1.Chronic VisualDisturbance and VisualLossSetareh ZiaiApril 2nd, 2009sziai@ottawahospital.on.ca

2. QUICK review 3. Basic Anatomy 4. Where is the problem?LMCC Objectives Pre-retinal: cornea (dystrophy, scarring, edema) lens (age-related, traumatic, steroid-induced) glaucoma Retinal: DM (diabetic retinopathy, macular edema) vascular insufficiency (arterial or venous occlusion) tumours macular degeneration Post-retinal: anterior to optic chiasm (if optic nerve = monocular) compressive optic neuropathy (intracranial masses, thyroid eye disease) toxic/nutritional (nutritional deficiencies, alcohol/tobacco amblyopia) optic chiasm lesions (pituitary adenoma) 5. Where is the problem? Pre-retinal: cornea (dystrophy, scarring, edema) lens (age-related, traumatic, steroid-induced) glaucoma Retinal: DM (diabetic retinopathy, macular edema) vascular insufficiency (arterial or venous occlusion) tumours macular degeneration Post-retinal: anterior to optic chiasm (if optic nerve = monocular) compressive optic neuropathy (intracranial masses, thyroid eye disease) toxic/nutritional (nutritional deficiencies, alcohol/tobacco amblyopia) optic chiasm lesions (pituitary adenoma) 6. Diagnosis based on:- focused ophthalmological history- monocular vs. binocular- acute vs. chronic- painful vs. painless- exam: start with gross examination- VA- slit lamp biomicroscopy +/- fluorescein- dilated fundus examination- VF testing- fluorescein angiography +/- other tests 7. **Remember for exam: sometimes, chronic visual loss in ONE eye isnoted incidentally some time later due toocclusion of normal eye: CHRONIC LOSSOF VISION CAN PRESENT ACUTELY!! 8. Corneal Causes-dystrophy- scarring - edema 9. The Cornea- allows light to enter theeye- provides most of the eyesoptical power- 0.5-0.8 mm thick- transparent due to itsuniformity, avascularityand deturgescence 10. Epithelium StromaEndothelium 11. Corneal Dystrophies- rare inherited disorders- progressive, usually bilateral- can affect any of the three layers of thecornea- affect transparency- age at presentation: first to fourth decades 12. Corneal Dystrophies- divided into:- anterior dystrophies: - epithelium - may present with recurrent corneal erosions- stromal dystrophies: - usually present with visual loss - if very anterior, can cause erosions and pain- posterior dystrophies: - endothelium - vision loss secondary to edema (endothelial dysfx) 13. Corneal Scarring- multiple causes:- trauma- infectious (eg., herpes)- post-surgical 14. Corneal Edema- most often caused by dysfunction of thecorneal endothelium:- dystrophy- trauma- infectious (eg., herpes)- post-surgical 15. CornealTransplantation 16. If the corneal stroma opacifies due to trauma or infection, or if there is swelling or an irregularity of the surface of the cornea, light cannot properly reach the retina.In some cases, a cornea from adeceased donor can be transplanted. 17. Corneal Transplantation 18. Lens-Related Causes(cataract) - age-related- traumatic - steroid induced 19. The Lens Lens- biconvex, avascular,transparent structure- sits inside a thincapsule, attached to theciliary body by thezonules- provides theremainder of the eyesoptical power (alongwith the cornea) 20. cataracts are due to the opacification of this normally clear structure 21. Age-Related Cataract- often affect the nucleus of the lens first:- yellowing, followed by a browning of the lens- eventually, liquefaction- causes myopic changes (increasedrefractive index of the lens) 22. Traumatic Cataract- most common cause of unilateral cataractin young individuals- most often caused by direct penetratinginjury to the lens- can also be caused by:- concussion- ionizing radiation to ocular tumours- infrared radiation (glassblowers) 23. Steroid-Induced Cataract- both systemic and topical steroids can bethe culprits- posterior part of lens affected first- children may be more susceptible- if lens changes develop, dose should bereduced to the minimum necessary- early opacities may regress withdiscontinuation of therapy 24. Glaucoma 25. Glaucoma disease of the optic nerve, often caused by an increase in intraocular pressure due to poor drainage of aqueous from the trabecular meshwork 26. Glaucoma if left untreated, glaucoma can lead topermanent damage to the optic nerveand resultant visual field loss can progress to blindness 27. Glaucoma by