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BackTable / ENT / Article
The Many Presentations of Fungal Sinusitis
Audrianna Wu • Updated Sep 30, 2024 • 33 hits
Fungal Sinusitis is an inflammatory infection caused by the inhalation of certain types of fungus. There are multiple differentials within fungal sinusitis, including allergic fungal sinusitis (AFS), acute fungal sinusitis, and chronic invasive fungal sinusitis (chronic IFS). Patients with fungal sinusitis often report generic symptoms, such as facial pain and headaches, making it difficult to distinguish one type of fungal sinusitis from another. In fact, many patients who are diagnosed with chronic IFS are first diagnosed with other forms of less severe fungal sinusitis.
To help you better recognize the many presentations of fungal sinusitis, rhinologist Dr. Ashleigh Halderman provides a quick overview of the different types, with special emphasis on how to differentiate chronic IFS from less severe diagnoses. 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
• Fungal sinusitis is defined as a sinus infection caused by inhaling certain types of fungus.
• There are many types of fungal sinusitis, including allergic, acute, chronic, and invasive fungal sinusitis. Chronic IFS is a rare but severe type of fungal sinusitis.
• Patients with chronic IFS often have other health conditions, such as a compromised immune system or diabetes.
• In most cases, patients with chronic IFS are first diagnosed with less severe forms of sinusitis.
• Many patients who present with chronic IFS have a long-term history of unusual symptoms without clear cause.
Table of Contents
(1) Types of Fungal Sinusitis: Invasive vs Noninvasive
(2) Differentiating Chronic Invasive Fungal Sinusitis from Other Types of Fungal Sinusitis
Types of Fungal Sinusitis: Invasive vs Noninvasive
The noninvasive category of fungal sinusitis includes fungal balls and allergic fungal sinusitis, whereas the invasive category includes acute fungal sinusitis, chronic invasive fungal sinusitis, and chronic granulomatous invasive fungal sinusitis. Chronic IFS is one of the more uncommon but more damaging forms of fungal sinusitis. Many patients who present with chronic IFS tend to be immunocompromised and have other pre-existing conditions, such as diabetes.
[Dr. Ashleigh Halderman]
The way that I categorize fungal sinusitis is there's two main categories. There's noninvasive and invasive. In the noninvasive forms, you're going to have your fungal ball and allergic fungal sinusitis.
Essentially, in a fungal ball, it's going to include usually one sinus, and we're not certain how or why it happens. One thing that seems to increase the risk is prior endodontal work. That might be changing, because I believe that they're no longer using the zinc as part of the amalgam. That was the thing that allowed this permissive environment, was theorized.
That is something that sort of increases as people age, the incidence increases of fungal balls. There's not really any immune response to the fungus. In allergic fungal sinusitis, obviously, that is allergy-based, or so we think. There is an IgE sensitivity to fungus in these patients, which is helping to propagate and allow that disease to form. Otherwise, the immune system isn't really combating it, or ignoring it, I guess.
…
Then the chronic forms, there's both chronic invasive fungal sinusitis, which is what we're going to talk about today and is pretty uncommon. Then you also have your chronic granulomatous invasive fungal sinusitis. The differences between those two, is that with chronic invasive, you're going to see some level of immune deficiency. It's usually not as profound as acute invasive, but it could be somebody who's on high-dose steroids for a while, or they have diabetes. I think most of the people that I've ever seen that have it have diabetes.
It's, again, very rare, but the mortality rate is real. It can be at a year, one paper said about 48% survival. It's landing there at about 50%, like acute invasive. Then chronic granulomatous tends to be an immunocompetent patient. There's not really any evidence of any degree of immunocompromise. I am suspicious that this might be on a spectrum. We at UT Southwestern had five cases of allergic fungal sinusitis that converted to chronic granulomatous. Actually, when they've gone back and looked at pathology for allergic fungal sinusitis cases in a study, a couple of studies I think have been done. Around 21% of AFS patients show some granulomatous sort of inflammation.
All of our patients, they were all young Black men. Those are typically the patients who tend to see the most significant or severe disease when it comes to allergic fungal sinusitis. I wonder, is there something there? It can also happen in isolation not associated with allergic fungal sinusitis. It tends to be much more favorable. I think mortality is pretty low. It's highly treatable.
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Differentiating Chronic Invasive Fungal Sinusitis from Other Types of Fungal Sinusitis
Distinguishing chronic IFS from other types of fungal sinusitis can be difficult. Many patients who are diagnosed with chronic IFS present a wide range of unusual symptoms and have a history of other conditions, including a previous fungal sinusitis diagnosis. Imaging such as MRI and CT scans can help to differentiate types of fungal sinusitis. As Dr. Halderman explains, biopsies and tissue culture analyses can also help to identify the source of the patient’s symptoms and inflammation.
