Congenital Diaphragmatic Hernia (CDH) Frequently Asked Questions
Common Questions After Receiving a CDH Diagnosis
How did my baby get CDH?
Congenital diaphragmatic hernia (CDH) is a birth defect, meaning it happens while the fetus grows in the uterus. During pregnancy, different organ systems develop and mature. CDH usually occurs during the 7th and 12th weeks of pregnancy as the diaphragm, esophagus, stomach and intestines are developing. As development continues, the diaphragm forms abnormally with a defect on either the right or left side. Through this defect in the diaphragm, some of the organs that are normally found in the abdomen move up into the chest cavity, leading to small lungs (pulmonary hypoplasia) and high blood pressure in the lungs (pulmonary hypertension).
Can CDH be prevented? What causes CDH?
There is no single known cause of CDH. It is not related to anything a mother did or did not do during the pregnancy and is not preventable.
How common is CDH?
CDH is rare and occurs in about 1 in every 2,500 newborn babies. That said, CHOC is a destination center for CDH and sees a very high volume of these patients each year. Our expert team is experienced in managing even the most severe forms of this disease.
Can babies survive CDH?
While CDH is a life-threatening illness, babies can survive with the appropriate treatment and care. It is therefore important to establish the diagnosis of CDH before birth. If your baby has been diagnosed with a diaphragmatic hernia, you should plan for a consultation with a team of pediatric CDH specialists, including a maternal-fetal medicine specialist, pediatric surgeon, fetal cardiologist, pediatric pulmonologist and neonatologist, to prepare for the birth and subsequent care of your baby.
What happens if CDH goes untreated?
If left untreated, CDH can lead to many life-threatening conditions. Two major problems associated with CDH are pulmonary hypoplasia and pulmonary hypertension. Pulmonary hypoplasia is a small lung that is sometimes inadequate to bring enough oxygen to a baby’s body. Pulmonary hypertension is the abnormal tight squeezing of the blood vessels in the lung. This causes the baby’s heart to pump extra hard against these tightly constricted blood vessels. When untreated, both pulmonary hypoplasia and pulmonary hypertension can lead to hypoxia (not enough oxygen for the body), heart dysfunction and sometimes even death.
How often will I meet with doctors during pregnancy?
Congenital diaphragmatic hernia can often be detected on fetal ultrasound in the second and third trimesters of pregnancy. Your baby’s specific diagnosis, which largely depends on the severity of their condition, will determine individual follow-up appointments during pregnancy. At The Fetal Care Center of Southern California, your detailed treatment plan will be developed in conjunction with your OB or MFM to determine the care for you and your baby.
Does it matter where I deliver my baby?
Babies with CDH require immediate care in a neonatal intensive care unit (NICU). Therefore, it is optimal that your baby is delivered in or near a hospital that has a level 3 or 4 NICU. This will allow for your baby to receive care immediately after birth from neonatologists, pediatric surgeons, specialized nurses, respiratory therapists, pulmonologists and other important members of the care team. CHOC offers the only Surgical NICU on the west coast. Our NICU is a Level 4 NICU, which is the highest level available because of the complex conditions we treat. While CHOC does not currently offer delivery services, you will be recommended to one of two nearby hospitals with leading delivery centers. Your baby will then be safely transported to the NICU after delivery.
Can I deliver my baby vaginally? Is it safe?
Yes it is safe. In fact, most babies with CDH are born vaginally, either naturally or through induced labor. Some babies, however, can also be born via cesarean if necessary. It is important to discuss options prior to birth with your delivery team.
Common Questions About CDH After Delivery
When will my baby have CDH repair surgery?
Babies will have surgery to repair the diaphragm a few days after birth once their condition is stable. After delivery, your baby will be brought to the NICU where they will remain for the rest of their stay. Our doctors will insert a breathing tube (also known as an endotracheal tube) into your baby’s mouth and a machine called a ventilator will help your baby breathe. Another tube will be placed into your baby’s nose and threaded through the esophagus into the stomach. This will keep your baby’s stomach empty so that their lungs have as much room in the chest as possible to expand.
How can I help my baby while they’re in the hospital for CDH treatment?
You’ve already made the best decision to help your baby by choosing CHOC for your baby’s CDH care. Although the national survival rate for CDH is roughly 60-70%, the survival rate for CDH patients at CHOC is 88%. Our team of multidisciplinary pediatric CDH specialists will work together to provide your baby with the best possible treatment for their condition. Learn more about CDH care at CHOC.
However, our specialists are only part of your baby’s team. At CHOC, our focus is on patient- and family-centered care, and we believe a baby’s parents or guardians are important partners in the care we provide. We encourage families to bond with baby during their hospital stay to help them as they receive treatment. Babies, even those being monitored or receiving support by machines, have very positive responses to their parents’ closeness—whether it is a gentle caress with a fingertip, skin-to-skin cuddling or reading a book. As each baby’s plan of care is unique, talk to your medical team about the best ways to bond with your baby during their NICU stay.
Does CHOC offer FETO treatment for CDH?
CHOC does not yet offer fetoscopic endotracheal occlusion (FETO) to repair congenital diaphragmatic hernias (CDH). However, we do work in collaboration with other CDH centers that offer this experimental procedure. If your baby is a candidate for FETO and you choose to undergo this special procedure, your care team at CHOC will help get you to a center that can perform FETO. Your care team at CHOC will work with their team to coordinate your fetal treatment and your return to CHOC for follow-up care through the remaining duration of your pregnancy.
