Tracheal atresia is an extremely rare anomaly and refers to a congenital absence of the trachea.
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Epidemiology
There may be a greater male predilection 5.
Associations
Associated anomalies can be present in up to 90% of cases 5:
congenital high airways obstruction syndrome (CHAOS): some authors consider this as synonymous with tracheal atresia although the syndrome can occur with tracheal stenosis or laryngeal atresia/stenosis
esophageal atresia with tracheo-esophageal fistula 7: tracheal atresia can occur in some of the subtypes
Pathology
Tracheal atresia falls under the spectrum of laryngeal-tracheo-bronchial atresia which in turn results either from an obstructing lesion (i.e. cartilaginous bar) or vascular insult with atresia of the airways which occurs during intrauterine development.
Radiographic features
Antenatal ultrasound
may show uniformly increased echogenic enlarged lung +/- pleural effusions +/- fetal ascites
markedly dilated fluid-filled bronchi may be seen
may also show ancillary sonographic features such as the presence of polyhydramnios
the fetal cardio-thoracic circumference ratio is decreased due to larger lung volumes
Differential diagnosis
General differential considerations include:
laryngeal atresia: sometimes can be impossible to differentiate on imaging
severe congenital tracheal stenosis
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