Chronic Visual Chronic Visual Disturbance and Visual Disturbance and Visual

LossLossSetareh Ziai Setareh Ziai April 2April 2ndnd, 2009, 2009

sziai@ottawahospital.on.casziai@ottawahospital.on.ca

QUICKQUICK review review

Basic AnatomyBasic Anatomy

Where is the problem? Where is the problem? LMCC ObjectivesLMCC Objectives

Pre-retinal: Pre-retinal: cornea (dystrophy, scarring, edema)cornea (dystrophy, scarring, edema) lens (age-related, traumatic, steroid-induced)lens (age-related, traumatic, steroid-induced) glaucoma glaucoma

Retinal:Retinal: DM (diabetic retinopathy, macular edema)DM (diabetic retinopathy, macular edema) vascular insufficiency (arterial or venous occlusion)vascular insufficiency (arterial or venous occlusion) tumourstumours macular degeneration macular degeneration

Post-retinal:Post-retinal: anterior to optic chiasm (if optic nerve = monocular)anterior to optic chiasm (if optic nerve = monocular)

• compressive optic neuropathy (intracranial masses, thyroid eye disease)compressive optic neuropathy (intracranial masses, thyroid eye disease)• toxic/nutritional (nutritional deficiencies, alcohol/tobacco amblyopia)toxic/nutritional (nutritional deficiencies, alcohol/tobacco amblyopia)

optic chiasm lesions (pituitary adenoma)optic chiasm lesions (pituitary adenoma)

Where is the problem?Where is the problem? Pre-retinal: Pre-retinal:

cornea (dystrophy, scarring, edema)cornea (dystrophy, scarring, edema) lens (age-related, traumatic, steroid-induced)lens (age-related, traumatic, steroid-induced) glaucoma glaucoma

Retinal:Retinal: DM (diabetic retinopathy, macular edema)DM (diabetic retinopathy, macular edema) vascular insufficiency (arterial or venous occlusion)vascular insufficiency (arterial or venous occlusion) tumourstumours macular degeneration macular degeneration

Post-retinal:Post-retinal: anterior to optic chiasm (if optic nerve = monocular)anterior to optic chiasm (if optic nerve = monocular)

• compressive optic neuropathy (intracranial masses, thyroid eye disease)compressive optic neuropathy (intracranial masses, thyroid eye disease)• toxic/nutritional (nutritional deficiencies, alcohol/tobacco amblyopia)toxic/nutritional (nutritional deficiencies, alcohol/tobacco amblyopia)

optic chiasm lesions (pituitary adenoma)optic chiasm lesions (pituitary adenoma)

Diagnosis based on:Diagnosis based on:- focused ophthalmological historyfocused ophthalmological history

- monocular vs. binocularmonocular vs. binocular- acute vs. chronicacute vs. chronic- painful vs. painlesspainful vs. painless

- exam: … start with gross examinationexam: … start with gross examination- VAVA- slit lamp biomicroscopy +/- fluoresceinslit lamp biomicroscopy +/- fluorescein- dilated fundus examinationdilated fundus examination

- VF testingVF testing- fluorescein angiography +/- other testsfluorescein angiography +/- other tests

**Remember for exam:**Remember for exam: sometimes, chronic visual loss in ONE eye is sometimes, chronic visual loss in ONE eye is

noted incidentally some time later due to noted incidentally some time later due to occlusion of normal eye…: CHRONIC LOSS occlusion of normal eye…: CHRONIC LOSS OF VISION CAN PRESENT ACUTELY!!OF VISION CAN PRESENT ACUTELY!!

Corneal CausesCorneal Causes- dystrophydystrophy- scarringscarring- edemaedema

The Cornea The Cornea

- allows light to enter the - allows light to enter the eyeeye

- provides most of the eye’s - provides most of the eye’s optical poweroptical power

- 0.5-0.8 mm thick- 0.5-0.8 mm thick

- transparent due to its - transparent due to its uniformity, avascularityuniformity, avascularity and and deturgescencedeturgescence

Epithelium

Stroma

Endothelium

Corneal DystrophiesCorneal Dystrophies

- rare inherited disordersrare inherited disorders

- progressive, usually progressive, usually bilateralbilateral

- can affect any of the three layers of the can affect any of the three layers of the

cornea cornea

- affect transparencyaffect transparency

- age at presentation: first to fourth decadesage at presentation: first to fourth decades

Corneal DystrophiesCorneal Dystrophies- divided into:divided into:

- anterior dystrophiesanterior dystrophies::

- epitheliumepithelium

- may present with recurrent corneal erosionsmay present with recurrent corneal erosions

- stromal dystrophiesstromal dystrophies::

- usually present with visual lossusually present with visual loss

- if very anterior, can cause erosions and painif very anterior, can cause erosions and pain

- posterior dystrophiesposterior dystrophies::

- endotheliumendothelium

- vision loss secondary to edema (endothelial dysfx)vision loss secondary to edema (endothelial dysfx)

Corneal ScarringCorneal Scarring

- multiple causes:multiple causes:- traumatrauma

- infectious (eg., herpes)infectious (eg., herpes)

- post-surgicalpost-surgical

Corneal EdemaCorneal Edema

- most often caused by dysfunction of the most often caused by dysfunction of the

corneal endothelium:corneal endothelium:- dystrophydystrophy

- traumatrauma

- infectious (eg., herpes)infectious (eg., herpes)

- post-surgicalpost-surgical

Corneal Corneal TransplantationTransplantation

If the corneal stroma If the corneal stroma opacifiesopacifies due to due to trauma or infection, or if there is trauma or infection, or if there is swellingswelling or an or an irregularityirregularity of the of the surface of the cornea, light cannot surface of the cornea, light cannot properly reach the retina.properly reach the retina.

