Transcription:
Case Report: Pulsatile Proptosis Due To Orbitocranial Trauma

Melanie Cole (Host): Today, we're discussing the case of a young woman who presented with pulsatile proptosis for years after a childhood orbital cranial trauma. Welcome to Better Edge, a Northwestern Medicine podcast for physicians. I'm Melanie Cole. And joining me in this panel are Dr. Stephen Magill, he's an Assistant Professor of Neurologic Surgery at Northwestern Medicine, and Dr. Liza Cohen, she's an Assistant Professor of Ophthalmology at Northwestern Medicine.

Doctors, thank you so much for joining us to share this case report today. Dr. Magill, I'd like to start with you. What types of problems affect the brain where you might benefit from having both a brain surgeon and an orbital surgeon?

Dr Stephen Magill: Within brain surgery, well, there's brain and spine surgery, and I'm primarily focused on brain surgery and, within that, I focus on the skull base. And the skull base is really the area between the brain and the face. And so of course, in the front, we have the brain just behind the eye. One of the most common problems is meningiomas that can affect the skull base and they can make the bone of the skull base get thicker, which can cause proptosis of the orbit. So anytime I have a tumor that is either compressing the orbit and especially when tumors invade the orbit, which meningiomas can do, and then they're involved with the intraconal part of the orbit, the muscles of extraocular movement, in those types of situations, it's really helpful to have the expertise of an orbital surgeon like Dr. Cohen or an oculoplastic surgeon.

Dr Liza Cohen: Thanks, Dr. Magill, for bringing up those issues where our specialties can work together. As an orbit specialist, I deal with problems involving the soft tissues within the eye socket, as well as the bones that surround those soft tissues. So occasionally, I encounter tumors of the orbit. In particular, ones that involves the bone that separates the orbit from the skull base. And we can work together in those types of cases to remove the tumors, reconstruct the orbital walls if necessary, and provide the best outcomes for patients.

Another situation in which an orbit specialist like myself and a neurosurgeon like yourself can work together are in cases of trauma. Oftentimes, patients who sustain significant face trauma will have either a fracture involving the roof of the orbit, that requires both of our specialties to work together or even patients who develop loss of vision from trauma to the optic nerve may benefit from a combined approach through the expertise of both an orbit specialist, as well as a neurosurgeon to decompress the bone around the optic nerve and save patient's vision.

Dr Stephen Magill: Yeah, I think trauma is definitely an important place. There's benign tumors, like I mentioned, as well as cancers often. We have a number of cases coming up, I think, where we have cancers involving within the orbit or around the orbit. And so when those windows invade into both the orbit and you need to get clean margins, we may be taking out a cancer that's involving the frontal lobe, but it goes down and invades into the orbit and the best surgery for cancer is to get clean margins. And so having an oculoplastic surgeon or an orbital surgeon like Dr. Cohen to help is really important or, vice versa, tumors that are in the orbit going up to the brain, maybe you need to take the dura or margin of the brain that's near the cancer. And so we often work together on those cases also.

In addition to trauma, cancer, things like that, another area that's very exciting where our fields overlap is in minimally-invasive surgery. And for some of these approaches in tumors, instead of having to do a large craniotomy or something, we can actually do approaches where we go through the eyelid and then go up towards the brain that way. So whenever I'm going to do an approach that involves working right around the orbit or going through the eyebrow or the eyelid, I like to involve one of our oculoplastic surgeons, because they can really help optimize that corridor. And then we can make a much smaller approach to get into or around the brain than we would have in the past. So that's an area that's really emerging, surgically, which is exciting.

Melanie Cole (Host): Thank you both for that summary. It's a very comprehensive approach. So Dr. Magill, I'd like to talk about the recent case of the young woman who presented with post-traumatic pulsatile proptosis. Describe this case for us. And then, Dr. Cohen, I'd like you to jump in with your role and how you both worked together on this case.

