Thyroid Ophthalmopathy/Thyroid Eye Disease has been known for 200+ years. During this Live Lecture we review clinical aspects of this auto immune disease, as well as review new findings to shed light on developments in treatments in the last 2-3 years and a look at possible future treatments.

Lecturer: Dr. James Fleming, Ophthalmologist from The University of Tennessee, USA


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DR FLEMING: Good morning! Or it’s good morning in Memphis. I’m Dr. James Fleming, at the Hamilton Institute at the University of Tennessee in Memphis. And the topic we’ve chosen today is thyroid eye disease, graves orbitopathy, or Graves’ disease, many terms we use for this process, that we all as ophthalmologists tend to see. The plan today is to do an overview of graves ophthalmology, and as Cybersight has requested, we have a series of questions embedded within this lecture. Those questions you can answer, and I’ll get a feedback on the thought about this. So let’s begin. And if you’ll give me just a minute, I’ll get my slides going. First, financial disclosures. And I wanted to put this in, because I do a lecture for AO North America. A lecture on orbital fractures. I donate that all to charity. The second has to do with thyroid eye disease, and I’ve been in a phase II double blind active study for thyroid eye disease. I guess the short answer is I’m trying to work myself out of this business, as we all probably are, but we’ll get into that a little later in the talk. A text was written by John Dutton and Barrett Haik a few years ago. I contributed a few chapters to this text. That’s the basis of when we started talking about thyroid eye disease and its management. So the first polling question is: what is the average age of onset of thyroid eye disease? So I would ask the audience to vote. And since this sometimes isn’t there, A, B, C, and D. I can’t vote. The panelists can’t vote. So let’s see what the group says. If you’re looking at the results, 30 to 45%. Let’s look at what the statistics show. Incidence in the United States is probably 0.4%. I learned years ago in different populations this goes up and down. I was in Panama and found out after a talk there that this is rarely seen. This varies throughout the world. Male to female ratio is as you see here. 5 to 6 to one. Usually onset in 40s and 50s. But this varies significantly. It’s the most common orbital disorder and the most common cause of exophthalmos. Historic background. Three people have been credited with describing the syndrome. Dr. Perry, Dr. Graves, and Dr. Basedow. How it obtained the name Graves’ disease is interesting literature to read. But it did. Graves orbitopathy. Even though we’ve known about this for two centuries, the science is just starting to move forward, because of the immune complex of this. I’ll use different terms because different people use different terms. The fundamental cause is an antibody that stimulates the TSH receptor on the thyrocyte. It was thought by researchers a while back that this was an IgG molecule. Other people might think different thoughts about this. We know there’s a genetic predisposition. We know it’s not Mendelian genetics, but it tends to show up more frequently in families. There is also a known environmental factor that worsens graves ophthalmology, and that is smoking, and that’s been shown in a number of literature articles. So classic theory of autoimmunity. Antigen presenting cells are activated by some type of outside source. This leads to T-cell activity and autoimmunity. We see death of tissue or injury to tissue, as this cartoon shows. Classic example is rheumatoid arthritis. You get all this autoimmune event, and then you get scarring and the arthritides that are associated with this. Thyroid eye disease is a little bit different. The T-cells interact with the antigen presenting cells, and then there’s upregulation of thyroid. So independent of the normal regulatory pathway. So the next polling question is: thyroid eye disease and this upregulation affects the orbit as a secondary target organ. So what does it do? Increase orbital fat, cause swelling, increase the thickness of the extraocular muscles, or all of the above? I’ll leave it to the participants to vote their choice. Very good. 84% say that, if you’ll notice, all of them — it’s there. And nobody said cause swelling. I’ll talk about that a little bit. The autoimmune mechanism is really clear, and if you look at the bottom of the slide, there’s been thought about the IgG receptors on the surface of the fibroblast. A number of people are working towards what is the autoimmune mechanism. It has not yet been elucidated in the scientific literature, but we’re closer than we were 20 years ago. So what does happen in the orbit associated with this? What happens is orbital fibroblasts appear to be the prime target of the autoimmune attack. The cell that is noted — it’s the retroorbital preadipocyte fibroblast. There has been measured increased orbital fat in a subset of these people and we know it. Another classic finding is the brow fat pad increase. T-cell mediated inflammatory response is occurring in the active form of the disease. So we see swelling and inflammation and acute orbitopathy. We do see swelling and inflammation. I’ve got some photographs of people with this. And the last is increased thickness of extraocular muscles, the increase of glycosaminoglycans. This is deposited and it’s shown pathologically, pretty common to see, in the group where you see enlargement of the muscles. This is a schematic thought of what’s going on. It’s from 2002. Antigen presenting cells activate T-cells, it upregulates thyroid, the T-cell activation gets into the bloodstream, finds the fibroblast and retroorbital area, and then the three things happen. There is edema, thickness in the muscle, proptosis, collagen synthesis, fibrosis, restrictive myopathy, lid retraction, which we see, and an increased orbital fat and proptosis. So all of these processes go on in the secondary target organ. What so in looking at what gets what, the best population-based study that I can find or come up with was out of the Mayo Clinic in 1994. The County right around Mayo Clinic has been studied for many years. He pulled that whole population. It doesn’t have an increased incidence of thyroid eye disease. But the findings are pretty clear. Lid retraction, 91%. Exophthalmos, 63%. Restrictive myopathy, 42%. Optic nerve dysfunction is the key one. 6 or 7% has been put forth and it tends to hold clear in most of the literature. When they did CT scans, they found 55% — not 63% had extraocular enlargement in the group they looked at. And the most interesting piece — and this is an argument I have with endocrinologists pretty often. Can you be euthyroid and have graves orbitopathy? 