Congenital Tracheoesophageal Fistula in the Adult

CASE REPORT

Congenital Tracheoesophageal Fistula in the Adult Jerry L. Acosta, M.D., and J. Stanley Battersby, M.D. ABSTRACT Congenital tracheoesophageal fistula can persist and remain undetected until adulthood. We have recently encountered such a case and reviewed the others reported in the English literature. Commonly, symptoms are present during infancy, but an occasional patient develops symptoms only later in life. Diagnostic confirmation may be quite difficult, and the esophagogram appears to be the most reliable test. Chronic pulmonary suppuration is unusual, and most tracheoesophageal fistulas may be satisfactorily repaired by way of a cervical approach.

T

he majority of tracheoesophageal fistulas, especially those associated with esophageal atresia, are life threatening in infancy. Early diagnosis and surgical treatment give quite satisfactory results [Z]. A tracheoesophageal fistula without associated esophageal atresia is compatible with life and may persist for a prolonged period before the diagnosis is established. We have recently encountered a patient with a congenital H-type fistula that was undiagnosed until she was 20 years old. Many fistulas between the respiratory and alimentary tract in the adult have been recognized, but most are acquired [17, 181. A few fistulas believed to be of congenital origin have been reported, with a preponderance of them communicating with a lobar or segmental bronchus [5]. The persistence of a congenital tracheoesophageal fistula into adult life is indeed uncommon; only 10 previous cases have been reported in the English literature. We wish to present our patient and to discuss the others in relationship to symptoms, diagnosis, and treatment. N. W. was a 20-year-old woman whose problems had commenced in infancy. In 1951, at 8 weeks of age, she was admitted to our Children’s From the Division of Thoracic Surgery, Indiana University School of Medicine, Indianapolis, Ind. Accepted for publication July 23, 1973. Address reprint requests to Dr. Acosta, Department of Surgery, Emerson Hall, Indiana University School of Medicine, 1100 W. Michigan St., Indianapolis, Ind. 46202. VOL. 17, NO.

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Hospital because of wheezing, choking, and spitting with feedings. Pneumococcal pneumonia was diagnosed by chest roentgenogram and sputum cultures. An esophagogram was interpreted as “filling of the trachea by overloading of the esophagus.” A tracheoesophageal fistula was not demonstrated. Between 1951 and 1957 she apparently had recurrent respiratory tract infections. In 1957 she became a ward of the court and was placed in a foster home. Over the next 3 years she was repeatedly moved from one foster home to another. Each move seemed to be precipitated by a continuous nocturnal cough which disrupted the household. During this period a tonsillectomy was followed by postoperative pneumonia. In January of 1961, at 9% years of age, she was again admitted to our Children’s Hospital for evaluation. Chest roentgenogram, skin testing for allergies, paranasal sinus roentgenograms, and bronchograms were all interpreted as normal. There was no evidence for cystic fibrosis. Persistent symptoms with chronic cough and mucoid sputum production prompted readmission eight months later. Esophagograms were done on two occasions and failed to reveal a communication between the esophagus and respiratory tract or gastroesophageal reflux. Bronchograms were again normal. Continuous, chronic respiratory symptoms prompted reevaluation again in 1966, when the patient was 14. Immunoglobulins were normal. Bronchograms failed to reveal bronchiectasis, and bronchoscopy disclosed inflammation of the right middle and lower lobe orifices. The patient did not return until 1971, when she was admitted at term gestation and had a normal, spontaneous delivery. The postpartum period was complicated by pneumonia. During a review of her respiratory difficulties, she related that eating had precipitated symptoms as long as she could remember. Solid food was better tolerated than liquids, which she ingested slowly and in small amounts to prevent coughing. A chest roentgenogram failed to reveal significant chronic parenchymal changes, but an air esophagogram was consistently present (Fig. 1). Bronchograms revealed only diffuse, mild tubular bronchiectasis and an anomalous bronchus to the right upper lobe. Bronchoscopy and esophagoscopy were performed under topical anesthesia with failure to visualize a fistula. Methylene blue contrast medium injected into the upper esophagus did not stain the trachea. An esophagogram demonstrated a fistulous communication between the esophagus and trachea at the thoracic inlet (Fig. 2). Because of these findings the patient was taken to the operating room on April 12, 1971. A cervical incision parallel with the right sternocleidomastoid muscle was used. Dissection easily exposed a tracheoesophageal fistula at the thoracic inlet. The fistula was oblique in its course, with the tracheal orifice lying about 1 cm. cephalad to the esophageal orifice. Division and closure

