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.