Johns Hopkins All Children's Hospital

Fall 2017

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4 Johns Hopkins All Children's Hospital | HopkinsAllChildrens.org Improving Survival for Infants with Congenital Diaphragmatic Hernia Pediatric surgeons at Johns Hopkins All Children's Hospital are pushing survival rates for infants with CDH above 90 percent. CONGENITAL DIAPHR AGMATIC HERNIA (CDH) is a defect that results in an often large hole in the diaphragm, more commonly on the left side, in about one in 3,000 pregnancies. Depending on its severity, CDH allows organs such as the intestines, stomach and even the liver to move into the infant's chest cavity, threatening lung development. Many believe the survival prospects for an infant with CDH are bleak, but Johns Hopkins All Children's offers hope. While the national survival rate for this condition is between 65 and 70 percent, infants treated at Johns Hopkins All Children's experience a survival rate of greater than 90 percent. A significant factor contributing to this increased survival rate is the approach taken by Johns Hopkins All Children's surgeons and medical staff. "When we look at an infant with a prenatal diagnosis of CDH, the No. 1 thing we do is believe they can survive," says David Kays, M.D., FAAP, FACS, professor of surgery, director of the CDH program and director of the extracorporeal life support program at Johns Hopkins All Children's. "We also believe they must have their CDH repaired. To the layperson this may seem obvious, but there is significant concern nationally that repair surgery makes an already sick child less stable. We know, however, that the more severe a child is on the spectrum of CDH, the more critical it is to perform the repair surgery." FOLLOWING THE PROCESS CDH presents on a spectrum of severity, based on how much the condition has affected an infant's lung development. "e more severe the patient is, the more reluctant most surgeons are to do the repair, out of fear of making the infant worse," Kays says. "is is where we differ from the national conversation. Surgery doesn't make little lungs into big lungs, but it helps little lungs be as big as they can. Repair surgery is critical to helping these infants use the lungs they do have." Kays' approach has five key components: Belief the infant can survive Prevention of any damage to the lungs through resuscitation and careful ventilation upon birth Repair surgery conducted at most optimal time for repair benefits, with minimized risk to the infant Use of high-quality extracorporeal membrane oxygenation (ECMO) Offering ECMO to even the most severely affected infants when needed LOOKING TO THE FUTURE "Our long-term goal is to have a large program hereā€”one that includes publishing research and teaching fellows and residents how to better manage these patients to improve survival rates for 4 Johns Hopkins All Children's Hospital | HopkinsAllChildrens.org Surgical Advances

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