Five Diagnoses Outside the Scope of Comprehensive Ophthalmology

Educational Objectives

The goal of this program is to improve the diagnosis and management of neuro-ophthalmologic diseases. After hearing and assimilating this program, the clinician will be better able to:

1. Determine whether referral to a neuro-ophthalmologist is appropriate for patients presenting with acute
ophthalmic symptoms.

2. Follow an algorithm for diagnosing the cause of
third nerve palsy.

3. Recognize clinical symptoms associated with
neuromyelitis optica.

Summary

Alternatives to neuro-ophthalmologic consultation: neuro-ophthalmologists scarce; for some diagnoses, comprehensive ophthalmologists should consult with specialists in neurology and general medicine rather than delay treatment; provide specialists with ophthalmic findings and presumptive diagnosis, then allow them to complete workup of patient; “neuro-ophthalmology is a team sport”

Five diagnoses requiring referral: (1) posterior ischemic optic neuropathy (PION) — anterior ischemic optic neuropathy (AION) is common cause of acute unilateral optic neuropathy in adults; has 2 forms (nonarteritic, 90% of cases; arteritic [giant cell arteritis], 10%); diagnosis requires disc swelling; PION rarely nonarteritic; in most cases, diagnosis incorrect or giant cell; if PION suspected, refer to team; (2) third cranial nerve palsy — requires referral for treatment of aneurysm; (3) chronic optic neuritis (ON) — if ON not improving, seek help from team; (4) Tolosa-Hunt syndrome — requires referral; (5) retinal migraine — diagnosis never made on first visit; no test or laboratory study can confirm diagnosis

Issues complicating diagnosis of above conditions: no tests or antibody markers establish diagnosis; misdiagnosis can result in death or blindness of patient; findings often absent on imaging studies (comprehensive ophthalmologist may therefore attempt diagnosis without aid of team); “retrospective diagnoses” (can be confirmed only after following differential diagnosis and observing imaging findings that appear after resolution)

Atypical features: if found in, eg, corneal ulcer, retinal condition, refer to appropriate subspecialist

Case example 1: 20-yr-old woman presents with acute unilateral painful loss of vision, relative afferent pupillary defect (RAPD), and normal fundus; diagnosis, retrobulbar ON; however, same presentation in 80-yr-old requires referral

Three forms of PION: arteritic, postsurgical (following spine or cardiac surgery), and nonarteritic; nonarteritic ischemic optic neuropathy is anterior

Management of case example 1: referral diagnosis of retrobulbar ON suggests to team that patient has multiple sclerosis (MS); however, since patient 80 yr of age, likely diagnosis giant cell arteritis; to avoid leading team astray, provide details of observed pathology (rather than presumed diagnosis) and differential diagnosis for these observations; otherwise, unnecessary and uninformative tests (eg, lumbar puncture, magnetic resonance imaging [MRI]) may be performed; retrobulbar optic neuropathy (rather than retrobulbar ON) is correct referral diagnosis; perform fluorescein angiography to observe blood flow and rule out choroidal perfusion defect; if no clear etiology for PION identified, order MRI because other retrobulbar optic neuropathies (eg, Aspergillosis, Mucormycosis, pituitary apoplexy, ophthalmic artery aneurysm) could present similarly to PION; if all alternatives ruled out, PION diagnosis can be made retrospectively (nerve turns pale on imaging after 6 wk)

Management of third nerve palsy: fundus examination unnecessary if patient presents with chief complaint of ptosis or diplopia; main concern is aneurysm; neuro-imaging in such cases “gives false sense of security”; if patient reports “worst headache of life,” begin with noncontrast computed tomography (CT); if subarachnoid hemorrhage diagnosed, order contrast CT angiography (CTA); if CTA negative, perform MRI and MR angiography (MRA) with and without contrast; MRI superior for diagnosing every cause of third nerve palsy other than aneurysm; if MRA or CTA is equivocal, do not wait for availability of neuro-ophthalmologist (activate “angiogram team”)

Case example 2: 25-yr-old woman presents with loss of vision, RAPD, and central scotoma, suggesting retrobulbar ON; however, patient failed to improve over time; in Optic Neuritis Treatment Trial, all patients (in steroid as well as placebo groups) showed improvement in visual acuity and visual field; if patient with ON does not improve, consider Leber hereditary optic neuropathy (especially in men) or neuromyelitis optica (NMO); vision poorer than 22/100 after recovery seen in only 3%; for NMO, look for enhancement of nerve; for patients with ON, normal MRI brain, failure to improve, and subsequent ON in other eye, image spine; look for longitudinally extensive spine lesions (not present in all patients with NMO); test for NMO-IgG antibody, and if positive, refer to neurology team

