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Podcast Transcript: Allergic Fungal Rhinosinusitis

with Dr. Amber Luong

In this episode of BackTable ENT, Dr. Shah and Dr. Agan speak about allergic fungal rhinosinusitis with Dr. Amber Luong, vice president of the American Rhinology Society and professor of otolaryngology at McGovern Medical School. You can read the full transcript below and listen to this episode here on BackTable.com.

Table of Contents

(1) Defining Allergic Fungal Rhinosinusitis (AFRS)

(2) Diagnosing AFRS & Regional Differences

(3) Clinical Presentation of AFRS

(4) Imaging for AFRS

(5) Distinguishing AFRS from Other Sinusitis Using Labs

(6) The Role of Steroids in AFRS Treatment

(7) Strategies for Surgical Success

(8) Post-operative Management of AFRS

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Allergic Fungal Rhinosinusitis with Dr. Amber Luong on the BackTable ENT Podcast)
Ep 73 Allergic Fungal Rhinosinusitis with Dr. Amber Luong
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[Dr. Gopi Shah]
This week on the BackTable podcast.

[Dr. Amber Luong]
I think it's those extreme patients that are the most challenging to clear of allergic fungal. Luckily, the kids often, or in my experience, more often than the older ones present with unilateral disease. If you can get that unilateral disease and you can do a really good job of clearing all the mucin, then those patients do really well. It's those, the ones that I remember over the years, the really expanded sinuses, the crazy CT scans, those are really difficult because it is so challenging to get all that mucin out because it's these unusual crevices, these areas where our instruments are not intended to get to at that depth. I think that those end up, and because of maybe how long it's been going on, those are really sometimes the most challenging.

[Dr. Gopi Shah]
Hello everyone and welcome to the BackTable ENT podcast, where we discuss all things ENT and we bring you the best and brightest in the field with the hope that you can take something from our show to your practice. Now a quick word from our sponsor. Cook Medical's Otolaryngology-Head and Neck Surgery Clinical Specialty strives to provide otolaryngologists with minimally invasive solutions to address unmet needs. Areas of focus include head and neck, otology, and laryngology, with products ranging from a full suite of interventional for endoscopy products and the Doppler blood flow monitoring system to the BioDesign Otologic Repair Graft and the Hercules 100 transnasal esophageal balloon. For more information, visit cookmedical.com/otolaryngology. Now back to the show. Good morning everyone and welcome to the Backtable ENT podcast. My name is Gopi Shah and I'm at Pediatric ENT here with my partner, Dr. Ashley Agan. Good morning, Ash.

[Dr. Ashley Agan]
Hey, good morning, Gopi. How are you?

[Dr. Gopi Shah]
I'm good. I'm good. I'm really excited for our guest today because I fangirled our guest at COSM. I was like, Dr. Amber Luong, Dr. Amber Luong, my name is Gopi Shah. I'm at Pediatric ENT. My God, I've listened to you and I'm really excited. I have this podcast. Can we please do it? She was kind enough to email back and respond. We have Dr. Amber Luong, MD, PhD. She is an otolaryngologist and rhinologist, a true surgeon-scientist. She serves as a professor and vice chair for academic affairs in the Department of Otolaryngology at the McGovern Medical School of the University of Texas Health Science Center at Houston with a joint appointment within the Center of Immunology and Autoimmune Disease at the Institute of Molecular Medicine. She currently serves on the board of directors and will be starting her tenure as the second vice president for the American Rhinologic Society in the fall of this year. Welcome to the show, Amber. Good morning.

[Dr. Amber Luong]
Good morning, Gopi. Good morning, Ash. Thank you for having me. Go women power!

[Dr. Gopi Shah]
Absolutely. Before we get into it, I wanted to just say I love the ARS. I love the women in rhinology subsection of the ARS.

[Dr. Amber Luong]
Oh, thank you.

[Dr. Gopi Shah]
Yes. You were part of starting that group. Yes.

[Dr. Amber Luong]
Yes. Gosh, it was about 10 or so years ago now. There was a group of us that we went to a happy hour and we were, sitting around and thinking, what a great opportunity because now we've got like this critical mass of women who are interested in rhinology. We had a lot of the common issues that we have with our men colleagues, but there are some unique aspects of being a woman in rhinology. We thought it'd be nice to get together. We developed these women in rhinology. I think it modeled after the women in otolaryngology, but it's sort of taken off in several different societies as well. It's nice to see, I think the otology group is developing one and the pediatric group may be so. It's been really a fun opportunity and a great group of women.

[Dr. Gopi Shah]
I'm getting emails from Patricia Loftus. She helps organize it. Every time I see an email from Patricia Loftus, it makes me smile and I get excited. I open them and I'm like, yes. Anyways. All right. Can you first tell us a little bit about yourself, Amber, and your practice?

[Dr. Amber Luong]
As you alluded to, I do have an opportunity to have a basic science lab where we are interested in understanding the pathophysiology of chronic rhinosinusitis with a particular interest in allergic fungal rhinosinusitis, meaning we're interested in understanding environmental triggers. I also have a very busy clinical practice. Patients in the clinic, as well as in the operating room, operating in both the ambulatory surgery center, as well as in the main hospital. We take care of your typical chronic rhinosinusitis and outpatient procedures, but also some of the CSF leaks and tumors. It's just very enriching. I'm very lucky. I get to do a little bit of everything.

(1) Defining Allergic Fungal Rhinosinusitis (AFRS)

[Dr. Ashley Agan]
Yes. Lots of variety. That's so awesome. Today, we specifically want to zero in and talk to you about allergic fungal rhinosinusitis. I guess let's just start out by defining terms.

[Dr. Amber Luong]
Yes. Allergic fungal rhinosinusitis is basically a subtype of the bigger chronic rhinosinusitis with nasal polyps. We have chronic rhinosinusitis with and without nasal polyps. When we start looking at the nasal polyp group, allergic fungal falls under that. The reason why it's been carved out is because of some of the unique features of this disease. Some of which includes sometimes such severe presentation that the sinus cavities are so expanded that you get these really impressive CT scans where the frontal sinus is expanded into the intracranial cavity, not necessarily invading it like you alluded to, but more just because it's been there for so long and expanded the sinus cavity outside of its normal dimensions. It can expand into the orbital area.

Luckily, oftentimes, it's still preserved within the sinus cavity, but you get these really dramatic CT scans. Also on CT scans, you get a presence of this really thick, it's been described as “peanut butter mucin,” really sticky, thick mucin. Then these patients have an allergy sensitivity. If you did skin testing or blood testing, they do have elevated IgE level to the various different fungal antigens. The other characteristic is that when you look at the total IgE level, it's like out the roof. You see elevated IgE levels in allergy patients, but maybe in the hundreds in allergic fungal rhinosinusitis, it's in the thousands. Almost following into that category of hyper IgE syndromes such that we see in the thousands. It's a really unique phenotype and subtype of CRS with nasal polyps.

(2) Diagnosing AFRS & Regional Differences

[Dr. Gopi Shah]
When you diagnose it, can you pretty much diagnose it then on CT or are you then in your workup checking off the-- are we still doing the Bent & Kuhn Criteria or how do you actually diagnose it clinically? Are you just looking at the scan and then look in the nose, if you see some peanut butter and you're like, Oh, that's probably AFS or are there certain things in your checklist that you want to make sure that you have?

