esophageal atresia and tracheoesophageal fistula

Oesophageal atresia is a rare birth defect that affects the baby's oesophagus, the tube that carries food from the mouth to the stomach.

In oesophageal atresia, the upper part of the oesophagus doesn't connect with the lower oesophagus and stomach (see diagram, left). It ends in a pouch, which means food cannot reach the stomach.

It often happens together with another birth defect called a tracheoesophageal fistula, which is a connection between the upper part of the oesophagus and the windpipe (trachea). This causes air to pass from the windpipe to the oesophagus and stomach, and stomach acid to pass into the lungs.

It's not known why the defect develops, but it's more likely to happen in babies of mothers who had too much amniotic fluid in pregnancy (polyhydramnios).

The defect means the baby won't be able to swallow and digest food safely, or at all.

Oesophageal atresia and tracheoesophageal fistula are thought to affect about one in 2,500-3,500 births.

Why prompt treatment is needed

Oesophageal atresia and tracheoesophageal fistula are life-threatening conditions.

If they're not corrected soon after birth, the pouch at the end of the oesophagus may fill with food and saliva, which can overflow into your baby's windpipe. Saliva and stomach fluids are inhaled, which can cause choking and pneumonia.

How the defects are repaired

An oesophageal atresia repair operation is usually done soon after birth.

Your baby will be taken to the neonatal intensive care unit, where they are given a general anaesthetic. They will receive nutrition into a vein (intravenously) and nurses may use a suction tube to stop fluid going into your baby's lungs.

Both oesophageal atresia and tracheoesophageal fistula can be repaired in the same operation.

The surgeon makes a cut on the right side of the chest, between the ribs, and closes off the abnormal connection (fistula) between the oesophagus and windpipe.

Then, the surgeon will sew together the upper and lower parts of the oesophagus.

Sometimes, your child will need to wait two-four months for the operation, in which case they'll need to have a gastrostomy tube temporarily fitted under a local anaesthetic. While you wait for surgery, your baby's oesophagus may be widened with a special instrument called a dilator, which will make the surgery easier.

After surgery

After surgery, your child will be kept in the intensive care unit and placed in an incubator. They may also need:

antibiotics
a machine to help them breathe (ventilator)
a tube into their chest to drain fluids and keep their lungs open
nutrition given to them intravenously
oxygen
pain medicine

You'll be able to take your baby home once they are taking food by mouth or via their tube and are gaining weight.

Risks of surgery

With any surgery, there is a small risk of bleeding and infection.

For this particular operation, the additional risks are:

collapsed lung (pneumothorax)
food leaking from the area being repaired
hypothermia (low body temperature)
narrowing of the repaired oesophagus (see below)
re-opening of the abnormal connection (fistula) between the oesophagus and windpipe

Outlook

Most children who have had surgery to repair oesophageal atresia and/or tracheoesophageal fistula will grow up to have normal lives.

However, there's a chance that the repaired part of the oesophagus narrows, in which case further surgery is needed to widen this (an endoscopy to inflate a balloon inside the oesophagus).

Your child may also experience heartburn and acid reflux, and some infants may have problems with breathing, swallowing and feeding, as well as growth problems, so need to be continuously monitored by specialists.