Transcription:
Complex Ophthalmology Cases: A Discussion With Neuro-Ophthalmology and Oculoplastics

Melanie Cole, (Host): Welcome to Better Edge, a Northwestern Medicine podcast for physicians. I'm Melanie Cole. And we have a complex case panel discussion for you today with Dr. Nicholas J. Volpe, he's the Chairman and Professor of Ophthalmology at Northwestern Medicine; and Dr. Liza Cohen, she's an Assistant Professor of Ophthalmology at Northwestern Medicine.

Welcome, Dr. Volpe and Dr. Cohen. I'd like to start this podcast today by defining your expertise in this field for other providers and the types of patients that you see. Dr. Volpe, would you please start for us?

Nicholas J. Volpe, MD: Sure. I am a neuro-ophthalmologist, and one can be a neuro-ophthalmologist either through ophthalmology or through neurology. I happen to be an ophthalmologist who did additional training in the specialty of neuro-ophthalmology, which deals with just like the intro to the word ophtho, neuro, the neurologic problems that affect the eye and, in particular, eye movement disorders, misalignment of the eyes, eye muscle disorders, optic nerve disorders and brain disorders that affect the way the eye either functions or moves. In addition to the nerves and brain and how they affect the eye and its function, the eye of course sits in the orbit and is surrounded by several important structures in the orbit that are involved in its function, including eye muscles and other tissues. And that's where, as you'll hear from Dr. Cohen, we have significant overlap in the types of patients that we care for.

Liza Cohen, MD: Yes. By the way, thank you so much for having us on this podcast. This is really exciting for us to participate in. So, I'm Dr. Liza Cohen. I'm an orbital and ophthalmic plastic surgeon. My practice comprises of really three main areas, orbital disease as Dr. Volpe alluded to, which includes everything from tumors, inflammation, trauma involving the tissues that surround the eye in the orbit; eyelid and periocular disease, including everything from droopy eyelids to skin cancers of the eyelid; other eyelid malpositions, both functional and cosmetic issues. And then, the third category of patients I see are patients with tear drain and lacrimal system problems, such as tear drain blockages. And I treat these problems both medically as well as surgically.

So, I'd like to start off the discussion by asking Dr. Volpe to talk about an interesting case that both of us co-managed together. Dr. Volpe, if you wouldn't mind telling us how the patient presented to you.

Nicholas J. Volpe, MD: Sure. And perhaps this patient is the best example of the single symptom that most often overlaps our expertise, and that is a presentation with binocular double vision. So, patients with binocular double vision develop the symptom because their eyes aren't working together. And with eyes that don't move together and have different angles to their position, the patient experiences double vision.

This particular patient is in her 50s with a history of breast cancer and presented to her oncologist with double vision. The oncologist recognized that one of her eyes wasn't moving normally and sent her along to me for an opinion about what might be causing the problem. In fact, when we evaluated her, as would be anticipated, we were able to identify an abnormality in the way the patient's eye moved. And in particular, she was unable to move her left eye to the left. And when an eye doesn't move to the left, you can imagine there are any number of different things that can cause that, particularly a nerve or a muscle problem.

I also recognize that her eye was slightly bulging or had proptosis. And with this combination of an impaired eye movement, an unfortunate history of cancer, and some bulging of her eye, I thought it would be very important to obtain an MRI scan of her orbit to see if there was any structural or space-occupying lesion that could potentially cause her to have this double vision and impaired eye movement. And sure enough, when we did this study, we identified a very lumpy or swollen lateral rectus muscle. The lateral rectus muscle, of course, is the one that moves the eye out or abducts the eye. And once this abnormality was identified, there was a strong suspicion with a very limited differential diagnosis for swollen muscles of a potential spread of cancer, and that's when I turned the case over to Dr. Cohen.

Liza Cohen, MD: Yes. So when this patient came to see me, she had already had an MRI scan of the orbits that Dr. Volpe had ordered. And in talking to the patient about her history, examining her myself, looking at the MRI, you know, the lateral rectus muscle was significantly enlarged. And there's very few things that can cause single muscle enlargement as Dr. Volpe alluded to. And so, given this patient's history of breast cancer with the possibility of spread to that muscle, we decided to perform a biopsy as this could potentially change the patient's treatment course. And she could receive targeted treatment to that lesion.

So, we performed a left orbitotomy. Since it was the lateral rectus muscle that was involved, we made a small incision hidden in the natural fold in the upper eyelid, and then basically dissected down to the orbital rim, performed a subperiosteal dissection into the orbit and then, incised the periosteum, identified the lateral rectus muscle, which was visibly enlarged and irregular and kind of grayish in appearance and took an incisional biopsy of the mass within the muscle. And then, we sent that to pathology. And the pathology did come back as demonstrating metastatic breast cancer, unfortunately.

