From the Guidelines
Severe tracheomalacia is primarily caused by a combination of congenital and acquired conditions that weaken the tracheal structure, with the most recent evidence suggesting that excessive dynamic airway collapse is a key factor in its pathogenesis 1. The primary causes of severe tracheomalacia can be categorized into congenital and acquired conditions. Congenital causes include incomplete or abnormal formation of tracheal cartilage rings during fetal development, often associated with genetic syndromes like VACTERL association, Ehlers-Danlos syndrome, or trisomy 21. Acquired causes, on the other hand, include:
- Prolonged intubation or tracheostomy, which can damage the tracheal wall
- Chronic inflammation from conditions like polychondritis or severe COPD
- External compression from vascular anomalies such as innominate artery compression or double aortic arch
- Trauma to the trachea
- Post-surgical complications following procedures near the trachea The pathophysiology of severe tracheomalacia involves weakening of the tracheal wall, causing excessive collapse during respiration, which can lead to significant morbidity and mortality if left untreated 1. Management of severe tracheomalacia depends on the severity of the condition, but may include continuous positive airway pressure (CPAP), surgical interventions like aortopexy, tracheal stenting, or tracheoplasty in severe cases where the airway collapse significantly impairs breathing 1. It is essential to note that the most recent evidence suggests that excessive dynamic airway collapse is a key factor in the pathogenesis of severe tracheomalacia, and treatment should be tailored to address this specific condition 1.
From the Research
Primary Causes of Severe Tracheomalacia
The primary causes of severe tracheomalacia can be attributed to several factors, including:
- Congenital abnormalities, such as esophageal atresia with or without tracheoesophageal fistula 2, 3, 4
- Abnormal division of the embryonic foregut, which can lead to a decrease in the ratio of cartilage ring to posterior membranous wall 2
- Association with other airway anomalies, such as laryngomalacia, bronchomalacia, and large laryngeal clefts 2
- Impaired airway protection reflexes, which can lead to aspiration and contribute to the development of severe tracheomalacia 3
Associated Conditions
Severe tracheomalacia is often associated with other conditions, including:
- Esophageal atresia-tracheoesophageal fistula (EA-TEF) 3, 4, 5
- Tracheobronchomalacia 4, 6
- Laryngomalacia, bronchomalacia, and large laryngeal clefts 2
- Vascular rings 5
Diagnosis and Treatment
Diagnosis of severe tracheomalacia can be made using various modalities, including:
- Clinical history and physical examination 2
- Pulmonary function testing 2
- Computed tomography (CT) scans 3
- Dynamic magnetic resonance imaging (MRI) 2
- Fiberoptic bronchoscopy 2, 3
- Treatment options for severe tracheomalacia include pharmacotherapy, positive pressure application, and surgery, such as aortopexy and tracheopexy 2, 3, 5