This lecture will cover the initial approach to diseases of the orbit. This will include basic surgical and functional anatomy, history and physical, and orbital imaging. It will also include common and unknown causes of orbital disease.

Part 2 of this course will be delivered at a later date and will cover the Surgical Management of Orbital Disease.

By Dr. Yasser Khan

Approach to the Diagnosis and Management of Orbital Disease – Part 1 11/15/2016 from Cybersight on Vimeo.

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(To translate please select your language to the right of this page)

DR YASSER KHAN: There. Now I can see myself talking. I was waiting for that. Okay, good. Okay. So the whole objective of this lecture is to develop an approach to taking a orbital history. Orbital disease can be actually quite complicated, and it’s very important to get a basic history and basic approach to orbital disease. And once you have your basic approach, then you can basically diagnose and manage any orbital disease that comes. Now, this is part I. Part II of this series will be more on treatment and surgical options of orbital disease, and that’s when I’ll have videos, and we can get into treatment. But for now, I want to start off today with just a basic approach on how to perform an orbital exam. On just being familiar with specific techniques and equipment used to investigate the orbit, recognize a patient with proptosis, and also develop a differential diagnosis based on your history and physical. This is poll question number one. What is your position? And if you can just basically answer — just give me an idea of exactly who’s online. And I think Lawrence, I believe that they’ll have access to these poll questions, I believe. Right? You’ll be able to answer them?

>> You have to hover your mouse above the screen. You have to launch the poll from your end.

DR YASSER KHAN: So… Always remember, when it comes to orbital disease, the orbit is a very small area. Let me see where my skull is. It’s a very small area. And basically you have this tiny little area in which houses the eyeball, plus multiple other structures that we’ll come to. And so it can get quite complicated, once you get into the orbit and start thinking about orbital disease. And so it’s very important to always recognize — just keep things simple. And if you keep things simple, then you’ll be able to manage your patients very well. It’s important to recognize and know the common orbital diseases in adults and children. Now, this may vary from area to area, country to country, Africa to Asia to North America, but there are certain general common things in orbital disease in both kids and adults. Okay? It’s very important to differentiate between acute, stable, and chronic orbital disorders, and also know when to refer. And this may not be possible sometimes, but know when to refer when you really don’t know what’s going on. Okay. So my general approach to orbital disease is basically almost like — you know, for those of you who know the French cartoon character Tintin, so that’s how I try to remember orbital disease, when I approach it. And there’s only really six things, to be honest, that can happen in the orbit. And that’s by TINTIN. The thyroid. I stands for inflammation, so orbital inflammation. N is neoplasm. T is trauma. I is infection. And N is newborn or congenital disorders. Okay. Good. So… Proptosis is a hallmark of orbital disease. And of course, I think most of us know this — proptosis is basically a forward protrusion of one or both the eyeballs. Okay? And when you see someone with proptosis, there’s basically three things you should always keep in mind, or three things that are very important, in approaching a patient with proptosis. The history and physical, the CT/MRI scan, and finally to do or not to do an orbital biopsy. So those are three things that you should always keep in the back of your mind.

(no sound from 3:45 to 4:16)

>> Dr. Khan, it seems that you muted yourself. Can you unmute yourself? Dr. Khan, you seem to be muted.

DR YASSER KHAN: Okay. Sorry. I guess I was muted. I apologize. I didn’t notice that. Do you want me to go back? Or just…

>> Maybe go back a few slides.

