In this section
As a young person with oesophageal atresia (OA) and tracheo-oesophageal fistula (TOF) your ongoing care is important.
As you become an adult, you will need to understand your care and monitoring needs, and you will need to transition from paediatric to adult health care.
The information in this booklet will help you learn about your condition, what surgery you had when you were a baby, and learn what problems may develop so you know when to see your doctor.
In normal development, babies are born with an oesophagus and a trachea.
The oesophagus (food pipe) is a tube that transports food, fluids and saliva from your mouth to your stomach.
The trachea (windpipe) is a tube made of cartilage that connects your larynx (voice box) to smaller tubes called bronchi which connect to your lungs. The trachea transports air in and out of your lungs as you breathe.
When you were diagnosed as a baby, you may have had a range of scans and tests to check for other problems. This is because OA/TOF is commonly associated with VACTERL association which may affect your heart, kidneys, spine and other organs.
It is not known what causes OA or TOF, but they usually occur together. In some cases, OA or TOF occur on their own without the other.
Sometimes OA/TOF is diagnosed before birth, but it is more commonly diagnosed soon after a baby is born.
There are five main types of OA/TOF. It is important you understand your condition when it’s time for you to move to adult health care – this will help you understand any problems that develop. You need to know:
If you are unsure what type of OA/TOF you were born with, your parents, carers or medical team will be able to provide you with this information.
Surgery performed to repair OA/TOF depends on the type of OA/TOF you had. You may have one or more operations, and most of your surgery would have happened when you were a baby.
If you are unsure what type of surgery you had, your parents, carers or medical team will be able to provide you with this information.
OA/TOF can only be repaired with surgery. The surgery is usually done in babies when they are only a few days old.
OA/TOF surgery involves the following two procedures, which are usually done through a cut between the fourth and fifth ribs on the right side of the chest (called a thoracotomy):
The surgery to repair H-TOF is slightly different to an OA/TOF repair because the oesophagus has developed normally and does not need to be repaired.
A long-gap OA is when the space between the upper and lower oesophagus is too large to repair straight away. A long gap often occurs in babies with pure OA, OA with a proximal TOF, and sometimes with OA and distal TOF.
Babies born with long-gap OA spend a much longer time in hospital. Long-gap OA often requires more than one surgery over a period of time (usually a few months) to completely repair the oesophagus. The complete repair of the oesophagus is often done in the first few months of life.
Babies born with long-gap OA may need one or more of the following surgeries:
There are a few options for an oesophageal replacement procedure:
If you have been treated in a paediatric hospital, you will usually go through a process called ‘transition’ that helps to prepare you for your move to care in an adult setting. Transition begins in your teenage years, usually around the age of 15, sometimes earlier.
There may be a transition lead who will work with you, your parents and carers, and your doctors to develop a transition plan. This transition plan will help you develop the skills (such as knowing more about your condition and gaining confidence to communicate with your care team/s) you need to independently navigate and take care of your health to the best of
Transition is important to empower you (and your parents and carers) to have a greater sense of control over the process and to maximise your capacity to live well and to achieve your goals.
People who have had surgery to repair OA/TOF generally have an excellent quality of life. However, oesophageal problems can develop at any time, sometimes without symptoms. To ensure any problems are picked up and can be treated before further complications occur, it is important you have the following regular check-ups:
Regardless of the type of OA/TOF you have, you will need life-long follow-up with a gastroenterologist (a doctor who specialises in the digestive system) for the ongoing monitoring of your oesophagus.
You will need regular gastroscopies to monitor your oesophagus for any changes. A gastroscopy is done under sedation (you are not awake for the procedure) and is a procedure where the gastroenterologist uses a small telescope-type instrument with a camera to look inside your oesophagus and stomach. These areas are examined and biopsies (small samples) of tissue may be taken for testing.
You will need to see your gastroenterologist more often if you develop any problems or if your doctor has asked to see you more often.
Having a good local GP who you trust and who understands your condition is important as they can help to coordinate your care needs as an adult in the community.
Your GP plays a key role during and after your transition from paediatric care, as they can communicate with your specialists and refer you to other healthcare professionals or supports if needed.
After the initial surgery as a newborn, some people who have had an OA/TOF repair have ongoing or life-long problems. In some cases, problems may develop in adulthood.
A common problem is gastro-oesophageal reflux.
Reflux is when the contents of the stomach are regurgitated (brought back up), either up the oesophagus or into the mouth.
The stomach contains acid to help digest food, so when reflux occurs the symptoms may include:
You should see your gastroenterologist if you have any symptoms of reflux.
If reflux is ongoing (chronic) and not treated, it can cause further problems such as oesophagitis, oesophageal strictures, Barrett’s oesophagus, and breathing issues.
Reflux can be treated with medicine to reduce acid in the stomach. If medicines do not help control your reflux symptoms, anti-reflux surgery may be required (fundoplication). This operation is done to strengthen the muscle in your lower oesophagus and reduce reflux. If you have already had the anti-reflux surgery as a child and have reflux symptoms, you may need to have another procedure to re-tighten or repair the initial fundoplication.
Sometimes you may have reflux with no symptoms. This is called silent reflux. Regular follow up with your GP and gastroenterologist is important in case you have silent reflux and experience no symptoms. Your doctors will be able to pick this up during your regular appointments.
Reflux of stomach acid can irritate the lining of the oesophagus causing inflammation. You will need to see a doctor if you develop any pain or have other symptoms of reflux.
An oesophageal stricture is a narrowing in your oesophagus. This usually occurs where the two ends of your oesophagus were joined together.
Swallowing can be difficult if the oesophagus narrows because it can slow or stop food moving down your oesophagus.
Treatment for an oesophageal stricture is to have this narrowed area stretched. This procedure is known as an oesophageal dilatation and is usually performed during a gastroscopy.
Oesophageal dilatation can be done:
Oesophageal dilatations are common. Some people with OA/TOF may have had many dilatations as a child and into adulthood following the repair of their OA/TOF. Others may only need a few, and some may not need any.
Chronic reflux can cause a permanent change in the cells that line the oesophagus – this is called Barrett’s oesophagus.
Barrett’s oesophagus is a pre-cancerous change in the cells of the oesophagus. It is important to continue regular appointments with a gastroenterologist so that any changes can be detected early.
If you are diagnosed with Barrett’s oesophagus, you may need more treatment for reflux and more frequent gastroscopies, so your oesophagus can be monitored for any further changes.
The risk of developing oesophageal cancer later in life is low, however it is more common in people with OA than in the general population.
Two types of oesophageal cancer have been reported in a very small number of patients with repaired OA: adenocarcinoma and squamous cell carcinoma.
It is important to continue regular appointments with your gastroenterologist, so any changes in your oesophagus can be monitored.
Common breathing problems may include:
If you have any breathing problems, you should see your GP as soon as possible or call an ambulance in an emergency.
If you have swallowing problems (dysphagia), you may experience food getting stuck when you swallow.
There are two main reasons you may have problems with swallowing:
If you have swallowing problems, you can try having a drink with meals to help move the food down. It is important to be reviewed by your gastroenterologist if you have any swallowing problems.
Anyone who has had an OA/TOF repair should maintain a healthy lifestyle into adulthood, including eating a healthy diet, exercising regularly and avoiding smoking or use of illicit drugs.
If you choose to drink alcohol, be aware it may cause increased problems with acid reflux. You may need to be more careful than your friends in order to drink safely and responsibly for your health.