What is congenital diaphragmatic hernia (CDH)?

Every person has a muscle barrier under the lungs called a diaphragm. The diaphragm is what separates the chest cavity from the abdominal cavity and is the primary muscle used when inhaling. A congenital diaphragmatic hernia occurs when there is a hole in the diaphragm. The hole (hernia) allows abdominal organs – such as the intestines, stomach, and liver – to slide up into the chest cavity. This can cause overcrowding in the chest cavity, leaving little space for the lungs to develop and mature properly. This overcrowding can sometimes push the heart over from its normal place in the middle of the chest, which can crowd the opposite lung and cause it to underdeveloped, as well.

Congenital diaphragmatic hernia happens in about 1 in every 2,500 live births. In most cases, about 90 percent, the hernia occurs on the left side, but CHD can happen on the right side or both sides. The hole can be small, allowing only a loop of bowel to push through, or severe, where most of the diaphragm is missing. If the hernia is large enough, your baby may need a prosthetic patch.

CDH is sometimes associated with other birth defects. Approximately 50 percent of babies with CDH will have another problem including issues with the central nervous system, cardiovascular, gastrointestinal, urinary, or skeletal systems.

Who gets it?

There is no known cause of CDH, although it tends to happen more often in boys than girls. It is also important to know that CDH is not related to anything a mother did or did not do during the pregnancy.

Why is it important to be cared for at a comprehensive children’s hospital?

UI Stead Family Children’s Hospital is home to Iowa’s only comprehensive children’s hospital. This means that all pediatric specialties are available under one roof, 24 hours a day, seven days a week. The Neonatal Intensive Care Unit (NICU) at University of Iowa Stead Family Children’s Hospital is a Level 4 unit – the highest level recognized by the American Academy of Pediatrics. This means that our NICU is equipped to care for the tiniest and most critically ill babies, offering the greatest range of neonatal services and support. UI Stead Family Children’s Hospital is also home to Iowa’s only pediatric ECMO. ECMO (extra corporeal membrane oxygenation) is a type of heart and lung bypass that can help your baby breathe if he or she is very ill. We want you and your baby to be cared for immediately after birth by individuals with pediatric expertise and all the necessary resources.

Will my child need surgery?

Chances are very good that if your baby is diagnosed with a congenital diaphragmatic hernia, some kind of surgery will eventually be required once the baby is stable enough for surgery. When – and what kind – will depend on the severity of the hernia.

Many babies with CDH are diagnosed during a routine prenatal ultrasound. If your doctor thinks your baby has CDH, he or she will refer you to a maternal-fetal medicine specialist. This is a doctor who specializes in high risk pregnancies. This doctor may conduct more tests, like a fetal echocardiogram and amniocentesis, to confirm the diagnosis and gain more information about your baby.

At birth, CDH can make it very hard for your baby to breathe. It can also affect your baby’s blood circulation due to abnormal development of the lungs and possible shifting of the heart. Most babies born with CDH require immediate breathing assistance. The neonatologist will pass a breathing tube into your baby’s airway. Another small tube will be put through your baby’s nose to his stomach to keep the stomach decompressed, or small in size. This will keep it from pressing on the lungs. The NICU team will also place an I.V. to give your baby fluids, medicines, and nutrition.

The pediatric surgeon will operate on your baby to close the hernia if and when your baby is stable enough for the surgery. During the operation, the surgeon will guide the abdominal organs back down into the abdomen where they should be. If the hole is small, it can be sewn closed. This is called a primary repair. If the hole is large, your baby may require a patch to be put in to help close it. After surgery, it’s likely your baby will still need help breathing with the breathing tube. The I.V. will stay in place to give your baby medicines like antibiotics and pain medications, and medicines to control blood pressure.

What else do I need to know?

Babies with CDH often cannot eat by mouth for several weeks after birth and nutrition is given through an I.V. NICU and surgery teams will look for signs that your baby’s body is ready before giving nutrition through the stomach. These signs include bowel sounds, passage of stool or gas, and a decreased about of drainage coming from the tube in your baby’s stomach. Moms hoping to breastfeed will be able to pump during this time. The NICU has breast pumps available for use, if needed.

Your baby will be able to go home once he or she can eat enough to gain weight and the lung function is sufficient enough to supply the body with enough oxygen. After discharge, the neonatologists, cardiologists, and pediatric surgeons will see your baby in the pediatric specialty clinic to make sure your baby is healing and growing well.

Your child’s evaluation:

If your doctor believes your baby has CDH, we will schedule an appointment at University of Iowa Stead Family Children’s Hospital in Iowa City. During this appointment, you will meet with a pediatric surgeon, members of the pediatric surgery team, members of the NICU team, a social worker, and a maternal-fetal medicine doctor. During this appointment, you will learn about treatment options, the possibility of surgery, pre-surgery care, and follow-up treatment.

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