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Congenital T E Fistula in the Adult

FIG. I . Posteroanterim and lateral chest roentgenograms demonstrating air esophagogram.

were performed using interrupted 4-0 silk inverting sutures. There was no evidence of inflammation or neoplasm about the fistula. The postoperative period was without complications, and the patient was discharged on the seventh postoperative day. At 1 year postoperatively she was completely asymptomatic.

Comment The first report of a congenital tracheoesophageal fistula in an adult was in 1929 [15]. The fistula was described at postmortem examination, and the patient had died of unrelated causes. The next case report appeared in 1933 [141. A roentgenographic contrast dye was swallowed to study the esophagus but produced a bronchogram effect on the chest roentgenogram; the tracheal orifice was localized by bronchoscopy. It is interesting that no treatment was rendered, and it was not until 1939 that the first successful closure of an Htype tracheoesophageal fistula was performed [ 101. A latent period of 20 years passed before the next patient was reported [ll]. Death occurred in this patient secondary to aspiration of a bolus of meat, presumably retrograde through a large, patulous fistula. Several cases of a congenital tracheoesophageal fistula in an adult have subsequently been reported and are included in the Table [4, 6-8, 12, 13, 191. The criterion for inclusion in the Table was the individual author's opinion that the defect was congenital in origin based on the characteristics of the fistula, such as its epithelial lining and the presence of submucosal muscle fibers, or the lack of findings

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FIG. 2. Esophagogram demonstrating contrast medium in tracheobronchial tree.

which would likely result from an acquired fistula, such as inflammation, neoplasm, or foreign body. The patients at the time of diagnosis ranged from 15 to 63 years of age, and the majority were female. Symptoms had commenced in infancy in most cases. A chronic cough with recurrent respiratory tract infections was almost always present. The characteristic pattern consisted of coughing with meals; solid food was better tolerated than liquids. Nocturnal accentuation of the cough also occurred. At least 2 patients manifested an adult onset of symptoms [6, 141. Delayed manifestation of a congenital defect is difficult to understand. Protection from aspiration by a redundant mucosal flap or membrane has been suggested [3, 10, 151. We have found that the tracheoesophageal fistula in neonatal patients has consistently demonstrated an oblique course, with the tracheal orifice cephalad to the esophageal orifice. The configuration in the adult group has been the same [4, 8, 151. This orientation of the fistula might function as a valve during swallowing, compressing the anterior esophageal wall against the fistula and occluding its lumen with either the passage of a bolus of food or the peristaltic wave. This mechanism might account for the greater tolerance to solid food than liquids demonstrated in these patients. One might also speculate that there is an increase in the size of the fistulous communication with growth, creating incompetence of this valvelike mechanism and accentuation of the symptoms as the patient matures.

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CASE REPORT:

Congenital TE Fistula in the Adult

SUMMARY OF REPORTED CASES OF CONGENITAL T E FISTULA IN T H E ADULT (AUTHORS INCLUDED)

Author &

Year

Age & Sex

Onset of Symptoms Esophagogram

Negus [15], 1929 McKinney [14], 1933 Krausey [ll], 1953 Caldwell [6 1954 Mat ey & Lemoine [13], 1954 Demong et al. [7], 1959

45, M

None

None

58, F

Recent

Positive

38, F

Unknown

None

54, M

25 yr.