[Dr. Amber Luong]
Yes, great question. Actually, the Bent & Kuhn Criteria has been a great criteria for us to make this diagnosis. One of those is, the nasal polyps we talked about, the eosinophilic mucin, CT changes, but it turns out that the more we are starting to talk as a community, a worldwide community, where we're starting to realize that there are several patients that you can check mark and meet them at Bent & Kuhn Criteria, but it either has a different presentation in different geographic locations or they're totally different things. It's confusing because in the South where we live, in the Southern regions, you get that classic allergic fungal, exactly what we talked about, this thick peanut buttery mucin, the fungal allergies, the expanded CT scans, but then when I talk to my colleagues, let's say in Canada, where they make the diagnosis of allergic fungal, they may have the fungal-specific IgE elevation, so a fungal allergy, but they don't get the same expanded CT scans.

They don't get the same really thick peanut butter, although they do get some sort of eosinophilic mucin. The question is whether or not these are the same diseases. I think those of us in the south that see the classic ones, I would say that it's probably going to turn out to be different than what they see up north, which is more of an eosinophilic serous with nasal polyps. I don't know if that's going to make any difference right now. Right now it's sort of just an academic discussion, whether or not those things are different. Down the road, as we start to really get down to the molecular, pathophysiology, that may have some implications because the more targets we identify, the more we really learn about this disease, maybe it turns out that there's a specific defect, which I'm starting to discover, that then could serve as a target for therapies.

There, it will be critical to really make that distinction, going away from what we're talking about, which is a phenotype, clicking off some of these characteristics to more of this endotype, where we're really starting to understand the molecular differences between one type of serous with nasal polyps and another type of serous with nasal polyps.

[Dr. Gopi Shah]
I wanted to ask about, do we know why there's that geographic difference? Does it just have to do with certain fungi being in certain places like in the south that aren't up north? Does it just have to do with that part of the environment? But then now I also want to make sure that we touch on endotypes and expand on that as a definition. I don't know what would makes sense to cover first.

[Dr. Amber Luong]
Yes, so, great questions! In terms of geographic distribution, we believe, so this was described many years ago by another fantastic woman rhinologist, Dr. BJ Ferguson. She was the lead author on this paper that talked about the geographic distribution of allergic fungal rhinosinusitis. It turns out that you do get much higher level of prevalence of AFRS in the south. I don't know if we know exactly why we do get this geographic distribution, but it sort of supports the concept that there is an environmental trigger to this disease process. That there must be an underlying genetic defect. Otherwise, everyone in the south would get it. There also must be a geographic, maybe the levels of fungus more than the actual presence of specific fungi, because a lot of these are very much prevalent everywhere. Staph aureus, similarly. Fungus, the different species are actually quite prevalent in most places. It's just that in the south where it's hot and humid, the duration of the year where you get these high levels of fungus is going to be longer than, let's say, in the north where you get these very distinct seasons and so you get a really cold front, so it kills all these spores while in Houston, we're happy if we get two weeks of where it drops into the 30s. Probably similar to Dallas, although it gets a little bit colder there. I think that's why we're seeing some of this distribution. Also, you'll see the distribution closer to like in the US around the Mississippi Basin.

There it's, again, humid, more prevalence, and higher loads of fungus. I think allergic fungal is unique in that it does really highlight this environmental component along with the probably genetic component of chronic rhinosinusitis more so than other endotypes.

[Dr. Gopi Shah]
Then just going back to the second part of Ash's question, in terms of terminology, nomenclature, are we still saying CRS with nasal polyps? Are we saying CRS with type II inflammation? How should we be teaching it to the trainees or discussing it with our colleagues? Is there a change in the way people are sort of referring to this?

[Dr. Amber Luong]
Gopi, I think the reason why we are in that, how do we talk about this is exactly to your point is that we're making this transition now. I think that still most people refer to CRS with and without nasal polyps because clinically that's a very easy thing to distinguish different types of chronic rhinosinusitis. We just don't have an easy way going. We're going to introduce a new concept now and term biomarker. We don't have an easy biomarker to link it to these different endotypes.

The difference between endotype and phenotype is, phenotype, we as clinicians can go in there, look and say, okay, we see a polyp, or okay, we see mucin. Things that we can see clinically. The endotypes are more challenging because that's describing different molecular pathways that lead to the same phenotype. The more we learn about this disease process, I think that that's where we're going to start seeing these different clear endotypes. Because even within that group that you sort of alluded to, that type II, and that pretty much covers 80% of CRS with nasal polyps and that's why I think we haven't really gone to, hey, CRS type II, because it's sort of falls under the same umbrella. I think as we're starting to really understand what causes aspirin, exasperated respiratory disease, while there is a defect in the arachidonic acid.

If we can find a clear biomarker, then we would be able to say this falls under AERD, aspirin exasperated respiratory disease. Even with allergic fungal, we're talking about this interesting phenotypic distribution, but we don't have a clear marker yet to talk about what the defect. In my lab, we've been really looking at this classic allergic fungal rhinosinusitis group, collecting tissue from these patients, collecting blood from these patients, and starting to see actual differences. One of the differences that we have found in AFRS patients is that they seem to be deficient in making an antimicrobial peptide. Now we're starting to dissect like, what is the pathway or the cells that are missing that prevent these groups from not having enough antimicrobial peptide or antifungal peptide, which explains why they get all this fungus debris in their sinuses.

Once we identify that, and then we come up with a test that help clinicians to easily identify that, then that will allow us to say this is an endotype of allergic fungal rhinosinusitis. Until we get to that point, I think we're going to still fall into those phenotypic descriptions with and without nasal polyps. I guess to answer your question, ultimately, is we're still using the word CRS with and without nasal polyps, but now having the appreciation that with nasal polyps, most of these patients have that type II inflammatory disease and so we're getting a little bit closer to endotyping these patients.

(3) Clinical Presentation of AFRS

[Dr. Gopi Shah]
Thank you for clarifying that. I think it would be helpful to take a step back now and think about how these patients present. What are they looking like when they walk into your clinic?

[Dr. Amber Luong]
Yes, so the whole spectrum. What's interesting about allergic fungal is that they can present quite young. Our typical CRS with nasal polyps, I think would present probably around 30s to 50 years of age. In allergic fungal, you should become more alert to a possible allergic fungal rhinosinusitis when you see a young person in their 20s. The youngest person that I saw present with allergic fungal is age 6. Very uncommon, but it can happen. It's not uncommon to see late teenage years and early 20s. They present with unilateral disease. Unlike another group of polyp patients in younger kids is cystic fibrosis.

Cystic fibrosis when they present is often bilateral and ultimately will have other symptoms associated with that. That's a different workup. You'll want to look at possible cystic fibrosis disease and looking for the gene associated with CFTR, the CFTR gene associated with cystic fibrosis. In allergic fungal, the kids can present with bilateral disease, but more often can present with unilateral disease. Going back, they're generally younger, often very limited symptoms like lower symptom burden than our other CRS with nasal polyp patients, which makes me think that these kids, because they present so much younger, they just think it's part of their life. They're just so used to their symptoms. It also highlights the chronicity of this disease.

