Ocular Syphilis

Hello, welcome to I3Ears, you're off the MOLGE O-CAPS and Board of View Podcasts. We're your host Ben Young and Andrew Powell. Please keep in mind that this podcast is meant for medical education only, not to diagnose that weird thing on your eye. We're off the MOLGE Resins who figure that reviewing for boards, the clinic, or O-CAPS is better when you don't have to do it alone. Each week we review a high yield topic and discuss the why and the how. This week we're going to be talking about ocular syphilis. As you know syphilis is a chronic multi-system infection originating from the organism trepanema paladum. It's one of the great masqueraders of medicine, and pretty much if you try to look for it in the BCSC, it'll pop up in almost every chapter of every book, especially on the differential diagnoses parts of. You have to make sure that your patient doesn't have it though, particularly when you're thinking about starting any kind of steroid treatment. Any time you have a patient that has inflammatory ocular disease, think about syphilis as we'll review in this episode. Before we get into ocular syphilis, we should review the stages of systemic syphilis. To help us do that, we brought on the hosts of the podcast Kriogs Over Coffee and Hobi Guine Board Review Podcast. So thanks Faye Kai and Nick Burns of Brown University and Kriogs Over Coffee for coming on. Yeah, of course. Yeah, thanks Ben for having us. So what syphilis do? What do syphilis? That's an excellent question. No, syphilis, as you said, has multiple different stages, so Faye and I will just quickly go ahead and review those. To start, primary syphilis is rarely seen truthfully because it's the presence of one or more painless shankers usually at the site of exposure. This generally happens within three weeks of infection and truthfully, these often heal on their own without treatment, though this is a very, very infectious stage of syphilis. We can then move on to secondary syphilis, which can manifest as mucocutaneous lesions like rashes, remember that syphilis is one of three diseases that can actually cause a rash on the palm and soul, as well as on other parts of the body. The other two, just to review, are Rocky-mounted spotted fever and kaksaki or hand foot mouth disease. This will usually fade about six weeks and can range anywhere between three months to six months after infection. You can also have things like condoloma lada on the genital area, mucous patches on the tongue, lyphanopathy, and constitutional symptoms, as well as alopecia. Again, secondary syphilis is very infectious. After secondary syphilis, there is a stage called latent syphilis, where really the only way that you can tell that somebody has syphilis is that they have reactive serologic testing. There are no symptoms per se of latent syphilis. The patients at this point are potentially infectious, but the longer time period that they've been latent, the less likely they are to be infectious. Finally, tertiary syphilis can occur three to fifteen years after initial infection. So this can be manifested as skin and bone manifestations, like gummies, which are soft tumor-like balls. They can also get neuro syphilis, which can actually occur on the earlier side, and this can be seen as syphilitic meningitis, or on the later side, like general parisis, tabby's or salus, which is poor balance, and lightning pain, the lower extremities, which can occur four to even twenty-five years after infection. Finally, they can also get cardiovascular syphilis. These include things like syphilitic aortaitis, which can lead to aortic aneurysms. But remember, during tertiary syphilis, patients are not usually infectious. I guess ocular syphilis is the other kind of, it's not really a stage per se, but it's something that can happen as far as we understand at any time point, but it's kind of gross. So we're going to turn this back over to you. Wow. Thank you for one dismissing our entire, no, okay? Wow. Thank you, Fay and Nick, for reviewing syphilis so comprehensively for us. If you'd like to hear more about syphilis, Fay and Nick did a whole episode on their podcast, dogs over coffee, kryags is like the O-caps for OB-GYN if you guys didn't know. And that is episode 20 for them, and I'll link that in the description below. Back to us, Andrew. So we can consider talking about ocular syphilis in two approaches, one being the syphilis that you might be born with if you're having a congenital form of it. That's a little different than the acquired form of syphilis. So to start with congenital syphilis, remember that it's typically transmitted to infants if the mother has either primary or secondary syphilis. Layton is much less likely to be transmitted though. It is always theoretically possible that we won't go too much into the systemic manifestations of syphilis because I'd be its whole own talk. But it can be helpful to remind you of Hutchinson's triad. The triad of congenital syphilis includes Hutchinson teeth, which are a small, widely spaced teeth with notches on them, eighth nerve deafness, and interstitial carotitis, which is the ocular manifestation that's pretty characteristic of syphilis. There are also many causes of having a pigmentary retinopathy, and syphilis, particularly congenital syphilis, is one such cause. There's also a subset of inflammatory ocular problems that can also cause high intracular pressure, and syphilis is another one of those. There is kind of a more unusual mnemonic that's already existed for a while that some people know is hot tonopens for the problems that have inflammation and increased intracular pressure. So for us, we've sort of reduced it down to HTPSS, like a website address or something, because that's, if you take out all the vowels in hot tonopen, you