Normal MRI in patient with optic neuritis: question patient about symptoms of NMO (ie, spine issues, intractable hiccups, symptomatic narcolepsy, or unexplained nausea and vomiting); correct diagnosis vital because if case incorrectly diagnosed as MS, disease treated with immunomodulatory therapy, which worsens NMO; for NMO, immunosuppressive therapy necessary (ie, chemotherapy such as rituximab or azathioprine, intravenous immunoglobulin [IVIG], or plasma exchange); data from Mayo Clinic show that, by month 30, 80% of people diagnosed with NMO become paralyzed or develop another optic neuropathy that causes blindness; correct diagnosis and treatment can prevent these devastating outcomes; testing for NMO antibody indicated for patients with unimproved, bilateral, sequential, or recurrent ON, >50 white cells per mm3 in cerebrospinal fluid, or transverse myelitis

Tolosa-Hunt syndrome: presents with painful ophthalmoplegia; imaging negative; pain responds almost immediately to steroids (however, this applies to many conditions); CT insufficient; MRI and team approach necessary; La Mantia et al — after reviewing all cases of Tolosa-Hunt syndrome in literature, found only 21% of cases correctly diagnosed

Case example 3: 76-yr-old woman with acute myelogenous leukemia had red eye and erythema on day 13 of induction chemotherapy; puffy eyelid and chemosis noted; CT read as nonspecific fat stranding and nonspecific sinusitis; patient failed to improve after taking antibiotics and steroid prescribed by resident; otorhinolaryngologist found pale necrotic tissue in nose; biopsy showed Mucormycosis; patient did not show signs of fungal infection because immune system compromised by chemotherapy

Case example 4: 60-yr-old woman with acute onset of loss of vision in right eye for 10 min; eye examination normal; patient never previously had migraine; diagnosed with ocular migraine

Retinal migraine: extremely rare; Hill et al — reviewed every case in literature labeled retinal migraine; only 5 cases met criteria defined by International Headache Society for migraine; rare diagnosis; cannot be diagnosed on first incidence (must have multiple attacks); test visual fields to determine whether visual loss has occurred

Case example 4 (continued): patient’s loss of vision consistent with amaurosis fugax; patient had hemispheric transient ischemic attack on following day and had stroke that night; misdiagnosis as retinal migraine resulted in $2 million malpractice judgment

Readings

La Mantia L et al: Tolosa-Hunt syndrome: critical literature review based on IHS 2004 criteria. Cephalalgia. 2006;26(7):772-81; Tolosa E: Periarteritic lesions of the carotid siphon with the clinical features of a carotid infraclinoid aneurysm. J Neurol Neurosurg Psychiatry. 1954;17:300–2.

Disclosures

For this program, members of the faculty and planning committee reported nothing to disclose.

Acknowledgements

Dr. Lee spoke at Ophthalmology in View: Vision, Innovation, Enterprising and Worldwide Impact, the VSEPS Education Fund 35th Annual Scientific Meeting, presented by the Virginia Society of Eye Physicians and Surgeons, and held June 8-9, 2018 in Virginia Beach, VA. For information about upcoming CME conferences from this sponsor, please visit: www.vaeyemd.org. The Audio Digest Foundation thanks Dr. Lee and the Virginia Society of Eye Physicians and Surgeons for their cooperation in the production of this program.

CME/CE INFO

Accreditation:

The Audio- Digest Foundation is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians.

The Audio- Digest Foundation designates this enduring material for a maximum of 0 AMA PRA Category 1 Credits™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Audio Digest Foundation is accredited as a provider of continuing nursing education by the American Nurses Credentialing Center's (ANCC's) Commission on Accreditation. Audio Digest Foundation designates this activity for 0 CE contact hours.

Lecture ID:

OP562102

Expiration:

This CME course qualifies for AMA PRA Category 1 Credits™ for 3 years from the date of publication.

Instructions:

To earn CME/CE credit for this course, you must complete all the following components in the order recommended: (1) Review introductory course content, including Educational Objectives and Faculty/Planner Disclosures; (2) Listen to the audio program and review accompanying learning materials; (3) Complete posttest (only after completing Step 2) and earn a passing score of at least 80%. Taking the course Pretest and completing the Evaluation Survey are strongly recommended (but not mandatory) components of completing this CME/CE course.

Estimated time to complete this CME/CE course:

Approximately 2x the length of the recorded lecture to account for time spent studying accompanying learning materials and completing tests.