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Tracheomalacia (TM) is a localized or generalized weakness of the tracheal wall, which creates airway obstruction, resulting in different degrees of possible respiratory symptoms. It can be isolated or associated with other anomalies such as anterior vascular compression, esophageal atresia with tracheoesophageal fistula (TOF), or gastroesophageal reflux. Although in some cases spontaneous improvement can occur, TM can lead to severe cough, respiratory distress episodes, or “near-death” spells (acute life-threatening events).
Among the preoperative investigations, fiberoptic bronchoschopy (FOB) plays the main role. In our institution, FOB is used with bronchography to additionally assess the dynamics of the trachea during the respiratory phases, providing quantitative measures of the airway and dynamic information regarding opening pressures.
In most cases, a computerized tomography scan is useful to study the relationship between cardiovascular and airway anatomy and to help define the best treatment. Treatment relates to the severity of symptoms and can vary from a conservative approach awaiting spontaneous improvement to airway stenting or aortopexy.
Aortopexy has been demonstrated to be effective in treating most cases of TM.
Its rationale is to bring the vascular structures away from the airways toward the sternum. Depending on the type of TM, different technical solutions can be adopted. If malacia is secondary to vascular compression, the pretracheal fascia is dissected from the posterior aspect of the aorta and innominate artery, creating a free space and relieving the compression on the airway. Second, in the presence of posterior collapse of the tracheal wall, mainly associated with TOF, or after fetal endotracheal occlusion,
All have advantages, but our routine approach is through a limited superior sternotomy, because it gives excellent exposure and easy access to the superoanterior mediastinum and pretracheal fascia, as well as safe control of the aortic arch in case of complications. Moreover, it is suitable for all morphologic variants.
Perioperative bronchoscopy can be performed to demonstrate the improvement of the tracheobronchial lumen. The pericardial drain is removed after 24 hours. Usually the patients are easily extubated in theater or shortly afterward and are expected to have a short and easily manageable postoperative hospitalization. Complications are rare, although pericarditis, pneumonia, bleeding, chylothorax, or phrenic or recurrent nerve lesions have been observed. Pericardial effusion is the most common one and may be life-threatening; thus, we prefer to perform echocardiography 2 to 3 weeks post surgery. Our experience indicates that the results of aortopexy relate to the group of patients with TM.
Patients with congenital TM, associated with TOF (group 1) or vascular compression (group 2), have better outcomes than patients with acquired TM, usually associated with other comorbidities, mainly cardiac anomalies (group 3). In the latter group we have observed an increased risk of mortality (9% overall), failure of the aortopexy and need for a redo surgery (4%), of stenting failure (5%), or a tracheostomy insertion/decannulation failure (9%).
The quality-of-life results have been excellent after a mean follow-up of 7 years in 73% of all patients, but we have observed a significant difference among groups, as 80% of the patients in groups 1 and 2 reported excellent results, compared with only 57% of group 3.