Congenital tracheoesophageal fistula without esophageal atresia

Abstract

Congenital tracheoesophageal fistula (TEF) without esophageal atresia, commonly referred to as H-type fistula, comprises only 4.2% of all TEF's. Four infants with this entity were surgically treated at Santa Rosa Children's Hospital during the period of 1974 to 1977, and their diagnosis, surgical management, and hospital course are described in detail. H-type TEF is characterized by a clinical triad consisting of paroxysms of coughing precipitated by feeding, gaseous distention of the gastrointestinal tract, and pneumonitis. Demonstration of H-type TEF can be difficult and may be accomplished only by repeated examinations. The esophagogram using cinefluoroscopy and image intensification is the primary diagnostic technique utilized. If this is nondiagnostic, then tracheobronchoscopy should be performed in conjunction with ancillary techniques which improve endoscopic yield. Surgical correction can be accomplished through a cervical approach in 80% of cases. Preoperatival treatment of H-type TEF is dependent upon a high index of suspicion, an aggressive diagnostic approach, and prompt surgical correction. Our series of patients demonstrates that early diagnosis is feasible.