Congenital tracheal stenosis

Congenital tracheal stenosis (CTS) is a rare congenital malformation characterized by narrowing of the trachea (windpipe) that is present from birth. The narrowing is most commonly caused by complete cartilaginous tracheal rings, where the normal C-shaped tracheal cartilages are replaced by O-shaped (complete) rings, eliminating the flexible posterior membranous wall of the trachea. This results in a fixed, rigid narrowing of the airway that can range from a short segment to involvement of the entire length of the trachea (long-segment tracheal stenosis). The condition primarily affects the respiratory system and can be life-threatening if severe. Key symptoms include stridor (noisy breathing), respiratory distress, wheezing, recurrent respiratory infections, cyanosis (bluish discoloration of the skin), and difficulty feeding. Symptoms may be present from birth or may become apparent in the first weeks to months of life, often worsening during respiratory infections when airway swelling further compromises the already narrowed trachea. The severity of symptoms depends on the degree and length of the stenosis. Congenital tracheal stenosis is frequently associated with other congenital anomalies, particularly cardiovascular malformations such as pulmonary artery sling (where the left pulmonary artery aberrantly courses between the trachea and esophagus), as well as other airway, cardiac, and gastrointestinal anomalies. Diagnosis is typically made through bronchoscopy and imaging studies such as CT angiography. Treatment depends on the severity and extent of the stenosis. Mild cases may be managed conservatively with close monitoring. Moderate to severe cases, particularly long-segment stenosis, typically require surgical intervention. The slide tracheoplasty has become the gold standard surgical technique, in which the stenotic trachea is divided, and the two halves are slid over each other to create a shorter but wider airway. This procedure has significantly improved outcomes compared to older techniques such as pericardial patch tracheoplasty. Outcomes have improved substantially in specialized centers, though the condition still carries significant morbidity and mortality, particularly in neonates with severe stenosis and associated anomalies.

Common questions about Congenital tracheal stenosis