The esophagus is in close proximity to surrounding vital organs and vessels, such as the trachea, aorta, and pulmonary veins. Advanced esophageal cancer frequently involves the trachea, with 20-36.6% of patients developing tracheal invasion[5, 6] and 5–10% developing TEF,[7] a potentially fatal complication. TEFs are generally more likely to occur after chemoradiotherapy for advanced esophageal cancer,[8] and once completed, they have an extremely poor prognosis, with an average survival time of approximately 1 month.[9]
The most difficult aspect of TEF management is the management of digestive fluids that flow into the trachea through the fistula. Most have a fatal course with repeated aspiration pneumonia during the course of management.
There have been a few cases of TEFs due to benign or postoperative complications that have been cured by innovative surgical procedures, such as the use of a muscle flap[8] or three-step surgery;[10] however, TEFs due to esophageal cancer are more complicated to treat because the patient is also receiving cancer treatment.
The treatment of TEF in esophageal cancer is a combination of chemotherapy or chemoradiation therapy (CRT) for the tumor and stenting, esophageal bypass, or the use of a muscle flap for TEF, all of which are often performed as palliative—rather than curative—treatments.
Esophageal stents and airway-esophageal double stents are often selected for TEF, especially to close the fistula and improve the quality of life.[11, 12] Although there are reports of improved survival,[13, 14] it is considered to be a palliative treatment, and associated disadvantages include the possibility of fistula enlargement and difficulty in surgical manipulation due to stenting. Surgical treatment may be an option, but most patients undergo esophageal bypass surgery[15, 16] or muscle flap coverage[9, 17] as a palliative treatment. There are reports of two-step surgery for TEF caused by lung cancer[18] and TEF caused by malignant lymphoma that was closed after continued chemotherapy;[19] however, we were unable to find any reports of radical surgery for TEF caused by esophageal cancer.
In the present case, the patient developed an upper TEF; however, prior tracheostomy allowed for the continuation of chemotherapy under strict airway and nutrition management, and successful chemotherapy enabled the en bloc resection of the TEF by pharyngo-laryngo-esophagectomy and R0 resection as conversion surgery. Currently, this patient remains alive without recurrence at 9 years after treatment.
To safely administer chemotherapy, it is necessary to reduce the risk of aspiration and pneumonia as much as possible by preventing the backflow of digestive fluids into the fistula and collecting digestive fluids that have entered the trachea through managements such as continuous suctioning over the cuff of a tracheal cannula, prohibiting saliva swallowing, and maintaining constant upper body positioning (Fig. 5). Generally, the creation of a permanent tracheostomy is considered if the trachea can be dissected cephalad of the suprasternal margin.[20] Before surgery, biopsy is performed at the level of the tracheal incision by bronchoscopy through the tracheostomy site and the tracheal incision level is determined based on the confirmation of negative biopsy results. This ensures the achievement of complete resection of the tumor. Sakai et al.[21] also reported no difference in the recurrence pattern in patients undergoing laryngeal cancer surgery, even when tracheotomy was performed first. If the TEF caused by the tumor is located caudal to the suprasternal margin, the tracheal cannula may not be long enough or a permanent tracheostomy may not be safely created, in which case stenting to close the fistula or esophageal bypass may be considered.
In this case, tracheostomy was performed first due to the presence of severe airway stenosis. Even in the absence of airway stenosis, however, creating a tracheostomy prior to other treatments is advantageous as it facilitates confirmation of the fistula site, allows for better management of aspiration and pneumonia, and facilitates biopsy to determine the level of the tracheal incision before surgery. When a patient with advanced esophageal cancer with tracheal invasion develops TEF, we believe that it is useful to create a tracheostomy before performing other treatments.
In patients with advanced hypopharyngeal and cervical esophageal cancer with upper TEF, the strict management of TEF by tracheostomy may facilitate subsequent treatment with chemotherapy and curative surgery.