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Eur J Cardiothorac Surg 1999;16:667-669
© 1999 Elsevier Science NL

Case report

Congenital bronchoesophageal fistula in the adult

2nd Thoracic Surgery Department ‘SOTIRIA’ Chest Diseases Hospital, Athens, Greece

Corresponding author. 24 Ellinikou Stratou str., GR-152 37 Philothei, Athens, Greece. Tel.: +30-1-779-3098; fax: +30-441-42820

	Abstract

Top Abstract 1. Introduction 2. Case report 3. Discussion References

 
Congenital bronchoesophageal fistulas, when not associated with esophageal atresia, are compatible with life and may persist until adulthood before diagnosis has been established. We report such a rare case of a 55-year-old Caucasian female with a history of repeated pulmonary infections, suffering from cough during the last 12 months due to a mass in the right lung. A bronchoesophageal fistula (type III according to Braimbridge and Keith classification) was incidentally discovered during thoracotomy which was resected and end-sutured. Following that, a right lower lobectomy was performed. The patient had an uneventful recovery. The final diagnosis of congenital bronchoesophageal fistula was established excluding all the reasons that lead to the acquired disease. The diagnostic and therapeutic procedures are analyzed and the relevant literature is reviewed.

Key Words: Bronchoesophageal fistula • Congenital • Surgical treatment

	1. Introduction

Top Abstract 1. Introduction 2. Case report 3. Discussion References

 
Congenital bronchoesophageal fistulas, if not associated with esophageal atresia or other respiratory abnormalities, are rare and compatible with life. They may persist clinically in apparent until adulthood.

The authors were not able to cite more than 150 cases in the international literature, although it has been estimated that the real number is higher of which most are case reports.

The pathogenesis is not absolutely clear. A persistent attachment between the tracheobroncheal tree and the esophagus, produced due to abnormal growth of the trachea during its severance from the esophagus, has been accused for it. On the other hand the acquired bronchoesophageal fistulas may be due to malignancy, trauma, infections diseases such as tuberculosis and esophageal diverticula. The chief symptoms are bouts of coughing after drinking and recurrent respiratory infections.

As soon as the diagnosis of ‘congenital bronchoesophageal fistula’ is established, any further treatment is surgical, as far as the general condition of the patient allows to.

	2. Case report

Top Abstract 1. Introduction 2. Case report 3. Discussion References

 
A 55-year-old Caucasian female visited her family doctor because of severe coughing for 12 months; for the previous 4 weeks she also had yellow-like expectorations. She reported that was ‘sensitive to catching a cold’. She had never been treated in a hospital, nor had she ever had serious health problems. The bouts of coughing in question were not associated with drinking.

Further evaluation included hematological tests, chest X-ray that showed a diffuse shadowing of the right lower lobe, while a chest CT scan revealed a mass measuring 2.5 cm in diameter, with irregular borders encircling the basal segmental bronchi of the right lower lobe (Fig. 1). Bronchoscopy revealed a slight inflammation of the bronchial mucosa, especially of the right basal segmental bronchi.

View larger version (60K): [in this window] [in a new window]   Fig. 1. Preoperative chest CT scan.

The histological examination of the resected lobe did not reveal any evidence of malignancy; the fistulous mucosa was lined by squamous epithelium. The 6-month follow-up was uneventful and the patient was in a very good general condition and has resumed all her regular activities.

	3. Discussion

Top Abstract 1. Introduction 2. Case report 3. Discussion References

 
Congenital bronchoesophageal and tracheoesophageal fistulas were first described by Gibson in 1696 and Negus in 1929. In 1966 Braimbridge and Keith [1] suggested the following classification: (i) type I, esophageal diverticulum with a large osteum and a fistula at its tip; (ii) type II, a short tract running directly from the esophagus to the bronchus or the trachea; (iii) type III, a fistulous tract connecting the esophagus to a cyst in the lobe, which in turn communicates with the bronchus; (iv) type IV, a fistula that runs into a sequestrated segment or lobe.

Most of the bronchoesophageal fistulas reported in the international literature are isolated case reports. The largest literature review by Risher et al. [2] includes 100 cases, however in both pediatric and adult age groups.

Sex distribution did not reveal any predominance and the highest incidence occurred in the 3rd decade of life.

The chief presenting symptoms are bouts of coughing after drinking (Ono's sign) [3–5] and recurrent respiratory infections. Hemoptysis and bouts of retrosternal pain are also reported, while some patients complain of vague non-specific gastrointestinal symptoms [5]. The duration of symptoms ranges from 6 months to 30 years [2,5]. The long silent interval until adulthood and the irregular character of the signs have received various explanations, including an occluding proximal fold of esophageal mucosa (flap valve), or a fistulous tract running upward from the esophagus into the bronchus that may close during swallowing [1,2], or spasm of the muscular layer occluding the lumen [3,6]. Another theory probably closer to reality is that there is not a late onset of symptoms, but that initially mild complaints have not been thoroughly investigated until complications appeared.

