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Retinal Vessel Occlusion

A retinal vessel occlusion is a blockage in a major artery or vein of the retina Retina The ten-layered nervous tissue membrane of the eye. It is continuous with the optic nerve and receives images of external objects and transmits visual impulses to the brain. Its outer surface is in contact with the choroid and the inner surface with the vitreous body. The outermost layer is pigmented, whereas the inner nine layers are transparent. Eye: Anatomy. Depending on the location, the occlusion can be classified as central retinal artery occlusion (CRAO), branch retinal artery occlusion (BRAO), central retinal vein occlusion (CRVO), or branch retinal vein occlusion (BRVO). Typically, a retinal vessel occlusion is a thromboembolic event. Risk factors include hypertension Hypertension Hypertension, or high blood pressure, is a common disease that manifests as elevated systemic arterial pressures. Hypertension is most often asymptomatic and is found incidentally as part of a routine physical examination or during triage for an unrelated medical encounter. Hypertension, diabetes mellitus Diabetes mellitus Diabetes mellitus (DM) is a metabolic disease characterized by hyperglycemia and dysfunction of the regulation of glucose metabolism by insulin. Type 1 DM is diagnosed mostly in children and young adults as the result of autoimmune destruction of β cells in the pancreas and the resulting lack of insulin. Type 2 DM has a significant association with obesity and is characterized by insulin resistance. Diabetes Mellitus, and cardiac valvular disease. Central retinal vessel occlusion is characterized by sudden, unilateral, painless loss of vision Vision Ophthalmic Exam and/or transient vision Vision Ophthalmic Exam loss ( amaurosis fugax Amaurosis fugax Transient complete or partial monocular blindness due to retinal ischemia. This may be caused by emboli from the carotid artery (usually in association with carotid stenosis) and other locations that enter the central retinal artery. Carotid Artery Stenosis). Treatment options are limited in all cases and usually ineffective. When the macula Macula An oval area in the retina, 3 to 5 mm in diameter, usually located temporal to the posterior pole of the eye and slightly below the level of the optic disk. It is characterized by the presence of a yellow pigment diffusely permeating the inner layers, contains the fovea centralis in its center, and provides the best phototropic visual acuity. It is devoid of retinal blood vessels, except in its periphery, and receives nourishment from the choriocapillaris of the choroid. Eye: Anatomy is involved, prognosis Prognosis A prediction of the probable outcome of a disease based on a individual's condition and the usual course of the disease as seen in similar situations. Non-Hodgkin Lymphomas is especially poor, leading to permanent vision Vision Ophthalmic Exam loss.

Last updated: May 30, 2022

Editorial responsibility: Stanley Oiseth, Lindsay Jones, Evelin Maza

Anatomy

  • The internal carotid artery Internal carotid artery Branch of the common carotid artery which supplies the anterior part of the brain, the eye and its appendages, the forehead and nose. Carotid Arterial System: Anatomy enters the skull Skull The skull (cranium) is the skeletal structure of the head supporting the face and forming a protective cavity for the brain. The skull consists of 22 bones divided into the viscerocranium (facial skeleton) and the neurocranium. Skull: Anatomy through the carotid foramen and branches into the ophthalmic artery Ophthalmic artery Artery originating from the internal carotid artery and distributing to the eye, orbit and adjacent facial structures. Eye: Anatomy, which further divides into:
    • The central retinal artery; supplies the inner ⅔ of the retina Retina The ten-layered nervous tissue membrane of the eye. It is continuous with the optic nerve and receives images of external objects and transmits visual impulses to the brain. Its outer surface is in contact with the choroid and the inner surface with the vitreous body. The outermost layer is pigmented, whereas the inner nine layers are transparent. Eye: Anatomy
    • The posterior ciliary arteries Arteries Arteries are tubular collections of cells that transport oxygenated blood and nutrients from the heart to the tissues of the body. The blood passes through the arteries in order of decreasing luminal diameter, starting in the largest artery (the aorta) and ending in the small arterioles. Arteries are classified into 3 types: large elastic arteries, medium muscular arteries, and small arteries and arterioles. Arteries: Histology (branch retinal artery); supply the outer ⅓ of the retina Retina The ten-layered nervous tissue membrane of the eye. It is continuous with the optic nerve and receives images of external objects and transmits visual impulses to the brain. Its outer surface is in contact with the choroid and the inner surface with the vitreous body. The outermost layer is pigmented, whereas the inner nine layers are transparent. Eye: Anatomy
  • The central retinal vein 
    • Drains either into the superior ophthalmic vein or the cavernous sinus directly