DR YASSER KHAN: Well, I think I’ll just quickly summarize. The slides are explanatory. So basically… Keep things simple with orbital disease. And the way I approach orbital disease is through TINTIN, where we have thyroid, inflammation, and neoplasm, and trauma, infection, and newborn. And really, there isn’t much more that can go on in the orbit, besides these pathological processes. Proptosis is the main hallmark of orbital disease. It’s a forward coming of both eyeballs, history and physical is extremely important in guiding how you will manage someone with orbital disease. A CT/MRI helps you establish the diagnosis, and a biopsy, if you need it, helps to confirm the diagnosis. Okay? How is proptosis diagnosed? Through either using the Hertel exophthalmometer, and for those of you who don’t know what it is — I think most of you would, but if you don’t, I can certainly show it to you after the lecture. The CAT scan is very useful, and also observing the globes from below. Basically if you have the patient look up and you basically look from underneath, you can see one eye is more out than the other. Okay? Okay. The Hertel ex-ophthalmometer measures the anterior projection of the eye from the lateral orbit, orbital rim, to the cornea. And I basically lightly push the instrument against the lateral orbital rim to measure. And I use the same base setting for each patient. Okay? Now, the average Hertel measurements are different — in general are different amongst the races. So basically, you know, the average measurement in a Caucasian is about 20 millimeters of protrusion. In Asians, Asians have larger orbits, so the Hertel measurement is less, at about 18. And Blacks generally have shallow orbits, so they’ll have higher proptosis. I’m sorry. Higher level of Hertel measurements. Okay. But in general, to be honest, in my opinion, the general numbers are not so important. Okay? Basically what’s more important is the asymmetry. So what I really look for is not necessarily how proptotic they are, but generally the asymmetry between the two. So anything more than 2 millimeters — so if one eye is more out than the other by 2 millimeters, that is significant. And basically what else is important is a change in globe position over time. So as time goes on, if they’ve noticed a change in one eye or the other, that’s significant. So the absolute numbers actually are not that important to me. Okay? And there are different kinds of proptosis. There is axial and non-axial. Okay. I’m going to actually pause, because I think some of my pictures are missing from these diagrams. From these slides. So just give me one second to reboot. Can you just mute my screen for one second, Lawrence? I’ll be 30 seconds. Okay. Sorry about that. Let’s try this again. Not from the beginning. Different colors. Okay. So, I mean, for those of you who didn’t see this, that’s the Hertel, where my arrow is. That’s the Hertel ex-ophthalmometer, and basically what I do is — by observing the globe from below, you can often tell how proptotic they are. And over time, I mean, you’ll get really good at being able to tell the amount of difference between one globe and the other. Okay? So we already covered this. Okay. So asymmetry — anything more than 2 millimeters is significant. So you can see by just looking at her from underneath, you can see there’s about a 3 millimeter difference in the one eye. So you don’t always need a Hertel. You can actually just look at it clinically. So change in globe position over time. So there’s basically axial proptosis, which is here, which is basically the eye going straight out, and then there’s non-axial proptosis, where something is acting on the globe to cause globe displacement, either up, down, left, or right. And this helps to locate the mass. Okay? So let’s talk about anatomy, in brief. So the dimensions of the orbit, on average, the volume is about 30 milliliters. The width of the orbit is about 40 — that’s basically from here to here, is about 40 millimeters. The height is about 35 millimeters, and basically the distance — and this is very important when you’re doing orbital surgery — the distance from the rim to the apex is anywhere from 45 to about 50 millimeters. I always, actually, in my mind, have the number 40. So when I’m operating, I will typically mark on my instruments the 40 millimeter mark, so that I know that if I’m below 40 millimeters, I’m getting very close to the apex, and I need to be careful not to cause damage to the optic nerve. So, in essence, the eyeball lives in the orbit. And the orbit is the house which contains the eyeball. Okay? And that’s very important. Because the eyeball does not live by itself. It’s got its neighbors. And these neighbors include veins and arteries and muscles and connective tissue and fat and the lacrimal gland. And so the eye is not alone. And that’s very significant when it comes to orbital disease. When I look at the anatomy — you don’t have to know every single nerve or vessel or fat or structure in the orbit. The key thing from looking at it from a diagnosis — even when you get into orbital surgery, which I’ll get to in my next lecture, is surgical spaces. Okay? So the orbit has certain spaces. There’s the extraconal space. The extraconal space is basically the space outside of the cone. Or the annulus of the eye. And basically the extraconal space is outside of the extraocular muscles, outside the cone, and typically contains structures like the lacrimal sac, lacrimal gland, you know, other connective tissue, arteries, and veins, and things like that. Okay? Then there’s the intraconal space, which is between the extraocular — the four extraocular muscles. And, I mean, really, the intraconal space has one extremely important structure. And what is that? Obviously that’s the optic nerve. Okay? So those are two important spaces. And they determine how you will — what the cause of the proptosis is, and how to treat it. Other spaces include the subperiosteal space, and that’s the space between the periosteum and the orbital rim or the orbital bones. And things that commonly can occur there are things like subperiosteal abscesses, when there’s an infection in the sinuses, that’s present in the orbit, you know, subperiosteal hematoma can also occur in the subperiosteal space. Then there’s a sub-Tenon’s space, which I think many of us as general ophthalmologists deal with all the time, when we do things like conjunctival surgery, and that’s basically sub-Tenon’s space, and that’s considered an orbital space, and just communicates with the optic nerve in the back. So that’s like the sub-Tenon’s space. Okay? And then there’s a space that I call the extraorbital space. And that space includes everything outside the orbit. So you can see — this is the orbit. So the extraorbital space is basically everything outside, which includes the brain, the nose, the sinuses, and the skin. Okay? So, again, I already covered this, but the intraconal space, again, just to emphasize the point, is optic nerve, orbital fat. And anything in the intraconal space will what? It will cause axial proptosis. Right? Because it’s gonna push the eye right out. Anything in the extraconal space, like the lacrimal gland, the superior oblique muscle, nerves and vessels, will cause non-axial proptosis. And so that sort of helps you in determining, you know, exactly what’s going on. And this is a nice way to sort of memorize that, as to exactly what’s going on. Okay? Hematoma, abscess, in the subperiosteal space. The extraorbital space we’ll get to later on. Okay? So when you’re thinking about orbital disease, always think about the different tissues found in the orbit. And these include nerves and muscles and veins and arteries, you know, glandular tissue like the lacrimal gland, connective tissue, which includes fat, and other pigment cells and red and white blood cells. Okay? And any of these can give you pathology. Okay? So let me just see what questions we have, before we go on into primary orbital disease. Yeah. So when you visualize proptosis — the question is: Why do you visualize proptosis from below versus above. Is there a difference? What happens when you’re looking from above is that the eyelid, the upper eyelid, basically covers the whole globe, so you can’t determine the proper position of the eyeball. When looking at it from below, you know, the patient’s looking up, the upper eyelid is retracted, and the lower eyelid, it does not cover both eyeballs, and so you can tell much, much easier. Okay? Okay. Good. So let’s move on. So there is some examples, when it comes to orbital disease — so it can be separated into primary orbital disease or secondary. Primary orbital disease is a disease that arises from structures within the orbit. Okay? So this is an example of a patient that came to me a few years ago, with a huge chronic orbital schwannoma, that basically caused her to have significant proptosis. So, you know, the orbit contains nerve-like structures that can cause disease. Just to give you examples of what can happen. Now, the orbit contains muscles, and of course we all know that orbital myositis, which is an orbital inflammatory syndrome, can occur as a result, causing proptosis, and you can see here, basically, on her left eye, how in particular her inferior rectus and her medial rectus muscles are enlarged. So she has two-muscle myositis. There’s also veins and arteries in the orbit and around the orbit, and certainly here’s a patient that presented with angle closure glaucoma. And basically the patient had high pressure, had a bit of proptosis, decreased vision, shallow anterior chamber, and tortuous corkscrew vessels. Basically episcleral congestion. And we saw a very shallow chamber, and did a CAT scan and MRI scan, and it ended up being a carotid-cavernous fistula. The lacrimal gland also is in the orbit, and definitely anything involving the lacrimal gland, like dacryoadenitis, can cause proptosis and orbital disease. The most common connective tissue orbital disease is, of course, thyroid eye disease, which is an autoimmune inflammatory disease. Causing inflammation of the extraocular muscles and the connective tissue, like fat, around the orbit. Okay? So secondary orbital disease is basically disease that comes from outside the orbit, into the orbit. Okay? And when you look at the orbit in general, things that surround the orbit are facial and skull bones, is the brain, very close to the orbit, in very close proximity to the orbit, there is the sinuses that are all around the orbit, and definitely disease in the sinuses can go into the orbit, like most commonly infection and orbital cellulitis, and there’s also facial and soft tissue. And here you can see this is an elderly man, typical Caucasian man with northern European ancestry, has a lot of sun damage, actinic keratosis, on his skin, and obviously you know that a person like this is at a high risk for getting squamous cell carcinoma. Okay? Be aware, also, of pseudoproptosis. And there are some things that can cause pseudoproptosis. So basically the eye is not proptotic, but they have pseudoproptosis. And this includes unilateral high axial myopia, and I’ve seen that several times, where the patient comes in and just has a bigger eye, 27 millimeters axial length, and basically it looks proptotic. Sometimes, also, it’s not the fact that the one eye is proptotic, but the other eye is enophthalmic. And this is often common in patients with breast cancer. Often chronically, when breast cancer invades the orbit, it can also cause an enophthalmic syndrome, and basically retraction of the eyeball. Any eyelid pathology, upper or lower