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Congenital Tracheal Anomalies: Complete Tracheal Rings, Tracheomalacia, and Vascular Compression

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Abstract

The normal trachea consists of approximately 15–20 cartilaginous rings situated in a “horseshoe” or “C shape” as well as a posterior membranous portion in a 4–5:1 ratio (Cummings et al. Otolaryngology – head & neck surgery [print/digital]. Philadelphia: Elsevier Mosby, 2005). Alterations in this ratio are seen in two of the most common congenital tracheal anomalies, tracheal stenosis and tracheomalacia. Congenital disorders of the trachea, while rare, can cause a wide range of symptoms with varying severity. The diversity in presentation mandates a high degree of clinical suspicion to identify these disorders. Management has evolved significantly, such that there is potential to address both morbidity and mortality in this patient population. Concomitant congenital anomalies may be present and can complicate management. A multidisciplinary approach to care is necessary to ensure the best airway, feeding, and overall outcomes for these complex patients. This chapter will focus on the clinical presentation, diagnosis, and management of congenital tracheal stenosis, most commonly caused by complete tracheal rings. Additionally, other causes of tracheal stenosis and disorders involving tracheal collapse, including both intrinsic and extrinsic causes, will be reviewed.

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Wilcox, L.J., Miller, C., Rutter, M.J. (2020). Congenital Tracheal Anomalies: Complete Tracheal Rings, Tracheomalacia, and Vascular Compression. In: McMurray, J., Hoffman, M., Braden, M. (eds) Multidisciplinary Management of Pediatric Voice and Swallowing Disorders. Springer, Cham. https://doi.org/10.1007/978-3-030-26191-7_36

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