So, Dr. Volpe and I consulted with the patient's oncologist. We got her in to see a radiation oncologist and she received radiation therapy to the left orbit in order to shrink the mass and help with her double vision. And it actually worked pretty well. She did have some residual double vision, so I asked her to see Dr. Volpe again to see if there was anything that could help relieve that. But she did experience a lot of improvement, even just with the radiation.

Nicholas J. Volpe, MD: So just coming back to this symptom, which is probably the most common one that overlaps our expertise, that is double vision, it obviously develops because of impaired eye movements. And, in this case, because of a muscle that actually wasn't working or it was not performing its function, so her eye was turned in or she had an esotropia. And there be any number of different ways that we could help her manage the symptom while she is being treated for a systemic disease. We'd anticipate probably at least a six-month period necessary for the muscle, if you will, to heal as much as it's going to. And then, we would have options to temporize either by using prism glasses or of course the patient could just wear a patch to block the double vision by covering one of her eyes.

In this case, a prism glass would expand the field of single vision, so she'd be able to see some single, but not when she looked in extreme directions. Ultimately depending on how she was doing systemically with her cancer, she would be an excellent candidate for my own area of surgical expertise, which is the management of double vision in adults through eye muscle or strabismus surgery.

And in this case, depending on another very important sign that Dr. Cohen and I both depend on, something called forced ductions to determine whether the eye is sort of stuck and can't move because of physical phenomena, or is it free and not moving because of weakness of a nerve or muscle, we could then do a combination of either strengthening or weakening, either that muscle or the muscles that it's paired with or opposes in the left eye or even working on the other eye to restore a more coincident or simultaneous movement of the eyes and relieve the double vision.

So, there's great options for these complex patients with orbital tumors. Specifically, that would be the overarching theme here of the kind of patients that would present either to a neuro-ophthalmologist or to an orbital specialist often with double vision or bulging of their eye. And then generally, these lesions are easily identified with imaging, but almost always have to be biopsied to secure a diagnosis because you can imagine that we'd not easily choose to radiate someone's eye socket without a tissue diagnosis as it was in this particular case.

So, that's I think a good segue into perhaps speaking a bit about the most common disease that overlaps Dr. Cohen's and my expertise, and specifically that is thyroid eye disease. So, there are a host of patients well known to both internists and ophthalmologists that develop thyroid eye disease classically in the setting of hyperthyroidism, but occasionally without any known thyroid disease that present with a very typical group of symptoms that might first come to Dr. Cohen's attention which I'll let her talk about in just a second, but may also come to my attention because the process primarily causes inflammation of the muscles and fat in the eye socket. That, as you can imagine, can impair eye movements and once again lead to double vision and, in rare cases, can cause the muscles to swell so much that they compress the optic nerve and cause vision loss that would present to a neuro-ophthalmologist. So, you can see here a disease that primarily affects the eye socket and its contents can have protean manifestations that would present to either of us; to me, with either double vision and or vision loss from optic neuropathy and to Dr. Cohen with the more classic orbital signs. And I'll ask her to comment about those at this point.

Liza Cohen, MD: Yes. So, I see many patients with thyroid eye disease, and the thing about this condition is that it can present in a variety of different ways. As Dr. Volpe mentioned, this is an autoimmune condition that affects the fat and the muscles within the orbit, and that surround the eye socket. And because the disease causes inflammation and fibrosis of these tissues, the most common presenting signs tend to be inflammatory signs, so edema, erythema of the eyelids and the ocular surface, eyelid retraction, or the eyelids being more open than they should be, difficulty moving the eyes and double vision, as Dr. Volpe alluded to, as well as bulging of the eyes or proptosis.

And so, patients can present with all of these symptoms with one or two of them. And that's really, where he and I work together. If I see a patient that comes to see me, they have some bulging of the eye, but they also have double vision, they might require multiple different forms of treatment in order to manage their symptoms. whether that be systemic medication, which can help manage their symptoms or whether it be surgery or just supportive medication. And then, there's also things that, you know, I'll let Dr. Volpe talk about that can be done to help alleviate double vision. But there's many different ways that we can treat these patients medically. There's medications such as targeted immunotherapies, which are on the horizon for our field with the newest one being teprotumumab, which has led to great success in terms of alleviating a lot of these patients' symptoms. There are other medications in the pipeline. There's always the non-specific anti-inflammatory medications such as systemic steroids and a few others. And then, there's always surgery that can be done in terms of relieving proptosis for which we can do an orbital decompression there's eyelid retraction surgery that can be done to normalize the position of the patient's eyes. And then, of course, aesthetic eyelid rejuvenation such as upper and lower blepharoplasty, which can be done for any puffiness or swelling that's bothersome to the patient. So Dr. Volpe, what sorts of things can you do for these patients with thyroid eye disease who come to see us?

