Lecture: Central Serous Chorioretinopathy and Pachychoroidal Diseases

During this live webinar, we discuss the nature of central serous chorioretinopathy and its relationship to pachychoroidal diseases. We also review the diagnostic testing required and potential treatment modalities for central serous chorioretinopathy.

Lecturer: Dr. Ramana S. Moorthy, Indiana, USA


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DR MOORTHY: Good morning. I hope you can all hear me. My name is Ramana Moorthy. I wanted to discuss with you this morning a topic that I have become very much interested in. On central serous chorioretinopathy and pachychoroid disease, and the spectrum of disease associated with these conditions. It’s kind of a new way to look at an old disease. By new, it’s probably maybe in the last five to six years that there’s been some elucidation as to, perhaps, the pathogenesis of these diseases, based on new instrumentation that we have readily available in our office, and based on high definition OCT, enhanced depth imaging, autofluorescence imaging, fluorescein angiography, and even wide field angiography. We’ll start with a poll question, first of all. It’s kind of a hard way to start a lecture, but I will begin and say: What is the average thickness of normal subfoveal choroidal measurements, using frequency domain OCT or spectral domain OCT? 150 microns, 250 microns, 320 microns, or 400 microns? So if you want to submit your answer… I’ll give you guys a few seconds here. All right. So that’s correct. The average thickness is typically around 250 microns. You guys are all experts, it seems like. So that’s the typical measurement. And these measurements are done using enhanced depth imaging OCT on our spectral domain machines. So this is kind of a standing electron micrograph of the choroidal vasculature, and you can see the lobular architecture of the choroid. The choroid plays a prominent role in these pachychoroid diseases, as the name may imply. The choroid is abnormally thickened, so we see a thicker than normal choroid. We may see this in other portions of the disease spectrum as well. So when we look at patients who have these types of diseases, we pay attention to — specifically — not just the presence of subretinal fluid that we may see on the OCT, but also at the level of the RPE and Bruch’s membrane complex here, and also at the choroid, specifically looking at the smaller vascular structures in the choroid, the Sattler’s layer, and the Haller’s layer as well. And we’ll see changes that we’ll talk about in a moment about these disease processes. Question two: In pachychoroid diseases, which OCT layer is effaced or absent? Sattler layer, Haller layer, nerve fiber layer, or Bruchs membrane/RPE complex? Where do you think the changes are? I’ll give you a few seconds here. So we’ve got kind of a mixed bag here. So we’ll talk about that. The smaller choroidal vessels become effaced in pachychoroid disease. We see larger vessels especially as the disease progresses and becomes more chronic. So the face of Sattler’s layer, especially with a thickened choroid, can be very helpful in differentiating these diseases from other causes of serous retinal detachments. In pachychoroid diseases, there are abnormal and permanent increases to choroidal thickness. The large Haller layer vessels apposed to Bruchs membrane complexes is very common, and you see effacement or absence of vessels of Sattlers layer. Why does this happen? This is the mystery. We still don’t know exactly why this occurs. Choroidal vascular congestion is thought to be one possible link, especially since there may be lengthened intrascleral portions of vortex veins that can impede the outflow of the choroid, and that can result in thickening of the choroid. Other things, such as excessive amounts of interstitial choroidal fluid, because there may be precapillary arterial hypertension, or altered intravascular osmolality, because of the presence of serum proteins or albumin. There may be alterations in choroidal interstitial fluid because of the presence of exogenous pharmacological agents, such as PDE inhibitors or corticosteroids, which in the case of CSC have been known to exacerbate CSC. The alteration in interstitial tissues in the choroid is another possibility, and these are things that we do not understand, based on the lack of histopathologic evidence in active disease phase. Here’s the third poll question. I promise we’ll get to the end of the poll questions here in the beginning, but we’ll have one more in the middle of the presentation. Pachychoroid disease spectrum includes which of the following diseases? Central serous chorioretinopathy, polypoidal choroidal vasculopathy, pachychoroid pigment epitheliopathy, or all of the above? Hopefully you guys have paid attention so far. So you guys all got it right. Obviously this is a pretty easy question. But it includes all of these things. And I’ll show you perhaps how these diseases kind of progress or manifest themselves. So