7% of the population that he looked at was euthyroid at the time when they were tested. Now, they may later come to thyroid ophthalmology. But this is what the data showed at that time. And it’s a pretty clear study of how this appears through time. So the clinical course of thyroid eye disease is also the thing we need to do. And I use the term Rundle’s curve. He was an endocrinologist in the 1930s. And he described the orbitopathy portion of this. How long does Rundle’s curve last? And here we get to vote again. So the group can vote. When you have an onset of thyroid eye disease, what are we looking at? This is the outlook for a patient — your prognosis is what they can expect to happen. So I’m waiting patiently for the group’s vote. 6 to 24 months was 62%, 30% think it’s 6 months, and a little bit on both sides. Nobody thinks it’s 2 months, and they’re absolutely right. Let me close that. Here’s been reported, and I’ll pull from the literature. And this is Rundle’s curve. This is the active phase and the chronic phase. And people talk about active phase and chronic phase. If you divide the active inflammatory phase, you can have acute or subacute. And that means they can be really hot, really inflamed, redness or inflammation, or this can be a slow, progressive thing. And so the active phase that everybody’s talked about is usually around 6 to 24 months. It holds up in the literature. However, the caveat is: this is quite variable. So it varies from patient to patient. And we’ve all seen this pretty commonly. There’s a variable outcome in this group of patients. The chronic phase or the fibrotic phase that these people have gone through Rundle’s curve and they’re at the other end, then the question comes down to: what’s the long-term prognosis? And in my review of the literature, really two long-term studies looked at proptosis. I haven’t looked at the extraocular muscle stuff. I just picked out proptosis. And said: Okay. What happens after they go to the chronic phase? 72% stay the same. Their proptosis doesn’t change. Increase by 23% slowly over a period of time, and decreases by only 5%. So it’s gonna get better because we fixed your thyroid gland and it’s all gonna go back to normal — really hasn’t held up. So let’s look at my drawing of Rundle’s curve. And I use this in clinical analysis of patients. Rundle was pretty good at describing this. What happens is, if you have a patient, the question I always ask: where are they on Rundle’s curve? Here, here, here? But most likely they’re not gonna return to baseline. What happens is you’re following these people and you’re trying to determine what’s gonna happen. The classic answer I tell the patient the first time I see them… I’ll do measurements. I’ll find out where you are. But I have one point on a graph. I can do history back and forth, look back at ID pictures six months or a year ago, and get some sense of where their proptosis is, but in a clinical setting, the best way to do this is follow them. Do a measure, and my usual follow-up is 2 to 3 months, unless they have acute problems. Measure them again, and try to isolate them on this curve and say where they are. The thing I’d like to say is… This whole space under the curve is the variability. Some people are very severe, some can have it very mild with certain changes. 91% get lid retraction. That may be their curve. They may not have any other findings associated with this. The other question is: What are the reported risk modifiers in thyroid eye disease? What has come out in the last 10 to 12 years? I’ll put these up. I’ll discuss them a little bit. But I would like everybody to take a shot at what’s going on here with these three entities. Do they think one stands out? All are involved? Whatever. Let’s see the group. It’s interesting when you do a webinar. There’s no feedback, except what I see over a period of time. So I’ll just be patient and look. Here we go. 64% think all of the above. There are a group that think low selenium levels, thyroidectomy. Risk factors are not causality. I have a daughter who works with the Center for Disease Control and we have this discussion all the time. Correlation does not equal causality. There’s correlations with problems. That’s not the cause, but they’re factors that you must take into account when you’re looking at it. The classic one is cigarette smoking. That’s been confirmed in multiple studies when they do analysis. They need to stop smoking. The severity of their thyroid ophthalmopathy is gonna increase significantly if they continue to smoke. Selenium. 