Johns Hopkins pediatric urologist John Gearhart discusses his team’s latest research comparing orthopaedic complications between nonstated and staged osteotomies for cloacal exstrophy, which was presented during the American Urological Association’s 2024 annual meeting. Cloacal exstrophy is a rare birth defect that occurs in one in 400,000 live births. Click here to view the results from this research.
Hi, I'm John Gerhardt. I'm the Jeff's professor of pediatric urology at the Brady Urological Institute of Johns Hopkins and the Charlotte Bloomberg Children's Hospital also at Johns Hopkins. Today, we're gonna talk about the use of osteotomy in the major congenital birth defect known as cloacal Troy. Choicy troy occurs one in 400,000 live births. So it's rare. We follow 100 and 75 patients with Cecy Troy here at, at the Bradi Institute and we see about seven new patients a year, which is the world's experience, but it's still a very rare birth defect. One of the major parts of the birth defect of cloacal atrophy is that the pubic bones are widely separated in a normal person. The pubic bones are separated about a centimeter. Uh in cloacal troy, the pubic bones on average are seven centimeters apart and what lies between the pubic bones that are open are the intestines and the bladder. So this is a major congenital birth defect and fairly easily diagnosed on prenatal ultrasound studies in order to bring the pubic bones together and to get the viscera, the bladder, the intestines back inside the pubic bones need to be brought together without tension. In the late eighties, early nineties people would do this operation in one step, putting the intestines back inside the bladder back inside, cutting the pubic bones in one spot and then bringing the pubic bones together. And this did improve the success rate of the treatment of cloacal. Actually from about uh 50% without an osteotomy to about 80% or so with an osteotomy. We noticed in a small series that we did with Doctor Paul Sponseller, our professor of pediatric orthopedic surgery here at Hopkins, that if we cut the pubic bones in two places, put some fixating pins on and gradually gradually over 2 to 3 weeks, brought the pubic bones into closer apposition. We could then place the intestines and the bladder back inside the abdomen without tension. This improved the security of the operation and also improved the safety of the operation. So, the purpose of the paper that we just presented at the American Neurological Association in San Antonio in May was to compare the single stage repair, putting everything back inside, cutting the bones, bringing it all together in one time versus doing the osteotomy, placing the fixator device and then gradually cranking things together over 2 to 3 weeks and then putting the bladder and the intestines back inside. And we looked at these two groups very carefully and we found that there's no higher risk, no higher complication rate by doing the osteotomy and gradually cranking uh the pubic bones together, uh and then putting the viscera and the bladder back inside. And this didn't surprise us a lot because we certainly felt it was safer. We've done this now in 100 and one total patients here at Hopkins and the success rates are over 90%. So we feel comfortable with this, that this is the best thing for the child. Uh This is better for the safety of the operation. It helps not only the pediatric orthopedic surgeons and the pediatric urologist, but also our pediatric general surgeons who are involved in this too. So we highly recommend this. We've published this uh in the journal of pediatric surgery just a few short months ago and we hope that this will be adopted by our colleagues at other major institutions around the world. Thanks for listening. Uh I'd like to thank Doctor Paul D Sponseller, my colleague of over 35 years for uh helping us with this and Doctor Isam Nasser of the pediatric surgery service, who is our colorectal surgeon, who's involved in the reconstruction of these uh young patients. And doctor Richard Radet, uh and doctor Robin Yang of the pediatric plastic surgery service who are intimately involved in helping us in the reconstruction of these infants. I hope you enjoyed this presentation and I hope this will uh add your clinical armament when you're dealing with your patients. Thanks so very much.
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