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Ann Thorac Surg 1995;60:151-155
© 1995 The Society of Thoracic Surgeons

Congenital Bronchoesophageal Fistulas in Adult Patients

Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital, Seoul, South Korea

Accepted for publication March 16, 1995.

	Abstract

Top Footnotes Abstract Introduction Material and Methods Results Comment Acknowledgments References

 
Background. Congenital bronchoesophageal fistulas have rarely been reported. Presented here is the 14-year experience of our hospital with this lesion.

Methods. The study comprises 13 patients; 9 had a Braimbridge type II fistula; 2, a type I fistula; and 2, a type IV fistula. The most frequent site of communication was between the middle esophagus and the right lower lobe of the lung, especially the superior segment. A fistulectomy, with or without pulmonary resection, was performed on each patient.

Results. All patients had complete relief of symptoms. No operative complications were observed.

Conclusions. Congenital bronchoesophageal fistulas in adults are usually diagnosed by an esophagography. Symptoms are often nonspecific, and the possibility of a congenital bronchoesophageal fistula should be considered in patients who complain of long-standing unexplainable respiratory symptoms such as coughing and frequent pulmonary infections. The surgical intervention is relatively simple. In many cases, a fistulectomy with simple closure of the openings in both the esophagus and the bronchus is all that is required. Pulmonary resection is needed in some patients with severe bronchiectasis and recurrent pneumonitis.

	Introduction

Top Footnotes Abstract Introduction Material and Methods Results Comment Acknowledgments References

 
Congenital fistulas constitute a small number of the abnormal communications between the esophagus and the respiratory tract in adults. Although there is little controversy over treatment, the pathogenesis and the delayed onset of symptoms in these patients can be explained only speculatively. Here we present our experience with 13 patients with a congenital bronchoesophageal fistula (BEF) and provide a review of the literature.

	Material and Methods

Top Footnotes Abstract Introduction Material and Methods Results Comment Acknowledgments References

 
A total of 13 patients were operated on for congenital BEFs from 1979 to 1992 in the Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital. Their hospital records were thoroughly reviewed, and data including age, sex, symptoms and physical signs, and methods of diagnosis were obtained. Operative findings and operative treatment were reviewed. The histopathologic specimens from each patient were also reviewed, and the diagnoses were confirmed.

	Results

Top Footnotes Abstract Introduction Material and Methods Results Comment Acknowledgments References

 
Of the 13 patients, 9 were male and 4, female. Age ranged from 16 to 62 years with a mean age of 36.2 years (Table 1).

The diagnosis of BEF was possible preoperatively in 11 patients, and in all of them, it was obtained by barium esophagography (Table 2). In the remaining 2 patients (patients 3 and 5), BEFs were found incidentally during operation for bronchiectasis. Esophagograms were not made for these 2 patients. Fiberoptic esophagoscopy was done in 8 patients, and in 5 of them, fistula openings were visible. Bronchoscopy was performed in 12 patients, but the fistula openings were visible in only 4, in 3 with the aid of methylene blue ingested into the esophagus. In bronchograms of 6 patients, the fistulas were visible in 4 patients. In patient 3, the fistula was missed during the preoperative reading but was found to be visible during a postoperative review of the film.

View larger version (165K): [in this window] [in a new window]   Fig 1. . (Patient 13.) Operative finding in patient who had a fistula from a diverticulum of the esophagus (E) (Braimbridge type I). The arrow indicates the fistula. (L = lung.)

For treatment, 5 patients underwent a fistulectomy only (see Table 3). We experienced no difficulty dissecting around the fistulous tracts, even in cases of marked pulmonary disease and dense intrapleural adhesion. No adherent lymph nodes were found around the fistula. After excision of the fistula, the esophageal opening was closed in two layers, and the bronchial opening was covered with pleura or pericardial fat after closure with absorbable sutures. The remaining 8 patients underwent pulmonary resection as well as fistulectomy because of severe parenchymal destruction by bronchiectatic changes or marked volume decrease.

