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229 Cards in this Set
- Front
- Back
ANATOMY: Vitreous
-How much of the eye's volume does the vitreous occupy? -What is the composition of vitreous? -What are the major parts of the vitreous? -Where are the points of firmest attachment of the vitreous? -When does liquefaction of vitreous begin? |
Vitreous
-80% of the eye's volume -Composed of hyaluronic acid, collagen, water -Central (core) vitreous, outer cortical vitreous (denser collagen fibers), anterior/posterior hyaloid, vitreous base -Vitreous base: 2 mm anterior and 3 mm posterior to ora serrata (strongest area of insertion of vitreous) -Other strong points of attachment: retinal vessels, optic n, macula -Liquefaction of vitreous begins as early as age 2 (premacular vitreous) --> produces precortical vitreous pocket |
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ANATOMY: Retina - Macula
-What are the histologic and anatomic definitions of the macula? -What causes the yellow color of the macula and what is the significance of the yellow color? -Define the size/location of the fovea, foveola, parafoveal area and perifoveal area |
Retina - Macula
-Anatomically: macula lutea (yellow spot) --> portion of posterior retina containing xanthophyll pigment -Histologically: area of retina with 2 or more layers of ganglion cells (5-6 mm diameter area between temporal arcades) -Yellow pigmentation is secondary to oxygenated carotenoids (lutein, zeaxanthin) --> causes hypofluorescence of this area during IVFA -Fovea: central 1.5 mm of macula -Foveola: central pit, 0.35 mm diameter, with slender and densely packed cones -Parafovea: 0.5 mm width ring surrounding fovea (GCL, INL, OPL are thickest here) -Perifovea: 1.5 mm wide area outside parafovea |
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Anatomy: Retina
-What are the layers of the retina (from innermost to outermost)? |
Retina layers
-ILM (true membrane) -NFL -GCL -Inner plexiform layer -Inner nuclear layer -Outer plexiform layer -Outer nuclear layer -ELM (not true membrane) -Photoreceptor inner/outer segments |
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RETINA: Anatomy
-What types of cones are in the fovea and what is the cone density in this area? -What types of cells are in the central fovea? -Which area contains the greatest density of rods? |
Retina
-Fovea contains red-green cones, density 140,000 cones/mm3 (NO rods) -Central fovea: contains only cones and supporting Muller cells -Rods: greatest density in zone 20 degrees from fixation |
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RETINA: Anatomy
-What are the light sensitive molecules in rods/cones derived from and what are they bound to? -Where are these molecules located and how are they removed? -What is the protein/gene responsible for removal of these molecules? -What disease results if this gene is defective? |
Retina
-Light sensitive molecules in photoreceptors are derived from vitamin A -Molecules are bound to an apoprotein (called opsin) -Located in photoreceptor outer segments -Removed by shedding from outer retina --> phagocytosed by RPE -ATP-binding cassette transporter of retina (ABCR) encoded by ABCA4 gene is involved in transport of the molecules to be degraded -If deficient --> Stargardt disease |
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RETINA: Anatomy
-What type of depolarizing response do photoreceptors exhibit with stimulation? -What is the ratio of synapses between cones: bipolar cells and rods:bipolar cells? -What do bipolar cells synapse with and what other cells are involved in generating action potentials to conduct to brain? |
Retina
-Photoreceptors depict GRADED response to depolarization --> proportional to amount of stimulating light -Cones: 1:1 synapse with bipolar cells (midget bipolar cells) -Rods: sometimes up to 100 rods can synapse with a single bipolar cells -Bipolar cells synapse with ganglion cells --> ganglion cells summate responses from amacrine cells and bipolar cells to develop action potentials |
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RETINA: Anatomy
-What makes up the ILM? -What makes up the ELM? -Which of these is a true membrane? -Which cells course through almost the entire thickness of the retina? |
Retina
-ILM: composed of footplates of Muller cells (true membrane) -ELM: composed of zonular attachments between photoreceptors and Muller cells -Muller cells course through almost entire thickness of retina |
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RETINA: Anatomy
-What is a cilioretinal artery? -Which artery is the CRA derived from? -What percent of O2 used by the fundus is supplied by the CRA? What supplies the rest? |
Retina
-Cilioretinal artery: retinal vessel derived from ciliary circulation, occasionally present, supplies portion of inner retina b/w optic n and macula -CRA derived from ophthalmic artery (first branch) -Retinal vessels supply 5% of O2 used by fundus; choroidal circulation supplies the rest |
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RETINA: Anatomy
-RPE: Location, cell type, cellular origin -What anterior segment structure is the RPE contiguous with? -How is the RPE different in the macula compared to periphery? |
RPE
-Single layer of hexagonal shaped cuboidal cells, neuroectodermal origin, located b/w Bruch's membrane and retina -Contiguous with ciliary body pigmented epithelium -Macular RPE: taller, denser cells with more pigment |
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RETINA: Anatomy
-What are the 6 functions of the RPE? -What part of the RPE cell functions in light absorption? -What type of light is absorbed best? -What time of day do rods shed discs and what time do cones do so? |
RPE
-Absorbs light -Maintains sub-retinal space -Forms outer blood-ocular barrier -Phagocytoses rod/cone outer segments -Retinal/poly-unsaturated fat metabolism -Heals and forms scar tissue -Melanosomes: RPE cell component that functions in light absorption (blue light absorbed more than red) -Rods shed discs at dawn, cones shed them at dusk |
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RETINA: Anatomy
-What happens to Bruch's membrane over time? -What happens to Bruch's membrane in pseudoxanthoma elasticum? |
Bruch's Membrane
-Lipids, oxidatively damaged materials build up in Bruch's throughout life -Pseudoxanthoma elasticum: increased fragility of Bruch's --> breaks/cracks develop that radiate outward from optic disc (resemble blood vessels --> called angioid streaks) |
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RETINA: Anatomy
-What vessels supply the choroidal circulation? -What are the 2 layers of choroidal vessels and how are they different? -Where do vortex veins leave the eye? What vessel do they drain into? -What are RPE cells exposed to more than any other cell population as a result of high choroidal blood flow? -What is the function of choroidal melanocytes? |
Choroid
-Circulation supplied by short posterior ciliary arteries -Haller layer: outermost, largest vessels -Sattler layer: smaller diameter vessels -4-5 vortex veins leave the eye at the equator --> drain into superior ophthalmic vein -Choroid has highest blood flow of any tissue --> RPE cells lying over choriocapillaris are exposed to highest O2 tension of any perfused tissue -Choroid contains melanocytes --> absorb excessive light transmitted through retina/RPE |
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RETINA: IVFA
-What is the molecular weight of fluorescein? -How is fluorescein eliminated from body? How long does it take? -What % of fluorescein is protein-bound? Is the bound or unbound portion visible with IVFA? -At what wavelength does fluorescein fluoresce? What wavelength is needed to excite it? -What type of light does fluorescein emit after excitation? |
IVFA
-Molecular weight: 376 daltons -Eliminated through liver/kidneys within 24-36 hrs -80% protein bound; 20% free (only free portion is visualized) -Fluoresces at wavelength of 520-530 (green) --> emits yellow-green light -Excites by light of wavelength 465-490 (blue) |
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RETINA: IVFA
-During which phase do the retinal and choroidal vessels fill? -What is the order of each phase of the IVFA and what happens during each phase? -When is the peak phase of fluorescence? |
IVFA
-Retinal/choroidal vessels fill during transit phase (lasts 10-15 seconds) -Choroidal phase -Arterial phase (arteries fill) -Arteriovenous phase (complete filling of arteries and capillaries, laminar filling of retinal veins) -Peak phase of fluorescence occurs 1 minute after dye injection --> greatest visualization of foveal detail -Late phase: staining of Bruch's, choroid, sclera |
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RETINA: IVFA
-What is autofluorescence? examples? -What are 2 causes of hypofluorescence? |
IVFA
-Autofluorescence: caused by naturally highly reflective substances (i..e, drusen) -Hypofluorescence: secondary to vascular filling defect (nonperfusion of artery, vein, capillary) OR blockage of fluorescence by presence of fibrous tissue, pigment or blood |
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RETINA: IVFA
-What are 5 major patterns of hyperfluorescence? -Describe appearance of each pattern |
IVFA - Hyperfluorescence patterns
-Leakage: gradual, marked increased fluorescence throughout angiogram --> borders become increasingly blurred, greatest intensity in late phase (i.e., CNV, leaking telangiectatic vessels) -Staining: fluorescence gradually increases in intensity, persists in late views ut borders remain fixed (i.e., scar, drusen, optic nerve tissue, sclera) -Pooling: accumulation of fluorescein in fluid-filled choroidal space; no dye seen in beginning of angiogram --> as dye leaks into space, margins of space trap fluorescein and appear distinct -Transmission/window defect: view of normal choroidal fluorescence through defect in pigment or loss of RPE pigment --> seen early (corresponding to choroidal filling), greatest intensity at peak of choroidal filling, fades in late phases |
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RETINA: IVFA
-What are side effects of IVFA? -What happens if dye extravasates into skin during injection? |
IVFA
-Side effects: temporary yellowing of skin, conj, urine -N/V, vasovagal reactions occur in 10% of pts -Rare anaphylactic or anaphylactoid (1%) reactions -Extravasation of dye into skin: local pain --> treat with ice cold compresses -Avoid in pregnant women -Fluorescein is transmitted in breast milk |
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RETINA: ICG
-What is the molecular weight of ICG? -What percent is protein bound? -What is ICG ideal for imaging? -What is the significance of a plaque on ICG? -What are focal hot spots on ICG? -Name 7 indications for ICG. |
ICG
-Molecular weight 775 daltons -98% protein bound (limited diffusion through choriocapillaris) -Ideal for imaging choroidal circulation -Plaques: late-staining vessels corresponding to occult CNV -Focal hot spots: indicate retinal angiomatous proliferation and polypoidal vasculopathy (CNV variants) -Indications for ICG: CNV, pigm epith detachment, polypoidal choroidal vasculopathy, retinal angiomatous proliferation, ICSC, intraocular tumors, choroidal inflammation |
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RETINA: ICG
-What allergies should you be aware of when administering ICG? -What are contraindications to ICG? |
ICG
-Use with caution in pts with iodine or shellfish allergy (contains 5% iodide) -Contraindications: liver disease, use of Metformin |
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RETINA: OCT
-What is the basis of OCT imaging? -What determines the brightness/darkness of tissue on OCT? -How does the retinal thickness analyzer compute thickness? |
OCT
-Based on imaging reflected light -Tissues with higher reflectivity (i.e., RPE) appear in brighter colors (red/white); less dense tissues (vitreous, intraretinal fluid) appear darker (blue/black) -Retinal thickness analyzer: identifies location of NFL and RPE at each point in scanned area --> calculates differences to determine retinal thickness |
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RETINA: ERG
-What type of response does an ERG evoke? -What are 5 different types of responses and does each require a dark or light adapted state? -What is the configuration and cell of origin of the a-wave and the b-wave? |
ERG