198 subjects — they found there was a decrease in selenium levels. And if there is a decrease in selenium levels, there probably is some help in dealing with that, when corrected for this. Again, it’s not causality. Does it affect the outcome? It may well. If you test for selenium, this is a fairly easy medication to obtain. In vitamin form. So that’s something to consider in your patient population. Statins. This is the data from JAMA Ophthalmology in 2014. They reviewed 8404 beneficiaries of a large managed care network. Patients received a 50% reduction in the hazard of developing Graves orbitopathy. It’s a retrospective analysis. It doesn’t create causality, but there is some correlation associated with this. There’s one other caveat with statins and steroids, and I’ll get into that in just a minute. Is there some correlation? Yes. Causality? Probably not. Thyroidectomy. This was another one that was looked at in this large study. Patients with Graves’ disease underwent a reduction in their hazard of developing thyroid eye disease. That is not a driver to have a thyroidectomy. There again is some correlation, but again, causality and correlation are two different things. So I pose it for the audience to look at, but understand that those three things are not drivers in this thing. But they’re something to be considered in an individual patient. There is some correlation associated with this. So let’s look at active phase disease management. I’m gonna take a second to talk about active phase disease diagnosis. And I think some people — I didn’t put it in the talk, because of the length of the talk. I use a clinical activity score to delineate active phase. I’ll give you the notations or websites so that you can look at those. Both put forth modified ways to look at thyroid eye disease and use a classification. Clinical activity score has been very good for me. But I have not bought into anybody’s measurement, except to say I continue to use the same one, because I can compare apples to apples. And continue to follow these patients over Rundle’s curve. I’m trying to delineate where they are in Rundle’s curve. Back to the active phase. Disease management, medical therapy. Obviously localized ophthalmic protective measures, artificial tears, if they need to patch at night, do different things, use lubricants, that’s what we do. Antiinflammatory treatment, corticosteroids have been used by a large number of people. Periorbital injections have been proposed. And then IV pulse steroid use. There is some correlation. And this is the caveat I put in there. Therapy with statins and IV pulse steroids together puts somebody at a moderate risk associated with this. Some people need to take that into consideration if they’re dealing with statins or the patient is on a pre-use of statins themselves. Immunosuppression. We thought this would change the world. In my experience, this has not been the great savior we wished it to be. Others might have had different experiences. But consistently, it has not been as good as I would have liked to have seen it. And targeted immunosuppression, we’ll get into that a little bit later. Orbital radiotherapy. It’s controversial, but it does have defined benefit with this. And then surgical intervention. I try to save for the chronic phase. People have had chronic changes. They’re burned out. They’re not an active disease. The only group that you do active intervention with are people are acute vision loss and you’re not able to deal with it. I’ll back up to steroid therapy for acute orbitopathy with vision loss. Usually go to 60 to 80 milligrams of oral prednisone for a week, 10 days, for the acute vision loss, and try to control them in that fashion. I may jump to orbital decompression if vision is going downhill. I have no problem with pulse IV steroid therapy, and I’ve got the dose and another couple of slides to put forth, and you can use that also. To give this group… EUGOGO is the European group on Graves orbitopathy. Go to their website. You can get more information. There’s whole text on this problem. So I’m trying to speed through updates and give you references to go further. The other is ITEDS, and that group is right there. That is their logo. And I invite everyone to join and look at that group also. So acute orbitopathy. Severe exposure, compressive optic neuropathy, globe prolapse. Real trouble. This is a small subset. 5%, 6% of the people at best. And what it has been defined as is a very… An orbitopathy — it’s a compartment syndrome. Here you have an orbital compartment and you’re compressing the compartment. Compression of the optic nerve, marked proptosis. These people are in real trouble. So the question is: thyroid eye disease affects the optic nerve dysfunction in what percent? I may have given away the answer. So you all get a free one on this one. And the interesting answer is… It’s a physical problem. It’s not directly within the optic nerve, in my opinion. Other people may say there is some dysfunction within the optic nerve. But it’s interesting. If you perform orbital decompressions, you do get a significant effect. So this is a 6%, 7% group. Small group, but an intense group that you have to really treat hard. So they improve with oral corticosteroids. I use 1 milligram per kilogram per day. The pulse level is 500 milligrams over three days and four cycles. A total of 6 grams. I put the risk factors… There have been a couple of reported liver failures associated with this. If you’re adding statins, look out. The combination of the two can give you trouble. And then acute orbitopathy. I do not like to do surgical decompression in this group. But if it’s necessary, it’s necessary. It’s interesting. The slide I show here, with the papilledema, because of the posterior aspect of this, papilledema is not the most common thing I see. It’s the optic nerve dysfunction measurements that you pick up. So don’t wait for the papilledema. Wait for the optic nerve function. Etiology. Orbital congestion with compression of the optic nerve. So these are that slide. Glycosaminoglycans, the infiltration of this thing, and look at this poor optic nerve. It’s been compressed within the apex of the orbit. Decompressions address this, because of the thinness of the bone in the medial area. But this is a compression phenomenon associated with this. Can you decompress this medically? Sure. And we need to try. The other caveat I’m gonna put in this talk is acute active phase disease management, steroid therapy, this is a very nice lady. An endocrinologist put her on 60 milligrams of prednisone for two months, and 40 pounds later, hypertension, diabetes, you name it. She’s got it. Very unhappy patient. Have we shortened her life? That is a very good question. How much do you use steroids, and how hard, and how long is a question each practitioner needs to balance between the side effects of the steroids