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What is Oesophageal Atresia?

• What is Oesophageal Atresia?
• What is a Tracheo Oesophageal Fistula?
• What sort of problems may occur once the Oesophageal Atresia has been repaired?
• Does Oesophageal Atresia always take the same form?
• Is it a modern condition?
• How frequently does it occur?
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What is Oesophageal Atresia?

Oesophageal Atresia (OA) is the term used to describe a congenital obstruction in the oesophagus in newly born babies. It is a congenital anomaly in which the oesophagus ends in a blind upper pouch. The oesophagus is commonly known as the gullet, foodpipe, and in babies born with Oesophageal Atresia this means that the gullet is not connected all the way to the stomach, and as result food and saliva cannot pass into the stomach. As you can see from the diagrams below, quite often the baby born with oesophageal atresia may also suffer from a Tracheo Oseophageal Fistula. (TOF)

What is a Tracheo Oesophageal Fistula?

Tracheo Oesophageal Fistula is the term used to describe the condition where a section of the baby’s Trachea (windpipe) is abnormally connected (that’s the fistula part) to the baby’s oesophagus.

Some babies are born with additional defects to the spine, heart, kidneys, anus and radial bone. If a baby has three or more of these anomalies, it has what is known as “VATER Association”.

Major surgery is required early in life. TOF/OA children are all different in that some may have few or no problems following their repair, whilst others have a lot of problems in their early years.

What sort of problems may occur once the Oesophageal Atresia has been repaired?

It should be remembered that all children born with a TOF/OA are different and that there are no hard and fast rules for the difficulties the children may encounter once they have been successfully operated on. The success rate of the surgery is virtually 100%!

Most TOF/OA babies grow up to live perfectly healthy normal lives, and apart from some dietary preferences, and of course the surgical scars, may be no different from any other child. Whilst some children are fortunate not to have any further complications, others may suffer from any of the following conditions;

• A very characteristic cough known as the “TOF Cough”.
• Due to the repair of the foodpipe the TOF/OA child may have abnormal swallowing mechanisms and will have to learn or still be learning to cope with their particular swallowing problems. Some may require a special diet, or fluid with their meal to assist in swallowing. Many TOF/OA children have developed unique coping mechanisms or have dietary preferences that they feel comfortable swallowing.
• Occasionally food may get “stuck” in the oesophagus and the child may appear to choke and/or be sick. Managing this situation may vary from the food being dislodged with a drink, by vomiting, or by seeking urgent advice from the medical profession.
• Some children may require a dilatation of the oesophagus from time to time in cases where the oesophagus develops strictures. I.e. where it appears to be narrowing.
• TOF/OA children may suffer from mild to severe gastro-oesophageal reflux, where the contents of the stomach can wash back up the foodpipe. They may very easily be sick. In a few TOF/OA children gastro-oesophageal reflux may be severe enough to warrant further surgery known as a fundoplication.

Does Oesophageal Atresia always take the same form?

No. There are five main types of abnormal conditions of Oesophageal Atresia and/or Tracheo- Oesophageal Fistula. These are presented below in a simple diagrammatic form.

Figure 1. Oesophageal Atresia with a distal tracheo-oesophageal fistula. (Abnormal connection between windpipe and stomach). The most frequently encountered anomaly.

Figure 2. Oesophageal Atresia with a proximal fistula. (Abnormal connection between windpipe and gullet. No connection from gullet to stomach.

Figure 3. Oesophageal atresia combined with fistulae from both oesophageal segments. (Abnormal connection from gullet and stomach to windpipe.)

Figure 4. Isolated Oesophageal Atresia without a fistula.(No connection between gullet and stomach)

Figure 5. Tracheo-oesophageal fistula of the”N” or “H” type. (abnormal connection between gullet and windpipe)

Figure 6. Repaired Oesophageal atresia

Is it a modern condition?

As with many problems of this type, very little information was recorded before the 20th Century. The earliest mention was in England in 1670 and the first detailed description of the most frequently encountered form of Oesophageal Atresia was written by Gibson in London in 1696. The first operation was not performed until 1888 in London and the baby died shortly after surgery. The major breakthrough occurred in 1941 when an American surgeon (Cameron Haight) achieved survival by joining two ends of the oesophagus and thus overcoming the obstruction. This work followed earlier pioneering efforts in Boston and elsewhere.

The surgical principles laid down by Haight have been the nucleus for information and surgical techniques since that day. The first two Australian babies to survive were operated on at the Royal Children’s Hospital Melbourne in 1949 and the father of one of these children was the first President of OARA.

How frequently does it occur?

The condition seems to occur about once every 3,000 births. We would therefore expect to find about 15 to 20 babies born in Victoria each year with Oesophageal Atresia.