the spectrum of disease includes these three disease processes. Some people believe that pachychoroid pigment epithelialopathy is the forme fruste of this spectrum of disease, and it may be the harbinger for central serous chorioretinopathy. Of course, we see different variations of central serous. With some patients having massive exudations, and then as the disease progresses and the patient ages, we can see the development of vasculopathies. And I credit the New York group that kind of have proposed this terminology, which others have adopted, and we see the adoption of these same terminologies to describe these disease entities in the literature. I think we’re somehow going backwards here. Sorry about that. So pachychoroid pigment epithelialopathy is the forme fruste. You can see the absence of normal fundus tessellation, you can see RPE changes mistaken for ARMD or pattern dystrophies, and the OCT can show scattered RPE elevations, small serous PEDs, the choroid is thickened, and the ICG, if performed, may show especially in the wide field views mid-phase hyperfluorescence suggestive of hyperpermeability. And fundus autofluorescence can show a granular hypoautofluorescence. Or a mixture of hypo and hyperautofluorescence. So this is an example of PPE. So when you look at a patient like this, you’ll say: What the heck is going on here? You can see the absence in the picture of the choroidal vascular pattern, and the absence of the fundus tessellations, and then you’ll see this funky almost drusen-like change, these yellow spots, diffuse RPE disturbances, and that’s confirmed on our ICT, where we see large areas of black light fluorescence. This patient did not have active subretinal fluid at this time, but may have gone on to have chronic disease in the future. Here’s another kind of appearance of the pachychoroid pigment epithelialopathy. You’ll see extensive areas of window defects throughout the posterior choroid, and then on the ICG. Here’s another person who has a significantly thickened choroid, and this is an enhanced depth image, as you can tell on the bottom of the screen here. Where it says EDI. You really need to look at EDI images to determine choroidal thickness. Standard images on frequency or spectral domain OCT will not give you the kind of depth in the choroid to evaluate the choroidal thickness accurately. And even with EDI imaging, getting the correct thickness is quite difficult if there are medial opacities that can obscure the inner phase changes that you see here, between the end of large choroidal vascular layers, the effacement of Sattler’s layer, though Haller’s layer is still present here, and you see the choroidal junction. And this thickness you can measure directly in the machine and see that it’s well over 300 microns. Typically these are in the 345, 350 micron range, as opposed to the normal choroidal thickness of 250. CSC you’re all familiar with, because it typically affects young, healthy people. Typically males more than females. There is some association with people who have personalities — so-called “type A” personalities. High-strung individuals. Individuals who are very uptight, if you will. And you can often see these patients. I have an interesting questionnaire that I ask patients to determine if they’re type A personalities. How does it make you feel if you’re in a traffic jam, or at a traffic light, and you’re the 10th car and you’re waiting for the cars in front of you to move? Do you become stressed? Do you become agitated? How does it make you feel standing in line at the bank if you’re 4th or 5th and waiting several minutes to be served by the teller? Even if the patient tells me… Oh, I’m not a high stress personality, that can give you clues as to what kind of person he is. And that kind of personality has been associated with CSC. Exogenous corticosteroid exposure is very important to determine, based on the patient’s clinical history, because that exposure may exacerbate underlying propensity towards central serous chorioretinopathy in patients who have this pachychoroid epitheliopathy. Patients can have metamorphopsia, micropsia. Some types of patients may be asymptomatic, because the serous retinal detachment is outside of the fovea. There is often pigment epithelial destruction on the exam, and when we look at fluorescein angiography, we can see the classic findings of focal leaks on fluorescein angiography. This is a beautiful example of a smokestack leak, and you can see on OCT the pigment epithelial detachment and overlying serous retinal detachment and this flow of fibrin coming out of — like a smokestack on the OCT. So one of the ideas that we know about pachychoroid disease and CSC, one of the new things that we understand — and some of this is really based on new imaging technology, and of course, some of this is based on new ways to try to treat CSC. And I’ll talk about all of the treatment paradigms that are available from the evidence-based results of various studies. So we know that