lid retraction, can also cause pseudoproptosis. So this is an example of a young teenager who came in with — referred for proptosis of the left eye. And when you look at her, she basically has no proptosis. CAT scan was normal. And so everything is fine. She has a bit of lagophthalmos here. So she basically had a lower eyelid retraction. And I ended up doing a hard palate graft on her, to lift her lower eyelid. And we’ll talk about those things next time, when it comes to treatment. So just to review a bit before we move on, the presence of proptosis suggests an orbital problem. History and physical will give you a differential diagnosis. Imaging is very important. If the diagnosis is unclear, get tissue. And your treatment is based on your diagnosis. So let me just see if I can answer some more questions. Well, how do you differentiate between proptosis in the eye that has ptosis in it? I mean, the key thing is — sometimes a patient comes in with ptosis in one eye, and they’ll come in questioning proptosis in the other eye, because the other eyelid looks retracted, in comparison to the ptosis eyelid. The best way to figure that out is by doing Hertel measurements, or basically looking at the eye from below. Measurement by ruler is not bad. I think measurement by ruler — to measure proptosis by ruler is basically the same as basically looking with your eye. So it’s not as accurate as a Hertel ex-ophthalmometer. So one question is: You know, whether you get — whether in thyroid disease, you get axial or non-axial proptosis. I mean, in thyroid disease, it depends. You can get either/or. Because it’s a connective tissue disorder, and if one or two muscles are affected, they can move the eye one way or the other. If the lacrimal gland is inflamed, it can push the eye down. So actually, in thyroid disease, you can get both axial and non-axial proptosis. Okay. So let’s move on. Okay. So… When it comes to orbital disease, history and physical exam is very important. I always look at — everywhere I go, I always teach my retinas and fellows about the 7 Ps. We all have our approaches to examining the orbit, and this is what I use. And the 7 Ps, three of which are in yellow, is history. So typically, when someone will present with orbital disease, they’ll either have pain — so the most important things are pain, progression, and past medical history. And basically when we go to examine someone for the orbit, we look at proptosis, palpation, pulsation, and periorbital changes. And I’ll go into this in detail, in 5 seconds. Or 2 seconds. So on history, pain, progression, and past medical history are very, very important. Okay? You know, the nice thing about pain — well, pain is never nice. Pain sucks, when it occurs. But the nice thing about pain from a physician’s point of view — it narrows things down. Because there aren’t a lot of things in the orbit that can cause pain. So inflammation causes pain. Infection causes pain. Any acute pressure changes in the orbit, like a retrobulbar orbital hemorrhage, causes pain. And finally, when a neoplasm gets bigger and bigger and bigger and starts to involve the bone or nerve, then you get pain. So beyond that, I mean, there isn’t anything else that can cause pain. So right away, that gives you about 4 diagnoses. The top two, inflammation and infection, being the most common. Okay? Progression is very important. Because it narrows down exactly what’s going on. So a rapid progression, anywhere from minutes to hours to a few days, there’s only a few things that can cause that. Retrobulbar hematoma can cause that. A very severe infection can cause that. Orbital pseudotumor or inflammation can cause that. Intermediate is anywhere from weeks to months. And slow progression of orbital disease is basically from months to years. And often many chronic orbital tumors like pleomorphic adenoma, that are slow growing, can typically cause pain over months to years. So progression — how fast orbital disease progresses is key in figuring out exactly what is causing — what is the process that’s causing the disease. So when it comes to — if you have a patient that comes to your office, and they have proptosis, which is how orbital disease usually presents, okay, if they have pain, then right away you know it’s either inflammation, infection, or hemorrhage. So you can basically focus in on those three things. Pain does not typically occur, commonly, otherwise. Neoplasm or tumor, metastatic disease, and congenital disease are very unlikely to cause pain. Now, metastatic disease, if it’s severe enough, it can invade the bone and cause disease that way, but typically it does not. Okay? Now, this is an example of a patient that comes in… Okay. That comes in basically with relatively acute proptosis. You can see usually in an emergency department or when I’m traveling, I don’t have a Hertel on me, so I just use the EyeCheck. And you can see probably about 3 millimeters of proptosis. Remember, it’s the asymmetry that’s important. We did a CAT scan. And he basically had a large subperiosteal retrobulbar hematoma, quickly expanding the periosteum, causing a lot of pain, and pushing on the optic nerve. Okay? So he basically had a spontaneous orbital hemorrhage. Pain, sudden onset, rapid progression over hours. Looking at these three things, really, and a history of trauma, really gives you a nice idea of exactly what’s going on, or diagnosis. Here’s a patient who comes in, who had pain. Progressed over days. And the proptosis also progressed over days. And, you know, he wasn’t feverish, he wasn’t sick, and basically this is orbital inflammation, also known as orbital pseudotumor, where it’s pain, it’s acute, and it’s progression over hours to days. This is a patient that basically has had a few months of mild eye pain. And very, very mild. And almost discomfort. And basically has had, over months, a slow progression of his eye getting larger and larger. Or more proptotic, according to him. Okay? And so obviously, if you do a CAT, so whenever you see someone with orbital disease, remember I said it’s history, physical, CT, MRI scan, and then biopsy? Those are three things you always keep in mind. We don’t often have all three available. Sometimes we just have history and physical. And don’t have a CT available and can’t do a biopsy, and so on and so forth. But in this case, we did a CAT scan. You can see all his muscles are enlarged. And basically, he has mild pain, not that severe, and actually — or no pain, in this case. And he has a progression over weeks to months. Really over months, in his case. And, you know, I should qualify that. With thyroid eye disease, most patients get a discomfort. They don’t get a pain. So when you ask them, they will say discomfort, or they’re uncomfortable, but they won’t say pain. And that’s important. And that typically is — because if this patient had a pain, then I would probably think more orbital inflammation, causing the proptosis, most likely. Or something else. This is a patient that came in, you know, that actually came in for a lower eyelid blepharoplasty, a cosmetic blepharoplasty, and we saw her, and because of the asymmetry, even when patients come to me for a lower lid blepharoplasty, I always touch the fat. I always touch the eyelid, and just sort of feel what’s going on. On the right eye, that did not feel normal. It was kind of hard. And I said… Well, this doesn’t feel normal. It doesn’t feel like normal fat. So I did a CAT scan. And you can see also — she probably has a slight proptosis. I mean, if you look at this, she probably has about 1 to 2 millimeters of proptosis. We did a CAT scan, and sure enough, she had this lesion there. Okay? Basically an orbital mass. This was not fat. Fat does not look like this. It looks like this. Okay? We did a biopsy. Ended up being lymphoma. And again, it was chronic, no pain, slow progression over months. So you can tell how pain is very, very important in narrowing down what exactly is going on. Because if she had a lot of pain, then you would think — well, this is probably infection. Or is it inflammation? Or is it hemorrhage? Right? I always mention the 8th P. And the 8th P is basically looking at old pictures. And the other thing I put down as an 8th P is looking at old pictures or neuroimaging. So CAT scan and MRI scan. I basically put them under an 8th P. And looking at old pictures is very important. Often patients will come, especially with thyroid disease, or a chronic tumor, like a pleomorphic adenoma, and they did not notice their eyes changing. And sometimes they don’t know how long they’ve been proptotic for. And to me, progression or the time it takes for a tumor to progress, is very, very important. So I always ask them: When did it start? And they often don’t know. Especially if it’s chronic. And so looking at their old pictures, I mean, I don’t know how it is in other countries, but I know here in Canada, we have a driver’s license, which typically gets renewed every five years. And that’s an easy way. So I basically ask them for their driver’s license, I take my 20-diopter lens, and I basically look at their driver’s lens to see whether there’s an eyelid retraction, whether I see some kind of proptosis, and that really tells you or gives you an idea of whether it’s chronic or it’s more acute. Past medical history is extremely important. Because it narrows things down. So obviously, if the patient comes in with a history of hyperthyroidism, elevated thyroid levels, hypothyroidism, Hashimoto’s thyroiditis, or something like that, that narrows it down to thyroid disease. Cancer anywhere in the body can basically also metastasize to the orbit. And I think I’ll come to this later, but I’ll mention it now. As you know, lung cancer is the most common metastatic cancer that metastasizes to the orbit, and both women and men. Of course, in women, breast cancer also is relatively common as metastasizing to the orbit. But cancer anywhere in the body is important. Okay? Trauma to the face is very important. Age and sex, of course, they’re very important, because there are some things that only affect you at certain ages, and some things that affect women versus men. Okay? And also be careful — I had some pictures, and I think if you have time, I’ll put them — or I may have it in unknowns. You’ll often get patients that will come in, and especially if they’re older — I mean, many parts of the world have very conservative cultures. But often a lot of the elderly people in this part of the world are also very conservative, and they just don’t tell. So I’ve had a patient, an elderly patient, about 75 years old, that basically had an orbital mass. We took a complete history, asked her about everything, she basically said nothing. She said everything’s fine. So we took her for a biopsy. When she was in the OR, she was lying down, we were basically — I was taking care of the orbit, I was marking it, injecting it, and the anesthesiologist was basically putting in ECG leads. So we basically open up her gown to put ECG leads in, and he saw basically this huge fungating mass coming out of her left breast. And she was a very intelligent lady. 70 years old. Hadn’t told her daughter about it. Hadn’t told anybody about it. And even her family doctor, who supposedly examined her, didn’t pick it up. So obviously, then, the diagnosis was obvious to me. She had this fungating breast mass. I went ahead and did the biopsy anyways, and of course it came back as breast cancer. So often these patients will not tell you. So it’s important to do a complete exam. I had a patient with melanoma to the orbit that, after I did a biopsy, she said — well, you