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BackTable / ENT / Podcast / Transcript #161
Podcast Transcript: Idiopathic Subglottic Stenosis Evaluation & Management
with Dr. Stephen Schoeff
In this episode, hosts Dr. Gopi Shah and Dr. Ashley Agan talk to Dr. Stephen Schoeff, laryngologist at Kaiser Permanente (Tacoma, WA), about subglottic stenosis in adults. You can read the full transcript below and listen to this episode here on BackTable.com.
Table of Contents
(1) Recognizing Idiopathic Subglottic Stenosis
(2) Eliciting a History from the Idiopathic Subglottic Stenosis Patient
(3) Physical Exam Findings in Idiopathic Subglottic Stenosis
(4) Labs & Imaging for Idiopathic Subglottic Stenosis
(5) Navigating Treatment Options for Idiopathic Subglottic Stenosis
(6) Steroid Injections for Idiopathic Subglottic Stenosis
(7) Endoscopic Dilation for Idiopathic Subglottic Stenosis
(8) Cricotracheal Resection for Idiopathic Subglottic Stenosis
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[Dr. Gopi Shah]
This week on the BackTable podcast.
[Dr. Stephen Schoeff]
I would say the biggest thing is just it's just to have that suspicion, and it's the same thing across all these different more rare conditions is it's just have this suspicion that there could be like there could be something more to it. Not every globus, not every mucus sensation is reflux. Not every person with some dyspnea is paradoxical vocal cord motion. That there's always this just- to have that suspicion that these are rare conditions, but certainly at least be aware that there's a possibility and be ready to look a little further if you need to.
[Dr. Gopi Shah]
Hi, everybody, welcome to the Backtable ENT podcast. We're a podcast that focuses on all things otolaryngology and we've got a really great show for you today. Thanks for stopping by.
[Dr. Ashley Agan]
We got a great show today. I'll be one of your hosts, Ashley Agan, joined by the lovely Gopi Shah. Good morning, Gopi.
[Dr. Gopi Shah]
Good morning. How are you, Ash?
[Dr. Ashley Agan]
I'm doing great. How are you?
[Dr. Gopi Shah]
I'm doing good. I'm really excited, because we have an awesome guest on today. We have Dr. Stephen Schoefff. He's a laryngologist practicing at Kaiser Permanente in Washington. He's here today to talk to us about idiopathic subglottic stenosis in adults. Welcome to the show, Steve. How are you?
[Dr. Stephen Schoeff]
I'm great, thanks.
[Dr. Gopi Shah]
First, we like to set up the show, just giving you a chance to tell us about yourself, your journey, your practice. How'd you get here?
[Dr. Stephen Schoeff]
Yes. I started out in Ohio. I went to Ohio State undergrad, grew up close to there and then went to University of Virginia for medical school and residency where I did a otolaryngology residency there, and then worked with Jim Daniero there who is a great mentor for laryngology and helped really start my interest particularly in idiopathic subglottic stenosis, and then went on to do fellowship at University of Wisconsin with Dr. Seth Dailey and Dr. Francis. Then from there joined Kaiser Permanente in Washington to build a laryngology practice there in what is otherwise largely a general practice comprehensive otolaryngology setup. Adding a lot of office procedures and a little bit more comprehensive management for voice airway and swallowing for our patients.
[Dr. Gopi Shah]
Just before we get into the discussion, I do want to give a couple of shoutouts to Dr. Jim Daniero. We were residents together at Jefferson. It's really awesome that you got to work with him. He's wonderful. We'll have to get him on the show at some point. I was just recently in touch with him. Then Dr. Seth Dailey also was on Backtable, one of the earlier episodes on why to join societies, and he was super cool. It's something I encourage everybody to check out. Of course, we have to give a shout out to Ally. Dr. Baheti. Ally Baheti is Dr. Steve Schoeff's wife, your wife, who's one of our main hosts on the Backtable IR vascular and interventional show. It's really cool to finally have you on, Steve. I feel like it's been a long time coming. We're super excited. All right. We're talking about idiopathic subglottic stenosis in adults. Tell us, how do these patients usually present to you? Is this a patient that's just going to come to you randomly in clinic? Are you seeing these patients in the hospital or in the ER?
(1) Recognizing Idiopathic Subglottic Stenosis
[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. Gopi Shah]
Are these patients usually younger or older? In Peds, we have somatic stenosis. You think about the NICU baby or child that's been intubated, right? Same risk factors you think of in majority of your adult patients, I would imagine. When I think of congenital, it's such a small, like in a child or an infant, I think it's such a small percentage. Are these kids, do they have a history? Are these patients younger or older when they present and have they had history of like, oh, I had a ton of croup when I was little? Anything like that in terms of risk factors?
[Dr. Stephen Schoeff]
Yes, it's a great question. Usually they don't have anything remarkable in their history. By definition, we're saying that they haven't had any prolonged intubation or anything of that sort in the timeframe before the symptoms started. Interestingly, I've had patients- I've seen patients present sort of de novo all the way from the early 20s to late 70s that they're presenting for the first time. I'm managing a patient who I think we started treating her at 77 or 78 years old.
[Dr. Ashley Agan]
That's a huge age range. What about male versus female?
[Dr. Stephen Schoeff]
That's another great question with this is this is considered really a female disease in terms of a dramatic female predominance. Some will argue that it can only occur in females. However, I have a patient I'm taking care of who is male, who presented with several years of gradual dyspnea and no intubation history and had a very classic appearance of subglottic stenosis. Fortunately for him, the male anatomy, the male airway just tends to be larger. It takes longer to really close off in the way that really restricts somebody, so that they're avoiding a crisis, so to speak. I would say it has somewhere 97 to 99% female predominance.
