Vascular Retinopathies
Avnish Deobhakta
Daniel J. Egan
THE CLINICAL CHALLENGE
Vascular retinopathies can represent a sentinel event for serious systemic illness. As such, once the diagnosis of a vascular retinopathy is made or suspected, careful examination and testing for the underlying cause is critical. For patients with ischemic retinopathy, secondary prevention of further vascular insults like stroke can prevent significant morbidity. Identification of these processes can be challenging for the emergency provider given the heterogeneity of the descriptions of symptoms by patients. The experience of visual changes described by patients overlaps between diagnoses. Therefore, maintaining a broad differential diagnosis with a systematic approach to the complaints is important to prevent future events or progression of vision loss.
PATHOPHYSIOLOGY
A general understanding of anatomy is helpful to get a clear grasp of the vascular processes that affect the retina. The ophthalmic artery is the first intracranial branch of the internal carotid artery and enters the orbit accompanied by the optic nerve. The retinal artery branches off to enter the cerebrospinal space supplying the retina. The central retinal artery provides blood flow to the center of the retina, including the macula and fovea, and its branches perfuse the rest of the retina. Notably, up to 50% of the population has a cilioretinal artery that arises from either the choroid or the posterior ciliary arteries and provides most of the macular circulation.1 In these individuals, cases of retinal artery occlusion (RAO) may have preservation of the middle field of vision owing to this variant. Acute retinal ischemia is usually embolic from an ipsilateral carotid artery, the aortic arch, or the heart (valvular disease or thrombus from atrial fibrillation). Patients with sickle cell disease also carry an increased risk of retinal ischemia in vaso-occlusive crises.
Transient Monocular Vision Loss (TMVL)
TMVL, also known as amaurosis fugax, is the most common form of retinal ischemia and the retinal equivalent of a TIA. Approximately 14 to 15 per 10 000 individuals experience this annually.2,3 TMVL typically presents with sudden, episodic, unprovoked, painless monocular blindness lasting seconds to minutes. The causes of TMVL vary, but all eventually result in dysfunction of the neural visual system itself (retina, optic nerve) or the vascular supply to it (choroid). The pathology is usually thromboembolic, causing occlusion of flow through the retinal artery or one of its branches, the primary source of the occlusion being the ipsilateral carotid artery or an inflamed ipsilateral temporal artery.4 As a nonvascular cause, demyelinating disease can present as TMVL secondary to optic neuritis. When chronic severe ipsilateral carotid artery stenosis is the cause of TMVL, it may be attributable to ocular ischemic syndrome.
Retinal Artery Occlusion (RAO)
Pathophysiologically, the occlusion lies within the retinal arterial circulation (either the central retinal artery itself or a distal branch of the central retinal artery) with the type of vision
loss dependent on the location of the clot. Central retinal artery occlusion (CRAO) is associated with profound loss, whereas a branch retinal artery occlusion (BRAO) has a partial scotoma corresponding to the location of the pathology. The cause of RAO is thromboembolic, and, like TMVL, often arises from similar sources. In ocular ischemic syndrome, critical stenosis of the carotid artery reduces central retinal artery perfusion pressure by up to 50%, placing these individuals at high risk for subsequent RAO. Additionally, giant cell arteritis (GCA), carotid artery dissection, vasculitis, sickle cell disease, and various hypercoagulable states can potentially be causative, though less likely.
loss dependent on the location of the clot. Central retinal artery occlusion (CRAO) is associated with profound loss, whereas a branch retinal artery occlusion (BRAO) has a partial scotoma corresponding to the location of the pathology. The cause of RAO is thromboembolic, and, like TMVL, often arises from similar sources. In ocular ischemic syndrome, critical stenosis of the carotid artery reduces central retinal artery perfusion pressure by up to 50%, placing these individuals at high risk for subsequent RAO. Additionally, giant cell arteritis (GCA), carotid artery dissection, vasculitis, sickle cell disease, and various hypercoagulable states can potentially be causative, though less likely.
Retinal Vein Occlusion (RVO)
In general, the major risk factor for RVO is systemic hypertension, along with advanced age (>65), smoking, and diabetes mellitus. Less common causes include hypercoagulable states (eg, multiple myeloma, polycythemia vera, and sickle cell disease), inflammatory diseases (eg, sarcoidosis), infectious diseases (eg, syphilis and tuberculosis), and pharmacologic causes (eg, oral contraceptive). RVO is the second most common retinal vasculopathy after diabetic retinopathy.5
Much like its arterial counterpart, RVO is broadly split into two types: central RVO (CRVO) and branch RVO (BRVO). In CRVO, the occlusion is thought to be caused by either a thrombus posterior to the lamina cribrosa of the optic nerve or turbulence initiated by central retinal arterial hypertension that compresses the nearby central retinal vein, the latter of which shares a sheath with its arterial counterpart. In BRVO, a similar thrombotic or inflammatory blockage can occur within one of the tributaries of the central retinal vein.