Positive

16, F

Infancy

Positive

15, F

Infancy

None

Ferguson & Schoemperlen [a], 1959 Lansden & Falor [12], 1960 Zack & Owens [19]. 1967 Bertelsen [4], 1970

23, F

Infancy

None

63, F

Infancy

Negative

32, M

Probably Positive childhood

48, M

Infancy

Positive

20, F

Infancy

Positive

k

Acosta & Battersby [present report]

Treatment Diagnosed postmortem None Diagnosed postmortem Death following esophagogram Thoracotomy; division & closure Thoracotomy ; pneumonectomy, division, 8c closure Thoracotomy; division & closure Cervical ligation Thoracotomy; division & c1osu r e Thoracotomy; division & closure Cervical; division & closure

Diagnostic confirmation of a fistula may be exceedingly difficult, as our patient's history has illustrated. A chest roentgenogram may show infiltrates or fibrosis, or it may be entirely normal if no acute pneumonitis is present. This study may, however, demonstrate an air esophagogram, most easily seen in the lateral projection (see Figs. 1, 2). This finding in an adult is indicative of an esophagorespiratory fistula. Endoscopy is used to visualize the orifices and is essential to exclude a neoplastic or inflammatory process. Bronchoscopy has been more helpful than esophagoscopy. Other maneuvers include injecting contrast medium into the upper esophagus with a catheter and bronchoscopic visualization of the tracheal orifice. Using general anesthesia and positive-pressure ventilation, one may detect air passing into the esophagus by esophagoscopy [l]. T h e apparent area of the fistula was noted as an outpocketing in the anterior wall of the esophagus in our

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ACOSTA AND BATTERSBY patient, but methylene blue contrast medium introduced into the upper esophagus could not be detected in the tracheal lumen. T h e most definitive and reliable confirmatory test is an esophagogram, which will usually demonstrate evidence of the fistulous communication in the adult. Regurgitation and aspiration must of course be excluded. Maneuvers which may be helpful during the procedure include placing the patient in a prone position or distal occlusion of the esophagus with a balloon catheter. Multiple and repetitive diagnostic procedures may be necessary to confirm the diagnosis, and persistence is of utmost importance if a high index of suspicion is present. T h e history of our patient demonstrates how elusive the diagnosis can be. Bronchograms should be used to evaluate the tracheobronchial tree. Although not all patients were evaluated with bronchograms, it is of interest that only one pulmonary resection was performed [7]. T h e development of saccular bronchiectasis or abscess formation is more frequent with congenital bronchoesophageal communications in the adult, and many of these patients require a pulmonary resection supplemental to repair of the fistula [9, 161. T h e treatment of tracheoesophageal fistula is clearly surgical. A cervical approach is recommended over thoracotomy if there is no destroyed lung tissue. T h e fistula could have been exposed by a cervical incision in several of the cases reviewed; an adequate, detailed description of the location was not outlined in all reports. That a cervical approach is adequate is supported by the findings that most of the congenital H-type fistulas are in the middle or upper one-half of the trachea [5]. A cervical approach would therefore be applicable in most patients, since we believe it is technically easier to dissect in the thoracic inlet from above than from below. Division and closure of these epithelium-lined tracts is recommended, and cure can be expected in almost all patients.

