Physical Examination, Complementary Tests, and Functional Status Assessment

Most adults with esophago-respiratory fistulas (ERFs) have the acquired form of disease comprising tracheo-esophageal, broncho-esophageal, or tracheo-broncho-esophageal fistulas. Their prognosis and management depend on whether the fistula is the result of a benign process or a malignancy, with the latter usually due to primary esophageal cancer. In one series of 264 patients with malignant ERF, 243 (92%) had esophageal cancer, 19 (7%) had lung cancer, and 2 (1%) had mediastinal tumor.¹ Results from studies show an overall frequency of ERF in patients with esophageal cancer of 5% to 10%, but fistulas may be more common toward the terminal stage of the disease.¹ Compared with patients without ERF, those with esophageal cancer and ERF present at a more advanced stage of disease, have more frequent involvement of the upper to mid-thoracic esophagus, and have a longer segment of tumor. Although the median time from diagnosis of esophageal cancer to the development of a fistula is approximately 8 months, our patient was diagnosed with ERF only 2 months after his initial diagnosis. ERF can be the presenting manifestation of cancer in approximately 6% of cases.¹ The median survival time after diagnosis of ERF is only 8 weeks.²

During initial evaluation of these patients, a thorough oncologic treatment history is important because the use of some antiangiogenesis drugs such as bevacizumab, along with radiation therapy, has been linked to the development of malignant ERF.³ Nonmalignant causes of ERF include complications of mechanical ventilation or indwelling tracheal or esophageal stents, complications from prior tracheal or esophageal surgery, granulomatous mediastinal infection (tuberculosis, syphilis, histoplasmosis), trauma (blunt or penetrating), and ingestion of caustic or foreign bodies. A large study from the Mayo Clinic found that esophageal surgery was the most common cause of nonmalignant ERF.⁴

Regardless of its origin, ERF is a life-threatening condition with severe pulmonary complications consisting of ongoing tracheobronchial bacterial contamination and impaired nutrition.⁴ Patients present with intractable cough, recurrent respiratory infections, rapid deterioration, and death, if left untreated.^2,5,6 In a study of 207 malignant trachea-esophageal fistulas (TEFs), symptoms and signs included cough in 116 (56%), aspiration in 77 (37%), fever in 52 (25%), dysphagia in 39 (19%), pneumonia in 11 (5%), hemoptysis in 10 (5%), and chest pain in 10 (5%).⁷ Our patient’s aspiration probably was worsened by his left recurrent laryngeal nerve palsy, causing an immobile left vocal cord (see video on ExpertConsult.com) (Video VI.27.2). Seen in approximately 10% of patients with ERF, this finding contributes to swallowing difficulties and periodic or constant aspiration.¹

Comorbidities

This patient had pneumonia, was hospitalized, and had a poor performance status (Eastern Cooperative Oncology Group [ECOG]/Zubrod 4).* His pneumonia resulted in sepsis, but no evidence was found of other organ dysfunction that could have interfered with anesthesia and perioperative care. He was severely malnourished, as manifested by his anemia and hypoproteinemia. Wound healing, in particular healing of the anastomosis in case surgery is performed, would be expected to be poor. Muscle wasting reduces respiratory reserve and the patient’s ability to breathe, cough, and clear secretions.⁸

Support System

This gentleman had a supportive wife. Married patients have been shown to have a lower risk of death than unmarried patients, independent of socioeconomic status.⁹ In addition, relevant to this case, married patients suffering from esophageal carcinoma report higher baseline quality of life with regard to legal concerns (e.g., having a will, advance directives) and friend and family support compared with single patients. Over time, married patients may have a decrease in pain frequency compared with single patients.¹⁰

Patient Preferences and Expectations

Our patient showed understanding of the gravity of his diagnosis. Patients with ERF have a poor prognosis, and many patients die from respiratory infection and malnutrition within a month of diagnosis.¹¹ Control of pulmonary contamination provides an opportunity for secondary treatment, as well as for improved survival and quality of life. Therefore treatment should include closure of the fistula and re-establishment of oral intake in a timely and cost-efficient manner, while minimizing the need for secondary medical interventions. Our patient wished he could eat and breathe better during the time he had left to live, but he did not want to be a burden to his wife. A significant proportion of caregivers of patients with esophageal cancer experience high levels of strain and psychological distress. Support and services targeted specifically at reducing the considerable strain of caring for patients with esophageal cancer are necessary, particularly for caregivers of patients from lower socioeconomic groups.¹²

