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Table of Contents 1.0 Introduction 2.0 Sudden loss of vision o 2.1 Sudden transient unilateral visual loss o 2.2 Sudden persistent unilateral visual loss o 2.3 Sudden bilateral vision loss 3.0 Progressive visual disturbances 4.0 Scintillations and flashers 5.0 Night blindness 6.0 Day blindness 7.0 'Floaters' - dots, points and lines 8.0 Headaches of extraocular origin with ocular symptoms 9.0 Cataract 10.0 Age related macular degeneration (AMD) o 10.1 Atrophic (Dry) AMD o 10.2 Exudative (wet) AMD o 10.3 Treatment of AMD References 1.0 Introduction This lecture attempts to provide a concise, problem-based approach to disorders that affect vision. Common causes of visual field loss, flashers, floaters and decreased visual acuity will be covered. Primary care management of these problems will be discussed where appropriate. [ top ] 2.0 Sudden loss of vision Patients with a sudden loss of vision usually seek medical aid urgently and the GP is often the first to see the patient in what may be a crucial 'treatment window'. The following section describes some common presentations of sudden visual loss and their implications. Practice Tip! As a general rule, any visual loss that is not clearly neurological needs urgent referral to an opthalmologist. Presentation Possible Causes Sudden Transient Unilateral Visual Loss Amaurosis fugax Giant cell arteritis Glaucoma Ocular Hypoperfusion Retinal migraine Uhtoff's phenomenon Obscuration due to raised ICP Sudden Persistent Unilateral Vision Loss Vitreous haemorrhage Retinal detachment Central retinal vein & artery occlusion Acute Glaucoma Optic neuritis Ischaemic optic neuropathy: Anterior o Giant cell arteritis o Non arteritic· Posterior Optic nerve compression Carcinoma or lymphoma Traumatic cataract - especially children Central serous retinopathy Sudden Bilateral Vision Loss Toxic optic neuropathy Occipital cortex infarction [ top ] 2.1 Sudden transient unilateral visual loss Sudden transient unilateral visual loss is usually apparent at time of onset. However some patients may only realise when they cover their good eye (in this case the visual loss is usually gradual). Golden Rule! All cases presenting with visual symptoms require assessment of visual acuity, visual fields, pupil response to light, anterior chamber depth and fundoscopy including red reflex. Amaurosis fugax: painless, uniocular visual loss lasts < 1 hour described as 'shutter' coming down over 1 eye caused by an embolus passing through the retinal arteries fundus examination - reveals refractive yellow cholesterol crystal in retinal arteries or non-refractive calcific emboli close to optic disc. Glaucoma: intermittent angle-closure glaucoma may cause unilateral vision loss brought on by pupillary dilation, e.g. during low light. Ocular hypoperfusion: present in patients with advanced extracranial vascular disease caused by activities that extract blood from the retinal circulation e.g. walking, standing after bending diagnostic features include:- venous stasis retinopathy (hypotensive retinopathy), with dilated retinal vein, branch occlusions, haemorrhages in the mid periphery and a central retinal artery pulsation stopped by minimal pressure on the globe. Obscurations: unilateral or bilateral visual loss lasting a few seconds occurs in patients with severe, raised intracranial pressure papilloedema (bilateral disc swelling) evident on fundoscopy. Uhtoff's phenomenon: unilateral or bilateral loss of vision occurs concurrently with rise in body temperature, eg after exercise caused by demyelinating optic neuritis. Retinal migraine: rare occurrence mainly in young adults vision loss sudden, uniocular and painless duration ~ 5 minutes thought to be caused by spasm of retinal arteries Other causes: giant cell arteritis (see later section) systemic lupus. [ top ] 2.2 Sudden persistent unilateral visual loss Traumatic cataract: Injury with initially unsuspected globe penetration (eg small sharp metal foreign body) may cause a uveitis and a loss of vision if the lens has been damaged. Be aware of this possibility especially in children with the added issue of developing amblyopia from the cataract. Vitreous haemorrhage fundus obscured due to vitreous haemorrhage. Retinal detachment: patient 1st perceives a dark shadow in peripheral vision if macula becomes involved, visual acuity is lost total detachment may be associated with afferent pupil defect Suspect the possibility of early detachment with the recent onset of flashes and floaters. Consider prompt ophthalmic referral in all cases because of the possibility of an early retinal hole before the retina becomes detached. Cental