Urology

A milestone in complex bladder surgery at Hopkins Children’s

Michael Triebsch leans in for a closer look at the portrait of a robust, dark-haired man, sporting the equivalent of early 20th century bling. He reads aloud the portrait’s plaque: “Diamond Jim” Brady. “I thought Brady was a doctor,” he says, turning. “Is that who I think it is?”

Pediatric urologist John Gearhart and pediatric orthopaedic
surgeon Paul Sponseller coordinate care for young patients

Yes, indeed. The famous Brady Urological Institute at Johns Hopkins is named for the American millionaire and businessman who in 1912 endowed the urological institute, in appreciation for the corrective surgery there that others had told him was impossible.

His portrait dominates the institute’s library at The Johns Hopkins Hospital. And, nearly 100 years later, his philanthropy combined with the contributions of the founding chief of pediatric urology at Hopkins, Robert Jeffs, and his successor, John Gearhart, has helped restore healthy futures to hundreds of children, thanks to another surgery that others once said was impossible.

In the library that morning, July 26, Michael and Ann Triebsch’s 10-year-old son, Adam, was photographed with Gearhart, in commemoration of the now 1,000 children with bladder or cloacal exstrophy who have been cared for in the Division of Pediatric Urology of the Brady Urological Institute. The young Louisville, Kentucky, resident had undergone, just weeks earlier, a series of scheduled corrective surgeries, led by Gearhart and pediatric orthopaedic surgeon Paul Sponseller.

Exstrophy surgery was considered experimental and cutting edge in the 1970’s, when Jeffs first performed a staged approach to correcting the congenital urogenital anomalies. Children with bladder exstrophy are born with their bladder outside of their body, due to the abdominal wall’s failure to close during development in utero.

With the very rare and more complicated cloacal exstrophy, children are born with many of their inner-abdominal structures exposed, including the bladder and intestines. Their genitalia can be split and their spine affected. Multiple surgeries over many years can help correct many of the related urogenital, intestinal and spinal issues.

Before Jeffs's pioneering approach in the 1970’s, surgical repair of bladder exstrophy was so limited in scope that for many affected males one solution was to remove their genitals and raise them as girls. Most children with cloacal exstrophy died soon after birth.

For children with bladder exstrophy, says Gearhart, “Jeffs took a major birth defect which consigned children to a reclusive, dismal existence and devised a novel technique to repair it, basically altering the lives of thousands of children throughout the world." Gearhart succeeded Jeffs as director of pediatric urology in 1996. Jeffs died in 2006.

Today, Jeffs's technique, since improved upon by Gearhart and his colleagues at Hopkins Children’s, is used throughout the world. Bladder exstrophy, while still a major birth defect, is now among the most readily repaired at birth, in the right hands. Further discoveries and surgical refinements by Gearhart and Sponseller have clearly defined the anatomical nature of this unique defect and improved the quality of life of children. Through their efforts the Brady Urological Institute has become the “center of excellence” for failed bladder-cloacal exstrophy patients who come from around the world for a “second chance” and sometimes a “third chance” of success.

The Triebschs delivered their son into Gearhart’s care at the recommendation of a former pediatric urology fellow at Johns Hopkins, Bruce Slaughenhoupt, who happened to be on call in the Kentucky hospital when Adam was born in 2000. “It was an incredible twist of fate that exactly the right person was exactly where we needed him that day,” says Ann Triebsch. “He instantly recognized Adam’s complex issues as cloacal exstrophy and told us that the place to go was Johns Hopkins and into the care of Gearhart and his team.”

The family now travels from Kentucky to Hopkins Children’s regularly for Adam’s ongoing surgeries. “It’s a long process,” says Triebsch. “We just love this place, though, and the team taking care of Adam is so kind and compassionate.”

Of the surgical milestone reached July 23 at Johns Hopkins, Gearhart says: “It’s a tribute to the great legacy started by Dr. Jeffs and upon which we’ve had the good fortune to build upon over the last 25 years. We took a birth defect that would otherwise consign children to a dismal future and made it repairable.”

A formal celebration is planned for the fall at Johns Hopkins. “Many thanks go out to the Brady family,” Gearhart continues, “and the many contributions over the years that have helped alter the lives of thousands of children throughout the world, and now over 1,000 at Johns Hopkins alone.”

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