Idiopathic laryngotracheal stenosis

Abstract

ILTS is a rare inflammatory disease that results in a cicatricial stenosis of the cricoid and upper trachea. It occurs almost exclusively in women and is without known cause. Patients present with dyspnea on exertion that progresses to dyspnea at rest, often with stridor. The diagnosis is usually made on the basis of patient history, physical examination, and radiography. Rigid bronchoscopy is usually reserved for the day of proposed surgery to confirm diagnosis and to plan the operative strategy. On occasion, the presence of active inflammation extending into the immediate subglottis or the patient's use of corticosteroids requires that surgery be postponed. In these cases, a patent airway is temporarily restored with careful bronchoscopic dilation. Single-staged laryngotracheal resection is successful in more than 90% of patients and is the most effective treatment when performed by experienced hands. Long-term follow-up shows stable airway and improvement in voice quality. Palliative procedures such as repeated airway dilations should be reserved for poor surgical candidates. Protective tracheostomy is rarely required.