Congenital Diaphragmatic Hernia
The diaphragm is the thin sheet of muscle inside the body that separates the chest from the belly. A congenital diaphragmatic hernia (CDH) happens if the diaphragm does not form completely before birth. This leaves a hole between the belly (abdomen) and chest. The condition is pronounced kun-JEN-ih-tul dy-uh-fruh-MA-tik HER-nee-uh.
In CDH, organs that should be in the belly go through the hole up into the chest. Most often, the intestines move into the chest. Sometimes, the stomach, liver and spleen also go through the hole.
With the organs from the belly in the chest, the lungs do not develop normally. The lungs are small, and the lung tissue itself is different. Because of these differences, babies with CDH have high blood pressure in their lungs (pulmonary hypertension). Some babies with CDH have other health problems, too.
How serious CDH is depends on your baby’s lung size and function, the health of other organ systems and their overall health. Most babies with CDH survive if they receive specialized care. Experts at Seattle Children’s are experienced in providing the care babies with CDH need, including breathing support; surgery; and comprehensive, long-term care.
Types of CDH
There are two main types of CDH:
- Hole toward the side of the chest (Bochdalek)
- Hole in front (Morgagni)
This webpage describes the most common type of CDH, Bochdalek hernias. They are 10 times more common than Morgagni hernias and they cause more severe problems. Morgagni hernias are usually discovered later in childhood.
CDH is present at birth (congenital). The problem happens early in pregnancy while the diaphragm is forming. Researchers are looking at possible causes, including the roles of certain chemicals, nutrients or changes in genes. CDH is not caused by something the mother did or did not do while she was pregnant.
Congenital Diaphragmatic Hernia at Seattle Children’s
We have treated many babies with CDH at Seattle Children’s. In a typical year, we take care of about 15 babies with this rare condition.
If you would like an appointment, talk with your pregnancy care provider for a referral to our Prenatal Diagnosis and Treatment program. If you would like more information or have a referral, call 206-987-5629 to schedule an appointment.
- If an ultrasound during pregnancy shows that your baby has a CDH, our Prenatal Diagnosis and Treatment team can do a detailed evaluation. We work with your obstetric team to plan your care. Finding a problem early gives you more time to learn about the condition, make decisions about care and prepare for what lies ahead.
- Careful coordination with your birth hospital helps prepare for the special care your baby will need during delivery, at birth and as they travel by ambulance to Seattle Children’s within a few hours of birth. Our transport team of physicians, nurses and respiratory therapists are national leaders in safely moving fragile newborns.
- Our Level IV Neonatal Intensive Care Unit (NICU) is skilled and equipped to care for the sickest newborns. Coordinated care continues through every step – as your baby has surgery, moves to a regular hospital room, goes home and comes back to Seattle Children’s for clinic visits.
- Our medical team focuses on how today’s treatment will affect your child as they develop and become an adult. We base treatment plans on years of experience and the newest research on what works best and most safely.
- When your baby is able to go home, our Surgery Pulmonary Follow-Up Clinic provides long-term care for children with CDH. This clinic gives families a single place where a team of experts checks their child’s health and provides treatment and advice.
- Our pediatric surgery team is one of the most experienced in the nation, with excellent outcomes. We have an 80% survival rate for babies with this very serious condition. Our rate for repeat CDH surgeries is one of the lowest in the world.
- We carefully evaluate your child to decide the best treatment for them. This includes the best type of breathing support and the timing of surgery to fix their diaphragm. We focus first on breathing support and improving blood pressure in your baby’s lungs. If needed, we have an Extracorporeal Life Support Program to provide heart-lung bypass support.
- Your team at Seattle Children’s will include specialists in caring for newborns (neonatologists) and their hearts (cardiologists) and lungs (pulmonologists). In 2019, U.S. News & World Report ranked Seattle Children’s among the best in the nation for these and other programs.
- Your child and family will also get care from experts in nutrition, social work, feeding, infant development, lactation support, emotional health and other specialties.
- Babies with CDH may have other health problems at birth. We have all the experts your family needs.
- Children do not react to illness, pain, medicine or surgery in the same way as adults. That is why our surgeons are all board certified in pediatric surgery.
- We take time to explain your child’s condition. We help you fully understand your treatment options and make the choices that are right for your family.
- Our doctors, nurses, child life specialists and social workers help your child and your family through the challenges of this condition. We connect you to community resources and support groups.
- At Seattle Children’s, we work with many children and families from around the Northwest and beyond. Whether you live nearby or far away, we can help with financial counseling, schooling for other children in the family, housing, transportation, interpreter services and spiritual care. Read about our services for patients and families.
Seattle Children’s leads research in the lab and in the clinic to improve treatment and quality of life for children born with conditions like CDH.