this choroidal exudation that occurs in CSC — that exudation of interstitial fluid in the choroid, then, is associated with the development of serous pigment epithelial detachment, and that will result in a breakdown of the pigment epithelium. The function of the pigment epithelium, so that the typical bumps that are present at the basal lamina of the RPE become pathological, and you see subretinal fluid developing, especially in the central region. The role of the choroid — there’s a great deal more that we are beginning to understand, based on some of these imaging modalities. We know that the choroid is hyperpermeable in CSC. Because there is significant choroidal hyperpermeability and hyperfluorescence on the ICG angiography. So this indicates that there may be hyperpermeability in CSC that occurs from stasis or ischemia or inflammation. And inflammation is less likely, because people have tried using antiinflammatories, and in fact, corticosteroids make this condition worse. So evidence based on staining of the inner choroid under ICG was originally described by Spaide, and going forward, there’s been increasing evidence to suggest that increased tissue hydrostatic can result in subretinal fluid. A fluorescein angiogram can show a leak. You probably won’t see a smokestack, because those only occur in 10% to 15% of patients, but you’ll see a focal area of leak that starts out very mild, initially, and then becomes dramatically hyperfluorescent in the late phase of the angiogram. And that focal leak that you see, increasing in size, tells you that’s what it is. Of course, in the differential diagnosis of fluorescein angiography, you need to keep in mind that there is possible neovascularization. How do you tell the difference? Typically in the case of choroidal neovascularization, you won’t see a dramatic enlargement in the size of the leak. Typically in a choroidal neovascular membrane that’s well defined, you’ll see a net of a certain size, and the net doesn’t necessarily increase in size, but becomes increasingly visible on fluorescein angiogram. Whereas here the leak starts small and gradually increases in size. It becomes dramatically leaking, and the subretinal fluid stains with the fluorescein dye, and there’s pooling of fluorescein in the subretinal space. The ICG angiogram is a little bit more non-specific, because it will show you leakage, but it won’t show you the kind of leakage that the fluorescein will, because you might see more significant obstruction. There may be underlying pachychoroid epitheliopathy. The thickening of the choroid was first kind of described and evaluated in great detail by Imamura and colleagues in 2009. And these areas of thickening don’t necessarily correspond to areas of staining on ICG. They may be the result of corticosteroid-induced vascular permeability, and secondary sympathomimetic induced dysregulation. Dysregulation of the choroidal vasculature perhaps results in increased congestion of the choroid, increased interstitial fluid of the choroid, and that results in the development of serous subretinal fluid. We can also see that in later cases of the disease with CSC, chronic CSC, we can see choroidal lobular ischemia. This is a case of more advanced choroidal vasculopathy, where you see ischemia on the ICG, surrounding the choroidal polyps. This level of choroidal ischemia can be fairly profound. You can also see increased amplitudes of pulsatile choroidal pathology. The RPE is secondarily affected due to the choroidal changes. The RPE abnormalities may be present, as I mentioned before, in asymptomatic eyes, particularly in fellow eyes. You might see RPE abnormalities. That’s quite typical of central serous. And these focal RPE defects bring into question: Which way does the fluid from the choroid flow? In 1984, Negi and Marmor suggested that the fluid flows out of the subretinal space to the choroid. But how do you explain that the presence of these RPE defects doesn’t necessarily do that? And how do you explain the response to focal laser treatment? This is still not particularly well understood, because based on osmotic pressures and hydrostatic pressures, based on osmolality of this choroidal interstitium, you would think the fluid in CSC would be moving from the choroid into the subretinal space. So there are still some not well understood ideas about the pathogenesis of this disease. There may be some hemoglobin factors. Endogenous cortisol levels are elevated in CSC. This was shown by other investigators. And exogenous corticosteroids, as we all know, have been recognized since the mid-’60s to exacerbate CSC. Haimovici in 2004 definitively established this relationship as a risk factor for CSC exacerbation. These corticosteroids exogenously appear to increase the adrenergic receptor transcription, and they impair vascular autoregulation. That results in this choroidal thickening. This can also secondarily — the corticosteroids can affect Bruch’s membrane, because they affect collagen synthesis. And if