Laryngotracheal stenosis is a congenital or acquired condition characterized by narrowing of the airway in the supraglottis, glottis, subglottis, and/or proximal trachea. This narrowing can be caused by systemic diseases (eg, granulomatosis with polyangiitis), iatrogenically, or without a clear cause (idiopathic subglottic stenosis). Symptoms include dyspnea, cough, stridor, and dysphonia. Diagnosis is based on examination of airway, pulmonary function testing, and sometimes blood tests to exclude underlying conditions. Treatment is based on the degree of stenosis and ranges from conservative (medical) management and endoscopic monitoring to endoscopic surgery and open airway surgery.
Laryngotracheal stenosis can be caused by systemic diseases such as granulomatosis with polyangiitis, relapsing polychondritis, or amyloidosis (1). It can also occur iatrogenically (eg, as a result of tracheostomy placement or prolonged intubation), or after direct traumatic injury to the airway. There is a subset of patients who develop subglottic stenosis without an identifiable cause, referred to as idiopathic subglottic stenosis, which appears to predominantly affect White female patients. In pediatric patients, a history of recurrent croup infections should raise suspicion for underlying airway stenosis.
Laryngotracheal stenosis is frequently described based on level of stenosis (eg, supraglottic, glottic) and amount of narrowing (2). There are several grading scales to describe the percent stenosis, none of which fully incorporate another important factor, the length of stenosis. These descriptions are important for determining the etiology of the stenosis as well as potential treatment plans.
References
1. Gelbard A, Francis DO, Sandulache VC, Simmons JC, Donovan DT, Ongkasuwan J. Causes and consequences of adult laryngotracheal stenosis. Laryngoscope. 2015;125(5):1137-1143. doi:10.1002/lary.24956
2. McCaffrey TV. Classification of laryngotracheal stenosis. Laryngoscope. 1992;102(12 Pt 1):1335-1340. doi:10.1288/00005537-199212000-00004
Symptoms and Signs of Laryngotracheal Stenosis
Symptoms depend on the degree of laryngotracheal stenosis and may include dyspnea, stridor, and cough (1). Dysphonia and odynophagia may also occur. Patients with mild stenosis (ie, slight narrowing of the airway) may be asymptomatic.
Symptoms and signs reference
1. Smith MM, Cotton RT. Diagnosis and management of laryngotracheal stenosis. Expert Review of Respiratory Medicine. 2018;12(8), 709–717.
Diagnosis of Laryngotracheal Stenosis
Laboratory testing
Endoscopic evaluation
Pulmonary function evaluation
Sometimes CT imaging
Clinical assessment should focus on evaluation of the patient's level of distress, including the degree of stridor and the presence of serious signs such as tracheal tugging, suprasternal retractions, or subcostal retractions. If the patient exhibits signs of severe distress, emergent management, including the placement of a tracheostomy, may be necessary.
Laboratory evaluation should include testing for antineutrophil cytoplasmic autoantibody (ANCA), particularly to work up granulomatosis with polyangiitis. Other laboratory tests (eg, autoimmune workup including anti-nuclear antibody or ANA) may be obtained to assess for other systemic diseases or infection, depending on the clinical presentation.
Endoscopic evaluation is the mainstay for diagnosing and monitoring laryngotracheal stenosis. It can be performed in the office using flexible bronchoscopy or in the operating room with direct laryngoscopy and bronchoscopy. In addition to assessing the level and extent of the stenosis, endoscopy also allows for tissue biopsies to assist in identifying the underlying cause.
Pulmonary function tests (PFTs), are useful for initially characterizing the degree of stenosis and monitoring disease progression (1). In particular, peak expiratory flow can be a valuable aid in monitoring progression of stenosis and determining the need for surgical intervention in patients with idiopathic subglottic stenosis.
CT imaging can be valuable for characterizing the extent of the stenosis and assessing the involvement of various cartilaginous structures. It is typically performed prior to any surgical reconstruction.