Nicholas J. Volpe, MD: Sure, thank you. You know, this is a whole new era in the management of this very, very, challenging condition that can be both disfiguring and as well, very disruptive to patient's life because of both the discomfort and the bulging and the proptosis, and then the symptoms that develop, particularly irritation, tearing, eye discomfort that Dr. Cohen talked about and then, the more structural problems that can develop because muscles are diseased or either swollen so much that they're affecting the optic nerve.

And the introduction of teprotumumab, which is an insulin growth factor receptor blocker, which really has revolutionized the management of this disease. We think we're going to slow it down and see less of the complications that we've seen over the last few decades in these patients. But the most concerning ones that do develop, and we're not sure yet whether teprotumumab is going to change the frequency of this is the stiff eye muscles develop restriction, as I mentioned. If we were to try to move the eye, it doesn't move normally, the eyes move at different speeds to different extents. And again, patients develop double vision.

Once the double vision has been judged to be stable, we can sometimes, again, manage it with prism, which are glasses which are surprisingly effective, even though the deviation seems to be very different in different directions. We can give great relief from prism. But also again, the use of adjustable sutures, strabismus surgery, in these patients can really restore eye movements by taking restricted muscles off the eye, reattaching them in different places so that they act differently and restrict differently, and thereby reestablish alignment. The use of an adjustable suture allows us to change the outcome of the surgery the next day to get the muscles just in the right position. This is something that's added as part of the rehabilitation Dr. Cohen would do with they eyelids, sometimes with a decompression. There is a small subset of patients that Dr. Cohen alluded to that, in their acute phase, the muscles get so big that they threaten the optic nerve. The apex of the orbit becomes very crowded and compresses the optic nerve. This is a medical and sometimes surgical emergency, which requires either prompt treatment with steroids and more likely orbital decompression where the bones are literally broken away from the eye socket to make more space for the muscles and the contents of the orbit. And that's a procedure that Dr. Cohen does to manage these patients. But from a neuro-ophthalmic perspective, we're good at identifying the optic nerve problem and as well managing the double vision.

I thought we'd spend the last couple of minutes just talking about two quick things. The first is the variety of things that present in the setting of closed head injuries that overlap our expertise. So obviously patients with closed head injuries have varying degrees of orbital trauma in addition to brain trauma. And once again, because of either bleeding behind the orbit and I'll leave that one to Dr. Cohen in just a second, or fractures to the orbit or damage to the eye muscles and/or damage to the nerves that control the eye muscles because of skull base fractures, these patients can present with a complex series of eye movement abnormalities and vision loss that often require our combined expertise to first localize and identify the problem and then decide if there are acute issues to be managed as well as how they'll be managed down the road. So, maybe Dr. Cohen can speak briefly about orbital trauma here and then we'll wrap up with a few comments about managing papilledema.

Liza Cohen, MD: Yes. Thank you, Dr. Volpe. So, as you alluded to, both of us see lots of patients present with trauma to the eye socket as well as the brain. And sometimes these injuries can coexist, for example, in the case of motor vehicle accidents. Oftentimes these patients can have many complex injuries involving both the face and the eye socket as well as the brain. And it really takes an astute clinician to make sense of things such as the extraocular motility, the optic nerve exam, the ophthalmic exam to really identify what a patient's injuries are. It's easy to chalk up double vision and abnormal eye movements to an orbit fracture. But sometimes these patients, as you alluded to, can also present with cranial nerve palsies, which as a result of skull base fractures, which may not be quite as noticeable on neuro imaging.

So, oftentimes when we evaluate these patients, some of the issues that we want to be concerned about as you mentioned, Dr. Volpe include bleeding within the eye socket or what we call a retrobulbar hemorrhage, which can happen from a number of injuries, including blunt trauma to the eye socket or a fall. They tend to be particularly common in patients who are taking blood thinner medications. But this can be a really serious injury that, if not treated promptly, can actually lead to permanent vision loss because the orbit is a confined closed space within four bony walls, and there's only so much expansion that can occur. And so, you can imagine if there's bleeding within the orbit, pressure gets placed onto the arteries that supply the optic nerve. And if that pressure's not released within about 90 minutes, the nerve can become permanently infarcted and the patient can unfortunately lose their vision. So, identifying these patients who have a lot of swelling, bruising of the eye, decreased vision, other measures of decreased optic nerve function, such as a relative afferent pupillary defect on the pupil exam can aid one in determining whether this patient needs urgent intervention. And, you know, if that's the case, oftentimes we need to decompress the eye socket either through a laceration if the patient has a laceration that's going into the orbit or via a lateral canthotomy and lysis where we make an incision in the outer corner of the eye and release the eyelid from the periosteum at the orbital rim to release the pressure within the orbit.

