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Key Points

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  1. Tracheal narrowing due to fixed (ie, complete tracheal rings) and/or dynamic (eg, tracheomalacia) airway obstruction can cause life-threatening respiratory distress in infants and children.

  2. Expedient investigations for tracheal stenosis must include echocardiography, cross-sectional imaging by computed tomography (CT) or magnetic resonance imaging (MRI), and bronchoscopy to determine the cause and define the extent of airway compromise.

  3. Optimal management of tracheal obstruction requires a multidisciplinary and yet individualized approach. Conservative management without surgical intervention may be warranted in select patients depending on severity of symptoms and extent of narrowing as the airway caliber in congenital tracheal stenosis (CTS) and tracheomalacia will improve with patient growth in many cases.

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Introduction

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Airway obstruction of the infant or pediatric trachea can precipitate acute and life-threatening respiratory compromise. While infections and foreign bodies are the most common causes of airway compromise in children, intrinsic airway narrowing due to CTS caused by complete tracheal rings or airway compression caused by anomalous cardiovascular anatomy and/or tracheomalacia represent the more challenging conditions for the pediatric surgeon to manage, especially in the setting of acute respiratory distress. Moreover, these 2 conditions may coexist in the same patient, presenting a complex clinical condition that requires thorough and systematic evaluation. This chapter will present the pathophysiology of airway obstruction, the diagnostic paradigm, and the surgical options for the treatment of tracheal stenosis and tracheomalacia.

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Disease Pathophysiology

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“Fixed” Tracheal Obstruction: Congenital Tracheal Stenosis

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Congenital tracheal stenosis is a rare condition defined as the congenital reduction in tracheal diameter due to the presence of abnormal and complete cartilaginous rings replacing the normal compliant C-shaped tracheal cartilages. It has been estimated that the incidence of CTS is approximately 1 in 65,000 live births. In addition to fixed narrowing due to small complete cartilaginous tracheal rings and a reduced mural compliance during respiration, there is hypertrophy of submucosal glands and connective tissue, further narrowing the tracheal cylinder in the affected segment. This malformation of the airway can be associated with a number of other congenital anomalies, including vascular or cardiac malformations in 25% to 70% of cases. Lung hypoplasia and total lung agenesis are also frequently associated with CTS, suggesting that lung and airway malformations may arise from a common developmental defect. Several classifications of CTS have been described including the morphologic classification by Cantrell and the functional classification by Anton-Pacheco being the most commonly used. According to the Cantrell classification, there are 3 morphologic types of the stenosis: diffuse hypoplasia, funnel type, and segmental stenosis (Fig. 22-1). This classification is particularly useful for determining the technique for reconstruction of the airway. The functional classification stratifies patients into mild, moderate, and severe categories according to presenting symptoms and age of presentation that could further help in defining prognosis and need for surgery. Interestingly, our experience representing more of a population-based patient cohort demonstrated that segmental narrowing ...

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