Orbital Tumors: The Value of an Endonasal Surgical Approach

Podcast Transcript

Intro: Neuro Pathways, a Cleveland Clinic podcast exploring the latest research discoveries and clinical advances in the fields of neurology, neurosurgery, neuro-rehab, and psychiatry.

Glen Stevens, MD, PhD: Schwannomas of the orbit are rare and account for only 1-6% of all orbital tumors. Although typically benign, these tumors can be difficult to manage, particularly in the narrow setting of the optic canal. From an ophthalmological standpoint, surgically assessing the tumor through the eye would further compress the optic nerve, potentially risking full loss of vision in the eye. Radiation therapy can be used in an effort to slow tumor growth. However, it does not remove the tumor and carries its own risk to the eye. A multidisciplinary team approach is key to removing these tumors without additional injury and to obtain successful outcome.

In today's episode of Neuro Pathways, we're discussing the complexities in the diagnosis and management of orbital schwannomas or other tumors associated with the orbit. I'm your host Glen Stevens, neurologist/neuro-oncologist, in Cleveland Clinic's Neurological Institute. I'm very pleased to have Dr. Pablo Recinos and Dr. Raj Sindwani join me for today's conversation. Dr. Recinos is a neurosurgeon in Cleveland Clinic's Neurological Institute, and Dr. Sindwani is a rhinologist in Cleveland Clinic’s Head and Neck Institute. Pablo and Raj, welcome to Neuro Pathways.

Pablo Recinos, MD: Thank you very much, Glen. Very happy to be here.

Raj Sindwani, MD: Thank you, Glen.

Glen Stevens, MD, PhD: So when I say I trust these guys with my life, I really mean it. A couple of years ago, I was diagnosed with a tumor at the base of my skull. I found the “Dream Team” of these two guys that did my surgery without complication, I have to admit. Here I am, I think probably three years later, tumor free and doing fine. So thank you, gentlemen. It's a pleasure to have you.

Pablo Recinos, MD: No greater privilege than to have a colleague as a patient.

Glen Stevens, MD, PhD: So schwannomas, particularly in the orbital region, are extremely rare and can cause serious damage or even vision loss. Given that's the case, Dr. Sindwani, can you start off today's conversation and tell us about some of the clinical manifestations seen in patients diagnosed with either orbital schwannomas or other tumors of the orbit?

Raj Sindwani, MD: Sure. Glen, and thank you again for having us. As you nicely pointed out in your introduction, anything that affects the orbit, which is essentially a bony box, can cause disturbance in the functioning of the eye. Of course, most notably, that is in vision loss. And indeed the 65 year-old lady that we're discussing, who had that schwannoma, did present with progressive visual loss in her right eye. She also was complaining of a pressure and pain sensation in the back of her eye, which led her to see an ophthalmologist.

Glen Stevens, MD, PhD: So what about other patients? Is that the portal? Most patients will come through ophthalmology, that have orbital tumors, I would assume?

Raj Sindwani, MD: Yeah, your assumption's exactly right. You have an eye problem, so you see an eye doctor. Now often they'll be treated for other things, like the patient that we're referring to, actually had cataract surgery, with the professionals who manage her thinking maybe the cataract was responsible for her declining vision. It was only when she didn't get better after that intervention, that she sought other help, saw one of our neuro-ophthalmologists, Dr. Kosmorsky, who ended up getting an MRI scan, which revealed the underlying diagnosis.

Glen Stevens, MD, PhD: If I'm just sitting there thinking about, "Could I have an orbital tumor?" Anything in particular that stands out symptomatically for these patients?

Raj Sindwani, MD: Well it's usually a progressive type of situation where you might have some minor visual changes here and there, but if they get better then it's probably nothing serious. In this case, it was clearly progressive to the point that she actually could not see very well and was almost blind in that right eye. Things like pain, double vision, even changes in color vision can be a subtle finding, and subtle sign that there could be something more sinister going on.

Pablo Recinos, MD: I would add that common things are common. Things like cataracts are very common and as we age, it's very common for people to suffer from cataracts. However, if a procedure is done for cataracts and there's no significant improvement in vision, that's a common story that people present with and need further workup and evaluation after a treatment of cataract may not have the improvement that would otherwise be expected.

Glen Stevens, MD, PhD: Yeah, it sort of reminds me, I've seen several cases over the years of individuals that presented with progressive weakness. Then they did an MRI of the cervical spine and they had spinal stenosis, had surgery, but did not get better. Then down the road, ended up being diagnosed with Lou Gehrig's Disease. I think that we see these common problems throughout medicine and we always just have to keep our radar high. Certainly with patients that aren't improving. So you touched on it a little bit and of course we have two individuals here from different disciplines, but talk about your multidisciplinary team that you formed.

