Congenital Anomalies of the Larynx, Trachea, and Bronchi

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Chapter 378 Congenital Anomalies of the Larynx, Trachea, and Bronchi

Because the larynx functions as a breathing passage, a valve to protect the lungs, and the primary organ of communication, symptoms of laryngeal anomalies are those of airway obstruction, difficulty feeding, and abnormalities of phonation (Chapter 365). Obstruction of the pharyngeal airway (due to enlarged tonsils, adenoids, tongue, or syndromes with midface hypoplasia) typically produces worse obstruction during sleep than during waking. Obstruction that is worse when awake is typically laryngeal, tracheal, or bronchial and is exacerbated by exertion. Congenital anomalies of the trachea and bronchi can create serious respiratory difficulties from the first minutes of life. Intrathoracic lesions typically cause expiratory wheezing and stridor, often masquerading as asthma. The expiratory wheezing contrasts to the inspiratory stridor caused by the extrathoracic lesions of congenital laryngeal anomalies, specifically laryngomalacia and bilateral vocal cord paralysis.

With airway obstruction, the severity of the obstructing lesion, the work of breathing, determines the necessity for diagnostic procedures and surgical intervention. Obstructive symptoms vary from mild to severe stridor with episodes of apnea, cyanosis, suprasternal (tracheal tugging) and subcostal retractions, dyspnea, and tachypnea. Chronic obstruction can cause failure to thrive.

Bibliography

Zoumalan R, Maddalozzo J, Holinger LD: Etiology of stridor in infants, Ann Otol Rhinol Laryngol 116:329–334, 2007

378.1 Laryngomalacia

Lauren D. Holinger

Clinical Manifestations

Laryngomalacia is the most common congenital laryngeal anomaly and the most common cause of stridor in infants and children. Sixty percent of congenital laryngeal anomalies in children with stridor are due to laryngomalacia. Stridor is inspiratory, low-pitched, and exacerbated by any exertion: crying, agitation, or feeding. Stridor results from the collapse of supraglottic structures inwards during inspiration. Symptoms usually appear within the first 2 wk of life and increase in severity for up to 6 mo, although gradual improvement can begin at any time. Laryngopharyngeal reflux is commonly associated with laryngomalacia.

Diagnosis

The diagnosis is confirmed by outpatient flexible laryngoscopy (Fig. 378-1). When the work of breathing is moderate to severe, airway films and chest radiographs are indicated. With associated dysphagia, a contrast swallow study and esophagram may be considered. Because 15-60% of infants with laryngomalacia have synchronous airway anomalies, complete bronchoscopy is undertaken for patients with moderate to severe obstruction.