In some cases, a cornea from a In some cases, a cornea from a deceased donor can be transplanted.deceased donor can be transplanted.

Corneal TransplantationCorneal Transplantation

Lens-Related Causes Lens-Related Causes (cataract)(cataract)

- age-relatedage-related- traumatictraumatic

- steroid inducedsteroid induced

The LensThe Lens

- biconvex, avascular, - biconvex, avascular, transparent structuretransparent structure

- sits inside a thin - sits inside a thin capsule, attached to the capsule, attached to the ciliary body by the ciliary body by the zonuleszonules

- provides the - provides the remainder of the eye’s remainder of the eye’s optical power (along optical power (along with the cornea)with the cornea)

Lens

cataracts cataracts are due to the opacification of are due to the opacification of this normally clear structurethis normally clear structure

Age-Related CataractAge-Related Cataract

- often affect the nucleus of the lens first:often affect the nucleus of the lens first:- yellowing, followed by a browning of the lensyellowing, followed by a browning of the lens

- eventually, liquefactioneventually, liquefaction

- causes myopic changes (increased causes myopic changes (increased

refractive index of the lens)refractive index of the lens)

Traumatic CataractTraumatic Cataract- most common cause of most common cause of unilateral cataract unilateral cataract

in young individualsin young individuals

- most often caused by direct penetrating most often caused by direct penetrating

injury to the lensinjury to the lens

- can also be caused by:can also be caused by:- concussionconcussion

- ionizing radiation to ocular tumoursionizing radiation to ocular tumours

- infrared radiation (glassblowers)infrared radiation (glassblowers)

Steroid-Induced CataractSteroid-Induced Cataract- both both systemicsystemic and and topicaltopical steroids can be steroids can be

the culpritsthe culprits

- posterior part of lens affected firstposterior part of lens affected first

- children may be more susceptiblechildren may be more susceptible

- if lens changes develop, dose should be if lens changes develop, dose should be

reduced to the minimum necessaryreduced to the minimum necessary

- early opacities may regress with early opacities may regress with

discontinuation of therapydiscontinuation of therapy

GlaucomaGlaucoma

GlaucomaGlaucoma

disease of the disease of the optic nerveoptic nerve, often , often caused by an caused by an increase in intraocular increase in intraocular pressurepressure due to poor drainage of due to poor drainage of aqueous from the trabecular aqueous from the trabecular meshwork…meshwork…

GlaucomaGlaucoma

if left untreated, glaucoma can lead to if left untreated, glaucoma can lead to permanent permanent damage to the optic nerve damage to the optic nerve and resultant and resultant visual field lossvisual field loss

can progress to blindnesscan progress to blindness

Glaucoma Glaucoma

by definition, glaucoma is a trimodal by definition, glaucoma is a trimodal disease, characterized by:disease, characterized by:

increased IOPincreased IOP optic nerve changesoptic nerve changes visual field changesvisual field changes

Goldmann Applanation TonometerGoldmann Applanation Tonometer

GlaucomaGlaucoma classification:classification:

primaryprimary: open-angle, angle-closure: open-angle, angle-closure secondarysecondary: inflammatory, traumatic, : inflammatory, traumatic,

neovascular, steroid-induced etc…neovascular, steroid-induced etc… congenitalcongenital

Risk Factors for GlaucomaRisk Factors for Glaucoma

ageage african-american heritageafrican-american heritage high IOPhigh IOP family historyfamily history myopiamyopia

Symptoms of GlaucomaSymptoms of Glaucoma

often asymptomatic often asymptomatic with late disease, with late disease, constriction of constriction of

peripheral, and later central visual fieldperipheral, and later central visual field with very high IOP, can have blurry with very high IOP, can have blurry

vision and halos around lightsvision and halos around lights

Glaucoma: Optic Nerve Glaucoma: Optic Nerve ChangesChanges

increased cup:disc ratioincreased cup:disc ratio thinning of neural rimthinning of neural rim progressive loss of nerve fiber layerprogressive loss of nerve fiber layer flame hemorrhages on discflame hemorrhages on disc

Primary Open Angle Primary Open Angle GlaucomaGlaucoma

most common (90%)most common (90%) usually bilateral (can be asymmetric)usually bilateral (can be asymmetric) prevalence increases with ageprevalence increases with age angle is open, eye is quietangle is open, eye is quiet increased resistance to aqueous drainage increased resistance to aqueous drainage

at the level of the trabecular meshwork is at the level of the trabecular meshwork is thought to be the main pathophysiologic thought to be the main pathophysiologic featurefeature

Treatment optionsTreatment options

goal is to stabilize the IOP to protect goal is to stabilize the IOP to protect the optic nerve against further damagethe optic nerve against further damage

options:options: dropsdrops laserlaser surgerysurgery

Glaucoma - MedicationsGlaucoma - Medications

mechanism of action:mechanism of action: decrease aqueous production:decrease aqueous production:

• beta blockers: timololbeta blockers: timolol• alpha agonists: brimonidinealpha agonists: brimonidine• carbonic anhydrase inhibitors: diamoxcarbonic anhydrase inhibitors: diamox

increase aqueous outflow:increase aqueous outflow:• miotics: pilocarpinemiotics: pilocarpine• epinephrineepinephrine• prostaglandin analogs: latanoprostprostaglandin analogs: latanoprost

Glaucoma - LasersGlaucoma - Lasers

usually when medical management usually when medical management failsfails ALTALT (argon laser trabeculoplasty), (argon laser trabeculoplasty), SLTSLT

(selective laser trabeculoplasty): for open (selective laser trabeculoplasty): for open angle glaucomasangle glaucomas

peripheral iridotomyperipheral iridotomy: for angle-closure : for angle-closure glaucomasglaucomas

high success ratehigh success rate

Glaucoma - SurgeryGlaucoma - Surgery

usually when medical management and usually when medical management and laser treatments faillaser treatments fail trabeculectomy: trabeculectomy: sub-conjunctival shunt of sub-conjunctival shunt of

aqueousaqueous drainage devices (valves) drainage devices (valves) cyclodestruction: cyclodestruction: last resort – destruction last resort – destruction

of ciliary bodyof ciliary body

Where is the problem?Where is the problem? Pre-retinal: Pre-retinal:

cornea (dystrophy, scarring, edema)cornea (dystrophy, scarring, edema) lens (age-related, traumatic, steroid-induced)lens (age-related, traumatic, steroid-induced) glaucoma glaucoma

Retinal:Retinal: DM (diabetic retinopathy, macular edema)DM (diabetic retinopathy, macular edema) vascular insufficiency (arterial or venous occlusion)vascular insufficiency (arterial or venous occlusion) tumourstumours macular degeneration macular degeneration

Post-retinal:Post-retinal: anterior to optic chiasm (if optic nerve = monocular)anterior to optic chiasm (if optic nerve = monocular)

• compressive optic neuropathy (intracranial masses, thyroid eye disease)compressive optic neuropathy (intracranial masses, thyroid eye disease)• toxic/nutritional (nutritional deficiencies, alcohol/tobacco amblyopia)toxic/nutritional (nutritional deficiencies, alcohol/tobacco amblyopia)

optic chiasm lesions (pituitary adenoma)optic chiasm lesions (pituitary adenoma)

THE RETINATHE RETINA

- neural tissue lining - neural tissue lining the inside of the eyethe inside of the eye

- converts the visual - converts the visual image into a image into a neurochemical neurochemical message and sends it message and sends it to the brainto the brain

- is made up of 10 - is made up of 10 anatomic layersanatomic layers

DiabetesDiabetes- diabetic retinopathydiabetic retinopathy

- diabetic macular edemadiabetic macular edema

Diabetic RetinopathyDiabetic Retinopathy

microangiopathymicroangiopathyaffects pre-capillary arterioles, capillaries affects pre-capillary arterioles, capillaries and post-capillary venulesand post-capillary venulesfeatures offeatures of::

microvascular occlusionmicrovascular occlusionleakageleakage

clinically, can be divided into:clinically, can be divided into:background DR (nonproliferative)background DR (nonproliferative)preproliferative DRpreproliferative DRproliferative DRproliferative DR

Diabetic Retinopathy: EpidemiologyDiabetic Retinopathy: Epidemiology239 million people by 2010239 million people by 2010

doubling in prevalence since 1994doubling in prevalence since 1994diabetes will affect:diabetes will affect:

28 million in western Europe28 million in western Europe18.9 million in North America 18.9 million in North America 138.2 million in Asia138.2 million in Asia1.3 million in Australasia1.3 million in Australasia

• #1 cause of blindness in patients 20-64 yrs#1 cause of blindness in patients 20-64 yrs

• prevalence increases with prevalence increases with duration of diabetesduration of diabetes and and patient agepatient age

rare to find DR in children < 10 yrs, regardless of durationrare to find DR in children < 10 yrs, regardless of durationrisk of developing DR increases after pubertyrisk of developing DR increases after puberty

EpidemiologyEpidemiologyWisconsin Epidemiologic Study of Diabetic RetinopathyWisconsin Epidemiologic Study of Diabetic Retinopathy

Between 1979-1980Between 1979-1980

1210 patients with Type 11210 patients with Type 1

1780 patients with Type 21780 patients with Type 2

predominantly white populationpredominantly white population

After 20 yrs, DR present in:After 20 yrs, DR present in:

99% of Type 1 99% of Type 1

60% of Type 260% of Type 2

WESDR: Frequency of retinopathy in WESDR: Frequency of retinopathy in subjects with type 1diabetessubjects with type 1diabetes

WESDR: Frequency of retinopathy in WESDR: Frequency of retinopathy in subjects with type 2 diabetessubjects with type 2 diabetes

Diabetic Retinopathy: Risk FactorsDiabetic Retinopathy: Risk Factors

duration of diabetesduration of diabetes: most important risk : most important risk factorfactorpoor metabolic controlpoor metabolic controlpregnancy: can be associated with rapid pregnancy: can be associated with rapid progressionprogressionHTNHTNnephropathynephropathysmokingsmokingobesityobesityhyperlipidemiahyperlipidemia