Histologic examination of the excised fistulas revealed squamous epithelial linings and submucosa with muscle layer in all patients. In patient 13, transition of the epithelial lining from squamous to columnar was found. The specimens from the 2 patients with a history of antituberculosis medication revealed no evidence of past or currently active tuberculosis.

None of the patients in this series experienced major postoperative complications, and all were followed up for more than 1 year (range, 1 to 8 years). All patients were free from symptoms after operation.

	Comment

Top Footnotes Abstract Introduction Material and Methods Results Comment Acknowledgments References

 
Congenital BEF without accompanying esophageal atresia is a rare anomaly that is usually diagnosed in adults. Such a fistula is 25% to 50% less common than a tracheoesophageal fistula [1, 2]. The literature review by Risher and associates [3] is the most recent and the largest series and includes 100 cases in both the pediatric and adult age groups. Kurashige and co-workers [4] collected data on 91 cases in adults that had been reported in Japan to 1981. Both reviews revealed no sex preponderance in incidence and a relatively even distribution of cases in all age groups, with the highest incidence occurring in the third decade of life. In the review of Risher and colleagues, 24% of the cases were in children.

Coughing on liquid ingestion, sometimes precipitated by a certain posture, is a typical symptom of this disease, and if present, the decision to perform barium esophagography and the subsequent diagnosis of fistula are not difficult. Halitosis, especially in patients who eat spicy foods, is very rarely reported [5]. However, many patients with congenital BEF have no typical symptoms, and some patients complain only of gastrointestinal or nonspecific respiratory symptoms such as coughing and frequent pulmonary infections. Some patients exhibit only belching or epigastric pain, which can be explained as a result of persistent air ingestion through the fistula. Therefore, we emphasize that the possibility of a congenital fistula should be considered and the esophagus investigated more frequently during diagnostic workups for patients with nonspecific, but long-standing, respiratory or gastrointestinal symptoms. Osinowo and co-workers [6] found some relationship between age at symptom onset and type of bronchial lesion. However, others [7] have objected to this suggestion, and we also could not confirm it.

Barium esophagography is considered the most sensitive and definite tool of diagnosis. Cineesophagography, if available, is preferable, and sequential positioning of the patient during the examination is important to find the fistula [8]. Esophagoscopy is less sensitive than esophagography. Because they are less sensitive, as shown in our series and those of others, bronchography and bronchoscopy are regarded as auxiliary means for evaluating associated pathologic conditions and excluding the possibility of an acquired fistula [9]. They are also important in the decision whether to perform pulmonary resection. Kurashige and associates [4] reported an episode of acute lidocaine hydrochloride intoxication during bronchoscopic examination that was thought to be caused by leakage of the drug into the esophagus through the fistula.

Surgical intervention for a congenital BEF is relatively simple. In many cases, a fistulectomy with simple closure of the openings in both the esophagus and the bronchus is all that is required. Because of concern about the possibility of leakage, some authors [10] prefer simple ligation or stapling to division or excision of the fistula. However, in the literature, we could find no report of a complication after excision of the fistula, whether the closure method was suturing or stapling. Pulmonary resection is needed in some patients with severe bronchiectasis and recurrent pneumonitis, but a prudent attitude must be taken [2, 11]. Becker and co-workers [9] reported a marked improvement in pulmonary function test results and rapid disappearance of radiologic changes after a simple fistulectomy in a patient who had had extensive roentgenographic changes and very poor pulmonary function preoperatively.

A common finding in our series and the series of Risher [3], Kurashige [4], and their co-workers was that the most frequent site of communication in the lung was the right lower lobe, particularly the superior segment. Shimada and coauthors [11] postulated that the thinner muscle layer and the lack of a soft tissue envelope around the esophagus below the carina result in the predilection for the fistula location to be the superior segment of the right lower lobe.

