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BackTable / ENT / Article
Diagnosing Chronic Invasive Fungal Sinusitis
Audrianna Wu • Updated Sep 30, 2024 • 34 hits
Diagnosing chronic invasive fungal sinusitis (chronic IFS) can be difficult, as patients with this condition tend to have generalizable symptoms, and are often not diagnosed until their condition has worsened to the point of irreversible damage. Rhinologist Dr. Ashleigh Halderman walks through the imaging tests, blood tests, and tissue biopsies that can help identify if a patient has chronic IFS and determine the best course of treatment.
This article features excerpts from the BackTable ENT Podcast. We’ve provided the highlight reel in this article, and you can listen to the full podcast below.
The BackTable ENT Brief
• Chronic invasive fungal sinusitis is often hard to distinguish from other forms of fungal sinusitis due to the generalizable set of symptoms most patients present with.
• Diagnostic workup of fungal sinusitis includes imaging screens (CT and/or MRI) and bloodwork to identify abnormal tissue or susceptibility to infection.
• Tissue biopsies are an important part of determining whether a patient has chronic IFS. Pathological examinations can help to detect common overlapping conditions, such as vasculitis or other autoimmune conditions.
• Anti-fungal medication can be an effective starting treatment for patients presenting with less severe symptoms before test results come back.
Table of Contents
(1) Challenges in Diagnosing Chronic Invasive Fungal Sinusitis
(2) Diagnostic Algorithm for the Chronic Invasive Fungal Sinusitis Patient
(3) The Role of Biopsy in Chronic Invasive Fungal Sinusitis
(4) Before the Biopsy: Pre-operative Considerations for Fungal Sinusitis Patients
Challenges in Diagnosing Chronic Invasive Fungal Sinusitis
Some symptoms frequently seen in chronic IFS patients include sinus headache, facial pain, paresthesia, and orbital symptoms. However, due to the generalizable nature of these common symptoms, it is often difficult to pinpoint this condition before it is too late. By the time most patients with chronic IFS are diagnosed, their condition has often progressed to the point of extensive tissue or organ damage, such as blindness.
[Dr. Ashley Agan]
In patients who are presenting with predominantly like a facial pain complaint, I feel like it would be very easy to miss this because it's so rare that most of us probably aren't thinking of chronic invasive. Because patients coming in with sinus headache and facial pain are pretty common, and the percentage of them that are experiencing a chronic invasive fungal sinusitis is probably very, very low. Also you don't want to miss this. Any other questions that help tip you off, or is it just the chronic worsening nature of it? Walk me through your clinic visit to where we can make sure that we're catching these. Because these are mostly presenting in the clinic, not in the hospital, like an acute, right?
[Dr. Ashleigh Halderman]
No. The ones that I've seen have all presented in the hospital actually. It's because unfortunately by the time they get to us is they've gone blind, or they've developed some type of orbital symptoms, if you will, or the imaging is very concerning for like a mass or a tumor. That's how they typically end up with us. If you were just going off of facial pain alone, it is difficult and you're right, the majority of those patients are not going to have chronic invasive fungal sinusitis. Again, if they don't have any possible immune deficiency, like they're not diabetic, they haven't been on steroids, all of these things, then that further sort of is like probably not the case.
There's been a few studies that have looked at sort of the findings, and I think you just have to have a suspicious eye. I wish that I could give you something more solid, but I guess I would have to say that just based off of my experience and my learning through practice, I've been better at spotting these things. It's taken some trial and error a little bit. I wasn't perfect and I still am not. When I'm looking at a case, again, usually a lot of the groundwork has been done and there's some pretty abnormal findings on the imaging, like soft tissue involvement of the orbit, destruction of the bone, involvement of the pterygopalatine fossa structures.
They're presenting with symptoms like pain or paresthesias, which absolutely should be very concerning for anybody. Paresthesias needs further investigation for sure. It's just sort of that clinical picture of all of those things. I think in isolation, pain without findings in those areas, without sort of anything concerning, I don't think you need to be too worried.