Raj Sindwani, MD: Sure, so even though, as you pointed out, Dr. Recinos and I as the “Dream Team”, our “Dream Team” is actually much bigger than just the two of us. It does include the neuro-ophthalmologist, oncologists, neuro-radiologist, other head and neck surgeons, and even other neurosurgeons who often will refer patients to us for a minimally invasive approach, through the nose, to get to the back of the eye or to different recesses of the head.

Essentially in this case, our neuro-ophthalmologist colleague Dr. Kosmorsky, after diagnosing through imaging, the schwannoma, appreciated that due to the location of this tumor, specifically at the back of the eye, meaning posterior and more in the medial quadrant, he realized that that's a very amenable location to an endonasal approach. Essentially what we do is we use the nose and sinuses, which as you know are largely contained with air, as a corridor to get to the back of the eye, gently take down the bone partitioning the nose from the eye, and access the tumor through that direct line approach to the orbit.

Pablo Recinos, MD: I would add that our experience has grown from starting with pituitary surgery. Which you started to talk about at the beginning and in your own experience. Pituitary surgery is the most common procedure that we do together. However, flanked on each side of the pituitary gland are the optic nerves, and then the orbit. So these are natural areas that we work in, both for tumors that arise from the intercranial space, but now expanded for ones that arise primarily within the back of the orbit. To work together to tackle problems in this area is a natural extension from where we have worked together.

Raj Sindwani, MD: That's a good point, Pablo, and really the approach of having two surgeons using multiple hands, with multiple instruments going through the nose is actually exactly the same that we use for pituitary tumors and other skull based tumors. Essentially this multi-handed technique is what allows us to safely dissect, reflect and retract structures within the orbit, in this case, to then safely remove the tumor without disrupting any of the normal critical structures that are nearby.

Glen Stevens, MD, PhD: So if we got in our reverse time machine and we went back 20-25 years, what would our surgical approach be? Or didn't we have a surgical approach that we could really do? Or was the morbidity/mortality too high?

Pablo Recinos, MD: We don't have to travel back that far, in fact, to envision that. Depending on where the problem arises from within the orbit, it may be amenable to a different approach. If something is at the very roof and perhaps on the lateral aspect of the orbit that may be best approached through a craniotomy, that's still the case. The orbital apex and the back of the orbit is such a narrow corridor that our ophthalmology colleagues, not infrequently, will say, "Well, is there another approach other than going through the orbit itself to get to that delicate area?" The main reason is that you want to minimize any traction, any manipulation of the optic nerve, which is frequently already severely compromised in these cases. That's why we come from a different angle such as through a craniotomy or through the nose.

The endonasal approaches are much more recent. For many of the cases that Dr. Sindwani and I have operated on, in fact the ophthalmologist reached out to us and said, "Do you have anything different? Because we believe that the morbidity associated with our approaches is simply too high." That has not been a very long standing thing, which is why I mentioned that we don't have to go back 20 years.

Glen Stevens, MD, PhD: I'm just curious, in terms of the surgery itself, Pablo. How's the separation from the nerve with the tumor? Is it complicated? Risk to the nerve with the surgery itself?

Pablo Recinos, MD: One of the things that we keep in mind, regardless of whether we're going through the nose or whether I'm just doing a craniotomy on a displaced nerve, is to not put more additional pressure on a nerve that's already being pressed. For example, in a case like this, the optic nerve was being displaced laterally, so all of our maneuvers have to be focused on coming around the tumor, but putting pressure towards the nose or away from the nerve so that the nerve is not stretched any more than it is at the beginning.

Glen Stevens, MD, PhD: Have you done any separation type surgeries? I know this is done in the spine with people that have malignant tumors to get it away from the spinal cord. Has anybody looked at this or you guys are pretty comfortable in removing the tumor and you don't need separation surgery for radiation?

Pablo Recinos, MD: I think that's a great question. The concept of separation surgery, as you alluded to, is to create more space between a delicate structure. In that case, the spinal cord and the area that's going to be radiated in question. An emerging area in the orbit which is not called separation surgery, but perhaps is similar in idea, is for example, with optic nerve sheath meningiomas. So tumors of the optic nerve sheath have typically responded very poorly to surgery because the tumor is intimately embedded with the vasculature of the optic nerve itself and by peeling the tumor off you de-vascularize the tumor. Therefore, the better outcomes have been with either observation alone or radiation.

An emerging area is, "Do we go in and open up the optic canal and expand the orbital apex prior to a procedure such as radiation which may cause swelling, even transiently, to minimize the possibility of visual worsening during that time."