[Dr. Ashley Agan]
Okay. No particular age is classic, but definitely female presenting with maybe a worsening shortness of breath, eventually stridor, and maybe some other nonspecific ENT symptoms.
[Dr. Gopi Shah]
Some mucus. Add-on- throw on some mucus.
[Dr. Stephen Schoeff]
Mucus is really a hallmark of this, which is really interesting as well. Once you get somebody's airway open, they're often- that's the thing that they notice the most. I would say that there's probably a bell curve in there. I would say, most patients are probably presenting more between- as you go to 30 to 70, 40 to 60, you're probably getting a higher concentration there in that range, that age range.
(2) Eliciting a History from the Idiopathic Subglottic Stenosis Patient
[Dr. Ashley Agan]
Let's think about the patient that hasn't been diagnosed yet. When you're going through your history taking, are there particular questions that you're asking to screen for this diagnosis in particular that's unique or is it your standard list of questions from your laryngologist?
[Dr. Stephen Schoeff]
Yes. I would say, I naturally am more suspicious of it, I would say, than probably the average ENT. I may ask a little bit more about progressive dyspnea, progressive inability to tolerate exercise. I would say the biggest thing about this condition is it tends to move along pretty slowly. Classic timeline is about two years from when somebody starts to have symptoms to when they actually are seeing a laryngologist. Usually by then, their airway is probably 60 or 70% narrowed. The biggest thing for me is that slowly progressive loss of exercise capacity. Because people will describe this, yes, initially, I just couldn't- I used to be able to walk a few miles, then I couldn't walk more than a couple miles. Then now I'm struggling to get up the stairs. I'm having to stop and take breaths as they're talking. Definitely, the people who are presenting in that way, you'll have that clinical suspicion as you hear them talk as well.
[Dr. Gopi Shah]
An adult, do they have sleep disordered breathing or sleep apnea type symptoms as well with this diagnosis?
[Dr. Stephen Schoeff]
Not typically, unless they just otherwise have that as a comorbid condition.
[Dr. Ashley Agan]
With the mucus, is it mostly just like, I've got that post nasal drip or I've got this mucus in my throat that I'm clearing all the time?
[Dr. Stephen Schoeff]
Yes. Yes. Usually the latter. They're going to feel that- just feel like mucus is caught. Really what's happening is that the normal ciliary mechanism is catching on that shell, and there's probably increased inflammation in that area. They're getting a double whammy of mucus that's holding up naturally and then having to deal with this extra inflammatory input. They're just feeling like, I'm always coughing up mucus. They'll often say they can't cough it out, which is I would say that's a little different than the average mucus complaint in an adult. Tends to be more, oh, I've got this postnatal drip and I'm clearing my throat or things like that. That really I have this mucus and I can't cough it out is relatively unique to this population, I'd say.
[Dr. Gopi Shah]
For the patient that's not diagnosed, but they've been dealing with more mucus, increased exercise tolerance. Now it's been going on for about a year. They've seen asthma, they've seen allergy, and they end up in your clinic, but not diagnosed as of yet. What's on the differential? With these symptoms, what else is on your differential usually?
[Dr. Stephen Schoeff]
Yes. Definitely I'm thinking about a number of different things, just the classic globus LPR-type profile. I tend to be on a more conservative side in terms of blaming everything on reflux. I tend to try and avoid that. I try and look for other explanations first, but definitely that is a potential to create similar symptoms. Paradoxical vocal fold motion is a big overlap here that non-infrequently, I do get patients have been temporarily diagnosed with that as well, particularly because they may have PFTs that would show some blunting of the inspiratory flow loop, but not quite the hamburger-type flow loop that when somebody is really, really narrowed in the early part of the disease. Paradoxical vocal fold motion is definitely something that can overlap symptomatically here. You'll see that inspiratory difficulty, maybe occasionally stridor, but that tends to obviously be more episodic. Then of course, there's what I might call like a functional dyspnea, which is just that obviously, at times I've had patients who- their exercise tolerance is just decreasing and it's this cycle where they're doing less and they've gained some weight, and they just can't breathe as well and can't do as much as they used to. Occasionally I am saying, oh, maybe this patient could have a stenosis and we'll do a full airway exam and find it. No, the airway is patent. It's just more that they've been conditioned, unfortunately.
(3) Physical Exam Findings in Idiopathic Subglottic Stenosis
[Dr. Ashley Agan]
Moving on to your physical exam in-clinic, can you walk us through that?
[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.
[Dr. Ashley Agan]
Can we back up just a little bit? When you pack the nose, what's on your pledgets when you're packing the nose, and how long do you let it sit there before you do your bronch?
[Dr. Stephen Schoeff]
Usually it's 4% lidocaine and a decongestant. I think we have phenylephrine in my clinic. I used Afrin previously. Usually we're leaving that for a few minutes. I skipped over. Occasionally some patients, particularly patients that are repeat patients, will do a nebulizer as well or if we have a chance to plan that. I found the nebulizers to be variable. Some patients really like them. Some patients hate it. Some patients feel so numb and gross and it just throws off the whole exam. I actually don't really start with a nebulizer, but that's definitely- can be part of the numbing routine as well.
[Dr. Ashley Agan]
Yes, I agree. I think there's that phenomenon when patients- when everything is super numb and then they just start throat clearing, and there's a little bit of panic and they're not feeling. I think that's really challenging finding the sweet spot.
[Dr. Stephen Schoeff]
Everybody's different. I have patients who love the nebulizer. I have patients who hate the nebulizer. I have patients who feel neutrally about it. It's just like, everyone needs their own secret sauce on numbing.
