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Retinal Artery Occlusion

RAO

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:

  1. Permanent non-arteritic RAO accounts for a majority of the cases. It is caused by thrombi or emboli secondary to atherosclerosis [6] [7] [8].
  2. Transient non-arteritic RAO is associated with transient monocular visual loss and has a good prognosis for regaining vision.
  3. Non-arteritic RAO with cilioretinal sparing results in only peripheral visual loss due to sparing of the cilioretinal artery and the macula [4].
  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].

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]
Fever
  • 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]
  • […] causes) include: • Carotid Artery Disease (including Carotid Dissection) • Heart Disease • Hypertension • High Cholesterol • Diabetes • Giant Cell/Temporal Arteritis (Symptoms you should ask about include: headache, jaw or shoulder pain (claudication), fever[optometrystudents.com]
  • Case presentation A previously healthy 8-year-old boy with a fever and cough lasting for 1 week was admitted to the Department of Pediatrics in Jinhua Hospital of Zhejiang University.[bmcpediatr.biomedcentral.com]
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]
Cat Scratch
  • CONCLUSION: Cat scratch disease may cause retinal artery occlusion in infected patients, leaving them with a permanent visual field defect.[ncbi.nlm.nih.gov]
Jaw Claudication
  • 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]
  • Patients who have giant cell arteritis are 55 or older and may have a headache, a tender and palpable temporal artery, jaw claudication, fatigue, or a combination.[msdmanuals.com]
  • MEDS : ASA, Plavix, nitroglycerine PRN and alfalfa pills ROS : denied headaches, jaw claudication, scalp tenderness, weight loss, and loss of appetite EXAM Best corrected visual acuities: 20/30 OD and HM OS. Pupils: greater than 2.5 LU RAPD OS.[eyerounds.org]
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]
Papilledema
  • Single episodes generally do not result in optic atrophy nor in permanent vision loss; however, multiple episodes can result in both. papilledemaPapilledema” is edema or swelling of the optic disc (papilla), most commonly due to an increase in intracranial[tedmontgomery.com]

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.

Dyslipidemia
  • Patients had a combination of systemic comorbidities such as diabetes (5), hypertension (4), dyslipidemia (5), and hyperhomocysteinemia (1).[ncbi.nlm.nih.gov]
  • Infarction of retina Caused by thrombotic or embolic occlusion of retinal arteries Sources of thrombosis are systemic hypertension, dyslipidemia, hypercoagulable states Sources of embolism are atheromas of cervical carotid bifurcation or abnormalities[kellogg.umich.edu]
  • Center) // Edited by: Alex Koyfman, MD (EM Attending Physician, UT Southwestern Medical Center / Parkland Memorial Hospital, @EMHighAK) & Justin Bright, MD (EM Attending, Henry Ford Hospital, @JBright2021) Case: An 82 year-old man with a history of dyslipidemia[emdocs.net]
Bartonella Henselae
  • PURPOSE: To report a case series of six patients suffering from branch retinal artery occlusion due to Bartonella henselae infection, in order to raise awareness to this etiology in the differential diagnosis of retinal artery occlusion.[ncbi.nlm.nih.gov]
Ischemic Changes
  • In all cases, initial OCTA images revealed typical ischemic changes in superficial and deep retinal capillary plexuses. Follow-up OCTA revealed increasing areas of ischemia in the RAO region and persistent narrowing of the arteries.[ncbi.nlm.nih.gov]
  • General Pathology Histopathological changes following BRAO occur due to ischemic changes in the retinal tissue. These ischemic changes may be seen in the corresponding retinal quadrant, depending on which vessel is occluded.[eyewiki.org]

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]

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

  • BACKGROUND AND PURPOSE: RAO is caused by various etiologies and subsequent vascular events may be associated with underlying etiologies.[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]
  • However, few previous clinical or epidemiologic studies have been performed because of the rarity of RAO.[ahajournals.org]
Sex distribution
Age distribution

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

  • Lack of randomization and intrinsic biases prevent any definite conclusions regarding the benefits and further research is warranted.[ncbi.nlm.nih.gov]
  • It is appropriate that they should have more proactive care as well, including blood pressure and lipids to prevent future heart disease.[waterdownoptometric.ca]
  • 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

Article

  1. Graefes AV. Ueber Embolie der Arteria centralis retinae als Ursache plotzlicher Erblindung. Arch Ophthalmol.1859;5:136–157.
  2. 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.
  3. Rumelt S, Brown GC. Update on treatment of retinal arterial occlusions. Curr Opin Ophthalmol. 2003;14:139–141.
  4. Hayreh SS, Zimmerman MB. Central retinal artery occlusion: visual outcome. Am J Ophthalmol. 2005;140:376–391.
  5. Lorentzen S. Incidence of cilioretinal arteries. Acta Ophthalmol. 1970;48:518–524.
  6. Chen CS, Lee AW. Management of acute central retinal artery occlusion. Nat Clin Pract Neurol. 2008;4:376–383.
  7. Rudkin A, Lee A, Chen C. Vascular risk factors for central retinal artery occlusion. Eye. 2009;24:678–681.
  8. 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.
  9. Hayreh S. Acute retinal arterial occlusive disorders. Progr Retin Eye Res. 2011;30:359–394.
  10. Hayreh SS. Prevalent misconceptions about acute retinal vascular occlusive disorders. Progr Retin Eye Res. 2005;24:493–519.
  11. Hayreh S, Kolder H, Weingeist T. Central retinal artery occlusion and retinal tolerance time. Ophthalmology. 1980;87:75.
  12. Pfaffenbach D, Hollenhorst R. Morbidity and survivorship of patients with embolic cholesterol crystals in the ocular fundus. Trans Am Ophthalmol Soc. 1972;70:337.
  13. Hayreh SS, Podhajsky PA, Zimmerman MB. Retinal artery occlusion: associated systemic and ophthalmic abnormalities. Ophthalmology. 2009;116:1928–1936.
  14. Greven CM, Slusher MM, Weaver RG. Retinal arterial occlusions in young adults. Am J Ophthalmol. 1995;120:776–783.

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Last updated: 2019-06-28 10:19