Patient education: Congenital hernia of the diaphragm (The Basics)

What is a congenital hernia of the diaphragm? — A congenital hernia of the diaphragm is a condition that happens when a baby is growing in the mother's uterus.

The diaphragm is the layer of muscle that separates the organs in the chest from the organs in the belly. Babies with a congenital hernia of the diaphragm have a hole in their diaphragm. Because of this, some of the organs from the belly might move through this hole into the chest (figure 1). In the chest, these organs can press on the lungs and keep them from growing normally.

A congenital hernia of the diaphragm can be mild or severe. A severe congenital hernia of the diaphragm is life-threatening.

What are the symptoms of a congenital hernia of the diaphragm? — Before birth, a congenital hernia of the diaphragm causes no symptoms. The doctor might find out your baby has it when they do a routine test, called an ultrasound, to check on your baby during pregnancy. An ultrasound is an imaging test that creates pictures of your baby inside the uterus.

After birth, the most common symptom that a baby might have is trouble breathing. This usually starts within a few hours after birth. Some babies have mild trouble breathing, but most babies have severe trouble breathing.

Is there a test for a congenital hernia of the diaphragm? — Yes. Often, the condition is found when an ultrasound is done during pregnancy. Then, after delivery, a chest X-ray is done to confirm that the baby has it. A chest X-ray is also done if a doctor thinks that a baby might have the condition after birth.

How is a congenital hernia of the diaphragm treated during pregnancy? — Your doctor will probably refer you to a different group of doctors with experience in caring for babies with this condition. There is no treatment that can be given during pregnancy. But the doctors will do regular tests to monitor your baby's health.

As you get closer to your due date, your doctor will probably talk with you about giving you medicines to help start your labor. This is called "inducing labor." Doctors usually induce labor when a baby has a congenital hernia of the diaphragm. That way, the doctors who take care of your baby can be ready to start treatment right after birth.

How is a congenital hernia of the diaphragm treated after birth? — Treatment depends on your baby's symptoms and how severe the condition is. It usually involves different steps.

First, the doctor will treat your baby's breathing and other lung problems. Treatments usually include:

●A breathing tube – A breathing tube is a tube that goes down the throat and into the lungs. The other end is attached to a machine that helps with breathing. Doctors use the term "intubation" for this.

●A nasogastric tube ("NGT") – This is a thin tube that goes in the nose and down into the stomach. The tube sucks up extra fluid and air in the stomach.

●An IV line – This is a thin tube that goes into a vein. It can be used to give medicines or fluids.

●An umbilical line – This is a thin tube that goes into a blood vessel in the umbilical cord.

If the breathing tube doesn't help enough, your baby might need to be put on a heart and lung machine. This machine, called "ECMO," takes over the jobs of the heart and lungs. It pumps blood from your baby's body, gives it oxygen, and pumps it back into their body.

After your baby's breathing and other lung problems are under control, your baby will need surgery. Surgery is usually done a few days after birth, but the timing depends on your baby's individual situation. During surgery, the doctor will put the belly organs back in the belly and close the hole in the diaphragm.

After surgery, babies usually need a breathing tube or need to be on a heart and lung machine while they heal.

Does a congenital hernia of the diaphragm cause long-term problems? — Sometimes. It will depend on your baby's individual situation. But many babies with a congenital hernia of the diaphragm will have long-term problems. These can include:

●Breathing or other lung problems

●Not gaining weight or not growing normally

●Digestive problems

●Learning problems or hearing loss

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