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Young patient presents with painless vision loss
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A 47-year old African American male presented to the clinic with a history of sudden painless vision loss involving his right eye. He reported that it began approximately 5 days prior but had remained stable since. There were no other ocular, visual or neurological symptoms reported.
He said he was told elsewhere that he had “bleeding in the back of his right eye” a year ago. At that visit elsewhere, he was asked to see a retinologist but he failed to follow through. His medical history was remarkable for uncontrolled type 2 diabetes mellitus (DM) diagnosed 16 years earlier with a reported hemoglobin HbA1C of 12% and a fasting blood sugar (FBS) of 290 mg/dL. He was taking metformin HCl 500 mg, two tablets twice daily for his DM. In addition, he also admitted to a medical history of hypercholesterolemia, for which he was not taking a medication, and hypertension, for which he was taking losartan potassium (50 mg daily).
Best-corrected visual acuity was light perception in the right eye (not improving with pinhole) and 20/20 in the left eye. Both pupils were round, equal and reactive to light with a trace relative afferent pupillary defect (APD) in the right eye. Confrontation fields were full to finger counting in the right and left eyes, and ocular motility of each eye showed no restrictions. Blood pressure was measured as 152/110 mm Hg, right arm sitting. Slit lamp examination of the anterior segment was unremarkable. Intraocular pressure was 23 mm Hg OD and 18 mm Hg OS.
Dilated stereoscopic fundus exam revealed normal physiological cupping with perfused neural retinal rim tissue in each eye. Arteriovenous nicking and arterial attenuation were noted in each eye but were more pronounced in the right eye. Maculae were flat and intact. Posterior pole assessment revealed scattered hemorrhages (primarily dot and blot plus a few flame shaped), as well as exudates and cotton-wool (CWS) spots in each eye. In addition, the right eye revealed segmented vascular and ischemic retina surrounding the macula with an associated “cherry-red spot” in the right eye. The peripheral retina was flat and intact 360 degrees in each eye without conditions predisposing to retinal detachment.
The patient was diagnosed with central retinal artery occlusion (CRAO) in the right eye secondary to retinal/systemic vascular disease. An optical coherence tomography was ordered. In addition, fluorescein angiography was recommended. Pending these results, further medical work-up would be planned.
Images: Semes LP
Based on the symptoms and clinical findings in this case, differential diagnoses included: retinal artery occlusion, hypertensive retinopathy (HTR), diabetic retinopathy (DR), radiation retinopathy (RR) and sickle cell retinopathy (SCR).
Working through the differentials
Based on the patient’s race and retinal findings, SCR was considered. The absence of “salmon patches,” sunbursts or proliferative retinopathy findings made this diagnosis unlikely. Additionally, the patient did not admit to a history of sickle cell disease. In addition to posterior segment findings, patients with SCR may present with anterior segment findings, such as comma-shaped vessels in the conjunctiva. Of note, sickle cell disease may contribute to the unique retinal ischemic finings noted in the right eye.
Vascular retinopathy noted in both eyes is also a common presentation of RR. It presents with associated variable retinal hemorrhages, CWS and exudates. The absence of radiation exposure either accidentally or therapeutically made this diagnosis unlikely.
Given the systemic blood pressure and vascular retinopathy, HTR was considered and may be a contributing factor to the clinical picture. The concurrent existence of diabetes and hypertension in the presence of retinal vascular manifestations adds to the diagnostic challenge. It may be difficult to discern if, in fact, the vascular picture is attributable solely to DM or hypertension. Generally, severe HTR presents with associated flame-shaped hemorrhages and CWS rather than dot-blot hemorrhages and exudates seen with DR. Sudden painless vision loss is not consistent with hypertensive retinopathy, per se. But the clinical picture is likely contributed to by systemic hypertension.
The current retinal vascular disease could be consistent with a CRVO, especially given the history of uncontrolled medical conditions. Although non-ischemic CRVO was a consideration, the patient’s visual acuity is inconsistent with the consequence of macular edema consistent with that diagnosis. Moreover, the presence of the trace APD and lack of the typical hemorrhagic retina diminished the diagnosis of CRVO.
Outcome
Optical coherence tomography (OCT) revealed a normal macular architecture of the left eye. However, the right eye revealed increased inner retinal thickness. No sign of intraretinal edema was noted in either eye. Funduscopically, the retina of the right and left eye showed scattered dot and blot hemorrhages and hard exudates in the posterior pole. Along with the retinal hemorrhages and exudates in the right eye, there was an overall appearance of retinal edema consistent with the mild thickening seen on OCT. The “cherry-red” spot indicating persistent choroidal perfusion of the macula stands in strong contrast to the background of the retina. These findings strengthened the suspicion of CRAO and confirmed the diagnosis in the right eye. This is likely the consequence of systemic vascular disease complicated by diabetes.
CRAO typically presents with acute monocular loss of vision ranging from counting fingers to light perception associated with a relative APD.
The initial sequelae include retinal opacification (whitening) within the posterior pole, representing inner retinal edema surrounding a cherry-red spot. Box-carring, retinal-arterial attenuation and optic disc edema may be noted. Inner retinal ischemia represents the late stage of a CRAO, evident by a thinning retina. Subsequent OCT is likely to show a diminished RNFL. Optic atrophy, retinal arterial attenuation and macular retinal pigment epithelial changes can be seen as consequences.
As a result of his condition and age, the patient was asked to seek medical care for further management and work-up of the underlying uncontrolled systemic conditions. He was advised to undergo a complete medical evaluation, in addition to a carotid Doppler, echocardiogram, CBC with differentials, SickleDex (Streck) and re-evaluation of FBS and HbA1c. Unfortunately, the patient failed to follow through.
Our patient presented with what we considered an unusual constellation of findings. Given the duration of diabetes, uncontrolled status and comorbidities, the patient was at risk for developing bilateral DR and HTR. These factors were likely contributory to development of a CRAO.
- References:
- Coady PA, et al. Br J Ophthalmol. 2015;doi:10.1136/bjophthalmol-2014-304900.
- Hayreh SS, et al. Arch Ophthalmol. 1982;100(10):1585-1596.
- Hayreh SS. Prog Retin Eye Res. 2014;doi:10.1016/j.preteyeres.2014.04.001.
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- For more information:
- 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: The authors report no relevant financial disclosures.