Bruch’s membrane is altered, this could play a role in the development of subretinal fluid, and they can also affect RPE by altering the water and ionic transport and impair barrier function of the basal structures of the RPE. Interestingly, topical corticosteroids and in some cases regional corticosteroids don’t appear to necessarily have a huge role in making CSR worse. Of course, I’ve not given patients with CSR periocular corticosteroids. I wouldn’t necessarily recommend it. There’s also been some interest in helicobacter pylori infection, and its association with CSC. It is thought that the helicobacter pylori is associated with thrombotic states, and this thrombosis can occur at the capillary level, and that can explain the effacement of the small choroidal vessels. That can explain the development of CSC. A French study found that in 40% of CSC patients, versus 20% of the general population, there was H pylori infection. If that was the case, treatment was performed for these patients, but this was done in multiple studies — this showed a mixed bag of results that didn’t really reduce the type of subretinal fluid absorption, nor did it significantly affect visual outcomes. One study showed that it did reduce the time, another did not, but visual outcomes were not affected, based on treatment of the H pylori infection. I think that was one of the questions about proton pump inhibitors, or H pylori-related care. There’s also some genetics at play. In the pathogenesis of CSC. 52% of the families of chronic CSC patients had CSC pathology in one study. And there’s a higher prevalence among certain racial groups. For example, whites, Hispanics, and Asians — you’ll see more CSC. Cytokine analysis was done of subretinal fluid in cases of CSC. VEGF was not found to be elevated in the CSR, and that may explain why when we give intravitreal Avastin, we get a mixed bag of results. Some people get better. Most don’t. The interleukins, IL-6, IL-8, were not elevated in this study. So inflammatory mediators don’t seem to play a role too much in the pathogenesis. There are also some insights related to imaging that we already touched on, specifically EDI imaging of the choroid. You can see that in active CSC, we’ll see significant thickening of the choroid. For example, greater than 368 microns of thickness. It’s interesting that choroidal thickness is reduced after therapy. And this was shown in a very elegant study. The OCT findings are interesting, because in active CSC, you may see outer nuclear layer thinning that correlates with the thickness of the epithelium. Patients that have 74 microns — where the outer nuclear layer is 74 microns, whereas the typical normal outer layer thickness is 125. There may be elongation of photoreceptors in cases of active CSC. You can also see white precipitates that occur, sometimes in the subretinal fluid, and sometimes in the deposits in the subretinal space, or within the outer segments of the retina. Etiology of this is unknown. Some people think it might be related to macrophages. The RPE detachment areas near the area of the leak — there may be detectable RPE breaches. This breach may be at the tip of the RPE. And you can often see evidence of these very dramatic spewing out of hyperreflective material into the subretinal space. I’ve seen this in multiple cases of CSC. Here are three examples of this spewing. You see this broad kind of spewing. It’s a very focal spew. The patient has the smokestack. And here’s one where it’s poorly defined in the RPE area of leak and pigmental vasculature is flattened. Here’s another variation of presentation of CSC, the so called serosanguineous CSC, where this patient actually looks like there’s fibrin and almost a bloody appearance to this area of pigmental detachment. This patient got better. Here’s the fluorescein angiogram of this patient. Typical CSC, you can see this focal area of leak, and a dramatic worsening. I followed this patient very carefully. This was before the advent of laser therapy. This patient on time domain OCT — you can see this old technology — and here he was about a month later. Spontaneous resolution. Serosanguineous CSC — sometimes you can see a bloody appearance. Frank subretinal blood should make you think twice about the possibility of CSC. You should really be thinking about neovascularization and chorioretinopathy. But this is different. That bloody appearance is very faint, reddish, choroidoretinal, rather than subretinal. You can see on OCT in active cases, especially after treatment, once the subretinal fluid is gone, that we see thinning of the fovea. And that thinning correlates to outer nuclear layer loss. This is shown in several studies. This autofluorescence can be very useful. It’s often in my opinion underrated in the management of some of these diseases. In acute CSC, where there’s just acute subretinal fluid, the fundus autofluorescence may be normal. But as the fluid is chronic and recurrent, you’ll see more dysfunction of the pigment