[Dr. Ashley Agan]
When you're when you're using when you're dripping lidocaine through your channeled scope, is that 4% as well?
[Dr. Stephen Schoeff]
Yes, it's 4%. There's an argument you could use 2% as well. That's typically what I'm using is 4%.
[Dr. Ashley Agan]
Then when you do your injection, say that one more time, what are you injecting and where are you injecting?
[Dr. Stephen Schoeff]
Usually that's 2% lidocaine, it's two or three milliliters. I'll use a 25 gauge needle and just inject the skin a little bit in front of the cricothyroid membrane, and then pop through, aspirate some air to prove that I'm there, and then push through as much as I can before the coughing kicks out the needle. Hopefully getting hopefully getting one or two cc's in there pretty quickly.
[Dr. Ashley Agan]
How long do they cough after that?
[Dr. Stephen Schoeff]
It varies. Usually not more than 10 seconds or 15 seconds. Most patients tolerate that really well. Occasionally, the supraglottis doesn't quite get numb enough there. You have the option of doing an extra transthy or hyoid injection of 2% lidocaine as well. I've used that once or twice if they don't get quite numb enough with that first injection. That just gets the distal superior laryngeal nerve fibers, like if you were doing an augmentation or some Botox injections through that technique.
[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.
[Dr. Ashley Agan]
Once you've spotted that, what happens next?
(4) Labs & Imaging for Idiopathic Subglottic Stenosis
[Dr. Stephen Schoeff]
Usually, ideally, we have a really good exam in-clinic, that we've evaluated the extent of the trachea to make sure this is really isolated to this area, which again tends to be the cricotracheal junction. Then we are going over just the nature of, okay, you have airway stenosis, which is a huge change for somebody, of course, who's coming in largely healthy, thinking that maybe they have- they know something's wrong, but they're trying to figure out what it is. That's a big deal, and just processing that initially with folks is always a first step. Then we start talking about what to do next. I don't necessarily feel like a CT is needed, even though sometimes patients come to me with one. As long as we have a really good exam in the clinic, I don't think a CT is going to add anything. Unless I'm concerned for something else going on or structural abnormality or something like that, which A-frame stenosis or something like that tends to be pretty obvious inside the airway as well. Then in terms of work-up, historically, previously I used to get more labs and I think generally the consensus is that we don't need a ton of lab work to rule out autoimmune disease, in part because many of the labs are so nonspecific. For me now, it's primarily a c-ANCA, and then that'll auto spin off into the MPO and PR3, I believe are the two subtests for GPA. I order that for everybody. To date, in somebody where I did not have suspicion for GPA, I've never seen that be positive, but there's always theoretically that possibility. I don't get ANA and things like that, just because it's not infrequent they're positive, but you send them to rheumatology or communicate with a rheumatologist and they say, what do you do with that? I generally don't, unless I am really suspicious that this is something different.
[Dr. Ashley Agan]
Yes, I've had that experience too. That positive ANA is really common and then you're just like, what are we looking at? What does it mean? Then the rheumatologist is like, nothing, it's not a big deal, why are you sending this patient?
[Dr. Stephen Schoeff]
Exactly.
[Dr. Ashley Agan]
Okay. Cool. Limited imaging, limited labs, because you can-- The exam is the big part, right? Being able to see it. Did you ever have to take anybody to the OR to be able to see the subglottis? Maybe rarely?
[Dr. Stephen Schoeff]
I don't think I've had anybody in practice that I've had to do that. Even if they're really not digging, going down below the vocal cords, we can adjust their position enough that we can get at least some image of the subglottis. I think there's one patient I did do a CT, just because I didn't have a perfect exam. She was pretty resistant to doing some of these more advanced numbing. It wasn't a totally clear history. I said, oh, all right. Let's get a CT scan. Sure enough then, she had some narrowing in the subglottis and she ended up doing really well with some treatment.
[Dr. Gopi Shah]
Because otherwise these patients, when they come to you in clinic, they're sleeping flat at home. It's more exertion induced. Getting a CT for somebody that maybe you didn't get a great exam on or that is really tight should be reasonable, right? Because it's pretty quick. Again, they should be able to lay flat for this.
[Dr. Stephen Schoeff]
Exactly.
[Dr. Gopi Shah]
Do you ever have that? Has that ever come up ever?
[Dr. Stephen Schoeff]
I don't think I've run into trouble there. In general, I keep pointing out this is gradual. These patients are usually not- are very rarely in extremis. The most extreme part of it is that they feel like nobody's listening to them. Then once they feel like somebody's listening to them, usually they're like, okay, well, I can get around the house and things like that, but obviously, they can't do a whole lot. I've seen people who really were fighting through their stenosis and come in with airways narrowed to three millimeters. That they were just going, going, going, because they were convinced that they were- they'd gotten out of shape and just keep pushing it. Yes, they can usually lie flat for CT or lie flat at home at least temporarily.
[Dr. Gopi Shah]
What percentage of your patients or do you think you've seen where they're on a bunch of asthma medications by the time? I feel like asthma, at least with pediatric ENT when it comes to airway foreign bodies, anything like that, that's the great diagnosis that blankets and masks everything until here we are six months later and we have a pneumonia, et cetera, other complications.
[Dr. Stephen Schoeff]
Absolutely. Yes. Usually almost everybody's been through asthma medicines. Usually they say, I stopped because they were not helping. Usually they'll get a couple of different asthma medicines, but then they'll get PFTs and the asthma specialist says, this isn't asthma. You don't have any lung restriction. If it's early enough in the course, they won't see any sort of upper airway restriction. Then again, they feel like they're being gaslit, told nothing's wrong. Sure enough, there is something this whole time.
