Key points about congenital diaphragmatic hernia
- a congenital diaphragmatic hernia (CDH) is caused by a hole in the diaphragm
- the intestines or stomach, and sometimes the liver and spleen, can move from the tummy area into the chest area
- if your baby has a CDH, they will need immediate treatment after birth
What is a CDH?
The diaphragm is a flat sheet of muscle that sits under the lungs, separating the chest from the tummy. The diaphragm helps us to breathe.
A CDH is caused by a hole in the diaphragm. The intestines or stomach, and sometimes the liver and spleen, can move from the tummy area into the chest area. This puts pressure on and squashes the developing lungs and affects their growth. CDHs can happen on either side of the chest but most happen on the left side of the chest.
An illustration comparing normal anatomy with a diaphragmatic hernia.
Source: KidsHealth
transcribeTranscript
The illustration shows two images of a baby lying on their back.
- On the left, the image is labelled Normal.
The lungs sit in the chest above the diaphragm, and the organs from the tummy sit below the diaphragm. - On the right, the image is labelled Diaphragmatic hernia.
Organs from the tummy have moved into the chest area through a hole in the diaphragm. These organs squash the left lung. - Text explains that a diaphragmatic hernia happens when organs from the tummy move into the chest through a hole in the diaphragm, squashing the left lung.
At the top right of the illustration is the KidsHealth logo with the website: kidshealth.org.nz.
Most pēpi with a CDH are very unwell when they are born. They can have severe breathing troubles and need treatment straight away.
During pregnancy, pēpi get oxygen from their mother’s bloodstream. After birth, your baby's lungs need to provide oxygen to the body so the organs can work properly. If the lungs are small or don't have much space to expand, they may not work properly.
Sometimes, the heart is also pushed over to the ‘wrong’ side of the chest. This can affect the growth of the lung on the opposite side.
CDH is a rare, serious condition.
Causes of CDH
CDH affects about 1 in 2,500 newborn pēpi. It's not caused by anything you have or haven't done.
CDH can happen on its own. But it's more common in pēpi who may have a heart defect, or in pēpi who have a gene or chromosome condition.
You can talk with a specialist team about your own baby's situation.
CDH diagnosis
Health professionals can often diagnose CDH using an ultrasound scan before birth. An ultrasound scan can show the organs from the tummy area in the chest. It can also show increased fluid around pēpi (polyhydramnios). Sometimes, CDH is not diagnosed until after birth.
Once the ultrasound scanning shows CDH, several health professionals will review the ultrasound pictures. A team of health professionals will care for you and your baby before, during and after birth.
Paediatric surgeons are doctors who specialise in children’s surgery. Neonatologists are doctors who provide medical care for newborn pēpi. A paediatric surgeon and a neonatologist will talk with you about your baby's condition. They will talk with you about possible complications and treatment.
Where to give birth to your baby with CDH
You'll need to have your baby at a hospital with a neonatal intensive care unit (NICU). These units have experience in caring for pēpi with CDH. You can usually look around the NICU before your baby is born. Talk to your healthcare team about arranging this.
Managing CDH
Immediate care after birth
Your baby will need immediate treatment after birth. So you may not hear your baby cry and you may not be able to hold your baby immediately after birth.
Treatment includes a breathing machine to help your baby breathe. The breathing machine is called a ventilator.
Your healthcare team will also put a tube into your baby's nose or mouth. The tube will go from the nose or mouth to the stomach. This will reduce the pressure so the lungs have room to expand during breathing.
Care in the NICU
Your healthcare team will transfer your baby to the NICU. They will place special lines into the blood vessels of the umbilical cord. This allows the healthcare team to watch your baby's blood pressure and take blood samples for testing. It also allows them to give your baby fluids and medicines. The healthcare team may also place intravenous (IV) lines in your baby's hands or feet.
Your baby will have heart scans to check on how well the heart is functioning. These heart scans are called echocardiograms. The results will help the healthcare team decide which medicines to give to support the heart.
Surgery
Your baby will need surgery to repair the hole in the diaphragm. The timing of surgery will depend on how well your baby's lungs and heart are working. The healthcare team will watch your baby very closely to decide the best time for surgery.
Before surgery
Most pēpi need several days of care to help them become stable before surgery. Some pēpi have problems with blood flow through the lungs. They may need extra medicine, such as nitric oxide, to help with this. Pēpi do best when their heart and lungs are working as well as possible before surgery.
The surgeon and anaesthetist will talk with you about the surgery. They will explain what will happen and answer your questions. You will need to sign a consent form before your baby has the anaesthetic and surgery. Make sure you ask the doctor questions and discuss any concerns you may have before you sign the consent form.
You can be with your baby until they are asleep from the anaesthetic.
After surgery
The surgeon will let you know how the operation went as soon as it is finished.
After the surgery, the healthcare team will watch your baby closely. They will support your baby's breathing until the lungs recover. How long this takes depends on how much the condition affected your baby's lungs and heart.
Your baby will have intravenous (IV) pain relief medicine. This is medicine that goes straight into a vein. Medicine may include:
morphine for pain relief
midazolam for any distress
The healthcare team may put a small tube into your baby's bladder to drain urine.
Feeding
The healthcare team will feed your baby through a vein. This is called total parental nutrition (TPN). Your baby will have TPN before and after the surgery. TPN will continue until your baby is ready to have small amounts of breastmilk or formula through a tube. As milk feeds increase, the TPN will slowly decrease until your baby is only having milk feeds. You will receive support to help establish sucking feeds when your baby is ready for this.
Guidance and support for you
If your baby has been diagnosed with a CDH, it's normal to worry. There will be a large healthcare team caring for your baby and this can feel overwhelming. So can all the interventions and medicines. But you will receive lots of guidance and support. Don't hesitate to ask your healthcare team any questions. For example, you can ask them what you can do to care for, and bond with, your baby.
Complications and survival
For some pēpi with CDH, treatment can be quite straightforward. But for other pēpi, treatment can be complex. There are several possible complications, and some pēpi may not survive. Survival depends on many things, including how the condition has affected the heart and lungs. Your healthcare team will give you information that is specific to your baby and their diagnosis.
Checks after leaving hospital
Before you leave hospital, your healthcare team will talk to you about what follow-up checks your baby needs.