Raj Sindwani, MD: In the ENT space this idea of decompression to relieve pressure, actually on the orbit, also has been done for quite some time in the setting of graves orbitopathy or thyroid eye disease. Where you have increased interocular pressure due to extra deposition of materials so that the patient's eye is bulging. We again use an endonasal corridor, would go in and decompress the bony medial wall of the orbit and incise the lamina, that's the investing soft tissue or fascia that houses the orbital contents, to relieve pressure, thereby decompressing it into the sinus. Effectively as Dr. Recinos was explaining, that trajectory and corridor we used to get to the tumor. Remember the start of the procedure is actually to decompress the orbit and the periorbita, so right away, you actually get a little bit of decrease in pressure on the optic nerve. Then we continue to decompress things medially to progressively take that pressure away, eventually removing the entirety of the tumor.

Glen Stevens, MD, PhD: So Raj, you had mentioned that we're not going to see an external scar on these patients, but you are obviously going in through the nares and you're going to be affecting tissue. What do we do coming out to make sure everything is sealed and heals properly?

Raj Sindwani, MD: Yeah, that's a good question, Glen. We have a lot of experience with accessing the orbit through the nose, with our history of the orbital decompressions we did for graves that we alluded to earlier. What we learned from that is that you actually don't have to reconstruct or put any barriers back in to separate the nose and the eye. The body heals that area on its own. Remember, most of these tumors are relatively small when we take them out. We've had great success in not reconstructing the medial wall of the orbit when that's a very limited approach that we use.

Now, if you take out the orbital floor or do a more complex or advanced surgery, sometimes reconstruction is required, but for these posterior apex lesions, usually no reconstruction is required. The other thing I'll point out on the way out, as you mentioned at the end of the surgery, we rarely if ever have to put any packing materials or any splints in place. We've had great experience going through the nose for a whole host of different types of pathologies and what we've learned is that we can be very minimal in our reconstructive and packing materials that are used. Which helps a patient from a recovery standpoint.

Pablo Recinos, MD: We do discuss with the patient, significantly, expectations after surgery. Ironically, in some of these patients that have had near complete vision loss, once they start regaining vision in the weeks to first few months of healing, they can have inflammation of the muscles that we worked around, which can cause double vision. They can go from not having vision to double vision. That usually is a transient thing, which ends up resolving over time. But it is important to educate the patients, "Hey, you might be seeing better out of that eye, but the eyes may not be moving perfectly in sync right away. And that's not a sign of, of concern. It just takes time for that inflammation to resolve."

Glen Stevens, MD, PhD: So we've mentioned schwannomas, meningiomas, any other tumors that we commonly see that you guys do this type of approach on?

Raj Sindwani, MD: Yeah, in the intraconal space at the back of the orbit, the most common pathology that grows there are actually hemangiomas, within that intraconal space. But we've seen a wide variety and we recently looked at our experience of over 15 of these intraconal or intra-orbital tumors. There were other weird and wonderful things. Metastases for example, from breast cancer, from prostate cancer, et cetera.

Glen Stevens, MD, PhD: So I was fortunately asleep during my surgery, but I heard from the OR nurses that there was quite a dance going on between you two. So I'm sure that there's a lot of interplay between who does what, when they do it. Talk about that a little bit.

Raj Sindwani, MD: Sure.

Pablo Recinos, MD: I can't imagine what it would have been like if you would have been awake with the three of us dancing, Glen, so I'm glad you were asleep for that part.

Raj Sindwani, MD: Two is company, three's a crowd in that case, I think, Glen. But you're exactly right. No longer is this type of work through the eye or the nose a serial partnership where the ENT would come in, make a hole, neurosurgeon comes in and takes out the tumor, then the ENT surgeon tags in. It really is a dance because both of us are there simultaneously. What that accomplishes is it allows us to have multiple instruments going in through the nose, as I mentioned simultaneously, to actually perform more complex and delicate maneuvers around critical structures. So while it's true that maybe at the beginning and towards the end, from the reconstruction standpoint, I may take the lead and Dr. Recinos during the tumor removal, we actually are working together. So you get the advantage of not just multiple hands and multiple instruments, but also two brains and two perspectives bringing brought to bear on each of the moves that we're performing.

Pablo Recinos, MD: The interesting thing is these techniques have evolved. One of the critical aspects of the evolution was the improvement in instrumentation. In pure rhinology or ENT procedures, the instrumentation was all focused on "Can I have one hand that does five things?" And as soon as the two of us started really working together, where you have three or four hand approaches, we realized we can do a lot more without a Swiss army knife type tool. Would you agree with that Raj?

Raj Sindwani, MD: No, absolutely, and that's been great to see the borrowing of instrumentation from your specialty to ours, and using some of the tools we've adapted going into the, the more neurological or neurosurgical spaces as well. There's been a lot of synergy that we've brought together through this multidisciplinary approach.

Pablo Recinos, MD: Yeah, that cross-pollination has definitely happened though by the two minds working together, and adapting tools that we've used in our individual disciplines, but then all of the sudden merging them into the same type of approach.

