Congenital respiratory-esophageal fistula in the adult. Report of nine cases and review of the literature.

Congenital tracheoesophageal or bronchoesophageal fistulas, if not associated with esophageal atresia, may not appear initially until adult life. Nine such cases (two tracheoesophageal and seven bronchoesophageal) are reported. The chief presenting symptoms were recurrent bouts of coughing, after drinking, and hemoptysis. In the majority of cases the duration of symptoms exceeded 15 years. The diagnosis was confirmed in seven patients by esophagography, in one patient by bronchoscopy, and in one patient the fistula was discovered incidentally during thoracotomy. The esophageal opening of the fistula was in the lower third in seven patients and in the middle third in two. Bronchoesophageal fistulas communicated with a segmental bronchus in four patients and with a main or lobar bronchus in three. Treatment involved excision of the fistula (five patients) or division and suturing (four patients). Postoperative follow-up revealed no long-term sequelae except persistent chronic respiratory failure in one patient. The respiratory failure had developed before treatment of the fistula. The analysis of this series and a review of the literature underline the high index of suspicion required in all cases of chronic cough and lung suppuration, to diagnose this benign condition before life-threatening complications occur.