[Dr. Ashley Agan]
Do you typically see them have exacerbations of their symptoms when they have a cold? If they have an upper respiratory infection, or COVID, or something like that?
[Dr. Stephen Schoeff]
It can definitely happen. Although I feel like it happens a little less than I would have expected, just in my experience. I had one patient who really did- she probably had 40% stenosis, had gradual symptoms and she ended up presenting because she had a really bad exacerbation. She was lucky in that we caught it earlier than average, I would say. Most patients don't get huge exacerbations unless they are really tight. If they're at 70% narrowing, then yes, a cold can tip them over the edge a little bit or really cold air and they start with some crusting, things like that. You can get that additional symptom on top, but I don't find it to happen too much, fortunately.
(5) Navigating Treatment Options for Idiopathic Subglottic Stenosis
[Dr. Gopi Shah]
For these patients, is there medical management? I think of reflux management. Are we throwing PPIs at these patients? What are we doing?
[Dr. Stephen Schoeff]
If you look at some of the papers from the early 2000s, that was the number one theory. That was common across, I think, ENT and laryngology was this idea that reflux was everything. There could be some impact, but I tell you, I've had a lot of patients come in and they're on high-dose PPIs and they tell me it makes zero difference. Reflux could be playing a role in the very initial onset of the condition, but it doesn't have a clear role in the ongoing symptom management. The biggest thing in terms of medical management, a few different things have been tried. I haven't seen a big trial of methotrexate, but I've heard that being tried occasionally, particularly if there's a thought that there's something else going on or just because that is this general immune management. Certainly, people will give these patients oral steroids, big doses of oral steroids. The patients I've seen who've been on oral steroids, unless they're in that acute, I had a cold and I had acute inflammation, don't seem to get any benefit from oral steroids in my experience. I've seen them used as a “temporizer" as you're getting somebody to the OR. I don't think patients seem to feel anything with that, other than they just maybe feel a little euphoric on their steroids. Then the other part of medical management gets into the Mayo Clinic's triple therapy approach after they do endoscopic treatment. It may be something we can get into a little better later once we've talked about some of the other interventions, but they do this post-intervention medication strategy.
[Dr. Gopi Shah]
What's your decision cutoff, like what's going to push you to recommend an intervention versus you know what? You have a little stenosis, you're doing okay. Because there's such a variable degree of symptoms, it seems like.
[Dr. Stephen Schoeff]
Exactly. Most patients, by the time they get to me, they are pretty symptomatic. That's a big part of it. That's a difference again between some of the iatrogenic type stenosis and things like that, because those can just settle at 20%, 30% sometimes, and those patients aren't that bothered by it. A lot of times, by the time I'm seeing an idiopathic patient, they're quite symptomatic. Again, not in extremis, but quite symptomatic. My general algorithm is, to everybody I meet, I start to talk through all the options. The standby operation to treat this for the last 30 or 40 years, this condition was first described in the '70s, but I think really in the 2000s is when it's really been clearly recognized for what it is. To start by just saying, well, the standard is operative endoscopic dilation. When you look at data, that probably lasts one to two years for most patients to keep them pretty open, and then they're coming back and wanting another dilation. Half of them will start there. Usually you're going to do a steroid injection there, and everybody's technique is different. Some people use lasers to make radial cuts. Some people use cold instruments. Some people use cryo. There's all kinds of things out there that people use to make the cuts. I'm of the opinion that there's probably not huge variation between those. We start there and say, that's pretty standard. I think that many people would offer that. Then we talk about what's the most definitive option. I think the data support the idea that the most definitive option is cricotracheal resection, which is when patients who, again, coming in with this background of being pretty healthy and things like that, you start talking about major airway surgery, they're not quite ready to go for it. We talked through the pros and cons of that. the best data suggests that it's quite durable. There's some anecdotal reports that maybe eight, 10, 12 years down the road can come back, but that hasn't really been published. That's something that's still out there in the ether as a little uncertain, but it's quite durable. The big drawback is that usually the pitch of the voice lowers, because of detaching the cricothyroid muscles. There's some variations out there that have been proposed, but generally, it's still seen as pretty likely to lead to a lower pitch voice, which for a woman in her 30s or 40s is a quite undesirable outcome. That's a big part of that consideration. Then when I talk about the third part of the management that I often will share with patients, which is steroid injections in the office being called different things, but I think we've all come down to this, SILSI, Serial Intralesional Steroid Injections, is the easiest way to describe it for the literature and for patients. Those can be delivered quite easily, again, with some of the similar techniques we were talking about in terms of numbing the airway and then either injecting through a working channel scope or through- transcutaneously. I'll bring up these three options with a couple caveats in there. There are some variations of endoscopic procedures like laser excision and a Maddern procedure that's out there as well. We can get into those a little more, but basically get these three broad approaches to management that I'll start with, and then start to discuss with the patient where their priorities are, what they feel like is better for them to start out with.
[Dr. Ashley Agan]
For your patient who is not ready for a procedure, it sounds like most of your patients are at that point, but let's just say there's somebody who's like, whoa, I just want to wait and watch. What's the natural progression of this? Do most people tend to have a progressive course in idiopathic subglottic stenosis to where you would expect that eventually they're going to come back and need something? Do some people stabilize at a certain stenosis that can be tolerable?
[Dr. Stephen Schoeff]
That's a great question. I think we still don't have that answer exactly. I've not had a patient elect observation.
