Idiopathic (unknown cause) inflammatory subglottic stenosis is a frustrating breathing problem seen mostly in women. It appears to be an autoimmune disorder that we have thought for many years to be an incomplete expression (forme fruste) of Wegener’s Granulomatosis, aka granulomatosis with polyangiitis (GPA). In a caseload of perhaps 125 patients, it appears that standard of care remains occasional outpatient dilation, steroid injection, and mitomycin C application. Most patients go a year or more between dilations. The rub: occasionally, dilations need to be done more frequently in an “active” case, or there may be reasons to avoid the brief general anesthesia required for dilation such as: morbid obesity, difficult jaw/tooth/neck anatomy, or even a phobia of general anesthesia.

“Office” Treatment Option for Inflammatory Subglottic Stenosis

In such situations, one naturally casts about for alternatives. Low dose methotrexate has not been very effective in our experience. And we work with a motivated rheumatologist open to considering rituximab, even with a negative ANCA study. Or the laryngologist might consider an office-based airway procedure as illustrated in the photos below. This particular person only requires dilation every 18 months or so, but it represents a major stressor for her given her fears, her size, and somewhat challenging anatomy. She is one of several offered the treatment shown below: steroid injection in the voice laboratory, followed by laser treatment.

Our thought is that in some, it may be the ridges and air turbulence that creates the feeling of airway restriction, or that these rings of stenosis “catch” mucus. In the case below, the clinician was almost dubious that what was accomplished would make that much difference to the person’s breathing. But upon calling her a week after the procedure below, and asking if the improvement was nil, subtle, small, medium, or large, she replied “large…a huge improvement.”

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60% Stenosis (1 of 5)

Estimated at a 60% stenosis, using the vocal cords as a reference.

Narrowing & erythema (2 of 5)

At closer range, the examiner sees concentric ridges of narrowing and erythema characteristic of this disorder.

Triamcinolone is injected (3 of 5)

With patient sitting in the chair with topical anesthesia, a needle is passed from anteriorly just below the cricoid. Here, triamcinolone is being injected into the posterior component of the stenosis.

Laser ablation (4 of 5)

The blue laser fiber (thulium laser) is seen just prior to beginning laser ablation. Note in particular the circular shape of the area of greatest stenosis, to compare with the next photo.

Stenosis is coagulated (5 of 5)

The anterior half of the ring of greater stenosis has been coagulated. Note that the shape of the opening is changed to more of an anterior-posteriorly oriented oval. Distal trachea is also more easily seen. Again, the patient reported that this improved her breathing to a large degree.

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Idiopathic Subglottic Stenosis Has Different Levels

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Post dilation (1 of 4)

Six months after dilation of idiopathic (inflammatory) subglottic stenosis, the patient has noted only slight deterioration, and breathing ability remains acceptable to her.

Closer view (2 of 4)

At closer range, the inflammatory component appears more evident.

Rich vascular pattern (3 of 4)

The rich vascular pattern accompanying the lesion is seen better and is a visual finding of inflammation.

"Sharing" the airway (4 of 4)

Here, the scope has been passed through the area of maximal narrowing and the patient becomes acutely aware of greater difficulty breathing. "Sharing the airway" is a way of 'measuring' it functionally. Note again the congested capillaries.

Another way to Inject Idiopathic Subglottic Stenosis

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Idiopathic inflammatory subglottic stenosis (1 of 3)

This patient is about to receive a triamcinolone (steroid) injection into her idiopathic inflammatory subglottic stenosis, while sitting in a chair under topical anesthesia. Dotted circle is for reference with Photo 2.

Priot to injection (2 of 3)

A needle has been passed through anterior neck skin and its tip rests out of sight, submucosally just inferior to the anterior cricoid ring. Note that the milky white medication has been infused submucosally within the dotted ring.

Injection (3 of 3)

Here, the 27-gauge needle traverses the trachea in order to inject the posterior tracheal wall. The submucosal white medication appears at the *.
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