Infants with mild TM can be successfully extubated after the repair of
the EA-TEF and do not require any ventilatory or other support. They
often remain symptomatic with recurrent or persistent symptoms (such as
“barking” cough and coarse wheeze), which tend to be amplified during
episodes of upper and/or lower respiratory tract infections.
Tracheomalacia tends to improve over time due to the increase in
diameter of the tracheal lumen and a reduction in airway collapsibility
that minimize the degree of obstruction relative to lumen size. However,
it is a slow process that even under the best circumstances continues
for years.
Patients with severe (especially extensive) tracheomalacia (with or
without bronchomalacia) may have severe, potentially life-threatening
symptoms due to the almost complete tracheal collapse, and they are
likely to require some type of ventilatory
support127–130. Such support is usually needed
immediately after extubation following the surgical repair of the
EA-TEF, but may be needed for weeks or months afterwards. Patients
without lung disease and/or respiratory muscle weakness respond well to
non-invasive positive airway pressure (continuous positive airway
pressure (CPAP) or Bi-Level Positive Airway Pressure (PAP)) that
prevents the complete collapse of the tracheal lumen. High flow nasal
cannula (HFNC) is a newer modality that has been shown to be effective
and safe71,131–133.
There is concern that non-invasive PAP in the immediate post-repair
period increases the risk of air leak in the area of the repaired TEF,
or damage to the esophageal anastomosis134. However,
in practice the various forms of PAP/HFNC have been used
safely135 and the RCWG felt that the risk of
respiratory decompensation in the immediate post-operative period,
outweighs the theoretical risks of non-invasive PAP. The situation is
more problematic for infants who may need prolonged continuous support
because in many countries (including the US) the non-invasive types of
ventilation are not approved for continuous use in the home environment.
Aortopexy has been used successfully for TM due to compression of the
anterior tracheal wall (e.g. compression by the innominate
artery)136. However, in EA-TEF the tracheal occlusion
is caused mostly by the intrusion of the posterior tracheal wall into
the tracheal lumen. Thus a posterior tracheopexy (pulling of the
posterior tracheal membrane with sutures that are secured onto the
anterior longitudinal spinal ligament) has been suggested as a more
definitive treatment. The technique has been modified to include
aortopexy of the descending aorta that is often causing severe
compression of the posterior wall of the left main stem bronchus36,71,100,127,136–140. These techniques are still
very much dependent on specific surgical and institutional expertise and
the evidence about their long-term effectiveness is still scant. Even
the timing of the procedure varies, with some centers performing these
procedures at the time of the initial repair of the EA-TEF, while other
consider them only for severe persisting malacia not responding to
non-invasive PAP. Thus, they did not receive a strong recommendation by
the RCWG. However, it was recognized that they could become the standard
of care in the future.
For patients who have repeated, life threatening cyanotic episodes
despite non-invasive PAP, and are not candidates for surgical repair, a
tracheostomy provides a secure airway that can be used with or without
positive pressure support141. Because most TEFs are
located near the carina, a regular tracheostomy tube that extends only
to the middle trachea may not prevent the collapse of the lower trachea.
Therefore a custom made tube that extends to the lower trachea may be
necessary (these tubes may also provide enough stenting of the main stem
bronchii)142.
The use of tracheal (and/or bronchial) stents for the management of
severe TBM was recently reviewed by the European Respiratory
Society143. Theoretically, stents would be the
“ideal” solution to the problem of severe TBM. However, they are
rarely used in infants because of lack of size-appropriate stents, stent
migration, difficulty of removal, and most importantly, because of the
need to replace them with a bigger stent as the trachea grows.
Bethanecol is a synthetic muscarinic stimulant that has been used in
certain cases of tracheomalacia. Stimulation of the muscarinic receptors
in the airways should normally produce bronchospasm, but it seems to
actually “open” an airway that is floppy due to malacia by increasing
the trachealis muscle tone. There are no RCTs on its use but in a small
series it appeared to be safe 144.