themselves. Radiation. To get into this, this is the dose. It’s 20 Grey, delivered to the entire bony orbit. Fractionated over 10 days. There’s other ways to do this, but this is the range in which it’s used. Does it work? Sure. In my experience, it works for acute inflammation. It takes a few weeks. So you’re gonna use this for acute orbitopathy. You’ve got to manage them with steroids also. But you can decrease the inflammation. But the point I want to make in this talk is… Look at the proptosis. It’s not gone. You haven’t changed the extraocular muscle dysfunction or the proptosis associated with this. The argument will be made you didn’t get them early enough and didn’t stop them before the proptosis became there. That is an argument that is made. You start looking at the literature, and there have been two prospective trials. There have been a number of retrospective trials. And retrospective trials are great, but there’s a bias already built into them by somebody’s treatment modalities that they pulled out. So do a prospective trial, and I believe it much more. The first one is out of the Mayo Clinic in 2001. And the arguments made about this… They said there was no difference in use of radiation. The problem with that trial, many people have pointed out, is they didn’t pick active disease versus chronic disease. So they weren’t separated out in that fashion. So there may be some flaws in the analysis of these patients. But they found no difference. Journal of Clinical endocrine metabolism, 2004, the Netherlands study, and in that group, they did show a response to radiation. In the long run, did it change the course of their Graves orbitopathy? That was in question, but it did decrease their inflammation. In their paper, there was a cost analysis and a change. And I invite people to read that paper and look at it and see what the cost and the benefit is. Because it does work, but it doesn’t fix all the problems. Radiation can decrease acute inflammation. Is it gonna cure their Graves ophthalmopathy? Probably not. So active phase management for me… It’s stop their smoking. Because that will change their thing. Look at the other risk factors, whether it’s the statins, if they’ve got it. Selenium. And then begin the documentation of locational Rundle’s curve. Most of these people do not come in in acute optic nerve compression. They come in with the other subsequent — or associated findings. Whether it’s lid retraction, proptosis, it’s mild. They’re known Graves patients. And what we do is follow them. And our group follows between 100 and 150 active Graves ophthalmopathy patients at any given time. Getting them through their acute changes associated with this. So stop their smoking. Start documenting it. Is there a cure for all these people? If they’ve got mild disease, we just follow them. They don’t need anything else. If they have moderate disease, we assess how much difficulty they’re having and try to get them to the other end of Rundle’s curve, to the fibrotic, burned out side, 6 to 12 months later. And look for the acute people. My plan is to follow them every 2 to 3 months during active phase, get them to the final phase, and then discuss what problems you have and what you recommend as far as treatment. So the chronic phase. People say a lot of this is cosmetic in the chronic phase. If you talk to a number of these people, they have orbital congestion. They feel their eyes all the time. They have discomfort associated with it. They have pain. And so in the phase where they have a chronic congestive orbit associated with the other end of Rundle’s curve, I use a treatment decision path. Because I’m an oculoplastic surgeon, this is where I do most of my intervention. I follow the active phases with our neuroophthalmologist, with our endocrinologist. We have a group of endocrinologists that see these patients also. And we keep everybody informed as a team to manage these patients. But it comes down to the chronic congestive phase on the other send of Rundle’s curve. I usually wait 6 months of stable measurements, and then I declare them stable. The question was asked earlier… What is the risk of reactivation? In our experience, it’s 5%. So if somebody’s gone through a series of changes, and they’ll go two, three, five years, and 5% will reactivate their autoimmune disease. That’s what I tell my patients. You look for it, and that’s where you go. So back to the decision path, if you’re going to intervene in the chronic phase, orbital surgery is done first, strabismus surgery is done second, and eyelid surgery. Some people just need strabismus surgery and a number of people just need eyelid surgery and they need no orbital surgery. So you put these people and see where they fall in the decision path. Do they jump to the third ring? Do they just need eyelid surgery? A large group does. 50% that have significant Graves orbitopathy might need orbital surgery. So this wall is more common for many people, looking at that. We’ll go through that type. Evaluation of the ophthalmopathy — I start with a CT scan. I do not perform the CT scan at the diagnosis. I perform a CAT scan to assess surgical intervention. So I’m looking for the size and depth of the orbit. A short orbit is much more problem than a long orbit. Size of muscles. Position of the sinuses. Do these people have chronic bad sinus disease? Do they not? Do they have large sinuses, small sinuses? What’s the thickness of the bone associated with these defects? Obviously large muscles, real problem. We know where we’re gonna go, as far as the decompression on this patient. And that’s right there. That’s where you’re gonna get the most bang for your buck. What I have learned to do — and I’m not proposing these groups. I just use these subgroups from my decision process, within orbitopathy. And if they have proptosis with significant restrictive myopathy, clinically significant diplopia, that’s group I to me. The group where I’m gonna have one discussion. Group II is proptosis with minimal restrictive myopathy. The reason for that is… And I’ll go back to this… Is the instance of