Congenital Diaphragmatic Hernia

What is Congenital Diaphragmatic Hernia?

Congenital diaphragmatic hernia (CDH) is a rare birth defect affecting approximately one in 3,000 fetuses. CDH occurs when the diaphragm (the muscle separating the chest from the abdomen) does not form properly during fetal development. This results in a hole in the diaphragm and allows the abdominal organs to migrate into the chest. This could mean having the intestines, stomach, spleen and sometimes liver in the chest. The presence of these abdominal organs in the chest compresses the lungs and prevents them from developing normally.
 
CDH is most often diagnosed before birth, around the 20-week ultrasound visit. There is a spectrum of severity in CDH patients, and the health risks and treatment often depend on the level of severity.

Why Choose Johns Hopkins All Children’s for Treatment of Congenital Diaphragmatic Hernia?

Dr. David Kays, director of Johns Hopkins All Children’s Congenital Diaphragmatic Hernia Program, and his multi-disciplinary care team are responsible for some of the best survival outcomes in CDH patients. The team treats each CDH patient differently and doesn’t have a “one size fits all” protocol.

Treatment includes:

  • Planned birth at Bayfront Baby Place, located on the third flood of Johns Hopkins All Children’s Hospital with easy, quick access to the neonatal intensive care unit 
  • Resuscitation and careful ventilation upon birth 
  • Variable timing of CDH repair (from six hours to six days of life) based on a scientific evaluation of each baby’s CDH severity, optimizing the benefits of repair, while minimizing the risks of surgery 
  • “Gentle” pressure-limited ventilation that eliminates pressure-induced lung injury and strict protection of the underdeveloped lungs
  • Utilization of ECMO when needed. Duration of ECMO is not pre-determined and may last a few days to a few weeks
  • Extremely high continuity of care during the course of hospitalization 
  • No routine paralysis, thus giving babies a chance to act as normal as possible in an abnormal environment 
  • Slow and deliberate recovery 
  • Discharge in one to three months

Read more about Dr. Kays' unique, research-based approach to treating congenital diaphragmatic hernia.

Appointments and More Information

For more information, visit Pediatric General Surgery. To make an appointment or to ask questions, please call 727-767-4170.