definition, glaucoma is a trimodal disease, characterized by: increased IOP optic nerve changes visual field changes 28. Goldmann Applanation Tonometer 29. Glaucoma classification: primary: open-angle, angle-closure secondary: inflammatory, traumatic,neovascular, steroid-induced etc congenital 30. Risk Factors for Glaucoma age african-american heritage high IOP family history myopia 31. Symptoms of Glaucoma often asymptomatic with late disease, constriction ofperipheral, and later central visual field with very high IOP, can have blurryvision and halos around lights 32. Glaucoma: Optic NerveChanges increased cup:disc ratio thinning of neural rim progressive loss of nerve fiber layer flame hemorrhages on disc 33. Primary Open Angle Glaucoma most common (90%) usually bilateral (can be asymmetric) prevalence increases with age angle is open, eye is quiet increased resistance to aqueous drainage at the level of the trabecular meshwork is thought to be the main pathophysiologic feature 34. Treatment options goal is to stabilize the IOP to protectthe optic nerve against further damage options: drops laser surgery 35. Glaucoma - Medications mechanism of action: decrease aqueous production: beta blockers: timolol alpha agonists: brimonidine carbonic anhydrase inhibitors: diamox increase aqueous outflow: miotics: pilocarpine epinephrine prostaglandin analogs: latanoprost 36. Glaucoma - Lasers usually when medical management fails ALT (argon laser trabeculoplasty), SLT(selective laser trabeculoplasty): for openangle glaucomas peripheral iridotomy: for angle-closureglaucomas high success rate 37. Glaucoma - Surgery usually when medical management and laser treatments fail trabeculectomy: sub-conjunctival shunt ofaqueous drainage devices (valves) cyclodestruction: last resort destructionof ciliary body 38. Where is the problem? Pre-retinal: cornea (dystrophy, scarring, edema) lens (age-related, traumatic, steroid-induced) glaucoma Retinal: DM (diabetic retinopathy, macular edema) vascular insufficiency (arterial or venous occlusion) tumours macular degeneration Post-retinal: anterior to optic chiasm (if optic nerve = monocular) compressive optic neuropathy (intracranial masses, thyroid eye disease) toxic/nutritional (nutritional deficiencies, alcohol/tobacco amblyopia) optic chiasm lesions (pituitary adenoma) 39. THE RETINA- neural tissue liningthe inside of the eye- converts the visualimage into aneurochemicalmessage and sends itto the brain- is made up of 10anatomic layers 40. Diabetes- diabetic retinopathy- diabetic macular edema 41. Diabetic Retinopathy microangiopathy affects pre-capillary arterioles, capillariesand post-capillary venules features of:l microvascular occlusionl leakageclinically, can be divided into:l background DR (nonproliferative)l preproliferative DRl proliferative DR 42. Diabetic Retinopathy: Epidemiology239 million people by 2010doubling in prevalence since 1994 diabetes will affect: 28 million in western Europe18.9 million in North America138.2 million in Asia1.3 million in Australasia #1 cause of blindness in patients 20-64 yrs prevalence increases with duration of diabetes andpatient age rare to find DR in children < 10 yrs, regardless of duration risk of developing DR increases after puberty 43. EpidemiologyWisconsin Epidemiologic Study of Diabetic Retinopathyl Between 1979-1980l 1210 patients with Type 1l 1780 patients with Type 2l predominantly white populationl After 20 yrs, DR present in: 99% of Type 1 60% of Type 2 44. WESDR: Frequency of retinopathy insubjects with type 1diabetes 45. WESDR: Frequency of retinopathy insubjects with type 2 diabetes 46. Diabetic Retinopathy: Risk Factors duration of diabetes: most important riskfactor poor metabolic control pregnancy: can be associated with rapidprogression HTN nephropathy smoking obesity hyperlipidemia 47. Classification of Diabetic Retinopathy Classified into 2 stagesNonproliferative Diabetic Retinopathy (NPDR)early stagealso known as background DR (BDR)further categorized based upon extent of DRl mild, moderate, severe, very severel Proliferative Diabetic Retinopathy (PDR) more advanced stage ***Macular edema May be present at any stage of DR 48. NPDR typically asymptomaticl fluctuating visual acuity: fluctuating blood sugarl decreased visual acuity:CSMEmacular ischemia review these patients annually 49. Mild