Gastrointestinal problems

The main symptoms that patients experience in adult life are swallowing difficulties (dysphagia) and reflux symptoms (heartburn and regurgitation of food). These symptoms are very common, occurring in up to 80% of adults who have had surgery for OA.

Such issues occur as a result of a loss of normal anatomy and nerve supply to the lower oesophagus:

• Surgery to repair OA involves bridging the gap between the upper and lower oesophagus. This almost invariably leads to some shortening of the oesophagus, which tends to pull the junction between the oesophagus and the stomach up above the diaphragm. This is known as a sliding hiatus hernia. Normally the oesophago-gastric junction (the point at which the oesophagus meets the stomach) lies below the diaphragm, in the abdomen.
• The nerve supply to the oesophagus consists of two parts. The left and right vagus nerves travel down along the oesophagus from the brain to supply organs in the chest and abdomen, including the oesophagus, and an intrinsic network of nerves in the wall of the oesophagus, which makes the oesophagus contract to create peristaltic waves pushing the food to the stomach. Although the vagus nerves usually remain intact after repair of OA, there is always an interruption of the network of nerves within the wall. The nerve supply to the lower segment of oesophagus is therefore invariably impaired. This leads to poor functioning of the muscles in the wall of the lower oesophagus.

The likelihood that an adult who underwent surgery for OA in infancy will suffer from continuing oesophageal symptoms depends on:

• The amount of scar tissue from surgery
• The length of the gap that had to be covered by the surgery
• The presence or absence of a hiatus hernia
• Whether or not an oesophageal replacement had to be used (36)

Whilst most people with repaired OA/TOF will have dysphagia, GORD or other gastrointestinal symptoms; one difficulty for the patient and doctor is that they may not be aware what is pathological. Adults born with OA/TOF have had a lifetime of adaptation to these symptoms, and no experience of a normal oesophagus, so it is often useful to use a reflux or dysphagia questionnaire in this instance to help determine symptoms fully.