In this lecture, Dr. Hayek presents a few oculoplastic cases, discusses the diagnosis and options in treating the patients.
Lecture location: on-board the Orbis Flying Eye Hospital in Chittagong, Bangladesh
Lecturer: Dr. Brent Hayek, Emory University, Atlanta, USA
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Dr. Brent Hayek: Thank you for once again for welcoming me to Bangladesh. And I — we’ve given, you know, some lectures, you have been here, and there’s a number of good presentations. And what I want to do this morning is kind of shift gears a little bit. Make this interactive again. And kind of use some of the thought process from yesterday’s talks on orbital tumors and imaging and oncology and talk to you, at least this morning, on a case presentation and kind of go over some options, clinical findings and, you know, things that we’ve kind of discovered along the way.
I do not have any financial disclosures. So, coming to our clinic was a 22-year-old Caucasian female. And she had a history of left-sided proptosis for over a six months period of time. She’s very healthy. She had really no past medical history. No trauma, no sinus history.
And why would that be important? You know, I’ll kind of start off a little bit. You know, sinus history can be important because things can expand from the sinus cavities into the orbit. As you know medially, next to the orbit, is the ethmoid sinuses, and inferiorly under the orbit is the maxillary sinus. And so, it’s not terribly uncommon to see sinus pathology affect the orbit. And once something grows into the orbit, the eye has nowhere to go but forward.
And so, trauma can be another situation where, either an acute trauma, where you may see — and this doesn’t particularly apply here — but in acute trauma, you may have an orbital hemorrhage. That is a common process that can happen. And, well, I would say an uncommon process, but it can happen. And you would see expansion of the orbit. Or it could be a fracture to the orbit, and it’s the other side that’s enophthalmos, but that doesn’t apply here. But one thing that can be also helpful in patients that come into your clinic with either eyelid or orbital issues is to be able to look at previous photos.
And so, she happened to have a driver’s license photo that showed that five years ago, she had some process that was at that time. So, this was six months that she’s noticed it. It’s actually been going on much longer. And that’s actually very common.
Also, one thing we want to know, particularly with orbital processes, right? Is double vision and any decrease in vision. Because that is a common orbital sign. So, on her exam, she had brisk pupils. There was no afferent pupillary defect. Her vision is good. Her confrontation to visual fields was full. Her extra ocular movement was full. And hertel is where you see that her right eye was 18 millimeters and her left eye was 24 millimeters. So, what is that difference that is normal? Are people perfectly symmetric? Yeah. 2 millimeters.
So, up to 2 millimeters is generally what we consider “Normal.” No one is perfectly symmetric. There are some movie stars, like Denzel Washington, which is thought to have a perfectly symmetrical face, and some other people such as that. Maybe Brad Pitt is thought to have a perfectly symmetrical face. But if you ever look into a mirror yourself and you really study yourself closely, you’ll find that we’re not perfectly symmetric. And oftentimes you may find in your oculoplastic clinic, if you do, so I, ptosis surgery or brow lift surgery or mid-face surgery or lower lid surgery, patients may come back and they’ll say, well, this is a little different, this is a little off.
If you show them previous photos, you’ll say, this was always different. But it’s now that you’re studying yourself after surgery that you’re able to pick this up. So, we’re not perfectly symmetric. So, she’s 6 millimeters different. So, she clearly has some process going on. We, you know, do a full eye exam. Her pressure is normal, her slit lamp exam is unremarkable. I think for all orbital processes, including Graves’ disease, it’s always good to look in the back of the eye at least once, even as oculoplastic doctors. It’s important look in the eye.
And so, her cup to disk was 0.5. So, it is a sharp, pink disk. She has no ocular optic nerve edema or pallor. And also, if there’s processes that are close to the globe, like a mass, you’ll see sometimes striae or coronal retinal striae, and it kind of gives you an idea of the location. And sometimes it is close to the macula, patients will receive that and they’ll have distortion of their vision. And she did not have that.
So, this is a clinical photo of her. And I won’t ask because it’s fairly obvious that the left side is the side that’s proptotic. Now, she’s pharmacologically dilated. Her pupil is normal. We just dilated that side to look in the back of that eye. It’s not a bad idea to actually look at both eyes at the same time. This clinical photo we just look at the back of the eye.