-Mass response evoked from entire retina with brief flash of light? -"Rod resopnse" (dark-adapted) -Maximal combined response (dark-adapted) -Oscillatory potentials (dark-adapted) -single flash "cone response" (light adapted) -30-Hz flicker response (light adapted) -A-wave: negative waveform, generated by photoreceptors -B-wave: ositive waveform generated by Muller and bipolar cells |
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RETINA: ERG
-How is the rod response (scotopic) ERG produced? -What is the typical ERG appearance in a normal eye? -How is the maximal combined response produced? -What is the ERG appearance? |
ERG
-Scotopic ERG: dark-adapt pt for 20 minutes --> stimulate retina with brief flash of dim light below cone threshold -ERG: prominent B wave, almost no A wave -Maximal combined response: dark adapt the pt --> stimulate with bright light flash to maximally stimulate both rods and cones -ERG: large A and B waves, oscillatory potentials superimposed on ascending B wave |
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RETINA: ERG
-How are oscillatory potentials produced? -When are they reduced? -How is a photopic (single flash cone response) ERG produced? -Why does a 30 Hz flicker response filter out rod responses so only cone responses are tested? |
ERG:
-Oscillatory potentials are isolated by filtering out slower ERG components -They are reduced in retinal ischemic states and some forms of congen stationary night blindness -Photopic ERG obtained by maintaining pt in light adapted state to suppress rods --> then stimulate retina with bright white flash -30 Hz flicker test screens out rod responses b/c rods only respond to stimulus up to 20 Hz |
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RETINA: ERG
-What is an early receptor potential (ERP), how is it evoked? -What retinal structure is responsible for producing a C-wave and when is it seen? -What percent of human ERG amplitude is generated by cones? |
ERG
-ERP: small response with no latency occuring before A wave, evoked by intense stimulus flash -C-wave: generated by RPE, occurs 2-4 seconds after stimulus |
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RETINA: ERG
-What is a foveal ERG? -When is it useful? -What is a multi-focal ERG? -When is it useful? |
ERG
-Foveal ERG: rods suppressed by presenting bright light to retina --> f/b stimulation of only foveal/parafoveal cones -Used when physical findings do not clearly correlate with loss of VA -Multi-focal ERG: tests cone-generated responses subtending 25 deg radially from fixation -Used to determine objectively whether macular dysfunction present (i.e., chloroquine toxicity) |
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RETINA: ERG
-What is a bright-flash ERG and when is it useful? -What is a pattern ERG and when is it useful? |
ERG
-Bright flash ERG: uses flash stimulus that is brighter than usual in eyes with opaque media -Allows judgement of retinal function in eyes with poor view of retina --> if unrecordable, suggests widespread retinal damage (no direct info about VA or optic nerve integrity) -Pattern ERG: alternating checkerboard stimulus presented to central retina -Correlates with optic n integrity; may be useful for recognition of early glaucoma but many confounding factors |
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RETINA: ERG
-What are 4 uses for ERG? |
ERG
-Diagnosing/following retinal dystrophies/degenerations -Evaluation of macular disease, determine if pathology limited to macula -Distinguish between retinal damage caused by diffuse vs focal process -Evaluation of chronic ischemic damage from vascular disease (i.e., inversion of B:A wave ratio or delay in 30Hz flicker response is an ominous sign in CRVO) |
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RETINA: EOG
-What is a normal light-dark EOG ratio (Arden ratio)? -What cells (rods or cones) dominate EOG amplitude? -What is EOG most specific for? -When is EOG most significantly reduced? |
EOG
-Arden ratio normally 1.85 or above (if < 1.85 --> subnormal, if < 1.3 --> severely subnormal) -Rods dominate EOG amplitude -EOG most specific for involvement of RPE when other studies show normal functioning retina (not useful test if retina itself is damaged) -Severely reduced in Best disease --> use to evaluate any yelow lesions or macular scars simulating Best disease |
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RETINA: VEP
-What is a VEP? -What type of stimulus is preferred when measuring VEPs? -What areas of visual function do VEPs test? |
VEP
-VEP: electrical signal generated by occipital cortex in response to stimulation of retina by light flashes or patterned stimuli -Best stimulus: checkerboard pattern (occipital cortex very sensitive to sharp edges and contrast; less sensitive to diffuse light) -VEP abnormality can reflect abnormality anywhere from retina to cortex -Often used to determine macular function (as most of visual cortex represents macular area of eye) |
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RETINA: VEP
-What are 5 indications for VEP? |
VEP - Indications
1) confirm diagnosis of optic neuropathy/demyelinating disease 2) assess misprojection of optic nerve fibers (i.e., in albinism) --> asymmetric VEPs between L and R) 3) Estimate VA in infants/nonverbal children (use checkerboard stimulus of decreasing size) 4) Detect/locate VF defects by comparing response to stimuli in different locations 5) Eval potential for reasonable VA in pts with opaque media |
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MACULA: ICSC
-Epidemiology? -Pathophysiology? -Symptoms? -Associations? |
ICSC
-Healthy men 25-55 yo; rare in AA pts -Altered barrier/pumping functions of RPE --> impaired fluid removal --> serous RD of sensory retina -Sx: blurry/dim vision, scotoma, dec color (usually VA > 20/30; can be corrected w/ hyperopic correction) -Assoc: type A personality, hypochondria, hysteria, neurosis, elevated steroid level |
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MACULA: ICSC
-3 most common IVFA patterns? -Which is most common and least common? |
ICSC
-Expansile dot: most common; small focal hyperfluorescent leak from choroid (early phase) --> increased size/intensity as FA progresses -Smokestack: least common (10%); central hyperfluorescent spot --> spreads vertically, then laterally (like smokestack) -Diffuse: no obvious leakage pattern on FA --> pts usually have large serous RD and extensive RPE changes |
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MACULA: ICSC
-Name 3 other tests that can be used to evaluate ICSC |
ICSC
-OCT: to follow, document resolution of SRF -Autofluorescence: hypoautofluorescence corresponding to site of focal RPE leak on IVFA -ICG: choroidal arterial/venous filling delays, venous dilation, hyperpermeability of vessels, multifocal hyperfluorescent patches early |
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MACULA: ICSC
-Name 4 diseases in the DDx of ICSC and ways to distinguish between them |
ICSC
-CNV assoc w/ AMD (large area of leakage as opposed to pinpoint) -Optic nerve pits (no pinpoint leak) -Idiopathic polypoidal choroidal vasculopathy (saccular outpouchings) -Idiopathic uveal effusion syndrome |
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MACULA: ICSC
-Prognosis? -Treatment options? -Criteria for consideration of laser photocoagulation? |
ICSC
-Prognosis: 80-90% spontaneous resolution in 3-4 months; poorer prognosis in chronic, recurrent cases or bullous CSC -40-50% have >/= 1 recurrence -Rx: laser --> rapid remission but no difference in final visual outcome long term -PDT: experimental -Criteria for laser: 1) lasts > 3-4 months, 2) need prompt restoration of vision, 3) recurrence in setting of pre-existing visual deficits from previous episodes, 4) permanent visual deficit in fellow eye, 5) chronic signs (cystic changes, widespread RPE changes) |
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MACULA: Optic pit maculopathy
-Appearance? Location? -What is the associated macular pathology? Treatment? |
Optic pit maculopathy
-Small, hypopigmented, yellow/white, round excavated defects in inferior temporal portion of cup -Causes serous macular RD (extends from disc to macula) -Rx: laser along edge of optic n (barrier to fluid migration) vs intraocular gas injection |
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MACULA: AMD
-Epidemiology of exudative vs nonexudative AMD? -Describe normal aging changes in: photoreceptors, RPE, choriocapillaris -Where are basal laminar and basal linear deposits found, respectively? -Risk factors for AMD? |
AMD
-85-90% of cases nonexudative; 10-15% exudative -Normal aging changes: 1) photoreceptors dec density/distribution; 2) RPE: loss of melanin, formation of lipofuscin granules, basal laminar deposits; 3) involution of choriocapillaris -Basal laminar deposits: lipid-rich material b/w plasma membrane of RPE (basal lamina) and inner aspect of RPE BM -Basal linear deposits: b/w RPE BM and choriocapillaris BM (i.e., within Bruch's membrane) -Risk factors: AGE!, +FHx, smoking, HTN, hyperlipidemia, cardiovasc disease, female, hyperopia, light iris color |
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MACULA: Dry AMD
-Hallmark finding? Other findings? -IVFA of PED? -Size of small vs large drusen? -Risk of progression to adv AMD in small/int drusen? many int or large drusen? -Among hard, soft or confluent drusen, which are more likely to progress to adv AMD? |
Dry AMD
-Hallmark finding: drusen; can also see geographic atrophy and RPE hyperpigmentation -PED: caused by thickening of Bruch's with drusen --> separation of RPE from Bruch's --> area fills rapidly with fluorescein on IVFA (pooling) -Small drusen: <64 um diameter; large: >125 um diameter -If many small/few int drusen: 1.3% risk of progression -If many int/large drusen: 18% risk of progression -Soft and confluent drusen more likely to progress than hard drusen |
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MACULA: Dry AMD
-What are 3 RPE abnormality patterns in dry AMD? -What happens to photoreceptors in areas of RPE atrophy? -IVFA patterns of atrophy vs hyperpigmentation of RPE? |
Dry AMD
-3 RPE patterns: 1) attenuation/atrophy of RPE in contiguous areas --> geographic atrophy; 2) non-geographic atrophy; 3) focal hyperpigmentation (inc risk of progression) -Photoreceptors overlying atrophic areas usually attenuated/absent --> vision loss -IVFA: atrophy shows WINDOW defect; hyperpigmentation shows BLOCKAGE |
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MACULA: Dry AMD
-What are regressed drusen? -What are calcified drusen? -Causes of hyperfluorescent lesions on IVFA -Causes of hypofluorescent lesions on IVFA |
Dry AMD
-Regressed drusen: disappearance of material that makes up drusen -Calcified drusen: pinpoint glistening areas within atrophy -Hyperfluorescence: drusen, atrophy, CNV, PED, laser scars, subretinal fibrosis, RPE tear -Hypofluorescence: hemorrhage, lipid, pigment (blockage) |
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MACULA: Dry AMD
-Name 4 diseases in the DDx of dry AMD -How does a preferential hyperacuity perimeter work? |
Dry AMD
-DDx: CSC (no drusen, RPE atrophy or PED), basal laminar/cuticular drusen ("starry night" on IVFA, vitelliform appearance of macula), drug toxicity (plaquenil --> mottling RPE) -Preferential hyperacuity perimeter: detects CNV to differentiate b/w neovasc and non-neovasc AMD -How it works: RPE elevation --> geometric shift of photoreceptors --> diff set of photoreceptor fields stimulated --> perception that dots in a line are in a different position relative to true location in space |
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MACULA: Dry AMD
-What 4 nutrients were found to decrease progression of AMD by AREDS study? -Which should be avoided in smokers? -Which pt population is this vitamin helpful for? |
Dry AMD
-AREDS: vitamin C (500 mg), vitamin E (400 IU), beta carotene (15 mg), zinc (80 mg) --> avoid beta carotene in smokers (increases risk of lung ca) -Most helpful for pts with intermediate or advanced unilateral AMD (25% dec risk of progression, 19% risk reduction in mod visual loss rate) -No improvement in pts with no/early AMD - |
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MACULA: Dry AMD
-What is the grading scale developed by AREDS? -Name 4 characteristics of individuals at high risk of progression of AMD, vision loss |
Dry AMD
-AREDS grading scale: 1 or more large drusen (1 pt), pigment abnormalities (1 pt), bilateral intermed drusen, no large drusen (1 pt), neovasc AMD (2 pts) --> more points = higher risk of progression at 5 and 10 yrs -High risk of progression: 1) extensive intermediate drusen, 2) at least 1 large druse, 3) noncentral geographic atrophy, 4) adv AMD in one eye -Consider supplementation for these pts! |