Classification of Diabetic RetinopathyClassification of Diabetic RetinopathyClassified into Classified into 2 stages2 stages

Nonproliferative Diabetic Retinopathy (NPDR)Nonproliferative Diabetic Retinopathy (NPDR)early stageearly stagealso known as background DR (BDR)also known as background DR (BDR)further categorized based upon extent of DR further categorized based upon extent of DR

mild, moderate, severe, very severemild, moderate, severe, very severe

Proliferative Diabetic Retinopathy (PDR)Proliferative Diabetic Retinopathy (PDR)more advanced stagemore advanced stage

• ***Macular edema***Macular edema• May be present May be present at any stage of DRat any stage of DR

NPDRNPDR

typically typically asymptomaticasymptomaticfluctuatingfluctuating visual acuity: visual acuity:

fluctuating blood sugarfluctuating blood sugar

decreaseddecreased visual acuity: visual acuity:

CSMECSME

macular ischemiamacular ischemia

review these patients annuallyreview these patients annually

Mild NPDRMild NPDR

Moderate NPDRModerate NPDR

Severe NPDRSevere NPDR

Proliferative Diabetic RetinopathyProliferative Diabetic Retinopathy

more likely to become more likely to become symptomatic than early symptomatic than early NPDRNPDR

may have decreased may have decreased vision, sudden vision loss, vision, sudden vision loss, floaters, cobwebs, flashes, floaters, cobwebs, flashes, dull eye ache dull eye ache

PDR can also affect visual PDR can also affect visual function by affecting the function by affecting the macula with resulting macula with resulting macular ischemia and/or macular ischemia and/or edemaedema

Proliferative DRProliferative DR

affects 5-10% of the diabetic populationaffects 5-10% of the diabetic population

neovascularizationneovascularization is the hallmark is the hallmark

NVD: neovascularization of the discNVD: neovascularization of the disc

NVE: neovascularization elsewhereNVE: neovascularization elsewhere

new vessels are not only extremely fragile new vessels are not only extremely fragile ((intraretinal or vitreous hemorrhageintraretinal or vitreous hemorrhage), but ), but often associated with fibrous proliferation, often associated with fibrous proliferation, leading to an increased risk of leading to an increased risk of tractional retinal tractional retinal detachmentdetachment

Advanced PDRAdvanced PDR

Tractional retinal Tractional retinal detachmentdetachment resulting from resulting from contraction of contraction of the fibrovascular the fibrovascular proliferative proliferative tissue on the tissue on the retinaretina

Panretinal Photocoagulation for High-risk Panretinal Photocoagulation for High-risk PDRPDR

goal is to induce goal is to induce involutioninvolution (or at (or at least least arrestarrest) of new ) of new vessels by creating vessels by creating areas of retinal areas of retinal ischemiaischemia

1200-3000 burns1200-3000 burns 4 sessions4 sessions

Vitrectomy for Vitreous Vitrectomy for Vitreous Hemorrhage / TRDHemorrhage / TRD

Diabetic Macular Edema (DME)Diabetic Macular Edema (DME)

retinal edema retinal edema threateningthreatening or or involvinginvolving the the maculamacula

diagnosis is made by slit-lamp exam, diagnosis is made by slit-lamp exam, confirmed by fluorescein angiography and/or confirmed by fluorescein angiography and/or OCTOCT

important observations include:important observations include:locationlocation of retinal thickening relative to the of retinal thickening relative to the foveafoveapresence and location of presence and location of exudatesexudates

DME and CSMEDME and CSME

Treatment of CSMETreatment of CSME argon laser applicationargon laser application

intravitreal steroid injectionintravitreal steroid injection

intravitreal anti-VEGF injectionintravitreal anti-VEGF injection

pars plana vitrectomypars plana vitrectomy

Ophthalmological Follow-UpOphthalmological Follow-Up Diabetic ScreeningDiabetic Screening

Type 1 diabeticsType 1 diabetics: :

Dilated funduscopic exam (DFE) 5 yrs after diagnosisDilated funduscopic exam (DFE) 5 yrs after diagnosisNewly diagnosed patients with Type 1 diabetes rarely Newly diagnosed patients with Type 1 diabetes rarely have retinopathy during the first 5 yrshave retinopathy during the first 5 yrs

Type 2 diabeticsType 2 diabetics: :

Type 2 diabetics typically diagnosed yrs after initial Type 2 diabetics typically diagnosed yrs after initial onsetonsetDFE at the time of diagnosisDFE at the time of diagnosisSignificant portion of newly diagnosed Type 2 Significant portion of newly diagnosed Type 2 diabetics have established DR at the time of diabetics have established DR at the time of diagnosisdiagnosis

Vascular InsufficiencyVascular Insufficiency- arterial occlusions (CRAO, BRAO)arterial occlusions (CRAO, BRAO)- venous occlusions (CRVO, BRVO)venous occlusions (CRVO, BRVO)

CRAOCRAO

most of the retina is supplied by the most of the retina is supplied by the central retinal artery central retinal artery (branch of the (branch of the ophthalmic artery, which is the first branch ophthalmic artery, which is the first branch of the ICA)of the ICA)

if this supply is interrupted (embolus, if this supply is interrupted (embolus, thrombosis, inflammation, vasculitis or thrombosis, inflammation, vasculitis or compression), the retina becomes compression), the retina becomes ischemicischemic

irreversible damage occurs after irreversible damage occurs after approximately 90 minutesapproximately 90 minutes