As you mentioned, Dr. Volpe, other issues that can come into play with orbital trauma include fractures of the orbit, which I see a lot of. Like I said, there's four bony walls of the orbit, and any of those bones can be broken in a number of situations. And, you know, occasionally, an extraocular muscle can become trapped in the bony defect, and that's actually a surgical emergency. Because if we don't release that entrapped muscle, the muscle can necrose and become permanently dysfunctional. But oftentimes that occurs in young children, and most of the time orbit fractures are not entrapped and, if they need repair, can be repaired at a later time. But oftentimes, those patients present with difficulty moving the eyes, whether due to muscle entrapment or just swelling or bruising of the muscles, which sit quite close to the bones within the orbit. And so, it does help to obtain a good ophthalmic exam, really assess the patient's eye movements. And sometimes, these patients don't fully recover their extraocular motility and they do have issues with double vision. And so, that's oftentimes where our areas of expertise can both come into play for these patients as I can often repair the fracture and, if the patient does have residual double vision, a neuro-ophthalmologist can assess these patients, prescribe prism, do strabismus surgery, things of that nature.

Nicholas J. Volpe, MD: I just thought that maybe to conclude we would spend just a couple of minutes talking about idiopathic intracranial hypertension or pseudotumor cerebri. And this is a pretty straightforward discussion on one hand; on the other hand, very complex. So, this is a condition that would almost exclusively present to first an ophthalmologist, then an neuro-ophthalmologist in which often young women often in association with weight gain develop raise pressure in their head, or elevated intracranial pressure, which manifests as papilledema or swelling of the optic nerve.

Now, while 90% of these patients will do fine and can be treated aggressively with medications like Diamox or acetazolamide, there are a subset of patients that do overlap our expertise and those are patients that present with a fulminant version of this condition that is dominated by vision loss. The swollen optic nerve becomes ischemic and threatens the patient's long-term visual prognosis by potentially causing permanent damage to the optic nerve. And while there are a couple of ways to manage this, including medically with steroids and even neurosurgically with a shunt, in our experience, one of the best ways to manage this acute form of fulminant idiopathic intracranial hypertension with vision loss is to perform a procedure called an optic nerve sheath fenestration. And this is a procedure which I'll let Dr. Cohen explain in just a minute in which we relieve the pressure on the optic nerve by opening the optic nerve sheath or meninges and letting the spinal fluid out to reduce the pressure. Dr. Cohen?

Liza Cohen, MD: Yes. Thank you, Dr. Volpe. So, as you mentioned, optic nerve sheath fenestration is a procedure that can be performed, basically where we perform an orbitototomy. Most commonly, I do this through a medial upper eyelid crease incision approach, so a small incision in the natural eyelid fold. We dissect back into the orbit, find the optic nerve, and create either a couple slits in the optic nerve or, most commonly, I actually excise a window of the optic nerve sheath in order to better allow for the egress of cerebrospinal fluid through that hole in the nerve sheath to alleviate the pressure on the patient's optic nerve and hopefully restore their vision. And the procedure's often very, very successful in terms of improving the patient's vision. Of course, it is a bit risky as you're making a cut on the optic nerve. But I think, you know, in the right set of hands and with an expert in orbital surgery, we can really achieve good outcomes for these patients.

Nicholas J. Volpe, MD: Thanks Dr. Cohen, for joining me during this discussion. I think as we started with the first case with proptosis and double vision from metastatic breast cancer, it highlights the often encountered overlap between our specialties in which orbital disease presents with either double vision, vision loss or ptosis. And these patients are very complex and the initial reaction may just be, "Well, we'll just get a CAT scan and MRI scan and go from there." But I'd like to emphasize the importance of identifying these patients early. And in fact, these are patients that are best cared for in tertiary centers in which expertise is present in both the specialties of neuro-ophthalmology and orbit and oculoplastic surgery. Dr. Cohen and I, for instance, work in hallways next to each. And we're constantly back and forth looking at patients, looking at MRI scans and coming up with plans to manage these patients with complex orbital and neuro-ophthalmic disease. So, thanks for collaborating and we'll look forward to our continued care of patients together.

Liza Cohen, MD: Thank you.

Melanie Cole, (Host): I'd like to thank you both for such a comprehensive, informative thought leader discussion today. To refer your patient or for more information, please visit our website at breakthroughsforphysicians.nm.org/ophthalmology. That concludes this episode of Better Edge, a Northwestern Medicine podcast for physicians. I'm Melanie Cole. Thanks so much for joining us today.