How long will my baby be in the hospital for CDH treatment?
Many parents want to know how long their baby will be in the hospital, but every case is different, and recovery is dependent on the severity of your baby’s CDH. Hospital stays for babies with mild CDH typically last between 3-4 weeks, while more severe CDH cases can range anywhere from 3-6 months and occasionally longer.
Can I breastfeed my baby with CDH?
CHOC is committed to supporting mothers who wish to breastfeed. Our nutritional recommendations encourage all mothers to breastfeed or pump breast milk for their bab. While parents may not be able to immediately breastfeed their baby with CDH, once our team feels the baby is ready, they will be fed very small amounts of breastmilk. If the baby is still on a ventilator when it is time to feed, they will be fed through a small tube placed into the stomach through the nose. This is called a nasogastric feeding tube or “NG tube.” If the baby is not on a ventilator, we will begin bottle feeding under the close supervision of our feeding team.
In the CHOC NICU, pumping rooms are available in the unit, as are portable pumps so that you may pump at your baby’s bedside. Our Nutrition Lab will store and track your breast milk for you. We have lactation consultant RNs, along with many of our nurses and dietitians, who are specially trained to work with you in the NICU.
How do I fly home if my baby is on oxygen?
Your baby’s doctor and care team will determine the best time for transport, and the right amount of oxygen for the trip. If your baby is on oxygen, CHOC will provide a doctor’s statement that showcases to airlines the necessity of your child’s oxygen tank and equipment.
Will my child have any long-term issues because of CDH?
Babies can have different types of problems requiring long-term care and follow-up. Long-term outcomes are usually better for babies with CDH in cases where the liver remains down in the abdomen when the defect forms before birth and when pulmonary hypoplasia and pulmonary hypertension are mild.
A few long-term issues that children might experience because of CDH:
- Many babies develop chronic lung disease and may require oxygen or medications to help them breathe long-term.
- Gastroesophageal reflux is a common long-term issue for babies with CDH. This is when acid and fluids from the stomach move out of the stomach and up into the esophagus. This can cause irritation, vomiting, feeding problems, lung problems and growth difficulties.
- Some babies have will have difficulty growing, known as failure to thrive. This is especially common in babies with more severe lung problems.
- Some babies experience developmental delays and may not roll over, sit, crawl, stand or walk at the same time as healthy babies. Developmental therapy is an important part of long-term care. Physical therapy, speech therapy and occupational therapy are offered at CHOC and as a part of the discharge plan to help babies achieve their greatest potential.
Will all my children have CDH?
There is recent evidence that genetics may be related to CDH. Parents who have had one child with CDH are at increased risk to have another child with the same birth defect. However, the likelihood that all your children will have CDH remains low.
Common Questions About CHOC’s CDH Care
What resources does CHOC have to support my family during my baby’s treatment?
We understand a baby’s CDH diagnosis can be scary for families and our team at The Fetal Care Center of Southern California is here to support you every step of the way. Our team of fetal nurse coordinators will work with you to schedule all needed appointments and coordinate treatment with your MFM or OB provider. Additionally, treatment at our Fetal Care Center brings your full treatment team to you within a single center at CHOC, making appointments easy for you and your family.
Once your baby is born and staying in the NICU, we set up video monitoring for families to watch over their baby 24/7, as well as private rooms for families to stay within our NICU as needed. Learn more about resources available within our NICU. Treatment at CHOC also provides access to the hospital’s full suite of pediatric services should your baby require treatment or care for any co-existing conditions.
CDH is a serious condition. As such, CHOC understands that some families may choose to travel out of state to receive treatment for their baby at CHOC. Even if you’re not from out of town, we know that many families will want to remain close to their babies during this time. CHOC offers extensive family resources and amenities both inside and outside the hospital to provide you and your family with access to everything they might need during your baby’s stay.
At CHOC we’re not just here to treat your baby or child, we know that every diagnosis affects the full family. That’s why we also provide supportive care services for families, which run alongside a patient’s treatment process.
Does CHOC have a long-term follow-up program for babies with CDH?
CHOC offers a long-term care program for babies with CDH. Our pediatric CDH specialist team has scheduled checkpoints with your child at 1-3 months, 4-6 months, 9-12 months, 15-18 months and annual touchpoints through age 16.
These long-term care follow-ups may include weight, length and head circumference measurement, chest X-rays, pulmonary function tests, RSV prevention, echocardiogram and cardiology follow-ups, and other services related to your baby’s CDH and development.
What research is CHOC doing to improve CDH outcomes and lives of children with CDH?
CHOC is committed to conducting research that helps save the lives of children, even if that means developing a unique solution for one child. Our pediatric general and thoracic surgeons regularly take part in and contribute to CDH studies, which lead to greater disease awareness and the development of innovative treatment solutions. On top of individual specialists’ research, CHOC is also a member of the CDH Study Group, an international cohort of CDH centers dedicated to improving outcomes. Our team has also developed the Consensus Guidelines for CDH management during extracorporeal membrane oxygenation by the Extracorporeal Life Support Organization.
Our expert’s recent publication include:
- Neonatal Respiratory and Cardiac ECMO
- Risk Characteristics of CDH Treated with ECMO
- Mortality Ratios for CDH Receiving ECMO Life Support
- Inborn vs Outborn Delivery in Neonates with CDH
- CDH Management with Extracorporeal Life Support