To relate to this, when you and I jump into a pool, you notice the symptoms right away and you're freezing, but after a while you just get used to it. I think that that's what happens with patients who have these chronic diseases. Maybe initially they may have noticed it, and if they present really young, they may not mention it to their parents or may not think anything of it. Then they just sort of get used to these symptoms. Some of these patients, I just remember because they're so shocking, polyps are coming out of the nose, boogers and mucin, all this really big gross stuff coming out of their nose. They'll tell you that they blow it out, but they will say, I've got a little bit of a headache. Then you get a CAT scan and as I alluded to these really expanded sinuses, almost barely see the bone that separates the sinus from the orbit and the brain. You see on the tissue window, this heterogeneic signal in your CT scan, really alluding to this really thick peanut buttery stuff that's inside their sinuses.

You look at the patient and they're like, yes, I've got a little bit of a headache and a stuffy nose. You're like, oh my goodness. Yes, so very low symptom burden polyps that can sometimes present on both sides or just on one side. Those are some of the highlights from that disease presentation.

[Dr. Gopi Shah]
Yes, I totally agree. Bulk of my kids were probably between 12 to 16 when they would present. Usually, they came in and nobody's ever asked them if they could smell and they'll tell you, "Oh, I haven't smelled in years." The biggest one was, and they may or may not have even noticed it, and they're there with family, is the proptosis or just some sort of asymmetry. There's always, not always, I'd say maybe 50%. Those are probably my 14-year-old adolescent, mostly males, with some sort of proptosis. Although every once in a while I'd see it. Youngest mine, I think, was six. That was a tough one because the workup was so indolent. You don't expect AFS. Their steroid response was a little bit more of where all of a sudden it was gone. They didn't have as much time to maybe develop all that peanut butter mucin, and you're working them up for CF, PCD, they have this throat clearing, maybe some cough. The younger they are, it can be a vaguer presentation because there's a lot more to think about. I agree that the symptoms are mild and finally, they just can't breathe or their mouth breathing or, and it's been going on for a long time and by the time they come up to you.

Now, every once in a while, and this is maybe twice, I think, in our children's ED, we've had AFS present with a history of vision loss. The vision loss, again, because the chronicity of it, it's been gone for weeks. That's heartbreaking because there's not too much once they have like an afferent pupillary defect or something where they're barely seeing and it's been going on for weeks. We've had two cases like that and that's very difficult. You do help them breathe better, but that vision's not coming back once it's been that long.

[Dr. Amber Luong]
Yes. Luckily, those don't happen too often, right, Gopi? I think I've only had in my career one child that completely lost their vision, but luckily it doesn't happen in that scenario so often. More often it's this very impressive CT scan where you're thinking that, oh my goodness, they are going to lose their vision or how can they see? You're able to get in there or there's got to be a CSF leak when you get in and you don't worry. Then you go in and there's no issues at all, but you're prepared for everything. You're prepared for seeing the optic nerve or you're prepared for a blood bath. You're prepared for brain fluid repair where you hope you can reach it with your instruments because the sinuses are so expanded that the typical instruments won't reach in that area. Knock on wood, that doesn't happen too often. That's unlike other CRS with nasal polyps patients. Those complications can happen more often, I think, than your other CRS with nasal polyps.

(4) Imaging for AFRS

[Dr. Gopi Shah]
That brings me to the question of imaging. Is there ever a role for CT with contrast or an MRI? Now, we talk about these really impressive CAT scans and like you said, if it's that anterior cranial fossa super pushed up or super thinned out and whatnot, MRI though, you tell me, because I always go back and forth. I'm like, would I go in if I think it's probably going to be fine, but then I'm like, do I need to be getting it? I feel like I should know, but I don't.

[Dr. Amber Luong]
I think when I was earlier in my career, I would get the MRIs. But after the experience with it, more often than not, it's not an issue. I don't normally get an MRI because it's so much more difficult to get the MRI. If I'm going to get the MRI, it's usually with contrast. Again, it's just so much more challenging to get the MRI. Luckily in a majority of cases, you won't have that concern about the separation. I would say if you don't see a lot of allergic fungal or you don't get that opportunity where you see them, I would tell you to get the MRI at least from time to time, just because it's quite impressive seeing the drop signal on the MRI, some of these characteristics, and just to sort of give you comfort going forward to not have to get the MRI, just to be able to see that. I don't get it now on a regular basis unless there's something in their history, i.e. vision loss or meningitis, which thank God, it doesn't happen very often.

If there's some other complications such as that, then I'll get the MRI, or because of some other issue, I am concerned about a prolapse or a loss of the integrity of the skull base or the orbit, then I may get the MRI, but most of the time, I don't.

[Dr. Gopi Shah]
I agree with that. I think unless there's a cranial nerve, something on my physical exam, which I've seen with expansion, or I'm not 100% sure clinically that this is what I'm dealing with, that I may get it. I have seen in the ER, especially that sometimes, again, the kids will present with AFRS and they come to the ER because of proptosis or something. All of a sudden, they come to the ER and they get this scan. Everybody's freaking out. They get an MRI. It gets even more confusing because of the drop signal. Then the patient's admitted because the read on the scan is fungal sinusitis in an otherwise healthy kid, but the patient's admitted and there's bells and whistles for "IFS," invasive fungal, or something else going on. I think that role of imaging can be very tricky.

[Dr. Ashley Agan]
The bone can get so thin on the CT scan that you're looking at it and you're like, is there bone there? Am I going to get in there and there's not going to be any bone? There always is. In my experience, it's just been pushed and it's just gotten so thin that you're looking at the CT and you're really worried that you're going to see an actual defect and usually there's not. The other thing that stands out is the couple of patients from residency who presented and family members would show a picture of them and be like, they look like a different person now because their actual craniofacial skeleton has expanded as well. Not common, but those are the ones that stick out, those extreme cases that have been going on for a long time and they finally present.

[Dr. Amber Luong]
Yes. I think it's those extreme patients that are the most challenging to clear of allergic fungal. Luckily, the kids, in my experience, more often than the older ones present with unilateral disease. If you can get that unilateral disease and you can do a really good job of clearing all the mucin, then those patients do really well. It's those, the ones that I remember over the years, the really expanded sinuses, the crazy CT scans, those are really difficult because it is so challenging to get all that mucin out because it's these unusual crevices, these areas where our instruments are not intended to get to at that depth. I think that those end up, and because of maybe how long it's been going on, those are really sometimes the most challenging. There's this one gentleman I'm thinking of, and also some social determinants of health, he's unable to do some of the things we need him to do. He's been quite challenging, but his vision is totally off. He's got a misalignment of his orbit, his eyes, so he sees double vision, so now he just relies on one. He has to rely on the METROLift to get him to the clinic, and it's hard for him to do saline irrigations on a regular basis. We end up sticking with oral steroids, which is just horrible. Now he's gained a ton of weight, and unfortunately, because of his social situation, his diet is not so good, so it's more likely that he gains the weight, and now you've got other medical complications.

These patients can be very challenging, and there's actually a great paper out of the MUSC group or Emory group, I can't remember which one, but they were talking about initially when there was less known about it, about how at least in their population, it was generally the lower income patient population that was getting allergic fungal. That makes it another level of challenge when you're dealing with that aspect on top of their disease process.

