On the development of idiopathic subglottic stenosis

Abstract

Idiopathic subglottic stenosis is a fibrotic narrowing of the airway at the level of the cricoid cartilage, which can result in severe dyspnea. There is an overwhelming female preponderance, and treatment usually involves dilation, tracheostomy or resection with reconstruction of the involved portion of the airway. The exact mechanism of action is unknown. Estrogen has been thought to play a role in the pathogenesis of this disease, but estrogen receptors have not been found in tissue specimens taken from afflicted individuals. A careful history taken from the patient often reveals a severe episode of coughing prior to the development of symptoms, and intraoperative examination can reveal impaction of the first tracheal ring within the lumen of the cricoid cartilage. Based on these observations, we surmise that an intermittent telescoping effect of the first tracheal ring within the lumen of the cricoid cartilage can lead to disruption of the local blood supply and trauma to the cricoid mucosa, with consequent mucosal edema, ischemia, and ultimately fibrosis. While estrogen has been shown to play a beneficial role in wound healing, abnormal wound healing may be potentiated by variations in estrogen receptor expression, and could also explain the female preponderance seen in this disease.