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Bronchomalacia and Tracheomalacia


Tracheomalacia and bronchomalacia refer to chondromalacia of a central airway, leading to insufficient cartilage to maintain airway patency throughout the respiratory cycle. These are common causes of persistent wheezing in infancy. Tracheomalacia and bronchomalacia can be either primary or secondary ( Table 416.1 ). Primary tracheomalacia and bronchomalacia are often seen in premature infants, although most affected patients are born at term. Secondary tracheomalacia and bronchomalacia refer to the situation in which the central airway is compressed by an adjacent structure (e.g., vascular ring; see Chapter 345 ) or deficient in cartilage because of tracheoesophageal fistula (see Chapter 345 ). Bronchomalacia is common following lung transplantation, assumed to be secondary to the loss of bronchial artery supply leading to ischemia of the bronchial cartilage. This form of bronchomalacia may take months to present following transplantation. Laryngomalacia can accompany primary bronchomalacia or tracheomalacia. Involvement of the entire central airway (laryngotracheobronchomalacia) is also seen.

Table 416.1
Classification of Tracheomalacia
From McNamara VM, Crabbe DC: Tracheomalacia, Paediatr Respir Rev 5:147–154, 2004.
PRIMARY TRACHEOMALACIA

  • Congenital absence of tracheal-supporting cartilages

SECONDARY TRACHEOMALACIA

  • Esophageal atresia, tracheoesophageal fistula

  • Vascular rings (double aortic arch)

  • Tracheal compression from an aberrant innominate artery

  • Tracheal compression from mediastinal masses

  • Abnormally soft tracheal cartilages associated with connective tissue disorders

  • Prolonged mechanical ventilation, chronic lung disease

Clinical Manifestations

Primary tracheomalacia and bronchomalacia are principally disorders of infants, with a male:female ratio of 2 : 1. The dominant finding, low-pitched monophonic wheezing heard predominantly during expiration, is most prominent over the central airways. Parents often describe persistent respiratory congestion even in the absence of a viral respiratory infection. When the lesion involves only one main bronchus (more commonly the left), the wheezing is louder on that side and there may be unilateral palpable fremitus. In cases of tracheomalacia, the wheeze is loudest over the trachea. Hyperinflation and/or subcostal retractions do not occur unless the patient also has concurrent asthma, viral bronchiolitis, or other causes of peripheral airways obstruction. In the absence of asthma, patients with tracheomalacia and bronchomalacia are not helped by administration of a bronchodilator. Acquired tracheomalacia and bronchomalacia are seen in association with vascular compression (vascular rings, slings, and innominate artery compression) or in association with the loss of bronchial artery supply in lung transplantation. Tracheomalacia is the rule following correction of tracheoesophageal fistula. Other causes of acquired tracheomalacia, which may persist after surgical correction include cardiomegaly. The importance of the physical exam cannot be understated; one study found that pediatric pulmonologists made a correct assessment of malacia based on symptoms, history, and lung function prior to bronchoscopy in 74% of cases.

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