Tracheal oesophageal fistula and oesophageal atresia

Oesophageal atresia (OA) is an abnormality that happens during development of a baby while still growing in the mother’s uterus, where the oesophagus (food pipe) has developed blind ending rather than being continuous from the mouth to the stomach.

Tracheal Oesophageal Fistula (TOF) is an abnormal connection between part of the oesophagus and the trachea (breathing tube).

Babies with OA have surgery in the first few days or weeks of life to repair this.

Diagnosis of an isolated TOF is more difficult, and can be missed.  Once found, a TOF is repaired immediately.

Following OA and TOF repair, there can be ongoing effects on the food pipe and windpipe.  These can impact throughout a person’s life.

During childhood, the children who have had surgery for OA and TOF are regularly reviewed by your surgical and gastroenterology team.

When you are nearing the age of 16, we will need to discharge you from children’s (paediatric) services and follow up.

Once you are discharged from the paediatric services, we recommend that your GP refers you to a gastroenterologist for surveillance endoscopy every 10 to 15 years. 

This is the camera test to look for changes in the lining of your oesophageal wall caused by reflux. You would have had one before you were discharged from Great Ormond Street Hospital. You may not always know you have reflux, as symptoms can be mild in early stages. It is important to treat reflux to prevent more serious problems later on.

Adult patients may often be offered a nasal endoscopy whilst awake. This is a safe procedure in adults and offers the advantage of being an outpatient procedure without the need for a general anaesthetic.

Gastro oesophageal reflux (often called reflux)

Reflux symptoms could start at any time in your life. Symptoms include burning pain in your oesophagus, acid taste in your mouth or an uncomfortable feeling in your chest. You should be aware that due to the OA and TOF you are more prone to suffer from reflux. If it continues for more than a few weeks and you haven’t had an endoscopy in the last 10 years you should ask your GP to refer you to a gastroenterologist for a review.

Oesophageal dysmotility 

Oesophageal dysmotility is common in people born with OA and TOF.  Your oesophagus works by contracting to push the food down, the same way you can feel your bowel contract when you really need the bathroom. In OA the nerves have been interrupted so this cannot happen as normal. The impact of dysmotility is variable. For many people it is hardly noticeable, managed by chewing food well and drinking plenty when eating. Some people may avoid certain foods they find difficulty, such as tough meat.

For a few people dysmotility may lead to more serious problems which limits their ability to eat a range of foods. Dysmotility can come and go.  If you are very tired or unwell, the dysmotility might be more noticeable.

If you find foods are getting stuck more than usual, chose softer, moist foods until you are feeling better.  If you are struggling to swallow foods that you can usually manage without a problem, and there is no obvious reason for this, speak to your GP about investigating for a stricture.

Strictures

Where the two ends of your oesophagus were joined by the surgery on your food pipe, the body will heal the stitch line and this causes the area to tighten up and cause a scar (in a similar way to how a cut on your arm will tighten as it heals). This narrowing caused by the scar is called a stricture.

Having a stricture means food and fluids can take longer to go down your oesophagus and can give you the feeling that food isn’t able to move down normally. Food may get stuck at the narrowing and you may bring them back up. Delay in treating the stricture can make the part of the food pipe above the stricture stretch and become baggy. This makes it even harder to push food down. It is therefore important that a stricture is treated. When a stricture is suspected, your GP or gastroenterologist (if you have one) can organise for you to have a contrast swallow x-ray at your local hospital.

Once diagnosed, the stricture will need to be stretched (a dilatation) which will be performed in a hospital by a gastroenterologist, interventional radiologist or surgeon.  If you have food stuck and are unable to move this with liquids, you should attend hospital and this can be sorted out more urgently.

Tracheomalacia

Your windpipe (trachea) is made out of cartilage, which is strong, flexible connective tissue In people born with OA and TOF the cartilage is softer, causing the trachea to be floppy rather than rigid. This floppiness in the trachea is called tracheomalacia. In young children it can cause problems with breathing, but usually improves with age. As a young adult though, you can still have a ‘TOF cough’. This is caused by the softer area of your trachea vibrating when you cough. A TOF cough is not serious. It is good to remember why you have it so you can explain it to health professionals if they ask.

Someone with tracheomalacia might be more prone to getting unwell with viruses as it is can be more difficult to move the mucus through the floppy area.  If you are getting more than two chest infections in a year or finding it difficult to clear and cough or cold you should discuss with your GP in relation to your OA and TOF.