Subglottic stenosis (SGS) management has changed dramatically in the last decade. An improved understanding of SGS’ underlying mechanism is spawning new research into how to control the inflammatory process at the core of the disease.
Explore This IssueAugust 2020
“The goal of treatment has shifted over time to include a more personalized plan of care based on early recognition of at-risk populations and the understanding of the divergent pathophysiology affecting the unique patient subgroups,” said Alexander Gelbard, MD, associate professor of otolaryngology–head and neck surgery and co-director of the Complex Airway Reconstruction Program at The Vanderbilt Bill Wilkerson Center for Otolaryngology and Communication Sciences in Nashville.
Understanding Idiopathic SGS
Over the last 10 years, researchers and clinicians have increasingly appreciated three distinct categories of SGS: SGS from iatrogenic injury, autoimmune stenosis, and idiopathic subglottic stenosis. All three arise from luminal narrowing at the level of the subglottis. Fibrotic remodeling of this region in the airway manifests as fixed extrathoracic restriction of pulmonary ventilation, which patients experience as symptomatic dyspnea. Although most airway stenosis appears similar on anatomic imaging and clinical examination, different mechanisms of injury are associated with differing rates of long-term tracheostomy dependence.
Iatrogenic injury from long-term endotracheal intubation is the most common cause of SGS (70% of cases), followed by autoimmune stenosis associated with collagen vascular disease (15%), such as granulomatosis with polyangiitis, formerly known as Wegener’s granulomatosis. Idiopathic subglottic stenosis (iSGS), however, develops without a known antecedent injury and is the least common variant (15%) (Laryngoscope 2015;125:1137-1143).
Idiopathic SGS is a recurrent fibroinflammatory disease that leads to upper airway obstruction and severe shortness of breath. “Unlike many disorders, iSGS has a pretty homogenously affected patient population,” Dr. Gelbard said. Patients who develop iSGS, he noted, are almost exclusively healthy women (98%), nearly all adults (age 40 to 50), and white. “Traditionally, because of high recurrence rates, more than half of the patients with iSGS required repeat surgical procedures within 12 months of their initial diagnosis,” he added.
Surgical iSGS Management
Surgical iSGS management includes two different types of endoscopic procedures: dilation with or without cuts or laser, and resection with lasers and medical therapies. “Endoscopic iSGS treatment generally includes lysis of scar tissue, judicious excision of scar, balloon dilation, serial dilation, or a combination of these techniques,” noted Michael Johns, MD, director of the University of Southern California Voice Center and clinical professor of otolaryngology–head and neck surgery at the Keck School of Medicine of USC in Los Angeles.