Diagnosis

Diagnosis of ADF should be suspected by clinical symptoms and should be corroborated by respiratory or digestive endoscopy or both. Exploration with radiological images can help in the case of small stulas.

Because the initial symptoms are quite non-­ speci c, diagnosis of esophageal respiratory s- tulas can be delayed in time. In some cases the diagnosis of benign stulas can be delayed even up to 18 months after symptoms have started [6].

If patients have a predisposing condition (i.e., a known esophageal tumor) once symptoms ensued, suspicious and clinical diagnoses are relatively easy. Patients complain of coughing, especially when swallowing liquids or solids, due to the passage of these to the respiratory system through the stula (Ono’s sign). Cough can be productive with sputum mixed with secretions and in the case of large stulas, with food debris. These patients often also have episodes of bronchitis, dysphagia, recurrent pneumonia that are dif cult to treat, and signs of malnutrition [34, 35]. Symptoms of cough, stridor, or hemoptysis are usually more frequent if the tumor is originally from trachea or bronchi, and rare when the primary tumor is originally from esophagus.

In the series of Burt et al. [5] that include 207 patients, the main symptoms were cough (56%), aspiration (37%), fever (25%), dysphagia (19%), pneumonia (5%), hemoptysis (5%), and chest pain (5%).

In patients with assisted mechanical ventilation, the appearance of sudden abdominal distention should make us think about the presence of astula.

The diagnosis of tracheal or bronchoesophageal stulas by respiratory or digestive endoscopy or by both [36] is usually successful. However, when the stula ori ce is small and surrounded by infammatory tissue, diagnosis is more dif cult and may require the use of swallowed contrast [37] for dynamic endoscopic inspection. Air bubbles or foamy secretions of biliary aspect during exploration can appear and help in locating the stula (Figs. 39.2a, b and 39.3). When the ori ce is larger, the endoscopic diagnosis is much easier. A chest X-ray or chest computed tomography (CT) scan can help with small stulas but are rarely essential. However, radiological control is essential in monitoring the evolution of the disease.

After the development of a malignant stula, the prognosis is dire, with most patients dying within 1 or 2 months due to respiratory infections and malnutrition. Therapy is aimed at alleviating symptoms and maintaining quality of life (QoL).

Ninety percent of patients with malignant ADF have advanced or metastatic disease with high ECOG scores (Eastern Cooperative Oncology Group). Most patients present with frequent lung contamination, bronchial obstructions and aspiration, and lung superinfections, which are dif-cult to treat. In most cases, these complications

cause aspiration pneumonia leading to sepsis, mediastinitis, ARDS, and a fatal outcome.

Treatment Options

Managing patients with ADF is always challenging. The rst thing to consider is the etiology, since benign conditions are approached differently than those of malignant origin. Table 39.1 suggests an algorithm for management.

In malignantADF, the main goals of the management are: to treat recurrent infections with antibiotics, seal the communication between the esophagus and the airway if possible, and ensure proper enteral feeding. We have to consider that usually patients are in an advanced cancer condition; they present malnutrition, and recurrent infections; and they have received chemotherapy or radiotherapy that impact also on their physical conditions.

Surgical procedures such as the occasional esophageal exclusion, esophageal bypass, andstula resection and repair, which in benign pathology can have good results, in the presence of a malignant stula carry a high morbidity/ mortality rate, with poor results [5].

Endoscopic procedures should be aimed at closing the communication and preventing esophageal fuid from passing into the bronchial tree in order to alleviate the symptoms and improve the quality of life of a patient with an unfortunate prognosis. The simultaneity of these procedures with the appropriate oncological treatment must be taken into account, especially considering the repercussion and incompatibilities between them.

Endoscopic treatment should be individualized and may include esophageal stenting, tracheobronchial stenting, or dual stenting (stenting of both sides).