Etiology

Central retinal artery occlusion/branch retinal artery occlusion

  • Embolism (most common cause of ipsilateral CRAO) 
    • Cardiogenic (under age 40) or atherosclerotic (over age 40 and most commonly of carotid origin) 
      • Risk factors include:  
        • Hypertension Hypertension Hypertension, or high blood pressure, is a common disease that manifests as elevated systemic arterial pressures. Hypertension is most often asymptomatic and is found incidentally as part of a routine physical examination or during triage for an unrelated medical encounter. Hypertension
        • Diabetes mellitus Diabetes mellitus Diabetes mellitus (DM) is a metabolic disease characterized by hyperglycemia and dysfunction of the regulation of glucose metabolism by insulin. Type 1 DM is diagnosed mostly in children and young adults as the result of autoimmune destruction of β cells in the pancreas and the resulting lack of insulin. Type 2 DM has a significant association with obesity and is characterized by insulin resistance. Diabetes Mellitus
        • Atrial fibrillation Atrial fibrillation Atrial fibrillation (AF or Afib) is a supraventricular tachyarrhythmia and the most common kind of arrhythmia. It is caused by rapid, uncontrolled atrial contractions and uncoordinated ventricular responses. Atrial Fibrillation
        • Cardiac valvular problems/ patent foramen ovale Patent Foramen Ovale A patent foramen ovale (PFO) is an abnormal communication between the atria that persists after birth. The condition results from incomplete closure of the foramen ovale. Small, isolated, and asymptomatic PFOs are a common incidental finding on echocardiography and require no treatment. Patent Foramen Ovale (leads to clots breaking off) 
    • Cholesterol Cholesterol The principal sterol of all higher animals, distributed in body tissues, especially the brain and spinal cord, and in animal fats and oils. Cholesterol Metabolism embolism; usually occurs after vessel catheterization
    • Septic embolism, as a consequence of infective endocarditis Infective endocarditis Infective endocarditis (IE) is caused by infection or inflammation of the inner lining of the heart (endocardium), most commonly affecting the heart valves. Endocarditis
  • Inflammatory conditions such as temporal arteritis Temporal arteritis Giant cell arteritis (GCA), also known as temporal arteritis, is a type of large-vessel vasculitis that predominantly affects the aorta and its major branches, with a predilection for the branches of the carotid (including the temporal artery). Giant cell arteritis is defined by inflammatory leukocytes in the vessel walls leading to reactive damage, ischemia, and necrosis. Giant Cell Arteritis and other forms of vasculitis Vasculitis Inflammation of any one of the blood vessels, including the arteries; veins; and rest of the vasculature system in the body. Systemic Lupus Erythematosus

Central retinal vein occlusion/branch retinal vein occlusion

Exact causes are not known, although there is an association with the following conditions:

  • Atherosclerotic conditions
    • Hypertension Hypertension Hypertension, or high blood pressure, is a common disease that manifests as elevated systemic arterial pressures. Hypertension is most often asymptomatic and is found incidentally as part of a routine physical examination or during triage for an unrelated medical encounter. Hypertension
    • Hypercholesterolemia Hypercholesterolemia A condition with abnormally high levels of cholesterol in the blood. It is defined as a cholesterol value exceeding the 95th percentile for the population. Lipid Disorders
    • Smoking Smoking Willful or deliberate act of inhaling and exhaling smoke from burning substances or agents held by hand. Interstitial Lung Diseases
    • Diabetes mellitus Diabetes mellitus Diabetes mellitus (DM) is a metabolic disease characterized by hyperglycemia and dysfunction of the regulation of glucose metabolism by insulin. Type 1 DM is diagnosed mostly in children and young adults as the result of autoimmune destruction of β cells in the pancreas and the resulting lack of insulin. Type 2 DM has a significant association with obesity and is characterized by insulin resistance. Diabetes Mellitus
    • Obesity Obesity Obesity is a condition associated with excess body weight, specifically with the deposition of excessive adipose tissue. Obesity is considered a global epidemic. Major influences come from the western diet and sedentary lifestyles, but the exact mechanisms likely include a mixture of genetic and environmental factors. Obesity
  • Hypercoagulable Hypercoagulable Hypercoagulable states (also referred to as thrombophilias) are a group of hematologic diseases defined by an increased risk of clot formation (i.e., thrombosis) due to either an increase in procoagulants, a decrease in anticoagulants, or a decrease in fibrinolysis. Hypercoagulable States state
    • Sickle cell anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types
    • Antiphospholipid syndrome Antiphospholipid syndrome Antiphospholipid syndrome (APLS) is an acquired autoimmune disorder characterized by the persistent presence of antiphospholipid antibodies, which create a hypercoagulable state. These antibodies are most commonly discovered during a workup for a thrombotic event or recurrent pregnancy loss, which are the 2 most common clinical manifestations. Antiphospholipid Syndrome
    • Multiple myeloma Multiple myeloma Multiple myeloma (MM) is a malignant condition of plasma cells (activated B lymphocytes) primarily seen in the elderly. Monoclonal proliferation of plasma cells results in cytokine-driven osteoclastic activity and excessive secretion of IgG antibodies. Multiple Myeloma
  • Chronic inflammatory conditions (e.g., systemic lupus erythematosus Systemic lupus erythematosus Systemic lupus erythematosus (SLE) is a chronic autoimmune, inflammatory condition that causes immune-complex deposition in organs, resulting in systemic manifestations. Women, particularly those of African American descent, are more commonly affected. Systemic Lupus Erythematosus ( SLE SLE Systemic lupus erythematosus (SLE) is a chronic autoimmune, inflammatory condition that causes immune-complex deposition in organs, resulting in systemic manifestations. Women, particularly those of African American descent, are more commonly affected. Systemic Lupus Erythematosus))
  • Pharmacological (e.g., oral contraceptive Oral contraceptive Compounds, usually hormonal, taken orally in order to block ovulation and prevent the occurrence of pregnancy. The hormones are generally estrogen or progesterone or both. Benign Liver Tumors pills)
  • CRVO is categorized into 2 types based on etiology: ischemic (hemorrhagic retinopathy Retinopathy Degenerative changes to the retina due to hypertension. Alport Syndrome) and nonischemic (venous stasis retinopathy Retinopathy Degenerative changes to the retina due to hypertension. Alport Syndrome)