floor medial wall decompression has a known instance of diplopia associated with — as a complication of the procedure. These patients already have diplopia. Not a complication. You’re just gonna move their double vision around a little bit, and then you can do strabismus surgery. So I will lean toward floor medial wall decompression for this group of people. And if they need further decompression, go to the lateral wall. Then consider strabismus surgery, then eyelid surgery. It’s an easier discussion when somebody already has double vision. If they don’t have double vision, then you have to decide what you’d like to do with these individuals. And I’ll go through the other type of decompression, and then we’ll talk about it a little bit further. Area of floor/medial wall decompression. This is the orbit. I’ve outlined what we call the strut, the junction between the medial wall and the floor. Medial wall, as you can see in this thing, that’s the honeycomb of the ethmoid cells, anterior and posterior ethmoid artery. Right in here… Junction to the anterior cranial fossa. This area can be decompressed, associated with this. Floor — you must identify — I’ll often denude the bone around the nerve and decompress the floor. The strut really is the medial wall of the maxillary sinus. And as the medial wall of the maxillary sinus comes up from below, it begins to form this strut, and it’s a structural piece that must be addressed a little differently, because of what you’re doing to the orbit and what you’re doing to the maxillary sinus. If you take enough of this out, you may have to deal with drainage of the maxillary sinus. But we address the strut, depending on how much decompression we want. I can grade it, as far as these areas. First, floor, second, medial wall, third, strut. If I want 6 to 7 millimeters in my hands, I’ve got to go for all three of these. If I want 2 or 3 millimeters, I can go to the medial wall or the floor. 2, the floor because it’s the easiest approach. And then the medial wall and then the strut, knowing I’m gonna create more diploma. Here’s the nerve. So the floor’s been removed. We identified the nerve. Removed the bone, kept the nerve intact. Transconjunctival approach, inferiorly expose the orbital floor, remove the floor bone, then if I want to extend medially and superiorly up the conjunctiva, near the caruncle and up, and allow for exposure of that… And if I can show it, that’s the top of the strut right there. We know where we’re dealing with… Medial wall above, floor has been removed below, and then make the decision about the strut. As you can see, this patient has fairly significantly diplopia. I use Afrin in the nose and in the sinuses to control bleeding, and then remove this bone in a piecemeal fashion with care to keep the orbital periosteum intact. The last piece I remove is the orbital periosteum. I incise it, release it, allow orbital fat. If you need more decompression, you can take the fat from the inferior medial orbital area and whoever does this surgery also knows the fat will prolapse anteriorly. So here’s our subject. Postdecompression. Here he’s beginning to go back. We performed strabismus surgery, realigned him. And gotten his decompression down. So we’ve taken him through three sets of surgeries. Floor, medial wall, and then on to strabismus surgery, and then lower lid surgery, upper lid surgery, to this position. This does not… What you’re getting is somebody with central vision, with no diplopia. He may well have restrictive diplopia, in lateral gaze, inferior gaze is the most common — just because the muscles are still abnormal. Another individual. Same problem. Marked retraction of the lower lids. Inability to look down very well. Looking up. This is three sets of surgery. In my hands, I use ear cartilage to build up a lower lid. Ear cartilage grafts within those lower lids to bring it back up. We’ve accomplished about 7 millimeters of decompression. Aligned his eyes fairly well. And gotten him through there. The problem is… I call this the tunnel. And these people are in for 6 to 8 months worth of serious surgeries, and we try to articulate this to the patient, to the family. One quick procedure is not gonna fix all this. They have to buy into the process. But if they do, you can take them from significant problems to fairly good outcome over a period of time. With the caveat… They’ve still got Graves’ disease. Do I do three wall decompressions? If I need more than 6, 7 millimeters, I go to three wall decompressions. As you can tell, this individual had a short orbit. And that’s what I look at. If you can’t decompress the medial wall, floor, lateral wall, in that amount of bone, what you can do is do three wall decompression, and you’ll see this term, malar augmentation. When God gave out malar bones in this individual, she didn’t get them. And so… What happened is we had to substitute malar bones and do lid retraction later on. This is a face-changing set of procedures. It’s doable. It’s a set of 5 to 6 procedures to get from A to B. Let’s talk a little bit about group 2. It’s the group that I enjoy doing. But I do a little bit different. In my hands… Treat these a little different. And what I’m talking about is a lateral decompression. And over the last few years, people have picked up using lateral decompressions for decompression, and this has been beneficial in my hands for the last ten years. Lateral wall decompression. Possible fat removal. There’s a very quiet area, inferiorly, laterally, in the orbits, you can remove fat and a little less consequences associated with this. If they need malar augmentation, you can add that to it, and the last is eyelid surgery. This is an individual who had bilateral lateral decompressions, and lower lid buildup. I will say that lateral decompressions — I perform one eye at a time. Floor, medial walls, I have no problem doing bilateral decompressions, because if it bleeds, it’s gonna go in the sinus and come out their nose. In this group, I had a very nice lady who had a huge vomiting episode at the postoperative time. I did bilateral lateral