Optic neuritis is an inflammation of the optic nerve, often affecting young
adults in one eye. Optic neuritis is frequently associated with multiple sclerosis. Other causes include infections, autoimmune disease, and injury to the optic nerve. The symptoms of optic neuritis include vision loss, reduced color vision, and pain on movement of the eye with recovery over weeks to a month, in most cases. The diagnosis is made on the basis of the patient history and an examination by an ophthalmologist. An MRI is important to look for lesions that indicate the patient may develop multiple sclerosis. Blood tests may be indicated. High-dose IV corticosteroids speed up recovery, but appear to have no effect on the long-term outcome. What is optic neuritis? Comment on thisRead 4 CommentsShare Your Story Optic neuritis is an inflammation that affects the myelin lining of the optic nerve, which transmits visual stimuli to the brain. The optic nerve is actually a nerve tract of axons that originate in the ganglioncells of the retina. Nerve tracts are the information pathways in the brain. The "optic nerves" are the only nerve tracts not located entirely within the brain. The optic nerves carry visual information from the retina to the brain stem, where the information is relayed to the area of the brain that recognizes vision (the occipital cortex). Optic neuritis can occur in children or adults and may involve either one or both optic nerves. Optic neuritis typically affects young adults ranging from 20 to 40 years of age. There is a strong female predominance. The annual incidence is approximately 6.4/100,000. What causes optic neuritis? The precise cause of optic neuritis is unknown, but it is thought to be a type of autoimmune disorder. The immune system is generally used to fight infection by creating a reaction that combats bacteria, viruses, fungi, and other foreign proteins. In autoimmune diseases, this reaction is mistakenly directed against a normal part of the body, creating inflammation and potential damage. In the case of optic neuritis, the optic nerve becomes swollen and its function is impaired. Inflammation and destruction of the protective myelin sheath that coats and insulates the optic nerve, plus direct damage to the nerve axons results in loss of vision. Optic neuritis is frequently an indication of multiple sclerosis (MS), a disease in which the immune system attacks the myelin sheath covering nerve fibers in the brain and spinal cord, resulting in inflammation and nerve damage. Optic neuritis typically resolves initially, but recurs. In 15%-20% of people who eventually develop multiple sclerosis, optic neuritis is their first symptom. The risk of developing multiple sclerosis following one episode of optic neuritis is approximately 50% within 15 years of the episode of optic neuritis. Studies vary, but between 27% and 70% of patients who have optic neuritis for the first time will have 2 or more MRI abnormalities consistent with MS. The 5-year risk of developing multiple sclerosis is 16% if the MRI is negative, 51% if there are 1 to 3 lesions. Neuromyelitis optica is another autoimmune condition in which demyelination mainly occurs in the spinal cord and optic nerve, but is less noticeable in the brain. In this infrequent condition, the patient can experience weakness orparalysis in the limbs and/or bladder and bowel dysfunction, as well as loss of vision. There are a variety of conditions that can affect the optic nerve causing symptoms similar to optic neuritis (optic neuropathies). Infection, trauma, hereditary conditions, toxic or nutritional problems, compressive lesions, arteritis, diabetes, glaucoma, etc. Treatment of optic neuropathies associated with these patients depends on the cause, and is directed at the underlying disease. What are symptoms of optic neuritis? The major symptom of optic neuritis is vision loss, usually in one eye, often developing within hours to a couple of days and peaking in 1 to 2 weeks. It may vary from a small area of blurring to complete blindness. Affected individuals may also notice distorted vision, reduced color vision, loss of contrast, and washed- out or less vivid vision than normal. Symptoms may be worsened by heat or exercise. Vision loss is usually temporary, but it may be permanent in some cases. Most people who develop optic neuritis experience eye pain that is worsened by eye movement. The intensity of the pain usually follows the course of the vision loss. Because optic neuritis usually affects one eye, patients may be unaware of subtle visual loss or changes in the color vision until they or doctors close or cover the healthy eye. What are signs of optic neuritis? The most characteristic findings on examination include reduced visual acuity (typically 20/25 to 20/190), a measurable change in peripheral