Clinical Presentation and Diagnosis

Clinical presentation

Central retinal artery occlusion

  • Sudden, painless, complete monocular vision loss
  • Patients may recall transient similar episodes (e.g., amaurosis fugax)
  • Fundoscopic examination reveals:
    • Pallor of optic disc due to narrowed arteries
    • Cherry red” spot in the macula Macula An oval area in the retina, 3 to 5 mm in diameter, usually located temporal to the posterior pole of the eye and slightly below the level of the optic disk. It is characterized by the presence of a yellow pigment diffusely permeating the inner layers, contains the fovea centralis in its center, and provides the best phototropic visual acuity. It is devoid of retinal blood vessels, except in its periphery, and receives nourishment from the choriocapillaris of the choroid. Eye: Anatomy (at the fovea Fovea An area approximately 1. 5 millimeters in diameter within the macula lutea where the retina thins out greatly because of the oblique shifting of all layers except the pigment epithelium layer. It includes the sloping walls of the fovea (clivus) and contains a few rods in its periphery. In its center (foveola) are the cones most adapted to yield high visual acuity, each cone being connected to only one ganglion cell. Eye: Anatomy centralis)

Central retinal vein occlusion

  • May be sudden or gradual, painless, monocular vision loss
    • Scotoma or other partial visual field defect
    • May be preceded by visual warning symptoms called “scintillations”
  • Fundoscopic examination reveals
    • Swollen optic disc (papilledema) and engorged retinal veins with hemorrhage
    • Blood and thunder” appearance due to extensive hemorrhage (ischemic type) 
    • Cotton-wool” spots: white deposits on the retina Retina The ten-layered nervous tissue membrane of the eye. It is continuous with the optic nerve and receives images of external objects and transmits visual impulses to the brain. Its outer surface is in contact with the choroid and the inner surface with the vitreous body. The outermost layer is pigmented, whereas the inner nine layers are transparent. Eye: Anatomy due to swelling Swelling Inflammation of the retinal nerve (ischemic type)

Diagnosis

Diagnosis of both conditions is usually clinical but additional investigations may be ordered.

  • Fluorescein Fluorescein A phthalic indicator dye that appears yellow-green in normal tear film and bright green in a more alkaline medium such as the aqueous humor. Pseudomonas angiography Angiography Radiography of blood vessels after injection of a contrast medium. Cardiac Surgery (to differentiate ischemic from non-ischemic forms of CRVO)
  • Optical coherence tomography Optical Coherence Tomography Glaucoma (used to assess the progression of retinal vein occlusion or response to treatment)
  • Carotid doppler Doppler Ultrasonography applying the doppler effect, with frequency-shifted ultrasound reflections produced by moving targets (usually red blood cells) in the bloodstream along the ultrasound axis in direct proportion to the velocity of movement of the targets, to determine both direction and velocity of blood flow. Ultrasound (Sonography) 
  • Erythrocyte sedimentation rate Erythrocyte Sedimentation Rate Soft Tissue Abscess ( ESR ESR Soft Tissue Abscess) and C-reactive protein (CRP): to rule out vasculitides Vasculitides Vasculitides are a group of conditions characterized by vasculitis, ischemia, and damage to the organs supplied by the affected vessels. The affected arteries are of different sizes and locations and vary by the type of vasculitis. Vasculitides