(5) Distinguishing AFRS from Other Sinusitis Using Labs

[Dr. Ashley Agan]
Yes, for sure. Other than imaging, do you get any other testing? Do you get labs where you're checking IgE levels? Do you always culture the peanut butter, thick eosinophilic mucin? What else is part of the workup?

[Dr. Amber Luong]
Yes, so at least in the South, and you probably see this, I don't do as much imaging, not much workup anymore because it's such a classic presentation. When I was starting out and trying to really understand this disease, I would get eosinophil levels, CBC with diff, and I still would get those in my fungal panel. I want to get a total IgE level. I do want to confirm that there's fungal hypersensitivity, and oftentimes it will be, especially here where it's much more classic presentation, so it's almost so obvious, but we do want to get that just to confirm it. Then a CBC with diff, however, in these patients, typically their serum eosinophil levels is normal.

That's another thing that we've been really investigating and trying to understand, that when you compare patients with allergic fungal to patients with CRS with nasal polyps that don't have allergic fungal, number one, they typically have normal serum eosinophil levels versus CRS with nasal polyps. Often they will have elevated serum eosinophil levels, and then number two, their asthma prevalence is very different.

In allergic fungal rhinosinusitis, we did both a retrospective study as well as a prospective study where we had those patients do spirometry to confirm or determine their asthma status. It turns out that allergic fungal rhinosinusitis patients, the prevalence of asthma is higher than the general population, which is about 8%, but it's not as high as that found in CRS with nasal polyps, which is somewhere between 60 to 80%. Allergic fungal balls, the prevalence of asthma is similar to CRS without nasal polyps, which is about 15 to 20%. It's a little bit higher, and it makes sense with the unified airway, so you get a higher prevalence, but it's not as high as what we see in other eosinophilic CRS with nasal polyp patients, which is somewhere between 60 to 80%.

We're trying to understand that and I think that's going to end up linking to this whole, what is the molecular defect in this patient population that leads to AFS when you're encountered with all this fungal load?

[Dr. Ashley Agan]
In my workup, I tend to, I'll get RAST. A lot of times they've already come in. Some of the patients who have already come in with allergy testing, whether it's skin or RAST. Sometimes on the way out, I'll just have them get a RAST panel, but I don't always know exactly what I'm looking for. I just want to have that sort of type one hypersensitivity and make sure there is some sort of mold or fungus that they're allergic to. I think I need to be a little bit more thoughtful about, maybe incorporating, like I never usually check eosinophil levels or IgE levels routinely.

[Dr. Amber Luong]
Yes, as I mentioned, the serum eosinophil levels is not as helpful, but it's just interesting to me. I just want to again, help differentiate between CRS with nasal polyps and an allergic fungal. It just helps me sort of just one additional data point, especially, in the younger patients, it's much more classic. There are some patients that present in their late 20s. There's that overlap potentially with the phenotypic presentation with CRS with nasal polyps. Some of these lab values help with that. Also with the total serum IgE levels, it sort of helps me put them on the spectrum of that AFRS. If they're really in the thousands, it makes me feel much more confident that this is allergic fungal because there's going to be a lot of patients, even with CRS with nasal polyps that just have fungal hypersensitivity, but it's not allergic fungal. It's those patients that have really elevated total IgE levels, along with these other characteristics that we talk about that make you lean more towards allergic fungal. There's never one lab that, again, if we did, it would be an endotype in a biomarker, but we don't have that one lab. These are data points that help to make the argument about whether or not this is your allergic fungal versus an eosinophilic CRS with nasal polyps. Also, it may be down the road when you start thinking about if you get this patient who you do have a difficult time managing for whatever reason, now we do have these other treatment options like biologics that some of these markers can be helpful when you're starting to think about different biologics that are available on the market.

Again, luckily, these patients, most of them do great with a good surgery upfront. I think that if you are lucky enough to catch someone with the initial presentation of allergic fungal, I would say that first surgery is really critical. If you are not set up to have the instruments, because there are some special instruments, some special frontal instruments, some of these and more expanded sinus instruments, I would say it's probably better for you to refer it on to a group that may just do more sinus surgery than you may. It's not because you're necessarily going to be unable to do it. It's just that you haven't invested in the instruments to help you get all that mucin. In my experience, that first shot, that first surgery is so critical because once they get down the road where they have a recurrence almost within a month after that initial surgery, it becomes way more challenging to get them under control.

Luckily, those biologics aren't that critical for these patients, but in those rare situations where they are really refractory and very difficult, and it maybe because they've got a couple of other things going on, then you may want to be thinking about these luckily other treatment options that have come into the market. These different delivery mechanisms for steroids, different steroids that are available, because once upon a time, really, we just relied on oral steroids. Now we're becoming much more sensitive to some of the long-term complications associated with the high doses of oral steroids we used to give. If you go back and look at some of the Bent & Kuhn papers, we were keeping patients on these steroids 30, 40 milligrams a day for months at a time. We don't do that anymore. At least, I would think that Ash or Gopi, do you? do those?

[Dr. Ashley Agan]
No.

[Dr. Amber Luong]
I know I don't.

(6) The Role of Steroids in AFRS Treatment

[Dr. Gopi Shah]
Where is your steroid? You have a patient that comes in. Do you do a trial of steroids for 10, 12 days, 14 days, get imaging? What is the role of oral steroids in your practice?

[Dr. Amber Luong]
Yes, so when a patient walks into my clinic and maybe they came in with a CT scan, so it's pretty clear that they have allergic fungal. Because we are interested in understanding the pathophysiology of this disease, I don't put them on oral steroids up front. If I was outside of the academic realm, I would tell you that this patient's going to need surgery and they're not going to respond with oral steroids alone. Given the fact that there are so much complications associated with steroids and that it would be beneficial for you to have perioperative steroids going into the surgery, I would tell you, think about the surgery date. Talk to your patients that, "Listen, you're going to need surgery. This is probably the first-line treatment," but save the oral steroids for that perioperative stage. I would tell you 40 milligrams at least, probably 40 milligrams. Obviously, if it's a child, that's going to be different. For your adult-sized patient, 40 milligrams, probably three days beforehand. You don't want to do it too much because then now it may change the presentation of your surgery.

Just enough to get the inflammation under control so that you're not having to deal with a lot of bleeding at the time of surgery makes it more likely that you're going to be able to get a good clean-out of the patient's sinuses. Then around day three or four of that oral steroid, do your surgery. Make sure you do a really nice job. Take the time. Some of these patients may take three or four hours to do. I remember when I was interviewing at the Cleveland Clinic and I just happened to be sitting in on a case with allergic fungal. This was a person who had come from the South and just relocated to Cleveland. The case that I was observing was seven hours just to get everything out.

Luckily, most of them don't go that long, but be prepared that some of this thick mucin, especially in areas that are really difficult to get to, may take an hour or two to just get. It's really important when you have that opportunity, it's that first surgery, you need to take the time. Otherwise, you're dooming this patient potentially to a lot of different surgeries down the road, a challenge, and not having the opportunity to have a situation where they had the one surgery, that's all they need, and they may need steroid saline irrigations. Then that's it for the rest of their disease lifespan.