So, I should have asked, what would you do next? As of yesterday, you know, we want to get a scan. So, a CT scan is the scan of choice. It’s fast. It’s cheaper. It’s more readily available. And if gives you a very good idea of what’s going on in the orbit. Because the fat, you can do contrast CT and see differences. Plus, compared to MRI, the bony anatomy in the sinuses we can see a little more clearly.
So, for multiple reasons, we always tend to get a CT scan. And which side is abnormal? You want to measure the size. And so, at least, you know, axial length and the width. So the, you know, there’s three dimensions that you can get in particular if you have coronal cuts. So, you want to know the size. You want to know the space that it’s in. Because it gives you — you can’t always — you really can’t tell most of the time what — there’s so many orbital masses, right? So, you can’t tell most of the time exactly what it is. But you can tell where it is, the size of it, and you can tell the sort of — is it well-defined? Or is it infiltrated? So, it gives you an idea of what it is, but it also gives you an idea for surgical planning purposes. If you’re going to do an orbitotomy and you want to remove the mass.
And so, once again, you’re correct, this is a CT scan. This is an axial cut. This is a soft tissue window. And you’re looking at a very well-defined large mass mostly in the intracoronal space. For orbital things, it’s very helpful to be able so far axial cuts and coronal cuts. And most of the time you get a head CT, you’re only going to get axial cuts.
And so, you will have to sometimes, then, get the patient to get another imaging test. They’ll have to get a CT of the orbit. Or a CT of the face. Or a CT of the sinuses. Because any of those protocols will include the orbit, and you’ll be able to get in the coronal cuts. And usually they’ll also be thinner slices. So, generally, you’ll get thinner slices which will be more helpful for you because you’ll get more resolution.
When I, for instance, look at this scan, you know, I want to know, you know, we talked about it. What kind of scan it is. Is it a CT or is it an MRI? Is it, you know, a coronal cut or an axial cut? Say, for instance, that there was no tumor here. And I’m looking for thyroid, you know, disease. Or I’m looking for medial wall fracture.
The axial cuts are great. Because perpendicular is the medial wall. So, I can look and I can see, is there a fracture in this wall? Versus a floor fracture, which is very common, the coronal cuts are very helpful because I do not see that well on an axial CT because it’s in a parallel plane. So that’s one thing, for instance.
Just knowing the sinus anatomy, knowing that these are the anterior ethmoids, these are the posterior ethmoids, this is the sphenoid sinus is very helpful. Because — and then knowing, you know, what the black is air, the white is bone. You know, looking at the septum. You can see a small septal deviation that’s anterior.
And then knowing how the lateral wall looks. And just assessing those things. And this is a contrast-enhanced image. So, you can see that now it becomes very hyper-dense on contrast. So, this gives you an idea — this is some sort of vascular lesion. And yesterday we talked about orbital tumors. What’s one of the most common orbital tumors that’s vascular? Right. A cavernous hemangioma would be the sort of top choice on the kind of differential.
The other thing that we were concerned is this tumor, or this mass is very large and it goes very deep. And so, this can be a difficult process. So, this is a coronal image. And you can see, once again, it’s a very well-defined, looks like an encapsulated mass that lights up well with contrast imaging. The other thing that you wanted to always look at — and I encourage our residents and fellow — always look at the sinuses. You know? Look at the inter-cranium space. Look into the temporal facet. See — you look at the bone, you know? And you really don’t see — you maybe — I would argue you see remottling of the bone. It’s hard to tell because the floor of the bone is very thin. Particularly in the medial aspect. But this has been there for a long time. I think she’s got remottling of the bone. There’s no erosions that you see in the bone, but remottling of the bone.
So, this is likely a benign tumor. But what they comment is, this starts to go into the palatine fissure. This starts to go into the intraorbital fissure. So, this mass is a sort of extending outside of the orbit. So, that’s what got us a little bit extra concern that this might not be a campus hemangioma, this might be something a little bit different.
So, large benign vascular lesion. Long-standing with recent progression. This has deep inferior medial orbit with extension into the intraorbital fissure. Going back, we see that. We see this going along into the intraorbital fissure.
