Clinical Presentation and Diagnosis

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Benign Fistula. If a fistula develops in a patient on a respirator, a sudden increase in secretions is often noted as saliva enters the airway. It becomes difficult to maintain a seal with the cuff. Pulmonary infiltrates and pneumonia follow. Respiratory insufficiency may worsen. Cough follows swallowing. With ventilation, air may be heard escaping into the pharynx and the abdomen may become distended. Gastric feedings may appear on tracheal suctioning. Gastric reflux into the lungs can be disastrous and eventually fatal. If the patient is receiving oral feedings, these will appear in the tracheal suctioning.

Chest x-ray commonly shows the esophagus to be dilated distal to the fistula and the stomach may be dilated (Figure 12-3). A swallow of water stained with methylene blue will appear in the tracheostomy. This test is to be interpreted with caution since aspiration of dye into the larynx produces the same result. Fluoroscopy by an experienced radiologist, with ingestion of a small amount of barium, usually delineates the level and approximate size of the fistula (Figures 12-4, 12-5).

The fistula may be visible directly through a tracheostomy if it is present. Bronchoscopy should be done promptly if a fistula is suspected. In a patient who is on a respirator, a flexible bronchoscopy may be performed through an endotracheal tube, which is withdrawn just sufficiently to allow visualization of the

figure 12-2 Postmortem specimen of the trachea of a patient on long-term ventilation through a tracheostomy tube with a high-pressure cuff. A large membranous wall fistula has formed (arrow). The tracheal stoma is superior. Note the circumferential cuff damage.
figure 12-3 Roentgenogram of a patient with postintubation tracheoesophageal fistula. A, Note the distended esophagus, typical of this condition. Fistula is visible as a radiolucent circle. B, After repair, a normal tracheal air column is seen. Arrows mark the glottic level in both roentgenograms.

fistula. The same may be done through a tracheostomy tube while continuing ventilation in both cases. Passage of a rigid ventilating bronchoscope via the larynx allows the best assessment of the entire airway and locates the fistula relative to the cricoid and carina. The lengths of the fistula and of the normal airway are measured. A postintubation fistula is usually clearly identifiable (Figure 12-6). If not, methylene blue in saline may be instilled into the upper esophagus with the caution already noted to avoid overfilling and aspiration. Esophagoscopy is less likely to offer a good view, especially of smaller fistulae. A postintubation fistula usually lies a centimeter or two below the level of a tracheal stoma, since the fistula is located at the cuff site (see Figure 12-1).

Chronic fistulae from other causes present with cough on fluid or food ingestion, pulmonary infection, and occasional hemoptysis. Contrast images and bronchoscopy are diagnostic but most important is the clinician's suspicion of a fistula. This is even more critical after severe chest trauma since a tracheo-esophageal rupture may go unrecognized, or a fistula may be delayed in its formation, until life threatening mediastinal sepsis is established.

Malignant Fistula. In an excellent review of 207 malignant esophagorespiratory fistulae, Burt and colleagues confirmed esophageal carcinoma as the primary neoplasm (78%).n The tumors were located principally in the upper thoracic esophagus and were principally squamous in histology. The incidence of fis-tulization in this series of esophageal carcinomas was 4.5%. Lung cancer accounted for 16% of the malignant fistulae, and in only 3 patients were tracheoesophageal fistulae related to primary tracheal neoplasms. Other neoplasms that were associated with a tracheoesophageal fistula were Hodgkin's disease, metastatic breast cancer, and laryngeal carcinoma.12,13

All patients with carcinoma of the upper- or midesophagus should undergo bronchoscopy in their initial work-up. If an abnormality is identified between the trachea and esophagus radiographically, on

figure 12-4 Postintubation laryngotracheal stenosis with tracheoesophageal fistula (TEF) in a 56-year-old man. He had suffered cricoidostomy and failed TEF repair prior to referral. A, Tomograms of stenosis. Anteroposterior view on the left shows a deformed larynx with maximum stenosis at the laryngotracheal junction. The lateral view on the right demonstrates a narrowed subglottic larynx, with the most severe stenosis just below this (arrow) and above the stomal tract. B, Barium swallow in the same patient. The preoperative view on the left shows the fistula (arrow). On the right is a postoperative view of the repaired esophagus. Laryngotracheal resection of the stenosis and reconstruction were performed at the same time as closure of the fistula. There is no aspiration. Strap muscle was interposed between the two suture lines.

computed tomography (CT) scan or, most definitively, by ultrasonography, an abnormality is likely to be seen bronchoscopically; that is, compression, induration, granularity, or infiltration. If tumor is identified or suspected, the possibility of a fistula may be anticipated. Cough, hemoptysis, fever, and aspiration all signal malignant fistulization. Bronchoscopy with the use of methylene blue and contrast esophagography will show the communication. Esophagoscopy is often less definitive because of the bulk of tumor adjacent to the fistula. The fistula may be tracheal (53%) or bronchial (38%), and a few are pulmonary (6%).11 Frequently, the fistula follows prior radio- or chemotherapy, as might be expected, since treatment destroys tumor which had previously destroyed normal tissue. Although the disease may well be localized rather than disseminated at the time of manifestation of a fistula, progression of aspiration, pneumonia, lung abscess, and asphyxiation can be rapid. The clinical course typically is measured in weeks and months.

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