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December 20, 2001
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Johns Hopkins Researchers Find More Extensive Bone Defects Caused by Bladder Exstrophy

Researchers at Johns Hopkins Children's Center and St. Vincent de Paul's Hospital in Paris have learned that bone defects associated with classic bladder exstrophy are more extensive than previously thought. Their findings, reported in this month's Urology, will enable surgeons to better correct these bone defects that cause the bladder to develop outside of the body.

"We believe surgeons already do a great job," said Children's Center Director of Pediatric Urology John P. Gearhart, M.D., who directed the research. "But this information will further help the few surgeons who do this procedure to provide a long-lasting fix for these children."

Classic bladder exstrophy, which occurs in approximately one of every 30,000 live births, is a defect that affects an infant's pelvic bones, genitorurinary ducts, and leaves a hole in the abdominal wall through which the bladder emerges.

The researchers reviewed abdominal CT scans from seven infants with classic bladder exstrophy as well as CT scans of 26 infants who had them taken for other reasons. The team then compared the geometry of pelvic girdle bones in the bladder exstrophy group of infants with the normal group, looking for statistically significant differences.

The team learned that pelvic girdle bone defects in bladder exstrophy cases were more extensive than previously thought. Among the most pronounced differences were an outward flare of the upper part of the hip bone and a rotation of the entire pelvic girdle from the bottom forward.

Gearhart says the research broadens surgeons' understanding of those bone defects so that more effective adjustments can be made in realigning and repositioning bones of the pelvis. Gearhart also says the findings suggest a possible explanation for the mysterious muscle weakness some women born with bladder exstrophy have shortly after they give birth.

Researchers at the Johns Hopkins School of Medicine and the Department of Pediatric Surgery at St. Vincent de Paul's Hospital in Paris also contributed to the study. It was funded by grants from the National Institutes of Health and the Johns Hopkins Department of Urology.

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