[Dr. Gopi Shah]
That's 40 milligrams of prednisone, I assume?

[Dr. Amber Luong]
That's what I do. Yes, 40 milligrams of prednisone, three to four days before surgery, and then you do your surgery. Then after that, so for me, I don't do that perioperative steroid. However, after surgery, I will do that steroid, but I don't do it as high as I used to. I'll do more of 30 milligrams, three days, 20 milligrams for three days, 10 milligrams for three days. If it was really bad, maybe I might do a 40 milligram and a little bit longer, three or four days. Some of that is just clinical, like how long I want to expose them to steroids. I'll see them at that first week and decide how they're doing, and then see if we can start then adding on the topical steroids.

In terms of, as I alluded to, over the years, we've really been lucky in rhinology that there's been a huge interest in industry for wanting to develop new therapeutics for us. Once upon a time, all we had was steroids and steroid sprays and saline irrigations. Over the last, I would say, 10 years, we now have devices that allow us to put steroids locally into the tissue, whether it's at the time of surgery or after surgery. We have different delivery mechanisms for steroids. We have different now biologics. It does increase our ability to provide these anti-inflammatory medications into our patients, but it does make it a little bit more challenging for us because now we have more options. What is the best thing to do for our patients?

For allergic fungal patients, as well as a lot of my eosinophilic patients, I will put in at the time of surgery, a device that releases steroids locally because we know that the surgery in of itself causes a lot of inflammation. I like the idea of adding steroids locally there. For allergic fungal, I will still add on the oral steroids because it's probably not enough, but I taper them off pretty quickly, as I sort of alluded to. I don't do the month-long steroids. That has worked out really well. Some of these steroid delivery devices have an open matrix. That would be a concern for me, right? If I've went through and spent all this time opening up the sinus cavity and then putting in something that may block off the sinus cavity. I like the fact that these various different delivery systems have an open matrix. I feel comfortable putting it in there. That way it helps handle the inflammation that I may have caused without blocking some of the mucin and allowing the patients to start irrigating immediately after surgery.

[Dr. Gopi Shah]
Is that something like a PROPEL stent?

[Dr. Amber Luong]
That's the primary one. The principle is that you're delivering steroids locally. I've had the opportunity to work with various different companies, so we may be seeing other delivery mechanisms down the road. Again, I think we're in a really interesting time. There's a lot more treatment options coming in, and there's a lot more funding or interest in various different industry partners wanting to help us deliver different treatment options for our patients. Yes, so I think the one that you named is one of them, but I know that there are others that are looking to enter the market as well.

[Dr. Gopi Shah]
Do you let those stay in until they're gone on their own, or do you end up taking them out if there's still a little matrix left at six weeks, four weeks?

[Dr. Amber Luong]
Yes, so if you're putting them up in the frontal because they don't get as much, I think air turbulence, they don't crust as much, at least in my experience, so they don't crust as much if they're in the frontals. In that situation, it's so challenging to try to get it all out that I don't worry about it because it is intended to be a biodegradable, and it should just dissolve on its own with the saline irrigation. I don't put too much effort into trying to get it out in the frontals unless I see that there's a lot of crusting, which is unusual.

In terms of those that are placed in the ethmoid cavities, they tend to crust quite a bit. In that situation, I will remove them at around two weeks because the data has shown that the current ones that are on the market release most of their steroids after two weeks. At two weeks, between two weeks and three weeks, it's before they started degrading so much where you're having to pick out these little toothpick-like matrix fragments. You're able to get it mostly all en bloc, so to say, with all their crusting, and you're not losing the benefits of the matrix, but not driving yourself crazy trying to pick all of that stuff out.

[Dr. Gopi Shah]
Is there a max number that you'll put in one side?

[Dr. Amber Luong]
Yes, I usually only use one.

[Dr. Gopi Shah]
I just have a question. Sometimes the frontals-- everything's so expanded, there's a lot of space afterwards, and maybe you have a posterior one and maybe one more anterior and I don't know. I'm just asking. I don't use them very often.

[Dr. Amber Luong]
Yes. I usually just use one. That being said, if you've got a patient where you're really expanded out and you're concerned, it might be maybe justified to putting more than that but I usually use one. I don't know if there's any great data for that. You will be irrigating, and there is cilia, and they've shown there is a little bit more wide distribution of the steroid, not just where the matrix hits the mucosa where you're going to have steroids. In that situation, I don't really see the justification for the expense of putting more than that in a sinus cavity. Then I've got other options after surgery for adding in my anti-inflammatory medications, the budesonide irrigations or fluticasone through different delivery mechanisms.

(7) Strategies for Surgical Success

[Dr. Ashley Agan]
I want to back up and talk a little bit more about the specifics of surgery. You mentioned one big thing is just having the right tools at your disposal to be able to reach and get into all these nooks and crannies where this thick peanut butter mucin is packed in there. Can we unpack that a little bit more?

[Dr. Amber Luong]
Sure. I think the one that's most challenging on a normal basis, yes, there's these patients that have these crazy expanded frontals that expand posteriorly and intracranial cavity area, but not actually in the cavity itself. The most common one that's really frustrating to deal with is the maxillary sinus. Most patients, it is involved. There are different tricks that I've learned over the years that I use. Going in and then just the normal suctioning.

Then, when you start getting into areas where it's really low on the floor and their sinus cavity is significantly lower than even the nasal floor, but oftentimes I don't necessarily have to do, let's say, a mega-antrostomy or, do anything like that because the polyps and the mucin has already expanded the opening of the maxillary sinus so much. But still, sometimes in those areas, lower and anterior, it's really challenging to get all that mucin out. The maxillary forceps has this curve and it has a Blakesley grasping handle at the end. That works really well in using that to mix the mucin around to try to dislodge it.

I also will use various different curettes, like a 90-degree curette sometimes can help me reach into those areas. Those instruments are designed for the frontal, but because of the curvature of the instruments, it allows me to get to some of the anterior, some of the lateral aspects of the area. It is really important for you to take advantage of your different angled scopes so you can see in those areas. I will go into my even the 70-degree scope just to get a good look to make sure I haven't missed anything. I use warm saline on syringes. After I think I've gotten everything out, I'll irrigate with lots of saline and sometimes the warm saline is helpful to help dislodge it.

There are more devices that will allow you to basically power wash all of your sinuses. I'll use that, a hydrodebrider. The poor man's way is to do syringes and irrigate it and those are very effective too. Maybe even after you feel like you've gotten a good clean out, I'll go and use the hydrodebrider. It's an instrument that also allows you to curve the ends in different angles and then you attach like a liter of saline and it allows you to just power wash. It looks like literally a power washer and you just power wash all your sinuses at the end of the case. I'll do that at the end of the case.

Other things at the maxillary sinus, if I feel like there's some areas where the mucin won't come out, I'll wick it out. I'll use like Afrin soaked cottonoids and I'll stuff that maxillary sinus with a ton of cottonoids and then sometimes that will wick out some of the mucin that's trapped in there. Then again, other frontal instruments can be helpful. There's a whole bunch of non-biting giraffes that you can try, again, just to get at those angles, just to get everything out. That can be the most challenging area.