Laryngotracheal stenosis is frequently missed or misdiagnosed as asthma, but symptoms of laryngotracheal stenosis are typically progressive and do not respond to bronchodilators. However, glucocorticoids, as used in asthma management, may transiently provide some clinical benefit and further confound the diagnosis.
Diagnosis reference
1. Carpenter DJ, Ferrante S, Bakos SR, Clary MS, Gelbard AH, Daniero JJ. Utility of Routine Spirometry Measures for Surveillance of Idiopathic Subglottic Stenosis. JAMA Otolaryngol Head Neck Surg. 2019;145(1):21-26. doi:10.1001/jamaoto.2018.2717
Treatment of Laryngotracheal Stenosis
Medical management
Office-based procedures (endoscopic monitoring and treatment)
Endoscopic interventions
Open airway surgery (rarely, tracheostomy)
Medical management of the underlying disease may improve stenosis caused by a systemic disease such as granulomatosis with polyangiitis. For other conditions, medical management is primarily adjunctive and used in combination with endoscopic procedures or surgical interventions, with the aim of reducing inflammation or scarring ( 1). Examples of agents that may be used include inhaled or oral glucocorticoids, sulfamethoxazole-trimethoprim, and nebulized saline treatments. Antireflux medications are recommended for patients with gastroesophageal reflux that may be impacting the stenosis. Additionally, topical (eg, mitomycin) or injected medications (eg, glucocorticoids) can be administered during surgical interventions to enhance procedural outcomes (). Examples of agents that may be used include inhaled or oral glucocorticoids, sulfamethoxazole-trimethoprim, and nebulized saline treatments. Antireflux medications are recommended for patients with gastroesophageal reflux that may be impacting the stenosis. Additionally, topical (eg, mitomycin) or injected medications (eg, glucocorticoids) can be administered during surgical interventions to enhance procedural outcomes (2).
Office-based procedures and disease monitoring through flexible fiberoptic visualization (endoscopy) is essential to the ongoing care of patients with stenosis. There has a been a rise in office-based treatments to reduce stenosis (eg, steroid injections and balloon dilations), and studies on efficacy and protocols are underway.
Endoscopic interventions, the mainstay of treatment, may include the use of rigid and balloon dilators, laser excisions, cryotherapy, and graft placements to treat stenosis (3). Adjuvant medications may be injected at the time of the procedure to decrease rate of restenosis. These interventions are typically performed under general anesthesia using rigid laryngoscopy and bronchoscopy.
Open airway surgery with resection and reconstruction of the stenotic area is often considered the definitive treatment for airway stenosis (4). This highly specialized procedure requires careful patient selection and perioperative management. In some cases, a temporary tracheostomy, stenting, or cartilage grafting may be necessary for successful treatment.
If a patient has stenosis that cannot be corrected in a timely manner or does not respond to other treatment modalities, a tracheostomy may be performed to bypass the stenotic area and facilitate breathing. A tracheostomy can be placed while the patient undergoes other interventions (with the goal of decannulation), or it may serve as a permanent solution for airway stenosis.
Treatment references
1. Hoffman MR, Patro A, Huang LC, et al. Impact of Adjuvant Medical Therapies on Surgical Outcomes in Idiopathic Subglottic Stenosis. Laryngoscope. 2021;131(12):E2880-E2886. doi:10.1002/lary.29675
2. Pan DR, Rosow DE. Office-based corticosteroid injections as adjuvant therapy for subglottic stenosis. Laryngoscope Investig Otolaryngol. 2019;4(4):414-419. Published 2019 Jun 10. doi:10.1002/lio2.284
3. Feinstein AJ, Goel A, Raghavan G, et al. Endoscopic Management of Subglottic Stenosis. JAMA Otolaryngol Head Neck Surg. 2017;143(5):500-505. doi:10.1001/jamaoto.2016.4131
4. Bitar MA, Al Barazi R, Barakeh R. Airway reconstruction: review of an approach to the advanced-stage laryngotracheal stenosis. Braz J Otorhinolaryngol. 2017;83(3):299-312. doi:10.1016/j.bjorl.2016.03.012
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