CRAOCRAO

CRAOCRAO presentation is with sudden and presentation is with sudden and

profound loss of visionprofound loss of vision RAPD is presentRAPD is present orange reflex from the choroid stands orange reflex from the choroid stands

out at the fovea, and contrasts with the out at the fovea, and contrasts with the surrounding pale retina (surrounding pale retina (cherry-red cherry-red spotspot))

must r/o temporal arteritis must r/o temporal arteritis

CRAOCRAO most commonly the result of most commonly the result of

atherosclerosis (atherosclerosis (thrombosisthrombosis)) but may but may also be caused by calcific also be caused by calcific emboliemboli

often in often in olderolder patients, with a hx of patients, with a hx of arteriosclerosisarteriosclerosis

may have had a hx of amaurosis fugax may have had a hx of amaurosis fugax (transient visual loss)(transient visual loss)

CRAOCRAO

OPHTHALMOLOGIC EMERGENCY!! OPHTHALMOLOGIC EMERGENCY!! treatment:treatment:

decrease IOPdecrease IOP paracentesisparacentesis ocular massageocular massage

goal: to send the embolus distallygoal: to send the embolus distally **remember to r/o giant cell arteritis! (**remember to r/o giant cell arteritis! (ESR, CRP, pltESR, CRP, plt)) poor prognosis: poor prognosis: 60% 60% 20/400 20/400

BRAOBRAO

BRAOBRAO sudden and profound sudden and profound altitudinal or altitudinal or

sectoral sectoral visual field loss visual field loss similar causes as CRAOsimilar causes as CRAO identify and treat associated medical identify and treat associated medical

conditions (HTN, DM, conditions (HTN, DM, hypercholesterolemia, smoking, hypercholesterolemia, smoking, vasculitis etc…)vasculitis etc…)

BRAOBRAO retinal cloudiness in ischemic arearetinal cloudiness in ischemic area +/- visible embolus+/- visible embolus also has a poor prognosis, unless the also has a poor prognosis, unless the

obstruction can be dislodged within a obstruction can be dislodged within a few hoursfew hours

CRVOCRVO

thrombosis of the thrombosis of the central retinal veincentral retinal vein sudden loss of vision in affected eyesudden loss of vision in affected eye severity of symptoms varies…severity of symptoms varies…

non-ischemic: non-ischemic: 75% 75% Ischemic Ischemic

most characteristic finding: most characteristic finding: retinal retinal hemorrhageshemorrhages

CRVOCRVO

CRVO CRVO

underlying associationsunderlying associations advancing ageadvancing age systemic conditions: HTN, DM, smoking, obesity, systemic conditions: HTN, DM, smoking, obesity,

hyperlipidemiahyperlipidemia glaucomaglaucoma inflammatory diseases: sarcoidosis, Behcet inflammatory diseases: sarcoidosis, Behcet

disease disease thrombophilic disorders: hyperhomocysteinaemia, thrombophilic disorders: hyperhomocysteinaemia,

antiphospholipid antibody syndromeantiphospholipid antibody syndrome

CRVO CRVO Treatment:Treatment:

treat associated medical conditionstreat associated medical conditions decrease IOP if elevateddecrease IOP if elevated pan-retinal photocoagulation pan-retinal photocoagulation

(laser) if:(laser) if:

• neovascularization (iris, angle, neovascularization (iris, angle, retina)retina)

• ……especially if ischemic CRVOespecially if ischemic CRVO

BRVOBRVO

thrombosis of a branch of the thrombosis of a branch of the central central retinal veinretinal vein

visual loss depends on the visual loss depends on the amount of amount of macular drainage compromised by the macular drainage compromised by the occlusionocclusion (peripheral occlusions may be (peripheral occlusions may be asymptomatic)asymptomatic)

characteristic findings in characteristic findings in one sector one sector of the of the retina:retina: dilatation and tortuosity of veinsdilatation and tortuosity of veins retinal hemorrhagesretinal hemorrhages retinal/macular edemaretinal/macular edema cotton-wool spotscotton-wool spots

BRVOBRVO

obstruction often at arterio-venous obstruction often at arterio-venous crossings: arteries and veins share crossings: arteries and veins share adventitial sheath… thickening of the adventitial sheath… thickening of the arteriole (arteriole (arteriosclerosisarteriosclerosis) compresses ) compresses the vein, eventually causing an occlusionthe vein, eventually causing an occlusion

often associated with:often associated with: hypertension (75%)hypertension (75%) diabetes (10%)diabetes (10%)

BRVOBRVO

prognosisprognosis: depends on amt of venous : depends on amt of venous drainage involved by the occlusion and drainage involved by the occlusion and severity of macular ischemia: severity of macular ischemia: within 6 mos, within 6 mos, about 50% of eyes have a VA of 20/30 or about 50% of eyes have a VA of 20/30 or betterbetter

main complications:main complications: chronic macular edemachronic macular edema neovascularizationneovascularization

laser photocoagulationlaser photocoagulation may be helpful in may be helpful in above casesabove cases

BRVOBRVO

Retinal TumoursRetinal Tumours

ocular tumours:ocular tumours: ciliary bodyciliary body: :