[Dr. Gopi Shah]
What does the typical patient look like that we need to be thinking about this diagnosis?
[Dr. Ashleigh Halderman]
It's always weird, for lack of a better term. Generally, the classic patient tends to be older. Most of the time they have diabetes and it's maybe not super well-controlled, but it's also not like DKA area. They're going to have symptoms for a very long period of time and they can be pretty nonspecific symptoms. The first case I treated or diagnosed chronic invasive fungal sinusitis on, was a woman in her 50s and she'd had a year of pretty severe left-sided facial pain. She had been treated for sinusitis, she had been treated with gabapentin, all these nerve-- They thought she had some type of neuralgia and nothing was really touching it. The pain was really severe throughout the whole trigeminal distribution. She went down the whole trigeminal neuralgia pathway. Then she went blind.
When she presented, there was pretty impressive destruction of the orbital apex and sort of the spheno-orbital area. Going back and hearing her story, it had been there this whole time for a year and they just missed what was causing it. That being said, there was bony destruction. It was pretty impressive. You could see some sort of soft tissue changes as well in the orbital apex area. I took her to the operating room and took tons of biopsies in the sphenoid area and along the lamina and whatnot, nothing. They couldn't find anything. I thought, this has got to be a cancer or something. There's got to be something there.
I think the next time I took her back, I actually went into her orbital apex and was getting tissue samples because that seemed to be the hot spot. I'd tried to avoid it before, because obviously you don't want to take out a person's optic nerve and I was still like very cautious. They saw some fungal elements on the frozen, which then they didn't see on the final pathology, but we had enough to go off of. She started on the antifungal therapy and her pain got way better. When you go into these cases, it's not necrotic. It's not necessarily slapping you in the face. It's like, this is blatantly abnormal. The tissue looked a little bit weird. It was maybe a little bit pale and thickened, if you will, but again, it wasn't necrotic.
We got her on antifungals and I sent the tissue for culture, nothing grew out. That's a common theme in these cases I find, is that sometimes it is hard to achieve a true diagnosis and get tissue to send for culture and to get it to grow out. We had to go just based off of a high level of suspicion. We hadn't found any cancer there. It had been going on for so long. She was diabetic. Her hemoglobin A1c wasn't awesome. She started getting better once we started treating her with the antifungals. I think here it was really sad too, because it had gone so long.
She did really well on her antifungals and then she just stopped taking them and disappeared for a little while. She came back with new facial pain and new headache again on that left side. That was very worrisome to me. It was already evolving her trigeminal nerve and her orbital apex. I already had a high level of concern that it had gone intracranial. I think by definition it had. I got imaging at that time and it had gone wild intracranially. She actually had sort of osteomyelitis type changes to the sphenoid wing. She ended up going with neurosurgery to get cleaned up, get cultures. We were able to get a positive culture at that time. She's just going to be on lifelong antifungals, but she's still alive.
[Dr. Gopi Shah]
Wow. In terms of your initial scope, Ash, what did you see when she first came to your clinic when you did endoscopy? What are their scope care findings? Do you see polyps? You said you don't see that necrotic dead tissue. Are there any tip-offs?
[Dr. Ashleigh Halderman]
No. For me, it was purely symptoms and imaging. Again, it was limited to the sphenoid and the orbital apex. With the scope exam, you're not going to be able to see that area. You might be able to see the sphenoethmoid recess. I had another case recently that I was highly suspicious of chronic invasive and there was periolith. We were taking cultures, but sending-- Again, the perioliths was the only thing. The tissue just looked a little bit pale and thickened and inflamed a little bit, I guess, but not necrotic.
…
[Dr. Ashleigh Halderman]
The chronic granulomatous, it's going to appear as a mass on imaging, whereas chronic invasive is going to appear as more infiltrative.
[Dr. Ashley Agan]
For your lady that had been sent for a workup for trigeminal neuralgia and everything, does that mean that her scans were clean? That there was no sinus disease that could be attributed to her symptoms and people were like, hey, it looks clean, let's send you to neurology?
[Dr. Ashleigh Halderman]
I think she had some mild findings in her sinuses, pretty mild. Maybe just a little bit of mucosal thickening. They did treat her for sinusitis, but it didn't make any difference. I think that they abandoned that and went for-- The pain was the predominant symptom. She wasn't really having any sino-nasal symptoms. I don't certainly blame anybody for that thought process. I think that the destruction of the orbital apex was a later finding. I think that if that had been present immediately, alarm bells would have been ringing.
[Dr. Ashley Agan]
Since it had been a year, maybe she got that CT early on, maybe there wasn't much there. Then it was MRI after MRI and you don't get great bony characteristics.
[Dr. Ashleigh Halderman]
Yes, predominantly intracranial MRIs, MRIs of the trigeminal. I can't tell you how many times you get one of those scans and there's no comments at all on the sinuses.
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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