Ocular ischemic syndrome

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Ocular ischemic syndrome
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Ocular ischemic syndrome is the constellation of ocular signs and symptoms secondary to severe, chronic arterial hypoperfusion to the eye. [1] Amaurosis fugax is a form of acute vision loss caused by reduced blood flow to the eye; it may be a warning sign of an impending stroke, as both stroke and retinal artery occlusion can be caused by thromboembolism due to atherosclerosis elsewhere in the body (such as coronary artery disease and especially carotid atherosclerosis). Consequently, those with transient blurring of vision are advised to urgently seek medical attention for a thorough evaluation of the carotid artery. Anterior segment ischemic syndrome is a similar ischemic condition of anterior segment usually seen in post-surgical cases. Retinal artery occlusion (such as central retinal artery occlusion or branch retinal artery occlusion) leads to rapid death of retinal cells, thereby resulting in severe loss of vision.

Contents

Symptoms and signs

Those with ocular ischemic syndrome are typically between the ages of 50 and 80 (patients over 65); [2] [3] twice as many men as women are affected. [3] More than 90% of those presenting with the condition have vision loss. [1] Patients may report a dull, radiating ache over the eye and eyebrow. [1] Those with ocular ischemic syndrome may also present with a history of other systemic diseases including arterial hypertension, diabetes mellitus, coronary artery disease, previous stroke, and hemodialysis. [4] [5]

The condition presents with visual loss secondary to hypoperfusion of the eye structures. The patient presents with intractable pain or ocular angina. On dilated examination, there may be blot retinal hemorrhages along with dilated and beaded retinal veins. The ocular perfusion pressure is decreased. The corneal layers show edema and striae. There is mild anterior uveitis. A cherry-red spot may be seen in the macula, along with cotton-wool spots elsewhere, due to retinal nerve fiber layer hemorrhages. The retinal arteries may show spontaneous pulsations.[ citation needed ]

Complications

If carotid occlusive disease results in ophthalmic artery occlusion, general ocular ischemia may result in retinal neovascularization, rubeosis iridis, cells and flare, iris necrosis, and cataract. The condition leads to neovascularization in various eye tissues due to the ischemia. The eye pressure may become high due to associated neovascular glaucoma. An ischemic optic neuropathy may eventually occur.

Causes

Severe ipsilateral or bilateral carotid artery stenosis or occlusion is the most common cause of ocular ischemic syndrome. [1] The syndrome has been associated with occlusion of the common carotid artery, internal carotid artery, and less frequently the external carotid artery. [6] Other causes include:

Diagnosis

Differential diagnoses

Treatment

Quick determination of the cause may lead to urgent measures to save the eye and life of the patient. High clinical suspicion should be kept for painless vision loss in patients with atherosclerosis, deep venous thrombosis, atrial fibrillation, pulmonary thromboembolism or other previous embolic episodes. Those caused by a carotid artery embolism or occlusion have the potential for further stroke by detachment of embolus and migration to an end-artery of the brain. [11] Hence, proper steps to prevent such an eventuality need to be taken.[ citation needed ]

Retinal arterial occlusion is an ophthalmic emergency, and prompt treatment is essential. Completely anoxic retina in animal models causes irreversible damage in about 90 minutes. Nonspecific methods to increase blood flow and dislodge emboli include digital massage, 500 mg IV acetazolamide and 100 mg IV methylprednisolone (for possible arteritis). Additional measures include paracentesis of aqueous humor to decrease IOP acutely. An ESR should be drawn to detect possible giant cell arteritis. Improvement can be determined by visual acuity, visual field testing, and by ophthalmoscopic examination.

At a later stage, pan-retinal photocoagulation (PRP) with an argon laser appears effective in reducing the neovascular components and their sequelae.

The visual prognosis for ocular ischemic syndrome varies from usually poor to fair, depending on speed and effectiveness of the intervention. However, prompt diagnosis is crucial as the condition may be a presenting sign of serious cerebrovascular and ischemic heart diseases. [5]

In 2009, the Undersea and Hyperbaric Medical Society added "central retinal artery occlusion" to their list of approved indications for hyperbaric oxygen (HBO). [12] [13] When used as an adjunctive therapy, the edema reducing properties of HBO, along with down regulation of inflammatory cytokines may contribute to the improvement in vision. [14] Prevention of vision loss requires that certain conditions be met: the treatment be started before irreversible damage has occurred (over 24 hours), the occlusion must not also occur at the ophthalmic artery, and treatment must continue until the inner layers of the retina are again oxygenated by the retinal arteries. [15]