Indications

Treatment must correct the two problems of airway contamination and poor nutrition. Reflux of gastric contents is diminished by placement of a gastrostomy tube, and adequate nutrition is sometimes facilitated by insertion of a jejunostomy tube. Treatment depends on whether the patient is resectable and medically fit for surgical therapy. Like our patient, most patients have advanced disease and can be treated only with palliative measures, which involve restoration of the swallowing mechanism and prevention of aspiration. The current standard of palliative therapy for patients with malignant ERF is endoscopic or radiologic placement of esophageal-covered self-expanding metallic stents (SEMS), allowing closure of the fistula. Less common treatment options for selected patients with malignant ERF include chemotherapy and radiation, surgical bypass, esophageal exclusion, and fistula resection and repair.¹³ Our patient’s functional status and concurrent aspiration pneumonia precluded surgical treatment, chemotherapy, or radiotherapy. We chose to proceed first with silicone airway stent insertion with the goal of palliating left main bronchial patency and covering the fistula, followed by esophageal stent insertion to help prevent aspiration and facilitate oral food intake.

Contraindications

No absolute contraindications to stent insertion or general anesthesia were noted. Given the patient’s age and extensive smoking history, a cardiology evaluation was requested before intervention. He was considered to be at intermediate risk (1% to 5%) for cardiac death or nonfatal myocardial infarction. Blood gas analysis showed a partial pressure of oxygen in arterial blood (PaO₂) of 55 mm Hg and an arterial carbon dioxide tension (PaCO₂) of 60 mm Hg on room air. The risk associated with this degree of PaCO₂ elevation is not prohibitive for a necessary intervention, although it should lead to reassessment of the indication for the proposed procedure and aggressive preoperative preparation. Although preoperative hypoxemia and postoperative pulmonary complications may be associated in patients undergoing surgery,¹⁴ hypoxemia generally has not been identified as a significant independent predictor of complications after adjustment for potential confounders.

Expected Results

Palliation for malignant ERF is usually achieved with endoscopic placement of esophageal, airway, or parallel (dual) stent insertion (in the esophagus and airway). Dual stent insertion appears to work better than a single prosthesis. Particular attention should be paid to airway compression or erosion caused by esophageal stents, prompting some authorities to initially place an airway stent before placing the esophageal one, especially if significant tracheobronchial obstruction is a matter of concern (Figure 27-2). Results from small case series show that respiratory distress can occur from severe airway obstruction caused by extrinsic compression after esophageal stent insertion.¹⁵ Silicone or covered metallic stents may be preferred for the airway to prevent recurrence of airway narrowing by growth of tumor between the wires of metal stents. For instance, one study evaluated the clinical benefits and complications of studded silicone stent insertion in 35 patients, of whom 6 had ERF. Three of 6 patients showed resolution of the fistula, and 3 other patients improved symptomatically.¹⁶ Symptoms of ERF usually improve after double stent insertion. ERF symptoms may recur as the result of stent-induced pressure necrosis of tracheal and esophageal walls. In addition to fistula enlargement, other complications described with stents placed in the esophagus, in the airway, or after dual stent insertion include pain, reflux, stent migration or fracture, restenosis, massive bleeding, aspiration, airway obstruction, tumor overgrowth, and food impaction.^1,17

Figure 27-2 A, Flexible bronchoscopy shows severe tracheal obstruction from esophageal carcinoma and associated tracheo-esophageal fistula (TEF). B, Rigid bronchoscopy immediately after the TEF is covered with a straight, studded silicone stent.

Team Experience

A multidisciplinary approach involving the gastroenterologist, the interventional pulmonologist, the oncologist, and the thoracic surgeon may be helpful. Patients with ERF are prone to develop worsening wall perforation and erosion because of the nature of malignant infiltration, stent-induced necrosis, or concurrent or previously applied radiotherapy and chemotherapy. Locations, the number of stents, and their type and size should be discussed.¹⁸ Some prefer to insert an esophageal stent, an airway stent, and a percutaneous endoscopic gastrostomy tube at the same time, during a single session with the patient under general anesthesia.¹⁹ Others perform procedures sequentially. Interventions performed by different services need to be coordinated, so good communication among treating teams is necessary.

Therapeutic Alternatives

Treatment is individualized and may include esophageal stenting, tracheobronchial stenting, simultaneous stenting of the trachea and esophagus, esophageal exclusion, esophageal bypass, fistula resection, and repair and radiation therapy.¹⁸ Direct surgical fistula closure or resection does not yield satisfactory results in this population. In general, surgical esophageal bypass can resolve respiratory contamination and allow fairly normal swallowing, but it should be reserved for patients who can tolerate a major operation. Esophageal stent insertion helps prevent aspiration and allows swallowing. This procedure can be offered to nearly all patients, regardless of their physiologic condition. The most effective reported treatments are esophageal bypass and esophageal stent insertion. When performed promptly after diagnosis, these treatments may improve survival and quality of life.¹³ Discussion of available treatment modalities is warranted among treating team members, and specific indications, contraindications, advantages, and disadvantages of each should be shared with patients and their families.