• melanomamelanoma choroidchoroid::

• melanomamelanoma• hemangiomahemangioma• metastasesmetastases

primary ocular lymphomaprimary ocular lymphoma retina and optic nerveretina and optic nerve::

• retinoblastomaretinoblastoma• astrocytomaastrocytoma• hemangiomahemangioma

Choroidal MelanomaChoroidal Melanoma most common primary intraocular most common primary intraocular

tumour in adultstumour in adults presentation usually in 6th decade:presentation usually in 6th decade:

asymptomatic vs. visual field defect and/or asymptomatic vs. visual field defect and/or decreased visual acuitydecreased visual acuity

signs: signs: raised, usually pigmented lesion visible at raised, usually pigmented lesion visible at

the back of the eyethe back of the eye may be associated with retinal detachmentmay be associated with retinal detachment optic nerve may be involvedoptic nerve may be involved

Choroidal MelanomaChoroidal Melanoma treatment:treatment:

consider size, location, activity of tumour, consider size, location, activity of tumour, state of fellow eye, general health/age of pt, state of fellow eye, general health/age of pt, pt’s wishes/fearspt’s wishes/fears• brachytherapybrachytherapy• external radiotherapyexternal radiotherapy• transpupillary thermotherapytranspupillary thermotherapy• local resectionlocal resection• enucleationenucleation• exenterationexenteration• palliativepalliative (may include chemo) (may include chemo)

Choroidal MetastasesChoroidal Metastases ……with choroidal melanoma, don’t forget with choroidal melanoma, don’t forget

general medical investigations!general medical investigations! mets TO the choroid:mets TO the choroid:

• most frequently from most frequently from bronchusbronchus in both sexes in both sexes and the and the breastbreast in women, rarely kidney or GI in women, rarely kidney or GI

CXR, rectal exam, mammographyCXR, rectal exam, mammography

mets FROM the choroid:mets FROM the choroid:• liverliver

hepatic u/s, GGT, ALPhepatic u/s, GGT, ALP

• lungslungs (rarely affected before liver) (rarely affected before liver) CXRCXR

Choroidal MetastasesChoroidal Metastases

usually present with visual impairment usually present with visual impairment only IF tumour is near the maculaonly IF tumour is near the macula

signssigns:: fast-growing, creamy coloured lesionfast-growing, creamy coloured lesion most often in posterior polemost often in posterior pole usually not very elevated (infiltrates laterally)usually not very elevated (infiltrates laterally)

Choroidal MetastasesChoroidal Metastases treatmenttreatment::

observe: if asxic or receiving systemic chemoobserve: if asxic or receiving systemic chemo radiation: external beam or brachyradiation: external beam or brachy transpupillary thermotherapytranspupillary thermotherapy systemic therapy for the primarysystemic therapy for the primary enucleation: for painful blind eyeenucleation: for painful blind eye

prognosis is poorprognosis is poor…… median survival: 8-12 mos for all pts, 15-17 median survival: 8-12 mos for all pts, 15-17

mos for those with breast camos for those with breast ca

RetinoblastomaRetinoblastoma most common malignant tumour of the eye most common malignant tumour of the eye

in childhood (1:20 000)in childhood (1:20 000) mean age of mean age of presentationpresentation: 8 mos if : 8 mos if

inherited, 25 mos if sporadicinherited, 25 mos if sporadic 60% present with leukocoria (white pupillary 60% present with leukocoria (white pupillary

reflex)reflex) strabismus (20%)strabismus (20%) occasionally: painful, red eyeoccasionally: painful, red eye if inherited: often bilateralif inherited: often bilateral

RetinoblastomaRetinoblastoma malignant transformation of primitive malignant transformation of primitive

retinal cells before their final differentiationretinal cells before their final differentiation

can be caused by can be caused by germinal mutations germinal mutations (can (can be passed on to the next generation), or be passed on to the next generation), or can be can be sporadicsporadic (66% of cases) (66% of cases)

RetinoblastomaRetinoblastoma this is a this is a clinical diagnosisclinical diagnosis, but , but CSFCSF and and

bone marrow bone marrow should be examined to check should be examined to check for metastatic disease if ON involved or if for metastatic disease if ON involved or if there is evidence of extraocular extensionthere is evidence of extraocular extension

rx:rx: smallsmall: cryotherapy, photocoagulation: cryotherapy, photocoagulation mediummedium: brachytherapy, external beam, chemo: brachytherapy, external beam, chemo large/advanced caseslarge/advanced cases: chemoreduction + local : chemoreduction + local

treatment, enucleationtreatment, enucleation metastatic diseasemetastatic disease: chemo (intrathecal if cells : chemo (intrathecal if cells

in CSF)in CSF)

RetinoblastomaRetinoblastoma prognosis: prognosis:

depends on extent of disease at diagnosisdepends on extent of disease at diagnosis overall mortality ~ 5-15%overall mortality ~ 5-15% ~ 50% of children with the germinal mutation ~ 50% of children with the germinal mutation

will eventually develop a second primary will eventually develop a second primary tumour (eg., tumour (eg., osteosarcomaosteosarcoma of the femur or of the femur or pinealoblastomapinealoblastoma))