NPDR 50. Microaneurysm 51. Moderate NPDR 52. Severe NPDR 53. Proliferative Diabetic Retinopathy more likely to becomesymptomatic than earlyNPDR may have decreasedvision, sudden vision loss,floaters, cobwebs, flashes,dull eye ache PDR can also affect visualfunction by affecting themacula with resultingmacular ischemia and/oredema 54. Proliferative DR affects 5-10% of the diabetic population neovascularization is the hallmark l NVD: neovascularization of the disc l NVE: neovascularization elsewhere new vessels are not only extremely fragile(intraretinal or vitreous hemorrhage), butoften associated with fibrous proliferation,leading to an increased risk of tractional retinaldetachment 55. Advanced PDR Tractional retinaldetachmentresulting fromcontraction ofthe fibrovascularproliferativetissue on theretina 56. Panretinal Photocoagulation for High-risk PDR goal is to induceinvolution (or atleast arrest) of newvessels by creatingareas of retinalischemia 1200-3000 burns 4 sessions 57. Vitrectomy for VitreousHemorrhage / TRD 58. Diabetic Macular Edema (DME) retinal edema threatening or involving themaculadiagnosis is made by slit-lamp exam,confirmed by fluorescein angiography and/orOCTimportant observations include: l location of retinal thickening relative to the fovea l presence and location of exudates 59. DME and CSME 60. Treatment of CSME argon laser application intravitreal steroid injection intravitreal anti-VEGF injection pars plana vitrectomy 61. Ophthalmological Follow-Up Diabetic Screeningl Type 1 diabetics: Dilated funduscopic exam (DFE) 5 yrs afterdiagnosis Newly diagnosed patients with Type 1 diabetesrarely have retinopathy during the first 5 yrsl Type 2 diabetics:Type 2 diabetics typically diagnosed yrs after initialonsetDFE at the time of diagnosisSignificant portion of newly diagnosed Type 2diabetics have established DR at the time ofdiagnosis 62. Vascular Insufficiency- arterial occlusions (CRAO, BRAO)- venous occlusions (CRVO, BRVO) 63. CRAO 64. CRAO most of the retina is supplied by thecentral retinal artery (branch of theophthalmic artery, which is the first branchof the ICA) if this supply is interrupted (embolus,thrombosis, inflammation, vasculitis orcompression), the retina becomesischemic irreversible damage occurs afterapproximately 90 minutes 65. CRAO presentation is with sudden andprofound loss of vision RAPD is present orange reflex from the choroid standsout at the fovea, and contrasts with thesurrounding pale retina (cherry-redspot) must r/o temporal arteritis 66. CRAO most commonly the result ofatherosclerosis (thrombosis) but mayalso be caused by calcific emboli often in older patients, with a hx ofarteriosclerosis may have had a hx of amaurosis fugax(transient visual loss) 67. CRAO OPHTHALMOLOGIC EMERGENCY!! treatment: decrease IOP paracentesis ocular massage goal: to send the embolus distally **remember to r/o giant cell arteritis! (ESR, CRP, plt) poor prognosis: 60% < 20/400 68. BRAO 69. BRAO sudden and profound altitudinal orsectoral visual field loss similar causes as CRAO identify and treat associated medicalconditions (HTN, DM,hypercholesterolemia, smoking,vasculitis etc) 70. BRAO retinal cloudiness in ischemic area +/- visible embolusalso has a poor prognosis, unless theobstruction can be dislodged within afew hours 71. CRVO 72. CRVO thrombosis of the central retinal vein sudden loss of vision in affected eye severity of symptoms varies non-ischemic: 75% Ischemic most characteristic finding: retinal hemorrhages 73. CRVO underlying associations advancing age systemic conditions: HTN, DM, smoking,obesity, hyperlipidemia glaucoma inflammatory diseases: sarcoidosis, Behcetdisease thrombophilic disorders:hyperhomocysteinaemia, antiphospholipidantibody syndrome 74. CRVO Treatment: treat associated medical conditions decrease IOP if elevated pan-retinal photocoagulation(laser) if: neovascularization (iris, angle, retina) especially if ischemic CRVO 75. BRVO 76. BRVO thrombosis of a branch of thecentralretinal vein visual loss depends on the amount ofmacular drainage compromised by theocclusion (peripheral occlusions may beasymptomatic) characteristic findings in one sector of theretina: dilatation and tortuosity of veins retinal hemorrhages retinal/macular edema 77. BRVO obstruction often at arterio-venouscrossings: arteries and veins shareadventitial sheath