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MACULA: Dry AMD
-What other molecules may be helpful for treatment of AMD? -What lifestyle changes are helpful? -What are 2 experimental treatments undergoing evaluation for AMD? |
Dry AMD
-Molecules studied for effectiveness in AMD: xanthophylls (lutein, zeaxanthin --> members of caroteinoid family, concentrated highly in macula), omega 3 fatty acids, eicopentaenoic acid -Lifestyle changes: obesity reduction, smoking cessation -2 experimental therapies: rheopheresis (membrane filtration to remove high density macromolecules), anecortave acetate ie. Retaane (angiostatic steroid with little mineralocorticoid or glucocorticoid activity) |
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MACULA: Wet AMD
-Hallmark lesion? How does it form? -Sx/signs? -Gold standard for diagnosis? |
Wet AMD
-Hallmark: CNV -Buds of neovasc tissue from choriocapillaris perforate outer Bruch's membrane --> proliferate within Bruch's and disrupt choriocapillaris, RPE and retina architecture -Sx: sudden dec VA, metamorphopsia, paracentral scotoma -Signs: elevation of retina, subretinal/intraretinal fluid/lipid/blood; PED; gray-green CNV lesion, CME, sea-fan pattern of subretinal vessels -Gold standard for diagnosis: IVFA |
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MACULA: Wet AMD
-What are 2 angiographic patterns of CNV? Describe differences between each |
Wet AMD
-Classic CNV: uniform, bright hyperfluorescence in early phase --> progressively intensifies --> dye leakage obscures boundaries in late phases -Occult CNV: 1) fibrovascular PED: irregular RPE elevation, stippled/granular fluorescence seen early with prorgessive leakage later; 2) late leakage from undet source: late phase fluorescence at RPE level, do not correspond to classic CNV or areas of RPE elevation |
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MACULA: Wet AMD
-For which type of CNV is laser helpful? PDT? -Which type can be observed? -Difference b/w serous PED and fibrovascular PED? -Define predominantly CLASSIC CNV |
Wet AMD
-PDT: for predominantly classic or purely occult CNV -Laser: for classic CNV only -Many occult with NO classic CNV lesions may be observed (do not progress) -Serous PED: smooth, dome shaped area with RAPID early homogenous filling on IVFA (retains boundaries and intensity throughout study) -Fibrovascular PED (occult CNV): irregular RPE elevation, stippled/nonhomogenous pattern of fluorescence, SLOW filling rate -Pedominantly classic CNV: CNV more than 50% of lesion and classic CNV occupies more than 50% of entire lesion |
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MACULA: Wet AMD
-Describe difference between "classic" and "occult" CNV vs "poorly defined" and "well defined" CNV -Why is this distinction important? |
Wet AMD
-Classic/occult classifications describe IVFA patterns -Poor/well defined describe how distinct boundaries are b/w entire CNV lesion and uninvolved retina --> important for laser treatment (should be applied to entire CNV area, can only be done if well demarcated boundaries) |
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MACULA: Wet AMD
-Name 6 conditions that mimic wet AMD |
Wet AMD
-Retinal arterial macroaneurysms: can have PRH, SRH, IRH --> can see heme surrounding macroaneurysm on IVFA or ICG -Adult vitelliform dystrophy: may look like PED or confluent drusen; staining of vitelliform material assoc with blocked fluorescence early in IVFA; good VA (unlike AMD) -Polypoidal choroidal vasculopathy: multiple, recurrent RPE detachments; more frequently assoc with VH than AMD, looks like peripapillary multifocal orange and nodular lesions, no drusen present -Choroidal tumors -ICSC -Inflammatory conditions (VKH, sarcoid, SLE,etc) |
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MACULA: Wet AMD
-Indications for thermal laser? -Findings of Macular Photocoagulation Study? -Will laser tx improve vision? -Highest risk of recurrence with which 3 situations? |
Wet AMD
-Laser: CNV with well demarcated boundaries (classic or classic/occult), extrafoveal/juxtafoveal locations (NOT subfoveal!) -MPS: treatment did not decrease pt's risk of maintaining good VA -Laser may decrease risk of additional severe vision loss, but does not improve vision -Highest risk of recurrence: 1) fellow eye shows active CNV/scar, 2) treatment fails to cover entire lesion; 3) photocoag not as intense as moderately white treatment standard |
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MACULA: Wet AMD
-How does PDT work? -Findings of TAP study? -Findings of VIP study? -What are the FDA-approved indications of PDT? |
Wet AMD
-PDT: systemic administration of photosensitizing drug --> application of light to affected tissue to incite local photochemical rxn --> creation of reactive O2 species --> capillary endothelial damage, thrombosis -TAP: PDT vs placebo for subfoveal CNV --> PDT pts less likely to suffer at least moderate vision loss at 1 and 2 yrs, predominantly classic CNV derived greatest benefit -VIP: PDT vs placebo for subfoveal CNV in AMD --> at 2 yrs, PDT eyes less likely to have mod/severe vision loss, greatest benefit for occult CNV w/o classic component OR lower baseline VA -In VIP study, lesions > 4 DD size, baseline vision > 20/50 had worse outcome with PDT -FDA approved indications: predominantly classic CNV with AMD, pathologic myopia, ocular histoplasmosis |
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MACULA: Wet AMD
-Why is VEGF thought to play a role in CNV? -What are the functions of VEGF? -Which chromosome is the VEGF gene on? Which isoform is most pathologic? |
Wet AMD
-VEGF: expression is increased in RPE cells in early AMD --> thought to initiate neovasc; high VEGF concentrations excised from CNV in AMD pts -Functions of VEGF: induce angiogenesis, vascular permeability, lymphangiogenesis, survival factor for endothel cells (prevents apoptosis) -VEGF gene on ch 6p21.3 -VEGF165 isoform most pathologic |
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MACULA: Wet AMD
-What is pegaptanib? -VISION trial results? |
Wet AMD
-Pegaptanib: binds VEGF165 with high affinity, specificity; preventsVEGF165 and larger isoforms from binding VEGF receptors; does not target all active VEGF-A isoforms -VISION: pegaptanib vs placebo in subfoveal CNV --> 70% loss <3 lines vision compared with 55% of placebo --> poor drug b/c pts still lost vision |
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MACULA: Wet AMD
-What is ranibizumab? -MARINA trial results? -ANCHOR trial results? |
Wet AMD
-Ranimizumab: antibody fragment (Fab) that binds to and inhibits all active forms of VEGF-A and degradation products -MARINA: ranibizumab vs placebo for subfoveal CNV --> 95% lucentis pts had visual improvement/stabilization compared to 62% of placebo; 40% experienced improved vision by 15 letters compared to placebo -ANCHOR: lucentis VS PDT for subfoveal CNV --> 95% lucentis pts maintained/improved VA compared w/ 64% of PDT pts after 1 yr -Both studies looked at monthly dosing of lucentis |
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MACULA: Wet AMD
-What is bevacizumab? -What 2 upcoming studies are looking at combination treatment? What treatments are being evaluated by each? |
Wet AMD
-Bevacizumab: full length monoclonal antibody to VEGF, initially used for colorectal cancer, used IV and intravitreal for AMD (off label), has 2 Ag binding domains (lucentis has only 1) -VERITAS: PDT + triamcinolone, PDT + Macugen -FOCUS: PDT + lucentis for predominantly classic CNV |
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MACULA: OHS
-Epidemiology? -Disease transmission? -4 clinical signs of OHS? -Sx? |
Ocular Histoplasmosis (OHS)
-Mississippi and Ohio river valleys -Carried on bird feathers, bats --> inhaled --> systemic dissemination --> eyes -4 clinical signs: 1) histo spots (punched out chorioretinal lesions), 2) juxtapapillary atrophic pigmentary changes, 3) NO vitritis, 4) CNV -Sx: asymptomatic until CNV develops; vision loss, metamorphopsia, paracentral scotoma --> need IVFA! |
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MACULA: OHS
-What did MPS show re: laser treatment of OHS? -Which type of CNV does not benefit from laser? -VOH study results? |
OHS
-MPS showed that laser treatment of extrafoveal CNV in OHS reduced risk of vision loss at 5 ys to 9% (44% risk if no treatment) -Laser involving foveal center for new/recurrent CNV does not benefit in OHS -VOH: case series of pts treated with PDT --> PDT may limit vision loss in eyes with subfoveal CNV and OHS (FDA approved for this indication) |
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MACULA: Angioid streaks
-What are angioid streaks? -IVFA appearance? -What is the main vision-limiting effect? |
Angioid streaks
-Dark red to brown bands radiating from optic n head --> represent discontinuities or breaks in a thickened/calcified Bruch's membrane -IVFA: look hyperfluorescent (overlying RPE is atrophic) -Can result in CNV --> vision limiting |
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MACULA: Angioid streaks
-How is pseudoxanthoma elasticum inherited? What gene? -Fundus findings? -4 other diseases assoc with angioid streaks? -What condition are eyes susceptible to following trauma? -Treatment? |
Pseudoxanthoma elasticum
-Auto recessive; ABCC6 gene (ch 16) -Fundus: disc drusen, peripheral round, atrophic scars, peau d'orange (stippled) fundus appearance, angioid streaks -Other diseases with angioid streaks: Ehlers-Danlos, Paget disease of bone, sickle cell anemia, beta thalassemia -Eyes susceptible to choroidal rupture following trauma --> safety glasses -Treatment: similar to AMD; no preventative treatment available |
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MACULA: Pathologic myopia
-What % of population is myopic? pathologically so? -How is high vs pathologic myopia classified? -Fundus findings? |
Pathologic myopia
-25% US population is myopic; 2% is high/pathologic -High myopia = > -6 D or axial length > 26.5 -Pathologic myopia = >-8 D or axial length > 32.5 -Fundus: tilted disc, peripapillary chorioretinal atrophy, lacquer cracks (spontaneous rupture of Bruch's elastic lamina - linear or stellate pattern), Forster-Fuchs spots (subretinal/intraretinal RPE hyperplasia), posterior staphyloma, lattice/cystoid degen, retinal thinning/hole, CNV |
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MACULA: Pathologic myopia
-Risk of CNV? -VIP Pathologic Myopia trial results? -What is expanding RPE atrophy? |
Pathologic myopia
-5-10% risk of CNV in eyes with axial length > 26.5 -VIP trial: PDT alone or in combination with other tx recommended for reducing vision loss in myopia-related CNV -Use for juxtafoveal/subfoveal lesions --> scar creep may involve fovea if laser used -Expanding RPE atrophy may develop around laser lesions --> can encroach onto fovea and cause vision loss (higher risk in pathologic myopia pts) |
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MACULA: ERM
-What is an ERM? Cellular composition? Location? -Difference b/w idiopathic and secondary ERM? |
ERM
-Semitranslucent, avascular fibrocellular membrane along ILM -Usually located in fovea or adjacent to fovea -Composed of RPE and glial cells (astrocytes, Muller cells) -Idiopathic: usually related to PVD, usually asymmetric and in pts > 50 yo -Secondary: due to retinal vascular occlusions, inflammation, surgery, trauma |
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MACULA: ERM
-Clinical appearance? (early vs late appearance, vascular changes, macular edema) |
ERM
-Initially: mild sheen/glint on retinal surface --> become more reflective, thick and opaque (obscure retinal details) --> cellophane maculopathy (contracture of ERM produces distortion/wrinkling of retinal surface, radiating striae) -Late: macular pucker (severe distortion/wrinkling) -Increased vascular tortuosity and straightening of retinal vessels 2/2 traction -CME can result |
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MACULA: ERM
-Treatment? -Critera for surgery? |
ERM
-Rarely spontaenously detaches --> resolution of sx -Most cases: mild sx, no surgery -Severe cases: VA < 20/50, intolerable distortion --> surgical removal by vitrectomy -50-75% of pts have improved (but not fully normal) VA after surgery |
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MACULA: Vitreomacular traction syndrome
-How does VMT occur? -What happens to the macula in VMT? |
