OTHER SERVICES & SPECIALTIES, NEUROLOGY & NEUROSURGERY

The Ophthalmologist and Systemic Disease: Sometimes It's More Than Meets the Eye

Nov. 3, 2021 - Eden McCleskey

Eyes may or may not be the window to the soul, but ophthalmologists know they can be an important reflection of a person's overall health. Sometimes they're the first harbinger of a crisis in the body, an early warning before more widespread, permanent or catastrophic damage can occur.

During the time-sensitive period when early recognition and intervention can make a lifesaving or life-altering difference, it often falls to an ophthalmologist, not an emergency department, to identify, triage and refer the patient for immediate diagnosis and treatment.

Recognizing this crucial and emerging responsibility and illuminating potential knowledge gaps will be a major focal point at the American Academy of Ophthalmology 2021 Conference Nov. 12-15. An entire day will be dedicated to understanding more about systemic disease manifestations of the eye.

Dr. Andrew Lee, chair of the Department of Ophthalmology at the Houston Methodist Blanton Eye Institute, has been invited to participate in a case-based learning module and panel discussion about two examples: acute retinal ischemia and neuromyelitis optica. He also will present the Hoyt Award to Dr. Valerie Biousse following her keynote address on acute retinal ischemia.

Dr. Lee sat down with Leading Medicine to discuss what ophthalmologists need to know and why this is the right time and place to spread the word.

Q: What are the symptoms and risks associated with acute retinal ischemia?

Acute retinal ischemia, or ARI, is a stroke in the eye. A transient ischemia attack (TIA) is similar to a stroke, but the symptoms do not last as long. TIA typically presents as sudden, painless, visual acuity loss and/or visual field loss in the affected eye, usually lasting only minutes but sometimes lasting an hour or more. As opposed to a stroke in the eye, the vision returns to normal after a TIA.

One of the big messages that we're trying to get out nationally is that an eye stroke is a stroke and that an eye TIA has the same significance as a brain TIA. Like a brain stroke, acute retinal ischemia is a stroke in progress. If you believe your patient has had one, they need to be seen as urgently as possible by a hospital's specialized stroke center.

The ophthalmologist is really in the best position to assess if the vision problems that the patient experienced is a sign of ARI/TIA or has some other explanation. Every patient who experiences vision issues can't and shouldn't go to the ER. This is why the current stroke guidelines for ophthalmology recommend that eye doctors should make time in our schedules for same-day appointments for transient or acute visual loss and for whenever a patient has a sudden onset vision problem that could be a sign of something more serious, like a stroke.

A TIA is a warning sign that a brain or eye stroke may be coming. Approximately a third of patients who have acute retinal ischemia (e.g. a branch or central retinal artery occlusion) will experience a brain stroke within the first 24 to 48 hours. Really, it's a fairly short window to intervene, and if you don't recognize it and triage appropriately, then you're missing the opportunity to find the cause, treat it and prevent a potentially lethal or devastating stroke from occurring.

Q: What is neuromyelitis optica?

Neuromyelitis optica (NMO), also previously referred to as Devic's disease, is a rare condition where the antibodies in the immune system damage the spinal cord and the optic nerves. It can lead to permanent blindness and paralysis (transverse myelitis) if not recognized and treated appropriately.

NMO can present three different ways. Some patients will get vision symptoms first without any myelitis symptoms. Some patients will get myelitis symptoms first without any vision symptoms. Some patients will get both myelitis and vision symptoms at the same time. Myelitis symptoms include weakness, pain or numbness/tingling of the face, arms and legs. Vision symptoms are typical of optic neuritis, an inflamed optic nerve. They come on quickly, within a matter of days, and range from painless loss of visual acuity and dimming of colors to eye pain and vision loss.

It's patients who experience vision symptoms without any myelitis symptoms that ophthalmologists need to be on the lookout for. There are a number of reasons why patients might experience optic neuritis symptoms, from benign to severe. It's the ophthalmologist's job to assess when optic neuritis might actually be NMO and refer to a hospital neurologist for immediate evaluation.

There is a diagnostic blood test for NMO (the aquaporin 4 channel antibody), and there are treatments that can prevent progression of blindness and paralysis.

Q: Are these the main systemic diseases ophthalmologists need to be aware of?

There are many systemic diseases that can present with symptoms such as double vision, droopy eyelid, loss of peripheral vision, bulging eye or difference in pupil size. When these neuro-ophthalmic presentations are acute and painful, it is imperative to recognize the severity of the problem and the possibility of a dangerous underlying etiology.

Acute retinal ischemia and neuromyelitis optica are dangerous because patients might present with ocular symptoms or signs alone, and the vision loss may be mild or transient. In addition, the ophthalmologist may be the first or only point of contact for these patients, and thus the first to intervene early and make a big difference in potential outcome. These cases are particularly high-risk and high-stakes encounters for the eye doctor, and this is one reason we're highlighting both conditions at this conference.

Another thing is, the latest guidelines and research for both these conditions aren't regularly published in ophthalmology journals; they're in stroke, cardiovascular or neurology publications. That makes it hard to know what the recommendations are as a practicing ophthalmologist. In the past, ophthalmologists would actually work these conditions up themselves, but now the complexity of the disorders, the order and timing of evaluation are so critical that we recommend consideration for admission to the hospital for further evaluation.

We're using this in-depth, interactive learning opportunity at the AAO annual meeting this year to emphasize to our target audience that both acute retinal ischemia and acute optic neuritis might be harbingers of stroke or inflammatory disease respectively.