Congenital Bronchoesophageal Fistula

Braimbridge and Keith classified BEF into four types (Figure 12-9).23 Type I seems to result, at least sometimes, from inflammatory changes in an esophageal diverticulum, which then secondarily fistulizes to a bronchus. Although some diverticula are undoubtedly congenital, traction diverticula are also likely represented in this group of lesions and the fistula itself may not therefore always be of congenital origin. Nonetheless, I retain their classification of "congenital" BEF's. Type I presents with a small fistula, at times inflamed, at the tip of an esophageal diverticulum. Type II is a simple fistula extending from the esophagus at an upward angle to a bronchus. This is by far the most common type. Next in incidence is type III, where the fistula connects to a cyst in the lung and thence to bronchi. Type IV is a fistula to a sequestrated lung, which is supplied by a systemic artery. The tract runs most commonly from the middle third of the esophagus, and less often from the lower third to the right lower lobe (either segmental or lobar bronchus), while half as often to the bronchi of the left lower lobe and in diminishing frequency to the bronchus inter-medius, left main bronchus, right middle lobe, and right upper lobe bronchi. In a review of 100 cases reported up to 1990, Risher and colleagues found only 5 cases of type III and 3 of type IV.24 Congenital fis-tulae are characterized by lack of inflammation, absence of adherent or inflamed lymph nodes, and a tract lined with squamous or columnar mucosa with adjacent muscularis mucosa.

Clinical Presentation and Diagnosis. Distribution of patients is about equal for males and females, with 75% of patients over 17 years of age. Paroxysmal cough, cough on ingestion of food especially after liquids, frequent respiratory infections, hemoptysis, and hematemesis are found.25-27 Symptoms may be largely of cough and respiratory infection for many years, leading to a delay in diagnosis from 5 to 30 years. Retrospectively, a long history is usually identified. Delay in appearance of severe symptoms has been attributed to the oblique course of the tract and to its possible initial obstruction either by a thin membrane, which later ruptures after inflammation, or to a mucosal flap valve in the tract. Marked delay in the discovery of a fistula can lead to death from recurrent pulmonary suppuration. Treatment is therefore urged as soon as diagnosis is made.

Diagnosis is made by contrast esophagography (Figure 12-10) and bronchoscopy, sometimes with methylene blue instillation into the esophagus. Esophagoscopy is less often definitive. Insufflation of gases into the trachea during esophagoscopy may aid in pinpointing a tiny fistula. Contrast should be introduced for esophagography with the patient in a position where cough ordinarily follows oral ingestion, rather than with the patient in a recumbent position. CT scan helps to assess pulmonary damage.

Bronchoesophageal Fistula

figure 12-9 Braimbridge and Keith's classification of congenital bronchoesophageal fistula: type I, wide neck diverticulum with inflammatory fistula at tip; type II, simple fistula, the most common type; type III, fistula with cyst; type IV, fistula with sequestration of lung.23

I II III IV

figure 12-9 Braimbridge and Keith's classification of congenital bronchoesophageal fistula: type I, wide neck diverticulum with inflammatory fistula at tip; type II, simple fistula, the most common type; type III, fistula with cyst; type IV, fistula with sequestration of lung.23

Treatment. Despite a few reports of alternative treatments, surgical excision of the tract and closure of the fistula at either end is the best choice (Figure 12-11). Right or left thoracotomy is selected according to the location of the fistula. Two layers of 4-0 Vicryl sutures are advised for closure of the esophageal end, or one layer of staples with a sutured second layer. One layer of Vicryl is used on the bronchial side. A substantial flap of healthy tissue, such as a pericardial fat pad or intercostal muscle, is pedicled between the two suture lines. Simple stapling without division of the tract is likely to result in recurrent fistula. Any irremediably

figure 12-11 Operative repair of type I congenital bronchoesophageal fistula via left thoracotomy. A Penrose drain encircles the dissected fistula and esophageal diverticulum. The esophagus is at the left, the lung at the right. The diverticulum was excised, both sides of the channel sutured, and intercostal muscle pedicled between.

injured portion of lung is resected at the same time. This has been necessary in many cases. Results of treatment are generally excellent.

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