VMT
-Caused by incomplete vitreous separation from the macula --> abnormal adherence of vitreous --> traction on macula -Macula may become distorted, cystic or detached; no ERM will be seen -OCT to follow/diagnose -Spontaneous resolution vs need surgery |
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MACULA: Macular holes
-Epidemiology? -Stage 0? -Stage 1? Prognosis? -Stage 2? -Stage 3? typical VA? -Stage 4? How differentiated from stage 3? |
Macular holes
-6-8 decade of life; F>M; younger age in myopic eyes -Caused by tractional forces assoc with perifoveal vitreous detachment -Stage 0: perifoveal vitreous detachment, subtle changes in macular topography, normal VA --> most do not progress -Stage 1: central vision loss, metamorphopsia; 1A (loss of foveal depression, small yellow spot), 1B (yellow ring in foveal center) --> horizontal splitting on OCT -Prognosis of stage 1: 50% resolve spontaneously when vitreous separates from retina (resolves traction) -Stage 2: progression of foveal pseudocyst to full thickness dehiscence --> posterior hyaloid STILL attached to foveal center -Stage 3: full thickness hole with rim of thickened, elevated retina; posterior hyaloid attached to disc but not to macula, +/- operculum; VA 20/200 -Stage 4: fully developed macular hole with Weiss ring (PVD) |
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MACULA: Macular hole
-IVFA appearance of stage 2,3,4 holes? -Gold standard for eval of macular holes? -What stage macular hole should be considered for surgery? |
Macular hole
-IVFA: shows circular transmission defect (secondary to loss of xanthophyll at site of hole, RPE atrophy in base of hole) in stage 2,3,4 holes -OCT: gold standard for eval of macular hole -Stage 1 holes have high rate of spontaneous resolution --> no surgery -Stages 2,3,4 unlikley to resolve spontaneously so consider surgery |
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MACULA: Valsalva retinopathy
-Pathophysiology? -Location of heme? -Prognosis? -DDx? |
Valsalva retinopathy
-Sudden rise in intrathoracic or intraabdominal pressure --> inc intraocular venous pressure --> superficial macular capillaries rupture --> hemorrhage under ILM -Can have vitreous and SRH also -Good prognosis -Must r/o peripheral tear or aneurysm along arteriole |
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MACULA: Purtscher retinopathy
-Causes of Purtscher and Purtscher-like retinopathy? -Fundus appearance? -IVFA? -Consequences for vision? -Pathophysiology? |
Purtscher retinopathy
-Causes: acute compressive injuries to head/thorax --> injury-induced complement activation --> occlusion of small arterioles -Purtscher-like: acute pancreatitis, fat embolism, amniotic fluid embolism, collagen vascular disease, childbirth -Fundus: large CWS, heme and retinal edema peripapillary location -IVFA: arteriolar obstruction, leakage -Vision loss, optic atrophy (may be permanent) |
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MACULA: Terson syndrome
-What is the clinical appearance and pathophysiology of Terson syndrome? -Visual consequence? |
Terson syndrome
-Abrupt intracranial hemorrhage --> acute rise in intraocular venous pressure --> ruptured peripapillary and retinal vessels --> vitreous, sub-ILM or subhyaloid heme -No visual consequence once hemorrhage clears |
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VASCULAR: HTN
-What BP defines HTN? -Which vesels are affected in hypertensive retinopathy? -What are FIPTs? -What are chronic HTN retinal lesions? Which of these are signs of ischemia? |
HTNsive retinopathy
-BP > 130/85 -HTN affects precapillary arterioles and capillaries -FIPTs: focal intraretinal periarteriolar exudates --> at precapillary level (smaller, less white than CWS); seen w/ acute HTN -Chronic HTN lesions: IRMAs, dot/blot heme, microaneurysms, hard exudates, venous beading, neovascularization (last 2 assoc w/ ischemia) |
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VASCULAR: HTN
-What is the utility of focal arteriolar narrowing, AV nicking in classification of HTN? -What is the IVFA appearance of FIPTs? -Why do coexisting HTN and DM cause more severe retinopathy? |
HTNsive retinopathy
-Focal arteriolar narrowing, AV nicking have little predictive value for actual HTN -IVFA of FIPTs: punctate hyperfluorescence -DM affected capillaries & HTN affects precapillaries --> insult to both precapillaries and capillaries acts in combination |
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VASCULAR: HTN
-What patient population gets HTNsive choroidopathy? -What are the 2 characteristic choroidal lesions? -IVFA appearance? |
HTNsive retinopathy
-Young pts w/ acute HTN episodes (pre-eclampsia, pheochromocytoma, renal HTN) -Elschnig spots: tan, lobule-sized patch --> over time becomes hyperpigmented, surr by margin of hypopigmentation -Siegrist streaks: linear hyperpigmented lesions (follow meridional course of choroidal arteries) -IVFA: focal choroidal hypoperfusion early --> multiple subretinal areas of leakage late |
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VASCULAR: HTN
-What are characteristics of HTNsive optic neuropathy? -Does HTNsive optic neuropathy depend more on severity or chronicity of HTN? |
HTNsive retinopathy
-Optic neuropathy: peripapillary flame heme, blurred disc margins, florid disc edema, retinal venous stasis, macular exudates -Optic neuropathy depends more on chronicity of HTN |
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VASCULAR: DR
-For which age group is DR the leading cause of blindness in the US? -What perfecnt of pts younger than 10 have DR? -Major findings of the WESDR? -What is one limitation of the WESDR? |
DR
-Leading cause of blindness in pts 20-64 yo -Rarely seen in pts < 10 yo (regardless of duration of DM) -Prevalence increases with duration of DM and pt age -WESDR (Wisconsin Epidemiologic Study of DR): after 20 yrs, 99% of T1DM pts and 60% of T2DM pts had DR --> 3.6% of younger onset pts (<30 yo) and 1.6% older onset pts legally blind -Limitation: primarily white, European population --> may not be applicable to non-whites (i.e., Hispanics --> higher prevalence of DR) |
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VASCULAR: DR
-What is the presumed pathogenesis of DR? -What are the 3 categories of conditions associated w/ vision loss in DR? |
DR
-Pathogenesis: retinal capillary changes (loss of pericytes, BM thickening) --> capillary occlusion (nonperfusion) & endothelial barrier decompensation (leakage, edema) -3 categories of pathology in DR: edema (leakage), ischemia (occlusion), neovascularization |
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VASCULAR: DR
-Features of NPDR? -How is DME diagnosed? What is the role of IVFA? -What are 3 important observations to make in evaluating DME? |
DR
-NPDR: changes do not extend beyond ILM --> microaneurysms, capillary nonperfusion, dot/blot heme, IRMAs, cotton wool spots, edema, hard exudates, venous beading -DME: note LOCATION of retinal thickening relative to fovea, location/presence of exudates, presence of CME -DME is diagnosed by slit lamp exam (IVFA may show leakage but does not show retinal thickening) |
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VASCULAR: DR
-What is the difference b/w focal & diffuse macular edema? |
DR
-Focal DME: focal fluorescein leakage from capillary lesions --> resorption leaves precipitated hard exudates (outer & inner plexiform layers) -Diffuse DME: widespread capillary abnormalities --> diffuse leakage 2/2 extensive breakdown of blood-retinal barrier |
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VASCULAR: DR
-What were the findings of EDTRS? -What was the primary outcome in EDTRS? -What are 3 definitions of CSME? |
DR
-EDTRS (early treatment diabetic retinopathy study): 1) focal laser effective for DME (dec vision loss, inc vision gain, dec thickening); 2) aspirin does not alter progression of DR or affect VA; 3) scatter laser for DR may be good for T2DM -Primary outcome in EDTRS: moderate vision loss -CSME: 1) thickening within 500 microns of foveal center; 2) exudates within 500 microns of foveal center w/ adjacent thickening; 3) thickening > 1 disc area in size located within 1 DD of center |
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VASCULAR: DR
-What is the only proven long-term treatment for DME? -In high-risk PDR, which should be performed first: focal laser or PRP? -In pt w/ DME, should cataract surgery be performed first or should DME be treated first? -Side effects of focal laser? |
DR
-Focal laser = only proven long term treatment for DME -High risk PDR: focal laser should be performed prior to PRP -Should treat DME prior to cataract surgery (retinopathy may progress after surgery) -Focal laser sfx: paracentral scotoma, transient edema/dec VA, scar expansion, foveolar burns, CNV, fibrosis |
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VASCULAR: DR
-Diabetic macular ischemia: clinical exam and IVFA findings? -What is indicative of vision loss in diabetic macular ischemia? |
DR
-Diabetic macular ischemia: IVFA shows capillary nonperfusion; exam shows enlarged, irregular foveal avascular zone, clusters of MAs around nonperfused capillaries -Vision loss occurs if foveal avascular zone is > 1000 um diameter |
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VASCULAR: DR
-What is the 4:2:1 rule? -What determines severe vs very severe NPDR? What is the risk of progression to NPDR in each? |
DR
-4:2:1 rule: 1) diffuse intraretinal heme/MA in 4 quadrants; 2) venous beading in 2 quadrants; 3) IRMAs in 1 quadrant -If 1/3 seen --> severe NPDR --> 15% risk of progression to high risk PDR in 1 yr -If 2/3 seen --> very severe NPDR --> 45% risk of progression ot high risk PDR in 1 yr |
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VASCULAR: DR
-What are the 3 stages of evolution of neovascularization in PDR? -What are the most important aspects of medical/risk factor mgmt of DR? |
DR
-PDR: new vessels evolve in 3 stages: 1) fine vessels, minimal fibrosis, cross ILM; 2) increased size/extent of vessels, inc fibrous tissue; 3) regression of new vessels, remaining fibrovascular proliferation along post hyaloid -Medical/Risk factor mgmt of DR: control HTN, control carotid artery occlusive disease (causes ocular ischemic syndrome, worsens retinopathy) -Other risk factors for DR: advanced diabetic renal disease/anemia; pregnancy -Most important factor in medical mgmt: good glycemic control! |
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VASCULAR: DR
-What type of patient population was used and what were the main findings of the DCCT and UKPDS? |
DR
-DCCT (diabetes control & complications trial): type 1 DM --> intensive BG control reduced retinopathy risk by 76%, slowed progression by 54% (also reduced risk of nephropathy, albuminuria) -UKPDS (UK prospective diabetes study): T2 DM --> intensive BG control sowed progression of retinopathy, reduced other microvascular complications; **ALSO showed that intensive BP control slowed progression of retinopathy** |
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VASCULAR: DR
-What is the mainstay of treatment for PDR? -What is the goal of this treatment? -In which areas of the retina should this type of treatment be avoided? |
DR
-PDR mgmt: thermal panretinal photocoagulation -Goal of PRP: regression of neovascular tissue & prevention of further neovascularization -Avoid PRP in the following areas: prominent fibrovascular membranes, vitreoretinal traction, tractional RD |
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VASCULAR: DR
-What are some side effects of PRP? -In what pattern should PRP be performed? |
DR
-PRP sfx: dec night vision, peripheral vision, dec VA, inc glare, worsened macular edema -PRP should be performed in sessions to decrease side effects; try to avoid horizontal meridians as long as possible to preserve horizontal VF needed for driving |
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VASCULAR: DR
-What was the purpose and findings of the DRS? -Definition of high risk PDR? |
DR
-DRS (diabetic retinopathy study): evaluated benefit of PRP to eyes with clear media, advanced NPDR or PDR -Findings: 50% or greater reduction in rates of severe vision loss in PRP eyes compared to untreated controls -High risk PDR: need 3/4 of the following: 1) VH or PRH; 2) any form of neovascularization; 3) neovascularization on/near disc; 4) moderate-severe extent of neovascularization |
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VASCULAR: DR
