TOF - Tracheo-Oesophageal Fistula and OA - Oesophageal Atresia

Oesophageal Atresia [OA] is a rare condition where a section at the top of the oesophagus [gullet] is closed off, forming a blind-end. This means food cannot pass from the throat to the stomach.

Tracheo-Oesophageal Fistula [TOF] is another rare condition, which tends to occur alongside oesophageal atresia. This is where the bottom end of the oesophagus is joined to the trachea [windpipe].

It is not known what causes OA and TOF. It is not due to anything that happened during pregnancy. It is very rare, occurring [in isolation] in between one in 3500 and one in 5000 births.

Surgical Treatment

To repair the TOF, surgeons separate the oesophagus from the trachea and repair the part of the trachea where the oesophagus was originally joined. The method used to repair the OA depends on the distance between the ends of the oesophagus. In most cases, the surgeons will cut the blind end of the oesophagus and then join the two ends together to form a continuous passage from the throat to the stomach.

In rare cases called long gap OA where the distance between the ends of the oesophagus are too large for the surgeon to be able to join them straightaway, different treatment is needed. An oesophagostomy may be formed. This is when the oesophagus is brought out through a hole in the throat. This allows the child to swallow saliva [and later food] until such time as the ends of the oesophagus can be joined together.

General Outlook after Surgery

If the OA and TOF occur on their own, with no other associated problems, the outlook for children who have them is good, with the majority growing up to live normal lives. There are however a number of problems TOF children can face.

Respiratory [breathing] problems can occur, which are sometimes serious enough to need a stay admission to hospital. These tend to improve with age, and have little effect on the child's breathing when older. Some children however, continue to have a distinctive TOF cough but this is not at all serious.

The area of the trachea which was repaired may become floppy [tracheomalacia] which can cause breathing problems. In severe cases this floppiness may also involve airways lower down in the lungs [bronchomalaecia]. Rarely, tracheobronchomalaecia may make it necessary for the child to have a tracheostomy and assistance with their breathing. This floppiness of the airways disappears as the child grows and the airways strengthen.

Some children who have had OA and TOF develop a problem with gastro-oesophageal reflux. This is where the contents of the stomach flow back up the oesophagus [gullet] causing pain, irritation and in severe cases breathing difficulties. This can often be managed with medication. Some cases may require an operation called a Nissen's Fundoplication. This involves tightening the top of the stomach to prevent the contents flowing backwards.

Feeding problems may also occur as the child gets older. This is due to the oesophagus narrowing where it was originally repaired. Sometimes it needs widening and this will be done in an operation under general anaesthetic. Some children also complain of problems with swallowing and need to have a drink with all food.

[Information based on that kindly provided by Great Ormond St Hospital]