Dr Stephen Magill: Yeah, this was a really interesting case was actually referred to me by neuro-ophthalmologist. This is a young woman in her early 20s who had been hit by a trailer when she was a child. They never really thought anything of it. I think she was two or three years old when she was hit. Over her childhood, her mother would report that sometimes whenever she would bear down really hard, her eye would start to bulge out and sometimes she'd do it as a joke, but it was just something that was sort of in the family lore. That stopped as she got older and developed and was living her life working. But she began to develop in her teenage years this sensation of pulsation in her eye that was pretty continuous. And it led her to develop a migraine syndrome and really disabling headaches. And she saw a number of surgeons in several other states and would tell this to them. And when you looked at her, it was very difficult to see the actual pulsation. So people thought maybe she's a little crazy or didn't really take her seriously. But these headaches were getting progressively disabling. And as you can imagine, even a very small pulsation that you can't see when you're trying to drive and your eye is moving back and forth, when you're trying to work, when you're trying to live your life, this could be really frustrating and disabling.

And so one of our neuro-ophthalmologists here at Northwestern, Dr. Volpi, saw her in the clinic and as he examined her very carefully and with his experience and skill, he was actually able to detect some of that pulsation. And so we got some imaging. And when we did the imaging, we had both a CT and an MRI, and what they found was there was a bony defect on the medial side of the orbit. And on the CT scan, some of the orbital contents were actually herniating up in towards the brain. So normally, the orbit is completely encased in bone, but when there was a defect, now they started to think, "Hey, maybe something is going on where the pulsations of the brain are actually being transmitted to the eye and this could be leading to her symptoms."

We then went to get an MRI to better characterize this defect. And one of our really excellent neuro-radiologists. who was reading the MRI, went back and looked at the CT. And on the CT, the orbital fat had been going up into the brain. But on the MRI, there actually was a cyst in her brain and the brain contents were pushing into the orbit. And so by comparing those two scans and with the excellent care and rigor that he had looking over it, you could actually see two different time points in her pulsation between heartbeats that just happened to be on the two scans. You could see there was actually a dynamic movement between the brain contents and the orbital contents pushing back and forth into the orbit in systole and then back in towards the brain in diastole with the heartbeat.

So when we saw that, you could see there was a dynamic radiologic finding that would explain her symptoms and fit with them. So at that point, we began to discuss options for treatment. And typically, when you have some of the brain contents herniating out of the brain and a little cyst that had developed in her brain, we call that a meningoencephalocele. Because it went into the orbit, it's an orbital meningoencephalocele. And the treatment for that really is to put in a firm barrier to resect the orbital meningoencephalocele and then a firm barrier to separate those contents. But this was a perfect case for us to do together, because we're working both with the orbital contents as well as with the brain that are going back and forth. And this is kind of a perfect example of where Dr. Cohen and I overlap. So at that point, I sent her to see Dr. Cohen. She agreed and we scheduled the case together.

Dr Liza Cohen: You described the case beautifully, Dr. Magill. In these types of situations where there's pathology that involves both the brain and the orbit, being at a center like Northwestern Medicine, where we have specialists who are used to doing these types of cases together and working together, really allowed for the patient to be provided the best multidisciplinary care possible. And we can talk a little bit about the surgery and how we kind of approached it. Dr. Magill, if you want to start with that.

Dr Stephen Magill: I think being at a place where we had the really excellent diagnostic, both the ophthalmologist and the clinicians who first detected this and then the radiologists, the neuroradiologist seeing the imaging correlate, and then us working together, and what Dr. Cohen and I do as we looked at the case and thought about it -- You know, my specialty is in minimally-invasive brain surgery and skull base surgery, and so I'm always looking to try to see is there a minimally invasive option. Could we go through the orbit? And so we had several conversations back and forth looking at the imaging. And because of the size of the defect and that it was on the medial side of the orbit, which can be difficult to get at, and it was very deep in the orbit, so an approach where you go through the orbit is not as good because you have to get back where it gets very narrow by the optic nerve and to put in a big implant to close it, there just wouldn't be enough room. So we needed to come from the brain side of the orbit to look there.

