Focal malacia may also occur in relation to a widened membranous wall or residual pouch after repair of TEF and esophageal atresia (Figure 6-12). Defective cartilaginous rings may also be found at this level. Filler and colleagues considered this association to be the most common cause of tracheomalacia in infants, although precise explanation is lacking.26 If severe collapse follows TEF repair, aortopexy may have to be considered.27 Since gastroesophageal reflux can also be present, antireflux surgery may be needed.In a rare case, agenesis or hypoplasia of the right lung may result in mediastinal displacement and rotation severe enough to result in compression of the remaining bronchus at its origin, which is analogous to postpneumonectomy syndrome (see Chapter 15, "Tracheobronchial Malacia and Compression").28,29
When a short segment of malacic trachea accompanies pulmonary artery sling rather than segmental congenital stenosis, it may be managed by tracheopexy (or aortopexy) in conjunction with division and reimplantation of the anomalous left pulmonary artery.30
Laryngomalacia quite often causes inspiratory, fluttering stridor in the newborn, but usually corrects itself in 1 or 2 years, although it may require tracheostomy—which in turn may lead to later tracheal stenosis. Since this book does not pretend to give comprehensive coverage of laryngologic problems, no more will be mentioned of congenital laryngeal lesions such as absence or deformities of laryngeal cartilages, cysts and laryngoceles, congenital nerve paralysis, or of hemangioma and lymphangioma of the larynx, the latter an extension of cervical cystic hygroma.
Tracheobronchomegaly (Mounier-Kuhn disease) appears to be of congenital origin, but most patients have not become overly symptomatic until midlife.31,32 Then, symptoms can often be traced back to youth. It has also been discovered in children. The condition is very rare and thought possibly to be due to absence
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