- Drs. Delphine Yung and Kendra Smith do research on new treatments for pulmonary hypertension, which usually affects babies with CDH.
- Our newborn specialists are leaders in research to improve outcomes for babies born very early or with complex conditions. See Pushing Limits, Saving Lives.
- We study and test innovative mechanical heart devices that support children with breathing problems or very weak hearts. Our goal is to develop safer, more effective devices.
- Many children with CDH have feeding problems and gastroesophageal reflux (GER). Our research helps parents and doctors decide when surgery or other treatment is best.
- We work to find the safest, most effective ways to reduce stress in the Neonatal Intensive Care Unit (NICU) and to relieve pain. Learn more about Seattle Children’s Neonatology research.
- Our patients have the option to take part in research studies of promising new treatments. These are called clinical trials.
Symptoms of Congenital Diaphragmatic Hernia
Babies with CDH may have serious breathing problems, starting soon after they are born. Breathing problems happen because:
- Their lungs are smaller than normal
- Their hearts cannot pump blood easily to the lungs because the walls of the blood vessels are too thick
- The organs that slip through the hole between the belly and chest are pushing on their lungs
Babies with CDH may also have:
- Increased work of breathing, for example, using belly muscles to get a breath in
- Rapid breathing
- Fast heart rate
Diagnosing Congenital Diaphragmatic Hernia
In most cases, doctors diagnose CDH from an ultrasound during pregnancy. If an ultrasound shows your baby has CDH, our Prenatal Diagnosis and Treatment team can help you prepare. We will work with you to plan for a safe delivery and your baby’s transfer to Seattle Children’s.
We will talk with you about the treatment your baby is likely to need after birth. You can get to know some members of the team who will be caring for you and your baby. At a visit to our hospital, you can see where we will care for your baby and learn about the resources we have for families.
Sometimes, CDH is not diagnosed before a baby is born. A doctor or nurse who checks your baby after birth may find:
- Irregular chest movements with breathing difficulty
- No breathing noises on the side that is affected
- The belly feels less full and looks flatter than normal
These tests may be done to find out why your baby is having trouble breathing:
- Checking oxygen levels across the skin (pulse oximetry) and with blood tests.
- X-ray. This can show that organs are in the chest instead of the belly. Your baby’s lungs may look smaller than normal and their heart may be pushed to 1 side.
- An ultrasound image of your baby’s heart (echocardiography). This can help tell how CDH is affecting their heart.
- Sometimes, providers find CDH in an older baby or a child who has had many colds or other illnesses that affect their breathing. This is less common. Usually, this is a Morgagni CDH. It requires surgery to close the hole in the diaphragm, but is much less serious than the more common type, a Bochdalek CDH.
Treating Congenital Diaphragmatic Hernia
Most babies with CDH need breathing support soon after birth. All babies with CDH need surgery to gently move their abdominal organs down from the chest and close the hole in the diaphragm. Since we do not deliver babies at Seattle Children’s, your baby will receive their first care at your delivery hospital. Within a few hours of birth, they travel by ambulance to Seattle Children’s.
The timing of surgery depends on your baby’s condition. Surgery is not done the day your baby is born. Your baby may have the surgery a few days after birth or weeks later, if that is safest for your baby.
- Most babies will need to be on a breathing machine (ventilator) before and after surgery to help them breathe. We give your baby sedation to help them be comfortable and prevent them from having memories of that time.
- Breathing support is needed because most babies with CDH have small lungs. They also have high blood pressure in their lungs (pulmonary hypertension). Their heart has trouble pumping blood to their lungs because the arteries to the lungs are thicker and stiffer than normal. Surgery to fix the diaphragm and move the organs back to the belly does not cure this problem, but it does allow space for the lungs. Lung size and blood pressure in the lungs usually get better over time.
- After surgery, your baby will stay on a ventilator until their lungs are ready to take over. Your child may need to be on the ventilator for days or for weeks. It depends on their condition.
- If problems are severe, your baby may need to be on a machine that does the work of their heart and lungs (ECLS or extracorporeal life support). The machine is sometimes called ECMO. “Extracorporeal” means outside the body.
- During the time from birth until surgery recovery, we do not usually feed babies with CDH by mouth. During this time, your baby will get all their nutrition through an intravenous (IV) line.
- If you are planning to breastfeed your baby, our nurses and lactation specialists will do everything we can to support you. To keep your milk flowing and to build a milk supply for your baby, we help you with breast pumping and storing your milk.
- Babies with CDH also need a tube that passes through their nose into the stomach (nasogastric tube or NG tube). This tube prevents the intestines from filling with air so they do not put more pressure on the lungs.
