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