So we ended up doing a more classic approach with a subfrontal craniotomy. And we did that because we wanted to have enough working room to actively fix the problem. And I think this really highlights one of the tenets of minimally-invasive surgery and multidisciplinary surgery, where you have a minimally-invasive approach, but you're not married to it, because each patient's pathology is different. And so it's important at a center of excellence to have the ability to do all the approaches, the classic open approach, like we chose here because it would actually give us better access to repair this entire defect. But we discussed it, went through everything, thought about minimally invasive ways. But given the location deeper in the orbit and on the medial side, we went with a subfrontal craniotomy. I opened up, dissected the brain away. I found the brain defect where the brain tissue, the orbital meningoencephalocele was herniating out, and I resected that. And then, I put in a vascularized reconstruction, which would prevent any spinal fluid leaking out, because obviously the brain floats in spinal fluid. So anytime there's an opening where that tissue had been herniating out, there's a risk of spinal fluid leak into the orbit. So we put a vascularized reconstruction in there. And then at that point, Dr. Cohen came in and took over the case. And I'll let you talk a little bit about your part, but her expertise with the orbit allowed her to design a reconstruction for the orbit. So I'll let you take over talking about your part of the case.

Dr Liza Cohen: Thank you, Dr. Magill. And one other point about this case in particular, and how we were each really able to use our areas of expertise was that this defect, as Dr. Magill mentioned, was quite large in the superomedial orbit, where there's a lot of critical structures in that area that can affect the vision and the function of the eye. And one of the muscles that moves the eye, the superior oblique muscle, was actually located right where the defect was. And so doing an approach coming in through the orbit, we would have had to work around that muscle. It would have been very difficult and that's another reason why we chose the open frontal craniotomy approach.

But when we were able to visualize the full defect and the connection between the orbit and the brain, the orbital tissue including the fat and the superior oblique muscle was herniating into the cranial cavity. And so we very carefully used instruments to basically push the orbital tissue back and reposition it in its normal anatomical location within the orbit. And then, we designed MEDPOR-coated titanium implant that we contoured to fit the normal shape of the orbital roof. And dr. Magill and I secured plate in place, making sure to cover all of the orbital tissue, not entrap the fat or the muscle as that could affect the movement of the eye and also make sure that the bone was completely covered so that the intracranial contents didn't herniate into the orbit.

Dr Stephen Magill: Yeah. Excellent. And, you know, one of the other interesting and good things about being part of a multidisciplinary team, when we do a lot of cranial reconstruction, we often use bare titanium, which has no problem when there's dura over the brain and some space underneath it. But one of the things I learned working with Dr. Cohen in this case, as we were deciding on what implants to use is that actually titanium can cause some inflammation within the orbit. And so by working together, we were able to select an implant that would really provide a strong structural barrier. But because of the coating that was on it, it would reduce inflammation, any risk of scarring to the superior oblique and those structures that were herniating out from the orbit. So I think having a multidisciplinary team, really from every aspect, helped us optimize the care for the patient.

One of the other things, I wish I could show the video of the podcast, but I'll just describe it to you. You know, this woman had just complained about these terrible pulsatile proptosis that you couldn't see. But once we got her under anesthesia and she was paralyzed, all of a sudden her right eye was bouncing in and out of her orbit, almost moving I would say seven or eight millimeters with each pulse once the muscles had been relaxed. And her eye and brain, the muscles in the orbit and everything, when she was awake, had enough tone to fight that. But when she was fully relaxed, you could really see what was causing her problems. And then once we had closed up and finished the reconstruction that Dr. Cohen just described, I took another video and you just take a video and the eye is just sitting there. And consistent with that, when she woke up, she had no pulsatile proptosis. It was completely gone. She has a little pain from the surgery, which we treated to take care of her getting her through it, but she did it really, really well.

Dr Liza Cohen: Yes, it really was a remarkable result that you could see immediately on the table at the end of the surgery, that we had solved this problem of her pulsatile proptosis. And we've both seen her back in followup since that time and she reports that that symptom is completely gone. Her headaches are also gone and she's able to function much more easily in terms of her activities of daily living and working, and is no longer bothered by this debilitating symptom.

