Malformation | Characteristics | |
Nose* | Nasal deformities | Choanal atresia or agenesis, septum deformities, turbinate hypertrophy, vestibular atresia, or stenosis. |
Pharynx* | Craniofacial anomalies | Anomalies causing facial retrusion are associated with upper airway obstruction, including Crouzon, Pierre Robin, and Apert syndromes. |
Tongue | Macroglossia and glossoptosis. | |
Larynx | Laryngomalacia | Most common cause of chronic stridor in infants. Almost all patients present by 6 weeks of age. Symptoms are more pronounced after upper respiratory infections. |
Laryngeal webs | 75% located in the glottic area. Complete webs cause respiratory distress at birth and partial webs produce stridor, weak cry, and different degrees of respiratory distress. Associated anomalies are common. | |
Laryngeal cysts | If located in supraglottic area, may cause respiratory distress and stridor. | |
Laryngeal clefts | Characterized by abnormal communication between the larynx and pharynx, sometimes extending downward between the trachea and esophagus. Patients may present with aspiration, cough, swallowing difficulties, respiratory distress, hoarse cry, or occasionally with stridor; often associated with other congenital anomalies. | |
Subglottic hemangioma | Presents as with stridor and respiratory distress, usually worsening during the first few months of life. Often associated with cutaneous hemangiomas. | |
Subglottic stenosis | May be congenital but more often acquired secondary to intubation. Usually located 2 to 3 mm below the glottis. | |
Vocal cord paralysis | Idiopathic or secondary to a neurologic disorder (including Chiari II malformation, hydrocephalus, meningomyelocele, hypoxic cerebral palsy, and cerebral hemorrhage)[1,2]. | |
Trachea¶ | Tracheal stenosis | Usually presents with stridor or both stridor and wheezing. If stenosis is significant, respiratory distress occurs. |
Vascular rings or slings | 74% of vascular rings are symptomatic. The airway compression usually is intrathoracic, causing expiratory stridor. Associated anomalies are common. | |
Tracheomalacia | Often associated with other congenital anomalies. May be secondary to a vascular ring or cysts. Worsens with upper respiratory infections, crying, coughing, or feeding. May cause severe spells with cyanosis. | |
Bronchi and distal airways¶ | Bronchogenic cyst | May occur at any point throughout the tracheobronchial tree. Typically present during childhood with recurrent coughing, wheezing, or pneumonia, but may become symptomatic during infancy or adulthood or present as an incidental finding on chest radiographs. |
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