- During recovery from surgery, a different kind of nose-to-stomach (NG tube) is used for gentle milk delivery.
There are different ways to do the surgery that moves organs back into the belly and closes the hole in the diaphragm. The choice will depend on your child’s condition. Your baby’s surgeon will talk with you about the options and recommend the best treatment for your child.
- At Seattle Children’s, most surgeons make a small cut (2 to 3 inches) in the belly to do the surgery. This is called open surgical technique.
- If a baby with CDH is very healthy, surgeons may use a small camera inserted into the chest to see the CDH. This allows the surgeon to fix the CDH with a few very small cuts (incisions) for the camera and their surgical tools. This is called thoracoscopic technique.
No matter which type of surgery your baby has, we will give them medicine to make them sleep without pain during the surgery (general anesthesia).
After bringing the organs into the belly, the surgeon will close the hole in the diaphragm in 1 of 2 ways:
- Sew it closed.
- Patch the hole, if it is too large to sew closed. We use a patch with 2 layers invented at Seattle Children’s. It has the highest success rate reported in the medical literature. This patch will stay inside your child’s body.
Surgery for CDH usually takes a couple of hours. It depends on:
- The size of the hole
- How difficult it is to move the organs
- How healthy your baby is overall
At the time of surgery, many children need a chest tube. This allows extra fluid in their chest to drain out rather than collecting inside. The tube usually needs to stay in place for a few days after surgery.
After surgery, your child will continue to receive the highest level of care in our Level IV Neonatal Intensive Care Unit (NICU). Seattle Children’s is the first hospital in Washington to have a Level IV NICU.
When your baby no longer needs a ventilator and is stable, they will move out of the NICU to a regular hospital room. During the first days or weeks after surgery, your baby will continue to get all their nutrition through an intravenous (IV)line. When your baby is ready, our nurses and feeding therapists help with the transition to feeding by bottle or breast. By the time they go home, many babies still need a feeding tube in their nose (NG tube) for at least part of their nutrition.
Every child’s recovery is different. Your baby’s hospital stay may be a couple of weeks or many months. Most babies stay 4 to 6 weeks. Your baby will be ready to go home once they:
- Breathe on their own or with a tube in their nose to provide a small amount of extra oxygen
- Can gain weight without IV nutrition
You will learn what kinds of food or medicine to give your child. Our nurses will help set up the supplies and equipment you need. You will have plenty of time to practice all of your baby’s care before going home.
Our Surgery Pulmonary Follow-Up Clinic team will coordinate your follow-up visits.
Some children will have no long-term problems after being born with CDH. But children who are born with very small lungs often need care into their teen years or longer. For these children, we have created a special clinic. The Surgery Pulmonary Follow-Up (SPF) Clinic:
- Provides ongoing treatment your child needs
- Refers you to the experts your child may need for a range of conditions, such as nutrition and neurodevelopmentalsupport
- Can be a resource for your child’s pediatrician
Ongoing health issues for some children include:
- High blood pressure in their lungs (pulmonary hypertension): If your baby continues to have this problem when they are ready to go home from the hospital, they may need oxygen and medicines for a while to help blood flow better from the heart to the lungs. Our heart and lung specialists will make sure your child gets the care they need.
- Gastroesophageal reflux (GERD): The backward flow of stomach contents into the esophagus is common in babies with CDH. This can be uncomfortable and make feeding more challenging. Reflux can also worsen some breathing symptoms. Your child may need medicine or (rarely) surgery to manage reflux.
- Feeding problems: Our occupational therapists provide feeding therapy for babies who are still developing feeding skills. Some babies have problems related to reflux. Many need a nose-to-stomach tube for at least part of their nutrition. This provides gentle, safe feedings until a child grows out of GERD and learns to eat safely.
- Heart conditions: These include being born with a hole in their heart or other abnormalities. Your child will get care from experts in our top-ranked Heart Center.
- Frequent, serious colds: The lungs of children with CDH are smaller and stiffer than normal during the first few years of life. They may be much more likely to get colds and take longer to recover than other children. We recommend special vaccines to prevent the most serious infections.
- Asthma: Your child has a greater chance of getting asthma. To help prevent symptoms, avoid common irritants like smoke, dust and some animals. Our SPF clinic watches for early signs of asthma in children with CDH to be sure it is treated correctly.
- Curved spine (scoliosis) if the chest wall does not form or grow properly. This sometimes happens with CDH.
Based on your child’s needs, the clinic team will connect you with healthcare providers from other areas of Seattle Children’s.
If you would like an appointment, talk with your pregnancy care provider for a referral. If you would like more information or have a referral, call 206-987-5629 to schedule an appointment with our Prenatal Diagnosis and Treatment program.
Providers: See how to refer a patient.