Macular DegenerationMacular Degeneration

MaculaMacula

1.5 mm1.5 mm in diameter in diameter central vision: central vision: BEST VISUAL ACUITYBEST VISUAL ACUITY colour visioncolour vision progressive destruction of the macular progressive destruction of the macular

area:area:

MACULAR DEGENERATIONMACULAR DEGENERATION

Macular DegenerationMacular Degeneration most common cause of irreversible most common cause of irreversible

visual loss in the developed worldvisual loss in the developed world exists in two forms:exists in two forms:

non-exudativenon-exudative (dry) macular (dry) macular degenerationdegeneration

exudativeexudative (wet) macular (wet) macular degenerationdegeneration

Non-exudative Macular Non-exudative Macular DegenerationDegeneration

lipid products arising from lipid products arising from photoreceptor outer segments are photoreceptor outer segments are found under retina found under retina can be seen with ophthalmoscope!can be seen with ophthalmoscope! called called « drusen »« drusen »

Exudative Macular Exudative Macular DegenerationDegeneration

new vessels from the choroid grow into new vessels from the choroid grow into the sub-retinal space; form a the sub-retinal space; form a sub-sub-retinal neovascular membraneretinal neovascular membrane

subsequent subsequent hemorrhagehemorrhage into the sub- into the sub-retinal space or even through the retina retinal space or even through the retina into the vitreous is associated with into the vitreous is associated with profound loss of visionprofound loss of vision

Macular DegenerationMacular Degeneration symptoms:symptoms:

since fovea is responsible for fine visual since fovea is responsible for fine visual resolution, any disruption will cause resolution, any disruption will cause severe visual impairmentsevere visual impairment• blurry/reduced visionblurry/reduced vision

• distorted vision (distorted vision (metamorphopsiametamorphopsia))

• reduction (micropsia) or enlargement reduction (micropsia) or enlargement (macropsia) of objects(macropsia) of objects

• VF loss (VF loss (scotomascotoma))

Macular DegenerationMacular Degeneration rx:rx:

non-exudativenon-exudative (usually slowly (usually slowly progressive):progressive):

• no actual medical treatmentno actual medical treatment

• use low vision aidsuse low vision aids

• high dose antioxidants MAY be high dose antioxidants MAY be beneficial (eg., vitalux)beneficial (eg., vitalux)

Macular DegenerationMacular Degeneration rx:rx:

exudativeexudative (can be rapidly progressive and (can be rapidly progressive and devastating):devastating):• intravitreal injections of anti-VEGF intravitreal injections of anti-VEGF

factors: bevacizumab, ranibizumabfactors: bevacizumab, ranibizumab• photodynamic therapy (injection of photodynamic therapy (injection of

photosensitizer into systemic circulation photosensitizer into systemic circulation followed immediately by laser targeting followed immediately by laser targeting new vessels in macular area)new vessels in macular area)

• combination of above treatmentscombination of above treatments

Where is the problem?Where is the problem? Pre-retinal: Pre-retinal:

cornea (dystrophy, scarring, edema)cornea (dystrophy, scarring, edema) lens (age-related, traumatic, steroid-induced)lens (age-related, traumatic, steroid-induced) glaucoma glaucoma

Retinal:Retinal: DM (diabetic retinopathy, macular edema)DM (diabetic retinopathy, macular edema) vascular insufficiency (arterial or venous occlusion)vascular insufficiency (arterial or venous occlusion) tumourstumours macular degeneration macular degeneration

Post-retinal:Post-retinal: anterior to optic chiasm (if optic nerve = monocular)anterior to optic chiasm (if optic nerve = monocular)

• compressive optic neuropathy (intracranial masses, thyroid eye disease)compressive optic neuropathy (intracranial masses, thyroid eye disease)• toxic/nutritional (nutritional deficiencies, alcohol/tobacco amblyopia)toxic/nutritional (nutritional deficiencies, alcohol/tobacco amblyopia)

optic chiasm lesions (pituitary adenoma)optic chiasm lesions (pituitary adenoma)

OPTIC NERVEOPTIC NERVE

1.2 million cells 1.2 million cells 80 % visual fibres80 % visual fibres 20 % pupillary fibres20 % pupillary fibres carries visual carries visual

information from information from the eye to the brainthe eye to the brain

OPTIC CHIASMOPTIC CHIASM

crossover of nasal fiberscrossover of nasal fibers

above the pituitaryabove the pituitary

internal carotids are just internal carotids are just laterallateral

from optic chiasm:from optic chiasm:

optic tract to the optic tract to the lateral geniculate bodylateral geniculate body

optic radiation optic radiation to the to the primary visual cortexprimary visual cortex

Anterior to Optic Anterior to Optic ChiasmChiasm

- compressive optic neuropathiescompressive optic neuropathies- toxic/nutritional optic neuropathiestoxic/nutritional optic neuropathies

Compressive Optic Compressive Optic NeuropathiesNeuropathies

INTRACRANIAL MASSES:INTRACRANIAL MASSES: optic nerve gliomaoptic nerve glioma

• typically affects young women, end of first decadetypically affects young women, end of first decade• associated with NF-1associated with NF-1

optic nerve sheath meningiomaoptic nerve sheath meningioma• most frequent in middle-aged womenmost frequent in middle-aged women• unilateral, gradual visual impairmentunilateral, gradual visual impairment

any any other orbital or chiasmal tumour other orbital or chiasmal tumour compressing any part of the optic nervecompressing any part of the optic nerve