And so, we could go in and we could try to take out this mass. But it would be sort of difficult. And so, what we decided to do was involve our ENT colleague. And we have interventional radiology. And so, their goal was to attempt embolization to the mass. And so sometimes this can be helpful to embolize the mass to control for bleeding purposes in particular. And the ENT doctor, their role was — our rhinologist — was to decompress the medial wall in the floor to create a larger space so it creates more exposure for us to go in and do an orbitotomy and be able to take out the mass. And that was the plan and that’s what we did.
And so, this is a type of anterior or frontal orbitotomy, where, on the backside of the eyelid through a transconjunctival approach we went to get the mass. And one of the things that you may see is that she has some blood out the nose.
So, the ENT colleague went first and went in. And now you can see the eye is sunk in. So, that’s because more space was created for us to go in and take out this mass. And there it is. And so, this really allowed for a more controlled way to get to the mass and get a more complete resection. Because our concern was that this could be something that was not a cavernous hemangioma and we wanted to get it intact and we wanted it not to bleed and give us a problem in control. Because she had very good vision.
When people have poor vision or no vision, it’s always more comforting to go into the orbit. Any time we go into the orbit, it’s always a concern. For instance, we have a partner who has been practicing 30 years of oculoplastics. And our chairman asked him, when are you — what time frame in your career are you comfortable going into the orbit, doing some of this? And he said, I’ll let you know when it comes — when it happens. So, I think all the time we get concerned, particularly when someone has good vision, they’re young, and we want to go in.
So, I do not know much about pathology. I will confess. And so, it’s always helpful to have a good pathologist to be able to look at somebody’s tissue. Because sometimes you may get an incorrect report. And so, I won’t go through here too much, but this, based upon that, was what we call a hemangiopericytoma. And so, that was based on immunohistochemistry. And so, with the advent of immunohistochemistry and some cytogenetics nowadays, you’re able to really more accurately tell exactly what type of tumor that you have.
And so, these are some of the immunohistochemical stains that were done. And she had some capsular extension up to the surgical margin. So, there is a small area where the tumor was not — though it looked grossly excised, there’s a small area where it may not have been completely excised. And that will be important for our discussion coming up.
So, this is actually a more old term. Hemangiopericytoma. Nowadays we consider this a solitary fibrous tumor. So, an SFT is thought to be the more correct term, but in the literature from the early 1900s, mid-century, there was a lot of, as you know in pathology, a lot of descriptive terms, a lot of times tumors have multiple terms, a lot of overlap. And so, we’re getting better with more accurate pathological and diagnostic testing like immunohistochemistry, cytogenetics, even molecular assays, we’re able to really define exactly what tumor type.
And so, solitary fibrous tumor was mostly a plural disease. Or at least thought so. But many of them occur outside. So, except — and this includes the CNS. And so, people have now said, you know, you should call this as a solitary fibrous tumor. So, it occurs along a histologic spectrum. There’s some very — I just removed one on a young Asian man from his lacrimal sac, which is very fibrous. It had no vascularity to it. But it was a solitary fibrous tumor.
And so, what is the goal of removing this? Well, this type of tumor does have, though rarely, some malignant potential. And there are some malignant forms. And there are some that can even metastasize. And so that was our concern. Did we do the correct surgical approach? And some may argue that you should have had more of a head and neck surgeon involved, or a neurosurgeon involved to make sure you more completely removed it.
But then the next question is, does the patient need any further treatment? Do they need radiation to control or eliminate the rest of that disease so they don’t get reoccurrence and that’s a malignant form of it? And I don’t think — we’re unclear fully of the answer of that. And then the time of that. And then the long-term surveillance. So, I think it’s always important, if you have a patient that you remove cancer, particularly a more malignant, aggressive form of cancer, that you have a surveillance plan. And that you follow them for a number of years, particularly for eyelid cancer, orbital cancer. You give them signs, you know, if you develop double vision, you develop vision issues, that you have them come see you sooner than later.