[Dr. Gopi Shah] Do patients have like other sinus nasal symptoms? Runny nose, nasal congestion, parotid drainage?
[Dr. Ashleigh Halderman]
Not predominantly. You can never say never, but they tend to really present with headache, facial pain and orbital symptoms and sort of less so. I think chronic granulomatous may present more with nasal symptoms, but chronic invasive, not so much. I think everyone that I've ever diagnosed with chronic invasive has had either the facial pain, facial numbness and orbital involvement, so diplopia or vision loss, or orbital apex syndrome, things like that.
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Diagnostic Algorithm for the Chronic Invasive Fungal Sinusitis Patient
Imaging scans can be helpful for detecting chronic IFS in patients. Dr. Halderman recommends starting with CT scans to screen the patients’ tissues. Chronic IFS patients tend to show abnormal tissue findings around the spheno-orbital and skull base regions. She also recommends using MRI scans to see if there is abnormal enhancement signaling in the tissue for these patients. Hemoglobin A1C tests may also help determine if the patient is susceptible to acidosis, a pre-existing condition that is commonly found in patients diagnosed with chronic IFS.
[Dr. Ashley Agan]
Just to review the imaging findings, so on CT, we said there's going to be more destructive lesions. Are you going to see erosion? You're not going to see expanse style changes. Walk me through some of the key CT and MRI findings. I know on MRI, you mentioned soft tissue changes, but just to have that organized.
[Dr. Ashleigh Halderman]
The majority of the patients are going to show some findings, like I said, around the spheno-orbital and skull base region. Bony destruction and abnormalities, both in the soft tissue of the pterygopalatine fossa, orbital area, orbital apex is what I've seen. Also there was a case that was involving the maxillary sinus and this person has sort of abnormal findings along the lateral aspect of the maxillary sinus bone and like some soft tissue density there as well. That heightened my level of concern, especially because he was having like these sinus findings. They weren't really severe, but he was having paresthesias. I was like, "This is weird, we need to biopsy this. I think it might be chronic invasive."
As far as the MRIs, you're going to see some abnormal sort of signaling. I believe that most of the time it does enhance, but if it's pretty far gone, there might actually be a decrease in sort of uptake and like abnormal enhancement going the other direction. It's not enhancing like you would expect it to necessarily, if the tissue has been compromised. Then for chronic invasive, it is enhancing and it can be pretty impressive. I know that from a case that we had in residency.
[Dr. Ashleigh Halderman]
If we lose some of the tissue viability and the tissue is just in general not healthy, I think you're going to have a loss of that. I believe typically it is enhancing on two.
[Dr. Gopi Shah]
For most of these patients, it would be appropriate to start with a CT, and then if you're seeing bony erosion or if you're seeing something that looks like a tumor, but you're not sure, then you get an MRI.
[Dr. Ashleigh Halderman]
I think you also get an MRI based off of the symptoms that they're having. If they're having pain and numbness, then I want to know what their cranial nerves look like.
[Dr. Gopi Shah]
For the AFS patient that we're concerned could have some areas that have converted to a more chronic invasive. Is there something on the CT or the MRI that tips you off to that? Because for those patients, correct me if I'm wrong, but I assume some of their CT is going to look like the classic AFS with like that expansive sinus looking picture. Can you comment on that part?
[Dr. Ashleigh Halderman]
Yes. Expansile, but not invasive, and not infiltrative. Anybody who has AFS, but is again, presenting with facial numbness or facial pain that's sort of really acute or it just doesn't seem to-- Because most people with AFS don't present with facial pain. It's been a long ongoing process, and so that's just not usually part of it. They've sort of adapted, as everything is expanding and building up, pain is not usually something that's reported. AFS obeys boundaries to an extent. It's going to be this pushing border that's sort of smooth and is not actually invading the tissue.
