Retinal artery occlusion is characterized by sudden, painless, monocular loss of vision, most often due to an embolus occluding the central retinal artery or its branches. The diagnosis of the condition depends on clinical presentation and the classic funduscopic appearance of the retina.
Presentation
Retinal artery occlusion (RAO) was first described in 1859 by von Graefe [1]. It mainly occurs due to the occlusion of the central retinal artery (CRA) or its branches, usually by an embolus and is reported to affect approximately 2 out of every 100,000 individuals in the United States [2]. The patients present classically with a sudden onset of unilateral, painless, visual loss which may rapidly deteriorate to the level of mere finger counting ability or even complete loss of vision [3] [4]. Up to 30% of individuals have a cilioretinal artery [5], a branch of the short posterior ciliary artery, which may lead to significant sparing of the vision as the cilioretinal artery supplies part or the entire fovea and is unaffected in the central retinal artery occlusion.
Clinically RAO has four different entities:
- Permanent non-arteritic RAO accounts for a majority of the cases. It is caused by thrombi or emboli secondary to atherosclerosis [6] [7] [8].
- Transient non-arteritic RAO is associated with transient monocular visual loss and has a good prognosis for regaining vision.
- Non-arteritic RAO with cilioretinal sparing results in only peripheral visual loss due to sparing of the cilioretinal artery and the macula [4].
- Arteritic RAO accounts for a minor percentage of RAO cases and primarily includes patients with giant cell arteritis. Patients are usually above the age of 55 years and may have a bilateral visual loss with other symptoms like a temporal headache, temporal artery tenderness, jaw claudication, and fatigue.
As already mentioned, the commonest cause of RAO is a thrombus or an embolus occluding the central retinal artery as it pierces the dural sheath of the optic nerve [9]. Retinal recovery after RAO depends on the dislodgement of the embolus or thrombus and on the tolerance time of the retina [10] [11].
Entire Body System
- Increased Energy
A fundus contact lens is used with the laser in single-burst mode. • Pulses are delivered directly to the emboli, beginning with the lowest power setting and then with increasing energy until either (1) achieving photofragmentation of the embolus within [slideshare.net]
Immune System
- Generalized Lymphadenopathy
Systemic evaluation revealed a history of chronic low-grade fever and generalized lymphadenopathy. HIV (ELISA) was positive, and other systemic comorbidities were ruled out. [ncbi.nlm.nih.gov]
Gastrointestinal
- Regurgitation
Mild mitral valve regurgitation was noted on echocardiogram. Hemoglobin 12.4gm%, 1h ESR 23mm (Westergren); Complete blood count, coagulation profile and serum lipid profile were normal. FBS † : 82mg/dl, TSH 4.4 µU/ml, Free T4 1.0 ng/dl. [ncbi.nlm.nih.gov]
Musculoskeletal
- Shoulder Pain
pain (claudication), fever, scalp tenderness, and weight loss) • Endocarditis • Intravenous Drug Use Because these are such serious systemic diseases, prompt referral to either the primary care physician or the emergency room should also be part of your [optometrystudents.com]
Eyes
- Visual Impairment
RESULTS: A 65-year-old woman developed dramatic visual impairment in her left eye secondary to BRAO after left internal carotid artery endovascular reconstruction with flow diverter stent implant; visual acuity was hand motion. [ncbi.nlm.nih.gov]
An abrupt blockage of blood flow (ischemia) halts proper retinal functioning, which results in visual impairment. Depending on the extent and location of the retina affected, the loss of vision may be relatively mild or very severe. [midatlanticretina.com]
On the positive side doctors are looking into laser surgery and how it may help rectify retinal damage and fix the visual impairment caused by the occlusion. [kanataoptometry.ca]
On the positive side, however, doctors are investigating laser surgery to help rectify retinal damage and visual impairment caused by the occlusion of the retinal arteries. [waterdownoptometric.ca]
It may lead to severe irreversible visual impairment. This condition mostly occurs in patients with high blood pressure, heart disease, diabetes or carotid atherosclerosis in elderly people (Varma et al. 2013 ). [springerplus.springeropen.com]
- Retinal Pallor