actually get the mnemonic. H stands for herpes, T stands for toxoplasmosis, P stands for Posner-slashman, and one S stands for sarcoid and the last S stands for syphilis. So now we can move on to acquired syphilis. So to remind you, acquired syphilis really can cause inflammation in any part of the eye, whether that be, it can be unilateral, bilateral, it can be granulomitus, or it can be non-granulomitus. So we're just going to go from front to back, and honestly, we're going to name pretty much every structure in the eye and say it can be inflamed. Hit it, Andrew. Okay. All right. So we'll start with the most classic thing, the interstitial caratitis. The interstitial caratitis that is pretty classic for syphilis actually can manifest as what's known as salmon patch caratitis, too. And that's not exactly like just having a patch of redness over the cornea instead of the conjunctiva. So the first thing that happens in interstitial caratitis is rapidly progressing corneal edema, followed by a deep vascularization of the deep stroma just next to decimase. That vascularization gives it a salmon colored or reddish hue to the entire cornea. And after a while, after several weeks, two months, blood flow through that new vascularization seizes, leaving behind empty ghost vessels in the cornea. You can also get what are called gamma of the iris, just like, you know, interstitial syphilis. You can get gamma, you can get them in the iris, or you can get papules, or nodules in the iris as well. And additionally, because the iris can become a flame, you can get posterior sneakier. Remember iris, two lens adhesions. Syphilis can also be a cause of lens dislocation, or ectopia lentis. It's also one of the known causes of intermediate uviitis, so it can be a masquerader for parseplenitis. So if you see someone with intermediate uviitis, I either have detritis or classic snowball snowbanks in their, in the parseplenitis, then consider syphilis under differential. One kind of classic way that can manifest is corioretinitis. It typically presents as a posterior placoid corioretinitis. And the pier, it has a very characteristic appearance where it's a plaque. And there is an arc at the edge of that plaque that's kind of like a leading edge of disease. So you'll see that there's one that's kind of more elevated, more granular than the rest of the plaque. It's usually one plaque, it can be multiple plaques. Its appearance is characteristic enough. It can be considered pathachnemonic for secondary syphilis. If you OCT over these plaques, you'll see that there's disruption of the ISO's junction. And if you look very carefully, you can even see spiral keats or inflammatory debris in the corioretinitis, because posterior placoid corioretinitis due to syphilis is caused by the spiral keats entering the corioretinitis via the corioretin causing direct inflammation of the photoreceptors. This can lead to decreased vision, of course, because of the destruction of the photoreceptors, but also photopsias, because as the photoreceptors are inflamed, they can cause flashes, because it's a direct photoreceptor inflammation. One more retinal manifestation of syphilis is focal retinitis. It can look very similar to acute retinal necrosis or progressive outer retinal necrosis, which are diseases more commonly associated with herpes viruses such as zoster or CMV. So if you see these in a patient, or see finding similar to that in the patient, keep surface in the back of your mind as a possibility if it doesn't quite match a typical pattern of ARN or PRN. And it can also manifest as retinal vasculitis affecting either arteries or veins. And remember, many ocular syndromes or diseases either affect one or the other, and you can distinguish vasculitis in that regard, but syphilis can do both. Either one. You can also get serious retinal detachments, papillitis, and neuroretinitis. Then you want to talk about the differential of neuroretinitis. So this is a reminder, neuroretinitis is inflammation of the optic nerve, as well as the retina. So it's both. You know, the classically what they say is you'll have optic dyskidema with a, quote, macular star. A macular star is just exudates left by fluid that was in the macula. And it kind of dries up in a star-like pattern because that star is the pattern of the nerve fibers of Henley, which is that outer plexiform layer as it kind of exits to fovia. So if you see neuroretinitis, then you know, there's a couple things that I'm differential, including syphilis. Other things to consider are Bartonella Hensley, which is one of the main ocular manifestations of cat scratch fever. So definitely consider Bartonella and test for it. If you see a patient with neuroretinitis, you can also test for sarcoid and lime. And then finally, the syphilis you can't affect the neuro-optimologic system almost in any way. It can give you individual cranial nerve policies. It can give you an atypical optic neuritis. The classic teaching in medical school is you can get an argyle, Robertson pupil, which to remind you are pupils that accommodate, so they constrictive accommodation, but they don't react to light. So these are patients with light near dissociation. In reality, according to the text that we we reference for these podcasts, it appears to be very, very rare, but you know, that's what's tested on boards and invent school. So you know, that's a pretty, that's a rundown of the ocular manifestations of syphilis. Basically, take a structure that you want to think about and it can get inflamed. Next, we'll just talk about the diagnosis of syphilis from an ophthalmologic standpoint and then finally a bit about the treatment of syphilis. So Andrew, what's the first step about trying to diagnose syphilis? Well, if you're ever in a position