Identifying Idiopathic Subglottic Stenosis: Signs & Symptoms

[Dr. Stephen Schoeff]
That's a great question. At some level, it's an all the above sort of answer, but idiopathic patients really do tend to present a little differently than some of the other causes of subglottic stenosis. The classic subglottic stenosis that most non-laryngologists think about is going to be more of your traumatic or iatrogenic subglottic stenosis. You're likely to encounter that patient in the hospital. They've been intubated for a while, or they've had a tracheostomy and then they have an A-frame deformity. A lot of times they're going to be in these higher acuity settings and a more rapid onset of symptoms or persistence of symptoms. Whereas, the idiopathic patients tend to get shuffled around quite a bit. They often are otherwise pretty healthy. They're showing up with some mild stridor, potentially, but often not even stridor at their first presentation to someone where they're presenting, say, to their primary care provider with increasing mucus in the throat, which we all know is a super common complaint, or increased cough, maybe slightly shorter breath, but pretty gradual, pretty slow onset. That's part of what makes it such a challenging condition is that they oftentimes get managed with some OTC meds, or maybe acid reflux meds, and albuterol inhaler and they're not getting better. Oftentimes, they'll end up actually getting sent over to allergy or pulmonology first. Then finally, the stridor becomes more noticeable. That's oftentimes when they may either end up in an urgent care or emergency room setting or in the ENT clinic.

[Dr. Ashley Agan]
Do most of your patients already have the diagnosis by the time they get to you?

[Dr. Stephen Schoeff]
Yes, I would say a good portion come to me with a diagnosis. Either they've been into an urgent care or an emergency room and are getting a scan, a CAT scan that might show it, or they're being seen by an ENT consultant, or getting into a general ENT clinic where a careful, flexible laryngoscopy exam might show it. It's also quite possible for these patients to get into an ENT clinic and say just a standard flexible laryngoscopy, it might not be evident. If the stridor isn't particularly noticeable, it can even be missed in that setting as well. I would say the majority of patients have at some point gotten a diagnosis before they end up in my clinic. Probably about, I don't know, I'd say 85 or 90%. Occasionally, I do get patients where they've had stridor, they've had dyspnea for a while and end up in my clinic and we're finding it for the first time.

[Dr. Stephen Schoeff]
Yes. Obviously starting out with just a routine head-neck exam. I feel like I'm answering the boards.

[Dr. Stephen Schoeff]
But routine head-neck exam. We didn't get too much into autoimmune disease yet, but that's always the overlap there is the potential for autoimmune disease. Looking to see is there a septal perforation, major crusting in the nose, any fluid in the ears, things that make you think of GPA, granulomatosis, polyangiitis, the former Wegener's, and sarcoidosis, a couple other- those can be the big ones. Then listening to the patient breathe, having them do forced inspiration. A rapid through the mouth inspiration can definitely help you to hear whether or not there's that core stridor. Then moving on to some combination of laryngoscopy or an office bronchoscopy. If I know coming in that somebody has stenosis on a CT, we're going to be set up already to plan a bronchoscopy in the clinic. Usually I'm just using a channeled scope where we're going to really spray their nose well, get them a little bit set up. Particularly if they have a small nose, we'll pack the nose just because the camera is a little bigger. Then we will go ahead and dribble some lidocaine onto the vocal folds with sustained phonation, and then that allows me to do a full airway exam down into the trachea and even see the main stem bronchi. In the patient where I don't know yet, say they just get sent to me for a nonspecific complaint, but I'm suspecting that and say I'm not set up with a channeled scope and for whatever reason don't have the ability to switch over to that, sometimes I'll just use transcutaneous, transcricothyroid injection of 2% lidocaine. Just prep them, discuss, tell them that I'm suspicious of something deeper in the airway and that it'll feel a little uncomfortable at first, a little injection of lidocaine and make them cough quite a bit at first to get the lidocaine all through the airway. Most patients actually tolerate that really well in terms of numbing the airway and then you're able to do a full airway exam. With that as well to see the subglottis and the distal trachea even with a normal or just a regular flexible laryngoscope.
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[Dr. Gopi Shah]
Then on the scope exam, when a patient does have idiopathic subglottic stenosis, what are you looking for? What does it look like? How does it look different than the patient who has had an intubation history or a reason for subglottic stenosis?

[Dr. Stephen Schoeff]
Oftentimes this idiopathic subglottic stenosis has a fairly trademark look, I would say, and it tends to be this almost spiral pattern to it. The usually slightly red tissue that's spiral. It may be eccentric, it may be concentric, you may have a pretty midline circle, but it may be that the left side is pretty involved, but the right side is less so. The main differences, I would say, that I feel like I see with idiopathic versus say an iatrogenic, which fortunately in my practice, iatrogenic subglottic stenosis is quite rare, that tends to be a thicker appearing scar. Usually doesn't have this spiral pattern to it. It's just usually concentric, thick. It looks more dense and may or may not be located right in this cricotracheal junction area, but obviously can vary a little bit more. Of course, you have the history piece of I was in the ICU for the last six weeks from those patients as well. Then the other one that I would say could be a little harder to differentiate is the autoimmune disease. I would say the patients I've seen who present with GPA that's otherwise been diagnosed, which is going to be by far the most likely thing to present in the subglottis, those patients, it looks horrible. It is extremely red, usually there's a lot of crusting. Oftentimes the vocal folds will be inflamed, and it just looks way more inflamed than I would say the average idiopathic patient looks. They may have a little bit of crusting, but usually it's dramatically more inflamed in somebody who's presenting particularly with a new diagnosis of GPA and even some of these other autoimmune conditions that may affect that area. I've seen lupus affect the subglottis as well, and just tends to really show up with a more significant inflammatory appearance.