thickening of thearteriole (arteriosclerosis) compressesthe vein, eventually causing an occlusion often associated with: hypertension (75%) diabetes (10%) 78. BRVO prognosis: depends on amt of venousdrainage involved by the occlusion andseverity of macular ischemia: within 6 mos,about 50% of eyes have a VA of 20/30 orbetter main complications: chronic macular edema neovascularization laser photocoagulation may be helpful in above cases 79. Retinal Tumours 80. ocular tumours: ciliary body: melanoma choroid: melanoma hemangioma metastases primary ocular lymphoma retina and optic nerve: retinoblastoma astrocytoma hemangioma 81. Choroidal Melanoma most common primary intraoculartumour in adults presentation usually in 6th decade: asymptomatic vs. visual field defect and/ordecreased visual acuity signs: raised, usually pigmented lesion visible at the back of the eye may be associated with retinal detachment optic nerve may be involved 82. Choroidal Melanoma treatment: consider size, location, activity of tumour,state of fellow eye, general health/age of pt,pts wishes/fears brachytherapy external radiotherapy transpupillary thermotherapy local resection enucleation exenteration palliative (may include chemo) 83. Choroidal Metastases with choroidal melanoma, dont forget general medical investigations! mets TO the choroid: most frequently from bronchus in both sexesand the breast in women, rarely kidney or GI CXR, rectal exam, mammography mets FROM the choroid: liver hepatic u/s, GGT, ALP lungs (rarely affected before liver) CXR 84. Choroidal Metastases usually present with visual impairmentonly IF tumour is near the macula signs: fast-growing, creamy coloured lesion most often in posterior pole usually not very elevated (infiltrates laterally) 85. Choroidal Metastases treatment: observe: if asxic or receiving systemic chemo radiation: external beam or brachy transpupillary thermotherapy systemic therapy for the primary enucleation: for painful blind eye prognosis is poor median survival: 8-12 mos for all pts, 15-17mos for those with breast ca 86. Retinoblastoma most common malignant tumour of the eyein childhood (1:20 000) mean age of presentation: 8 mos ifinherited, 25 mos if sporadic60% present with leukocoria (white pupillaryreflex) strabismus (20%) occasionally: painful, red eye if inherited: often bilateral 87. Retinoblastoma malignant transformation of primitive retinal cells before their final differentiation can be caused by germinal mutations (can be passed on to the next generation), or can be sporadic (66% of cases) 88. Retinoblastoma this is a clinical diagnosis, but CSF andbone marrow should be examined to checkfor metastatic disease if ON involved or ifthere is evidence of extraocular extension rx: small: cryotherapy, photocoagulation medium: brachytherapy, external beam, chemo large/advanced cases: chemoreduction + localtreatment, enucleation metastatic disease: chemo (intrathecal if cellsin CSF) 89. Retinoblastoma prognosis: depends on extent of disease at diagnosis overall mortality ~ 5-15% ~ 50% of children with the germinal mutationwill eventually develop a second primarytumour (eg., osteosarcoma of the femur orpinealoblastoma) 90. Macular Degeneration 91. Macula 1.5 mm in diameter central vision: BEST VISUAL ACUITY colour vision progressive destruction of the macular area: MACULAR DEGENERATION 92. Macular Degeneration most common cause of irreversiblevisual loss in the developed world exists in two forms: non-exudative (dry) maculardegeneration exudative (wet) maculardegeneration 93. Non-exudative Macular Degeneration lipid products arising from photoreceptor outer segments are found under retina can be seen with ophthalmoscope! called drusen 94. Exudative Macular Degeneration new vessels from the choroid grow into the sub-retinal space; form a sub- retinal neovascular membrane subsequent hemorrhage into the sub- retinal space or even through the retina into the vitreous is associated with profound loss of vision 95. Macular Degeneration symptoms: since fovea is responsible for fine visual resolution, any disruption will cause severe visual impairment blurry/reduced vision distorted vision (metamorphopsia) reduction (micropsia) or enlargement(macropsia) of objects VF loss (scotoma) 96. Macular Degeneration rx: non-exudative (usually slowlyprogressive): no actual medical