Glen Stevens, MD, PhD: So for the person that's been the serial person, how do we convince them to join a team?

Raj Sindwani, MD: Well, I think as you look through the literature and just to see how large high volume centers like ours and others across the country have evolved, I think the outcomes really would be what sets off the alarms that people should look to innovate and try to do things differently. The surgeries that we're talking about, compared to traditional approaches which may require craniotomy or a cut near the eye for example, we do all of these cases, yours included, as you know, Glen, without a cut or bruise on the nose. The direct line approach that we use in scenarios where it's indicated is a great advantage, because it actually does allow you not to disrupt normal structures and more directly get right to where the pathology is.

Glen Stevens, MD, PhD: My wife says, "Thank you for making me as handsome coming out as I was going in."

Pablo Recinos, MD: Well, she didn't have any extra requests, Glen. One way or the other. We were not allowed to make any adjustments in you. One of the things, just to add I think, is a common shared vision, Glen. I think, certainly in neurosurgery, historically dealing with skull-based issues had been a much more solitary endeavor. One surgeon really taking on the challenge by him or herself. I think now we're turning the page where we're seeing a much greater advantage or we're able to accomplish a lot more through the multidisciplinary approach. Through working together as a team, but it really does require a different mindset that has to be there from the beginning. I know Dr. Sindwani, actually, we were partners from the very beginning, but before that he actually went through some building steps, which made it a little bit more evident on the importance of being on the same page.

Glen Stevens, MD, PhD: Excellent. So we're going to give a little data before it's published, if we're able to, here. I understand you have a series of patients where you've done surgery on orbital schwannomas. Can you tell us a little bit about what you're series shows?

Raj Sindwani, MD: So we've published on this already, just a variety of tumors involving the orbital apex, but what we're currently looking at is specifically intraconal pathology. Not just schwannomas, but hemangiomas and other things as well, where we use this corridor approach going through the nose, splitting between the medial rectus and the inferior rectus and entering, and actually operating within that ever so complicated intraconal space. As you would imagine, these are very, very rare tumors. The series that we have is between 15, and up to 20 we're looking at now, that we have done through this exact approach and reporting not just the nuances of our technique, but also the outcomes that we've had. Which really have been amazingly impressive to watch.

Pablo Recinos, MD: I think that, although Raj mentions that these are rare, yes they are, but they're perhaps not as rare as one would think. One of the issues that we've encountered, even at our own institution, was education of some of our colleagues to not throw in the towel before considering some of these approaches, which were not part of what they would be thinking would be an option. As people become more aware that some of these techniques can be an alternative approach with really good outcomes, then we start seeing a few more of these cases then than we originally saw.

Glen Stevens, MD, PhD: Excellent. You kind of almost asked my last question and that was going to be, what advice would you like to leave our listeners in terms of treatment options that are available for orbital schwannomas or other orbital tumors? I think you kind of nicely answered that. But would you like to add anything else, or Raj?

Raj Sindwani, MD: Yeah, I would just say, in addition to the awareness that depending on the location of the tumor, going through the nose is absolutely a viable option and maybe even the preferred or best option. I think the other thing I would point out is that time is of the essence. We talked about how the optic nerve is a very sensitive structure, so how much pressure it's under and how long that pressure is there does impact outcomes. We always set the stage that we're doing surgery to decrease ongoing vision loss or progressive loss. We can't always guarantee that the vision that has been already lost is going to come back. Now fortunately in our experience, we've had many cases where that has been the case, that you've had reversible improvement in the vision, but that's not always the case, so I would just let people know that time is of the essence when you have one of these issues.

Pablo Recinos, MD: I would add that, for our colleagues, for example, not to be disappointed or to stop too early, if treatment of common things is not resulting in the desired outcome. To consider further exploration of the problem through imaging is one of the most common ways because often that's how these are found. At that point to just be aware that there are really good alternatives to get back to this really challenging area, which is the orbital apex and the posterior intraconal space.

Glen Stevens, MD, PhD: Well, Pablo and Raj, thank you very much for joining us today. It's been very insightful conversation and I appreciate your time. Thank you very much.

Raj Sindwani, MD: Our pleasure. Thank you, Glen.

Pablo Recinos, MD: Thank you.

Outro: This concludes this episode of Neuro Pathways. You can find additional podcast episodes on our website, clevelandclinic.org/neuropodcast. Or subscribe to the podcast on iTunes, Google Play, Spotify, or wherever you get your podcasts. And don't forget, you can access real-time updates from experts in Cleveland Clinic's Neurological Institute on our consult QD website. That's consultqd.clevelandclinic.org/neuro, or follow us on Twitter @CleClinicMD, all one word. And thank you for listening.