Idiopathic progressive subglottic stenosis: findings and treatment in 52 patients

Rarely, patients develop severe idiopathic subglottic stenosis. In 34 years, we have observed this disorder in 52 patients. All but 1 of the patients were female--a finding that suggests a hormonal cause. Without treatment, the airway progressively narrows--in some cases, until the patient requires tracheotomy. Laser submucosal resection and rotation mucosal flaps open and stabilize the airway and provide effective palliation. However, unlike traumatic subglottic stenosis, which has been cured with this technique, the idiopathic form causes submucosal fibrosis that regenerates spontaneously. Thus, treatment helps, but does not cure, the patient. The characteristic pathological finding is of submucosal dense fibrotic tissue with evidence of chronic inflammation. The clinical findings and treatment are here discussed.