vision, decreased perception of brightness in the affected eye, and loss of color vision out of proportion to the loss of visual acuity. A disturbance in reaction of the pupil (afferent pupillary defect or APD) is usually detectable if the other eye is either unaffected or involved to a lesser degree. The optic nerve can easily be visualized with an ophthalmoscope. In one-third of the cases, there is visible swelling of the optic nerve, and there may be enlargement of the blood vessels around the nerve. This condition is called papillitis. In about two-thirds of patients, inflammation is entirely retrobulbar, causing no visible changes when the physician examines the optic nerve with an ophthalmoscope. This is called retrobulbar neuritis. How is optic neuritis diagnosed? Optic neuritis is suspected based on the characteristic history of eye pain and vision loss. The standard exam includes visual acuity, pupillary reduction, visual field evaluation, color vision testing, and visualization of the optic disc by direct and indirect ophthalmoscopy. A person experiencing a first episode of optic neuritis should undergo an MRI of the brain to look for the central nervous system lesions associated with MS. The MRI may also show an enlarged optic nerve. Visual symptoms usually progress for the first couple weeks, and then start to improve within the first month. If the course of recovery is not typical, then there are a number of blood tests like the sed rate, thyroid function, antinuclear antibodies, etc. that can be performed to look for other causes of optic neuritis/neuropathy. What is the treatment for optic neuritis? Comment on thisRead 17 CommentsShare Your Story If a definite cause (such as infection or underlying other disease) is determined, appropriate therapy for that cause can be instituted. The Optic Neuritis Treatment Trial, a multicenter randomized trial with 15 years of follow-up, showed that oral steroid (prednisone) alone, had no benefit on recovery to normal visual acuity. High-dose intravenous steroids, which involve some risks and can have significant side effects in some patients - e.g. elevated blood sugar, depression, insomnia, gastrointestinal symptoms, etc. - did speed up the initial recovery of vision in the acute phase. However, there was no conclusive evidence that oral or intravenous steroids had any long-term benefit on visual acuity, visual field or contrast sensitivity five years later. What is the prognosis for optic neuritis? The prognosis depends on the underlying cause. Most episodes resolve spontaneously, with return of vision in two weeks to three months. About 90% of people with optic neuritis will recover most of their vision within six months of onset. However, about 14% will have a recurrence of optic neuritis in the affected eye, and 12% will develop optic neuritis in the other eye within 10 years. If the patient has one or more abnormal lesions on the MRI, the risk of MS within 15 years is 72%.
Andrew A. Dahl, MD, FACS . Optic Neuritis. http://www.medicinenet.com/optic_neuritis/page5.htm
RETINAL VEIN OCCLUSION Retinal vein occlusion (RVO) is a common vascular disorder of the retina and is one of the most common causes of blindness after diabetic retinopathy. RVO has been recognized as an entity since 1855 and has been noted in numerous publications. However, the pathogenesis and management of this disorder remains somewhat of an enigma. An article in the Canadian Journal of Ophthalmology2007 noted "Research into CRVO is fraught with challenges, from accurate disease classification to its treatment; even the most prestigious trials have become controversial." [1]
RVO is classified according to where the obstruction is located. Obstruction of the retinal vein at the optic nerve is referred to as central retinal vein occlusion(CRVO), and obstruction at a branch of the retinal vein is referred to as branch retinal vein occlusion (BRVO). The two forms have both differences and similarities in pathogenesis and clinical presentation. RVO is commonly subdivided into nonischemic and ischemic types. Such a distinction is relevant to the clinician, since two thirds of patients with the ischemic type develop the dreaded complications of macular edema, macular ischemia, and neovascularization that lead to blindness. Most investigators accept that these two entities represent varying degrees of the same underlying disease process. Yet, other clinicians and researchers argue that ischemic and nonischemic types are distinct clinical entities. This controversy is predominantly the purview of the ophthalmology consultant. The purview of the emergency physician should center on emergency department (ED) diagnosis and recognition of the clinical scenario, so that prompt ophthalmologic evaluation and management can commence at the time of the ED visit.