decompressions and I had to evacuate hematomas from both sides from count fingers, I got her vision back. But that was more life changing than I wanted to do. So what’s a lateral decompression? It’s the lateral sphenoid wing. If you use the new high speed drills that go 60,000 to 100,000 RPMs, you can do a fairly significant decompression. I will thin the lateral rim. I will fine the takeoff of the roof. Bob Goldberg talks about removing this area too. The fossa above the lacrimal gland is absolutely game to remove bone. Take the bone all the way back to the level of the superior orbital fissure, expose this area over to the inferior orbital fissure, and remove this. There are a number of ways to address this. Some people take the rim off. Some people don’t. I prefer the rim off because of exposure. I have no problem with some people leaving the rim on and doing this with a high speed drill. The problem you run into with 60,000 or 80,000 RPMs is you better protect the lateral rectus muscle. I saw one case done elsewhere. Got into the lateral rectus muscle. This causes a strabismus that’s very difficult to fix. I use ribbons over the muscle to protect it, so if the drill jerks into the wrong area, it goes into the ribbon or the patty and the muscle is spared. So be careful in that area. I’ve had CSF leaks associated with this area. I really don’t worry about them. I have exposed dura. It’s not a major problem. You can plug a CSF leak, because it’s not open to a sinus. It’s not something you want. The dura exposure, as long as you don’t open the dura, you’ll do fine. Here’s a lateral decompression. I prefer an upper lid crease incision. You can do it through lateral lid splitting. Either way is fine. Expose the lateral orbital rim. My preference is to cut the rim free. This is a high speed drill with a fine cutting bit that’s used in this area to cut away the rim. It exposes the deep orbit. From that, I do a series of maneuvers. Use the high speed drill. I will use a rongeur, and just expose the lateral sphenoid wing, and proceed to remove bone until it’s completely taken back to the area near the superior orbital fissure. And see what you get. The other is I’ve found this to be helpful to me. I take the lateral rim out. I’ll take the rim and with a high speed drill thin it to half its thickness. People have talked about plating the rim back in place. I’ll put a small hole above and below on both sides. This side in the donor area and the rim piece. And just sew it in with a 4-0 nylon. The beauty of that is it allows the limb to shift laterally. I reported that a number of years ago with a plate. But I prefer just letting it kind of float laterally. And it helps. There is one caveat in closing this. I’ll let the lids find their own place. I will not reattach the lateral canthal tendon to the orbital rim. You release the tendon, it’ll pull forward, and that is helpful in the decompression also. You can combine this with a lid retraction repair or a recession of levator muscle, and I’ll do that in 30% of the cases I do. I’ll go after the upper lid at the same time. This is a case of bilateral laterals. I wanted 3 to 4 millimeters. This is sphenoid wing drillout. You’ll see her incision here in her upper lid. I’ve recessed her later a little bit. Like 3 millimeters. And I’ve gone after the sphenoid wings. That’s all that was done to this patient. You can get a pretty good result if you do it — one side, wait 6 weeks, and do the other side, is my preference. You can do some fat removal. This is an Asian individual that — she has a prominent eye and a short orbit. I’m not trying to make a deep orbit patient out of this, but I’m trying to deal with the lid retraction. At the same time as the lateral decompression, and do both procedures simultaneously. Do one, six weeks later, do the other. Supplemental procedures, preseptal or postseptal fat, to relieve orbital pressure and add to the decompression is done. Probably don’t do as much as I used to. And then malar augmentation or orbital rim augmentation. This is a porous polyethylene implant. If you look for these, you can find it. It replaces orbital rim and really what you’re doing is advancing the malar area by 5 to 7 millimeters. It corrects a lot of the perceived exophthalmos, because they have malar hypoplasia. So if your patient has a short orbit, what you’re doing is basically lengthening their orbit. No malar bone. What you’re doing is building up this area. And giving support to the lower lid as well. And you combine this with the lateral decompression, if you do a lateral incision, so I’ll do lateral decompression, remove the lateral bone, put in a malar implant. And this is an individual who’s had bilateral lateral decompressions and malar implants. Malar hypoplasia is a big problem. There’s no support for that lid. Let’s talk a little bit about the future and then we can get into the discussion. Because people have opinions about Graves orbitopathy. My prediction is these patients will not require major surgical management of the disease. The nice answer is I’m trying to work myself out of business. I know how to do orbital decompressions. I’ve done them for years, probably 300 orbital decompressions. I would love to quit. Because these patients need something. This needs to be changed to a medical disease. Because it is an autoimmune disease. So here’s my discussion about immunotherapy. And this article was published in the New England Journal of Medicine in May 2017. Teprotumumab is the drug. It’s not available commercially. It’s in phase II studies. We did it in a double blind study. It’s a monoclonal antibody that binds to the TSH receptor on the orbital fibroblast. This is an IgG molecule in the class of autoantibodies that activate cell surface receptors. So it’s addressed directly at fibroblast activity. If you read this article, and I invite you to, in the New England Journal of Medicine… I’ll give you the caveat. I had 11 patients within the study. We didn’t know who got the drug or who didn’t. Double blind study. The p-value is 0.001 for proptosis. For the other findings