Related Research Articles

<span class="mw-page-title-main">Retinopathy</span> Medical condition

Retinopathy is any damage to the retina of the eyes, which may cause vision impairment. Retinopathy often refers to retinal vascular disease, or damage to the retina caused by abnormal blood flow. Age-related macular degeneration is technically included under the umbrella term retinopathy but is often discussed as a separate entity. Retinopathy, or retinal vascular disease, can be broadly categorized into proliferative and non-proliferative types. Frequently, retinopathy is an ocular manifestation of systemic disease as seen in diabetes or hypertension. Diabetes is the most common cause of retinopathy in the U.S. as of 2008. Diabetic retinopathy is the leading cause of blindness in working-aged people. It accounts for about 5% of blindness worldwide and is designated a priority eye disease by the World Health Organization.

The National Eye Institute (NEI) is part of the U.S. National Institutes of Health (NIH), an agency of the U.S. Department of Health and Human Services. The mission of NEI is "to eliminate vision loss and improve quality of life through vision research." NEI consists of two major branches for research: an extramural branch that funds studies outside NIH and an intramural branch that funds research on the NIH campus in Bethesda, Maryland. Most of the NEI budget funds extramural research.

<span class="mw-page-title-main">Amaurosis fugax</span> Medical condition

Amaurosis fugax is a painless temporary loss of vision in one or both eyes.

<span class="mw-page-title-main">Electroretinography</span>

Electroretinography measures the electrical responses of various cell types in the retina, including the photoreceptors, inner retinal cells, and the ganglion cells. Electrodes are placed on the surface of the cornea or on the skin beneath the eye to measure retinal responses. Retinal pigment epithelium (RPE) responses are measured with an EOG test with skin-contact electrodes placed near the canthi. During a recording, the patient's eyes are exposed to standardized stimuli and the resulting signal is displayed showing the time course of the signal's amplitude (voltage). Signals are very small, and typically are measured in microvolts or nanovolts. The ERG is composed of electrical potentials contributed by different cell types within the retina, and the stimulus conditions can elicit stronger response from certain components.

Anterior ischemic optic neuropathy (AION) is a medical condition involving loss of vision caused by damage to the optic nerve as a result of insufficient blood supply (ischemia). This form of ischemic optic neuropathy is generally categorized as two types: arteritic AION, in which the loss of vision is the result of an inflammatory disease of arteries in the head called temporal arteritis, and non-arteritic AION, which is due to non-inflammatory disease of small blood vessels.

Posterior ischemic optic neuropathy (PION) is a medical condition characterized by damage to the retrobulbar portion of the optic nerve due to inadequate blood flow (ischemia) to the optic nerve. Despite the term posterior, this form of damage to the eye's optic nerve due to poor blood flow also includes cases where the cause of inadequate blood flow to the nerve is anterior, as the condition describes a particular mechanism of visual loss as much as the location of damage in the optic nerve. In contrast, anterior ischemic optic neuropathy (AION) is distinguished from PION by the fact that AION occurs spontaneously and on one side in affected individuals with predisposing anatomic or cardiovascular risk factors.

<span class="mw-page-title-main">Ophthalmic artery</span> Artery of the head

The ophthalmic artery (OA) is an artery of the head. It is the first branch of the internal carotid artery distal to the cavernous sinus. Branches of the ophthalmic artery supply all the structures in the orbit around the eye, as well as some structures in the nose, face, and meninges. Occlusion of the ophthalmic artery or its branches can produce sight-threatening conditions.

Neovascularization is the natural formation of new blood vessels, usually in the form of functional microvascular networks, capable of perfusion by red blood cells, that form to serve as collateral circulation in response to local poor perfusion or ischemia.

<span class="mw-page-title-main">Hollenhorst plaque</span> Medical condition

A Hollenhorst plaque is a cholesterol embolus that is seen in a blood vessel of the retina. It is usually found when a physician performs ophthalmoscopy, during which a plaque will appear as a small, bright crystal that is refractile and yellow. This is a medical exam finding, and is not a medical condition, though it may be related to cardiovascular conditions such as atherosclerosis of the internal carotid artery. It was first described by American ophthalmologist Robert Hollenhorst in 1961.