[Dr. Gopi Shah]
That can sometimes, like you said, be 30 to 45 minutes extra per sinus. There's a reason that these cases, the one that you described was seven hours. Sometimes it's just-- I tend to use the saline on the syringe and the different angled sections to irrigate that thick peanut butter because as we know, you can't grab it. It's just going to bite through. You can't suction it. It's not going to come out. It's always going to beat you. You have to make the openings really big and then I'll try to find a plane between the sinus wall and the outer surface of the peanut butter or whatever fungal ball unit and then just try to pump it.

I was going to ask you, in terms of navigation, do you always use navigation for your surgery as well as do you like navigating instruments like navigating microdebriders and those kinds of tools as well?

[Dr. Amber Luong]
Yes. For allergic fungal-- Well, number one, we're at an academic center. I think we probably tend to use the navigation probably more frequently than in private practice. I don't think that you have to use the navigation for all of your cases. Allergic fungal is one of those that I use it, even if I wasn't teaching, more often than not, just because of some of those characteristics we talked about, some of the areas where the skull base can be really thinned out, the unusual anatomy caused by the expansion of the sinus cavities.

I will more often use navigation. I don't like the microdebrider so much in terms of all of my dissections. I think that's just the way I trained. I trained more with the sharp instruments and sharp dissection and using my microdebrider more for the soft tissue. That being said, I think that there's definitely a role for using a microdebrider and it can be a lot quicker. It's just that I enjoy the dissecting aspect of it. In allergic fungal, sometimes that's not even a possibility. There, the microdebrider is more helpful because all those partitions are gone. All you're dealing with are polyps anyway. There's nothing for me to sharply dissect.

Yes, in that situation, a microdebrider. I don't like relying on-- To be fair, full disclosure, I haven't used a navigation microdebrider just because in my mind, I don't want to be navigating while I'm microdebriding. I'd rather be like, if I'm concerned, I should use a different instrument to tell me that that's skull base rather than to have my microdebrider up against the skull base and using that as my reliance on whether or not I should continue micro-debriding. That's probably just a philosophy more than anything.

[Dr. Gopi Shah]
I like it when I'm with a trainee. It makes me feel better maybe when I'm watching them use a guided one. That being said, I find that I use them less and less even with trainees as time goes on, just because-- You're right, I think you're looking at the endoscopic screen and so the image guidance is helpful, but I don't want to be looking at that the whole time. I also don't want the trainee necessarily to get dependent on that either. I go back and forth on it.

[Dr. Ashley Agan]
Do you have any preference or comments on different types of navigation? I'm trying to think of the types that we have at our hospital. Do we have BrainLab, I believe, is one of ours.

[Dr. Gopi Shah]
We had Medtronic in the past and now we have Stryker. One of our other hospitals in Plano has the Clarence system. There's so many different systems out there.

[Dr. Ashley Agan]
Yes, there's so many different ones.

[Dr. Gopi Shah]
Does it matter?

[Dr. Amber Luong]
I don't think it matters. I think it's whatever you're comfortable with. We have been really lucky and we also, similar to you guys, have a whole spectrum of different navigations at different locations. We have the Medtronic. We also have the different versions of the Medtronic. Ones where you can fuse it with the neurosurgeons because in our pituitaries we will use that Medtronic version. We have several different Medtronics and then we also have the Stryker system. We also have the ACCLARENT system. We also once upon a time had the Fiagon system.

We've been really lucky to be able to play with them and I find it really helpful because each of these have a little bit slightly different navigation, registration, different features. I think the Eclarent has this auto-segmentation feature. The Stryker system has the rings and being able to, for your trainees and even for yourself to think up through the frontal sinus pathway and even putting that on and developing a pathway to go to various different areas and then utilizing the onlay of these rings and the pathway onto your nasal endoscopy view guide you up into the various different areas you're going to.

All of these systems are being advanced and upgraded as we speak almost every day. There's a lot of research into these navigation systems and a lot of them overlap in terms of these features. Those I just highlighted a couple of them. Obviously, the Medtronic system is a tried and true and so many of our trainees are very comfortable with it. I think it's really about your comfort level, what makes you be able to use it but not relying on it and still being dependent, knowing your anatomy, but also being able to utilize this navigation to get to those areas, to really do that good job, especially with allergic fungal as we alluded to where the anatomy can be quite distorted and you really want to make sure you do a good job.

That navigation system can make you feel more comfortable to get into those last crevices, to get into that, making sure that you do see that most anterior inferior portion of the maxillary sinus and you've cleared that out. Otherwise, within a month they're going to come back in the maxillary sinus and then you're going to be wondering why, and in reality it's just you never got to it.

[Dr. Ashley Agan]
Talking about the sinus surgery and doing that really good job, if the patient just has like frontal and anterior ethmoid disease on your primary surgery, so the first one they've ever had, are you also opening up, for example, the sphenoids as well, "the full house FESS"?

[Dr. Amber Luong]
Yes, I haven't had the privilege of just having just one, that limited type of allergic fungal and I think it's because we're in an academic center. I think we're actually at the academy meeting coming up. I'm involved in the program committee. We have a great debate that is scheduled where they're going to be talking about this whole, "Do you do a more focused surgery or the full FESS if you have disease on one side? Do you do one sinus past the disease or do you just focus on the disease?"

Actually, Dr. Troy Woodward is going to be debating Dr. Devyani Lal at one of the great debates for the academy meeting in Philadelphia to address that very point. For me, I'm probably more in the camp of, especially if it's on that one side, I'll go ahead and open all of them. That's another key point. If they have unilateral presentation and they have a deviated septum, which they will, I will not try to fix that deviated septum because I don't want to instrument the other side.

Now, does it necessarily protect them? Not necessarily. I have had patients where they had unilateral presentation and then ultimately developed allergic fungal on the other side. I have had enough where they present with unilateral disease and it doesn't go over to the other side. I don't want to be the one that adds a big load of fungus onto the other side where they then develop allergic fungal on that side.

To answer your question, I will do the full FESS on the one side if they have allergic fungal, even if it's limited to, let's say, the ethmoid and the frontal but I won't go beyond to an unaffected sinus side.

[Dr. Gopi Shah]
I had that debate in my head and I think for me, it's age. For 14 and up, I will usually go ahead and do everything on that side. When they're younger, 10, 12, that six-year-old is going to be rare. The 10, 12, it was probably about a third of my kids with AFS. I always went back and forth on it because we're going to be back. We know that this isn't going to be their first surgery. I feel like in kids, it tends to come back fast depending on how compliant they are with their rinses, meaning sometimes within one to two years, we might be back in the OR.

Is it because I didn't do the full FESS to begin with? Is it because they're younger and have the "more robust immune system"? Is it because they're not rinsing? I'm not sure. I do find that as they get a little bit older and once they understand their process, they get it. They know when they rinse, they feel better. They know when they don't rinse, it comes back. We're on a much better pattern and they have a longer time between recurrence. I find that also the same question I have in my CF kids, PCD kids. I don't know. I go back and forth on it.

[Dr. Amber Luong]
That's a great point. It's just that age group is so difficult because there's so many other reasons why they would recur. To your point, they're teenagers. They're not going to be that interested in taking care of health diseases. they barely bathe on a regular basis, at least my guys. With soap.