associated with this disease, it shows great promise. It’s a number of years from getting on the market. It’s an infusion. But I guess the answer back is… This is again an autoimmune disease. Is there another drug or other drugs that can address this? We know it’s an autoimmune disease, because steroids and the other things that have been used have an affect on this process, but it’s not as targeted as some things ought to be. So in the next 10 to 15 years, let’s see if we can put ourselves out of business, as far as major orbital surgery. I think the lid and the muscle surgery will probably continue, because we see the patients after those things have begun to show up. And we’ll have a set of patients, as Dr. Bartley said, 7% were euthyroid and still had ophthalmopathy associated with it. So on that note, I’ll thank you for this short rendition of thyroid eye disease. Textbooks have been written about this. But I thought we’d just take a tour and begin the question session. Prior to the procedure I got a series of questions, and please write your questions now and we’ll talk for a minute. I tried to elicit some answers within that. How do I address the active eye disease? I watch them, I put them on Rundle’s curve, I use the clinical activity score. EUGOGO has another version of that. Both of them are very good at doing it. Look at them on the internet. Get the scoring card. Score the patients as they come in. And then watch them for a period of time. Do I use steroids? The answer is absolutely. Do I use them long-term? You saw my complication list. I’ll use it acutely. I do not put everybody on steroids. Because of Rundle’s curve — it’s different in every individual. Will I use radiation? I personally save that for the very severe. I know there are other people who feel like that’s a very first-step… What I’m looking for is evidence-based medicine, clinical trials that are proactive, that go forward, that can help in the course of this disease. So I’ll start with the first question. Should we include statin in medical treatment? Statin has its own set of complications associated with this. I invite you to read the article. If you’ve got the slide, you can look at it. I personally have not added statins in my medical therapy, but it’s something to consider. Again, if you add statins, look out for steroids. The next one is… Can you talk about the course of decompression surgical education? I’ll see if I can take that one on. Decompression… Is something… A decision that the patient and I have after they’ve stabilized, and I want stable measurements for six months unless they have an acute problem. Once I get that, six months of stable findings, the decision process — how much decompression do I want? 5, 6, 7 millimeters? I need to go to floor and medial wall. If I need 3 millimeters and worry about eyelid, then I go to lateral wall. Most people with 6, 7, and 8 millimeters of proptosis have a fair amount of diplopia. Some don’t. I will discuss the diplopia associated with that with the patient, and they have to accept the risk of that. These things are just sliding around. So it’s an individual discussion with the patient. Postoperatively, they’ll have numbness in their face if you do floor/medial wall for 2 to 3 weeks, and this usually resolves if you keep the nerve intact. When do you administer periocular steroids? It’s an interesting group. It’s usually my severe diabetics. Because they have so many side effects associated with PO steroids. It’s not been my best way to do it, but in the diabetics, I get around the systemic side effects associated with this. Steroids are like horseshoes and hand grenades. You do not need to put it in the orbit. Just put it beside the orbit. Right in front of the orbital septum. I use Kenalog. The problem is there’s a known group of people that increase intraocular pressure. You can’t take them back out. So you’re into the use of steroids for a period of time. And you’re doing a diabetic population that has some risk factors. IV pulse steroids… I’ll put it there. A number of people have liked this. I have no problem with it. I advise you to try it, if you wish, and see if this is something you would do. It has some problems, with the fact that you have to set it up to get it done. I’ve just been very happy with PO steroids because I’m trying to run down Rundle’s curve. I’ll pose the question: I use IV pulse steroids to stop the course of the disease? I have not found that that’s been my best activity with that. Find selenium helpful, supplementation during the active phase, if they have a low selenium level, absolutely. I would put it in. Has there been prospective studies to say it makes a big difference? I’m not sure of that. It’s easy. It’s a multivitamin. Just look for the multivitamins with selenium in it. Say you can take these, and it may help it. Retrobulbar triamcinolone for orbitopathy? I put it peribulbar, right in front. It’s close to there. Lateral orbital wall angle on CT predispose… This thing is jumping around. Excuse me. Predispose the patient for more severe disease? I’m not familiar with that. They said a shallow orbit. Absolutely. Because you’re starting from a proptotic eye to begin with. Should tarsorrhaphy be done for exposure keratopathy? Sure. You’re looking for Rundle’s curve. You’re looking for the chronic phase, and if you need to do tarsorrhaphy to get them down the road, sure. Don’t use it long-term, though. There are too many other options. I did this with facial nerve palsies — it’s like a horse where you put blinders on the side. Their field of vision is affected greatly. Thank you, doctor. I remember a patient that we had steroids, made him manic, and needed to manage him with the help of psychiatrists every time he came in with acute inflammation. We wound up using radiotherapy. There’s a number of things people use. All of these have effects and side effects. Radiotherapy is not benign also. So this is a complex disease. And we don’t have full control of this disease. How long should corticosteroids be performed for acute orbitopathy? Would you consider another