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THE RETINA12RETINAL DISEASESRarely occur in isolationFrequently affected by systemic diseases / RETINOPATHIESVASCULAR DiseasesINFLAMMATORY DiseasesDEGENERATIVE ConditionsRetinal DetachmentTumors- RetinoblastomaMacular disorders

3HYPERTENSIVE RETINOPATHY Modified Keith, Wagner & Barker (1939) ClassificationCorrelates directly with degree of systemic HTN and inversely with prognosis for survivalGRADE I: Mild to moderate narrowing/ sclerosis of smaller arterioles (generalized arteriolar attenuation)

GRADE II: Moderate to marked narrowing, broadening of the arteriolar light reflex & AV crossing changes

GRADE III: Retinal arteriolar narrowing, focal constriction & AV crossing changes, retinal oedema, cotton wool patches, flame shaped haemorrhages.

GRADE IV: All above features + Papilloedema4HYPERTENSIVE RETINOPATHY

5HYPERTENSIVE RETINOPATHY

7HYPERTENSIVE RETINOPATHY

8HYPERTENSIVE RETINOPATHY

9HYPERTENSIVE RETINOPATHYGrading of Arterio-sclerotic changes:Grade I- Broadening of arteriolar light reflexGrade II- Arterio-venous crossing defectsGrade III- Copper wire appearanceGrade IV- Silver wire appearance

10HYPERTENSIVE RETINOPATHYArterio-venous Crossing changes:Salus signGunns signBonnets sign / Distal banking

Obstruction of arteriolar circulationEmbolus + Superadded spasm Complete obstructionAtheroma at bifurcation of Carotid arteryDiseased Mitral/ Aortic valveEmbolus- CommonFrom the heartCalcific from valvesVegetations from valves; esp. in Bacterial endocarditisThrombus - MIFrom carotid artery - Cholesterol emboli (HOLLENHORST PLAQUES) frequently asymptomaticFibrino-platelet emboli Retinal TIA/ Amaurosis fugax, may be ass. with cerebral TIA on same side and contralateral signsCalcific emboli

RETINAL ARTERIAL OCCLUSIVE DISEASECENTRAL RETINAL ARTERY OCCLUSIONOther Causes: Systemic hypertension seen in two thirds of patientsDiabetes mellitusCardiac valvular disease seen in one fourth of patients

Atherosclerotic changesGiant cell arteritisHypercoagulable stateRare causes - Consider in younger patients Behet disease Syphilis Sickle cell disease MigraineHydrostatic arterial occlusion

13CENTRAL RETINAL ARTERY OCCLUSIONOccurs at lamina cribrosaSudden and complete retinal ischaemia, tissue rapidly dies and vision is lostFundus: Pale cloudy retinaArteries thread likeVeins slightly constricted near the discCherry red spot at the macula

CENTRAL RETINAL ARTERY OCCLUSION

CENTRAL RETINAL ARTERY OCCLUSIONClinical features:Painless catastrophic loss of vision in secondsAmaurosis fugax ( transient visual loss ).VA: CF to PL+ ( 90% )Afferent pupillary defectCherry red spot: opacification of superficial NSRMacular sparing in 25% due to cilio-retinal arteryAttenuated retinal arteriolesCattle tracking / Boxcar segmentation can be seen in both arteries and veins. This is a sign of severe obstruction.

16CENTRAL RETINAL ARTERY OCCLUSIONTREATMENT Rarely helpsTo be done within 6 -Relieve spasm and drive embolus into less important branchFirm ocular massage in supine position IOP &Blood flow Dislodges embolusI/V Acetazolamide or MannitolHyperbaric oxygenParacentesisPeripheral vasodilators (Inhalation of Amylnitrite, pentoxifylline/ Sublingual Isosorbide)Invasive treatment: selective catheterization of the ophthalmic artery with administration of fibrinolytic drugs.Results are often disappointingBranch retinal artery occlusion. Multiple emboli are visible in of the affected arterial branches (arrows).

CENTRAL RETINAL VEIN OCCLUSIONCommon & easily diagnosed

Clinical features:variable visual lossretinal hemorrhages, dilated tortuous retinal veins, cotton-wool spots, macular edema, and optic disc edema.

