OA/TOF - Oesophageal atresia and/or tracheo-oesophageal fistula

All surgery carries a small risk of bleeding during or after the operation. Even if the gap between the ends of the oesophagus is quite small, it can still be difficult to join together. After the operation, the join may leak or it may narrow over time, but these can both be treated. Your child will be reviewed regularly for a long time after the operation.

Every anaesthetic carries a risk of complications, but this is very small. Your child’s anaesthetist is a very experienced doctor who is trained to deal with any complications. For more information read our factsheet on anaesthesia and risk in infants.

Your baby will go to the intensive care unit (ICU) to recover. For a while after the operation, your baby will need help with breathing and will be connected to a ventilator. All babies are closely monitored after the operation. Your baby will be connected to monitors to check breathing, heart rate and oxygen levels. Pain relief will also be given through a ‘drip’.

Your baby will be transferred to a hospital ward when they no longer need intensive care. While your baby’s intestines recover and start to work, they will have an intravenous drip of fluids. Feeding usually starts a few days after the operation, with breast or bottled milk given through a naso-gastric tube (tube passed through a nostril, down the oesophagus and into the stomach). As your baby recovers, you will be able to breast or bottle feed.

Some babies’ intestines take a little longer to recover, so need to be fed through a tube into the veins (total parenteral nutrition or TPN). Naso-gastric feeding is then tried when your baby’s intestines start to show signs of recovery.

Once your baby is feeding properly and gaining weight, you will be discharged home.

If your OA and TOF baby has no other associated problems, the outlook is excellent. Most children grow up to live normal lives. The outlook for children with OA and TOF who have other difficulties, such as extreme prematurity and heart conditions will vary depending on how severe these other problems are.

There are a few problems which may happen after surgery that you should know about. They happen most often in the first few years after the operation and improve as your child grows older.

Caring for your baby does not begin when you take your baby home. You can share in your baby's care while still in hospital. Don't be afraid to ask questions. It is often hard to fully understand things when you are worried or anxious. People caring for babies and children in hospital understand that all parents are worried and anxious when their child is not well and needing medical attention. Confused feelings are normal at this early time.

Many parents speak of their deep disappointment that they were unable to take their baby home from the maternity hospital in the usual way. Sometimes people can feel confused and angry. Most parents feel frightened to see their baby connected up to tubes and machines. Some parents may not want to see their baby until they are well on the road to recovery and free of equipment. If that is the way you feel, remember that these feelings are perfectly normal. The sooner parents are involved with their baby the better. The more you know your child and care for them in hospital, the easier it will be when you take your baby home.

Parents are the real experts in the care of their child. Every TOF child, like every other child, is different. Though you need the help of other experts for a time, you are the most important people in the team caring for your child. A multidisciplinary team of experts will help you look after your baby by monitoring their progress during regular clinic visits.

Your child may experience a number of problems in the early years. Problems include swallowing difficulties and a higher number of childhood coughs and colds.

It is important to keep in contact with your baby's doctor if you are worried about anything.

Many children with OA/TOF experience some form of feeding difficulty, however the amount of difficulty varies between children. Feeding will improve as your child gets older.

When you take your baby home you will be given a feeding program suitable for your baby and their specific needs. You should start feeding orally as soon as your child is allowed. Many children can breastfeed. As many children with OA/TOF suffer from reflux, they often cope better with smaller more regular feeds.

Solids should be introduced around 6 months, as they would with any other baby, unless you are told otherwise by your doctor.

As your baby grows you will add solids and increased varieties of foods. Often children with OA/TOF have difficulty progressing with their food textures from very smooth runny purees to more solid or lumpy foods.

OA/TOF children may take longer than usual with the introduction of solids into their diet. In order to give your baby a good start to feeding, your child needs to stay on smooth purees and "mushy foods" for a longer period of time to reduce the risk of choking. Feeding should be done under close supervision to reduce the risk of choking.