know, I’ve had these bumps all over my body. On her breasts, on her back, on her neck, on her leg, and these were all melanoma lesions. And she died, unfortunately, about a month or two months after. But, again, people hide things. So it’s very important to take a good history. Okay. So history is pain, progression, past medical history. Let’s move on to physical exam. Which is the other four Ps. So… There’s four things to look at, when it comes to the orbital exam. One is proptosis, palpation, pulsation, and periorbital changes. Okay? So the four Ps. This is an image of eye patient with axial proptosis. You can see she has bilateral axial proptosis. Goes straight out. And, you know, there’s only two things. So when you see somebody with axial proptosis, remember I had mentioned early on — it kind of narrows in as to what’s going on? So anything that pushes the eye out — there’s only two things that can really cause it. Thyroid disease, with bilateral muscle enlargement, and any kind of optic nerve pathology or optic nerve tumor can push the eye straight out, causing axial proptosis. Other tumors, which are in the intraconal space, so between the recti muscles, like cavernous hemangioma, meningioma, glioma, they can basically push the eye out straight. So right away, you know what? Most likely it’s thyroid, but it could be an optic nerve tumor, and you narrow in on that. Non-axial proptosis — it’s also significant, because it tells you exactly what’s going on. So depending on the direction that the eyeball is deviated, so downward, laterally, medially, upward, it tells you what could be going on. You can see this man has obviously significant proptosis. And his eyeball is pushed down. And this is a chart that is nice to know. I mean, I think you guys probably already can figure this out. You don’t need a chart to tell you what. But just to give you an example, any large extraocular muscles will cause axial proptosis, intraconal mass, like I said. But looking at non-axial proptosis, when the eye is not straight out, if the eye is pushed down — so what’s superior? There’s the frontal sinus. There’s the orbital roof. There’s the lacrimal gland. So any kind of encephalocele, brain pathology, frontal sinusitis, anything like that, a superior dermoid cyst, anything will push the eye down. Laterally, of course, any pathology in the ethmoid sinus, like an ethmoid sinus mass, like a nasopharyngeal carcinoma, abscess, hemorrhage, will push the eye out. Anything that pushes the eye up is orbital fat, lymphoma can cause that, and medial, of course, you have enophthalmos and breast cancer. So it really isolates, as to what’s going on. Okay? Now, as I mentioned before to you with proptosis, any asymmetry is important. Any asymmetry measuring more than 2 millimeters is significant. Okay? Now, coming to the second P, which is — of the four Ps, of physical exam, is palpation. Okay? So when it comes to palpation, I typically start with the orbital rims, and move to the eye. I actually feel — this is going back to medical school, you know, and the nice thing about oculoplastics and orbital disease is that, when examining the eye, we just go to the slit lamp, and we don’t really touch the eye, except to open up the eyelid. With oculoplastics and orbit, I actually encourage my fellows, my residents, to actually touch the patients and touch their rim, touch their eyeball, feel any mass, feel the texture of the mass, the shape, the size, feel if there’s any tenderness, any temperature, so you’re actually practicing real medicine, unlike much of ophthalmology, that kind of takes us away from it, sometimes. That’s just my bias, of course. Let me move on. Third is pulsation. Which is feeling a pulse. Like a bruit or thrill or something like that. Again, this is all taking you back to basic medical school. Pulsation is not very common. So it’s not a common physical thing in orbital disease. But when it’s present, it’s diagnostic, obviously. There’s only one or two things that can cause pulsation. It’s typically arterial or venous malformation of some kind, or some kind of vascular mass — can cause pulsation. Okay? And, you know, there’s a thing of pulsatile proptosis, where you touch the eye like this, and you can feel a pulse. Obviously any orbital vascular lesion, like a congenital malformation, an acquired fistula, like a carotid-cavernous fistula, you know, sometimes you get certain cancers that are very destructive, and they either destroy the orbital bone — so absence of the orbital bone can also cause you to feel a pulsation. Okay? Here’s that patient we saw early on. Basically where you can feel a bruit or thrill when you press on his eyeball. And an earlier question was asked — somebody asked a question — how did we know to order a CAT scan when we saw this patient? The thing is, when I show you a picture like this, you’re just seeing one shot. So when you actually see him, if you see corkscrew vessels and episcleral venous congestion, you see shallow chamber, and you feel a bruit, then you know — you know what? There’s something going on that’s not right. This is not your typical angle closure glaucoma picture. And in that case, then you order a CAT scan, which leads to an MRI scan. And get the diagnosis. So this patient ended up having angiography, which did show basically an enlarged — now, to be honest, I don’t know how to interpret these either. So don’t ask me how to interpret these. They all look like beautiful paintings to me. But I did go through this with a radiologist, and basically this is an enlarged superior ophthalmic vein, apparently, and that’s a dural fistula that it’s draining into it, and this is a left meningeal artery. Basically it showed that the patient had a dural sinus fistula, and actually this was something which the neuroradiologist treated angiographically, and the patient did very well. Okay? The other thing to look for in physical exam is periorbital changes. So changes around the eyeball or around the orbit. Okay? I’m probably gonna go a bit over time. I apologize for that, but we started a little bit late, so I hope that’s okay with people. So when it comes to periorbital changes, look at skin, conjunctiva, eye, and bone. Okay? And that’s simple. So skin — what can happen with skin? Here’s a patient that came in with basically extraocular muscle limitations. Okay? Basically he had almost a frozen orbit on the left side. And you can see here — here’s more pictures of this — you can see here the patient’s looking up. The patient’s looking to his left. So the patient cannot abduct. Okay? Looking at the left eye. Again, cannot look down. So basically a frozen orbit. And you can see a scar here. So we asked the patient, you know, for any history of what happened. So I saw that, and we asked the patient — what is that? The patient apparently had a large squamous cell carcinoma that was excised and repaired with a skin graft. And it was very close to the orbit. And so we did — basically we did a CAT scan. And it ended up being that the patient had recurrent squamous cell carcinoma of the skin that actually invaded the orbit and caused a frozen orbit. And squamous cell carcinoma is known for that. It’s one of the cancers that can actually undergo perineural, so it can spread along the nerves, and actually enter the orbit, you know, and if any cancer is going to do that, usually squamous cell will do that. And basically, he had surgery 18 months earlier. So he had squamous cell carcinoma, he had it excised, had it fixed, and then about 18 months later, he comes up with pain and diplopia. Now, why does squamous cell carcinoma cause pain? Because what happens with squamous cell carcinoma is it invades the nerves, and that’s when it’s really painful. And you’ve really lost the battle. I’ve had several patients who I’ve even exenterated, that have had squamous cell carcinoma that basically spread down to their face, and have just lived in excruciating pain, because of the perineural spread. Okay? This is the patient’s left orbit. And you can see his muscles are enlarged. All the more enlarged. You can see here… And there’s — I don’t have all the pictures, but the other pictures show that there’s basically invasion with intraorbital spread. Here’s a lady that was referred to me for red eyes. For bilateral chronic red eye. Okay? And a young lady. You can see she must be about maybe 35, 36. And when you look at — when you basically look at her inferior fornices, she has basically — if you look closely, it’s not really your typical red eye. And basically it goes all around her eye, and looks almost like a conjunctival salmon patch. And that should tell you what it is. Right? So basically always look at it, because conjunctival lymphoma can masquerade as a red eye. And she basically had bilateral orbital lymphoma. And I’ve seen this actually several times. I’ve seen at least 10 conjunctival lymphomas in my career. So they’re not common, but they’re not uncommon. This is actually a patient that Dr. Bob Kurstin, who was my mentor, who I trained with — this is one of his pictures, and this is a patient that basically had extrascleral extension of his ocular melanoma. So that can also happen, where it spreads out, and it’s quite destructive and quite rapid. And that will be painful, because it invades the nerves. So that’s basically extraorbital extension and also extraface extension, kind of. Okay. So the game plan for examining orbital disease — evaluate the proptosis. Palpate the rims. Check for any pulsations. And look for clues around the orbit. Okay? And let me just pause now, and I’ll take some questions. Okay. Any management questions I’ll deal with later on. For sure. And definitely in question and answer, also, those that want to stick around afterwards, I can stay around and answer questions. That’s not a problem at all. Anything for surgical questions we’ll definitely cover in the next session, when it comes to treatment, and surgical treatment in particular, of orbital disease. Okay. Okay. Okay. Good. So we’ll move on. So some of these questions we’ll come to as we move on, okay? Good. Okay. And, of course, when it comes to orbital disease, don’t forget the eye, which is really what we’re all about, isn’t it? So always remember: Check for vision. Check for pupils, visual field, color vision, extraocular motility, and also retropulsion. All of those are important, and they can definitely all be affected by orbital disease. So we already reviewed this. When it comes down to approaching orbital disease, history, physical, make a differential diagnosis, narrow your choices down. So thyroid eye disease is the most common cause of unilateral or bilateral proptosis. Determine if urgent or elective referral is required. And these you guys probably now know like the back of your hand. So let’s skip that. Good. So what is the most important diagnostic test in orbital disease? The MRI scan, CT scan, orbital ultrasound, orbital angiogram, OCT, or visual fields? Okay. Let me get this here. Okay. Yeah. So, I mean, in my… Yeah. I think most of the people have it right. The one most important diagnostic test in orbital disease really initially is the CAT scan. Okay? The MRI scan is important for looking at the apex, getting more detail of the muscles. The orbital ultrasound is not used anymore. You know, if someone doesn’t have a CAT scan, you can maybe get a rough idea what’s going on, but I don’t use it anymore. The OCT and visual fields are important, but they’re more important for the eye and to find out if the orbital disease is actually damaging the eye. And the orbital angiogram does not exist. That was a trick question. Okay?