Retinal pallor corresponds to the area supplied by the affected artery. Occasionally, an embolus (cholesterol, fibrinoplatelet, calcific) can be seen within the artery, and cotton wool spots may eventually occur around that area. [patient.info]
Urogenital
- Polyuria
Convulsions, Drowsiness, Flaccid paralysis, Malaise GI: Anorexia, Diarrhea, Metallic taste, Nausea, Vomiting, Hepatic disease, Melena Blood: Aplastic anemia, Agranulocytosis, Leukopenia Paresthesia, Thrombocytopenia, Thrombocytopenic purpura Renal: Hematuria, Polyuria [link.springer.com]
Neurologic
- Temporal Headache
Patients are usually above the age of 55 years and may have a bilateral visual loss with other symptoms like a temporal headache, temporal artery tenderness, jaw claudication, and fatigue. [symptoma.com]
- Recurrent Headache
A 60-year-old woman with a history of recurrent headaches and blurred vision presented with bilateral optic disc edema. Optic neuritis was suspected, and intravenous methylprednisonlone was administered. [ncbi.nlm.nih.gov]
Workup
The diagnosis of RAO is suspected based on the clinical presentation and can be confirmed with clinical evaluation, fundoscopy, and fluorescein angiography. An ophthalmic examination may reveal a relative afferent pupillary defect while the funduscopic appearance of a pale, opaque fundus with a red fovea (cherry-red spot) is confirmatory. Occasionally, a cholesterol embolus (known as Hollenhorst plaque) may also be seen. In cases where the central retinal artery is spared and only a major branch is occluded, visual loss may be restricted to the corresponding sector of the retina. Fluorescein angiography detects the lack of perfusion in the occluded artery.
If non-arteritic permanent RAO due to thrombus or embolus is suspected then the physician should inquire about risk factors for atherosclerosis, a past history of vascular disease, cardiac disease, smoking, transient ischemic events, angina, and renal disease [12] [13]. In young patients, if there is no suspicion of atherosclerotic events, other etiologies like vasculitis, hemoglobinopathies, myeloproliferative disorders, hypercoagulable conditions, and the use of oral contraceptives should be excluded [14].
After confirming the diagnosis of RAO, carotid Doppler ultrasonography and echocardiography are performed to locate the source of the embolus and to prevent further embolization. An electrocardiogram (ECG) is ordered to detect atrial fibrillation and a 24-hour Holter monitoring may be indicated in patients with suspected arrhythmia. Laboratory tests are useful to diagnose the etiology and may include complete blood count, erythrocyte sedimentation rate (ESR), fibrinogen levels, antiphospholipid antibodies, activated partial thromboplastin time (aPTT), prothrombin time (PT), lipid panel, blood culture, and work up to exclude sickle cell disease.
Imaging studies such as echocardiography or carotid Doppler ultrasonography are performed urgently while magnetic resonance angiography (MRA) and computed tomography (CT) angiography can be performed later to detect atherosclerotic disease.
Treatment
A vasodilator (nicotinic acid) was used as the primary treatment. OCTA imaging and a visual field examination were performed to assess the retinal perfusion changes before and after treatment. Retinal artery occlusion was considered. [ncbi.nlm.nih.gov]
In comparison, visual improvement after conservative treatment according to three different stages is shown in Table 9. Obviously, the results after treatment with LIF are better than with conservative treatment. [ajnr.org]
Time from symptom onset to treatment ranged from 1-312 hours with median time to treatment of 9 hours for both groups. [jneuro.com]
Prognosis
The use of this as a non-invasive examination can improve the prognosis of patients and future studies investigating the treatment of central retinal artery occlusion. [ncbi.nlm.nih.gov]
Prognosis Recovery of useful vision is related directly to the rapidity of treatment and presenting visual acuity. [emedicine.medscape.com]
Etiology
Retinal artery occlusion is an extremely rare diagnosis in the pediatric population and the etiology with risk factors of retinal artery occlusion are poorly understood in younger individuals. [ncbi.nlm.nih.gov]
With regard to etiology, 62 patients (41.1%) had LAA, the most common etiological factor. About 40% of our patients had a RAO of undetermined etiology. [journals.plos.org]