A blender or small mincer is really helpful when preparing "mushy" food.

We recommend that you give your baby a wide range of food. Frequent and early exposure to a wide range of tastes has been shown to play a major role in what food children will eat.

Common problem foods for children with OA/TOF include:

• meat (unless pureed or minced)
• bread/doughy foods
• hard raw vegetables (carrots, apples)
• foods with skins (grapes, tomatoes).

When you introduce new foods, always blend, mince or chop them finely. When your child gets used to eating the new food, gradually make the sizes bigger. Also let your child watch you cut it up. Give them a nibble while you are doing it. As they grow, they won't be afraid to eat new things if you have been introducing them to food gradually.

Some children complain of problems with swallowing and need to have a drink with all food. This is often caused by the oesophagus not being coordinated.

Try to make sure that meal times are quiet and peaceful (this is not always easy with a young, energetic family). When children are in a hurry or excited, swallowing problems are more likely to occur. During social occasions keep an eye on your child. Things like nuts and potato crisps left at the child's level can be a significant problem.

The Speech Pathologist and Dietician can give more individualised advice on feeding. They can help you with advice on weaning and suggest the types of foods that are most suitable for your child as they grow older. The Speech Pathologist can help with problems with swallowing including troubles with textures and also help with exercises to help with chewing and swallowing at meal times. The Dietician can help with ensuring your child’s intake is meeting their nutritional needs and recommend higher energy options or additions if there are concerns with growth.

Sometimes the oesophagus becomes narrow at the site of the join (anastomosis) - this is called a stricture. Babies with a stricture have problems swallowing and may have increased vomiting or may choke or gag with feeds. Stricture is treated with dilation (stretching the narrowed food pipe). This procedure is done under a general anaesthetic either in the radiology department or with the help of an endoscope in the operating theatre. The purpose is to widen the narrowed oesophagus to help with feeding.

Children with OA/TOF often develop a problem with gastro-oesophageal reflux disease. This is where the contents of the stomach flow back up the oesophagus causing pain and irritation. This may cause a baby to vomit, but not always. As acid reflux can irritate the oesophagus and increase the chances of stricture, your child will be started on an anti-reflux medication after the initial operation to reduce the risk of this developing. Your child will need regular surveillance endoscopies to monitor their reflux.

In addition to a possible abnormal connection (fistula) between your baby’s windpipe (trachea) and food pipe (oesophagus), the windpipe in children with OA/TOF is unduly floppy due to a lack of properly developed cartilage to support its walls. This is known as "tracheomalacia" (or soft trachea) and may cause noisy breathing at rest or with activity, a loud "barking" cough, reduced ability to bring up phlegm/sputum and breathing difficulties.

Many, but not all children with OA/TOF suffer from what has become known as the "TOF" cough. This is a harsh, brassy, "barking" cough, which sounds as if the child is very sick. Remember, this does not mean that your child is sick and often family and friends need to be reassured that your child is fine.

Sometimes due to their floppy windpipe, your child might experience a sudden breath-holding episode or "blue spell". This usually happens during eating, if a piece of food becomes stuck in the food pipe. The lump of food puts pressure on the windpipe significantly narrowing it so that air cannot move in and out. As a result, breathing becomes extremely difficult and your child may change colour, becoming a dusky red or even blue. Please contact your doctor immediately if this happens.

Children with OA/TOF are more likely to experience prolonged colds and even pneumonia in the first few years of life. This is because they cannot clear their secretions effectively due to their floppy windpipe. Although this is likely to improve by the early school years, chest physiotherapy and measures to increase mucus clearance can help. Physiotherapy exercises can be taught at the hospital by a trained physiotherapist. These can be done at home and help when your baby has a cold. We would recommend that your child also have the influenza vaccine every year to prevent serious pneumonia.

In time most respiratory symptoms improve. It is important though that your child be regularly reviewed throughout childhood so that any ongoing problems can be identified and managed so that your child’s wellbeing is maintained.