-What is the appearance of concerning and non-concerning NVI? -What are the 2 main sequelae of PDR that require surgical mgmt? |
DR
-NVI: 1) small isolated tufts of NV at pupil border --> common; 2) contiguous NV of pupil, iris collarette and/or angle --> needs PRP -2 main sequelae of PDR requiring surgery: VH, tractional RD |
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VASCULAR: DR
-What was the purpose and findings of the DRVS? -What is the mechanism of a tractional RD in DR? |
DR
-DRVS (diabetic retinopathy vitrectomy study): role of early (1-6 mo) vs late (1 yr) vitrectomy in pts w/ VH and vision loss -Findings: Pts w/ T1DM and severe VH clearly showed benefit w/ early vitrectomy but no benefit seen w/ mixed or T2DM only pts -Note: if no PRP has been done, early vitrectomy generally recommended in pts w/ VH regardless of type of tDM -Tractional RD: secondary to vitreous traction of fibrovascular prolifertive tissue --> causes hemorrhage, schisis, RD or macular heterotopia |
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VASCULAR: DR
-5 most common indications for PPV in DR? |
DR
-Indications for PPV: 1) dense, nonclearing VH; 2) tractional RD; 3) combined tractional and RRD; 4) diffuse DME w/ post hyaloid traction; 5) recurrent VH despite maximal PRP |
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VASCULAR: DR
-What are some clinical features assoc with poorer VA outcomes after laser? -What are the settings for focal laser? Where is it applied? |
DR
-Clinical features assoc w/ poorer VA after laser: diffuse DME/leakage, foveal hard exudates, diffuse macular ischemia, CME -Focal laser: 500-3000 um from center; 50-100 um spot size, 0.1s duration |
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VASCULAR: DR
-What is time-table for follow up in pts w/ T1DM, T2DM or pregnant diabetic pts? -What is follow up protocol based on age of pt? |
DR
-T1DM: rarely exhibit retinopathy before 5 yrs after dx -T2DM: most pts have retinopathy at dx, need exam at dx -Pregnancy: need exam during 1st trimester (f/u afterwards case-by-case basis) -Less than 30 yo at dx: need exam within 5 yrs of dx; annually thereafter -30 or older at dx: need exam at time of dx, annually thereafter |
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VASCULAR: Sickle cell retinopathy
-What is the pathophysiologic change in sickle cell hemoglobin? -Which hemoglobinopathies are most frequently assoc with retinopathy? -Pathophysiology of retinopathy? |
Sickle cell retinopathy
-Sickle cell hemoglobin: valine substituted for glutamic acid in 6th position of beta chain (causes substitution of adenine for thymine) -Most frequently assoc w/ retinopathy: Hb SC disease (33% incidence of retinopathy), SThal & SS disease -Pathophysiology of retinopathy: peripheral arteriolar occlusion, capillary nonperfusion, neovascularization (at border of perfused/nonperfused segment), vitreous hemorrhage, tractional RD --> most commonly occurs w/ SC and SThal |
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VASCULAR: Sickle cell retinopathy
-What are the 3 most common findings of nonproliferative sickle cell retinopathy? -Pathophysiology? |
Nonproliferative sickle cell retinopathy
-Salmon patch hemorrhages: intraretinal hemorrhages occurring s/p peripheral arteriolar occlusion -Refractile spots: old, resorbed hemorrhages w/ hemosiderin deposition -Black sunburst lesions: localized, spiculated, perivascular areas of RPE hypertrophy, hyperplasia & pigment migration into retina (have hemosiderin deposition --> may be related to hemorrhages) |
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VASCULAR: Sickle cell retinopathy
-Describe the 5 pathogenic stages of proliferative sickle cell retinopathy -What are 2 differences b/w PDR and PSR? |
Proliferative sickle cell retinopathy
-Stage 1: peripheral arteriolar occlusions -Stage 2: peripheral arteriovenous anastomoses (dilated capillary channels) -Stage 3: pre-retinal sea fan neovascularization at border of nonperfused area -Stage 4: vitreous hemorrhage -Stage 5: tractional RD -Characteristics of PSR not found in PDR: peripheral (as opposed to postequatorial) neovascularization; frequent autoinfarction of neovascular tissue (white sea fan) |
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VASCULAR: Sickle cell retinopathy
-What are 3 other ocular abnormalities in SS or SC disease? |
Sickle cell retinopathy: other ocular abnormalities
-Comma sign: comma-shaped thrombi dilate and occlude conj capillaries -Disc sign: dark red spots on disc surface (small intravascular occlusions of capillaries on disc) -Angioid streaks (unclear etiology) |
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VASCULAR: Sickle cell retinopathy
-Mgmt of black pts w/ hyphema? |
Sickle cell retinopathy
-Hyphema: sickle screen for black pts w/ hyphema --> if positive, IOP control may be difficult and increased risk of ischemic optic neuropathy --> needs early AC washout, caution w/ CAIs for IOP mgmt (may worsen sickling through systemic acidosis) |
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VASCULAR: Sickle cell retinopathy
-How should laser be used in mgmt of PSR? |
Sickle cell retinopathy: Laser
-Do not use laser to close feeder vessels to neovascular frond --> may cause hemorrhage, CNV, retinal tears -How laser should be done: peripheral scatter laser, low intensity burns to ischemic peripheral retina --> may lead to regression of NV frond |
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VASCULAR: Sickle cell retinopathy
-Where does RD typically begin in sickle cell retinopathy? -What are precautions needed during vitreoretinal sugery in pts w/ sickle cell disease? |
Sickle cell retinopathy
-Causes of RD: begins in ischemic peripheral retina, tears start at base of sea fan; may be precipitated by laser -Precautions for surgery: avoid adjunctive epinephrine in local anesthesia, avoid encircling buckles, avoid removing EOM, need adequate hydration and supplementary O2, caution with expansile gases |
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VASCULAR: ROP
-What are the criteria (weight, age) for ROP eval? -When should the first evaluation be done? -In which cases is a single examination sufficient? -What % of infants w/ birth weight < 1251 or 1000g (respectively) have signs of ROP? |
ROP
-Criteria for eval: birth weight < 1500g or GA < 30 weeks **Also may screen pts w/ unstable clinical course, birth weight 1500-2000g or GA > 30 wks -First exam: 4-6 wks postnatal age OR 31-33rd wk postconceptional age --> then every 1-2 wks thereafter -Single exam sufficient only if fully vascularized retina seen bilaterally -66% of infants w/ birth weight < 1251g & 82% with birth weight < 1000g have signs of ROP |
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VASCULAR: ROP
-What are criteria for conclusion of ROP exams? -What is the criteria for prethreshold disease? |
ROP: Criteria for concluding exams
-Zone III vascularization attained w/o history of zone I/II disease -Full retinal vascularization -Postmenstrual age 45 wks & NO prethreshold disease (i.e., stage III ROP in zone II or any ROP in zone I) -Regressing ROP |
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VASCULAR: ROP
-What is the mgmt of infants with threshold disease? -When does normal nasal & temporal retinal vascularization occur? -What cells give rise to capillary endothelial cells? |
ROP
-Infants w/ threshold disease should receive cryo or laser within 72 hrs of diagnosis -Nasal retinal vascularization: complete by 36 wks -Temporal retinal vascularization: complete by 40 weeks -Mesenchymal cells: give rise to capillary endothelial cells |
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VASCULAR: ROP
-What are the 2 distinct processes by which vascularization occurs? Where does each process occur in the retina? |
ROP
-Vasculogenesis: de novo formation of new vessels --> zone I disease -Angiogenesis: formation of new vessels by budding from existing vessels --> zone II disease |
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VASCULAR: ROP
-What is the presumed pathophysiology of ROP? -What factors aside from high O2 exposure may increase risk of ROP? |
ROP: Pathophysiology
-Exposure of vascular precursor tissue to high concentration of O2 --> arrest of development --> area of retina w/o blood supply --> mesenchymal tissue forms shunts --> neovascularization -Risk factors for ROP: genetic predisposition, low birth weight, short gestational period, high O2 concentration exposure |
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VASCULAR: ROP
-Define the 5 stages of ROP |
ROP: Staging
-Stage I: flat demarcation line b/w vascular and nonvascularized retina -Stage 2: elevated demarcation ridge b/w vascular/nonvascular retina -Stage 3: ridge + extraretinal fibrovascular proliferation (blood vessels grow through ILM) -Stage 4: subtotal RD (4a does not involve fovea; 4b involves fovea) -Stage 5: total RD with funnel |
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VASCULAR: ROP
-Define the 3 zones of ROP -Which zones have the most favorable prognosis? |
ROP: Zones
-Zone I: posterior retina within 60 deg circle centered on optic nerve (twice the radius of optic disc --> foveola distance) -Zone II: from edge of Zone I cricle to nasal ora serrata -Zone III: remainder of fundus outside zones I, II -Best prognosis: Zone III involvement --> the more posterior the zone, the more vasculogenic the mechanism, the more nonperfused retina there is and therefore worst prognosis |
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VASCULAR: ROP
-What is the definition of "plus disease"? -What is "threshold disease"? -What is "rush disease"? What % of infants < 1251 grams develop threshold ROP? |
ROP
-Plus disease: retinal vascular dilation & tortuosity in posterior pole (actively progressing phase of disease) -Threshold disease: >5 contiguous clock hours or 8 cumulative clock hours of extraretinal neovascularization + plus disease + zone I or II -Rush disease: plus disease + vascularization in zone I or posterior zone II --> rapid progression -7% of infants < 1251 g develop threshold ROP |
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VASCULAR: ROP
-What % of eyes have spontaneous regression? -What is the appearance of regressing ROP? |
ROP
-85% of eyes have spontaneous regression -Appearance of regression: clear zone of retina beyond shunt --> straight vessels that cross shunt --> AV feeder extending into avascular retina |
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VASCULAR: BRVO
-Exam findings in BRVO? -Most common location? -If not at typical location, what dx should be considered? -4 risk factors for BRVO? |
BRVO
-Exam: superficial retinal heme, edema, CWS -Most common location: superotemporal retina (63%) at AV crossing site -If not at AV crossing site --> consider retinochoroiditis -Risk factors: glaucoma, HTN, CV disease, increased BMI at age 20 |
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VASCULAR: BRVO
-Pathophysiology of venous obstruction? -Most important factors guiding visual prognosis? -What criteria are generally needed to see neovascularization s/p BRVO? Overall, what % of eyes with BRVO develop NV? -What % of pts will have VA >/= 20/40 at one year? |
BRVO
-Pathophys: thickening of arterial wall --> crompression, thrombotic occlusion of adjacent vein -Visual prognosis: determined by extent of macular edema, capillary nonperfusion (retinal heme can also temporarily reduce VA) -Neovasc results in 40% of eyes that have >5 DD of ischemia (overall, 1% of eyes w/ BRVO develop neovasc) -50-60% of pts have VA >/= 20/40 at 1 year |
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VASCULAR: BRVO
-What is an indication for scatter laser in BRVO according to the BVOS? |
BRVO
-Scatter laser (PRP): perform when posterior segment neovasc develops --> BVOS showed that ischemia w/o neovasc was not an indication for treatment (need to observe and if develop neovasc, give laser) |
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VASCULAR: CRVO
-Describe fundus appearance, IVFA appearance and clinical exam findings of nonischemic CRVO |
CRVO: Nonischemic
-Fundus: mildly dilated, tortuous retinal veins, hemorrhages, disc edema, retinal edema -IVFA: minimal areas of nonperfusion, rare anterior segment neovasc -Exam:good VA, mild APD and VF changes, +/- macular edema |
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VASCULAR: BRVO
-What are 2 indications for laser in BRVO? What study provided the basis for these criteria? -What areas are targeted by laser? |
BRVO
