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This chapter presents some of the congenital tracheal and vascular anomalies that can be seen in children and ultimately lead to difficulties with recurrent respiratory infections, secretion management, and breathing. These problems tend to be complex and are best handled by a multidisciplinary approach that includes pediatric surgery, gastroenterology, neonatal intensive care, pulmonary, radiology, pediatric intensive care, cardiac intensive care, cardiology, cardiac surgery, pediatric anesthesia, operating room teams, otorhinolaryngology (ORL), plastic surgery, and interventional radiology.


The trachea is formed from hyaline cartilage (“C” shaped) rings anteriorly and laterally and smooth muscle posteriorly. Airway development begins as a bud off the primitive esophagus during the fourth week in utero.1,2 Anomalies with separation of the airway and esophagus are thought to account for many of the congenital anomalies found in the airway and the esophagus, including esophageal atresia (EA), tracheoesophageal fistula (TEF), laryngeal clefts (LC), and laryngotracheal esophageal clefts (LTEC).2

The airway has different structure and has different surrounding structures throughout its course in the mediastinum. Taking these differences into account is important for assessing the airway and for communicating to other providers. For these reasons, the airway can be separated into separate regions based on the surrounding anatomy. T1 is the extrathoracic trachea located above the clavicles and up to the cricoid cartilage. T2 is designated as the area below the clavicles to the takeoff of the innominate artery and can usually be easily visualized during bronchoscopy; T3 is typically the lower third of the trachea with the carina as the triangle-point between the takeoffs of the two mainstem bronchi.3 The right mainstem is divided into the proximal and distal right mainstem (R1 and R2), the bronchus intermedius, and the right upper lobe. The left mainstem is divided into L1, L2, and L3 (Fig. 52-1).4

Figure 52-1

Segments of the trachea. This is used to describe areas of malacia or compression from great vessel anomalies.

This classification system helps when documenting areas affected by compression or airway abnormalities. The picture shown in Figure 52-2 highlights the different segments of the trachea with its associated vascular neighbors.5 Upper T1 is covered by the thyroid, lower T1 is situated in between the carotid arteries, upper T2 is under the brachiocephalic vein, lower T2 tends to be most affected by compression of the innominate artery, upper T3 is in close proximity to the transverse aortic arch, and lower T3 is where the arch crosses to become the descending aorta.6 The carina, R1, and L1 tend to be affected by anterior pulsations of the pulmonary artery, where L2 can be affected posteriorly by pulsations of the descending aorta. L3 and R2 tend to be unaffected by surrounding vascular structures.6 The distal trachea (T3) can also be ...

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