Johns Hopkins All Children's Hospital

Often, very sick children are thought to need time to build their strength before invasive surgery. For infants born with severe congenital diaphragmatic hernia (CDH), an often fatal developmental defect of the diaphragm, one physician at Johns Hopkins All Children's Hospital in St. Petersburg has found another way, and it is saving many more young lives. Rewriting the Book on Congenital Diaphragmatic Hernia "NATIONALLY, THESE KIDS are born and put on a ventilator. ey are resuscitated, and if they get better they are repaired at roughly four to six days of life," explains David Kays, M.D., a pediatric surgeon who recently joined Johns Hopkins All Children's Hospital as director of the new Congenital Diaphragmatic Hernia Program. "What has evolved nationally is that the sicker the child, the more physicians delay the repair—but that can result in worse survival. ey have it exactly backwards. We have found that the more severe these babies are, however, the earlier the repair should be done." Kays and his team scientifically define the severity by combining lung size measurements before birth with physiologic data obtained in the first hour after birth. "When indicated, we offer parents the choice to have the repair done in the first four to 12 hours," he added. "For the most severe patients, early repair gives them a distinct survival advantage." SAVING NOAH One of the first patients Kays operated on after joining Johns Hopkins All Children's was a baby named Noah. A routine prenatal ultrasound showed that his intestines, stomach and most of his liver had moved up into the chest through a very large diaphragm defect. Doctors in North Carolina said he would likely not survive and offered termination as the family's first option. e family relocated to St. Petersburg so they could give birth as close to Kays and his surgical team as possible. "Noah was on the severe end, but that's what we specialize in—the most severe kids," Kays recalls. "I reassured Noah's mom that there was hope for a good outcome." Soon after Noah was born, his diaphragm hernia repair was successfully completed, and he was placed on extracorporeal membrane oxygenation for about three weeks with limited mobility. Noah did well and is now expected to lead a normal life. "His brain appears to have survived in great shape, which is central to a great outcome. A future risk is the development of a recurrent diaphragmatic hernia because his left diaphragm is about 96 percent Gore-Tex patch, which doesn't grow as he grows," Kays says. "Over his lifetime, he has about a 10 to 20 percent chance of a reoccurrence." A UNIQUE, RESEARCH-BASED APPROACH e national survival rate for severe CDH is 30-50 percent, but Kays' approach achieves greater than 90 percent survival. "We are conceptually rewriting the book on surgical timing of CDH," Kays says. "e most exciting part of this work is seeing the real survival potential of these kids, even the severe ones." Kays is likely the only physician performing early repair of CDH based on scientific measurement of disease severity. Based on work he did while at the University of Florida in Gainesville, he presented and authored a report in the prestigious Journal of the American College of Surgeons in April 2016. For severe patients, the process typically involves four or more operations. "We tried this a lot of different ways," Kays explains. "We were conceptually open-minded to the different approaches to repair timing. We reviewed our experiences, analyzed our data and then published what we learned, hoping to teach it to other physicians. We hope to help other physicians think about this disease in new ways that will save more children." David Kays, M.D., holds Noah shortly before his discharge from the hospital. Summer 2016 15 Innovation

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