Congenital Diaphragmatic Hernia

A congenital diaphragmatic hernia (CDH) is an opening in a baby’s diaphragm that causes the organs in the abdomen, such as the stomach and intestines, to develop in the chest cavity. If left untreated, CDH can prevent a baby from breathing properly, slow lung development and cause problems with the heart and digestive system.

At the SSM Health Cardinal Glennon St. Louis Fetal Care Institute, we understand the anxiety and worry you may have about a diagnosis of CDH. Our doctors are specially trained to help you and your baby, providing the timely treatment that puts you at ease and gives you baby the best start in life.

Our fetal care team is part of a Collaborative CDH Care Team which provides babies with CDH seamless care from prenatal diagnosis through childhood and beyond. After your delivery, we will care for your child in the Neonatal Intensive Care Unit (NICU) where they will receive extensive breathing support and other necessary interventions.

How is CDH Diagnosed?

A CDH is typically detected through a routine ultrasound which allows doctors to check the position of your baby's lungs and heart. In some cases, the CDH is not diagnosed until after the baby is born. Babies with CDH will have difficulty breathing right after birth. As a result, a chest x-ray after birth is performed to evaluate the lung and which organs have been displaced into the chest area.

How is the Severity of CDH Diagnosed?

We are able to assess the severity of this condition with the help of ultrasounds and other tests, including:

  • Fetal MRI to obtain a more detailed view of your baby’s organs
  • Fetal echocardiogram (echo) to assess the structural functioning of your baby’s heart

During our evaluation, we measure your child’s lung-to-head ratio, the liver position, the total lung volume, and the response of the fetal lungs to oxygen. We may also look for any abnormalities suggestive of a genetic cause of CDH.

While we do not know exactly what causes CDH, in up to 40% of cases, the CDH is associated with other birth defects, the most common being a congenital heart defect. In up to 20% of cases of CDH, there is an underlying genetic cause due to a chromosome defect or genetic syndrome.

What Happens at Delivery and Beyond?

Most babies with less severe CDH can be delivered vaginally. In this case, your baby is quickly evaluated by our neonatologists (pediatrician specialized in newborn care). Once your baby is stabilized, a surgery to repair the hole in the diaphragm can be performed.

After surgery, your baby must learn to breathe and eat without assistance. This can take several weeks. Our neonatologists and pediatric surgeons will work closely with the your family to navigate your baby’s time in the hospital, and follow-up care as their lungs continue to develop.

EXIT Procedure

In severe cases of CDH, we may recommend a special delivery procedure called EXIT (Ex Utero Intrapartum Treatment). During EXIT, you will give birth by C-section while asleep under general anesthesia. This will allow the surgeon to evaluate your baby’s airway and lung function while your baby’s placenta and umbilical cord remain attached. During this procedure, a breathing tube and ventilator machine is used to assist your baby's breathing. Once stabilized, your baby is fully delivered by cutting the umbilical cord.

When is ECMO Necessary?

If a baby's CDH causes severe lung and heart problems, then ECMO, or Extracorporeal Membrane Oxygenation, may be used shortly after delivery. It's a machine which allows the body to exchange vital gases like oxygen while bypassing the heart and lungs. As a result, ECMO supports a baby's circulation and gas exchange, giving their heart and lungs an opportunity to rest.

Continuum of Care

CDH is a complex condition that requires long-term follow-up care. Because CDH can cause underdeveloped lungs and problems with the heart and digestive system, it’s important to monitor your baby’s health throughout their development. Once you go home, a Collaborative CDH Care Program coordinator will assist with scheduling your baby’s follow-up clinic appointments and any future medical procedures or surgeries that may be needed. This continuum of care ensures that the specialists working with our CDH patients know the intricacies of your child’s unique medical situation and can provide the best level of care.

We understand that CDH can be a scary diagnosis. That’s why we’re available to help 24 hours a day, 7 days a week. For more information or to schedule an appointment, call us at 314-268-4037 or toll free at 877-SSM-FETL (877-776-3385).

While we can’t change the diagnosis, we can provide you expert care and support, helping your baby get the most out of treatment and life.

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