[Dr. Gopi Shah]
My kids too.

[Dr. Amber Luong]
I have to remind them.

[Dr. Gopi Shah]
My kids too!

[Dr. Amber Luong]
You just wait until the 13-year-old. I'm like, "Lucas, I know you took a bath, but you have to use soap, okay?" You're like a teenage boy and so you're changing. I think it's hard because that disease process does affect the younger population. Sometimes not one gender is worse than the other, but they've got other priorities in their life. They're thinking about college or thinking about other things. It's hard for them to be thinking about this. I don't know if the recurrence is because of their age and just age by itself. If they did everything, they wouldn't recur. Is it because of their disease process or their compliance on it?

I think that's actually a great research project that we should look into and think about like, what is it that's giving them the recurrence, the high recurrence?

[Dr. Gopi Shah]
Yes. Just rounding out the surgical discussion, what else do we need to know about that surgery? Are you sending tissue for path? Are you always culturing the peanut butter? Anything else, any other pearls from the operating room?

[Dr. Amber Luong]
Yes. I send tissue more for my research aspect, but I send it just because our pathologists want to, but just to confirm the eosinophils in the tissue. It's not so, it doesn't play into a lot of my decision-making if I'm pretty confident that it's allergic fungal. I don't send the mucin anymore. Once upon a time, I would send it just for the Charcot-Leyden crystals showing the presence of fungus but now that there's papers that show that you can find fungus if you just look for it in everyone.

If you use reducing agents that break up the mucin, you can find fungus in 100% of patients as well as 100% of healthy control patients. That whole process is pointless. That doesn't really play into the diagnosis so much more than, let's say, if you saw the fungal debris by itself, that characteristic isn't enough. In those patients where it's not so clear, I would send the mucin and maybe have them look, but I don't do that so much anymore.

[Dr. Gopi Shah]
Do you send any cultures?

[Dr. Amber Luong]
No.

(8) Post-operative Management of AFRS

[Dr. Gopi Shah]
Do you have antibiotics, orals, or topicals ever? Is there ever a rule for that?

[Dr. Amber Luong]
After surgery, because of those steroid-polluting devices, they do crust, and so it makes me think of staph. I will put them on a post-operative 10-day course of Duricef, 500 milligrams BID for 10 days, just to protect them from the crusting that happens on the implants. If I don't use those implants, I don't use antibiotics anymore.

[Dr. Gopi Shah]
Do you ever do the ointment in the rinses, like Mupirocin or Bactroban, any role for that stuff?

[Dr. Amber Luong]
It just depends on how much mucin I'm seeing. Once upon a time, as they were transitioning off the oral steroids to the budesonide topical steroids, I would put in the Mupirocin because there was a lot of data that suggested that staph and fungus go together. I will more likely in my AFS patients, instead of just transitioning them to just Budesonide saline, I'll transition them to budesonide/Mupirocin saline irrigations and do that for a couple of months and then start bringing them down to something more simple to try to keep their regimen as easy as possible. Because again, we're dealing with a young population. The simplest what we can make it for them, the more likely they're going to do it.

[Dr. Ashley Agan]
How do you prescribe your Mupirocin and budesonide irrigations? How do you tell them as far as, how do you get the ointment to dissolve and how much are you putting in there? Are you just doing the Pulmicort Respules or, can you explain that part?

[Dr. Amber Luong]
For the budesonide, I use the Pulmicort Respules. It's two milligrams in two ccs, I believe is my dosing. I'll have to go back in because now I'm confused because they've been having to change it. Ultimately, it's one Respule in one bottle of 240 cc. One Respule, one bottle for my Budesonide, half the bottle on one side, half the bottle on the other, and ideally twice a day. Then for my Mupirocin, if you're lucky enough to find a compounding pharmacy, we usually will try to do 200 milligrams of mupirocin powder that dissolves into the saline. Oftentimes, because it's off-label use, and so make sure patients know it is off-label use, sometimes we get pushback from the insurance. Then we are able to find, sometimes have them cover 20 milligrams of Mupirocin powder because it does dissolve a lot easier. Otherwise, we are stuck with the Bactroban ointment and one tube in the ointment and just warming it up slightly, mixing it. Obviously, it can't be too hot, otherwise you can't get it. I have them warm it up slightly, try to get as much in, and then once it's at room temperature to irrigate their nose. In that situation, I don't know if it's more for me or for them that it's really working.

[Dr. Ashley Agan]
My ointment recipe is like, okay, put a dime-size amount. It's an approximate for sure. I think that making it as easy as possible because I think at the end of the day, it's consistency. At first I'd say, "Try to, maybe put your head back on the couch and drop the budesonide in directly," or "Rinse out your nose with half the bottle with regular and then put the ampulla and then rinse the rest out, so that it's more concentrated." Now I'm just like, "What, put it all in there and just make sure you do it and do it like homework. You do it Monday to Friday and if you take the weekends off, that's fine. That over four weeks will be better than, doing it all at once in four days."

Yes, I agree. I'm like the worst in terms of flossing and that stuff. My flossing is their rinsing. We have to sit down and we have to talk about my dental care and their sinus care so that we are on the same page because it is something every day. Anyways, sorry, tangent. Yes. I like that, Gopi.

[Dr. Gopi Shah]
Gosh. One last question about antibiotics. Are you doing ever three to six weeks of an anti-staph oral antibiotic? Is that in vogue, out of vogue? Nobody ever did it and I made it up. Is that still happening?

[Dr. Amber Luong]
I think that's when like things fail. I wouldn't say that that's the first line, but for allergic fungal, I haven't used it. I think you're alluding to like doxycycline or some of the macrolides, people talk about macrolides. The macrolide where it's a longer course, that's more probably for your CRS without nasal polyps that people describe that population that it may play a role. Then when other things fail, people will use the doxycycline for your CRS with nasal polyps. The data is not very good. That's where we're trying to explore options that are typical go-to treatment options don't work, but I don't think the data is very good. I think that that's why when you go to guidelines, all of those things are like options and weak data.

[Dr. Gopi Shah]
For the patients doing their budesonide rinses, is that forever? Is that patient dependent on how they're looking when them? How do things usually happen post-op? What's the rest of the story after surgery?

[Dr. Amber Luong]
I think that with their life, with me, it's forever. Ideally, I'd like to get them to a saline spray and a nasal steroid spray. I'm not naive to realize that most patients after probably around 10 years, they start tapering off and maybe they'll come see you once a year. Then they'll tell you, "Doc, I have to be honest, I use it like a couple of times a week. Every once in a while, I may use a steroid spray," whatever. Then you look in their nose and then you have to decide and you tell them, give them the feedback, "Hey, this has worked and obviously this has worked for you. Do whatever you're doing because it's working."

We don't have that great registry long-term because honestly, patients either move after a certain number of years or they stop seeing you. I don't know all the lifespan of an AFS. I assume that the fact that I lose so many as they get older, I assume that that's because they've gotten better, but I don't know. Maybe they're just moving to a different city after they got their job because they've graduated from college. I don't see that many older AFS patients that tell me they had surgery coming from, let's say, somewhere else.