Eales disease is a type of obliterative vasculopathy, also known as angiopathia retinae juvenilis, periphlebitis retinae or primary perivasculitis of the retina. It was first described by the British ophthalmologist Henry Eales (1852–1913) in 1880 and is a rare ocular disease characterized by inflammation and possible blockage of retinal blood vessels, abnormal growth of new blood vessels (neovascularization), and recurrent retinal and vitreal hemorrhages.

Optic neuropathy is damage to the optic nerve from any cause. The optic nerve is a bundle of millions of fibers in the retina that sends visual signals to the brain. [1].

<span class="mw-page-title-main">Cotton wool spots</span> Medical condition of the eye

Cotton wool spots are opaque fluffy white patches on the retina of the eye that are considered an abnormal finding during a funduscopic exam. Cotton wool spots are typically a sign of another disease state, most common of which is diabetic retinopathy. The irregularly shaped white patches are a result of ischemia, or reduced blood flow and oxygen, in the retinal nerve fiber layer, which is located in the distribution of the capillaries of the superficial layer of the retina. These areas with reduced blood flow reflect the obstruction of axoplasmic flow due to mechanical or vascular causes and the consequential accumulation as a result of decreased axonal transport. This reduced axonal transport can then cause swelling or bulging on the surface layer of the retina, increasing the potential for nerve fiber damage.

Central retinal artery occlusion (CRAO) is a disease of the eye where the flow of blood through the central retinal artery is blocked (occluded). There are several different causes of this occlusion; the most common is carotid artery atherosclerosis.

<span class="mw-page-title-main">Blurred vision</span> Medical condition

Blurred vision is an ocular symptom where vision becomes less precise and there is added difficulty to resolve fine details.

<span class="mw-page-title-main">Central retinal vein occlusion</span> Medical condition

Central retinal vein occlusion, also CRVO, is when the central retinal vein becomes occluded, usually through thrombosis. The central retinal vein is the venous equivalent of the central retinal artery and both may become occluded. Since the central retinal artery and vein are the sole source of blood supply and drainage for the retina, such occlusion can lead to severe damage to the retina and blindness, due to ischemia and edema (swelling).

<span class="mw-page-title-main">Persistent fetal vasculature</span> Medical condition

Persistent fetal vasculature(PFV), also known as persistent fetal vasculature syndrome (PFVS), and until 1997 known primarily as persistent hyperplastic primary vitreous (PHPV), is a rare congenital anomaly which occurs when blood vessels within the developing eye, known as the embryonic hyaloid vasculature network, fail to regress as they normally would in-utero after the eye is fully developed. Defects which arise from this lack of vascular regression are diverse; as a result, the presentation, symptoms, and prognosis of affected patients vary widely, ranging from clinical insignificance to irreversible blindness. The underlying structural causes of PFV are considered to be relatively common, and the vast majority of cases do not warrant additional intervention. When symptoms do manifest, however, they are often significant, causing detrimental and irreversible visual impairment. Persistent fetal vasculature heightens the lifelong risk of glaucoma, cataracts, intraocular hemorrhages, and Retinal detachments, accounting for the visual loss of nearly 5% of the blind community in the developed world. In diagnosed cases of PFV, approximately 90% of patients with a unilateral disease have associated poor vision in the affected eye.

<span class="mw-page-title-main">Retinal vasculitis</span> Medical condition

Retinal vasculitis is inflammation of the vascular branches of the retinal artery, caused either by primary ocular disease processes, or as a specific presentation of any systemic form of vasculitis such as Behçet's disease, sarcoidosis, multiple sclerosis, or any form of systemic necrotizing vasculitis such as temporal arteritis, polyarteritis nodosa, and granulomatosis with polyangiitis, or due to lupus erythematosus, or rheumatoid arthritis. Eales disease, pars planitis, birdshot retinochoroidopathy, and Fuchs heterochromic iridocyclitis (FHI) can also cause retinal vasculitis. Infectious pathogens such as Mycobacterium tuberculosis, visceral larva migrans can also cause retinal vasculitis. Drug-induced vasculitis may involve retina as well, as seen in methamphetamine induced vasculitis.

Sickle cell retinopathy can be defined as retinal changes due to blood vessel damage in the eye of a person with a background of sickle cell disease. It can likely progress to loss of vision in late stages due to vitreous hemorrhage or retinal detachment. Sickle cell disease is a structural red blood cell disorder leading to consequences in multiple systems. It is characterized by chronic red blood cell destruction, vascular injury, and tissue ischemia causing damage to the brain, eyes, heart, lungs, kidneys, spleen, and musculoskeletal system.