PATOPHISIOLOGY Visual morbidity and blindness in retinal vein occlusion (RVO) are due to macular edema, macular ischemia, and neovascular glaucoma. The precise pathologic event in RVO is intraluminal thrombus formation, which can be associated with the abnormalities of blood flow, its constituents, and vessels consistent with the Virchow triad. CRVO has been likened to a neurovascular compartment syndrome at the site of the lamina cribrosa or closure of the final retinal vein located at the optic nerve. The nonischemic type of CRVO is thought to be the milder clinical entity. Seventy-five to eighty percent of patients present with this form. Neovascularization is rare, at less that 2% incidence. Conversion to the ischemic type is common. However, the ischemic type is associated with marked decreased vision. This entity affects 20-25% of patients. Neovascular glaucoma is typical in the latter. In both types, blockage of the retinal vein occurs, but the nonischemic type is able to maintain better relative blood flow to the retina through collaterals, preventing the dreaded complications known of the ischemic type. The ischemic type of CRVO predisposes to anterior neovascularization called rubeosis irides. With this, high- pressure neovascular glaucoma develops. Neovascularization in the back of the eye can lead to vitreous hemorrhage and retinal detachments. RVO is essentially a blockage of a portion of the venous circulation that drains the retina. With blockage, a pressure build up occurs in the capillaries, leading to hemorrhages and leakage of fluid and blood. This can lead to macular edema with leakage near the macula. Macular ischemia occurs when these capillaries, which supply oxygen to the retina, manifest leakage and nonperfusion. Neovascularization is the most devastating pathologic complication with the development of abnormal blood vessel growth. Histologically, CRVO is found to have fresh recanalized thrombus at, or just posterior to, the lamina cribrosa. The thrombi have a mild lymphocytic infiltration with prominent endothelial cells noted. Loss of retinal layers are noted and consistent with retinal ischemia. BRVO is noted for a histopathologic picture of arteriolar disease as the underlying pathology. BRVO occurs almost always at arteriovenous crossing, and it is thought that a arteriosclerotic artery compresses the retinal vein at a branch point leading to turbulent flow, endothelial damage, and vein thrombosis and obstruction.
CLINICAL PRESENTATION HISTORY The evaluation of patients with unilateral visual loss should include a pertinent and thorough history of present and past medical history. Questions pertinent to the present medical history should include an inquiry into the rate of onset of visual loss, possible trauma, unilateral or bilateral in character, and if redness is present or not. Central retinal vein occlusion (CRVO) is essentially a diagnostic finding of painless unilateral loss of vision. In some cases, this loss of vision is subtle in character, with intermittent episodes of blurred vision. In other cases, it may be sudden and dramatic. The nonischemic type is often the more subtle of the two, while the ischemic type is prone to the more acute clinical presentations. Nonischemic CRVO - Subtle, intermittent visual loss; painless; mild-to-moderate visual loss Ischemic CRVO - Acute visual loss; pain may be present; marked visual loss BRVO is similar in presentation to CRVO. BRVO is often noted with an onset of blurred vision or visual field defect. Vision loss may be subtle. Patients with small occlusions of a branch retinal vein may often be asymptomatic. Larger obstructions can lead to significant visual loss. It is uniformly a unilateral disease. Nine percent of cases are bilateral.
PHYSICAL The performance of a pertinent physical examination and mandatory evaluation for visual acuity and visual field testing is prudent. An ophthalmoscopic examination is diagnostic. An article in the American Journal of Ophthalmology 2007, notes that the finding of an afferent pupillary defect, in ischemic CRVO, is of high diagnostic precision. [4]
Nonischemic central retinal vein occlusion Mild vision loss, usually better than 20/120 measured Rare afferent pupillary defect Ophthalmoscopy findings consist of variable dot and flame hemorrhages in all 4 quadrants, optic nerve swelling, retinal vein engorgement and tortuosity, cotton wool spots are few Ischemic central retinal vein occlusion Marked visual loss, usually 20/200 to only hand motion Afferent pupillary defect Ophthalmoscopy findings of extensive retinal hemorrhages in all 4 quadrants, optic disc is edematous, retinal vein markedly edematous and engorged Macular edema is often severe. Bleeding may result in vitreous hemorrhage. Retinal detachment may occur. Branch retinal vein occlusion Patients with BRVO have retinal hemorrhages confined to the distribution of the retinal vein. The ophthalmoscopic examination may note triangular and flame-shaped hemorrhages. Mild obstruction of a branch may only show scant hemorrhage. Complete obstruction may have extensive hemorrhage noted on examination, with cotton wool spots.
CAUSES Local disease processes include the following: trauma, glaucoma (history of glaucoma is 5 times more likely to have CRVO), and orbital structural lesions. Rarely, is local ocular disease seen in BRVO. When it is apparent in BRVO, one can consider toxoplasmosis, Behet syndrome, ocular sarcoidosis, and macroaneurysms. Systemic disease processes include the following: hypertension, atherosclerosis, diabetes, glaucoma, elderly, fasting, hypercholesterolemia, hyperhomocysteinemia, SLE, sarcoidosis, tuberculosis, syphilis, protein C resistance (factor V Leiden), protein C and S deficiency, antiphospholipid antibody disease, multiple myeloma, cryoglobulinemia, leukemia, lymphoma, Waldenstrom macroglobulinemia, polycythemia vera, and sickle cell disease. In CRVO, a positive association has been found in ACE inhibitor use with atrial fibrillation. A counterintuitive finding was noted in a small study in 2007, where warfarin and aspirin use was linked with a surprising propensity to develop CRVO. A negative association can be found with the use of estrogen in postmenopausal women. BRVO has a strong association with hypertension.