retinal vein occlusion relatively common fundoscopy reveals dilated, tortuous veins, flamed shaped haemorrhages and soft exudates visual loss occurs when macular is involved. Central retinal artery occlusion painless, sudden loss of vision (visual acuity reduced to light perception only) pupil unreactive to light (afferent pupil defect) fundus near-normal in early stages retinal arteries may appear smaller and macula may have cherry-red spot later stages - retina displays pale, cloudy swelling due to infarction if seen within minutes, emergency treatment involves massaging the globe, intravenous acetazolamide and paracentesis of the globe (urgent, immediate referral for the ophthalmologist to perform this procedure is required). Acute glaucoma: severe pain, haloes around lights, nausea and vomiting globes are hard to touch and very tender acute glaucoma is a medical emergency and the patient should be sent to a hospital with a specialist eye unit immediately. Optic neuritis: visual loss occurs over several days pain on eye movement often described as a "lace curtain" over eye but degree of vision loss varies visual field loss (esp. central scotoma) common fundoscopy may reveal swollen optic disc sometimes including haemorrhages and exudates afferent pupil defect partial recovery between 2-6 weeks after onset. Ischaemic optic neuropathy: sudden painless loss of vision in the elderly fundoscopy reveals pale, swollen optic disc, haemorrhages may be present visual field loss caused by infarction of the optic nerve head important to refer early as may be caused by giant cell arteritis which needs corticosteroid therapy ASAP afferent pupil defect usually present. Optic nerve compression: not usually of sudden onset decreased visual acuity poor colour vision central scotoma pale optic disc a relative afferent pupillary defect is usually present. Carcinomatous or lymphomatous: unilateral or bilateral vision loss may be suspected in patients with known lymphoma, carcinoma or leukaemia. Central serous retinopathy: typically affects younger men impaired central vision with distortion and micropsia macular lesion may be apparent on fundoscopy. [ top ] 2.3 Sudden bilateral vision loss Toxic optic neuropathy: painless bilaterally symmetrical progressive scotomatous field defects colour vision loss can be due to ethanol, methanol, tobacco, ethambutol, quinine. Occipital cortex infarction: presents with bilateral homonymous hemianopia with variable macular sparing patients are cortically blind and hence may be unaware of their visual loss until fully examined. [ top ] 3.0 Progressive visual disturbances Progressive permanent cloudiness of vision. May be due to: cataract vitreous opacities. Mobile opacities May be due to: vitreous floaters retinal detachment. Central scotoma May be due to: macula haemorrhage, macular hole, central serous retinopathy macular degeneration. Peripheral field losses May be due to: inflammation glaucoma intraocular, orbital and brain tumours. [ top ] 4.0 Scintillations and flashers Posterior vitreous detachment is the most common cause of scintillations and flashers. Scintillations may also be associated with fainting and migraines. In migraine, a scotoma associated with flickering sparks and zigzags (which may move through visual fields) may be present. This is followed by headache minutes - hours later. Retinal detachment and choroiditis also cause scintillations. Caution! Patients with acute onset of flashers or floaters should be seen promptly by an ophthalmologist to exclude a retinal tear or detachment. [ top ] 5.0 Night blindness The causes of night blindness include: decreased vitamin A intake (due to malabsorption, gastritis, liver disease etc) advanced glaucoma macular degeneration (diminished light to dark adaptation). [ top ] 6.0 Day blindness Patients complaining of day blindness may have central opacities of the cornea or lens. Albinism and total colour blindness are other causes of day blindness. [ top ] 7.0 'Floaters' - dots, points and lines "Floaters" are caused by vitreous opacities, primarily due to haemorrhages, cellular opacities, parasites and pigment. As the eye moves, these distracting floaters move synchronously, with a slight lag caused by inertia of the vitreous gel. Caution! All complaints of recent or increasing floaters that come to the patient's attention are significant and require ophthalmoscopic examination. Serious retinal traction is likely when floaters are accompanied by 'flashers' (streaks of light). [ top ] 8.0 Headaches of extraocular origin with ocular symptoms Migraine: most significant of the vascular headaches aura and/or scintillating scotoma a frequent prodrome headaches usually