An exception to all reasons which lead to the acquired disease such as malignancy, trauma, foreign body, esophageal diverticulum, infections diseases (TBC) and the presence of adherent lymph nodes, lead to the diagnosis of the congenital nature of the fistula [1,3,4].

Smith [7] gives an embryologic explanation for the development of these fistulas. He states that they are the result of persistent attachment between the tracheobronchial tree and the esophagus produces by rapid elongation of the trachea and its separation from the esophagus.

The most useful diagnostic investigation is esophagography with dilute barium. Esophagoscopy and bronchoscopy are less sensitive [2–4]. Many authors install methylene blue into the esophagus during bronchoscopy, or infuse saline into the esophagus during esophagoscopy, coupled with positive pressure ventilation to promote bubble formation at the site of the fistula [3,6].

Chest CT scan may be utilized to rule out the presence of a neoplasm and to define the extent of pulmonary disease, which may require resection. Even though 10% of bronchoesophageal fistulas remain undiagnosed or undiscovered intraoperatively [3].

The most frequent anatomical type is a connection between the lower third of the esophagus and the right lower lobe (40%), while no fistulas regarding at the left upper lobe have been reported [1,2].

There is no controversy regarding about the necessity for surgical management of congenital respiratory-esophageal fistulas, as far as the general condition of the patient allows to. The two main methods of treatment are division and suturing of the ends of the fistula and complete resection. Because of the concern about the possibility of leakage, some authors 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. The insertion of a muscular or pleural flap has been postulated by most authors, to prevent any refistulization [8]. Less efficient forms of treatment have included occlusion of the esophageal opening with biologic glue or a Celestin tube [9], or by using sodium hydroxid and acetic acid solution via the bronchoscope and the esophagoscope [10]. We believe these techniques should be considered only when the patient's condition does not allow a thoracotomy. Pulmonary resection is required in cases with severe bronchiectasis and recurrent pneumonitis, nevertheless a prudent attitude must be taken [3,5]. The prognosis after surgical repair is excellent. Complications or recurrences are seldom reported [2–5].

Congenital bronchoesophageal fistula is compatible with life if not associated with esophageal atresia or other abnormalities and they may persist until adulthood before they become clinically apparent. The main symptom is bouts of coughing after drinking. The most useful diagnostic investigation is the esophagography with barium. Even though they are benign diseases, life-threatening complications might occur and they must thus be treated surgically as soon as the diagnosis or suspicion is established.

	References

Top Abstract 1. Introduction 2. Case report 3. Discussion References

 

1. Braimbridge M.V., Keith H.I. Oesophago-bronchial fistula in the adult. Thorax 1965;20:226-233.
2. Risher W.H., Arensman R.M., Ochsner J.L. Congenital bronchoesophageal fistula. Ann Thorac Surg 1990;49:500-505.[Abstract]
3. Azoulay D., Regnard J.F., Magdeleinat P., Diamond T., Rojas-Miranda A., Levasseur P. Congenital respiratory-esophageal fistula in the adult. J Thorac Cardiovasc Surg 1992;104:381-384.[Abstract]
4. Kim J.H., Park K.-H., Sung S.W., Rho J.R. Congenital bronchoesophageal fistulas in adult patients. Ann Thorac Surg 1995;60:151-155.[Abstract/Free Full Text]
5. Subrato D., Mir B.A., Fonseca P. Congenital bronchoesophageal fistula in an adult. Chest 1998;114:1784-1786.[Abstract/Free Full Text]
6. Acosta J.L., Battesby J.S. Congenital tracheoesophageal fistula in the adult. Ann Thorac Surg 1974;17:51-56.[Medline]
7. Smith D.C. A congenital broncho-esophageal fistula presenting in adult life without pulmonary infection. Br J Surg 1970;57:398-400.[Medline]
8. Gerzic Z., Rakic S., Randjelovic T. Acquired benign esophagorespiratory fistula: report of 16 consecutive cases. Ann Thorac Surg 1990;50:724-727.[Abstract]
9. Laforet E.G. Treatment of oesophagobronchial fistula (Letter). J Am Med Assoc 1978;239:2388.
10. Parry G.W., Juma A., Dussek J.E. Broncho-esophageal fistula treated effectively without surgical resection. Thorax 1993;48:189-190.[Medline]

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This Article Abstract Full Text (PDF) 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): Christophoros Kotoulas Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Lazopoulos, G. Articles by Lioulias, A. Search for Related Content PubMed PubMed Citation Articles by Lazopoulos, G. Articles by Lioulias, A.