Man presents with sudden onset decrease in central vision in left eye
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A 43-year-old man presented with a chief complaint of a sudden onset “gray spot” associated with a decrease in central vision in the left eye 2 days earlier.
The patient had no ocular history. His medical history was significant for hypertensive emergency and non-ST-elevation myocardial infarction (NSTEMI) in the setting of COVID infection 4 months prior, after which he was started on several antihypertensive medications and a statin.
Source: Julia Ernst, MD, PhD, and Jeffrey L. Marx, MD
Examination
On exam, best uncorrected distance visual acuity was 20/25 in the right eye and counting fingers at 6 feet in the left eye. Pupils were equal, round and briskly reactive to light bilaterally with no relative afferent pupillary defect. IOPs were normal. Extraocular motility and confrontation visual fields were full bilaterally.
Slit lamp exam of the anterior segment was unremarkable for both eyes. The posterior segment exam of the right eye showed a few scattered drusen in the macula, flat fovea and one nasal flame hemorrhage. A few intraretinal hemorrhages and pigment clumping were observed in the periphery. Fundus exam of the left eye revealed a large foveal pigment epithelial detachment with preretinal and subretinal hemorrhages and pinpoint exudates. Other findings included a tortuous superior retinal artery, scattered intraretinal hemorrhages and pigment clumping in the periphery.
Imaging
On the initial visit, fundus photo (Figure 1) and OCT of the macula (Figure 2) of the left eye were taken. OCT of the left macula demonstrated significantly increased foveal thickness, with subretinal fluid, exudates and intraretinal fluid. Fundus fluorescein angiography (FFA) was deferred due to the anticipated blocking effect from the large preretinal hemorrhage. OCT of the left macula a week later (Figure 3) showed improvement in central foveal thickness, decreased subretinal hemorrhage and resolved subretinal fluid.
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Unilateral central vision loss
The patient’s symptoms of sudden onset unilateral central vision loss with a multilayer retinal hemorrhage in the setting of hypertension were highly suggestive of ruptured retinal artery macroaneurysm (RAM). Multilayer retinal hemorrhage could also be caused by trauma. However, the patient denied any recent history thereof. A possible underlying condition could have been branch retinal vein occlusion leading to RAM with subsequent rupture. Macular hemorrhage can also be present in several other conditions such as exudative age-related macular degeneration, proliferative diabetic retinopathy (PDR) and complicated posterior vitreous detachment (PVD). However, these etiologies were unlikely given the patient’s young age for AMD, no underlying diabetes for PDR and no other exam findings of PVD.
Workup and management
The patient underwent an extensive workup for hypertension and management of hyperlipidemia following his episode of hypertensive emergency and NSTEMI, which was managed by his primary team. According to the patient, his blood pressure was well controlled since. On presentation to the clinic, he underwent multimodal imaging including fundus photography and OCT of the macula. He was given the option of treatment of ruptured RAM with macular hemorrhage with pneumatic displacement with or without intravitreal tissue plasminogen activator (tPA), but he opted for observation. He was followed closely and seen within a week with improved central foveal thickness, decreased subretinal hemorrhage and resolved subretinal fluid. He was seen again a month later with further improvement in anatomy. However, his visual acuity remained the same, at the level of counting fingers. He remains under close observation.
Discussion
Retinal artery macroaneurysm is a focal dilation of the retinal artery, ranging from 100 µm to 250 µm in diameter, that forms within the first three branches of the arteriolar tree. They are most commonly located in the superotemporal retina, and they are associated with varying degrees of hemorrhage, exudation and retinal edema. They can be congenital or acquired, and as such, they can be associated with a variety of conditions including von Hippel-Lindau syndrome, Coats disease, Eales disease, systemic hypertension, arteriosclerosis, diabetic retinopathy, retinal vein occlusion and retinitis, among others. The pathology behind RAM involves hyaline degeneration of the vascular walls associated with hypertrophy of the muscularis layer, loss of autoregulation and elastic recoil, which eventually leads to arterial dilatation.
Risk factors are female gender, age older than 60 years, arteriosclerotic disease and systemic hypertension. RAM is usually unilateral and singular; however, multiple arterial aneurysms can be present in up to 20% of cases, and in about 10%, they are bilateral. The most important risk factor associated with RAM is systemic hypertension, which is present in 75% of patients. We believe that this was a significant contributor to etiology of RAM in our patient, along with arteriosclerosis and abnormal lipid levels.
RAMs are usually asymptomatic and discovered incidentally on funduscopic eye exam. They are unlikely to affect vision unless they extend to the macula. In this case, RAM rupture caused macular edema and hemorrhage, which led to central vision loss. The hallmark funduscopic finding of ruptured RAM is blood at multiple layers, including preretinal, intraretinal, subretinal, sub-internal limiting membrane and vitreous. Fundus exam of our patient revealed large preretinal and subretinal hemorrhages, which are typical for this condition.
The most commonly used imaging modalities in diagnosis of RAM are OCT and fundus fluorescein angiography (FFA). OCT usually shows a circular intraretinal lesion with a hyperreflective wall. FFA is regarded as the most helpful tool for diagnosis of RAM as it can reveal pathognomonic immediate filling of a saccular dilation in the arteriolar wall. In our patient, FFA was not pursued due to the blocking effect of preretinal hemorrhage over the location of interest, which would most likely preclude good visualization of the pathology.
RAM usually undergoes spontaneous involution in up to 75% of cases. In about one-third of patients, RAM might undergo vascular leakage and retinal edema, which might require treatment to prevent central vision loss. Our patient had submacular hemorrhage, which has the poorest outcome as it can lead to macular hole formation, subretinal neovascularization or neurosensory detachment with subsequent atrophy. Nevertheless, even in vision-threatening RAM, it may be closely observed for 3 months due to the high chance of spontaneous resolution. Systemic workup for hypertension and vascular disease is warranted. Treatment is recommended for complicated or non-resolving RAMs. However, there are no consensus guidelines for treatment. Currently, the options include laser photocoagulation, intravitreal injection of anti-VEGF agents, Nd:YAG laser hyaloidotomy, pars plana vitrectomy, or pneumatic displacement with or without intravitreal tPA. The patient had subfoveal hemorrhage without vitreous hemorrhage. Hence, he was a good candidate for pneumatic displacement with or without intravitreal tPA. This procedure involves the use of perfluorocarbon gas with intravitreal tPA, which is thought to lead to thrombolysis and hence clearance of the subretinal hemorrhage, followed by prone positioning. However, given its unproven success rate and postoperative burden, our patient opted for observation.
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- For more information:
- Edited by Jonathan T. Caranfa, MD, PharmD, and Angell Shi, MD, of New England Eye Center, Tufts University School of Medicine. They can be reached at jcaranfa@tuftsmedicalcenter.org and ashi@tuftsmedicalcenter.org.
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