When somebody who has maybe some atypical symptoms who has AFS, you get imaging, then I'm going to look at invasion. Is this no longer a pushing border and it's starting to infiltrate into the orbit or the pterygopalatine fossa or intracranially? You're going to look, do they have a V2 numbness? I'm going to look along the course of V2 and see, is it enhancing? Does that appear abnormal? Do I need to be concerned that something is happening along this nerve? It's these very subtle things, but that's what you would be looking for.
[Dr. Gopi Shah]
They could have some areas of imaging that look like classic AFS, and then some areas where it's like, oh, maybe this is actually starting to invade here.
[Dr. Ashleigh Halderman]
Absolutely. I think that the majority of these cases, there's a lot of clinical evidence. It's just the picture is not right. Something doesn't resonate right. The clinical presentation and the history is so essential in these cases. I would say that just every time I've seen something like this, something's just been off and it is a complete picture that you put together with all of these modalities, your sort of history, the clinical exam, imaging, and ultimately biopsy.
They have looked at sort of those markers that they can use for pulmonary fungal diseases and the answer is we have no idea how. It's just too early. It hasn't been looked at for fungal stuff. I would not bother ordering those, because I don't know how to interpret them.
[Dr. Gopi Shah]
What about just labs that evaluate their immune system? Would you want to look at like your ANC, like their hemoglobin A1C, or stuff like that?
[Dr. Ashleigh Halderman]
That's fair. There's no labs that are going to diagnose positive fungus here, other than tissue biopsy with culture and seeing the fungal elements. However, you can evaluate their metabolic state and BMP or CMP. You want to see if they're acidotic and if they're in a DKA, that's a huge piece of evidence. The hemoglobin A1C to get an idea of what their sugar control has been like over a three-month period. All of those are really important just to paint a fuller picture. Again, I think I've seen this develop in folks whose hemoglobin A1C was like a seven-point something. It doesn't have to be horrendous. I don't think seven-point something is great, but it's not 11 or 14 or–
[Dr. Gopi Shah]
We've seen worse, yes.
[Dr. Ashleigh Halderman]
Then as far as the classic other immune signs, for chronic, if a person does, and there was one study where patients had developed sort of chronic invasives in the setting of hematologic malignancy, so it is possible. I think that they actually found that an A1C level of greater than 1,000 was associated with improved survival. I think that the A1C is something I'm mostly going to be looking at in an individual who is on like chemotherapy or has a hematologic malignancy. Insofar as you can correct that and follow it, then yes, that could be really, really helpful.
The Role of Biopsy in Chronic Invasive Fungal Sinusitis
In addition to imaging and lab results, tissue pathology is key to determining if a patient has chronic IFS. Dr. Halderman recommends obtaining biopsies to screen the patient for infiltrative changes in their tissue. Many patients with chronic IFS also show signs of vasculitis, autoimmune processes, or other conditions that can only be detected through pathological tissue examinations.
[Dr. Ashley Agan]
Ashleigh, what's on our differential here? I know we've discussed tumor, we want to get biopsy. Usually we think path and biopsy or think in tumor. Do inverted papillomas have a similar presentation? What else is in your box of three to four things that may prove to have something similar to some of these findings?
[Dr. Ashleigh Halderman]
Like you said, malignancy, typically not just your run-of-the-mill sinus disease. Inverted papilloma would not be something I would expect, unless it had converted to a cancer, because then you can very easily see a lot of infiltrative changes then into the tissue. Again, that comes back to malignancy. I would say that it's some other things– Vasculitis, pseudotumor, some autoimmune process, those are going to be on my differential as well. IgG4 disease, I think those are the main ones just because this is so unusual.
[Dr. Ashley Agan]
All basically requiring some tissue pathology to figure out what's going on.
[Dr. Ashleigh Halderman]
Yes, and a multidisciplinary care team and approach to sort of evaluating everything and talking to your colleagues to see, hey, is there something that I'm missing? What do you recommend here?
Before the Biopsy: Pre-operative Considerations for Fungal Sinusitis Patients
Before sending patients in for biopsies and pathological examinations, Dr. Halderman recommends taking the patient’s current condition and symptoms into careful consideration. For more severe cases, immediate biopsy is recommended to confirm the patient’s diagnosis and determine an effective treatment plan for the patient to recover. For less severe cases, Dr. Halderman may start the patient on anti-fungal medications and take a slower management approach before sending the patient to the operating room.
