Idiopathic subglottic stenosis – a familial association and review of practice

Abstract

Abstract Objective Idiopathic subglottic stenosis describes subglottic stenosis where no inflammatory, traumatic, iatrogenic or other causative aetiology can be identified. The present study aimed to outline our institution's experience of patients diagnosed with idiopathic subglottic stenosis and describe a very rarely reported familial association. Methods A retrospective review was conducted of prospectively maintained medical records from 2011 to 2020. Patient clinical, radiological and intra-operative data were reviewed to assess for defined endpoints. Results Ten patients with idiopathic subglottic stenosis were identified in this series. One familial pairing was identified, with two sisters presenting with the condition. Successful treatment with carbon dioxide laser and dilatation was achieved in most cases. Conclusion Idiopathic subglottic stenosis represents a rare, clinically challenging pathology. Management with endoscopic laser and balloon dilatation is an effective treatment. This paper highlights a very rare familial association, and describes our experience in treating idiopathic subglottic stenosis.