My baby has been diagnosed with oesophageal atresia

This information is to help you understand the condition and what treatment and care your baby will have at Sheffield Children’s NHS Foundation Trust on the Neonatal Surgical Unit (NSU) and on the Paediatric Intensive Care Unit (PICU).

What is oesophageal atresia (OA) and tracheo-oesophageal fistula (TOF)?

It is a rare condition which can occur in 1 in 3500 to 1 in 5000 births. The oesophagus (food pipe) often referred to as the gullet, has not formed properly. This means food cannot pass from the mouth to the stomach. The oesophagus has a gap (an atresia) in it.

Some babies have just oesophageal atresia (pure OA) but the majority of babies who are born with oesophageal atresia also have trachea-oesophageal fistula. This is where one end of the oesophagus joins itself to the trachea (wind pipe).

How was my baby diagnosed with oesophageal atresia?

Your baby may have had problems breathing, swallowing or feeding shortly after they were born. A tube (nasogastric tube) would have been passed through your baby’s nose in an attempt to pass it down in to the stomach. An X-ray taken following this would have shown the nasogastric tube to be in the pouch (blind end) of the oesophagus and not in the stomach. This confirmed your baby has oesophageal atresia.

How will my baby be cared for initially after the diagnosis of oesophageal atresia has been made?

Your baby will have a special tube called a ‘Replogle’ tube passed through their nose and in to the upper pouch of the oesophagus. This tube helps to remove the saliva from the oesophagus, to keep your baby safe and comfortable.

A plastic tube, often called a ‘drip’ (intravenous cannula) will be inserted in to your baby’s vein to give intravenous fluids, antibiotics and other medicines your baby may need. Your baby will not be able to feed and will need an operation.

Your baby will be attached to a monitor for recording their heart rate, breathing rate, oxygen level and blood pressure. Some babies may have breathing difficulties when they are born and may require some extra help. This will be explained to you in more details by the doctors

How will my baby’s oesophageal atresia be treated?

Your baby will need an operation to repair the oesophageal atresia (OA) and, if present, the tracheo-oesophageal fistula (TOF). The surgeons will explain the operation to you and you will need to give consent. You will also be seen by an anaesthetist because your baby will need a General Anaesthetic for the operation.

Pure OA treatment

If you baby has pure OA they will have an operation on their abdomen (tummy) initially. They will have a special feeding tube put in their stomach called a gastrostomy. This will all be explained to you at the time.

OA and TOF treatment

If your baby has OA and an associated TOF, the operation your baby will have is performed through the chest. This may be done as keyhole surgery (thorascopically) or as open surgery.

The fistula (TOF) will be repaired by separating the oesophagus from the trachea, and the trachea will be repaired at the place where the oesophagus had joined it. The OA will be repaired by joining the 2 ends of the oesophagus to form a continuous passage from the mouth to the stomach.

A nasogastric tube will be passed into your baby’s stomach at the end of the operation.

In rare cases the gap between the 2 ends of the oesophagus is too big to enable the ends of the oesophagus to be joined. This is called long gap oesophageal atresia. If this is the situation the surgeons will talk to you about how they will treat and manage your baby.

What happens after the operation?

Usually after the operation your baby will need help with their breathing for a short period time to help with pain, healing and to enable your baby to rest. Your baby may need to spend a period of time on the Paediatric Intensive Care Unit (PICU). This will be explained to you at the time.

Your baby will be given pain relief and will be continued to be monitored closely. Your baby will be able to start milk soon after the operation. If your baby had a TOF they will initially have their milk slowly through the nasogastric tube. After a few days they should be able to start having small amounts by mouth by bottle. The volume of milk your baby has will be slowly increased as they recover and show us signs they can take and tolerate their milk well.

As your baby recovers you will be able to commence breast feeding if you wish to do so. If your baby had a pure OA they will have their milk via their gastrostomy. This too will be slowly increased as your baby tolerates having the milk. Occasionally some babies need to have intravenous feeding whilst having milk feeds introduced. This is called parenteral nutrition (PN) which provides all the nutrients your baby needs to grow which they would normally get from milk. PN is given via a special ‘drip’ in their veins. This will be explained to you at the time.

What milk can my baby have?

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 breast milk we will support and help you in expressing your breast milk and establishing breast feeding. We have facilities on NSU for expressing your breast milk 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.

Will my baby have other problems?

Sometimes babies with OA and TOF can have associated medical problems, genetic problems or both. These can include problems with the heart, kidneys and spine. Your baby will be closely examined by the doctors, have X-rays and ultrasounds (scans) and other investigations as necessary to check if they do have any other problems. This will be explained to 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. Some babies with OA and TOF are only in for a few weeks, others can be in for months.

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 you baby is tolerating milk feeds and gaining weight they can go home.

Your baby will be followed up initially on NSU by the specialist neonatal surgical nurse and then by a paediatric surgeon in our outpatient clinics here at the Sheffield Children’s Hospital or at your local hospital. Your baby will also have regular follow up with the respiratory paediatricians here at this hospital.

What is the long term outlook for my baby?

The majority of babies with OA and TOF do not have long term complications from this condition. They grow up to lead a normal life. If they do have problems these commonly are related to the following issues.

Breathing

Your baby may be prone to chest infections and asthma but this will be monitored and treated if it is a problem. The trachea may be floppy by the operation site, this is known as tracheomalacia. Your baby may develop a cough which is known as a ‘TOF cough’.

It may sound worrying but is not serious. They may have noisy breathing and this will need to be monitored and may require treatment if it becomes a problem.

Difficulties swallowing

The oesophagus may narrow where it was joined (known as an oesophageal stricture) and this can cause problems with feeding. This is treated by having the stricture dilated (stretched) under a general anaesthetic.

Your baby may have problems swallowing certain foods when they are older. Drinking plenty of fluids with the meal and chewing slowly will help. Support for this will be given as necessary by your surgical team and the speech and language therapists.

Gastro oesophageal reflux (GOR)

This is when the contents of the stomach comes up the oesophagus causing pain and irritation. Medicine and supportive measures can help with this. Occasionally they may need further surgery.

Are there support groups?

TOFS

St George’s Centre
91 Victoria Road
Netherfield
Nottingham NG4 2NN

0115 961 3092
info@tofs.org.uk
Website: www.tofs.org.uk

BLISS

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

68 South Lambeth Road
London SW8 1RL

www.bliss.org.uk