-2 indications for laser: 1) chronic macular edema with intact perifoveal capillary perfusion; 2) posterior segment neovascularization -BVOS: showed argon laser improved vision in eyes w/ macular edema (VA 20/40 - 20/200) but intact foveal capillaries -Laser pattern: grid laser to areas of capillary leakage seen on IVFA |
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VASCULAR: CRVO
-Describe fundus appearance, IVFA appearance and other clinical exam findings of ischemic CRVO |
CRVO: Ischemic
-Fundus: severe dilation, tortuosity of retinal veins, more extensive 4 quad heme, retinal edema, CWS -IVFA: at least 10 disc areas of capillary nonperfusion in posterior pole -Clinical: poor vision, dense APD, dense central scotoma, poor visual prognosis, up to 60% develop iris NV |
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VASCULAR: CRVO
-Pathophysiology? |
CRVO
-Pathophysiology: thrombosis of CRV at or posterior to level of lamina cribosa -May be secondary to compression by atherosclerotic CRA --> turbulence, endothelial damage, thrombus (similar to BRVO) |
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VASCULAR: CRVO
-Most common age of presentation? -3 most common systemic associations? -Other meds, systemic conditions assoc with CRVO? |
CRVO
-90% of pts >50 yo at diagnosis -Systemic associations (noted by Eye Disease Case-Control Study): DM, HTN, open angle glaucoma (in one or both eyes) -Meds: OCPs, diuretics -Other systemic conditions: polycythemia vera, dysproteinemias, vasculitis, hypercoagulable state --> suspect if young pt w/ CRVO |
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VASCULAR: CRVO
-What were the findings of CVOS re: benefit of grid laser for macular edema & PRP for iris NV s/p CRVO? -Most important predictive factor for iris NV found by CVOS? |
CRVO
-Grid laser: CVOS showed that grid laser reduced IVFA evidence of macular edema but did not improve visual acuity -PRP: does not decrease risk of developing iris NV; should be performed only in pts w/ at least 2 clock hours of iris NV on undilated gonio -Most important predictive factor for iris NV: poor visual acuity (also: large areas of capillary nonperfusion, intraretinal blood) |
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VASCULAR: Carotid occlusive disease retinopathy
-What is one method of differentiating between CRVO and this entity? |
Carotid artery occlusive disease
-Ophthalmodynamometry: CRVO shows normal CRA pressure but carotid disedase will show low CRA pressures |
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VASCULAR: Precapillary retinal arteriole obstruction
-What vessel supplies blood to inner retinal layers? -What is a clinical manifestation of a precapillary retinal arteriole obstruction? -What is the significance of a single cotton wool spot in an asymptomatic patient without diabetes? |
Precapillary arteriole obstruction
-Central retinal artery supplies inner layers of retina (in 15-30% of pts, cilioretinal artery also supplies blood to portion of retina/macula) -Cotton wool spot (NFL infarct w/ inhibition of axoplasmic transport in axon) --> acute finding reflective of precapillary obstruction -Single cotton wool spot in asymptomatic nondiabetic pt --> work up for underlying systemic etiology |
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VASCULAR: BRAO
-Acute clinical appearance? -Chronic appearance? -What are 3 types of emboli? |
BRAO
-Acutely: edematous opacification of inner retina supplied by occluded artery -Chronically: recanalization of vessel --> restoration of perfusion, but persistent VF defect -3 types of emboli: 1) cholesterol (carotid arteries); 2) platelet-fibrin (arteriosclerosis); 3) calcific (cardiac valves) |
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VASCULAR: CRAO
-Presentation? -What causes a cherry red spot? -When is VA better than 20/40? -What is cause of NLP vision s/p CRAO? |
CRAO
-Sudden, complete, painless monocular vision loss -Cherry red spot visible due to edema, opacification of retinal NFL/ganglion cell layers surrounding fovea --> obscure underlying choroidal vessels (no NFL in fovea so choroidal vessels still visible) -If patent cilioretinal artery --> can have VA 20/40 or better -NLP vision: usually secondary to partial/complete ophthalmic artery occlusion + CRAO (choroidal & retinal vascular insufficiency) |
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VASCULAR: CRAO
-How long does it take for irreversible damage to occur to sensory retina? -Pathophysiology of CRAO (thrombosis vs emboli)? -What is the leading cause of death in pts w/ retinal arterial obstruction? |
CRAO
-90 minutes till irreversible damage to retina -Pathophys: most cases due to thrombus (atherosclerotic) at lamina cribosa; emboli seen in 20% of pts -Emboli may cause amaurosis fugax -Leading cause of death in these pts: cardiovascular disease |
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VASCULAR: CRAO
-What disease accounts for 1-2% of cases of CRAO? When should testing for this disease be performed in CRAO pts? -What % of eyes develop iris neovasc? How long does it take to develop? Treatment? |
CRAO
-GCA accounts for 1-2% of cases --> get ESR/CRP if emboli not seen on exam -NVI develops in 18% of eyes 1-12 wks s/p CRAO --> mgmt: PRP |
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VASCULAR: Ocular ischemic syndrome
-Pathophysiology? -Presentation? -IVFA findings? |
Ocular ischemic syndrome
-Pathophys: chronic, severe carotid artery obstruction (>90%) OR chronic ophthalmic artery obstruction 2/2 atherosclerosis -Presentation: weeks-months of vision loss, orbital pain, prolonged recovery s/p exposure to bright light, NVI (2/3 of pts), AC reaction -IVFA: 60% w/ delayed choroidal filling, 95% w/ delayed AV transit time, prominent arterial staining |
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VASCULAR: Ocular ischemic syndrome
-ERG findings? -5 year mortality? -Most frequent cause of death? -Mgmt for NVI? -Definitive treatment? |
Ocular ischemic syndrome
-ERG: decreased a and b wave amplitude -40% five year mortality -Majority of death secondary to cardiovascular disease -PRP used to treat rubeosis (effective in 35% of eyes) -Definitive treatment: carotid artery stenting, endarterectomy |
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VASCULAR: Vasculitis
-Early fundus findings? -Which vessels involved? -What is Eales disease? -What is IRVAN syndrome? |
Vasculitis
-Early findings: perivascular infiltrates, sheathing of retinal vessels (thickening of walls) -Veins involved earlier and more frequently than arteries but usually both involved -Eales disease: idiopathic occlusive retinal vasculopathy in males, peripheral retina w/ neovascularization and VH --> assoc w/ tuberculin hypersensitivity -IRVAN: idiopathic retinal vasculitis, aneurysms, neuroretinitis |
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VASCULAR: CME
-Pathophysiology? -Which retinal layers are involved? Which cells? -IVFA pattern? |
CME
-Pathophysiology: abnormal perifoveal retinal capillary permeability --> leakage of fluid into extracellular cystoid spaces -Inner nuclear & outer plexiform layers; edema in and b/w mullerian glia -Flower petal pattern seen on IVFA -Other IVFA findings: hyperfluorescent dilated capillaries w/ late petaloid leakage, **disc staining** |
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VASCULAR: CME
-When is the peak incidence of Irvine-Gass syndrome? -Natural course? -What drug is helpful for prophylaxis? -What is Acular (ketorolac) effective for based on clinical trials? |
CME: Irvine-Gass
-Peak incidence: 6-10 wks s/p CE -Natural course: 95% resolve spontaneously over 6 months -Topical/systemic indomethacin: effective for reducing incidence of angiographic CME -Acular: improves VA in eyes w/ chronic aphakic/pseudophakic CME |
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VASCULAR: Coats' disease
-Clinical appearance? -IVFA appearance? -Pathophysiology? |
Coats' disease
-Clinical: retinal telangiectasia (vascular dilations), fusiform capillary aneurysms, microaneurysms, exudation, exudative RD, leukocoria -IVFA: capillary nonperfusion, telangiectasias, leakage -Pathophysiology: incompetence of abnormal vessels --> leakage of serum/blood components --> exudation, RD (variable) |
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VASCULAR: Coats' disease
-Systemic associations? -Inheritance? -Epidemiology? -Disease course? |
Coats' disease
-Sporadic -Progresses over time w/ increased exudation -85% of pts are males -No associated systemic vascular abnormalities |
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VASCULAR: Coats' disease
-Mgmt? -DDx? |
Coats' disease
-DDx: familial exudative vitreoretinopathy, ROP, von Hippel disease, facioscapulohumeral muscular dystrophy -Mgmt: laser, cryo or RD surgery |
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VASCULAR: Juxtafoveal retinal telangiectasias
-Clinical appearance? -Main cause of visual loss? -Pathophysiology? |
Juxtafoveal retinal telangiectasias
-Clinical: focal gliosis, telangiectasias of capillary bed confined to juxtafoveal area (usually temporal) -Vision loss: secondary to capillary incompetence --> exudation -Pathophys: structural abnormality of retina (similar to diabetic microangiopathy) |
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VASCULAR: Juxtafoveal retinal telangiectasias
-What are features of Groups 1, 2 & 3? (laterality, clinical appearance, associated abnormalities, mgmt) |
Juxtafoveal retinal telangiectasias
-Group 1 ("localized Coats' disease"): M > F, unilateral, Leber miliary aneurysms (circinate exudates) -Group 2 (most common): M=F, bilateral, thickening 2/2 gliosis, 1/3 of pts w/ abnormal glucose tolerance test -Group 3: bilateral, assoc with capillary obliteration --> progressive vision loss -Mgmt: laser effective for group 1 but NOT effective for groups 2/3 (leaky vessels are not the predominant feature in these cases) |
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VASCULAR: Retinal arterial macroaneurysm
-Inherited vs acquired? -Which vessels are typically affected? -Distinguish between white & red infarcts -What % of cases are bilateral? -What % of cases are assoc w/ systemic HTN? |
Retinal arterial macroaneurysms
-Acquired -Second order retinal arterioles -White infarct: embolic/thrombotic occlusion of arteriole -Red infarct: hemorrhage at any level (pre/sub/intra/vitreous) -10% bilateral -2/3 assoc w/ HTN |
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VASCULAR: Retinal arterial macroaneurysm
-Frequency of recurrence? -Mgmt? |
Retinal arterial macroaneurysm
-Recurrence uncommon (spontaneously closes following hemorrhage) -Mgmt: large spot size laser burns to areas of leakage if edema seen in macula & affects vision |
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VASCULAR: Capillary hemangioblastomas
-Clinical appearance? -Laterality? -Pathophysiology? -Inheritance? |
Capillary hemangioblastomas
-Clinical: spherical orange-red tumor fed by dilated, tortuous retinal artery & drained by engorged vein -50% bilateral -Autosomal dominant -Pathophys: leakage of plasma from tumor --> serous RD or exudate in macula --> vision loss, neovascularization of disc/retina/iris |
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VASCULAR: Capillary hemangioblastomas
-Describe clinical features, areas of involvement/systemic workup and cause of death for the following... -Von Hippel disease? -Von Hippel-Lindau? -Mgmt? |
Capillary hemangioblastomas
-Von Hippel disease: limited to the eye -Von Hippel-Lindau: eye + CNS/visceral involvement (visceral cysts, cerebellar, brainstem and spinal cord hemangioblastomas) -Most important systemic workup: renal cell carcinoma, pheochromocytoma, cerebellar hemangioblastoma (leading causes of death) -Mgmt: enlarge over time, so treat with laser, cryo --> may cause massive exudation and worsening of disease! |
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VASCULAR: Congenital AVM
-What is a racemose angioma? -Laterality? Inheritance? -IVFA? -What is Wyburn-Mason syndrome? |
Congenital AVM
-Racemose angioma: no intervening capillary bed b/w AV connection -Unilateral, nonhereditary -IVFA: NO leakage! -Wyburn-Mason: retinal lesion + similar ipsilateral AVM of brain/orbit/face/mandible |
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VASCULAR: Retinal cavernous hemangioma
-Inheritance? -Clinical appearance? -IVFA? |
Retinal cavernous hemangioma
-Sporadic vs autosomal dominant -Clinical: grapelike clusters of thin-walled saccular angiomatous lesions of inner retina or optic nerve head (blood flow derived from retinal circulation, stagnant) -IVFA: slow filling of lesion, plasma-erythrocyte layering 2/2 to sluggish flow, NO leakage! |