epithelium. And with that dysfunctional pigment epithelium, you’ll see hyperautofluorescence of the RPE, as you see in the top picture here. And as the disease process waxes and wanes, you’ll see more pigment disruption. And this pigment disruption that you see in the bottom picture may help to explain to the patient — why am I not seeing properly? And this is a good question from these young, healthy patients, because they may be seeing 20/20, but it’s not a very good quality vision, because they had significant RPE dysfunction throughout the central macular area. So that can be very helpful. Whitish retinal precipitates may be seen in autofluorescence as well. And we think these may be correlated with the precipitates seen on OCT. This might be macrophages. There may be accumulation of shed outer segments in this serous retinal detachment that may show up this way. You can also see the late RPE dysfunction and atrophy type of autofluorescence. This is more common in chronic and so-called malignant cases of CSC, which you see in the pictures on the right here. You can see these flask-shaped deformities, where there’s tracts of subretinal fluid that have been there, and large serous exudative detachments of the retina, leaving behind these flask-shaped deformities. There may be hyperautofluorescence of the margins and hypoautofluorescence centrally, where the fluid has been chronically present, resulting in significant disruption and damage to the pigment. Remember that the hyper and hypoautofluorescence probably comes from the accumulation of lipofuscin. When stimulated with autofluorescence lighting. Whereas in the case of hypoautofluorescence, we’re seeing a window defect. There’s loss of pigment epithelium. And so you’ll see this hypoautofluorescence appearance and more substantial damage with this RPE atrophy. You can look at multifocal ERG. If you have these available, it will show much more widespread retinal dysfunction than you might expect, and this can help to explain to the patient why the patient will have increases in appearances of the disease. And it can show where the actual damage is. Because it’ll show reduced retinal sensitives retinal sensitivities in areas of RPE irregularity and ellipsoid zone disruption. You can see an example here. Adaptive optics have been more recently utilized to evaluate the exact appearance of the photoreceptors. And core density is decreased even if the ellipsoid zone is intact. Cone density is about 42380 in CSC, versus 67900 in controls. There have been some elegant studies done on this. I would ask that you look at these articles. It addresses the pathogenesis of the disease and helps in understanding some of the newer techniques utilized in studying this disease. The adaptive optics — the cone density really underscores the clinical relevance of this issue. There can be a significant reduction in the number of cones. This is something we didn’t really understand. We suspected this in the early days, but now we have objective evidence. Next poll question: The quality of evidence for therapeutic efficacy is best for which method of therapy for recurrent CSC? Diode micropulse laser, systemic eplerenone, photodynamic therapy, or systemic antiglucocorticosteroids? That’s right. Let’s move on to the next slide and we’ll discuss it. But photodynamic therapy is the answer. Why is that? Observation… You know, there’s actually some fair evidence, quality of evidence, if you look at the natural history of the disease. Most cases of acute CSC resolve in 1 to 4 months. A longer duration results in more permanent loss of cones, as we saw. So in some patients that have disease process with CSC, serous retinal detachments, that are lasting for more than a month or two, it makes me a little leery about leaving those patients alone. I start with risk factor modification. And I start with a conservative approach. And follow the patient who has an acute episode. So I typically will tell them to discontinue any exogenous corticosteroid use. And there’s good evidence to suggest that this has been — if there’s exogenous corticosteroid use, anything, topical, nasal sprays, systemic, regional injections — these things, if you stop them, there’s a good likelihood that the exacerbation in subretinal fluid will stop. I also suggest a reduction in physical stress. Using physical exercise, mindfulness meditation, et cetera. This might sound like an old wives tale, but long-term, you don’t want these patients to get exacerbations. If you can do things to avoid that, especially elevations in stress and endogenous release of cortisol that may exacerbate the choroidal vascular abnormalities that are already dysregulated, that may have a significant role in reducing this pathology. Reducing stress levels, regular exercise, and other things that you may have experienced with this yourself, personally, that may help the patient reduce stress, is useful. Avoiding phosphodiesterase inhibitors, such as sildenafil, tadalafil, that may be helpful as