Dr Stephen Magill: And one of the other things I would add too, working together with Dr. Cohen, you know, anytime you do orbital reconstruction, and because I deal with the orbit fairly regularly as a skull base surgeon, I always worry about entrapping any of the muscles. So at the end of the case, Dr. Cohen opened the eye and checked to make sure the eye could move fully in every direction and that our implant didn't do it. And I think about having a multidisciplinary team really lets us be thorough from implant selection, from surgical planning, from all of that, to what we do in the operating room. Instead of just one set of eyes looking at things, we have two sets of eyes at each stage in the case. And then even at the end of the case, making sure that there's no entrapped muscles while the patient is still asleep with a forced duction test. All these things work together for us to preserve vision and really prevent complications.

Melanie Cole (Host): Thank you both. And I'd like to give you each a chance for a final thought. This is absolutely fascinating, and I am quite sure that other providers are going to find it as interesting as I do the way that you're working together. And Dr. Cohen, starting with you, as you're telling us what's important to note when working to preserve vision and prevent complications, what would you like other providers, neurosurgeons, ophthalmologists to learn about this case? What would you like from your perspective to be the key takeaways?

Dr Liza Cohen: I think the key takeaways from this case are, first identifying, that there's a problem, so listening to the patient, obtaining the appropriate imaging. And once the problem has been identified that overlaps different specialties, in the case of this patient, an orbital surgeon and a neurosurgeon, proper referral to a center such as Northwestern Medicine that can provide this type of very specialized multidisciplinary care for patients like this.

And the other thing that's important to take away is that, as Dr. Magill mentioned, our fields in medicine are constantly evolving and there's always new ways that we can approach things and get creative and no two cases are exactly the same. So really using each of our areas of expertise, we're able to work together to determine the best course of management for a particular patient. And things are constantly changing. And there's lots of opportunity for growth in terms of how we can work together in the future as well.

Melanie Cole (Host): Really an exciting time in both of your fields. And this case is so interesting. Dr. Magill, for your final thought here, expand just a little bit more or reiterate for us this multidisciplinary approach and how it advances surgical practice and care for patients for better outcomes.

Dr Stephen Magill: As I think about this case, as I think about what having all of us involved really brought to the patient, I think Dr. Cohen really highlighted, the first was listening to the patient, taking them seriously, really astute diagnosis. But not just being able to diagnose a problem, but by having a multidisciplinary team, we could prove that there was a defect that would fit with her symptoms with our excellent astute neuroradiologists. And then as we work together and thinking about the case and as Dr. Cohen and I think about different cases together, it's really at the intersection of disciplines where we make progress. And so when I see a case and say, "Hey, take a look at these films. What do you think? How should we do this? What is the best approach?" And then by working together, I see what she can do and she can see what I can do, which gives us a totally different perspective on the problems that we deal with everyday.

At those intersections, that's where we can make progress. And I think in skull base surgery and in minimally-invasive surgery approaches going through the orbit, approaches going around the orbit, working in a minimally invasive fashion with a faster recovery for the patients, all of these things come out of these conversations, of working together, of seeing cases like this and building these relationships. And I think that's really why I love to work at a place like Northwestern, where we can constantly push to be better and be surrounded by really excellent colleagues on all fronts that help us continually grow and then also provide a venue and a sounding board for creativity, where we can consider what other options could we have for this. And I think those discussions and that dynamic interaction is actually where progress is made.

And I think in surgery, you can become very stale. "This is how I was taught to do it. This is how I will always do it for the next 25 years." And if you're stuck on your own and you're not working in a multidisciplinary setting, then you do what you know you can do that's safe. But when you're surrounded by really brilliant colleagues and creative thinkers, and you put these problems together and you share cases together and have this dynamic back and forth, that's where you can start to really make progress and think, "How can we do things differently?" And then you can have the boldness to actually go in and start doing that, because you know you're working with someone who can address those issues that are outside of your specialty. And so I think these areas where there's overlap between neurosurgery and skull base surgery and orbital surgery, oculoplastics are really exciting and it's a really fun place to work. And I think it makes a difference for the patients too. We really can provide the best outcomes for them.

Melanie Cole (Host): Well, you certainly can. And you're both very involved in advancing medicine. I thank you so much for joining us and sharing this case report on pulsatile proptosis due to the orbital cranial trauma. To refer your patient or for more information, please visit our website at breakthroughsforphysicians.nm.org.

That concludes this episode of Better Edge, a Northwestern Medicine podcast for physicians. I'm Melanie Cole.