WORK UP LABORATORY Extensive laboratory testing for retinal vein occlusion is usually not indicated. The yield from general laboratory tests for central retinal vein occlusion (CRVO) is so low that many authorities do not advocate these simple blood tests, as well as, extensive thrombophilia workups. Such laboratory workup may be prudent in unusual case presentations or in bilateral CRVO, where systemic causes are more prominent etiological factors.
IMAGING Fluorescein angiogram of the retinal vein is advocated by some clinicians and investigators.
OTHER TESTS Focus other testing on specific ophthalmic evaluation. Visual acuity is mandatory in virtually all instances. Initially consider at least a gross evaluation of visual fields. Formal testing can follow at a later point. Tonometry is useful.
TREATMENT EMERGENCY The cornerstone of emergency department (ED) treatment for retinal vein occlusion may rest with the awareness of the disease entity by the ED physician. When considered in the proper clinical scenario, expeditious consultation by an ophthalmologist in the ED, can enable definitive diagnosis and so that appropriate intervention and follow-up care is ensured. Dexamethasone intravitreal implant (Ozurdex) is FDA-approved and indicated for treatment of macular edema following branch retinal vein occlusion or central retinal vein occlusion (see Medications). Treatment remains controversial at the present time. The Canadian Journal of Ophthalmology 2008 noted "No intervention has emerged as the standard of care. Current management in most centers is close observation for complications and treatment as they arise." [5] Another 2008 study notes that many different interventions have been advocated, but evidence is lacking as to their merit. Several randomized clinical trials are underway at the present time. [6]
Current literature and practice for the treatment of CRVO remains controversial. Vitreous Retina Macula Consultants of New York, a large and well-established retina practice group in New York City, advocates laser photocoagulation for managing rubeosis irides. [7] For the treatment of macular edema, they promote intraocular triamcinolone or bevacizumab (Avastin) in CRVO. In a small prospective, multicenter randomized, double-masked, placebo-controlled trial, intraocular injection of ranibizumab (0.5 mg) resulted in rapid improvement in 6-month visual acuity and macular edema, with low rates of ocular or nonocular safety events. Improvement required repeated injections. Further long-term study and follow up is needed. [8]
In one pilot study, simultaneous intravitreal injection of triamcinolone/tPA effectively reduced macular edema and improved the best corrected visual acuity (BCVA) in patients with CRVO without serious complications, although repeated treatments were required in 75%. [9]
Dalteparin was found superior to aspirin in improving visual acuity and preventing neovascularization in CRVO in a 2008 study. [10] Radial optic neurotomy (RON) has shown good results in improving visual loss in severe CRVO in a limited 2008 study. [5] Of note, a single case study in 2008 noted the use of hyperbaric oxygen, and subsequent visual improvement in a patient who refused other intervention. [11]
Current literature and practice for the treatment of BRVO is controversial as well. Vitreous Retina Macula Consultants of New York note that there is "no known medical treatment" for branch retinal vein occlusion. [7] A small study in 2008 noted intravitreal triamcinolone and grid laser photocoagulation effective in the treatment of BRVO. [12] In another 2008 study, triamcinolone acetonide retrobulbar injection was found effective for BRVO. [13] Pars plana vitrectomy was effective when the macular edema was resistant to improvement with triamcinolone alone.
MEDICATION In addition to corticosteroids, the drug bevacizumab (Avastin) is a monoclonal antibody Food and Drug Administration (FDA) approved in colon and breast cancer. Its use is experimental for macular edema in CRVO. CORTICOSTEROID These agents have anti-inflammatory properties and cause profound and varied metabolic effects. In addition, these agents modify the body's immune response to diverse stimuli. View full drug information Triamcinolone (Amcort)
Useful in treatment of inflammatory and autoimmune reactions. By reversing increased capillary permeability and suppressing PMN activity, it may decrease inflammation. View full drug information Dexamethasone intravitreal implant (Ozurdex)
Corticosteroids suppress inflammation by inhibiting multiple inflammatory cytokines, resulting in decreased edema, fibrin deposition, capillary leakage, and migration of inflammatory cells. Indicated for treatment of macular edema following branch retinal vein occlusion or central retinal vein occlusion.