What is congenital diaphragmatic hernia (CDH)?

Congenital diaphragmatic hernia (CDH) occurs when the diaphragm muscle — the muscle that separates the chest from the abdomen — fails to close during prenatal development, and the contents from the abdomen (stomach, intestines and/or liver) migrate into the chest through this hole.

When the abdominal organs are in the chest, there is limited room for the lungs to grow. This prevents the lungs from developing normally, resulting in pulmonary hypoplasia (or underdeveloped lungs). This can cause reduced blood flow to the lungs and pulmonary hypertension (high blood pressure in the pulmonary circulation), as well as asthma, gastrointestinal reflux, feeding disorders and developmental delays.

CDH can occur on the left side, right side or, very rarely, on both sides. It can be life-threatening.

After a doctor has diagnosed CDH in a pregnancy, the next step is to be referred to a prenatal diagnosis center for additional testing and information. If a patient is referred to our Center for Fetal Diagnosis and Treatment (CFDT), a comprehensive multidisciplinary evaluation will be scheduled.

The health of a baby born with CDH can change unexpectedly, making it critically important to deliver within the hospital, where postnatal care will be received and all aspects of specialized care are immediately available in one location.

Treatment for congenital diaphragmatic hernia

Treatment for congenital diaphragmatic hernia involves guiding the organs back into the abdomen and surgery to close the hole in the diaphragm.

For babies born with CDH, every little detail matters and can impact the outcome. It is important that your baby be treated by a team with experience caring for babies with CDH. At Children’s Hospital of Philadelphia (CHOP), we see a high volume of babies with CDH each year. Your baby will be cared for using optimal care guidelines developed by our experienced multidisciplinary team.

For babies with the most severe cases of CDH, treatment before birth may allow the lungs to grow enough before birth so these children are capable of surviving and thriving. Fetoscopic endoluminal tracheal occlusion (FETO) is a fetal surgery procedure that may improve outcomes in babies with the most severe cases of CDH.

After delivery, babies with severely compromised or fragile lungs may require extracorporeal membrane oxygenation (ECMO), a temporary heart-lung bypass technique used to oxygenate the blood and allow the lungs to rest.

Surgical repair of CDH after delivery depends on your baby’s progress in the days following birth, and can occur as early as three days of life. Babies with CDH are extremely sensitive to noise and movement, so surgical repair of CDH is often performed by our Neonatal Surgical Team in the N/IICU so your baby does not have to be transported to the operating room.

Long-term follow-up by a team of experts is important to provide the best clinical care to your child.