Drug abuse retinopathy is damage to the retina of the eyes caused by chronic drug abuse. Types of retinopathy caused by drug abuse include maculopathy, Saturday night retinopathy, and talc retinopathy. Common symptoms include temporary and permanent vision loss, blurred vision, and night blindness. Substances commonly associated with this condition include poppers, heroin, cocaine, methamphetamine, tobacco, and alcohol.

References

  1. 1 2 3 4 De Graeve C, Van de Sompel W, Claes C (1999). "Ocular ischemic syndrome: two case reports of bilateral involvement". Bull Soc Belge Ophtalmol. 273: 69–74. PMID   10546383.
  2. ↑ Friedberg MA, Rapuano CJ. The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease, J.B. Lippincott, 1994.
  3. 1 2 "Ocular Ischemic Syndrome." Archived 2009-09-27 at the Wayback Machine Accessed October 25, 2006.
  4. ↑ Chen KJ, Chen SN, Kao LY, Ho CL, Chen TL, Lai CC, Wu SC (August 2001). "Ocular ischemic syndrome". Chang Gung Medical Journal. 24 (8): 483–91. PMID   11601190.
  5. 1 2 Mizener JB, Podhajsky P, Hayreh SS (May 1997). "Ocular ischemic syndrome". Ophthalmology. 104 (5): 859–64. doi:10.1016/s0161-6420(97)30221-8. PMID   9160035.
  6. ↑ Alizai AM, Trobe JD, Thompson BG, Izer JD, Cornblath WT, Deveikis JP (December 2005). "Ocular ischemic syndrome after occlusion of both external carotid arteries". Journal of Neuro-Ophthalmology. 25 (4): 268–72. doi: 10.1097/01.wno.0000189831.92504.2d . PMID   16340491.
  7. ↑ Koz OG, Ates A, Numan Alp M, Gultan E, Karaaslan Y, Kural G (January 2007). "Bilateral ocular ischemic syndrome as an initial manifestation of Takayasu's arteritis associated with carotid steal syndrome". Rheumatology International. 27 (3): 299–302. doi:10.1007/s00296-006-0194-4. PMID   16944156.
  8. ↑ Hamed LM, Guy JR, Moster ML, Bosley T (June 1992). "Giant cell arteritis in the ocular ischemic syndrome". American Journal of Ophthalmology. 113 (6): 702–5. doi:10.1016/s0002-9394(14)74798-1. PMID   1598963.
  9. ↑ Munch IC, Larsen M (Aug 2005). "[The ocular ischemic syndrome.]". Ugeskrift for Lægerer. 167 (35): 3269–73. PMID   16138965.
  10. ↑ Bigou MA, Bettembourg O, Hebert T, Cochener B (Jan 2006). "[Unilateral ocular ischemic syndrome in a diabetic patient.]". J Fr Ophtalmol. 29 (1): e2. doi:10.1016/s0181-5512(06)73751-2. PMID   16465117.
  11. ↑ Kaiboriboon K, Piriyawat P, Selhorst JB (May 2001). "Light-induced amaurosis fugax". American Journal of Ophthalmology. 131 (5): 674–6. doi:10.1016/S0002-9394(00)00874-6. PMID   11336956.
  12. ↑ The Undersea and Hyperbaric Medical Society (UHMS), Hyperbaric Oxygen Therapy Committee. Guidelines: Indications for Hyperbaric Oxygen. Durham, NC: UHMS; 2009.
  13. ↑ Butler FK, Hagan C, Murphy-Lavoie H (2008). "Hyperbaric oxygen therapy and the eye". Undersea & Hyperbaric Medicine. 35 (5): 333–87. PMID   19024664. Archived from the original on January 13, 2013. Retrieved 2010-11-09.{{cite journal}}: CS1 maint: unfit URL (link)
  14. ↑ Wright JK, Franklin B, Zant E (2007). "Clinical case report: treatment of a central retinal vein occlusion with hyperbaric oxygen". Undersea and Hyperbaric Medicine. 34 (5): 315–9. PMID   18019081. Archived from the original on January 13, 2013. Retrieved 2009-05-26.{{cite journal}}: CS1 maint: unfit URL (link)
  15. ↑ Butler Jr, FK (2010). "Hyperbaric oxygen for central retinal artery occlusion". Wound Care & Hyperbaric Medicine. 1 (3): 25.
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