19Recent onset central retinal vein occlusion, showing extensive hemorrhages in the posterior pole giving the "blood and thunder appearance"

CENTRAL RETINAL VEIN OCCLUSIONPredisposing factors:HypertensionCardiovascular diseasesHypercoagulabilityHyperopiaHigh IOP

Broadly, CRVO can be divided into 2 clinical types, ISCHEMIC AND NONISCHEMIC. All these classifications take into account the area of retinal capillary nonperfusion and development of neovascular complications. 21CENTRAL RETINAL VEIN OCCLUSIONComplications:Macular edema > Cystoid macular edemaReduced VANeovascular glaucomaIVFA:Non-ischemic: Ischemic: , areas of capillary non-perfusion : 50% develop NVG22CENTRAL RETINAL VEIN OCCLUSIONA number of clinical and ancillary investigative factors are taken into account for classifying CRVO, including vision at presentation, presence or absence of relative afferent pupillary defect, extent of retinal hemorrhages, cotton-wool spots, extent of retinal perfusion by fluorescein angiography and electro-retino-graphic changes. CENTRAL RETINAL VEIN OCCLUSIONNonischemic CRVO is the milder form of the disease. It may present with good vision, few retinal hemorrhages and cotton-wool spots, no relative afferent pupillary defect, and good perfusion to the retina. Nonischemic CRVO may resolve fully with good visual outcome or may progress to the ischemic type. Ischemic CRVO (hemorrhagic CRVO) with marked hemorrhages and edema

CENTRAL RETINAL VEIN OCCLUSIONTreatment:PRP to prevent NVGMacular grid for macular edema26BRANCH RETINAL VEIN OCCLUSION

Occlusion of the inferior temporal vein

Peripheral Retinal Degeneration Microcystoid degeneration:- Tiny vesicles with indistinct boundaries on a grayish-white background.Snowflakes Degeneration:- minute glistering yellow-white dots which are frequently found scattered diffusely in the peripheral fundus.

RETINAL DEGENERATIONBENIGN (Do not cause retinal breaks)Snow flake: near ora serrataPaving stone: Focal chorioretinal atrophyReticular pigmentaryEquatorial drusensPeripheral microcystic

Retinal Tears U-Tears (arrowhead tears):- Apex of which is pulled anteriorly by the vitreous, the base remaining attached to the retina.Incomplete U- Tears:- May be linear, L-Shaped, I-Shaped.

DETACHMENT OF RETINASeparation of neuro-sensory retina from RPE d/t accumulation of SRF in potential space

Depending on mechanism of SRF collection Retinal Detachments are classified intoRhegmatogenousTractionalExudative

RHEGMATOGENOUS RDSecondary to a full thickness defect in the sensory retina which permits SRF derived from synchitic (Liquified) vitreous gel to gain access to this subretinal space.

RHEGMATOGENOUS RDCommonest causeTypes of breaks-Horse shoe shaped tears: Periphery,> in upper quadrantsRound holes: Less peripheral and also in maculaGiant tears: Involving >1/4 of retinaDialysis/Disinsertion: Large, sharply defined & choroid shines throughPredisposing factorsMyopiaPrevious intraocular surgeryFamily H/O RDTraumaInflammationsRHEGMATOGENOUS RDClinical featuresShallow RD- non specific D/V as retina gets some nourishment from SRFTransient flashes of light especially seen nasallyIncrease in no. of black spotsCurtain/ Veil obscuring field of visionGross D/V when macula affected or large bullous RD

Retinal detachment in the temporal mid-periphery without macular involvement

Rhegmatogenous retinal detachment involving the macula.

Total retinal detachment

RHEGMATOGENOUS RD - ManagementRetinal breaks to be localized 50% cases have > 1 breakUpper temporal quadrant affected the mostProphylaxisEarly detection of breaks & treatment by cryotherapy or photocoagulation and take care of risk factorsLong term follow up by repeated I/O and examination with 3 mirror CL to detect further breaksSurgical Management depends upon extent, duration and condition of retina.Horseshoe-like break with bridging vesselsurrounded by laser spots

Principles of managementIdentification of breaks and traction if presentSealing of breaksRelease of traction ( Vitreal/ Pre- retinal)Drainage of SRFEnsuring chorio-retinal apposition for atleast 2 weeks by External tamponade (silicon buckle, tyre, sponge or bands)Internal tamponade ( Air, gas -sulpur hexafluoride, perfluorocarbons or liquids as silicone oil)PARS PLANA VITRECTOMY

Non - Rhegmatogenous RDTractional:- Sensory retina is pulled away from the RPE by contracting vitreoretinal membrane the source of SRF is unknown.

Exudative:-SRF derived from the choriocapillaries gains access to the subretinal spaces. TRACTIONAL RETINAL DETACHMENTSecond most commonEtiology:PDRPVRROPTrauma45EXUDATIVE RETINAL DETACHMENTFluid collection beneath Retina and choroidEtiology:Degenerative macular disease > SRNVMInflammation eg uveitis in VKH dz, Posterior scleritisInfectionsChoroidal tumorRPE disease > leakSystemic vascular disease- hypertension & eclampsia46EXUDATIVE RETINAL DETACHMENT

PIGMENTARY RETINAL DYSTROPHYSlow degenerative disease, bilateralStarts in childhood, leads to blindness in middle/ advanced ageDegeneration mainly of Rods, starts at equator with anterior & posterior extensionMacula affected very lateSymptoms: Night blindness (most prominent)Visual field concentric contraction- Tubular visionLoss of vision central, 50-60 yrs of age,Associated with Glaucoma, Myopia, Cataract & KeratoconusRETINITIS PIGMENTOSA-FUNDUSSmall jet black pigment cells (Bone corpuscle like) around equator, spreading anteriorly & posteriorlyRetinal veins- sheathing with pigment along the courseTesselated fundus d/t migration of pigment cells upwardsPigments often lie over vessels Retinal vessels extremely narrow/ attenuatedDegeneration of Ganglion cellsOAPale waxy yellow disc (Consecutive OA)