[Dr. Stephen Schoeff]
That's across training and practice. I don't think I've seen someone elect to do nothing. The general sense is this will progress. There probably is some point at which it may stop for many patients. There are rare reports of patients with this condition dying from acute plugging events. It's certainly not completely, there's not a guarantee that it's going to stop in a way that would be nonfatal. I would bet that for many patients, it may get to a four millimeter airway and then just by the nature of air passing through, it may stop. I can't say for sure. I generally advocate doing something. Most patients-- I've never had a patient where I had to convince them to do something.
[Dr. Ashley Agan]
Right. They're coming to you for a solution. Exactly. Your three options are steroid injections, a dilation, or a cricotracheal resection. Those are the three big categories.
[Dr. Stephen Schoeff]
Those are the three big categories. Exactly. Within that dilation discussion, I'm usually offering an endoscopic laser resection of tissue, plus or minus dilation is where I've settled. That's based on some data that's been produced particularly out of Mayo as well as the North American Airway Collaborative Comparative Outcomes. I generally am offering a laser resection if we're doing an endoscopic operation with steroid injection. If somebody's the occasional patient I get who's only at 40 or 50 percent stenosis or even slightly less, if we're really lucky, I will offer them to start with steroid injection in the office with a biopsy done in the office just again to rule out autoimmune disease. Everybody gets a biopsy, in my opinion, just to make sure. I've had one and maybe two patients where we started with that approach and were able to avoid the OR altogether. She had a really good response to that.
(6) Steroid Injections for Idiopathic Subglottic Stenosis
[Dr. Ashley Agan]
That's interesting that steroid injections are helpful, but oral steroids aren't helpful at all. Why do you think the concentration that you're able to deliver with an injection is just so much higher or any thoughts on that? Because we love giving steroids at ENT. To hear that steroids don't work is like, what?
[Dr. Stephen Schoeff]
It's a really interesting question. There's definitely a debate within our field. I'd say most laryngologists I know are using these, but there's a few and a few very prominent laryngologists that are pretty skeptical. There's a couple of factors. One of which is what's the underlying problem? What's going on here? Dr. Gelbard at Vanderbilt has led a lot of the high-level basic science research into this. I don't want to ignore his colleagues there, but he's the person who's, I think, spearheading it right now. He's shown that there is this lack of epithelial integrity, some invasion by bacteria. There's a little bit of a chicken or the egg question, what starts the process? We're observing the process after it started. What's going on there? Ultimately, you end up with this inappropriate fibroblast process as well that's laying down scar where you shouldn't be laying down scar and an alteration of the immune response in the sub-epithelial tissue. The steroid injection side, how that addresses it, the theory from what I understand, came from what we see in keloids and hypertrophic scar and approaching those. Dr. Franco and Dr. Dailey have both been big early adopters. Dr. Franco was the first person to describe it. Then Dr. Dailey also has been an early adopter of the steroid injections in the office and seeing really good results with these since about 2014 or so. Dr. Franco recently presented, I believe it was a poster actually, but some basic science showing that the fibroblast activity actually has changed with steroid injection. I don't think we get that from oral steroids or at least we can't give oral steroids long enough to achieve that response. Basically a fibroblast activity is changing from laying down scar to actually removing scar. That's hugely valuable, of course, when we're talking about really just needing a few millimeters of airway and needing some improvement in that scar deposition.
[Dr. Ashley Agan]
For your steroid injections, whether you decide to do initial treatment of the serial injections or at the time of your endoscopic dilation in the OR, what steroid do you use and how much do you end up giving? Do you have a special needle you like?
[Dr. Stephen Schoeff]
Yes. I'm using Kenalog 40. I think that's pretty standard across the field. We like that deposition. Of course, that's what I think a lot of facial plastic surgeons and folks have used in keloid as well. I use that just with a-- There's an Integra laryngeal injector in the OR. Usually I'm removing the tissue and then injecting steroid, and then possibly dilating a little bit after that just to make sure the airway is completely open for somebody coming out of anesthesia. Then in the clinic, I'm using either a 25-gauge needle trans-cricothyroid with a physician assistant driving a camera or I'm driving a working channel scope with a sclerotherapy needle through the channel that is, I believe, a 20, 23-gauge that we're getting into the tissue subepithelially and infiltrating with the Kenalog.
[Dr. Ashley Agan]
You just try to do several little injections around the edges of it?
[Dr. Stephen Schoeff]
Exactly. Yes. Just finding places where there is more scar deposition, where there's more tissue and filling that tissue. I generally aim to do about one milliliter, plus or minus. It just depends. Patients who have minimal stenosis, we're oftentimes doing less than that. Then usually it's done in a series. If somebody's starting out with treatment that way, we're doing it in a series of three or four injections over the course of three or four months. Oftentimes after somebody is taken to the OR, I offer a series of injections afterward as well. There's evidence that helps to improve the interoperative interval. For some patients, it really keeps them out of the OR entirely. We had a good number of patients at University of Wisconsin who stopped going to the OR with this approach. I've had that experience as well, taking over practice from a surgeon who is treating patients with dilation every six to 12 months or so based on their symptoms. A number of them have switched to steroid injections and it's been three plus years that I've been here and they've never had to go back to the OR. Some patients respond to it beautifully. Not everybody does. We still don't quite have a handle on why those patients who don't respond to it really well don't. Certainly there's some variability in that.
[Dr. Ashley Agan]
It's every- once a month for three to four months?
[Dr. Stephen Schoeff]
Correct. Then after that, and this is where I think there's a lot of variation, I think a lot of laryngologists are using that approach if somebody elects endoscopic treatment and doing this postoperatively. I think there is quite a bit of variation around the country in terms of how we approach it after that. Whether somebody- folks are followed or folks are told just to come back if their symptoms start to worsen again. There's, I think, a huge variability in terms of how that practice is approached.
