Picture of Congenital Subglottic Stenosis

Congenital subglottic stenosis is defined as subglottic stenosis in the absence of a history of intubation, other laryngeal trauma, or extrinsic compression, such as a vascular malformation like a double aortic arch. The child may present at birth, in the neonatal period, or in the first few weeks or months of life. It is caused by failure to recanalize the lumen of the larynx and trachea in embryonic life, thus resulting in atresia, webs, or stenosis. Not unexpectedly, there is an increased incidence of other lesions in the airway if one area is already affected.

Symptoms :The children appear reasonably well with normal growth and development, but they come to the physician's attention because of stridor. The stridor may have an expiratory component as well as the normal inspiratory component , ie, biphasic. The child may also present with dyspnea, a brassy or barky cough, hoarseness, and/or weak or unusual cry. A prolonged episode or unusual course of croup may also bring the child to attention.

The physical examination findings depend on the amount of obstruction in the airway. The child may have only a minimal amount of obstruction, and normally may be without symptoms. But obstruction of the airway may rapidly increase when the edema caused by an URI is superimposed on the already compromised airway. For example, 1 mm of edema in the subglottic region will decrease the cross-sectional area of the airway by over 50%.

Obviously, if the original obstruction is severe, the child will present early and with more severe symptoms, including presentation of stridor in the delivery room.

Evaluation of the child with possible congenital subglottic stenosis may include the use of endolateral neck x-rays to look for narrowing in the subglottic region. Bronchoscopy, or the more correct term laryngoscopy if only the laryngeal structures are visualized, is needed for complete evaluation of the airway, to rule out other anomalies, and for definitive diagnosis.
Bronchoscopy shows marked soft tissue swelling 2-3 mm below the vocal cords. Occasionally, the lumen is eccentric with greater involvement of one area of the wall. Click Here