I'm assuming that at some point in time, their immune system has changed and it's no longer that big of issue or that these patients are amazingly compliant and using their saline sprays and saline steroid sprays their whole life and they don't come back. I think it's probably the latter. Something happens and maybe they become less robust. As Gopi, I think you were alluding to when they're young and their immune system is so revved up, but then maybe as they get older, our immune system changes and it becomes less of an issue.

[Dr. Ashley Agan]
Going to that point, what's your surveillance like? What's your post-op like that first year, but what's it at two, three years? We're not quite turned the corner, but they need to come see you.

[Dr. Amber Luong]
Yes, probably once a year. Once they're stable and I've got them on saline irrigations and a nasal steroid spray, I tell them once a year, but then I honestly tell them, "Listen, it's more probably a social visit for me than it is for you, but I would love to see you once a year just to make sure you're still in the right path." Some of them will take me up on the offer and some of them, they after a couple of years or just once a year, stop coming or they've moved on to a different area.

[Dr. Ashley Agan]
Then let's say them and maybe like a grade two polyp, but they're like, so the polyp that's maybe at the level of the middle turbinate, but they don't have any symptoms. What do you do for those patients?

[Dr. Amber Luong]
Yes. If, again, their symptoms, unfortunately, aren't that great in terms of linking it up to what we see. If it's just a polyp, maybe I would consider doing a Medrol dose pack. If I see mucin, I'm more likely to use a little bit higher and longer extended steroid. We do have some steroid-eluting depots that are available that may come into play down the road. I don't like them so much for our situation because if there's a lot of eosinophilic mucin, I don't know if putting in a steroid depot is going to be enough for that patient population. I'll try the oral steroids first.

Then again, it just depends on how angry, because if it just looks like water balloon polyps, those that you sometimes you're like, "I think a Medrol dose pack is probably going to be enough," because honestly, if I went in and just popped them, they probably would go away. Then sometimes if they're amenable, I can do an in-office just a quick polypectomy too.

[Dr. Ashley Agan]
If you do like a Medrol, do them back at six weeks, do you see them back in a year, six months? How does that follow up? Are you like, "I got to follow this grade two polyp."

[Dr. Amber Luong]
I think it's just a bit-- Again, it depends on what that nasal endoscopy looks like and how reliant these patients are. Some of my patients, I can tell that they're like, "Well, I see this peanut butter" or "The stuff in my irrigations," and they're signed up for the patient portal. I'm like, "Okay, just drop me a note on the patient portal that you feel good about it." or if I'm really concerned then I'll be like, "Okay, let's do the steroid. Then I want to see you back at least four to six weeks after you've restarted your steroid irrigations, you've finished this course of steroids, and I wanted to see how you're doing and then we'll set you free again."

That situation is going to be how well I know this patient, how feasible it is and how reliable are their symptoms and their nasal endoscopy.

[Dr. Ashley Agan]
It's tricky too when the patient's like, "I feel good." Then you look in there and you're like, "It doesn't look as perfect as I want it to look." There's that little something. "Do I treat that? Do I treat my exam or do I treat the patient's symptoms?" It can be tricky.

[Dr. Amber Luong]
Yes, exactly to your point. Maybe it's just that if they just restarted or use their steroid irrigations more regularly if it's a pretty small polyp or-- There's some polyps that just look really bland. They may respond to that and then you can save your oral steroids. I agree with you, Ash. It's just, that's a hard one, but you have to remember that this is still a quality-of-life disease. If you don't think that polyp is going to ultimately lead to a florid exacerbation, then it's okay to become more conservative with their treatment.

[Dr. Ashley Agan]
Then, lastly, can we talk about biologics? Do biologics, have a role in treatment of AFRS?

[Dr. Amber Luong]
Yes, a very exciting area. In allergic fungal rhinosinusitis, it's not very clear. There's actually a couple of clinical trials that are ongoing right now because all the clinical trials that were done with biologics, excluded AFS patients specifically. Even though you have the indication for polyps for these biologics, that patient population was not specifically examined. There's actually several trials and more to come that are specifically targeted against allergic fungals. There is a one for, that's actively enrolling right now for dupilumab in patients who've already had a sinus surgery in the past and now are recurrent polyps, and they're enrolling. It's a randomized control trial. If you have patients who you think might be candidates, we are one of the sites for doing that.

There's a handful of other sites across the country that is also involved. We're about to launch another one that is also looking at dupilumab, but at the time of surgery. That's going to be launching sometime soon. Then a couple of the other companies, although they haven't done the trials, they are looking at the data. Anti-IgE, you would think that would be a strong role for that. Just keep in mind that those biologics trials specifically excluded allergic fungal.

That being said, it does still fall under that type two inflammatory disease process. If you do happen to have a patient with allergic fungal that doesn't respond to other treatments, and you feel like you've done a really good job with the surgery and they don't have any other surgical needs that need to be addressed, then I think a biologic is reasonable, but the right choice is still unclear, especially for this patient population.

[Dr. Ashley Agan]
Then, is there a role at all for immunotherapy?

[Dr. Amber Luong]
Yes, so actually UT Southwestern is probably the area that the institution has looked at this question the most, and I think the ultimate answer is if it doesn't seem to change the disease process and the life of this allergic fungal rhinosinusitis, but if they are an allergic patient, then immunotherapy can help for allergy symptoms. In terms of the course of allergic fungal and how they do, it doesn't seem to have much of an effect.

[Dr. Gopi Shah]
I find that a lot of AFRS patients don't always have the classic sneezing, watery, some of them do, but there's a handful of them that don't. I always ask them like, "If you have those symptoms, it might help you with those." but it's a lot of resources and time and missed work days, missed school days, depending on if they're doing shots or drops and things like that. I feel like we took so much of your time and I could keep going.

[Dr. Amber Luong]
I love this disease process. When you were saying that we were going to talk about this and we're going to spend an hour, I'm like, it depends on what questions you ask because as you can see, we can spend hours on this and fun, fun.

[Dr. Ashley Agan]
Any final pearls or tips to close us out?

[Dr. Amber Luong]
No, but allergic fungal rhinosinusitis is an amazing disease. I've built my career on it and love it. If you have any questions, feel free to send me an email on it, and happy to answer it. Thank you so much for giving me the opportunity to talk about it.

[Dr. Gopi Shah]
Thank you for taking the time.

Podcast Contributors

Dr. Amber Luong discusses Allergic Fungal Rhinosinusitis on the BackTable 73 Podcast

Dr. Amber Luong

Dr. Amber Luong is the vice president of the American Rhinology Society and a professor of otolaryngology at McGovern Medical School in Houston, Texas.

Dr. Ashley Agan discusses Allergic Fungal Rhinosinusitis on the BackTable 73 Podcast

Dr. Ashley Agan

Dr. Ashley Agan is a practicing ENT and assistant professor at UT Southwestern Medical Center in Dallas, TX.

Dr. Gopi Shah discusses Allergic Fungal Rhinosinusitis on the BackTable 73 Podcast

Dr. Gopi Shah

Dr. Gopi Shah is a practicing ENT at UT Southwestern Medical Center in Dallas, TX.

Cite This Podcast

BackTable, LLC (Producer). (2022, October 11). Ep. 73 – Allergic Fungal Rhinosinusitis [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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