[Dr. Gopi Shah]
Meaning, come back, we'll be scoping you every three to six months until we need to intervene versus just come back and see me if you're getting the mucus and having more dyspnea and exertion and your original symptoms that you had.
[Dr. Stephen Schoeff]
Exactly. There are a lot of different approaches, as I've said. Some folks will use PFTs. Some people will use a peak flow meter. I like to use a peak flow meter, because it's so simple and can approximate for a patient if they see a decline in their peak flow out that they know that can corroborate their subjective symptoms, and it's cheap. Then classically with the dilation approach, a lot of patients would get a dilation and just be told, okay, when you start to feel tight again, give me a call. We'll do another dilation. Whereas I tend to follow- offer to patients to come back in and starting out, coming back in a little more frequently, three months. I have some patients who have found that if they come in every six months and get a steroid injection, they're pretty good. They're thrilled because they were getting dilations every nine to 12 months. Now they're getting a steroid injection every six months. Between those injections, they still feel good. Whereas with the dilation, after a couple of months, they were just biding time until the next dilation.
(7) Endoscopic Dilation for Idiopathic Subglottic Stenosis
[Dr. Gopi Shah]
Can we talk a little bit more about the dilation technique? You're doing this in the OR. Is this with general anesthesia using rigid bronchs and suspension or is the patient still awake? You're doing topical, flexible scopes. How do you do it?
[Dr. Stephen Schoeff]
My approach is to do this under general anesthesia with paralysis, rocuronium paralysis on board as well, but we do this graduated approach. I've got a team there that's gotten really comfortable with these cases as well. We have a great team set up, and we start with induction, just using propofol, getting the patient quite deep, ensuring that they can be masked safely. We always have that ability to back out. Then we're giving rocuronium paralysis to allow for rigid instrumentation. Then I go in with a Dedo typically, or sometimes a smaller laryngoscope, depending on the patient, and start supraglottic jet ventilation. Just running a jet through the sideboard of my Dedo. From there, we actually use an oxygen mixture so we can usually ventilate with jet at 30% while removing tissue with the laser. We don't actually have intermittent apnea, but sometimes patients don't tolerate that as well, particularly patients who are more obese, for example. We will either do intermittent apnea with ventilation at 100% or just open the tissue some with a laser and or a balloon dilation and then intermittently intubate with a 502 through the laryngoscope.
[Dr. Ashley Agan]
What laser do you like to use?
[Dr. Stephen Schoeff]
I use a CO2 laser with an AcuBlade pattern generator, usually typically a line for that. Using the UltraPulse mode, there is some evidence that the continuous mode laser does have a higher risk of edema and tissue injury that could create a postoperative issue. Fortunately with the UltraPulse, I've not encountered that.
[Dr. Ashley Agan]
Rather than just doing radial incisions, you're actually removing the scar as much as possible.
[Dr. Stephen Schoeff]
Yes, exactly. It's, again, a protocol that has been described at the Mayo Clinic. A number of surgeons there have been using it for years now where they essentially use a radial cut to identify the depth of the scar and then using a laser to remove that scar tissue as much as possible in this wedge fashion. Usually leaving a small wedge of tissue in between, usually three or four tiny little bridges in between with the idea that a complete circumferential excision would create risk of encouraging scar formation.
[Dr. Ashley Agan]
Got you. Then after you've removed these small segments, then you inject those areas with your steroid.
[Dr. Stephen Schoeff]
Exactly. When you're doing the laser excision, I don't know how much the steroid does at that time. I think it's more beneficial in the postoperative setting. Typically, I'll inject steroid at that point. If I feel like it's still creating a little bit of a blab in the tissue or that the patient might feel restricted at all coming out of surgery, then I'll do a balloon dilation, or for some reason I'm just finding that I'm just not able to get as much tissue out as I'd like to.
[Dr. Gopi Shah]
Then in the OR, what red flags should we keep in mind for an airway emergency or do you always have a trach tray available or you said, hey, we mask them down, because if we can't mask them, we at least have the opportunity to back out. Can you tell us a little bit more about some of those things that we should be thinking about?
[Dr. Stephen Schoeff]
Yes, absolutely. I always have a trach tray in the room. I don't typically have it open unless I have a particular concern about a patient. Obviously we have the preoperative exam to have a sense of how narrow the patient is and then considering other anatomic features in terms of their ability to be masked. If somebody has significant adiposity, cervical adiposity, or large tongue, OSA type features. Knock on wood, I've not actually encountered the patient that I can't mask, which is really good. Realistically, the vast majority of these patients are going to be able to be masked unless they're really, really narrow or crusting in some way. There is the option of salvaging with an LMA. For some reason, oral or oropharyngeal structures are giving you trouble with the mask. You can put in an LMA. If you have trouble with rigid exposure, that's really I think, the big next branch point. There's going to be the occasional patient that just cannot be exposed with rigid exposure with any laryngoscope. I've run into that once over my career. That patient can often be treated, though, with an LMA and then using a flexible scope. You can use a laser fiber, but at least can get a balloon dilation done that way, and steroid injection. That's really how our pulmonology, interventional pulmonary colleagues treat most patients as well, of course. they'll do some rigid bronchoscopy, but that's the bread and butter for most interventional pulmonology treatments. That's a very viable option. I always talk through with my anesthesia team what our plan A is, and then plan B, plan C, and then plan D or E is always tracheostomy. We try and avoid that.
[Dr. Gopi Shah]
What about post-procedure instructions? You had mentioned the Mayo post-op protocol.