[Dr. Ashley Agan]
For the patient who's in the hospital, what's your order of operations? How quickly do you need to go to the operating room to get PATH? Is there anything that you do in the meantime while you're waiting as far as like starting medications?
[Dr. Ashleigh Halderman]
It depends how they present. Now if they've gone blind or are sort of losing vision, I think you need to act on things immediately. If they are started on antifungals before I get them to the operating room, that's okay by me, because preserving their functionality is goal number one. Then getting the diagnosis to sort of help support that is also a priority, but you need to do what you need to do to keep the patient as whole and well as possible. I feel like a lot of times we are not the front line. These aren't the people that they're not immediately coming to us.
There's been some stuff that's been done and you're just sort of walking into the situation and maybe a little bit later on the scene than some other folks, or infectious disease has been consulted and they're like, you need to get ENT involved. We arrive at some point. I always see my role in this as being the person that goes and procures tissue and sends that for the appropriate testing.
[Dr. Gopi Shah]
For tissue, this might sound like a silly question, but do you need a lot? Because when I think of sino-nasal biopsies, the lesion is very vascular. It's like get what you can and get out and pack. It doesn't sound like it would be too bloody. You could probably get what you need to, but how much tissue do you like to get? Because I feel like just a little Blakesley size, you're going to end up with, we don't have enough specimen or something. Does that ever pop into your head when you do these? I don't know. I'm always like, do I have enough? Do I have enough?
[Dr. Ashleigh Halderman]
Oh gosh, yes. I've been in the situation more than once, where they're looking and they can't find fungal elements. For that chronic invasive, and I think that our chairman who's a rhinologist has also said that he's had cases where like you just could not get pinned down fungus. I send a lot, any area that I can. In cases where they're pterygopalatine fossa or the orbital apex, I try to get to those areas and get samples, because disease is present and it shouldn't be there. I do chase these things a little bit. Not to the extent that like, if they're not blind, I'm not going to go blind them for the sake of like, but I am going to get a little bit into the orbit. I'm going to sample some of the orbital fat in a safe place. I do try to send as much as I can.
You of course always want to make sure that you send the tissue for culture. I can't emphasize that enough. This is sort of an interesting area where sending it for some of the like MRA analysis, of the 16S mRNA would probably be a great idea. The reason that I say that, is because it can detect it at such a smaller level, but it also can get the answer back to you, I think more quickly than routine standard cultures can. If you have that feasibility, this might be the case where you want to submit tissue for that sort of testing.
[Dr. Gopi Shah]
Are you doing frozens as well?
[Dr. Ashleigh Halderman]
I have. When I've done it, it's usually been on the second go around, because I'm wanting them to tell me that I've sampled something that can give them a diagnosis. As we know, in cases that are not fulminant acute invasive, it can be really hard to detect fungus in frozen section. I don't always rely on that. I think the second time around, usually I am, because I just want to know, did I get something? Please tell me there's something here so that I don't need to keep sending more, right?
[Dr. Ashley Agan]
Yes. Otherwise you're sending tissue for pass and then fungal culture.
[Dr. Ashleigh Halderman]
Yes, for permanent. Right. Because ultimately they need to be able to run like the GMS stains on it to really do a thorough job. It's going to take the special staining, that's what we're really relying on. If they can give me a preliminary, yes, there's fungus here, so that if we haven't started antifungals, we can start the person on antifungals, then that's really helpful. If I need to make decisions based off of that, then maybe I will send a frozen. It's a case by case situation.
Podcast Contributors
Dr. Ashleigh Halderman
Dr. Ashleigh Halderman is an Assistant Professor and practicing ENT specializing in rhinology and skull base surgery in the Department of Otolaryngology at UT Southwestern in Texas.
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 26). Ep. 164 – Chronic Invasive Fungal Sinusitis: Diagnosis & 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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