Practical safety in the diagnosis and treatment of congenital isolated tracheoesophageal fistula

Abstract

The rareness of H-type tracheoesophageal fistula in conjunction with its unspecific clinical presentation and wide range of anatomical presentation makes its diagnosis and treatment a problematic topic for both ear, nose and throat (ENT) specialists and pediatric surgeons worldwide. Symptoms and clinical signs of H-TOF are easily misleading. Diagnostic methods, most of the times, are dependent on the physician's experience; therefore, various errors may be made. We analyzed our experience in managing H-TOF cases over the last 15 years. Advice and strategies of action for health professionals directly involved in the diagnosis and treatment were identified, but also errors and mistakes while managing 6 cases. We analyzed 'red flags' but also important steps in the practical safety concerning this rare congenital malformation. Choosing the surgical access for division of the fistula throughout the cervical or thoracic approach is sometimes difficult. A scrupulous perioperative planning is mandatory. A dynamic overview of the patient's presentation never underestimating the subtlety of H-TOF presentation should be conducted for its early recognition and achieving best outcomes.

11 Cases of H-Type Tracheoesophageal Fistula: How Diagnosis and Treatment May Be Improved

“…If an esophagogram was done earlier on in another clinic without success, the investigation was not repeated. The sensitivity of esophagogram was low as it was also described in many other studies before [1,6,13,14,16]. The esophagogram allows to rule out differential diagnosis such as esophagus stenoses, gastroesophageal reflux, esophageal diverticula or swallowing disorders.…”

“…Key ongoing discussions on H-TOF concern which diagnostic methods are necessary, how to diagnose H-TOF as fast as possible, what the various risks of the different methods are and how best to proceed when H-TOF is diagnosed [6,7,10,12]. In this investigation, 6 out of 11 patients had an esophagogram, which was successful in 3 cases.…”

“…The disadvantage of a tracheobronchoscopy of course is, that the child needs full anesthesia. Based on the known success rate of tracheobronchoscopy for H-TOF diagnosis compared with esophagogram diagnosis observed in this study one could argue that on urgent suspicion of H-TOF, one should first resort to tracheobronchoscopy and to an esophagogram second and only if further investigation of a negative tracheobronchoscopy is required [6,10,12].…”

“…The symptoms are sometimes misguiding, which may lead to delayed diagnosis. Thus, the time of diagnosis varies from infancy to adulthood [6][7][8][9][10][11]. Diagnostic measures comprise an esophagogram with a contrast agent or tracheobronchoscopy.…”

Management of a double H-type tracheoesophageal fistula without esophageal atresia: a case report

“…Isolated tracheoesophageal fistula, or H-type tracheoesophageal fistula, (TEF) is a rare congenital disease affecting 4 to 5% of the general population of newborns worldwide [1,2]. An early diagnosis is still a challenge for pediatricians because of the non-specificity of symptoms [3,4]. TEFs are frequently attributed to more common etiologies, like gastroesophageal reflux (GER), swallowing disorders, or poor feeding techniques.…”