Key Points

• Lower laryngeal and upper tracheal circumferential fibrous stenosis of idiopathic origin is occasionally seen, principally in women. Patients are characterized chiefly by the fact that they have no known cause of these stenoses, however the lesions do share typical features such as location, configuration, clinical evolution, pathology, and response to surgical treatment.
• Autoimmune and connective tissue disorders (such as Granulomatosis with polyangitis) must be ruled out
• Palliation is provided by various forms of dilation, but is not curative and often leads to tracheostomy.
• Idiopathic subglottic stenosis is most often successfully managed by single-stage resection and reconstruction. Subsequent progression of the lesion is rare and initial good results tend to remain stable.