well. Illicit use of drugs such as ecstasy, which can definitely exacerbate CSR, are things to bring up to the patients. Helicobacter pylori… The quality of evidence for that being effective in CSR is very poor. It’s interesting that there is now a new study going on, on antiglucocorticosteroids. The quality of evidence for ketoconazole is pretty good, but now we have some new clinical trials. There was a retrospective study done that showed in 23 patients that there was a significant percentage of those patients had complete resolution with finasteride of their subretinal fluid, but once they discontinued the drug, a lot of them relapsed. Beta blockers are ineffective. For the treatment of CSC. They don’t have a substantial role in the management of symptoms. There’s very poor evidence in terms of carbonic anhydrase inhibitors. There was one study that showed some more rapid resolution compared to controls. That’s the only study. Anti-VEGF agents. Again, the quality of evidence is poor. There’s paucity of clinical trials. In the reports that are present, they have small sample sizes with poor follow-up. It’s hard to demonstrate a positive clinical effect, and in one case, PDT was found to be superior to ranibizumab. Laser photocoagulation in at least two randomized controlled trials found focal argon laser directly to the leak site was found to be effective when the focal leak site was easily identified, and particularly when it was outside of the fovea. There’s faster resolution of subretinal fluid. Usually within 6 weeks, as opposed to 16 weeks in the other trials. There does not seem to be substantial visual benefit. It did not seem to reduce the recurrence rate. There are many other case series with similar results for acute and recurrent CSC, but there appears to be no significant benefit compared to traditional treatments of CSC. And there does seem to be some potential risk. 10% of patients develop CNV later. So this is best reserved for the well defined extrafoveal area. Quality of evidence for photodynamic therapy in the treatment of CSCs is quite good. There are now multiple studies with large patient groups where even half dose PDT versus 30% PDT… 131 patients were followed for a year, and half of those got half dose PDT versus 30% dose PDT. It was found to be very helpful. Density of 600 watts per square centimeter. Reducing the photodynamic therapy, the laser… It may be really helpful too in reducing the risk of damage to the choroid, but still having a positive effect. I usually do this with full dose, but half dose PDT is effective. What’s interesting, of course, is that foveal RPE atrophy may occur after PDT. This was shown in 2009. Choroidal thinning may occur after PDT as well. And this may be a negative, because this choroidal thinning may then result in abnormalities of the choroid. This is a pigment epithelial detachment, and where my arrow is, there’s a small area of focal leak. In mid to late phases of the fluorescein angiogram, you can see the black areas of hypofluorescence, indicating disease. This patient was treated with phototherapy and had complete resolution. Here’s the autofluorescence image of that patient. Before and after treatment. You’re gonna see before treatment the subretinal fluid. You’ll see the margin of the subretinal fluid. Here’s the posttreatment. You can see the disruption of the pigment epithelium. Where the subretinal fluid was, you can see clearly the retinal vasculature, and you can see the detachment. Right now micropulse diode laser treatment, which has become increasingly commonly used for other disease processes, such as diffuse diabetic macula edema, there is not much evidence to show this is helpful. Small numbers of patients have been studied with this, using confluent micropulse diode laser in areas of well defined leaks. They showed resolution of subretinal fluid in a month, and there is a randomized controlled trial looking at a very small number of patients treated with the micropulse diode laser versus traditional argon laser. The suggestion was that micropulse diode laser resulted in faster visual recovery. With better contrast sensitivity and no scotomas. But again, there was a heterogeneous patient mix, inadequate follow-up, and varied outcomes were not easy to compare, so the quality of evidence of this is not particularly good. There has been increased use in eplerenone and spironolactone use of CSC, and the quality of evidence is fair. Because we know that aldosterone and the mineralocorticoid receptor may play a role in this disease. This was originally suggested by Marmor back in 1984. Spironolactone has a higher binding affinity to the corticosteroid receptor than eplerenone does, but seems to have more side effects too. But both drugs in a prospective study — eplerenone at 25 milligrams for CSC and then there was a crossover study after 30 days for another 30-day period with 50 milligrams a day — both of these showed reduced subretinal fluid and central subfoveal thickness. So I think