Congenital diaphragmatic hernia (CDH) is typically discovered during the 20-week ultrasound. The experts in the CDH Center at Johns Hopkins All Children's Hospital in St. Petersburg, Florida, can answer your questions about CDH and guide you through your baby’s treatment options.
Infant without Congenital Diaphragmatic Hernia
Infant with Congenital Diaphragmatic Hernia*
*The illustration above showing an infant with CDH shows Left CDH, with the small bowel, colon, stomach, spleen and portion of the liver in the chest. The heart is also shifted to the right, causing additional compression and growth restriction of the right lung. Artist retains copyright and reproduction rights (©Robert Margulies)
What is CDH (congenital diaphragmatic hernia)?
Congenital diaphragmatic hernia (CDH) is a birth defect in which the diaphragm—the muscle that separates the abdomen from the chest—does not develop completely. This causes a hole where abdominal organs can migrate into the chest. When that happens, it doesn’t leave enough space for the lungs to develop normally, making it hard for the baby to breath.
When is CDH found?
CDH is typically found during the 20-week ultrasound. CDH affects about one in every 3,000 pregnancies, occurring with relatively high frequency for a birth defect.
Why choose us?
Johns Hopkins All Children’s created the first inpatient unit in the country dedicated solely to the treatment of CDH in St. Petersburg, Florida. Infants treated here experience a survival rate greater than 90%, even in severe cases of CDH*, which exceeds national benchmarks. Whatever you may have been told about your child’s chances with CDH, we urge you to speak with our team and learn about options for your baby.
Each CDH case can vary greatly
The hole caused by the undeveloped diaphragm may be small and allow a limited amount of intestine into the chest. Or it may be large and allow a greater amount of abdominal contents into the chest. This can include the intestine, stomach, spleen and liver. The amount of abdominal contents that migrate into the chest affects whether the lungs are moderately small or severely small. Our experience has shown that the vast majority of CDH infants do have enough lung to survive, but it requires expert management to help those with very small lungs survive.
CDH Treatment: Expert care to help lungs grow
Treatment requires surgery to gently move the organs that have migrated back into the abdomen and close the hole in the diaphragm. This creates space in which the lungs can then grow, with careful care and monitoring from your baby’s physician team.
Our team uses a “kinder, gentler ventilation” for CDH infants born with critically small and fragile lungs. This process prevents harm to the delicate lungs of our patients. Extracorporeal membrane oxygenation (ECMO), a long-term form of heart-lung bypass, is used when a baby’s lungs aren’t able to rid the body of carbon dioxide or provide the body with enough oxygen. Babies requiring ECMO have their CDH repaired within the first 36 hours after ECMO initiation.
Optimal timing for repairing CDH
David Kays, M.D., has spent decades studying the precise timing for CDH repair, which allows lungs to grow to the fullest extent possible. The success of CDH treatment often depends on whether other anomalies are present. CDH by itself or with other non-life-threatening conditions, such as a single kidney instead of two, can often result in a successful outcome with proper management.
In 10 to 12 percent of cases, we find bilateral CDH—meaning it is on both sides of the abdomen—or CDH with other life-threatening conditions such as severe heart defects or major genetic or chromosomal defects. We evaluate cases where additional life-threatening conditions are present on an individual basis, but the very low survival and high-risk outcomes in these patients is directly related to the additional anomalies.
*While the majority of infants with CDH have an isolated CDH defect or only minor associated anomalies, infants with severe CDH plus a highly severe second anomaly, such as uni-ventricular heart defect, severe chromosomal defects, or severe bilateral CDH, face complex physiologic challenges that are addressed on a case-by-case basis.
Questions? Give us a call
Our team is here to answer questions and discuss concerns you have about your baby.
Request an appointment
Submit a request for an appointment and our team will reach out to you to discuss options.
Traveling for care?
We treat children from across the country, and the world, in the CDH Center at Johns Hopkins All Children’s in St. Petersburg, Florida. We have several resources to assist you and your family as you travel for care, including information about requesting a room at one of several Ronald McDonald Houses on our campus.
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Read inspiring stories about our families:
Congenital diaphragmatic hernia (CDH), a rare birth defect, occurs in about one in every 3,000 live births. It is even more unusual for a family to have more than one baby with CDH. But parents Ally and Jimmy were comforted by the care of David Kays, M.D., and the Center for Congenital Diaphragmatic Hernia at Johns Hopkins All Children’s Hospital.
When David Kays, M.D., was a child, his mother gave him a book about medicine, helping to set him on the path to becoming a surgeon. Now he is one of the best in the nation at treating congenital diaphragmatic hernia (CDH), and serves as medical director of the hospital’s Center for CDH, the nation’s first inpatient unit solely dedicated to treating CDH. He talks about his journey to medicine, and how he came to specialize in CDH.
Babies with congenital diaphragmatic hernia (CDH) are often diagnosed before birth, but for Birdie, the diagnosis didn’t come until shortly after she was born. She and her family were referred to the Center for CDH at Johns Hopkins All Children’s, where babies like Birdie receive the specialized care they need to grow and thrive.
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