where you're thinking that you have to try sending a syphilis study, you should also be thinking about all the other things that syphilis can masquerade as and in general, you're always safe sending things for syphilis, things for sarcoid, and things for tuberculosis all in the same breath just as a reflex whenever you're thinking, hmm, inflammation in the eye, what are my go-to's? So think of sarcoid syphilis and TB all in the same breath. Once you're more established that it's probably something related to maybe a sexually transmitted disease, I always remember also to send for HIV studies as well. More specifically, there are a whole bunch of syphilis studies. Then how can we wait through this forest of acronyms of sexually transmitted acronyms? I'm afraid a step one first aid. So moving on to treatment now, okay. So, you know, I'll just broadly go over the diagnostic tests used for syphilis. There are non-trippinimal tests, those are VDRL and RPR, and then there's tests that let are trippinimal tests, that's FTA, ABS. So to categorize those two, so VDRL, RPR, so VDRL and RPR, again, your non-trippinimal tests, those tests will increase with disease activity, and then the levels reported by those tests will decrease as a disease is treated, or if it becomes latent, so basically as the disease becomes less active. Hence, VDRL and RPR are useful for following disease activity. However, it's important to know that there's a lot of things that can cause false positives in VDRL. Initially, I was going to list out all those things, but the list got so long that I just figured it was worth just saying that. All my huge numbers used to call "cause false positives," you can't rely on that. In contrast, trippinimal tests, with the main one being FTA, ABS, becomes positive only after someone has entered the second stage of syphilis, but then remains positive. False positives are much rarer in FTA, ABS, but it can occur in cases where a patient has another spire key, so it includes Lyme, a leptosporesis, if they have an autoimmune disease, malaria, or leprosy. Those four are things to think about that can cause a false positive in FTA, ABS. If it helps you to remember the difference between the two, I remember that VDRL vanishes, so if it's treated, the levels will go down. Whereas FTA is fixed, so VDRL vanishes, FTA is fixed. That helps you remember how each level will appear at different time courses in the patient's disease. Besides drug blood for these tests, Andrew, if you see someone diagnosed with some ocular manifestation of syphilis, is there anything else you'd do besides drug blood? If you have ocular syphilis, remember that counts as neuro syphilis. We have to consider the eyes as an extension of the CNS, so your diagnostic testing needs to extend down to the spinal cord and it's fluid too. So, rope in your friendliest neurologist and ask them to poke needles into people's specs. Right, so any patient with syphilitic UVitis needs to be treated as if they have neuro syphilis. And that leads us into the treatment of syphilis, so because they need to be treated as if they have neuro syphilis, you know, IM, penicillin is just not enough. They need 10 to 14 days of IV penicillin, which required admission and medicine/ID co-management. Just to come back to congenital syphilis, that also needs to be treated for around 10 days. But in that case, IV or IM is acceptable for congenital syphilis. Then, what if my syphilitic patient is allergic to penicillin? So, there are no proven alternatives for penicillin and treatment of syphilis, so if a patient is allergic to the recommendation, I'm in standard care, is to desensitize them to penicillin and treat them with penicillin. Another thing to think about when you're treating syphilis is that you have to monitor them pretty closely, because you have to observe for a generous Hirshheimer reaction to remind you from medical school days. This is an intense hypersensitivity response to the antigens produced by the treponeum as the spirochetes die during the first 24 or so hours of treatment. As a result, they can have increased inflammatory response, so it can look almost like a disease is worsening, and that may require local or systemic steroids. In some cases, you can use eye drops to treat them, you may require more aggressive steroids or systemic steroids. Now, as soon as you've diagnosed syphilis, patch yourself on the back, you've found the elusive masquerade. You should feel proud because you've pinned down a pretty elusive disease, then you have to alert local authorities, because this is a notifiable disease. Local governments and local health agencies really would like to know if syphilis is popping up in their communities. So let them know, and then you have to identify your patient's sexual partners and then talk with them about trying to get them treated as well. That's all we have for the super fun topic of ocular syphilis. If you like what you heard, you can follow us on Twitter @eyes4earys with number four. Or you can check out our website, which is eyes4earys.net, but the word four. If you'd like to support the podcast, it really helps us if you give us a reading and review on iTunes. And one more big thank you to Fei, Kai, and Nick Burns of the podcast, Kriyogs River Coffee. Again, if you know people who are into OBGYN or who are doing OBGYN, and want a podcast that reviews high-yield OB topics in quick 10 to 15-minute chunks, then we highly recommend Kriyogs River Coffee to them. Kriyogs River Coffee really helped us when we were starting our podcast and gave us a lot of advice and tips on how to make ours. So if you want the best in OBGYN review, we highly recommend them. And thank you to our listeners for supporting us just by listening. We'll see you next week. Bye. Bye.