eplerenone is readily available. And using this 25 milligrams daily can be helpful. There was only one case series that was done. I don’t know if there were any retrospective studies, but again, although the evidence is somewhat underwhelming, my clinical appearance has been that in maybe one quarter of patients that I use eplerenone, I see some improvement in CSC, in reduction of subretinal fluid. There are some patients where — when I stop the drug, the fluid tends to recur. And those patients probably would do better with phototherapy. Long-term concerns include potential for neovasculature, and you have to watch the serum potassium levels. These are potassium sparing diuretics. Nausea, leg cramps, thirst, dehydration. Orthostatic hypotension. These are all common side effects. So because of side effect profile concerns, I use this in a very limited way for treatment of CSC. Chronic CSC is a different animal all together. It’s a different kind of disease. You see diffuse, much more serious damage to the pigment epithelium. All the large flask-shaped deformities you see here on the autofluorescence imaging. You can see substantial loss of pigment epithelium in the periphery. This is a 52-year-old Asian woman with chronic CSC with incidental chronic resolved interior pathology. Here’s a patient with bilateral prominent CSC in both eyes, substantial loss of pigment epithelium. So these are things obviously you want to prevent. You don’t want patients to get to this level. That’s why using aggressive photodynamic therapy early, if you can, may be the best option for these chronic or late cases. You’ll see substantial disruption of pigment epithelium in this patient. With the flask-shaped deformities. There’s dramatic pigment epithelial changes. And of course, you can see this chronic exudative detachments in patients with CSC lasting more than six months. Here’s the angiography pattern, you can see areas of non-perfusion in the retinal detachment. And this patient was treated with photodynamic therapy. And it improved with resolution. So in a nutshell, the CSC has a broad spectrum of disease process. The vast majority of cases that we see don’t fall into the latter categories of really severe disease. Late complications of the pachychoroid pigment epitheliopathy can get neovascularization. Typically these are type 1 subretinal pigment epithelium CNV. The OCT shows broad shallow elevation of RPE suggestive of CNV. And the ICG shows plaque-like hyperfluorescence of type 1CNV and hyperpermeability lesions. Eventually you can see polypoidal choroid disease developing as well. This is an example of the pachychoroid neovascularization. This patient has thickening in the subretinal space and you see the classic polypoidal appearance here. I think the ICG is probably the most effective way to demonstrate these problems. On fluorescein angiogram, it’s often difficult to tell a polyp. So I think the ICG is very, very useful. Especially in the early phases, as the choroidal vessels fail, we see this cluster of problems show up. And here you see the presence of more chronic polyps, and subretinal fluid. And you see this type 2 neovascularization. In the chronic recurrences of CSC, and with the chronic neovasculopathy, you get this starting in the macula. As you see in this wide field. Here the patient still has active CSC going on. It may be in the central area. Higher magnification here. You can see the fluorescent angiogram in this patient. I put this in because of the wide field imaging technology. It’s increasing in prevalence in our practices and can be very helpful in grading the severity of the CSC or the neovasculopathy. So you can see multiple areas of peripheral RPE changes, as well as this central RPE destruction in this patient. Here’s another example of type 1 CNV with polyps. You can see on the earlier photograph that I showed you, where there’s chronic late stage angiopathy, with the development of polyps. There’s another variation of the theme here. Focal choroidal excavation is another variation, which is very rare. This is a patient who presented with an asymptomatic choroidal excavation, which are probably another manifestation, I think, of pachychoroid disease. These are thought to represent areas where the choroidal vasculature has collapsed. And they are conforming — this is a conforming choroidal excavation. It conforms to the contour of the choroidal excavation. Non-conforming ones, you’ll see the retina elevated over the subretinal fluid. But if you look at the fellow, the choroidal thickness above it, the choroidal thickness is substantially greater. The fellow eye had a very thickened choroid. Without excavation. The thickness was 600. Dramatically thickened. Here’s the fluorescein and OCT angiogram. You can see the relative hypofluorescence of the area of excavation indicating loss of choroidal vasculature. And you can see also that this patient truly had this pachychoroid area, and this is shown on the ICG angiogram. Dramatically decreased