modality? I use a relatively short course. If you look at the IV pulse thing, it’s for a short period of time. I really use PO steroids for a week or two, and then start withdrawing it, or look at whatever other modalities I need. If I have a severe problem, I’ll go on to radiation therapy, if I’m not in the chronic phase, or consider decompression, if their vision is down, because I don’t have the beauty of being able to wait. Again, look at their severity of active disease. Do not use all these modalities for mild to moderate disease. They’re gonna get through all this. When would I consider rituximab? I’ve used it two or three times. I have not been greatly impressed. The old answer is when your back is against the wall and you’ve used everything else. I’ll probably pull it out. I’m looking for new drugs that are aimed better at the disease process. What’s the duration of steroid therapy, periorbital or periocular? Same answer. I use it a few weeks and then I’m off of it. You saw my picture. 40 pounds and diabetes. If you’re at two months, three months, you’re really not changing the course of the disease. Periocular, one or two injections, and I’m done. How do you treat retraction of upper lid medically? That’s an interesting question. It’s been proposed to use Botox to cause paralysis of the levator muscle. It has its own side effects. It lasts three to four months. If you have somebody with exposure, I’ve used it a couple times. It is not really predictable. So if you have somebody with real exposure, you can try. You can give double diplopia and other things. It’s been reported — occasionally I’ll pull it out, but it’s not a great treatment for it. Surgical is the correction for upper lid retraction once it’s stable. During decompression… If the dura is open, is there a chance of CSF leak? Of course there is. I’m not a neurosurgeon. Can I manage the patient? If you’ve exposed dura and there’s a small leak, I’ll put gel foam in it. If there’s no leak from there, because it’s an enclosed space on a lateral, I don’t greatly worry about it. If you see the dura and it’s intact, there’s no problem at all, because you’re doing a lateral decompression. If you need to, you can call in a neurosurgeon. They can put in a lumbar drain later on. Lower their pressure and it will self-seal in a group of people. The more complicated problem is somebody that performs a medial wall decompression, gets an incidental CSF leak. Now you do need a neurosurgeon. You do need to manage it. Because CSF leak goes into the ethmoid. They’re at high risk for meningitis. If you’re doing a lateral, the risk for meningitis is almost nil, because it’s not to an open area where there’s bacteria. It’s to the orbit. If the patient has mild unilateral lid retraction with euthyroid, how do I treat? Back to Rundle’s curve. Watch them for a period of time. And then the discussion comes down to a six month stable finding. Is this symptomatic to the patient? Do they wish something done? If it’s 2 millimeters or more, I’ll address it as a functional problem, because they get some lagophthalmos at bedtime. They have to use lubricants. It’s all patient individual treatment. What should the time difference be between different surgeries? Orbital floor decompression… I send to my strabismologist probably 6 to 8 weeks, and they want to get one or two stable measurements before they proceed. They’re looking for stability in the course of the disease. My lateral decompressions I’ll do one side and then wait six weeks and do the other. Eyelids… I want all the swelling and the inflammation to subside, and then I’ll address the upper or lower lids accordingly. EUGOGO classifies thyroid eye disease as moderate to severe and sight threatening. How do we differentiate between moderate and severe disease in this classification? I’ll take this a little bit down the line. Use either the EUGOGO or the ITEDS or the clinical activity score and start looking at those. Pull those scores up and start plugging them into your patients, is the first thing. The next is… My severe disease has to do with severe exposure or optic neuropathies. Do I have a decrease in vision, color vision, field defect, afferent pupillary defect? All those are signs of optic neuropathy. Is surgical decompression repeatable during the course of the disease? Absolutely. You have three walls. If somebody comes in that’s been treated elsewhere, the first thing I do is a CT scan and see how complete is the decompression. Has one wall been worked on? Two or three. Once you’ve got to three walls, I think you’re done. You may consider some of the other types of treatment. Orbital fat removal. And then you go from there. Has radiation been used? All the other things that are out there. You’re in trouble after you’ve taken three walls and you’re still having problems. Do you consider low to dose methylprednisolone? No, I do not. It’s back to Rundle’s curve. There’s a number of people that have just lid retraction and nothing else. I’ll let these people show where they’re going and what they’re doing before I pull the trigger on known side effect-producing medications, drugs. How do you decide how much decompression is needed? This is an interesting question. Hertel measurements are variable by facial contour. I look at how much lid retraction there is. How much I’d like to achieve. And I look back at their history. And what I mean by that is… Most people have old pictures. Old photographs. And I say bring in your old photographs and let me see how much proptosis has occurred in you. As you saw, I put up an Asian patient, and she’s got a fairly proptotic eye to begin with. That’s the way her face is. So I’m trying to get back toward normal. So I want to look at old pictures, let them bring them in, look at it and see how much decompression I want to be aiming for. Is it 2, 3, or is it 6, 7, 8? Okay? Thank you very much! I appreciate it. And I’ll welcome any further questions later on. Thank you. I’m signing off.

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August 11, 2017

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