>> Dr. Khan, you can push these to the audience.

DR YASSER KHAN: So orbital imaging. Almost all patients with proptosis require it. And I do understand in some parts of the world this may not be available, and therefore the ultrasound may have a role in these parts of the world. A good history and physical may also have a role in some parts of the world, and also, when in doubt, and there’s no imaging, and you can actually feel the mass, then maybe doing a biopsy, if you have no other choice, would also be good. And x-ray is not very useful in determining orbital pathology. You can discover foreign objects and things like that, on an x-ray, but it’s not really a very useful test in orbital disease. Okay? So all patients with proptosis, if possible, require some kind of imaging. And imaging has two purposes. Diagnostic information. What is the mass? And also surgical information. How would I approach this mass, to biopsy it? Okay. With orbital imaging, there’s three imaging things. One is the CAT scan, MRI scan, and ultrasound. So I’ve covered this. Okay? And the CAT scan gives you — is excellent for viewing orbital bones and most orbital structures. The CAT scan gives you spatial anatomy. It allows you to determine, view the orbital spaces, very nicely. It allows you to determine exactly where the mass is, in relation to the eyeball and the orbital structures. And really, when approaching surgically, it clearly tells you what the surgical space is, so you know exactly how to plan your surgical approach. The MRI scan is basically good for — I mean, it doesn’t use radiation. Its main indication, in my mind, is when viewing the orbital apex or the orbital cranial/brain junction, or sometimes if it’s a vascular lesion, or it involves the muscles and you need further clarification. Okay? What is the most common cause of proptosis, unilateral or bilateral, in adults? Is it lymphoma, orbital cellulitis, trauma, thyroid disease, or orbital inflammatory syndrome? Okay, yeah. It looks like everybody is getting this correct. Basically the most common cause of unilateral or bilateral proptosis is thyroid disease. By and far. And you always have to — I mean, when you see a painless proptosis, you have to rule out thyroid disease until proven otherwise. Okay? So common causes of — so now we’ll sort of delve into — we’ve talked about the basic history and physical and imaging when it comes to orbital disease. Now we’ll sort of dive into causes of orbital disease. Okay? And of course, like I said, consider thyroid eye disease as a cause of proptosis always, in any kind of orbital disease. Okay? Now, when it comes to orbital disease, you have to sort of differentiate whether it’s in adults or children. Because typically speaking, adults and children will have different spectrums of orbital disease. Okay? And understanding the disease process will help you manage it. Now, we can do — thyroid eye disease is a whole lecture by itself. And I’m not gonna cover it here. But I’ll just briefly touch on it, because it is the most common orbital disease that we all face, I think, internationally, really. Not just here. It’s the most common cause of proptosis. It’s an autoimmune disease, associated with systemic thyroid disease. About 90% of patients have hyperthyroidism. Less than 2% have hypothyroidism. And that number actually — it’s actually about less than 5% are actually euthyroid. So basically the bottom line to remember is that about 90% of the time, it’s hyper. There are some patients who also will have hypothyroidism, and will have euthyroid. Which means that their thyroid hormone levels are normal. And many of these patients with normal thyroid hormone levels will, at some point in their life, often convert into a hyperthyroid stage and get Graves’ disease. So just because people’s thyroid levels are normal doesn’t mean that they don’t have Graves’ disease. Women are 5 to 6 times more likely to get thyroid eye disease. Which is not a surprise, because women do get autoimmune diseases much more commonly than men do. The onset is typically in the early 40s or mid-60s, although we do see patients in their 20s and their 30s getting it. But typically it’s late 30s or 40s, is what I see. And sometimes the second spectrum is patients in their 60s. Okay? The onset is usually gradual, with variable progression. Pain is not present. It’s not a big feature at all. Discomfort is. Smoking a major risk factor. And so I always warn patients that smoking increases their risk of eye disease. Patients who have thyroid disease, who have hyperthyroidism, smoking increases their risk of eye disease about 8 times. And thyroid eye disease or orbitopathy, is twice, two times more common, in patients who smoke than not. So whenever I see a patient with thyroid disease, I always tell them to stop smoking and really encourage them to stop smoking, because it does make a difference. Symptoms include discomfort, fullness, pressure, tearing, photophobia, blurred vision, periocular swelling, and diplopia, if their muscles are really involved. Typically their proptosis is asymmetric. It can be symmetric sometimes. And it varies from patient to patient. They can get axial or non-axial proptosis. And the eyelid signs are often present. Basically upper or lower lid retraction. So this is a typical example of patients with almost axial proptosis. It’s asymmetric, and she also has significant eyelid signs with bilateral eyelid retraction. But worse on the left eye. And you can see, if you do the proptosis check, she probably has about 3 to 4 millimeters of proptosis, with the right one being worse than the left. But it’s interesting. If you looked at her just straight, you’d think that the left eye is more proptotic than the right eye. And that’s what eyelid retraction does. It’s almost like it optically fools — it’s like an optical illusion. It fools you and it basically indicates that one eye is — it basically gives you the image of being proptotic, when you may not be. So it causes — it creates pseudoproptosis, when in fact you can see her right eye is the eye that’s more proptotic. Another example of a patient with eyelid lag and left upper eyelid retraction. And when you see someone with an eyelid retraction, 99%, you’re sure this is Graves’ disease. Because there aren’t too many other things. There are some rare tumors and trauma in surgery, but there aren’t too many things that can cause eyelid retractions, except for hyperthyroid or Graves’ disease. Okay? So, I mean, this is basically eyelid retraction, eyelid lag, lower lid retraction. These are all common signs and symptoms of thyroid disease. A lot of patients with thyroid disease will get a temporal flare, where the temporal eyelid goes up, like this. And that also is very common. Okay? You can see a nice contour here, and that flares up. Another patient with thyroid disease — you can again see that it looks like his left eye is more proptotic. But in essence, it’s his right eye that is more proptotic, and he even has some corneal scarring. Because of chronic lagophthalmos. So often patients will present with axial proptosis. But a significant amount of inflammatory signs. And corneal exposure. So this patient had a lot of lagophthalmos, a lot of injection and corneal exposure. Okay? And, of course, they’ll have extraocular motility restrictions, as the muscles are quite large. Two things to watch out for with thyroid disease is corneal pathology and optic nerve dysfunction. So whenever patients come in, I always check their color vision. I check their pupils. Obviously their vision. And I will often do visual field and sometimes an OCT of the optic nerve now as well, to rule out any optic neuropathy. I don’t always image my thyroid disease patients. If it’s very obvious they have a systemic disease, and it’s quite obvious that there’s nothing going on, I don’t image routinely. If I have any doubt, or if I feel that their proptosis is significant, or if they have visual loss, then I will image them, to make sure everything’s okay in the orbit. We can see this patient was imaged, and we can see huge recti muscles. And so these are patients that — and that’s the optic nerve. So they’re very close. So these are patients that you need to monitor closely for any optic nerve compression. So in summary, then, thyroid disease is a self-limiting disease. The typical burnout — it typically burns out in about 1 to 2 to maximum 3 years by itself. I always reassure the patient that this is an autoimmune disease. You have to stop smoking, you have to control your thyroid hormone levels, your TSH. I typically follow them with their endocrinologist or their family doctor, and I wait ’til they’re stable for at least six months, and then I treat surgically whatever is left behind. Meaning eyelid retraction, proptosis, and things like that. Okay? Let me see if I can just take some questions on thyroid disease, if there are any. Okay. There’s a question asked — why would thyroid disease affect an eye more than the other tissues in the body? It’s unknown. There are certain growth factors and certain elements of the extraocular muscles and the fat that people are studying that basically attract more of the immune reaction. And that’s why people are trying to modulate those factors. But it really is nothing that’s been confirmed, as to why it occurs in the eye more than any other structure in the body. Okay. If thyroid hormones are normal, can we rule out thyroid eye disease? No, you can not. Because the patient could be euthyroid, and at some point in the future present with thyroid disease. Present with hyperthyroid disease. The best imaging method, like I said, to evaluate proptosis, is a CAT scan. There’s a question asked: What do I mean by burnout? I’m sorry. What I meant by burnout, and thanks for clarifying that, is that thyroid disease, after about 1 to 3 years, it ends by itself. The whole active inflammatory phase and process stops. It basically burns out, meaning the disease finishes by itself. So if you did nothing for 1 to 2 years, maybe 3 years, thyroid eye disease will eventually stop. The whole inflammatory process will eventually stop. But then we’ll be left with all the scarring and all the side effects