[Dr. Stephen Schoeff]
Yes. They've done this so-called triple therapy, which is Bactrim, proton pump inhibitor, and steroid inhaler. I offer that to every patient that I treat. I haven't had anyone take me up on it, at least all three. a lot of it's probably how you couch it or how you sell it. For me, I probably don't push it really hard. I saw a presentation from the Mayo team that showed that probably the Bactrim is the most influential. As I understand, they backed off prescribing proton pump inhibitors if patients don't have overt reflux or pH study proven reflux. The Bactrim is probably the most impactful, which makes sense with what we've seen in terms of translocation of bacteria. That was based on, I believe, some patients with GPA responding well to Bactrim. In Western Washington, there is not enthusiasm for long-term antibiotics. It's culturally, there's a lot of enthusiasm for alternative medicine out here, but not a ton of enthusiasm for big doses of pharmaceuticals. I think being driven a part of it, I've had a couple of patients elect Bactrim, but not frequently. Again, I'm not telling them that this is the way to go and this is the absolute only options. There's always that element of it, and you can get into that in terms of which patients are electing cricotracheal resection or these different options, too. a lot of it is how it's counted by the surgeon probably, and our bias is one way or the other in terms of what we think is the better option.
[Dr. Ashley Agan]
Is that protocol just for patients who are having like a dilation or an excision of the scar, or does that apply to the intralesional steroid injections or not really?
[Dr. Stephen Schoeff]
In theory, it could apply to either. It's been called endoscopic resection with medical therapy, I think. ERMT or EMRT. I hope I don't upset anybody over there if I'm missing some of the details here of their protocol. They designed it where they do an endoscopic resection and then put patients on medical therapy. There is some evidence that people get longer interoperative intervals that way for sure by a reasonable margin, particularly compared to dilation alone.
[Dr. Ashley Agan]
How long are they on those medications?
[Dr. Stephen Schoeff]
My understanding, indefinitely. Again, that's where I don't have a lot of patients who are ready to sign up for that.
[Dr. Ashley Agan]
Yes. I could see how that would be a hard sell. Any other post-procedure instructions that are important for you in particular or are there any supplements that your patients take that are found to be helpful?
[Dr. Stephen Schoeff]
Number one is the acute post-procedure. What are the things that could be really bad? When you're doing jet ventilation, there's a theoretical risk of pneumothorax. That's been described in the literature, and certainly I've heard of it happening. If somebody has-- I don't check an x-ray after every case. Personally, I know there are some surgeons who do. My experience has been that it's very rare for that to happen. Unfortunately, in my practice, I haven't observed it. Encouraging people, if they have dyspnea, if they're feeling really short of breath, that's a red flag. If they're in PACU and they're feeling short of breath, that's a problem. They should be breathing a lot better than they were. I check on patients, make sure that they are breathing better, meaningfully better than they were. If they're not, then we need to evaluate that. The other thing would be subglottic or laryngeal edema. Again, you'd be hearing-- They might come out of the OR with nothing, and then if the PACU says, hey, they're starting to get more stridor, they're starting to sound worse, that's a big red flag, and you need to be evaluating that and looking for swelling. The vocal folds are probably the bigger risk in that situation that you get glottic edema. In rare scenarios, that would mean reintubation, or ICU monitoring, and ideally avoiding the tracheostomy, if at all possible, but that's always your final pathway. Those are the big things in the acute, immediate, postoperative. Some surgeons will give patients prednisone bursts. If I feel like there's a little bit of laryngeal edema, but nothing significant, I may give somebody a short prednisone taper, because that's certainly laryngeal edema response to prednisone. No question. Then otherwise talking to folks about management of that mucus sensation, usually Mucinex and acetylcysteine. A couple of patients have actually elected a saline nebulizer, nebulized saline that helps to clear out that mucus and vent out the mucus. Personal steamers, all these different mucus options can definitely help with some of the symptom management. Then, like I said, I'm following the patients pretty closely. Usually I'm meeting them again within a month from surgery. Most patients elect to at least try steroid injections. Most of the time if we're doing an endoscopic procedure, I'm meeting the patients about a month after surgery.
[Dr. Gopi Shah]
For these patients, whether at initial presentation or post-op, voice and swallowing is usually fine. This is more of a breathing issue, yes?
[Dr. Stephen Schoeff]
Exactly. Some patients will have some involvement of the infraglottic vocal folds. Some patients do experience this as hoarseness, worsening symptoms. Swallowing would be very rare to have any issues there. The vast majority of the time, it's going to be a breathing issue.
(8) Cricotracheal Resection for Idiopathic Subglottic Stenosis
[Dr. Gopi Shah]
Just transitioning to CTR and open airway. Are there certain hard indications for CTR? Is there a certain amount of stenosis or, hey, now we're in the OR every two to three months? When do you start to that, hey, we're here now?
[Dr. Stephen Schoeff]
Yes, that's really driven by the patient scenario. I think some patients are very interested in that upfront. I would say it's a minority, but some are. Then I don't know that there is necessarily a cutoff, but certainly if patients are just needing constant, really frequent interventions, then you're really thinking about some of these more aggressive surgical options, whether that's a CTR or this what you might call an endoscopic CTR, which is the Maddern procedure that's mainly been developed at Cleveland Clinic. We're going in with a micro-debrider actually and removing the scar, and micro-debrider and then putting in a stent with a graft, either split the skin or a buccal mucosa to try and reline that tissue. It's a middle ground between the options, so to speak. They just published a series of almost 30 patients or so that have had pretty good results on average. That's definitely something that's out there as a potential middle ground between cricotracheal resection and the associated risks and potential issues there and the endoscopic options.
[Dr. Gopi Shah]
Do you ever have patients where tracheostomy is the best option? Who are those patients?