References

1. Battersby, J. S. Congenital anomalies of the esophagus. A.M.A. Arch. Surg. 71:560, 1955. 2. Battersby, J. S.. Jolly, W. W., and Fess, S. W. Esophageal atresia: A comprehensive study of 210 patients. Bull. SOC.Znt. Chir. 5-6:415, 1971. 3. Berman, J. K., Test, P. S., and McArt, B. A. Congenital esophagobronchial fistula in an adult. J. Thorac. Surg. 24~493,1952. 4. Bertelsen, S. Congenital oesophago-tracheal fistulas in the adult. Scand. J . Thorac. Cardiovasc. Surg. 4:80, 1970. 5. Blackburn, W. R., and Amoury, R. A. Congenital esophagopulmonary fistulas without esophageal atresia: An analysis of 260 fistulas in infants, children and adults. Rev. Surg. 23:153, 1966. 6. Caldwell, K. P.S. Congenital tracheoesophageal fistula in the adult. J. Znt. Coll. Surg. 22587, 1954. 7. Demong, C. V., Grown, 1. B., and Heitzman, G. C. Congenital tracheoesophageal fistula without atresia of the esophagus. Am. Surg. 25: 156, 1959. 8. Ferguson, C. C., and Schoemperlen, C. B. Congenital tracheoesophageal fistula in an adult. Ann. Surg. 149:582, 1959.

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Congenital T E Fistula in the Adult

9. Frater, R. W. M., and Dowdle, E. B. Congenital esophagobronchial fistula. Arch. Surg. 89:949, 1964. 10. Imperatori, C. J. Congenital tracheoesophageal fistula without atresia of the esophagus. Arch. Otolaryngol. 30: 352, 1939. 11. Krausey, B. M. B. Congenital tracheo-oesophageal fistula without oesophageal atresia-with resultant asphyxia1 death. Br. Med. J . 1: 1433, 1953. 12. Lansden, F. T., and Falor, W. H. Congenital esophago-respiratory fistula in the adult. J . Thorac. Cardiozmsc. Surg. 39:246, 1960. 13. Mathey, J., and Lemoine, A. Tracheal diverticulum and congenital oesophagotracheal fistula without oesophageal atresia. Thorax 9: 106, 1954. 14. McKinney, R. Tracheo-esoDhagea1fistula, probablv connenital, in a woman 58 years of age. Ann. Otol. Rhinol. Layngol. 42~1227,1933. 15. Negus, V. E. Society's proceedings. J . Laryngol. Otol. 44:184, 1929. 16. Ware, G. W., and Hall, A. Congenital tracheoesophageal fistula in the adult. J . Thorac. Surg. 36:58, 1958. 17. Wesselhoeft, C. W., Jr., and Keshishian, J. M. Acquired nonmalignant esophagotracheal and esophagobronchial fistulas. Ann. Thorac. Surg. 6: 187, 1968. 18. Wychulis, A. R., Ellis, F. H. Jr., and Andersen, H. A. Acquired nonmalignant esophagotracheobronchial fistula. J.A.M.A. 196:117, 1966. 19. Zack, B. J., and Owens, M. P. Congenital tracheoesophageal fistula in the adult. Arch. Surg. 95:674, 1967.

NOTICE FROM T H E SOUTHERN THORACIC SURGICAL ASSOCIATION The Twenty-first Annual Meeting of the Southern Thoracic Surgical Association will be held at the Williamsburg Inn and Lodge, Williamsburg, Va., November 7-9, 1974. Reservations may be made by writing to the Reservations Manager, T h e Williamsburg Inn and Lodge, Williamsburg, Va. 23 185. Members wishing to participate in the scientific program should submit abstracts-typed double-spaced and in triplicate-to William H. Lee, Jr., M.D. (Chairman of the Program Committee), Medical College Hospital, 55 Doughty St., Charleston, S.C. 29401. The deadline for submission of abstracts is June 1, 1974. The Council of the Southern Thoracic Surgical Association has ruled that all slides used during presentation of papers and discussions must be of 35-mm. size. If additional information is required, inquiries should be directed to Dr. Lee. Application for membership in the Southern Thoracic Surgical Association, on forms provided by the Association, should be sent directly to George R. Daicoff, M.D. (Chairman of the Membership Committee), University of Florida College of Medicine, Gainesville, Fla. 32601. The deadline for application to membership is September 1, 1974. Papers that are accepted for the program will be considered for publication in T h e Annals and must be submitted to the Editor by October 15, 1974. W. BROOKS, M.D. Secretary-Treasurer

JAMES