Urology

Bladder Exstrophy Central

John Gearhart, master of repair and
reconstruction.

For about 10 hours on April 7, pediatric urologic surgeon John P. Gearhart operated on a 2-year-old boy from Israel with bladder exstrophy, a major birth defect occurring once in 40,000 live births in which the bladder develops inside out. The protruding bladder splits the two pubic bones, and the skin of the lower abdominal wall, which is supposed to cover the bladder, doesn’t, leaving the bladder exposed and leaking on the top of the abdomen.

This particular boy had already been through two failed surgeries in the Middle East. “The state of his ladder was so bad, and it was just so small,” Gearhart explains, “that we had to make him a new one.”

To do that, Gearhart borrowed about 10 inches of tissue from the boy’s large intestine. He then connected greeters from the kidneys into the new bladder so that urine could drain properly. Finally, he attached one end of the boy’s appendix—a hollow, muscular tube—into the new bladder and the other side into the base of the navel. By passing catheter into his navel, the boy will now be able to empty his urine properly.

Johns Hopkins Children’s Center surgeons, led by Gearhart, perform these manmade bladder procedures about 20 times a year.

Since they began operating to correct these abnormalities more than 30 years ago, they’ve tracked 962 cases—and still counting. They successfully repair about 98 percent, and about 75 percent are done at an early enough age so that the child can live without a catheter.

Most of the patients referred to Gearhart (who alone has done 223 surgeries) need repair of operations performed at other hospitals that have failed. “Most surgeons,” Gearhart says, “just haven’t seen very many bladder exstrophies. Our team treats more cases than any center in the world.”

These researchers also have been conducting long-term follow-up. In girls with the condition—who are three times less likely to have it than boys—they are looking to see if the surgical repairs done at birth improve fertility and lower the risk of having a miscarriage or uterine prolapse later in life.

To discuss a case or refer a patient call +1.443.287.6499.

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