Antenatal diagnosis of congenital diaphragmatic hernia

Your baby has been diagnosed with a congenital diaphragmatic hernia. This information is to help you understand the condition and what will happen after your baby is born and comes to Sheffield Children’s Hospital for treatment and care.

What is congenital diaphragmatic hernia (CDH)?

The diaphragm is a muscle that separates the chest from the abdomen, and helps with breathing.

In a baby with congenital diaphragmatic hernia the diaphragm has not formed properly leaving a hole. This hole allows some of the bowel and sometimes other organs (for example: liver or stomach) to move up into the chest from the abdomen (tummy). The presence of the bowel being in the chest prevents the lungs from developing normally, and working properly when your baby is born. After your baby is born they will need an operation.

Babies who have CDH also sometimes have a problem called pulmonary hypertension. This is when the baby’s blood vessel in the lungs does not relax after birth. This makes it difficult for the heart to pump blood into the lungs to get the oxygen to the other organs. Organs need oxygen to work so it is important for this problem to be detected and treated.

What causes congenital diaphragmatic hernia, and how common is it?

We do not know what exactly causes this condition. It is not caused by anything you have done before or during pregnancy.

CDH is rare and occurs in about 1 in 2500 babies. It is more commonly seen on the left side of the diaphragm but it can occur on the right.

Unfortunately not all babies with CDH survive. Babies who have CDH can sometimes have other associated anomalies (conditions) too. If your baby is found to have other anomalies their prognosis is not as good as the babies who do not have other anomalies.

What will happen during my pregnancy?

You and your baby will be carefully monitored by the obstetricians. You will have regular scans and tests to check you and your baby are progressing well. You may also be offered special additional tests. This will include amniocentesis to check your baby’s chromosomes, an MRI (a special type of scan) and a scan to check your baby’s heart in more detail.

You will be able to meet the specialist doctors who will look after your baby when they are born. This will be the neonatologist (baby doctors) and a paediatric (children’s) surgeon. You will have an opportunity to visit the Neonatal Intensive Care Unit (NICU) at the Jessop Wing in the Royal Hallamshire Hospital in Sheffield, and the Neonatal Surgical Unit (NSU) at the Sheffield Children’s Hospital where your baby will be cared for after their birth. The aim is for your baby to be born at the Jessop Wing. Your obstetrician will advise when and how your baby will be born.

What will happen when my baby is born?

The neonatologist and the specialist baby team will be present when your baby is born. They will pass a special tube into your baby’s windpipe and attach it to a ventilator (a breathing machine) to help your baby to breathe. Your baby may be given some medicine to stop them moving. The neonatologists will take your baby to the Neonatal Intensive Care Unit (NICU).

On NICU the nurses and doctors will:

• pass another tube (called a nasogastric tube) into your baby’s stomach to allow the air and fluid that is in your baby’s stomach to drain out. This will help relieve the pressure on your baby’s lungs
• put special lines into your baby’s veins (intravenous cannulas often referred to as ‘drips’) to give your baby intravenous fluids and medicines
• give your baby medicine to sedate and to make them feel comfortable, to prevent infections and if necessary to help with their blood pressure
• take blood tests, X-rays, perform scans and closely monitor your baby

Sometimes your baby will need more specialist types of support to help their breathing. This can be nitric oxide ventilation and high frequency oscillatory ventilation. If these do not help, your baby will need to be referred to a specialist centre for ECMO (extra-corporeal membrane oxygenation). The doctors will explain to you in more detail at the time if your baby needs these types of treatments

When will my baby have surgery?

The neonatologist and the surgeons will closely monitor your baby. When they think your baby is ready for surgery they will be transferred to the Paediatric Intensive Care Unit (PICU) at Sheffield Children’s Hospital for continuing care and surgery.

Your baby will be transferred there by Embrace, a specialist transport team who are experienced with transferring babies with CDH.

What does the surgery to repair the congenital diaphragmatic hernia involve?

Your baby will have an operation under a general anaesthetic. The anaesthetist and the surgeon will explain this to you in detail at the time and you will need to give consent.

During the operation the surgeon will move the bowel, and the other organs that are in the chest back into the abdomen. They will close the hole in the diaphragm with sutures (stitches). If the hole is too big for using only sutures the surgeon will use a ‘patch’ which is a special piece of material, to close the hole.

What will happen after surgery?

Your baby will still need the support of the ventilator with their breathing after the operation. The length of time your baby will need support will depend on the condition of your baby’s lungs and how well your baby recovers from the operation.

Your baby will be given pain relief and will be closely monitored. Your baby’s breathing support will be gradually weaned as your baby recovers. When your baby does not need the support of the ventilator they will be transferred from PICU to NSU. Your baby may need to continue with extra oxygen for a period of time.

Will my baby be able to feed and what milk can my baby have?

Your baby will not be able to have milk feeds prior to the surgery. Initially your baby will have intravenous fluid and, or parental nutrition (PN) via their intravenous lines. PN provides all the nutrients your baby needs to grow which they would normally get from milk.

Your baby will begin milk feeds a few days after surgery, initially it may be via a nasogastric tube (NGT) or small amounts by bottle. The volume of milk your baby has will be slowly increased as they recover from the operation and show us signs they can tolerate their milk well. As you baby recovers you can commence breast feeding if you wish to do so.

Breast milk is very important for every baby but especially if your baby is poorly. If you wish to breast feed or give your baby your breast milk we will support and help you in expressing your breast milk and establishing breast feeding. Your midwife can help you to start expressing after your baby has been born. We have facilities on NSU for expressing and there are infant feeding specialists in the hospital who can advise and support you with this as well as the NSU nurses. Your breast milk can be frozen until your baby is ready to have it. You can also use your colostrum and breast milk for your baby’s mouth care.

If you do not wish to express your breast milk or are unable to do so, your baby can have formula milk of your choice. Occasionally the surgeons may wish your baby to have a special prescription milk formula. This will be discussed with you at the time.

How long will my baby be in hospital?

We are unable to predict how long your baby will be in hospital. The average length of time CDH babies are in NSU for is 5 weeks. Your baby’s progress will be closely monitored by the surgeons, nurses and the other teams involved with your baby’s care. You will be updated regularly on your baby’s progress and will be able to ask and have questions answered throughout your stay on NSU.

When your baby is tolerating milk feeds and gaining weight they can go home. Sometimes this is with additional support such as oxygen or tube feeding.

What is the long term outlook for my baby?

This will depend on your baby’s lungs, how long they needed ventilation for and whether they have any other problems. The outlook for CDH babies is becoming more positive. Your baby will need long term follow up with the surgeons, the respiratory paediatricians and sometimes other specialist doctors.

Are there support groups?

CDH UK

The Denes, Lynn Road
Tilney all Saints
Kings Lynn
Norfolk
PE34 4RT

Telephone: 0800 731 6991
Website: www.cdhuk.org.uk

Bliss

Group which offers general support to families of babies born early or sick

68 South Lambeth Road
London SW8 1RL

Website: www.bliss.org.uk
Facebook group: BLISS