Virtually all therapies used in patients with EA-TEF are directed to specific problems/symptoms and are based on empiric evidence. The RCWG rated the following:Airway Clearance: There was strong agreement that age appropriate airway clearance techniques (e.g. manual chest physiotherapy, High Frequency Chest Wall Oscillation, Positive Expiratory Pressure valve) should be used routinely to aid the clearance of secretions. There are no studies comparing these techniques in the EA-TEF population (or in tracheomalacia in general). Therefore, the selection relies on the availability of the various devices, and on institutional practices163–165. The Positive Expiratory Pressure valve technique may be particularly helpful in patients with tracheomalacia and/or bronchiectasis, by stenting collapsible airways open and aid airway clearance166.Anti-GE Reflux therapy: The association of GER with respiratory morbidity and the effectiveness of empiric anti-GER treatment remain highly controversial58,61,167. This is in part, due to the fact that respiratory symptoms from the upper and/or from the lower airways can be caused by miniscule amounts of refluxate and as a result the usual diagnostic studies for GERD may likely be within the normal range. The RCWG did not address in detail the management of GER, GERD and aspiration because they have been recently reviewed in detail in the INoEA Consensus Guidelines on Gastrointestinal Complications of EA-TEF19,167. However, a moderate agreement was reached on trying empirically anti-GER therapy for patients with persistent respiratory symptoms based primarily on the fact that the prevalence of GER is very high in EA-TEF52,168.
Bronchodilators : The use of bronchodilators in EA-TEF remains controversial. On one hand there is evidence of increased airway hyperreactivity among patients with EA-TEF that warrants the use of bronchodilators15,68. Albuterol is also being used to improve mucociliary clearance169. On the other hand, chronic “wheezing” in patients with EA-TEF is often caused by the collapse of the tracheal lumen that cannot be prevented or reversed with bronchodilators. Moreover, the overrelaxation of the tracheobronchial smooth muscle may actually worsen the obstruction (Figure 3A)170. Thus, although, a trial with bronchodilators for symptomatic children is warranted, the RCWG does not recommend β2-agonists as standard therapy for EA-TEF. However, ipratropium bromide (an anticholinergic agent) has been shown to be associated with improved symptoms in children with TM (possibly due to decreased effect on the tracheobronchial muscle tone and/or by reducing the amount of secretions) and it may be considered as an alternative of albuterol171..
Inhaled corticosteroids (ICS) : The role of steroids in TBM is rather controversial and there was weak agreement on their routine use because the inflammation in EA-TEF tends to be neutrophilic and therefore less likely to respond to steroids63. Furthermore, there is some evidence that chronic use of high doses of ICS may actually cause or exacerbate existing tracheomalacia172 . Thus of the routine use of ICS should be reserved for documented or highly suspected airway hyperreactivity.
Mucolytics : Mucolytic agents (Dornase alpha, hypertonic saline, N-Acetylcysteine) are often used in patients with tracheomalacia on the assumption that the impaired airway clearance probably results in the accumulation and possibly thickening of secretions. Dornase alpha and hypertonic saline are commonly used in the management of cystic fibrosis-related chronic bronchiectasis, but their effect in non-CF bronchiectasis is not as well defined173. Thus, the RCWG did not support its routine use.