[Dr. Stephen Schoeff]
Occasionally there are patients who are just fed up with it, are either considered too high risk. Diabetes is always considered a big risk factor for cricotracheal resection. Not in my current practice, but I have seen patients who either had a mucus plugging event and presented emergently and gotten a tracheostomy or were in a place where there wasn't somebody who could do jet ventilation and things like that. I just found that once they got used to a tracheostomy, they said, oh, this is actually easier than going back to the OR, and things like that, and actually found it to be pretty manageable. I have seen a couple of patients who actually, ultimately prefer, or at least have elected just to use a tracheostomy and bypass the issue.
[Dr. Gopi Shah]
Sounds like that's the minority though.
[Dr. Stephen Schoeff]
Correct. Yes, absolutely.
[Dr. Gopi Shah]
When you think about your patient population, specifically for the idiopathic folks, what's more common as far as procedure wise, do you feel like excising the scar and doing the dilation is the most common road? I feel like just from an outside looking in, that seems to be less invasive than, the cricotracheal resection, but also more aggressive than just an injection. I don't know. What do you think?
[Dr. Stephen Schoeff]
Yes, that's been my experience. I inherited some patients who had been previously treated with dilation alone. Then if we needed to go back to the OR, they've been interested in adding the laser resection. Then, again, surgeon biases play a role here. I hope for the better, not for worse, but surgeon biases certainly can impact how patients, what they elect. Many of my patients have elected steroid injections in the office as an adjunct to endoscopic procedures. Many of them have been really happy with the change in experience. all the patients that have continued to elect them certainly have with the change in experience from this gradual decline that they experience going into repeat dilation versus being able to stay open. Even when those patients that are getting steroid injections, even when they do say, I feel like it's tight enough that I just want to go to the OR, they're going to the OR at 40%, 50%. They're not going to the OR at 75% or 80%, where they're barely making it in. That's been a big impact in the positive for most of the patients I treat.
[Dr. Ashley Agan]
Do insurance coverage questions come into it? Are patients pretty much free to select whatever pathway works best for them or sometimes is there a discussion of coverage that has to come into play that also, dictates what happens next?
[Dr. Stephen Schoeff]
That's a great question. For me in the environment I'm in, it doesn't come up as much since I'm in an HMO capitated model. That's a big question for patients who are, say, on a high deductible plan. I would think in that, if they're doing steroid injections frequently on a high deductible plan, they're probably going to hit their high deductible every year. That's going to be really cost intensive. we see the same thing for Botox injections and things like that too, that one of these injections could bill a couple thousand dollars to somebody on a high deductible. That will definitely impact their decision making. I'm not aware of insurance really blocking these, but again, I'm fortunate in that I'm in a model where we don't have as much of that back and forth. I certainly see our message boards and know that that's not always the case. I feel fortunate in that regard.
[Dr. Ashley Agan]
When you think about the procedures, there's probably different costs when you think about going straight to a CTR, which is probably more expensive upfront, but maybe less expensive over time versus needing to have serial dilations versus serial injections. In a HMO capitated model, eventually there may be pressure to steer patients towards a more cost effective option. I don't know. I'm just talking out loud.
[Dr. Stephen Schoeff]
I think that's a great question. Kaiser Washington is relatively small compared to like Kaiser Northern California and Southern California. Those laryngologists, they certainly have not been pressured one way or the other. I talk with them a bit and a lot of them are using steroid injections and some combination of endoscopic management, occasional CTRs. I don't, that's a really interesting big picture question when you get into particularly managed care and optimization of, health outcomes versus cost of the system. Fortunately at some level, this is such a rare condition and such a, if you're talking health system wide, drop in the bucket.
[Dr. Ashley Agan]
Yes. It's a small patient population.
[Dr. Gopi Shah]
Yes. As we start to wrap things up, Steve, any final pearls that you want to leave our listeners with or thoughts on the topic?
[Dr. Stephen Schoeff]
I would say the biggest thing is just, it's just to have that suspicion. It's the same thing across all these different, more rare conditions is just have this suspicion that there could be something more to it. Not every globus, not every mucus sensation is reflux. Not every person with some dyspnea is paradoxical vocal fold motion. Just to have that suspicion that these are rare conditions, but certainly at least be aware that there's a possibility and be ready to look a little further, if you need to. I had a colleague I was talking to and I was saying, gosh, I just wish people would just inject lidocaine into the airway more often. He was like, what are you talking about? That's not normal. I was like, well-- But there were times where just getting the airway a little more numb and actually taking a really good look can diagnose something that can really help the patient. Again, the earlier we find this, the more options patients have, the more they can be treated effectively and not have to suffer through this horrible progression and feeling like they're not being heard.
[Dr. Gopi Shah]
Thank you so much for coming on Steve. It was wonderful to have you.
[Dr. Stephen Schoeff]
Absolutely. Thank you so much for having me.
Podcast Contributors
Dr. Stephen Schoeff
Dr. Stephen Schoeff is a laryngologist at Kaiser Permanente in Tacoma, Washington.
Dr. Ashley Agan
Dr. Ashley Agan is an otolaryngologist in Dallas, TX.
Dr. Gopi Shah
Dr. Gopi Shah is a pediatric otolaryngologist and the co-host of BackTable ENT.
Cite This Podcast
BackTable, LLC (Producer). (2024, March 5). Ep. 161 – Idiopathic Subglottic Stenosis Evaluation & Management [Audio podcast]. Retrieved from https://www.backtable.com
Disclaimer: The Materials available on BackTable.com are for informational and educational purposes only and are not a substitute for the professional judgment of a healthcare professional in diagnosing and treating patients. The opinions expressed by participants of the BackTable Podcast belong solely to the participants, and do not necessarily reflect the views of BackTable.
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