Abstract
Tracheal pathology in children are primarily congenital. They can be considered primary or de novo, when this is seen as an inherent defect within the cartilages of the tracheal segment. While segmental cartilage defects are very rare, there are often occasions when one or more cartilages can be considered missing from the length of trachea, contributing to airway abnormality. Secondary tracheal pathologies can often be seen in relation to disorders affecting nearby vascular elements or thoracic cage in general. In general, the pathological entity of tracheal disorders can be classified into either tracheomalacia or tracheal stenosis.
Highlights
Long segment congenital tracheal stenosis (LSCTS) is a rare congenital abnormality occurring in about 1 in 100,000 live births [3]
In LSCTS more than 50% of the length of the trachea is stenosed, always due to complete tracheal rings (Figure 3)
Symptoms range from respiratory failure soon after birth requiring extracorporeal membrane oxygenation (ECMO) support to mild to moderate severity like noisy breathing, respiratory distress, or recurrent chest infections
Summary
Primary tracheomalacia can often be diffuse, with weakness affecting many cartilaginous arches, especially in the distal half of trachea This is often seen in preterm, or in neonatal phase, with clinical presentation often needing ventilation to support the respiratory system in these small babies. Pathologies of nearby vascular tree, namely vascular rings and innominate artery compression syndromes, can often lead to localized area of tracheal compression and persistence of tracheal weakness, thereby causing tracheomalacia. This may persist for a very long period, even after relief of compressing elements, especially if such a relief had happened later in life
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