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VASCULAR: Retinal cavernous hemangioma
-Mgmt? |
Retinal cavernous hemangioma
-Asymptomatic --> no treatment unless recurrent VH develops (use laser, cryo) |
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VASCULAR: Radiation retinopathy
-What disease does it most resemble? -Timeline b/w exposure & onset, degree of exposure required? -Mgmt? |
Radiation retinopathy
-Resembles diabetic retinopathy --> CWS, hemorrhages, microaneurysms, macular edema, perivascular sheathing, neovascularization -seen ~18 months after exposure (sooner w/ brachytherapy), need 30-35 Gy doses -Mgmt similar to diabetic retinopathy |
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CHOROID: BDUMP
-Clinical appearance? -Important systemic association? -IVFA appearance? |
Bilateral diffuse uveal melanocytic proliferation (BDUMP)
-Clinical: diffuse thickening of choroid, red/brown discoloration, serous RD, cataract (may look like large nevi) -Paraneoplastic disorder --> assoc w/ cancer of ovaries, uterus, lung -IVFA: hypofluorescence of areas of melanocytic proliferation, may see leopard spot pattern in areas of chronic subretinal fluid |
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CHOROID: Perfusion abnormalities
-Appearance/shape of ischemic areas? -Why does choroidal ischemic occur if the choroid has a rich circulation? -Findings in HTN/eclapsia? -Findings in GCA? |
Perfusion abnormalities
-Ischemic areas are: vertically/horizontally hemispheric (if 2/2 intraorbital vascular occlusion) or triangular, in oblique meridian of occluded vessel (if 2/2 intraocular vascular occlusion) -Malignant HTN/eclampsia: blood flows in terminal fashion in choriocapillaris (little collateral flow after focal occlusion) -HTN/eclampsia: Elschnig spots (small patches of atrophy/pigmentary hyperplasia), Siegirst streaks (linear aggregations of Elschnig spots) -GCA: occlusion of short posterior ciliary arteries --> triangular areas of hypoperfusion in any meridian |
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CHOROID: Perfusion abnormalities
-Name 3 other disorders that can cause retinal/choroidal vascular occlusion -Name 3 iatrogenic causes of choroidal vascular occlusion |
Perfusion abormalities
-3 other disorders that cause vascular occlusion: Wegener's (30-50% of pts have ocular manifestations), TTP, DIC (microembolic occlusion due to platelet emboli --> caused by rapid deceleration of blood flow and larger volumetric flow within choroid) -Iatrogenic causes: laser, PDT, ocular compression w/ cataract surgery |
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CHOROID: Choroidal hemangioma
-Clinical appearance? -ICG pattern? -Visual consequences? |
Choroidal hemangioma
-Clinical: reddish orange, well circumscribed tumor, varying thickness -ICG: hyperfluorescence in midphase 2/2 dye within and leakage from tumor vessels --> late phases, dye washes out and leaves hyperfluorescent ring in adj tissues -Visual consequences: hyperopia, serous RD |
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CHOROID: Choroidal hemangioma
-Sturge-Weber: how is associated hemangioma different from choroidal hemangioma? -Mgmt of choroidal hemangioma? |
Choroidal hemangioma vs Sturge-Weber
-Sturge-Weber: diffuse "tomato catsup" fundus, underlying choroidal markings not visible; ipsilateral port wine stain (nevus flammeus) -Mgmt of choroidal hemangioma: laser, cryo, radiation, PDT |
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CHOROID: Uveal effusion syndrome
-Pathophysiology? -Associated eye conditions? -Consequeces of disease? -IVFA? -Mgmt? |
Uveal effusion syndrome
-Pathophysiology: abnormal scleral composition/thickness (nanophthalmos, scleritis, etc) --> reduced transscleral aqueous outflow -Assoc w/ glaucoma, hyperopia -Consequences of disease: choroidal/CB thickening, RPE changes, exudative RD -IVFA: leopard spot pattern of hypofluoresence, NO leakage -Mgmt: Scleral surgery --> anatomic correction but may not improve visual outcome (chronic changes may occur) -Suspect in young, hyperopic pt w/ recent dx of ICSC or RD without retinal hole |
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INFLAMMATION: APMPPE
-Epidemiology? -Laterality? -Clinical appearance (level of retina, appearance of lesion)? -Pathophysiology? -IVFA? -Prognosis, treatment? -Ddx? |
Acute multifocal placoid pigment epitheliopathy
-Epi: healthy pts, 2-3rd decade, viral prodrome in 1/3 -Bilateral (one eye first, then other eye days later) -Clinical: early blockage (nonperfusion of choriocapillaris or blockage 2/2 RPE lesions) --> late staining -Unclear if disease is primarily of pigment epithelium or if RPE secondarily involved -Prognosis: improvement within weeks --> good visual prognosis -Ddx: serpiginous choroidopathy |
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INFLAMMATION: Serpiginous choroidopathy
-Clinical appearance? -Symptoms? -IVFA? -Prognosis, treatment? |
Serpiginous choroidopathy
-Clinical: serpiginous (pseudopodial) or geographic (maplike) pattern of scars in posterior pole --> acute, look like gray-yellow discoloration of RPE -Sx: decreased VA, central/paracentral scotoma -IVFA: early hypofluorescence, late staining (similar to APMPPE) -Prognosis: recurrent, chronic, may develop CNV in scarred areas -Mgmt: potent immunosuppressives --> initiate immediately if suspected |
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INFLAMMATION: MEWDS
-Clinical appearance? -Laterality? -Epidemiology? -IVFA? -VF? -ERG? -Mgmt? -Ddx? |
Multiple evanescent white dot syndrome
-Clinical: multiple small gray-white dots at deep retina/RPE, posterior pole; may have transient foveal granularity (tiny yellow dots at level of RPE) -80% unilateral -Epi: F>M, 2-5th decades, myopes, viral prodrome in 50% -IVFA: each spot shows wreath of gray-white dots -VF: enlarged blind spot -ERG: decreased a-wave amplitude -Mgmt: No treatment --> improves in 2-6 weeks -Ddx: idiopathic enlargement of the blind spot syndrome (no retinal lesions) |
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INFLAMMATION: Birdshot choroidopathy
-Clinical presentation? -Sx? -Epidemiology? -ERG? -IVFA? -Prognosis, treatment? |
Birdshot choroidopathy
-Clinical: yellow, ovoid "birdshot" chorioretinal lesions (nasal retina), disc edema, vascular sheathing, vitritis in 100% -Sx: blurry vision, floaters, photopsias -Epi: F>M, 4-6th decades, HLA-A29 positivity in 90% -ERG: reduced/extinguished -IVFA: "quenching" - dye rapidly disappears from retinal circulation -Prognosis: chronic, bilateral, prone to recurrence --> vision loss 2/2 CME (1/3), macular CNV, optic atrophy -Mgmt: intravitreal triamcinolone, intravitreal fluocinolone acetonide implant, systemic immunosuppressives |
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INFLAMMATION: Multifocal choroiditis and panuveitis syndrome
-Clinical? -Sx? -Epi? -Ddx? -Mgmt? -Prognosis? |
MCP syndrome
-Clinical: vitritis, disruption of peripapillary RPE, multifocal choroiditis --> evolve into "punched out" chorioretinal scars similar to OHS -Sx: dec VA, floaters, photopsias, VF defects (enlarged blind spot) -Epi: bilateral, F>M, 2-6th decades -Ddx: OHS, other infectious etiologies (syphilis, Tb) -Mgmt: corticosteroids -Prognosis: Poor visual outcomes, subfoveal CNV in 20% (leading cause of visual loss) |
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INFLAMMATION: Punctate inner choroidopathy
-Clinical? -Sx? -Epi? -IVFA? -Ddx? -Prognosis? |
Punctate inner choroidopathy
-Clinical: small, round yellow-white lesions at level of RPE/inner choroid --> may coalesce to form serous RD; mild disc edema; no vitritis/iritis -Sx: bilateral central vision loss, photopsias, scotomata (correspond to location of lesions) -Epi: 90% F, myopia, young -IVFA: late staining of lesions -Ddx: OHS, MCP -Prognosis: spontaneous improvement w/o treatment, good prognosis but 1/3 may develop CNV |
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INFLAMMATION: Acute zonal occult outer retinopathy
-Clinical? -Sx? -IVFA? -ERG? -Epi? -Ddx? -Mgmt? |
Acute zonal occult outer retinopathy
-Clinical: may have normal exam initially --> later, depigmentation of large zones of RPE (corresponds w/ location of scotomata); vitritis -Sx: vision loss, visual field loss, photopsias, sometimes enlarged blind spot -IVFA: retinal, optic nerve head capillary leakage -ERG: decreased rod/cone amplitudes under scotopic/photopic conditions -Epi: F>M, young -Ddx: CAR, RP (in late cases, based on fundus appearance) -Mgmt: no treatment, most pts retain good VA in at least 1 eye |
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INFLAMMATION: Vasculitis - Bechet's
-Classic triad of sx? -Pathophysiology? -Epidemiology, genetics? -Ocular manifestations? -Mgmt? -Prognosis? |
Bechet's disease
-Classic triad: recurrent aphthous oral ulcers, genital ulcers, acute iritis w/ hypopyon -CNS disease in > 50% of pts -Pathophysiology: cyclical systemic occlusive vasculitis (post segment vasculitis, hemorrhages, necrosis, edema, vitritis --> can lead to ischemia and neovascularization) -Epi: M>F, Japan, southeast Asia, Mid East, Mediterranean, HLA-B5101 -Mgmt: corticosteroids, PRP (for neovascularization), immunomodulators for severe disease -Prognosis: poor --> multiple episodes of vasculitis |
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INFLAMMATION: Vasculitis - SLE
-Clinical? -Pathophysiology? -Epidemiology? -Mgmt? |
SLE vasculitis
-Clinical: varied --> CWS, hemorrhages, vasculitis, choroidopathy (serous elevations of retina/RPE) -Pathophys: autoimmune vascular disease --> B cell activation, autoantibody production, immune complexes --> microangiopathy -Mgmt: corticosteroids, immunomodulators, plasmapheresis, anticoagulation, PRP (prevent NV) --> should be done in conjunction w/ rheumatologist |
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INFLAMMATION: Intermediate uveitis - Pars planitis
-Ddx of intermediate uveitis? Pars planitis... -Clinical appearance? -IVFA? -Epidemiology? -Mgmt? |
Intermediate uveitis
-Ddx: MS, syphilis, Lyme, sarcoid Pars Planitis -Clinical: inflammatory exudates on pars plana ("snowbanks"), aggregates of vitreous cells ("snowballs"), diffuse vitritis --> may lead to peripheral NV, CME (1/3 of pts, leading cause of vision loss) -IVFA: difuse peripheral venular leakage, lade staining, neovascularization -Epi: young adults & children (ages 25-35, 5-15) -Mgmt: steroids for CME, peripheral laser or cryo for NV, PPV for RD, immunomodulatory therapy if severe |
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INFLAMMATION: Panuveitis - Sarcoid
-What % of sarcoid pts have ocular manifestations? -Anterior and posterior segment manifestations? |
Panuveitis - Sarcoid
-50% of pts have ocular manifestation of disease -Ant: granulomatous uveitis, iris nodules -Post (multiple!): intermediate uvietiis, vasculitis, periphlebitis, choroiditis, optic nerve edema |
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INFLAMMATION: Panuveitis - VKH
-Epidemiology, genetics? -Skin, neurologic signs? -What is Harada disease? -IVFA? -Mgmt? |
Panuveitis - VKH
-Epi: F>M, pts w/ darker skin pigmentation, HLA-DRB*0405 assoc in Japanese -Bilateral granulomatous panuveitis w/ skin (vitiligo, alopecia, poliosis) & neurologic sx (meningeal signs) -Harada disease: isolate posterior segment disease (no systemic findings) -IVFA: multiple punctate hyperfluorescent dots, leakage of dye into subretinal space -Mgmt: responds well to systemic steroids --> good prognosis but may be complicated by cataracts, CNV, glaucoma |
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INFLAMMATION: Panuveitis - VKH
-Describe the 4 stages of disease -What is Sigiura sign? -What is a "sunset glow" fundus? -What are "Dalen Fuchs" nodules? |
Panuveitis - VKH
-Stage 1: prodromal phase (flulike sx w/ meningismus, tinnitus, dysacusis) -Stage 2: uveitic phase (acute onset bilateral granulomatous uveitis, vitritis, disc edema, yellow-white RPE exudates, serous RD in posterior pole) -Stage 3: acute uveitic phase (2-6 weeks of diminished uveitis, depigmentation of skin/uvea, sunset-glow fundus, Dalen-Fuchs nodules (punched out) -Stage 4: final phase (chronic, recurrent inflammation --> cataract, glaucoma, CNV) -Sigiura sign: perilimbal vitiligo -"Sunset glow" fundus: orange-red discoloration 2/2 gradual depigmentation of choroid -Dalen Fuchs nodules: focal aggregates of epithelioid histiocytes admixed w/ RPE --> b/w Bruch's & RPE |
