Tracheo-oesophageal fistula (TOF)
TOFs are either congenital or acquired. Congenital TOFs present shortly after birth and are treated by paediatric surgeons. This article is about the presentation and treatment of TOFs in adults.
A TOF is an abnormal connection between the trachea (windpipe) and the oesophagus (gullet). A TOF can arise at any level along the length of contact between the oesophagus and the trachea. In adults, the vast majority of TOFs are acquired due to a variety of conditions. This includes malignancy as well as trauma from interventions to the tracheostomy and oesophageal stenting. TOFs can also occur as a complication of successful treatment of oesophageal malignancy, particularly after chemoradiotherapy and stenting.
Presentation and diagnosis
Presentation depends on the size of the fistula.If the TOF is tiny, there may be no symptoms. If it is large enough to allow saliva or fluid to pass from the oesophagus into the trachea, the presentation is usually with a recurrent cough, particularly on eating and drinking, as well as recurrent chest infections.
The diagnosis can be made with radiological imaging, either with a contrast swallow or CT scan as well as on bronchoscopy and oesophagoscopy.
This depends on the underlying cause of the TOF as well as its position and size. TOFs are rare and require the expertise of a multidisciplinary team for best outcomes. The skills required include the expertise of interventional gastroenterologists, respiratory physicians, thoracic surgeons and upper gastrointestinal surgeons.
The ideal treatment for a TOF is to close it surgically, ensuring that the underlying disease is also treated. The surgery can be technically challenging requiring experience in operating on the trachea and oesophagus as well as the ability to reconstruct the oesophagus using the stomach or the colon. High TOFs also requires the expertise of being able to operate in the neck.
There are very few centres in the UK that have the expertise in treating TOFs. Professor Mughal and his team at UCLH have developed expertise in this area over the last six years and have successfully treated eight patients with a variety of TOFs. The following are two examples.
Miss A presented at the age of 29 with symptoms suggestive of asthma. When she failed to improve from treatment for asthma she underwent further investigations including a CT scan, which showed a small TOF situated at the root of her neck. After detailed assessment she had an operation through the neck to close the fistula with interposition of muscle tissue between the separated oesophagus and trachea. She made a straightforward recovery and was discharged a week later with her symptoms resolved.
Small hole (fistula between windpipe and gullet) seen on bronchoscopy on the left & CT scan on the right
Miss B, who was 55, was known to have a condition called scleroderma which affects the oesophagus and results in swallowing difficulties. Her swallowing worsened, and she underwent an endoscopy which showed an abnormal area in the oesophagus from which biopsies were taken and showed a B cell lymphoma. She had successful chemotherapy but unfortunately developed a TOF.
She underwent complex surgery to closure of the fistula along with the removal of the diseased oesophagus and reconstruction by shaping her stomach into a tube which was placed under the breastbone and pulled up to join the healthy remaining oesophagus in the neck. Although she developed a leak from the join in the neck, this eventually healed, and she is now able to swallow properly and has been cured of her fistula.
A view of the windpipe through a bronchoscope showing a large hole communicating with the gullet