How to manage neuro-ophthalmic emergency cases

Recognizing the signs and symptoms and preparing a plan are key to effective treatment.

By Mark L. Moster, MD

When a patient presents with visual loss, diplopia, anisocoria, headache or ocular pain, you must decide whether it is an emergency requiring immediate evaluation and treatment, or if the condition is routine.

If you do not have a plan, attending to a possible neuro-ophthalmic emergency can disrupt the patient-schedule flow. These incidents require phone calls, referrals for consultation and detailed conversations with the patient.

One indicator of a neuro-ophthalmologic emergency is an acute presentation. The challenge lies in determining whether it threatens the patient’s vision, general health or even life.

Here is a list of ophthalmologic presentations most threatening to vision or overall health.

1. Papilledema may present in a patient with or without symptoms of elevated intracranial pressure (e.g., headache, transient visual obscurations). Even a patient with typical features of idiopathic intracranial hypertension needs urgent neuroimaging with at least a CT scan and, preferably, MRI and magnetic resonance venography (MRV) to look for a mass lesion or venous sinus thrombosis. If neuroimaging is negative, set up a lumbar puncture soon to rule out a meningeal inflammatory process and document the level of intracranial pressure.

2. Transient monocular visual loss lasting for minutes in the recent few days, without classic features of migraine, should be considered an emergency. It likely represents a retinal transient ischemic attack. If the patient is older than age 55, giant cell arteritis is a strong possibility that requires immediate erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and platelet count. Patients with retinal transient ischemic attack (TIA) are at risk for early stroke and are best evaluated immediately by a specialist. This may best be done at a stroke center or an observation unit associated with an emergency room.

Patients should have diffusion weighted MRI, noninvasive evaluation of the carotid arteries and early intervention to prevent stroke. Patients with central retinal artery occlusion should have the same emergency work-up if they present within a few days of onset of symptoms.

3. Horner’s syndrome, particularly if the onset is acute in nature, and especially if the patient complains of discomfort around the face or head, must be considered a sign of internal carotid artery dissection. Ask about recent trauma or severe coughing or sneezing, transient vision loss, neck or face pain. This requires urgent imaging of the carotid artery with MRI/MRA or CT/CTA with emergency evaluation by a physician with stroke expertise if the imaging shows a dissection.

4. Third-nerve palsy isolated in nature may be a sign of an enlarging aneurysm at the junction of the posterior communicating artery and the internal carotid artery. Unless the patient experiences a complete sparing of the pupil along with complete involvement of the extraocular muscles, she or he will require emergency room evaluation with CT/CTA or MRI/MRA. Isolated mydriasis without ptosis or ophthalmoplegia is not a similar emergency.

5. Severe headache with either sudden visual loss or involvement of cranial nerve 3, 4 or 6 may be signs of pituitary apoplexy. Emergency MRI with close attention to the suprasellar region is mandatory as is neurosurgical admission if the scan is positive for pituitary apoplexy.

6. Acute diplopia or nystagmus that does not fit a pattern of an isolated cranial neuropathy may be the result of a brainstem infarction. This requires immediate evaluation by a stroke expert.

7. Acute orbital apex or cavernous sinus syndrome may be a signs of a vision- or life-threatening condition. Patients present with multiple cranial nerve involvement and diplopia with or without visual loss. In a patient with diabetes or renal failure, consider the possibility of mucormycosis. Emergent evaluation with neuroimaging and immediate biopsy and treatment can be vision- and life-saving.

8. Giant cell arteritis must be considered in anyone age 55 or older with visual loss (particularly ischemic optic neuropathy), diplopia or headache. Ask about systemic symptoms such as polymyalgia rheumatica, jaw claudication, headache, fever, malaise, decreased appetite and weight loss. Obtain emergent ESR/CRP/platelets. If suspicion is high, start high-dose oral steroids or admit the patient for IV steroids immediately and arrange for a biopsy of the temporal artery.

9. Traumatic optic neuropathy requires urgent imaging to rule out an optical canal fracture that may be an indication for surgery. Although steroids have been used in the past, they have not been proven to help and are contraindicated in the presence of brain injury because they may worsen neurologic outcome.

CONCLUSION

Simply waiting for an emergency to arrive and dealing with it chaotically is poor preparation. Establish measures in advance.

Identify key players in your area on whom you may need to rely. Depending on where you practice, these may include neuro-ophthalmologists, emergency-room doctors, neurosurgeons, stroke specialists, neurologists and ENT specialists.

Identify the best emergency room for your patients and, when you send them, be sure to include details of what you are concerned about so they get the appropriate evaluation and treatment. Setting this process up in advance will alleviate a lot of grief and anxiety when a patient with an emergency does arrive. OM

About the Author
	Mark L. Moster, MD is a Professor of Neurology and Ophthalmology at Wills Eye Hospital and Thomas Jefferson University School of Medicine in Philadelphia. Disclosure: Dr. Moster has no conflicts to disclose.