Hypertensive Retinopathy
In the context of long-standing and poorly controlled hypertension, the retina experiences progressive microvascular changes. Physiologically, retinal vessels have unique features that distinguish them from a generic vasculature, including the absence of a sympathetic nerve supply and the presence of a blood-retinal (brain) barrier. Long-standing elevated blood pressure damages retinal vessels, causing decreased neurosensory retinal function.6 Damage to the endothelial cells leads to angiogenesis and neovascularization, which may further impair vision. Risk factors for this condition include any that are related to hypertension, including genetic factors and smoking.
APPROACH/THE FOCUSED EXAM
The American Heart and National Stroke Associations have amended the definition of stroke to include brain, spinal cord, or retinal cell death attributable to ischemia. As such, patients with acute vascular retinopathies are evaluated similarly to patients experiencing other stroke syndromes, including an assessment of risk factors. For all patients with a complaint of visual disturbances, the emergency provider should obtain a thorough medical history, including past ophthalmologic history, medications, and history of similar problems in the past. Focusing on the present illness, it is important to clarify certain key elements: onset of symptoms, monocular or binocular symptoms, duration of symptoms, associated symptoms (eg, headache or neck pain), and neurologic deficits. Furthermore, because GCA is associated with vascular retinopathies, a review of systems for this disease is critical. These patients are over age 50 and may report a history of jaw claudication, headache (particularly in the region of the temporal artery), tenderness of the scalp, constitutional symptoms, arthralgias, and fever (see Chapter 49). The physical examination will focus primarily on the eye, including a thorough examination of the retina, usually performed by an ophthalmologist after pupillary dilation. When GCA is suspected, measurement of inflammatory markers is recommended (erythrocyte sedimentation rate [ESR], C-reactive protein [CRP], and platelets).
Visual acuity and an assessment of the visual fields may uncover deficits. A comprehensive neurologic examination including cranial nerves is warranted. Given the association with underlying vascular disease, evaluation of the carotid arteries for bruits or evidence of other vascular disease on examination is important. Auscultation of the heart and assessment with electrocardiogram may identify a causative dysrhythmia like atrial fibrillation.
Transient Monocular Vision Loss
Patients will occasionally endorse a “vertically dropping black curtain” when describing their visual disturbance, although others may simply describe a “graying or darkening” of vision instead.
Symptoms are typically “negative” symptoms with loss of vision and do not characteristically include colors or flashes of light. It is important to keep in mind the inconsistency of symptomatic descriptions by patients. Most patients will report these episodes lasting anywhere from 2 to 30 minutes. Patients typically present for evaluation after symptoms have resolved. As a result, examination of the retina is expected to be normal.
Symptoms are typically “negative” symptoms with loss of vision and do not characteristically include colors or flashes of light. It is important to keep in mind the inconsistency of symptomatic descriptions by patients. Most patients will report these episodes lasting anywhere from 2 to 30 minutes. Patients typically present for evaluation after symptoms have resolved. As a result, examination of the retina is expected to be normal.
Retinal Artery Occlusion
Like TMVL, this condition presents with sudden, painless, often profound, monocular vision loss that does not resolve. RAO is considered a stroke of the retina, and assessment of these patients should mirror a general stroke evaluation. Medical history will often reveal some combination of cerebrovascular risk factors like hypertension, hyperlipidemia, diabetes mellitus, or tobacco use. Examination includes a complete neurologic examination. Visual acuity often demonstrates profound vision loss when the occlusion is central. Some preservation of vision is possible with a BRAO or in patients with a cilioretinal artery. On assessment of the pupils, the affected eye will usually have an afferent pupillary defect. On fundoscopic examination, the retina demonstrates a white appearance because of retinal edema. The most obvious clinical finding for a CRAO is a cherry red spot noted in the central fundus, which is seen in 90% of patients (Figure 41.1) and related to preserved choroidal blood flow to the fovea.7 In other cases, a frank embolus (termed a “Hollenhorst plaque”) can be seen within the lumen of a vessel (Figure 41.2). In BRAO, segmental white discoloration of the retina is seen. The optic nerve should appear normal in both CRAO and BRAO.