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.