An incomplete or frustrated form (forme fruste) of Wegener’s granulomatosis,* which we believe to be the cause behind some cases of inflammatory subglottic or tracheal stenosis. Unlike full-fledged Wegener’s, this forme fruste variant may or may not necessarily involve the sinus and nasal cavities, and in the author’s caseload of about 60 patients, it has not ever progressed to involve the lungs and kidneys. Such patients can go for years with only the need for intermittent dilation of the subglottic or tracheal narrowing. This disorder may be the same as what some call “idiopathic subglottic stenosis,” for which some have recommended cricotracheal resection and reanastomosis as treatment.
*Newer terminology is granulomatosis with polyangiitis (GPA)
Subglottic / Tracheal stenosis (1 of 4)
Inflammatory subglottic/tracheal stenosis, thought to be the result of an incomplete expression of Wegener's granulomatosis (no history of trauma).
Tracheal stenosis (1 of 3)
View from level of vocal cords shows residual lumen of airway at only ~35% of normal. The dotted circle shows how big the lumen should be.
Tracheal stenosis (2 of 3)
Closer view shows the characteristic appearance of mucosa and the inflammatory nature of the stenosis (pinkness).
Subglottic stenosis, before dilation (1 of 2)
This individual has undergone at least a dozen prior dilations, each of which provides dramatic relief from noisy breathing and exercise intolerance. Here the patient is halfway to needing re-dilation, due to the typical inflammatory stenosis that is seen. Compare with photo 2.
Subglottic stenosis, after dilation (2 of 2)
One week after one of this patient's dilations (with Kenalog injection and topical Mitomycin C), showing a dramatic widening of her airway; compare with photo 1. After a number of years, inflammatory lesions such as this sometimes "burn out," and the interval between dilations increases.
Subglottic stenosis (1 of 5)
Middle-aged woman with unexplained shortness of breath and noisy breathing, due to this idiopathic inflammatory and very high subglottic stenosis. The patient initially declined dilation due to her anxiety. She also had granularity of the nasal septum and a positive ANCA profile for Wegener’s granulomatosis.
Subglottic stenosis, worsened (2 of 5)
Five months later, the symptoms became intolerable, and the stenosis was noted to be slightly narrower and with a greater posterior component. The patient agreed to dilation.
Subglottic stenosis, worsened (3 of 5)
Same exam as photo 2; this close-up view shows more clearly the inflammatory nature of this stenosis.
Subglottic stenosis, after dilation (4 of 5)
Five days after outpatient dilation, triamcinolone injection, and topical mitomycin C application. The patient’s symptoms have vanished, the harsh inspiratory noise is no longer heard, and the size of the airway, though still not normal, is more than doubled. Compare with photo 2 of this series.
Inflammatory subglottic stenosis (1of 4)
Middle-aged woman with chronic difficulty breathing for about two years. Initially treated unsuccessfully for asthma, and recently found to have this clearly inflammatory subglottic stenosis. No prior history of trauma or endotracheal intubation. Workup for Wegener’s granulomatosis was technically negative, and biopsy showed the usual “acute and chronic inflammation.”
Closer view indicates a forme fruste of Wegener's granulomatosis (2 of 4)
Closer view, showing the clearly demarcated (dotted line) area of inflammation and narrowing. Normal tracheal rings are seen in the shadow, below the stenosis. Negative ANCA notwithstanding, we believe this is a forme fruste of Wegener’s granulomatosis.
Inflammation on septum (3 of 4)
As is often the case in persons with inflammatory subglottic stenosis of unknown cause, there are some signs of inflammation in the nose, too, usually on the septum, denoted here with “S.” Right side of the septum is subtly abnormal.
Subglottic stenosis (1 of 5)
High-grade subglottic stenosis in a woman diagnosed syndromically with forme fruste Wegener's granulomatosis-related subglottic stenosis. With a narrowing less than 50%, she is very symptomatic. Her breathing is noisy, and her forced inspiration time is about 3 seconds. Compare with photo 4.
Flexible scope (3 of 5)
Here the distal chip flexible scope has passed through the narrowing not only to see into distal trachea, but also as a breathing test. The patient becomes very aware of reduced space and this indicates a marginal airway.
Post-dilation (4 of 5)
A week after dilation, triamcinolone injection and mitomycin C application, the patient says breathing is now normal, and forced inspiration time is only a second in duration--normal. Compare with photo 1.
Planning on periodical injections (1 of 4)
Three months after successful dilation, the patient says breathing is still normal. We are planning an injection of triamcinolone every 3 or 4 months, attempting to lengthen the interval between dilation procedures. Please note that the *s (right of photo) are for comparison with photo 2.
Long-acting steroid (2 of 4)
A needle has been passed into the anterior neck to place submucosal long-acting steroid. Note the bulging and blanching at the *s and compare with photo 1.
Needle going into the posterior of stenosis (3 of 4)
Here the needle has been passed translumenally and into the posterior portion of the stenosis.
View inside left nostril (1 of 4)
This man has Wegener’s Granulomatosis, with sino-nasal, subglottic, and pulmonary effects, and is on immunosuppressive therapy with very good clinical results. This view is just inside his left nostril and our focus – seen better in the next photo – is the stippled vascular pattern sometimes seen in auto-immune disorders. (S = septum, and T = inferior turnbinate.)
Narrow band light (2 of 4)
Under narrow band light, the unusual vascular pattern of both septum and turbinate becomes much more obvious.
Distant view (3 of 4)
In this distant view, his subglottic stenosis looks just like the many other examples in Laryngopedia of forme fruste Wegnener's. The stenosis seen with both entities are visually indistinguishable.
Powerful gag reflex (1 of 4)
The usual method of dripping topical anesthesia into larynx, subglottis, and trachea from above through an Abraham cannula is difficult due to a powerful gag reflex. Here, the tip of a short 25 gauge needle has entered through the cricothyroid membrane, just below the anterior commissure.
Patient inhaling (2 of 4)
2% lidocaine has just begun to squirt through the needle with the patient inhaling.
Patient coughs (3 of 4)
Half a second later, the topical anesthetic is splattering (see streaks) and as the patient coughs. This will fully anesthetize the area topically.