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Unilateral optic disc edema in a young male
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A 32-year-old African-American male presented to the urgent care department of the clinic with a physician-directed referral letter in-hand stating a diagnosis of “papilledema in the left eye.” The patient described symptoms of constant peripheral blurry vision in the left eye only for the last 2 weeks. He described his central vision to be clear. In addition, he disclosed that colors appeared less bright in the affected eye.
Upon questioning, the patient admitted to a sharp pain in both eyes in right gaze only. He also reported an intermittent headache, occurring about three times per day, lasting about 5 minutes on each occasion, with no associating pattern for the last week. He denied double vision.
While the patient reported his most recent appointment with a primary care provider was 10 years prior, he indicated a medical history pertinent for an oromaxillary fistula, diagnosed and repaired approximately 1 year prior. He denied using medications or having any allergies. His social history was remarkable for occasional alcohol consumption and former cigarette smoking. In-office blood pressure was 112/78 mm Hg.
Entering uncorrected visual acuity was 20/20-1 OD and 20/20-1 OS, with a slower response in the left eye. Pupils were equal in size, round and reactive to light with a 1+ afferent pupillary defect in the left eye. Motility in each eye was full, and he reported a “discomfort” of both eyes in right gaze only. He denied any significant pain on eye movements.
Images: Theisen K
While confrontation visual fields were full to finger counting in the right eye, 24-2 Humphrey visual field testing (Carl Zeiss Meditec) demonstrated a superior altitudinal visual field defect in the left eye only. A red cap comparison test revealed 20% to 40% relative dyschromatopsic desaturation on the left side. Objectively, the patient was able to correctly identify all screening plates in each eye via Hardy Rand Rittler (HRR) testing. He had no apparent proptosis or globe displacement.
Anterior segment evaluation was unremarkable with the exception of small, eccentric, linear anterior stromal scars in both eyes, which the patient attributed to having “broken glass in each eye” years prior. Goldmann applanation tonometry was 14 mm Hg OD and OS. Dilated fundus examination for the right eye was unremarkable with no crowding of the optic disc. Significant findings for the left eye included optic disc edema and hyperemia without accompanying hemorrhaging.
The top two leading differential diagnoses in this case of unilateral optic disc edema without accompanying disc hemorrhaging were nonarteritic anterior ischemic optic neuropathy (NAION) and optic neuritis.
Nonarteritic anterior ischemic optic neuropathy
NAION represents the most common acute optic neuropathy in the elderly, typically affecting individuals older than 50 years, although no age is immune from the condition. Symptoms of NAION include sudden onset painless vision loss, frequently upon awakening. Visual acuity may be reduced, and an arcuate or altitudinal visual field defect, particularly inferior, is common. The level of color vision loss tends to be proportionate to the level of decrease in visual acuity.
In an acute presentation, an ischemic event involving the anterior portion of the optic nerve head leads to unilateral optic disc edema. Disc hemorrhaging typically accompanies the edema. On average, the optic disc edema resolves within 4 to 8 weeks with subsequent retinal nerve fiber layer (RNFL) loss and diffuse or segmental optic disc pallor.
Well known risk factors for NAION include hypertension, diabetes and a disc at risk, among others. Some suggest NAION in young individuals may be the initial manifestation of elevated cholesterol levels. There is no well accepted treatment for acute NAION.
Optic neuritis
Optic neuritis, on the other hand, is the most common acute optic neuropathy in individuals younger than 50 years. Demographic features show a predilection for young Caucasian females. Typical symptoms of optic neuritis include sudden onset unilateral vision loss, pain upon eye movements and dyschromatopsia, which tends to be disproportionate to the level of decrease in visual acuity. Optic neuritis may be inflammatory, infectious, infiltrative (secondary to conditions such as multiple sclerosis, syphilis and sarcoidosis, respectively) or idiopathic.
Approximately two-thirds of all optic neuritis cases are posterior optic neuritis, in which the optic disc appears normal. Of the remaining one-third of cases that are anterior optic neuritis, a very small percentage has accompanying disc hemorrhaging. In the case of anterior optic neuritis, the optic disc edema and subsequent loss of the RNFL begins around 1 month and continues for 3 to 6 months. Treatment options for acute optic neuritis are variable and case dependent.
This patient’s management
The recommended management for this case was to obtain an MRI of the brain and orbits with and without contrast, chest X-ray and laboratory testing to rule out diabetes, hyperlipidemia, sarcoidosis and syphilis. He was to follow up in 1 week to monitor for stability of the condition and to discuss case comanagement following the additional recommended testing.
One day after the initial examination, he called the urgent care on-call resident, stating his vision appeared to be worsening. All testing, including optical coherence tomography and Humphrey visual field, was repeated and stable.
His newly assigned medical insurance posed significant time delays in additional testing. Meanwhile, he was evaluated for stability and resolution of the disc edema. He presented for a 1-week follow-up and monthly follow-up examinations thereafter, until demonstration of RNFL thinning. Interestingly, while his acuity remained approximately stable, the RNFL continued to increase above baseline for at least 10 weeks. It was not until the 4-month follow-up that the RNFL showed significant thinning from baseline.
Eventually all additional requested testing was completed. Laboratory testing for syphilis, diabetes and hyperlipidemia was unremarkable, and hypertension was ruled out. MRI testing was unremarkable, including the absence of any enhancement of the optic nerve, demyelinating plaques or small vessel disease. For these reasons, a diagnosis of atypical NAION was concluded. A coincidental granuloma of the right lung without bilateral hilar lymphadenopathy was identified with chest CT, while serum ACE levels were within normal limits.
Yet another interesting facet of this case is that, without treatment, the visual field defect spontaneously improved to near normal levels 5 months after the initial event.
Although NAION typically presents in patients older than 50 years, younger individuals are not immune to the condition. This case represents atypical NAION in a young adult male without optic disc hemorrhaging, optic disc edema persisting beyond the typical 2 months and significant visual field improvement. In the atypical acute setting of NAION in young patients, other organic causes for unilateral optic disc edema, most notably optic neuritis, must be ruled out.
- References:
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- Hayreh SS, et al. Graefes Arch Clin Exp Ophthalmol. 2007;245(8):1107-1121.
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- For more information:
- Kelli Theisen, OD, recently began a career in academics as a clinical instructor at Illinois College of Optometry. She can be reached at ktheisen@ico.edu.
- Leo P. Semes, OD, is a professor of optometry, University of Alabama at Birmingham, and a member of the Primary Care Optometry News Editorial Board. He may be reached at: lsemes@uab.edu.
Disclosure: Theisen reports no relevant financial disclosures.