of the inflammatory process. And that’s what I mean by burnout. It typically burns out by itself. It finishes by itself, basically. Okay? Good. So let’s move on. And finish up. So when it comes to management of thyroid disease — and this is probably a future lecture, because I think thyroid disease is a very major topic, but initially when patients come in, they have some mild foreign body sensation. They have mild eyelid retraction. I follow them closely with some ocular lubrication. I reassure them that their disease will burn out, will finish by itself, between 1 to 2 years, so to be patient. And I see them every 6 months to a year. Sometimes they come in with acute, progressive inflammatory symptoms. They have eyelid edema. They have retraction. Severe proptosis. Corneal compromise. Diplopia. Compressive optic neuropathy. And for this, typically what I do is — obviously I make sure that there’s no optic neuropathy going on. Sometimes if it’s really severe, I will put them on oral prednisone. I start with 60 to 80 milligrams, depending on how big they are, and I taper down over about 3 months. And sometimes when it’s really progressive, and they have significant optic nerve neuropathy, then I’ll give them steroid at a high dose, and I’ll book them for surgery, to do an orbital decompression. So in the active stage, what I do is I monitor the patient. If everything is fine, if the cornea is okay, if the nerve is okay, I just monitor them. I give them some ocular lubrication, and like I said, if it’s severe, there’s three things: Either steroids, or if they’re intolerant to steroids, or they’re having a lot of side effects like diabetes and other side effects with steroids, or if I can’t control them on steroids, then I will typically move on to immunosuppression. So non-steroids, like methotrexate, and things like that, and typically I don’t do that myself. I typically combine my care with an internal medicine specialist or a rheumatologist for these very strong immunosuppressive drugs. I will sometimes use radiation, focused orbital radiation, if it’s really severe and nothing helps, and also decompression, if need be. And we can definitely talk about this at our next talk, when it comes to surgical management of thyroid disease and radiation. Okay? In the chronic stage, I reassure them, give them tears, if there’s no change in 6 to 9 months, then I talk to them about cosmetic improvements. So whether it’s decompression for severe proptosis, eyelid retraction or eyelid lengthening, or strabismus surgery. Remember, with thyroid disease, as you know, you always treat the orbit first. Then you do strabismus surgery, if need be, and the last thing you do is eyelid surgery. Okay? Okay. So we covered this already. Another patient. You can see. And I followed her along. Okay? I mean, there’s really not much you can do at this point, until she’s more stable. And you can see after she’s stabilized, all I did was an eyelid lengthening, of her left upper eyelid. And you can see how dramatically that… I’m sorry. That improved her appearance. So you can make a big difference in patients’ lives. We covered this before, but this is a patient with — moving on — with orbital inflammatory disease or pseudotumor. Acute onset of pain, rapid progression. It’s basically the opposite of thyroid disease. And we’ll come to the treatment later on, but basically pseudotumor is treated with steroids, primarily, and patients do respond quite well. Lymphoma is a great masquerader of anything. Lymphoma can masquerade as multiple orbital processes, and there’s been sometimes with my fellows — I often have bets as to whether this is lymphoma or it’s a pseudotumor or it’s something else. Because it can look like anything. It’s the most common malignant orbital neoplasm in adults, and you can often palpate it. It’s usually smooth, mobile, it’s salmon-colored. Even when you look at it surgically, it looks pinkish in color. Gradual onset, slow progression, painless orbital mass, either axial proptosis or non-axial, 20% can be bilateral, and it’s basically a good malignant cancer to get, because it’s very sensitive to radiation treatment. Lymphoma is. And so what I do with orbital lymphoma is I will send them to their family doctor for systemic evaluation. If they have no systemic disease, about 80% of lymphomas, even more so, orbital lymphomas, are confined to the orbit only. Less than 20% have systemic spread. If they do, then they need to combine treatment with radiation and chemotherapy. If there’s no systemic involvement, which is most of the time, then I simply will go and do a biopsy, confirm lymphoma, and send them for radiation therapy. This is a patient that came in with conjunctival lymphoma. And again, it was all around the globe. It’s bilateral. And she ended up going to radiation and it cleared up. Here’s a patient that was referred to me for left upper eyelid ptosis. I saw the patient and I said… Listen, something’s not right. He had a bit of fullness right in his superior sulcus right there, a bit of fullness that wasn’t noticed by the referring doctor. I went and I felt the area right there, and I felt a bit of fullness and firmness. I did a CAT scan, and the patient had diffuse, basically, lymphoma. Unfortunately, the referring doctor didn’t lift the eye. Otherwise he would have seen this lymphoma that was there. And again, like I said, you have to work them up systemically. Make sure that they’re okay. Do a biopsy. Confirm the lymphoma. And then send them for radiation. If there’s systemic spread, then they also need chemotherapy as well. Okay. We talked about the metastatic tumors to the orbit. Usually they cause non-axial proptosis. If they invade bone or nerve, they’ll cause pain and inflammation. Okay? Progression can be days to weeks, when it comes to metastatic tumors. And eyelid swelling and chemosis is common. Always ask for history. That’s where past medical history is very important. Lung is more common in males, breast in females. Other things like prostate, colon, and intestinal stuff can also metastasize to the orbit. Okay? And like I said, unlike primary malignant tumors of the orbit, these can cause pain. This is a patient with relatively quick proptosis. You can see she has almost like a turban on, because she’s lost her hair. So you know that she’s gone for breast cancer treatment. And you can see her bone scan shows lytic lesions. Meaning she has metastatic breast cancer. And you can see a very destructive lesion right… Quite destructive, occurring in the orbit. You can see some bone destruction there, and that’s what’s causing her pain. So she had metastatic breast cancer. Optic nerve tumors. There’s really three main ones. There’s a meningioma, there’s sphenoid ring meningioma, and there is basically an optic nerve glioma. That most commonly occurs in children and not adults. Optic nerve meningioma is the most common optic nerve tumor. And it’s common. Usually in a middle aged man or woman. Vision loss is obviously very common. Very slow progression. And typically they have axial proptosis. You can see these optociliary shunts. So basically — I mean, when it comes to meningioma, meningioma in adults is benign. In children, it’s significantly malignant. And aggressive. A glioma, optic nerve glioma, if you recall, in adults, it’s a malignant diagnosis, and in children, it’s a benign diagnosis. So often in these patients, with meningioma, if it’s just the one eye affected, the meningioma is not spreading across the optic chiasm to the other side — then we simply just watch these patients. And I’ve got about three patients, young patients, that I’m actually watching. And I see them every year with a MRI scan to make sure that there’s no progression to the other eye. And their vision actually is 20/40, 20/50. So it’s not that bad. And I just monitor them. So you don’t necessarily do surgery, unless it’s spreading or there’s a risk of causing visual loss to the other eye. Other secondary orbital tumors, like periocular tissues, like skin, eye, sinus, can all cause disease as well. This is not my picture. It’s, again, my preceptor, Dr. Kurstin’s picture. Here’s a patient that he saw in, actually, Saudi Arabia. This is many, many years ago. That came to him from the desert, and basically had maggots growing around his orbit. And that basically were almost at his apex. The patient survived. Lost the eye and everything, but he basically survived. But this is a classic picture of what can happen sometimes when you let things go. And why? Because he didn’t have any pain. Right? Often with tumors and some processes, if there’s no pain, patients don’t show up. I will end off with this. I know we’re a bit over, and I apologize for that, but I will end off with this. And that’s the orbital diseases in children. Okay? There are some general differences between children and adults. Malignancy is a less common cause of proptosis in kids. Congenital, more common. Thyroid orbitopathy, although it does occur, is a rare cause of proptosis in children, and the infection is of course more common in kids. Now, the one thing I need to emphasize, and that’s why I’m going through this — is that in any child with proptosis, please consider rhabdomyosarcoma always in the back of your mind, because obviously if it’s a baby, it’s retinoblastoma, but when they’re older children, always consider rhabdomyosarcoma. I’ve had children as old as 15 be diagnosed with rhabdomyosarcoma. So always have that in the back of your mind, okay? Because if you miss that diagnosis, the child is dead, basically. So it’s a very important diagnosis not to miss. Okay? The two most common causes of proptosis in kids are congenital, either dermoid cyst or capillary hemangioma. Infection, preseptal cellulitis is common in children, as is orbital cellulitis. Because children have underdeveloped orbits, and so the spread of infection from the sinus into the orbit is easier than in adults, so they get orbital cellulitis and orbital infections at a greater rate than adults do. Okay? So a dermoid cyst is a choristoma. Which is normal tissue in an abnormal location. So often if you open up a dermoid cyst, you will see hair and skin and things like that. A hamartoma, which