Braimbridge and Keith [12] classified congenital BEFs into four types as follows: type I is associated with a wide-necked congenital diverticulum of the esophagus; type II is a short tract running directly from the esophagus to the bronchus; type III is a connection to a cyst in the lobe; and type IV is a fistula to a sequestrated segment of the lung. Risher and co-workers [3] found type III and type IV BEFs to be very rare (only 5% and 3% of all cases, respectively), but the Japanese report by Kurashige and colleagues [4] showed a higher incidence of type III (32.8%). Our series includes two type I (15%) and two type IV (15%) fistulas but no type III fistulas.

The type IV lesion can be regarded as a form of congenital bronchopulmonary-foregut malformation, originally named by Gerle and co-workers [13]. The embryogenesis of this category of malformation is explained as an abnormality of ventral foregut budding [14, 15]. Smith [1] gave an embryologic explanation for the development of these fistulas; they are the result of persistent attachment between the tracheobronchial tree and the esophagus produced by rapid elongation of the trachea and its separation from the esophagus. He also raised the possibility of the role of a localized intrauterine infection resulting in adhesion between the embryonic bronchus and the esophagus. These hypothetical explanations differ from those for tracheoesophageal fistulas, which may arise from incomplete fusion of the lateral foregut plates that are responsible for the separation of the trachea from the esophagus proximal to the stem bronchi [16].

It may be difficult to differentiate a congenital BEF from an acquired one, especially if advanced pulmonary disease exists. In fact, only the type II BEF is not subject to debate about its congenital origin [7, 16]. The type I fistula may represent an acquired fistula secondary to infection and perforation of a congenital diverticulum of the esophagus. Long-standing pulmonary suppuration in the type III and IV fistulas may result in fistula formation to the esophagus. Generally, the criteria for the diagnosis of a congenital fistula are as follows: (1) the absence of past or present surrounding inflammation; (2) the absence of adherent lymph nodes; and (3) the presence of a mucosa and a definite muscularis mucosae [1, 3]. The mucosa may be lined by squamous or columnar epithelium. Transition of the epithelial lining of the fistula from squamous (esophageal) to columnar (respiratory) is another criterion according to some [2, 4].

There have been many explanations for the delay in onset of symptoms until adult life. These include (1) an occlusion of the opening by an esophageal tissue fold or a ``flap valve,'' (2) the presence of a membrane that subsequently ruptures, (3) the action of gravity (upward direction of the fistula from the esophagus to the bronchi) preventing spillage of the esophageal contents into the respiratory tree, (4) adaptation of patients to the minimal symptoms, and (5) spasm of the smooth muscle in the fistula wall [1, 6, 8, 17]. However, none of these is well supported by pathologic or radiologic findings. Smith [1] speculated that the action of gravity was the most likely explanation. However, this factor does not seem to have contributed to the symptom delay in most of our patients (the fistula opening in the esophagus was caudal to that in the bronchus in only 4 of 13 patients) and in most of the patients discussed by others [2, 8]. As pointed out by Bekoe and associates [8] the frequent need of pulmonary resection for marked bronchiectasis or interstitial pneumonitis (8 of 13 patients in our series) can be attributed to a long period of symptom tolerance by the patients.

	Acknowledgments

Top Footnotes Abstract Introduction Material and Methods Results Comment Acknowledgments References

 
This study is supported by grant 01-94-056 from the Seoul National University Hospital Research Fund.

	Footnotes

Top Footnotes Abstract Introduction Material and Methods Results Comment Acknowledgments References

 
Address reprint requests to Dr Kim, Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital, 28 Yongon-Dong, Chongno-Gu, Seoul, 110-744, South Korea.

	References

Top Footnotes Abstract Introduction Material and Methods Results Comment Acknowledgments References

 

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12. Braimbridge MV, Keith HI. Oesophago-bronchial fistula in the adult. Thorax 1965;20:226–33.
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This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Joo Hyun Kim Kay-Hyun Park Joon Ryang Rho Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Kim, J. H. Articles by Rho, J. R. Search for Related Content PubMed PubMed Citation Articles by Kim, J. H. Articles by Rho, J. R.