hemicranial, above eye, brow, temple and occiput photophobia may occur. Ophthalmoplegic migraine: neurologic deficits (oculomotor nerve paralysis) follow hemicranial migraine paralysis may last for 12-24 hours aneurysms, basilar tumours and arteriosclerosis must be ruled out. Sinusitis: may cause eye pain. Giant cell temporal arteritis: radiating pain from temple temporal arteries are tortuous, nodular, pulseless and painful when pressure is applied pain on mastication, brushing hair, wearing a hat low grade fever, muscle aches and weight loss associated with a clinical picture similar to polymyalgia rheumatica erythrocyte sedimentation rate elevated. treatment - systemic steroids in high doses (80mg prednisone/day for 46 weeks). Trigeminal neuralgia: may have localized eye pain. Brain / Intracranial: The following may all cause pain localised "behind the eye": increased intracranial pressure o headache o vomiting o papilleodema o fixed pupil o unilateral mydriasis meningitis brain tumours. [ top ] 9.0 Cataract Cataracts are opacifications of the lens. Lens opacities appear grey with oblique illumination and black in light reflected from the fundus. Patients present with symptoms such as blurred and unclear vision, monocular diplopia, photophobia and dazzling. Vision may be improved in low light because dilation of the pupil may allow patients to see around the lens opacity. Cataract has highest incidence in patients who: are aged 65+ have a history of ocular trauma have a history of uveitis have diabetes mellitus have genetic diseases such as myotonic dystrophy and galactosaemia have a history of radiation or glucocorticoid therapy. Patients suspected of cataract should be referred to an ophthalmologist for surgical extraction and replacement of the lens. Surgery is extremely successful with very low associated risk. [ top ] 10.0 Age related macular degeneration (AMD) Incidence most common cause of irreversible blindness in people over 65 years 25% of people 65+ and 40% of people 80+ have some form of AMD 90% of all AMD cases are atrophic (dry) and 10% are exudative (wet) exudative AMD produces the most severe visual loss. Risk factors: age genetic influences environmental o smoking o lack of antioxidants in diet o nutritional supplements such as vitamin A, C & E and minerals such as zinc, copper and selenium may reduce risk [ top ] 10.1 Atrophic (Dry) AMD Dry AMD is characterised by gradual cell (rods & cones) death in the retinal pigment epithelium and the choroid. Fundoscopy may reveal drusen (pale spots deep within the retina, as distinct from the pale superficial retinal spots due to exudate in hypertension or diabetic retinopathy), pigment clumping and atrophy around the macula. Dry AMD is occurring in increasing prevalence in Australia and as yet no truly effective preventative measures are available. Treatment of dry AMD is not satisfactory and early detection of a change to the exudative state is of primary importance. (Illustration: Optic drusen around the macula in atrophic age-related macula degeneration. Photo courtesy of Dr Alex Hunyor.) [ top ] 10.2 Exudative (wet) AMD Wet AMD is due to aberrant choroidal angiogenesis. The newly formed vessels in wet AMD are weak and prone to haemorrhage, causing sudden, severe visual loss. Fibrovascular scarring is the end-stage of the disease. (Illustration: Choroidal neovascularisation in exudative age-related macular degeneration. Photo courtesy of Dr Alex Hunyor.) [ top ] 10.3 Treatment of AMD Laser treatment is the mainstay of therapy for exudative AMD. It aims to cauterise new vessels, thus reducing oxidative stress and preventing further neovascularisation. Generally these treatments offer reduced progression of the scotoma from the retinal damage, but little prospect of improvement. New and experimental treatment options include photodynamic therapy, pharmacological therapy and surgical intervention. [ top ] References 1. King, J. 2000, 'Sudden loss of vision: a GP's guide to the many causes', MedicineToday, vol. 1, no. 7, pp. 76-83 2. Reich, J. 2000, 'Sudden loss of vision in an elderly woman', Australian Family Physician, vol. 29, no. 9, pp. 870-871 3. Nuffield Institute for Health, 'Management of Cataract', Effective Health Care, vol. 2, no. 3, pp 1-12 4. Wun, Y.T., Lam, C.C. & Shum, W. K. 1997, 'Impaired vision in the elderly: a preventable condition', Family Practice, vol. 14, no. 4, pp. 289-292 5. Pau, H. 1978, Differential Diagnosis of Eye Diseases, Georg Thieme Publishers, Stuttgart 6. Kanski, J.J., 1989, Clinical Ophthalmology, Butterworth & Co. Ltd, Hong Kong 7. Hunyor, A.P., 2001 Age related macular degeneration and diabetic eye disease. Chatswood Retinal Service.