pulmonary arteries in children. The anatomic disposition of the pulmonary arterial sling, however, does not generally permit this transposition without division and reimplantation of the anomalous artery. The distortion of the artery may also compress the resected trachea and cause recurrent obstruction.23
Congenital tracheomalacia that is not the result of compression by vascular structures, by an extrinsic mass, or related to congenital TEF, occurs only rarely. It is infrequently documented in convincing fashion. Primary congenital tracheomalacia is recognized early in life, sometimes in previously apparently healthy infants. It is characterized by progressively noisy respiration, a "seal-bark" cough, episodic cyanosis, increased respiratory rate, intercostal retraction, and stridor which is most noticeable on expiration.24,25 Symptoms worsen with agitation and respiratory infections. Apneic spells may occur. Fluoroscopy may be of diagnostic help but bronchoscopy (preferably rigid) is the key diagnostic technique, showing tracheal narrowing from front to back, indistinct tracheal rings, and expiratory collapse of distal trachea and main bronchi (Figure 6-11 and Figure 3 [Color Plate 12]). Symptoms of primary tracheomalacia often clear by the second year of life, with stability of the trachea then seen on bronchoscopy. In severe cases, prolonged stabilization of the airway is obtained by tracheostomy. Growth of cartilage eventuates in recovery in most cases, in 2 or 3 years. In others, silicone stents (Y stent for lower trachea and main bronchi) can provide long-term patency. Expandable stents are not advisable because of possible permanent tracheal injury and growth problems.
figure 6-10 Anatomic relationships with anomalous left pulmonary artery sling. A, Anterior view showing the aortic arch and ligamentum arteriosum. Note position of vagus and recurrent laryngeal nerve. B, Transverse-sectional view of the left pulmonary artery sling behind the trachea. A = aorta; BC = brachiocephalic artery; E = esophagus; LCC = left common carotid; LPA = left pulmonary artery; LS = left subclavian artery; PA = pulmonary artery; T = trachea; V= vagus nerve.
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