Publication Exclusive: Man presents with slow, progressive vision loss

A 61-year-old man visiting from the Ivory Coast presented to the New England Eye Center for slow, progressive vision loss in both eyes starting 2 to 3 years before presentation. His medical history was significant only for hypertension and gout. His family ocular history included his father, who was blind in one eye possibly due to a retinal detachment. He was evaluated by his local ophthalmologist on several occasions, but an etiology was never determined. Before his evaluation at the New England Eye Center, our patient was seen at another eye clinic in the United States and was told he may have a maculopathy.

Examination

On exam, best corrected visual acuities were count fingers at 8 feet eccentrically in the right eye and 20/200 in the left eye. Pupils were round, equal and reactive without relative afferent pupillary defect. The patient was unable to identify the Ishihara control plate in either eye. Extraocular movements were full bilaterally. Anterior segment exam was remarkable only for 1+ nuclear sclerosis bilaterally. Dilated fundus exam was remarkable for a white linear scar extending from the inferior arcade to the temporal aspect of the macula in the right eye. The cup-to-disc ratio was 0.65 bilaterally, and there was moderate temporal pallor of both optic nerves.

What is your diagnosis?

Progressive vision loss

The differential for bilateral disc atrophy with progressive vision loss is fairly wide but can be categorized into compressive, mitochondrial, vascular, infectious vs. noninfectious optic neuritis, and infiltrative.

Given the presentation of bilateral progressive vision loss, a compressive lesion is high on the list. Pituitary lesions, such as an adenoma or a craniopharyngioma, meningioma or slow-growing aneurysm are most common. Dominant optic atrophy could also be considered in the setting of bilateral progressive vision loss with large cup-to-disc ratios and temporally pale optic discs; however, there was no family history of unexplained bilateral vision loss.

Noninfectious causes of optic neuropathies, including sarcoidosis and demyelinating (including neuromyelitis optica), as well as infectious causes of optic neuritis, including TB and syphilis, were considered, but the time course did correspond with this category of diseases. Bilateral anterior ischemic optic neuropathy was also considered, but our patient lacked the acute onset of vision loss commonly consistent with this disease. As for non-arteritic anterior ischemic optic neuropathy, there is rarely progression. Arteritic anterior ischemic optic neuropathy could progress without the appropriate treatment; however, the progression is typically rapid, and patients often have other systemic symptoms. Infiltrative causes such as lymphoma and leukemia were also on the differential but less likely given a negative review of systems.

Click here to read the full publication exclusive, Grand Rounds at the New England Eye Center, published in Ocular Surgery News U.S. Edition, October 10, 2015.