RETINITIS PIGMENTOSA

RETINITIS PIGMENTOSA

RETINITIS PIGMENTOSAProgressive posterior cortical cataract Complete opacification of the lensERG & EOG Subnormal/ Extinguished (Seen earlier than appearance of signs & symptoms; D/D Secondary RP where ERG EOG is subnormal after advanced changes in retina)Condition is genetically determinedMajority Recessive; Consanguity of parentsOccasionally Dominant hereditary (Through generations & is less severe form of the disease)Sex linked (Rare- visual prognosis is very poor)

RETINITIS PIGMENTOSAOther associationsLaurence Moon Biedl Bartum Syndrome (Obesity Hypogoandism, Mental retardation & Polydactyly)Deafness (Ushers Syndrome)Cardiac conduction defectsAbetalipoproteinemia

Treatment Unsatisfactory

RETINOBLASTOMAEpidemiology:Rare, occurring in 1:20 000 childrenLife threatening2/3 of cases within 3 years of life / mean 12/181/3 are bilateralInheritance:Inheritable: Germline mutation - one inherited inactive allele on Chr 13 q14Non-inheritable: Somatic mutation - both alleles are inactivated by a mutation5454RETINOBLASTOMAClinical presentation:LeucocoriaSquintUveitisIris nodules / HeterochromiaAsymptomaticOrbital inflammation & ProptosisSecondary GlaucomaNystagmusVisual impairment5555RETINOBLASTOMAClinical Stages :Quiescent stageGlaucomatous stageStage of extra-ocular extensionStage of Metastasis

Growth patterns:EndophyticExophyticMixed / Diffusely infiltrating

Retinoblastoma, intraocular stage (leukocoria).

Retinoblastoma, intraocular stage

Retinoblastoma, glaucomatous stage

RETINOBLASTOMAPathology:Microscopy:Small polygonal cells Flexner Wintersteiner rosettesDegenerative changes: Necrosis & calcificationGross:Exophytic: growing towards choroidEndophytic: growing towards vitreousSpread: > ON > Brain> Emissary vein > sclera > Orbit6161Flexner-Wintersteiner rosettes in retinoblastoma

RETINOBLASTOMATreatment:EnucleationIntravenous chemoreductionRadiotherapy External beam teletherapy / BrachytherapyPhotocoagulationCryotherapyChemotherapy if metastaticThermochemotherapyPhotodynamic therapy

6464Age-related macular degenerationEpidemiology: Leading cause of irreversible visual loss in the industrialized world Unknown causeRisk factors: Family history, sex (females), race (Caucasians), smoking, age > 50 years.Classification:Non-exudative ( dry ): 10%Exudative ( wet ): 90% of legal blind patients.6565Two types of macular degeneration exist, the "dry" form and the "wet" form. The dry, or nonexudative, form involves atrophic and hypertrophic changes in the retinal pigment epithelium (RPE) underlying the central retina (macula) as well as deposits (drusen) on the RPE. Patients with nonexudative ARMD can progress to the wet, or exudative, form of ARMD, in which abnormal blood vessels called choroidal neovascular membranes (CNVMs) develop under the retina, leak fluid and blood, and ultimately cause a blinding disciform scar in and under the retina.

6767Early stage of AMD with large confluent drusen

Dry AMD with geographic atrophy

Exudative ARMDClinical features:Present with subretinal fluid, pigment epithelial detachments (PED), subretinal lipid, or flecks of subretinal hemorrhage in the affected eye, in addition to RPE changes and drusen. Reduced VA: Choroidal Neovascularisation.Exudative maculopathy.

7070Wet AMD with choroidal neovascularization

Disciform scar

TREATMENTLASER PHOTOCOAGULATION OF THE CNVM REPRESENTS THE BEST-STUDIED AND STANDARD TREATMENT FOR THIS DISORDER.

IVFA : CNV or SRNVM is extrafoveal, juxtafoveal or subfovealExtrafoveal: > or = 200um from FAZ/ ArgonJuxtafoveal: < 200um from FAZ / KryptonSubfoveal: below FAZ / ControversialPost laser recurrence rate of 50% in 2 years 7373Other modalitiesPhotodynamic therapy Intravascular dyes cause vascular occlusion by a photochemical reaction. Transpupillary thermotherapy Transpupillary thermotherapy (TTT) involves slowly heating the subfoveal choroidal neovascular complex with infrared (810 nm) diode laser light to occlude the CNVM Antiangiogenic agents AntiVEGF Intravitreal Macular translocation

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