fluorescence, and you can see similar indications on the fellow eye. So that shows you that focal choroidal excavations are another manifestation of these pachychoroid diseases. As I mentioned, there isn’t any specific therapy for choroidal excavations. In non-conforming cases where there’s subretinal fluid, there has been some suggestion that anti-VEGF therapy might be useful, because it’s thought that these non-conforming areas might be associated with choroidal deepithelialization. So that’s the end of my talk. I hope this has been helpful and not too confusing. I want you to keep in mind that when you’re assessing the thickness of the choroid it’s very important to use spectral OCT imaging in order to determine whether or not pachychoroid disease is present. Understanding that photodynamic therapy is probably the most effective way and has the best evidence for the treatment, especially central serous. And recurrent disease. Acute disease, if it’s extrafoveal, well defined, can still be treated with focal laser. And the evidence for use of antiglucocorticosteroids is still weak. And is pending additional clinical studies. The evidence for eplerenone appears to be relatively weak to fair for the treatment of central serous chorioretinopathy, at least in my experience, and finally, the micropulse diode laser treatment is, again, not well studied to date. Although there are clinical studies that will be investigating this, especially in chronic recurring CSC. Thank you for your attention. I’ll take questions at this time.

>> Thank you, Dr. Moorthy. You can go ahead and stop sharing your screen. We have about five questions, if you want to open those up. It’s a Q and A right next to share.

DR MOORTHY: What is the difference between serous RD and neurosensory detachment? That’s a technicality. At least the way I look at it, neurosensory retinal detachments have different pathogenesis. Exudative retinal detachments — you’ll see subretinal fluid and elevation of the retina. The outer segments are off the RPE. Autoregulation is a feature of retinal microcirculation only. Is it present in the choroid too? Yes. There is significant autoregulation of the choroidal vessels. It is thought that the vasculature of these vessels may have significant autoregulation. So I think that that’s something that we’re beginning to understand. Is there an explanation for why topical steroids don’t worsen CSR? Maybe it’s because the earlier corticosteroids that were studied didn’t necessarily penetrate. Perhaps difluprednate, which is a much stronger topical steroid, may have an effect on CSR and may exacerbate CSR. I have many uveitis patients with weak corticosteroids systemically, topically, et cetera — more than a handful of patients that have both CSC and uveitis. Those are very, very difficult patients to treat. Those cases, photodynamic therapy has been a very good modality. Do you recommend use of antiandrogen for CSCR management? Again, the eplerenone and spironolactone, those have been shown to be of some benefit. In terms of the testosterone-related agent, I have no experience with that. I don’t know that there’s much evidence about those. But antiglucocorticoids — those are still being investigated. Is there an explanation for why topical steroids… I addressed that. Are there risk factors for serosanguineous? That’s an excellent question. I think it’s such a rare occurrence that I don’t know there are any particular risk factors. Exogenous exposure to corticosteroids is probably the most significant one. Is there a role of pregnancy in aggravating this condition? That is an excellent question. Pregnancy is an interesting immunologic state. And it has an effect on many different conditions. As you know with patients who have moderate diabetic retinopathy, for example, we’ll see dramatic worsening of diabetic retinopathy in the second and third trimester. In uveitis, there may be reduction in inflammatory activity. And there are significant flares after the child is delivered. I don’t know that I have seen patients with CSCR in pregnancy. But there is probably significant hormonal alteration, enough that there may be variation in disease activity. If someone has active CSR when they’re pregnant, that makes it difficult to decide how to treat. Obviously using exogenous agents… Eplerenone and those kinds of medications, for obvious reasons, you want to avoid that. You want to avoid photodynamic therapy, for obvious reasons. In that case, focal laser treatment is absolutely essential. It may be the best treatment modality. But I haven’t seen any active cases with pregnancy. I don’t know if anybody else here has seen it.

>> So that looks like all the questions, Dr. Moorthy. So this is probably a good place to stop. Thank you very much.

DR MOORTHY: Thank you, everybody, for your attention. I hope this was instructive.

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September 14, 2018

Last Updated: October 31, 2022

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