Laboratory tests are useful to diagnose the etiology and may include complete blood count, erythrocyte sedimentation rate (ESR), fibrinogen levels, antiphospholipid antibodies, activated partial thromboplastin time (aPTT), prothrombin time (PT), lipid [symptoma.com]
Epidemiology
Huisingh, 1 Gerald McGwin Jr, 1,3 Martin L Thomley 1,2 1 Department of Ophthalmology, University of Alabama School of Medicine, Birmingham, AL, USA; 2 Retina Consultants of Alabama, Callahan Eye Foundation Hospital, Birmingham, AL, USA; 3 Department of Epidemiology [dovepress.com]
Two thirds of patients experience 20/400 vision while only one in six will experience 20/40 vision or better. [1] Epidemiology [ edit ] Risk factors for CRAO include the following: being between 60 and 65 years of age, being over the age of 40, male gender [en.wikipedia.org]
Diagnosis: Central Retinal Artery Occlusion (CRAO) EPIDEMIOLOGY Age > 40 years old. [eyerounds.org]
This review will describe the pathophysiology, epidemiology, and clinical features of CRAO, and discuss current and future treatments, including the use of tPA in further clinical trials. [nature.com]
Pathophysiology
The results suggest that we must consider SVD etiology as well as large vessel disease mechanisms in the pathophysiology of BRAO. [ncbi.nlm.nih.gov]
CRAO is the same pathophysiology in the retinal circulation. [hbot.com]
The underlying pathophysiology is usually secondary to blockage of the retinal artery from a retinal emboli. The most common retinal emboli types are calcific, platelet-fibrin or cholesterol, also commonly referred to as Hollenhorst plaques. [octscans.com]
Pathophysiology of Retinal Artery Occlusion These retinal arteries carry oxygen and nutrient rich blood to the retina. If it is occluded then the retinal tissue is starved of both oxygen and nutrients. [healthhype.com]
Date of Submission 12-Jan-2015 Date of Acceptance 25-Mar-2015 Date of Web Publication 25-May-2015 Dear Editor, The occurrence of postoperative visual loss (POVL) after spine surgery in prone position, although rare, has been described.[ 1 6 7 ] Various pathophysiologic [surgicalneurologyint.com]
Prevention
It is appropriate that they should have more proactive care as well, including blood pressure and lipids to prevent future heart disease. [waterdownoptometric.ca]
Treatment for such a clot could help to prevent a stroke. If a CRAO occurs in an elderly patient, temporal arteritis might be the cause. [iowaretina.com]
Lack of randomization and intrinsic biases prevent any definite conclusions regarding the benefits and further research is warranted. [ncbi.nlm.nih.gov]
What can I do to prevent retinal artery occlusion? Measures used to prevent other blood vessel (vascular) diseases, such as coronary artery disease, may decrease the risk of retinal artery occlusion. [gulfcoastretina.com]
References
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- Leavitt JA, Larson TA, Hodge DO, Gullerud RE. The incidence of central retinal artery occlusion in Olmsted County, Minnesota. Am J Ophthalmol. 2011;152:820–823.
- Rumelt S, Brown GC. Update on treatment of retinal arterial occlusions. Curr Opin Ophthalmol. 2003;14:139–141.
- Hayreh SS, Zimmerman MB. Central retinal artery occlusion: visual outcome. Am J Ophthalmol. 2005;140:376–391.
- Lorentzen S. Incidence of cilioretinal arteries. Acta Ophthalmol. 1970;48:518–524.
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- Rudkin A, Lee A, Chen C. Vascular risk factors for central retinal artery occlusion. Eye. 2009;24:678–681.
- Schmidt DP, Schulte-Mönting J, Schumacher M. Prognosis of central retinal artery occlusion: local intraarterial fibrinolysis versus conservative treatment. Am J Neuroradiol. 2002;23:1301–1307.
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- Hayreh SS. Prevalent misconceptions about acute retinal vascular occlusive disorders. Progr Retin Eye Res. 2005;24:493–519.
- Hayreh S, Kolder H, Weingeist T. Central retinal artery occlusion and retinal tolerance time. Ophthalmology. 1980;87:75.
- Pfaffenbach D, Hollenhorst R. Morbidity and survivorship of patients with embolic cholesterol crystals in the ocular fundus. Trans Am Ophthalmol Soc. 1972;70:337.
- Hayreh SS, Podhajsky PA, Zimmerman MB. Retinal artery occlusion: associated systemic and ophthalmic abnormalities. Ophthalmology. 2009;116:1928–1936.
- Greven CM, Slusher MM, Weaver RG. Retinal arterial occlusions in young adults. Am J Ophthalmol. 1995;120:776–783.