THYROID EYE DISEASETHYROID EYE DISEASE

Thyroid Eye DiseaseThyroid Eye Disease may occur in the absence of biochemical may occur in the absence of biochemical

evidence of thyroid dysfxevidence of thyroid dysfx autoimmune reaction (IgG Abs) causing:autoimmune reaction (IgG Abs) causing:

inflammation of EOMs: pleiomorphic cellular inflammation of EOMs: pleiomorphic cellular infiltration associated with increased secretion infiltration associated with increased secretion of GAGs and osmotic imbibition of waterof GAGs and osmotic imbibition of water• muscles can become up to 8 times their original muscles can become up to 8 times their original

size!!size!!

no relation to severity of thyroid dysfx!no relation to severity of thyroid dysfx!

Thyroid Eye DiseaseThyroid Eye Disease

main findings: (not all are always present!)main findings: (not all are always present!) soft tissue involvementsoft tissue involvement lid retractionlid retraction proptosisproptosis optic neuropathyoptic neuropathy restrictive myopathyrestrictive myopathy

Thyroid Eye DiseaseThyroid Eye Disease vision loss from:vision loss from:

exposure keratopathyexposure keratopathy• due to severe proptosis resulting in incomplete lid due to severe proptosis resulting in incomplete lid

closure → chronically exposed cornea → corneal closure → chronically exposed cornea → corneal ulceration & exposure keratopathyulceration & exposure keratopathy

optic neuropathyoptic neuropathy• affects 5% of ptsaffects 5% of pts• compression of ON or its blood supply by compression of ON or its blood supply by

congested (enlarged) EOMscongested (enlarged) EOMs• can lead to severe, can lead to severe, permanentpermanent visual impairment visual impairment• rx with steroids, surgery if neededrx with steroids, surgery if needed

Toxic/Nutritional Optic Toxic/Nutritional Optic NeuropathiesNeuropathies

nutritional deficienciesnutritional deficiencies alcohol-tobacco amblyopiaalcohol-tobacco amblyopia

Nutritional DeficienciesNutritional Deficiencies

pts with extremely poor diets, often in association pts with extremely poor diets, often in association with alcohol-tobacco amblyopiawith alcohol-tobacco amblyopia

usually due to B12 deficiency in combination with usually due to B12 deficiency in combination with cyanide toxicitycyanide toxicity

symmetrical VF losssymmetrical VF loss if early, can be treated with high-dose vitamins if early, can be treated with high-dose vitamins

and restoration of « well-balanced diet »and restoration of « well-balanced diet » eventually leads to optic atrophy and permanent eventually leads to optic atrophy and permanent

vision lossvision loss

Alcohol-Tobacco AmblyopiaAlcohol-Tobacco Amblyopia affects heavy drinkers, cigar and pipe smokers: deficient in affects heavy drinkers, cigar and pipe smokers: deficient in

protein and the B vitamins protein and the B vitamins symptomssymptoms: insidious, bilateral, progressive visual : insidious, bilateral, progressive visual

impairment + dyschromatopsiaimpairment + dyschromatopsia signssigns: symmetrical VF defect, may have pale (or normal) : symmetrical VF defect, may have pale (or normal)

discsdiscs rxrx: 1000 units of hydroxocobalamin qweekly X 10 wks + : 1000 units of hydroxocobalamin qweekly X 10 wks +

multivitamins + « well-balanced diet »multivitamins + « well-balanced diet » pxpx::

good in early cases if comply with rxgood in early cases if comply with rx advanced cases: optic atrophy and permanent visual advanced cases: optic atrophy and permanent visual

lossloss

Optic Chiasm LesionsOptic Chiasm Lesions- pituitary adenomapituitary adenoma

Pituitary AdenomaPituitary Adenoma presentation usually in early adult life or presentation usually in early adult life or

middle agemiddle age symptoms:symptoms:

h/ah/a visual symptoms: very gradual onset (often visual symptoms: very gradual onset (often

not noticed by pt until very well-established)not noticed by pt until very well-established)• VF defectVF defect: usually, bitemporal hemianopia, worst in : usually, bitemporal hemianopia, worst in

the superior field, and extending inferiorlythe superior field, and extending inferiorly• colour desaturation colour desaturation across vertical midlineacross vertical midline• optic atrophyoptic atrophy: in 50% of cases with field defects : in 50% of cases with field defects

caused by pituitary lesionscaused by pituitary lesions

Pituitary AdenomaPituitary Adenoma investigations:investigations:

MRI: coronal, axial and sagittal sections before MRI: coronal, axial and sagittal sections before and after gadolinium injectionand after gadolinium injection

CT: demonstrates enlargement or erosion of CT: demonstrates enlargement or erosion of the sellathe sella

endocrinological investigation: PRL, FSH, TSH, endocrinological investigation: PRL, FSH, TSH, GHGH

Pituitary AdenomaPituitary Adenoma treatment options:treatment options:

observationobservation medical: dopamine agonists (bromocriptine)medical: dopamine agonists (bromocriptine) surgerysurgery radiotherapy: often used as an adjunctradiotherapy: often used as an adjunct gamma knife stereotactic radiotherapygamma knife stereotactic radiotherapy

Visual Field DefectsVisual Field Defects

Merci