophy, so each patient case that the MIBEC team encounters is a valuable opportunity to refine their CPRE technique and expertise. Many of these families gathered at CHOP’s campus in early June for the International Exstrophy Conference hosted in partnership with the Association for the Bladder Exstrophy Community (A-BE-C). Over the weekend, they shared their experiences of living with bladder exstrophy and heard updates about MIBEC’s progress.

Waiting for completion of the 12-hour-long surgery is stressful for parents and caregivers, and it’s just as exhausting for the surgical team. Every nuance of the CPRE procedure is under scrutiny so that the MIBEC surgeons can standardize critical steps, timing, and technical details.

For instance, Dr. Canning, who also is a professor of Urology in Surgery at Perelman School of Medicine at the University of Pennsylvania, recalled a “revelation” that occurred during one surgery he was performing that led to improved safety and consistency during a particular stage of the procedure. A Wisconsin colleague noticed that the blood supply to the infant’s penis became compromised when they attempted to pull the pubic bones together. The team made adjustments and subsequently modified the operation’s algorithm and monitoring techniques to avoid penile injuries.

Following the surgeries, the MIBEC team stays in close touch with the infants’ parents and caregivers. While Gianni was recovering at home after his operation in the fall of 2015, his parents would measure his urine output and take him for ultrasounds every few days. They sent the results to Dana Weiss, MD, an attending pediatric urologist at CHOP, who recognized that Gianni wasn’t voiding enough on his own. His bladder would fill up, but the urine would shoot back up through his ureters into his kidneys, putting him at risk for urinary tract infections. To solve this complication, Gianni had another surgery six months ago to re-implant his ureters.

“He’s been great ever since,” said his mother, Lisa Lafrazza-Garrow. “Looking at him, you would never know that he has had six surgeries so far. He likes anything to do with throwing balls, riding his bike outside, going to the playground. And he loves doughnuts.”

Because the CPRE operation usually is performed in the U.S. on babies who are only weeks to months old, the urologists must wait until children like Gianni reach their fourth or fifth birthdays to see who is truly able to hold their urine and who cannot. The urinary continence rate of children who underwent CPRE is currently about 20 to 25 percent, and the surgeons expect the continence to improve as the children age.

In the meantime, the MIBEC team will continue to track the children’s continence rates, review and compare video clips, and gather research data that will help them to improve patient outcomes. One project underway involves taking samples of the infants’ microbiome — the community of bacteria that is growing on their bladder plate — and analyzing them to better understand how to lower post-surgery infection rates, which stand at 60 percent.

“As families are learning about this work, our three institutions are getting more referrals,” Dr. Canning said. “The families want to come to a place that is studying this carefully and invested in solving this critical problem … It is expensive to do, but the payoff is in the little children who now are given a better chance of voiding normally.”

More Fertile Learning Opportunities Internationally

Across the world in India, this model for sustained collaboration has taken shape over the last decade as the International Bladder Exstrophy Consortium. It is based on a long-term commitment from visiting U.S. surgeons and a competent, skilled host team who are dedicated to reducing the burden of disease in this part of the world, which has about five times the number of live births with bladder exstrophy than in the U.S.

Aseem Shukla, MD, director of Minimally Invasive Surgery, division of Urology at CHOP, showed the International Exstrophy Conference audience photos from Civil Hospital in Ahmedabad, India, where they take care of every child for free. A group of clinicians from CHOP, Cincinnati Children’s Hospital, and Sidra Medical and Research Center in Qatar travel there once a year in January and stay for two weeks to complete as many bladder exstrophy closures as they have time to accomplish. They also meet with about 80 families for consultations and rigorous follow-up care.

“Not only are we doing our very best for these children,” Dr. Shukla said, “we also are learning an amazing amount of information on an accelerated time curve. Even in a busy U.S. hospital, we might see five to 10 bladder exstrophy closures a year. In India, we see 15 in a week. This is fertile ground for interchange and sharing of best practices.”

Dr. Shukla, who also is associate professor of Surgery in Urology at Penn, and colleagues reported the team’s outcomes for 75 of those patients in a scientific abstract that won a clinical research prize at the Societies for Pediatric Urology Annual Meeting held in May. Based on their findings, the study team concluded that the collaborators successfully performed reconstruction in older children (mean age was 3 years for the CPRE procedure), and about 25 percent of these patients are showing some level of urinary continence.

As word has spread about the center for bladder exstrophy at Civil Hospital, families come from all over the Indian state of Gujarat, which has a population of 60 million people, to get help. Many comprise the lowest socio-economic strata in India, so they are tremendously compromised when a child is born with bladder exstrophy.

Dr. Shukla shared the story of a boy born with bladder exstrophy in central India who was asked to leave his joint family hut because of the overwhelming smell of urine. He also was dismissed from several schools because he left wet spots everywhere. The ostracized 14-year-old met the team of visiting surgeons, and they decided to repair his bladder exstrophy, even though he was much older than the majority of patients they had treated.

“Afterward, he did exceedingly well,” Dr. Shukla said. “He now experiences a couple hours of continence at a time. For him, the village was transformed. He is back in school getting an education. The family has a picture of the surgical team on their wall that neighbors come and bow to, as if a divine intervention had happened in their lives. These are the impacts we can make with the work we do.”

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