is a capillary hemangioma, is normal tissue in an abnormal quantity. Okay? So dermoid cyst is the most common orbital tumor. Usually occurs in the first month of life. It’s located in a supratemporal location. It’s painless. Proptosis is uncommon, because basically it’s right at the suture. Right at the superior orbital rim. And excision — you can excise it whenever you want to, or whenever the parents want to. Another example of a dermoid cyst right there. Okay? Sometimes a dermoid cyst, although temporal, can also be medial, causing a medial sort of elevation or a medial mass. I’m sorry. That also can be excised. And they do come out very easily. It’s a very typical classic appearance. It’s white. If you cut into it by mistake, it’s got this pasty appearance to it. Okay? A capillary hemangioma is not present at birth, and occurs after birth. It’s cutaneous or can be orbital. The upper eyelid and forehead are more commonly involved. And typically, the rule is that it goes away by itself. Involutes by itself. And that’s the rule. Typically by the time they’re about 7 or 8 years old. So you have the initial growth phase. Then you have a stable phase, at about 6 months — at about a year. And then from about a year onwards, it starts regressing. And some have actually regressed quite well. You can see here’s a baby that was initially born with… So different degrees of capillary hemangiomas. Okay? And like I said, onset typically occurs about 6 weeks after birth, rapid enlargement from 6 to 9 months, and involution begins at about 1 year, complete by about 8 years. Okay? Sometimes if it’s really large, or it’s causing visual loss, that’s when you have to go in and do something. Okay? The key thing, as you know, with children, is to follow them for amblyopia, astigmatism, so correct the uncorrected refractive error, and bony orbital asymmetry. Treatment, depending on what goes on or where they are in the course of their disease, includes intralesional steroids, oral prednisone, and surgical resection. There has been some — for the last maybe about 10 years, people have put these patients on an oral beta blocker, that has had some pretty good results, and some people, actually, if it’s small enough, that’s basically first line treatment now. Is that these children are put on a beta blocker, like propranolol. Typically when I do that, I do team up with a pediatrician, and put the child on, and it actually has shown a pretty good evidence, if it’s small enough, and not so intrusive, to involute faster with a beta blocker. This is a child that was born, and at about age 8 or 9, you can see significant decrease. So they do involute, and they do go away. Okay? More pictures of involution. You can see how it clears up. I spoke about orbital cellulitis a little bit. It’s the most common cause of childhood proptosis. And I spoke about why kids get it more often than we do, as adults, because their sinuses are underdeveloped. Their orbital bones are much thinner. The orbit is not fully developed, so any kind of respiratory infection can spread into the orbit and cause cellulitis. And you can see here a patient presents with proptosis, motility restriction, and chemosis. And we do a CAT scan. Ends up having a subperiosteal abscess. Preseptal cellulitis is more common in children. It’s an infection of the eyelids and soft tissue, anterior to the septum. Any kind of insect bite or skin wound can cause it. If there’s any orbital signs, then it becomes orbital cellulitis. Okay? Here’s a patient with a bit of a cut in the skin. And eventually develops like a preseptal cellulitis. So with rhabdomyosarcoma, any painful proptosis or progression from days to a few weeks — you have to think about rhabdomyosarcoma. It’s a malignant tumor of orbital soft tissue. Very malignant. 4% of childhood orbital masses. So it’s not uncommon. Mean age is 8 to 9 years old, and like lymphoma in adults, it’s a great imitator. It can imitate as anything. It causes non-axial proptosis, and if the tumor is isolated to the orbit, there’s a 90% cure rate. Which is great. It’s really — and I’ll come to that in a second. Here’s a child with basically rhabdomyosarcoma in the inferior orbit that was treated and biopsied. Okay? And the child did very well. Typically, this is a rhabdomyosarcoma that basically spread quite rapidly, and was left untreated. Okay? And the child died shortly thereafter. Unknowns. Let’s go through this. Here’s a patient with an orbital inflammatory syndrome. Was diagnosed with orbital cellulitis. Did not respond. I typically — when these patients come in, if they have — if they’re kind of feverish or I’m talking on the phone to somebody, I’ll put them on antibiotics for about two days, and if they don’t improve, then I’ll put them on steroids. Patients who come in with orbital cellulitis are usually very, very sick. Okay? And are very toxic. So you know that. Patients with orbital inflammation are typically not that sick. Don’t have a fever. Typically speaking are not that toxic or are not that sick. Okay? This is a 65-year-old patient who was, again — I mentioned him to you. Came with ptosis and diagnosis and ended up having lymphoma. This is a patient, 19-year-old patient, referred for a left upper eyelid ptosis. You know, I looked at her. She had a bit of proptosis going on in the left eye. Things just didn’t seem well. Ordered a CAT scan. Ended up having a large dermoid cyst, basically, that was invading her brain. Pushing against it, I should say. This is a patient with a preseptal cellulitis. And an abscess, basically. The globe position is normal. The motility was normal. And the vision is normal. So it’s not orbital cellulitis. Okay? This is a patient with orbital cellulitis. You can see quite significant proptosis and involvement of all periocular tissues. And high fever. This is a patient that presented with a bit of swelling, and basically pain with excursions and mild limitation upgaze. He was diagnosed with orbital cellulitis, treated with IV antibiotics, and did not improve after about 5 or 6 days of therapy. A CAT scan was done. Showed a very enlarged superior rectus muscle. The patient actually had orbital myositis, was treated with prednisone, and did quite well after. So, looking at these pictures, who do you think has orbital cellulitis and who do you think has orbital inflammation? It’s hard to tell sometimes the difference between the two. But the key thing to remember is patients with orbital cellulitis will be thick and will be toxic. So the woman in the picture above had orbital cellulitis, and the man below actually did not have cellulitis, but had actually orbital inflammatory syndrome. She had a high fever. Just was unwell and looked a lot sicker than he did. And they were both treated with antibiotics initially. She improved. He did not. He was put on steroids and he improved quite rapidly after. Patients, again, were referred for a right ptosis. So whenever you get a patient with a ptosis, always look at them. Touch them. Make sure there’s nothing in their superior orbit or their superior sulcus. And you can see he had bilateral, actually, lymphoma. Here’s a patient that was referred to me to rule out tumor. Okay? This patient ended up having a capillary hemangioma. So it actually was not even a lymphoma. But you can see very similar. These are two different kids. You can see on the right side is the child I showed you with rhabdomyosarcoma. And on the left, you see a child with capillary hemangioma. So it’s quite frightening, sometimes, because an orbital capillary hemangioma does not always manifest on the skin. And they can have very similar pictures, but this is benign and this is deadly. So it’s very important to always think of rhabdomyosarcoma in the back of your mind, because you can’t say this is capillary hemangioma, because you’ll basically kill the child. So just always remember that. This is a 25-year-old man who came in. His vision was improving. So his vision was improving, and he came in with a diopter shift. And a bit of optic nerve swelling. Optic nerve head edema. And he basically had a cavernous hemangioma in his intraconal space, pushing his eyeball out, basically creating a 3-diopter axial hyperopic shift. So he was myopic to begin with. No wonder he had an improvement in his vision, because he had this hyperopic shift of this tumor, pushing against his eye. Patient presented with left ptosis. She basically had quite significant GPC, and she had been like this for about a year. She basically had a contact lens stuck in her fornix for over a year, causing GPC, or giant papillary cell conjunctivitis. And then that was taken out and she was treated and she did very well. And that’s the end of the presentation. Thank you for sticking around. I apologize. I went a bit long. For those of you who are around, I’m happy to stay on and answer any questions. I can go through the questions now, and then… I don’t know if everybody is still around. I hope you are. I can answer questions and go from there. Okay? Do you recommend selenium in the first stages of Graves’ disease? Selenium… There’s been some studies done, and actually, there’s a large randomized study going on still in the US on selenium. Which is basically a vitamin. Basically. In the early stages of Graves’ disease. On whether it prevents the progression of Graves’ disease and progression and onset of complications and things like that. You know, I mean… To be honest, there’s one large prospective study going on. The results thus far have been inconclusive. I don’t think there’s anything to lose, in my opinion. So I will definitely put patients on this, because there’s really not much to lose. Okay? With biopsy of lymphoma, how would you do it? If the patient can clarify how… Basically, when I do a lymphoma, I always inform the pathologist I’m doing it. I don’t put the specimen in formalin. I will actually send it fresh. Because often the pathologist likes to do immunohistochemistry and cytology on these specimens, to find out exactly what the phenotype of the lymphoma is. So I will often send lymphomatous tissue fresh. I never do an excisional biopsy, meaning I never take the whole thing out. I will do an incisional