Clinical Manifestations:
The spectrum of clinical manifestations of LPAS is very wide ranging from no symptoms to mild respiratory distress and noisy breathing on exercise, recurrent chest infections and swallowing problems.28,29 At the other end of the spectrum neonates and infants may present with life-threatening disease due to critical airway narrowing. The diagnosis should be suspected if intubation results in an abnormally high endotracheal tube that cannot be pushed down due to undiagnosed tracheal stenosis (figure 3). Often hypercarbia and ventilation difficulties may be out of proportion to oxygenation issues.28 Neonates may, however, be symptom-free even in the presence of severe tracheal narrowing due to lower respiratory flow rates which do not lead to turbulent flow.30 Symptoms of upper airway obstruction and stridor often present after 6 months of age When the patient becomes more ambulant for example following an intercurrent chest infection where the diagnosis can be made as an incidental finding.Older patients may present with an asymptomatic murmur, chronic cough,dyspnea, wheeze or choking sensation.28,29 The most important prognostic factor is the extent of trachea bronchial disease, especially a long segment of tracheobronchial stenosis, rather than the associated cardiac defects.29