Differential Diagnosis

  • Vitreous hemorrhage Vitreous Hemorrhage Hemorrhage into the vitreous body. Diseases of the Vitreous Body: intraocular bleeding in or around the vitreous body Vitreous body The transparent, semigelatinous substance that fills the cavity behind the crystalline lens of the eye and in front of the retina. It is contained in a thin hyaloid membrane and forms about four fifths of the optic globe. Eye: Anatomy usually from trauma, a retinal tear, or any disease causing neovascularization. Patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship present with floaters Floaters Chorioretinitis and cloudy vision Vision Ophthalmic Exam. Treatment includes surgery and avoiding anticoagulants Anticoagulants Anticoagulants are drugs that retard or interrupt the coagulation cascade. The primary classes of available anticoagulants include heparins, vitamin K-dependent antagonists (e.g., warfarin), direct thrombin inhibitors, and factor Xa inhibitors. Anticoagulants. Vitreous hemorrhage Vitreous Hemorrhage Hemorrhage into the vitreous body. Diseases of the Vitreous Body is differentiated from a vessel occlusion due to a more gradual onset and the presence of floaters Floaters Chorioretinitis
  • Retinal detachment Retinal detachment Retinal detachment is the separation of the neurosensory retina from the retinal pigmented epithelium and choroid. Rhegmatogenous retinal detachment, the most common type, stems from a break in the retina, allowing fluid to accumulate in the subretinal space. Retinal Detachment: separation of the neurosensory retina Retina The ten-layered nervous tissue membrane of the eye. It is continuous with the optic nerve and receives images of external objects and transmits visual impulses to the brain. Its outer surface is in contact with the choroid and the inner surface with the vitreous body. The outermost layer is pigmented, whereas the inner nine layers are transparent. Eye: Anatomy from the retinal pigment epithelium Epithelium The epithelium is a complex of specialized cellular organizations arranged into sheets and lining cavities and covering the surfaces of the body. The cells exhibit polarity, having an apical and a basal pole. Structures important for the epithelial integrity and function involve the basement membrane, the semipermeable sheet on which the cells rest, and interdigitations, as well as cellular junctions. Surface Epithelium: Histology. Classically, the condition presents as a curtain descending over the eye causing painless vision Vision Ophthalmic Exam loss, but can also cause floaters Floaters Chorioretinitis and flashes early on. Most common causes are aging, hypertension Hypertension Hypertension, or high blood pressure, is a common disease that manifests as elevated systemic arterial pressures. Hypertension is most often asymptomatic and is found incidentally as part of a routine physical examination or during triage for an unrelated medical encounter. Hypertension, and trauma. Diagnosis is with direct eye exam and ultrasound. Treatment is surgery by laser or freezing. 
  • Cataracts: opacification of the lens Lens A transparent, biconvex structure of the eye, enclosed in a capsule and situated behind the iris and in front of the vitreous humor (vitreous body). It is slightly overlapped at its margin by the ciliary processes. Adaptation by the ciliary body is crucial for ocular accommodation. Eye: Anatomy in the eye, which leads to a decrease in vision Vision Ophthalmic Exam. Cataracts often develop slowly in one or both eyes Both Eyes Refractive Errors. Risk factors are age and eye injury. Symptoms may include blurry or double vision Vision Ophthalmic Exam, trouble with bright lights, halos around light, faded colors, and trouble seeing at night. Cataracts can be visualized as a cloudy lens Lens A transparent, biconvex structure of the eye, enclosed in a capsule and situated behind the iris and in front of the vitreous humor (vitreous body). It is slightly overlapped at its margin by the ciliary processes. Adaptation by the ciliary body is crucial for ocular accommodation. Eye: Anatomy on eye exam. Curative treatment is surgical replacement of the lens Lens A transparent, biconvex structure of the eye, enclosed in a capsule and situated behind the iris and in front of the vitreous humor (vitreous body). It is slightly overlapped at its margin by the ciliary processes. Adaptation by the ciliary body is crucial for ocular accommodation. Eye: Anatomy.

References

  1. Hedges III, T. R. (2018). Central and branch retinal artery occlusion. UpToDate. Retrieved September 24, 2020, from https://www.uptodate.com/contents/central-and-branch-retinal-artery-occlusion
  2. Covert, D. J. , & Han, D. P. (2019). Retinal vein occlusion: Epidemiology, clinical manifestations, and diagnosis. UpToDate. Retrieved September 24, 2020, from https://www.uptodate.com/contents/retinal-vein-occlusion-epidemiology-clinical-manifestations-and-diagnosis
  3. Hayreh SS, Podhajsky PA, Zimmerman MB. (2009). Retinal Artery Occlusion Associated Systemic and Ophthalmic Abnormalities. Ophthalmology.

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