treatment use low vision aids high dose antioxidants MAY be beneficial (eg., vitalux) 97. Macular Degeneration rx: exudative (can be rapidly progressive anddevastating): intravitreal injections of anti-VEGF factors: bevacizumab, ranibizumab photodynamic therapy (injection of photosensitizer into systemic circulation followed immediately by laser targeting new vessels in macular area) combination of above treatments 98. Where is the problem? Pre-retinal: cornea (dystrophy, scarring, edema) lens (age-related, traumatic, steroid-induced) glaucoma Retinal: DM (diabetic retinopathy, macular edema) vascular insufficiency (arterial or venous occlusion) tumours macular degeneration Post-retinal: anterior to optic chiasm (if optic nerve = monocular) compressive optic neuropathy (intracranial masses, thyroid eye disease) toxic/nutritional (nutritional deficiencies, alcohol/tobacco amblyopia) optic chiasm lesions (pituitary adenoma) 99. OPTIC NERVE 1.2 million cells 80 % visual fibres 20 % pupillary fibres carries visualinformation fromthe eye to the brain 100. OPTIC CHIASMcrossover of nasal fibersabove the pituitaryinternal carotids are justlateralfrom optic chiasm: optictract to the lateral geniculate body opticradiation to the primary visual cortex 101. Anterior to OpticChiasm- compressive optic neuropathies- toxic/nutritional optic neuropathies 102. Compressive OpticNeuropathies INTRACRANIAL MASSES: optic nerve glioma typically affects young women, end of first decade associated with NF-1 optic nerve sheath meningioma most frequent in middle-aged women unilateral, gradual visual impairment any other orbital or chiasmal tumour compressing any part of the optic nerve THYROID EYE DISEASE 103. Thyroid Eye Disease may occur in the absence of biochemicalevidence of thyroid dysfx autoimmune reaction (IgG Abs) causing: inflammation of EOMs: pleiomorphic cellularinfiltration associated with increased secretionof GAGs and osmotic imbibition of water muscles can become up to 8 times their original size!! no relation to severity of thyroid dysfx! 104. Thyroid Eye Disease main findings: (not all are always present!) soft tissue involvement lid retraction proptosis optic neuropathy restrictive myopathy 105. Thyroid Eye Disease vision loss from: exposure keratopathy due to severe proptosis resulting in incomplete lidclosure chronically exposed cornea cornealulceration & exposure keratopathy optic neuropathy affects 5% of pts compression of ON or its blood supply bycongested (enlarged) EOMs can lead to severe, permanent visual impairment rx with steroids, surgery if needed 106. Toxic/Nutritional OpticNeuropathies nutritional deficiencies alcohol-tobacco amblyopia 107. Nutritional Deficiencies pts with extremely poor diets, often in associationwith alcohol-tobacco amblyopia usually due to B12 deficiency in combination withcyanide toxicity symmetrical VF loss if early, can be treated with high-dose vitaminsand restoration of well-balanced diet eventually leads to optic atrophy and permanentvision loss 108. Alcohol-Tobacco Amblyopia affects heavy drinkers, cigar and pipe smokers: deficient inprotein and the B vitamins symptoms: insidious, bilateral, progressive visualimpairment + dyschromatopsia signs: symmetrical VF defect, may have pale (or normal)discs rx: 1000 units of hydroxocobalamin qweekly X 10 wks +multivitamins + well-balanced diet px: good in early cases if comply with rx advanced cases: optic atrophy and permanent visual loss 109. Optic Chiasm Lesions - pituitary adenoma 110. Pituitary Adenoma presentation usually in early adult life ormiddle age symptoms: h/a visual symptoms: very gradual onset (oftennot noticed by pt until very well-established) VF defect: usually, bitemporal hemianopia, worst in the superior field, and extending inferiorly colour desaturation across vertical midline optic atrophy: in 50% of cases with field defects caused by pituitary lesions 111. Pituitary Adenoma investigations: MRI: coronal, axial and sagittal sections beforeand after gadolinium injection CT: demonstrates enlargement or erosion ofthe sella endocrinological investigation: PRL, FSH, TSH,GH 112. Pituitary Adenoma treatment options: observation medical: dopamine agonists (bromocriptine) surgery radiotherapy: often used as an adjunct gamma knife stereotactic radiotherapy 113. Visual Field Defects 114. Merci