biopsy. Basically give them just a bit of tissue. And I typically do it fresh. So they can do cytology. Having said that, I’ve spoken to a lot of the pathologists over the years, and they say that even if you put the specimen in formalin, they may not be able to do cytology, but they’ll be able to give me a diagnosis as to whether it’s lymphoma or not. And sometimes a pathologist is not around, and if you don’t put a specimen in formalin, it will basically degrade and be contaminated and will not be good. So if you don’t have a pathologist available or it’s not close by or whatever, then just put it in formalin and send it. Okay? How do you differentiate between a pseudotumor and orbital cellulitis? So basically — good question, Javid. And I think I already answered this, but basically, again, patients with orbital cellulitis will be sick. I mean, they’re typically sick patients. Patients with orbital inflammation have a lot of pain, but they’re not ill. And that’s how I primarily differentiate. A CAT scan — you know, both pseudotumor and infection will give you that diffuse infiltrative appearance to the orbit, so you can’t really use that to differentiate. I think the main thing is — sorry, the other thing is, on CAT scan, often patients with with orbital cellulitis will have their sinuses — like, will have diffuse sinus disease. And that’s basically where the bugs come from. They come from the sinus into the orbit. Whereas patients with pseudotumor will not have diffuse sinus disease on CAT scan. Okay. Why do we do eyelid procedure — why do we do eyelid procedure before strabismus surgery? Actually, we don’t. Ideally, if a patient has a lot of diplopia, if a patient — in Graves’ disease, if the patient has diplopia, and they have significant restrictive strabismus, where they have inferior rectus restriction, basically, in upgaze, or any other extraocular muscle abnormalities, that has to be fixed first. Because, as you know, the eyelids are intimately related to the extraocular muscles. Especially superior rectus and the inferior rectus. So you have to release — or you have to do inferior rectus recession before you do any kind of eyelid surgery, because part of the eyelid inferior retractor muscles, you know, are formed by parts of the inferior rectus muscles. So always in thyroid disease you do the orbital surgery first, so you do decompression first, then you fix the muscles, and then the eyelid is always done last. Otherwise, if you don’t do it last, then you’ll change things. Is it necessary to look for a level of antithyroid antibodies in the management of thyroid disease? Yeah. Good question. I didn’t basically go into a lot of detail on thyroid disease and management or whatever, because I’ll do that at a later time in a different lecture, but basically yes. I routinely will order a thyroid — TSH, 3T4, and I will also order antithyroid antibodies, because that confirms my diagnosis. Especially if the thyroid hormone levels are normal, like they’re euthyroid, or it’s not obviously a thyroid disease, and I suspect something. Okay? Did you say a contact lens under the eyelid for a year? Yes. The patient had a contact lens in the fornix for a year. As you saw, the patient’s pretty young. And I don’t know if you know teenagers. When you’re 16, 17, you ignore a lot of things. And she apparently did, and this contact lens was hidden, basically, in her fornix, causing a very significant giant papillary conjunctivitis. I will — do you inject steroids directly into the capillary hemangioma? Yes, I will. And if it’s large enough. If it’s causing amblyopia. And, you know, causing a lot of astigmatism, then I will inject some Kenalog into the steroid, and I’ve had some pretty good results with regression. If it’s severe enough, I will give them oral prednisone. Okay. I don’t know if everybody’s still here, but I will answer a few more questions. Let me know when you want me to sign off. What dose of steroids do you use in orbital inflammatory syndrome? Depending on their weight — you know, there are some guidelines per weight, but basically when it comes to orbital inflammatory syndrome, if it’s a normal sized man or a woman, I will start them off with 80 milligrams per day. Okay? So basically I will continue that for about 3 to 4 days, depending on how severe they are, and after 3 days of giving them 80 milligrams, I will come down to 60 milligrams, and then I’ll taper them every 6, 7 days. I’ll taper them by basically 5 to 10 milligrams. So I’ll go 60 for about 6 days, 50 for 6 days, 40 for 6 days, then I’ll go 35, 30, 25, 20, all basically a 6 or 7-day decrease, and finally, when I get to 10 milligrams per day or 5 milligrams per day, then I’ll prolong those. 10 for 3 weeks, 5 for 3 weeks, and 2.5 milligrams for another few weeks. But basically, in your mind, keep a 6-month schedule. So if you taper them too fast, they’ll rebound. So I basically have them on steroids for about 6 months. Obviously, for the last few months of the 6 months, they’re on 2.5 or 5 milligrams, but I’ll have them on a low dose for that long. I’ll make sure that their sugars are managed by their family doctor if they’re diabetic. Even if they’re not. I’ll make sure that they’re taking their calcium, and all the precautions. I have an adult female patient with proptosis and diplopia with MRI negative and thyroid disease negative. What do you do next? I would definitely do a CAT scan of the orbit, for sure, next. Because sometimes — I mean, it gives you a better spatial idea of where the orbit is. So that’s what I would do. I would do a CAT scan. And IV steroid for thyroid actually works very well. I didn’t mention it, because it’s often not practical for people, but typically what I do is, when someone comes in with acute thyroid eye disease, has a lot of diplopia, has a lot of proptosis, has a lot of acute — I’m sorry. The question was: Somebody asked a question — what about using IV steroids for thyroid eye disease. So yeah. If someone comes in with acute inflammatory signs, congestion, a lot of diplopia, I will actually give them a 6-day course of IV steroids. So I’ll have them go to a clinic. People will put in an IV saline lock, and I’ll basically give them 500 milligrams daily IV of IV methylprednisolone or something like that. 5 milligrams for 6 days. And then I’ll see them back after. Typically sometimes you’ll need about 2 or 3 of these 6-day treatments. And I’ve had some pretty good results with that. The nice thing about giving IV steroids is that you avoid much of the systemic side effects. You avoid the stomach irritation. It’s rapid. It goes right… It’s intravenous, so it’s a rapid effect. And so it works very well. And so that’s what I do more often. But in some parts of the world, you can’t arrange that easily, and that’s why I didn’t mention it. But definitely IV steroids do work. The other question: Is there any role for IV methylprednisolone for orbital pseudotumor. Of course there is. You know, but the thing with IV methylprednisolone is — often, with thyroid disease, if they have acute inflammatory symptoms, you can give them the IV steroids for a 6-day course and then see them and they’ll do better. The thing with IV methylprednisolone for orbital pseudotumor is, because the inflammatory reaction is different, although it is autoimmune, but it’s a different reaction, I think that they need steroids longer. Especially if it’s severe. So I think that you can’t give somebody IV steroids for months and months. Right? So, I mean, if it’s really severe inflammatory disease, then I think that I would give them 80 milligrams — sorry. I would give them 1 gram, 1 gram methylprednisolone for 3 days. Once a day for 3 days. And then switch them to oral steroids, and I think that will give them that kickstart in treating their orbital inflammatory disease. What is the best route for fat decompression in thyroid eye disease? I mean, we’ll cover that for sure in part II, when we do the surgical management. But I basically use a transconjunctival approach, when I do my fat decompressions. Or a transcaruncular approach. But we’ll cover that in the next session. Another person is asking: Can you elaborate more about enophthalmos of the non-proptotic eye? What happens sometimes is that, especially with breast cancer, breast cancer causes — as it spreads to the orbit, it causes a retraction, almost like — what’s known as cirrhotic breast cancer — causes a retraction or a fibrosis — I’m sorry. It’s a fibrotic process. It causes fibrosis of the orbit, and that causes retraction of the eyeball, so you actually get enophthalmos. And so that’s a problem. And so the other eye appears to be proptotic, then, in comparison. Thyroid eye disease is an autoimmune disease. And so the antibodies are produced in relation to the… As a response to the immune factors that are floating around in the bloodstream. And… Yeah. It was asked… With capillary hemangioma, do you use propranolol? Yes, we do. I typically do not use it myself, because when it comes to children, I don’t bother getting into systemic things. I will work with a pediatrician who will then put the kids on a beta blocker, which is propranolol. I have not used timolol topically for hemangiomas. And I do inject right into the capillary hemangioma. So I think that’s it. I will actually… What I’ll do is I will email — I will provide everybody with my email. If you have any questions at all, ever, just email me. Let me see. How do I do that, here? Let me see. Yeah. So I’ll give you my email, and if you have any questions, just please email me at any point in time. I’m happy to help any day of the week. Or hour of the night. Or day. And happy to answer all the questions. But you guys actually have been very good, and asked some very interesting questions. And thanks for your patience in staying extra. Did this go through? I don’t know if it went through. Can you hear me now? I’m unmuted here. Okay. Well, thanks, folks, and I’m sure Cybersight can provide you with my email. There’s a process in which you can ask questions, if you have any. Thanks, everybody. And we will let you know next time, for the next session. Thanks. Bye.

November 15, 2016

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