So, in follow-up, we have this young, healthy female with a biopsy-proven solitary fibrous tumor and hemangiopericytoma with extension to the surgical margin. She now has some hypoglobus. So, her eyeball is lower, and some enophthalmos. And with some double vision. So, I always council my patients, any time we’re going to do orbital surgery, you may end up with double vision. Because that is a known risk factor for orbital surgery. And that makes sense.
So, she was seen by our radiation oncologist for evaluation, and their recommendation was to do external beam radiation to do posterior orbit after we reconstructed the floor and the medial wall. And so, she underwent a secondary repair. Basically a controlled fracture. Almost a controlled blowout fracture, of the inferior. And we used this product called MEDPOR TITAN MAX. MEDPOR is polyporous ethylene. It’s been around for a long, long time. It’s a great product because it’s biologically inert and it works well and there’s a lot of use for it.
But there are other — there are many implants that, you know, you could use. And just to show you an example of the implant that we used. This is — it’s actually three layers. There’s a polyporous ethylene layer, and then there’s a tight titanium sheet and then there’s another layer. And it’s layered where the top portion that faces the orbit is very smooth. And the portion that faces the sinus mucosa has got little pores in it. And that allows for vascular ingrowth of the sinus tissue to help. And then it has these — and we maybe use one or two. I usually don’t use. So, this faces the medial wall and goes up along the medial wall. And this goes along the floor.
And we generally have to kind of custom cut this with some heavy scissors to make that fit. So, after that she went and saw radiation oncology. And she had some post-operative surveillance that showed no residual tumor. And she underwent a fairly, you know, a fair amount of radiation. So with lymphoma, we sort of talked about today — or yesterday — do they have high doses of radiation or low doses of radiation? For lymphoma.
Say, for a MALT lymphoma or follicular cell lymphoma which are common lymphomas that you would see in the orbit, would you say that they require a higher or a lower amount of radiation? Who says higher? Who says lower? Lower. Lower. Yes. Yes.
In fact, over the decades, if you start to look in the literature — so, the most common type of lymphoma that we see in the orbit is MALT lymphoma, EMZL lymphoma, and over the decades the amount of radiation keeps dropping. And now, generally, it’s accepted to do 20, 24 gray, compared to 60 gray of lymphoma. And even now there is a prospective design study being done at MD Anderson, which is a top cancer institution in the U.S., and they’re doing what’s called Boom-Boom radiation and they’re giving 4 gray. Just 4 gray. And they’re seeing great results with that, most of the time. So, that’s two — so, we have to divide this up. So, this patient would be coming for like a month. So, they’re getting a couple gray each time. So, this patient with Boom-Boom gets 2 gray, and they come back and they get two more and they’re done. So, that makes it easier both on the patient, in terms of logistics, and as well — but still most people are doing 20, 24 gray of — and maybe a little bit more, depending.
So, she’s actually had, now — this is an older presentation. She’s had actually now seven years of follow-up with no — and she, after a few years she moved. But, you know, I’ve kind of sort of kept in touch and he’s had no reoccurrence. Her vision remained good, which is always something that I wipe my brow. And she had some mild diplopia and upgaze, and she developed some cicatricial lower lid ectropion which was offered to be fixed.
So, generally when someone has a cicatricial lower lid entropion from fracture repair approach like this, we’ll usually have to use a spacer graft. Because if we just release that scar and the eyelid comes up, afterwards they’ll heal scar back down. So, we kind of have to put a spacer graft.
Our choice, generally, is ear cartilage. You can use hard palate. I know that’s a very common one that’s used as well. So, yeah. So, she continued with further follow-up. She had some mild lid retraction entropion, but she declined further repair at the time.
So, sort of the summarize, hemangiopericytoma, now what we consider solitary fibrous tumor is a rare orbital tumor with potential for local reoccurrence and some malignant behavior. So, did we follow the correct approach surgically? And was that mobilization needed? I would argue, yes. You could do a more substantial surgery with greater morbidity to ensure complete removal, potentially. But she’s been followed for a normal amount of time and has done very well.
In — we don’t fully know the answer. The recommendation by our radiation oncologist was to do external beam radiation which she’s done well with, and she’s done long-term surveillance and has done well with that as well. So, any questions so far? Any questions on that?