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INFLAMMATION: Panuveitis - Sympathetic ophthalmia
-How to distinguish from VKH? -Incidence? -Clinical appearance? -Prognosis, treatment? |
Sympathetic ophthalmia
-Clinical: bilateral granulomatous panuveitis, disc swelling, choroidal thickening -Distinguish from VKH by eliciting h/o penetrating ocular injury/ocular surgery -Occurs in < 1% of pts w/ positive history --> days - decades following initial insult -Prevention: enucleation of injured eye within 2 weeks of injury -Mgmt: corticosteroids -Poor prognosis |
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INFLAMMATION: Infiltrative uveitis - intraocular lymphoma
-Clinical appearance? -Epidemiology? -Distinctive finding on exam? -Confirmation of Dx? -Prognosis, mgmt? |
Infiltrative uveitis - intraocular lymphoma
-Clinical: bilateral iritis, vitritis, retinal vasculitis, creamy yellow sub-RPE infiltrates --> may be mistaken for uveitis -Distinctive finding: large, solid, confluent RPE detachments -Dx: PPV, cytologic exam of specimen -Epi: 6-7th decades -Mgmt: systemic evaluation for CNS involvement -Prognosis: poor --> low 5-yr survival rate |
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INFLAMMATION: CMV retinitis
-Patient population? -Clinical appearance? -Mgmt? (IV vs intravitreal) -Complications? |
CMV retinitis
-Most common ocular infection in AIDS pts (CD4 < 50) -Clinical: retinal opacification, hemorrhage, exudate & necrosis; periphlebitis, frosted branch angiitis possible (very early on, lesions may resemble CWS) -Mgmt: IV ganciclovir/foscarnet (may switch to PO if respond well); intravitreal injection not yet FDA approved, does not treat systemic infection -Complications: RD in 40-50% within 1st year --> needs PPV, sil oil tamponade -HAART --> leads to immune recovery uveitis in 20% of AIDS pts w/ h/o CMV retinitis (CD4 cells 100 or more) |
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INFLAMMATION: Necrotizing herpetic retinitis - ARN
-Organisms? -Clinical presentation? -Complications? -Laterality? -Mgmt? -When is risk of RD highest? |
ARN
-VZV or HSV -Clinical: healthy pt w/ ocular pain, dec VA --> iritis, episcleritis, vitritis, large areas of retinal necrosis that coalesce and spread centripetally -Complication: retinal breaks and RD can occur in necrotic areas -Bilateral at onset in 20% (w/o treatment, fellow eye commonly involved) -Mgmt: IV acyclovir, PO famciclovir/valaciclovir until resolution; Intravitreal foscarnet/ganciclovir; prophylactic laser -RD risk highest 8-12 wks after onset |
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INFLAMMATION: Necrotizing herpetic retinitis - PORN
-Patient population? -How to distinguish from ARN? |
PORN
-Immunocompromised pts -Compared to ARN: more rapid progression, no vitritis, sparing of retinal vessels |
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INFLAMMATION: Endogenous bacterial endophthalmitis
-Clinical appearance? -Organisms? -Most common associated etiologies? -Mgmt? |
Endogenous bacterial endophthalmitis
-Clinical: begins as focal/multifocal chorioretinal lesion --> spreads to vitreous -Organisms: Strep, Staph, Serratia, Bacillus -Assoc systemic etiologies: endocarditis, GI/GU infections -Mgmt: culture vitreous and extraocular sites/blood; IV and intravitreal abx |
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INFLAMMATION: Candidal endophthalmitis
-Risk factors? -Clinical appearance? -Mgmt? -Prognosis? |
Candidal endophthalmitis
-Risk factors: indwelling catheters, chronic abx, immunosuppression, hyperalimentation, recent abd surgery, DM -Clinical: mild-mod inflammation, yellow-white choroidal lesions --> coalesce into mushroom shaped nodule projecting through into vitreous -Mgmt: IV fluconazole, voriconazole (ampho B does not penetrate well), intravitreal ampho B, voriconazole -Prognosis: good vision if macula not involved |
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INFLAMMATION: Aspergillus endophthalmitis
-Pt population? -Clinical? -Mgmt? -Prognosis? |
Aspergillus endophthalmitis
-Immunosupressed, IV drug users, h/o liver transplantation -Clinical: more severe inflammation than Candida, larger chorioretinal lesion and rapid progression; may have large yellow infiltrate in/near macula, subhyaloid/subretinal hypopyon -Mgmt: PPV w/ cultures/injection of ampho B -Prognosis: VA usually poor |
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INFLAMMATION: Tuberculosis
-Most common site of intraocular Tb? -Clinical findings? -Mgmt? |
Tuberculosis
-Choroid = most common initial site of intraocular Tb -Clinical: choroidal tubercles --> polymorphic yellow-white lesions. initially flat, > 1 DD size, variably pigmented -May also have vitritis, papillitis, overlying serous RD -Mgmt: 4-drug therapy w/ isoniazid, rifampin, pyrazinamide, ethambutol/streptomycin (similar to pulmonary Tb) |
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INFLAMMATION: Syphilitic chorioretinitis
-Which stage of syphilis does this occur in? -Testing? -Characteristic finding? -Mgmt? |
Syphilitic chorioretinitis
-Occurs in secondary stage of syphilis -Testing: RPR/VDRL (may be negative in HIV pts), FTA-ABS confirms prior exposure but not necessarily active infection -If + FTA-ABS and neg VDRL in pt w/ syphilitic uveitis, consider LP w/ CSF analysis as pt may have neurosyphilis even w/ neg VDRL -Characteristic lesion: yellow placoid chorioretinal lesion in posterior pole -Mgmt: same as for neurosyphilis, steroids of limited value |
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INFLAMMATION: Cat-scratch disease
-Organism, mechanism of infection? -Systemic findings? -What is Parinaud oculoglandular syndrome? -Typical retinal findings? -Mgmt, prognosis? |
Cat-scratch disease
-Bartonella henslae --> acquired thru contact b/w cat saliva & mucocutaneous surface/open wound -Parinaud oculoglandular syndrome: conj inflammation + preauricular lymph nodes -Retinal findings: neuroretinitis (disc edema + macular star pattern of exudate), yellow-white retinal infiltrates -Mgmt: doxy, cipro, erythromycin --> good prognosis w/ or w/o tx |
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INFLAMMATION: Toxoplasmic chorioretinitis
-Epidemiology? -Type of organism? -Features of congenital toxo? |
Toxoplasmic chorioretinitis
-T. gondii (obligate intracellular parasitic protozoan) --> if congenital, acquired thru exposure to tissue cysts, oocytes in uncooked meat or contaminated cat feces -Epi: most common cause of posterior segment infection worldwide --> 25% of post uveitis cases in US -Congenital toxo: retinochoroiditis is most common manifestation (others: hydrocephalus, seizures, rash, hepatosplenomegaly, lymphadenopathy) --> occurs in 3/4 of cases -Other features: 58% involve macula, 65-85% bilateral, + maternal IgM suppors dx |
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INFLAMMATION: Toxoplasmic chorioretinitis
-Clinical presentation? -What entity does toxo resemble in immunosuppressed pts? -Mgmt? |
Toxoplasmic chorioretinitis
-Clinical: unilateral decrease in VA = most common sx --> DFE shows unifocal area of inflammation adj to old chorioretinal scar (pathognomonic) -Focal condensation of vitreous inflammatory cells overlying lesion -In immunocompromised pts, can resemble necrotizing herpetic retinitis -Mgmt: neuroimaging in AIDS pts (intracranial toxo seen in 29% of those w/ chorioretinitis), observe if small, extramacular lesion; treat for 6-8 wks if sight-threatening -Regimen: triple therapy (pyrimethamine, sulfadiazine, folinic acid) vs Bactrim monotherapy, low dose prednisone |
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INFLAMMATION: Toxocariasis
-Epi? -Organism, mechanism of infection? -Ocular manifestation? -Systemic manifestations? -Mgmt? |
Toxocariasis
-Epi: children, young adults w/ severe unilateral ocular inflammation -Toxocara canis --> acquired via ingestion of soil/vegetables infected w/ ova --> migrates to liver/lungs --> systemic dissemination -Ocular disease: severe uveitis or post segment granuloma (may have fibrocellular stlak extending from disc to granuloma), inc intraocular antibodies to T canis -Mgmt: intensive local, systemic steroids --> antihelminthic therapy not effective, disease worsened by death of nematode -Systemic manifestations: visceral larval migrans, fever, eosinophilia (uncommon) |
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INFLAMMATION: Lyme disease
-Organism, method of transmission? -Early stage: ocular and systemic manifestations? -Later stages: ocular and systemic manifestations? -Diagnosis? -Mgmt of early vs advanced disease? |
Lyme disease
-Borrelia burgdorferi --> transmitted by tick bites -Early: follicular conjunctivitis -Systemic: fever, malaise, arthralgias, target rash -Late: various levels of uveitis, vasculitis, optic neuritis, keratitis -Late systemic: neurologic, musculoskeletal disease -Dx: dark-field microscopy to identify spirochete; antibody testing w/ screening ELISA & confirmatory Western Blot -Mgmt (early): tetracycline, doxy, PCN -Mgmt (late): IV ceftriaxone, PCN |
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INFLAMMATION: DUSN
-Epidemiology? -Causative organisms? -Pathophysiology? -Acute vs end-stage manifestations? -Mgmt? |
Diffuse unilateral subretinal neuroretinitis
-Epi: healthy, young pts -Organisms: toxocara canis, baylisacaris procyonis, ancylostoma caninum --> disease due to single nematode migrating w/ in subretinal space -Mgmt: laser to nematode -Acute: crops of gray or yellow-white outer retinal lesions --> location helps localize nematode -Late: optic atrophy, diffuse RPE changes, abnormal ERG, arterial narrowing **Consider this diagnosis in any unilateral presentation of white dots! |
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CONGENITAL: Color vision abnormalities
-Define: trichromatism, dichromatism, protanopia, deuteranopia, tritanopia |
Color vision (cone) abnormalities
-Trichromatism: normal color vision (can use a mixture of 3 primary colors - red, green, blue - to match any color of light) -Dichromatism: have only 2 of the primary colors -Protanopia: abnormality in red-sensitive cone pigment (confuse reds and greens) -Deuteranopia: abnormality in green-sensitive cone pigment (confuse reds and greens) -Tritanopia: abnormality in blue-sensitive cone pigments (confuse blues and yellows) |
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CONGENITAL: Color vision abnormalities
-Which disorders make up the largest group of color-deficient persons? -What colors do these patients have trouble distinguishing? -What are some differences b/w hereditary and acquired color vision deficiencies? |
Color vision (cone) abnormalities
-Anomalous trichromatism (protanopia & deuteranopia) = largest group of color deficient persons --> trouble distinguishing pastel greens & pinks (but CAN distinguish pure red from pure green) -Hereditary color vision problems: almost always x-linked recessive RED-GREEN abnormalities (males more affected than females) -Acquired color vision problems: usually BLUE-YELLOW abnormalities (males and females equally affected) |
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CONGENITAL: Color vision abnormalities
-Define achromatopsia -What are the 2 forms of achromatopsia? -Clinical presentation (triad)? -ERG findings? |
Color vision (cone) abnormalities
-Achromatopsia: absence of color discrimination -2 forms of achromatopsia: rod monochromatism & blue-cone monochromatism -Clinical triad: nystagmus, photoaversion, poor VA -ERG: normal rod ERG but absent conventional cone responses |
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CONGENITAL: Color vision abnormalities
-Pathophysiology of rod monochromatism? -Inheritance? -Fundus findings? -How to distinguish b/w rod monochromatism & albinism? |
Color vision abnormalities - rod monochromatism
-Pathophysiology: no cone function at all (see world in shades of gray) -Autosomal recessive -Fundus: lightly pigmented, absent macular granularity --> can be confused w/ ocular albinism -How to distinguish from ocular albinism: ERG will show normal cone responses in albinism |
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CONGENITAL: Color vision abnormalities
-Pathophysiology of blue-cone monochromatism? -Inheritance? |
Color vision (cone) abnormalities - blue-cone monochromatism
-Pathophysiology: pts have only blue-sensitive cones (few in number, not found in fovea) --> visual function mimics rod monochromatism -Caused by loss of function of both red and green cone pigment genes on X chromosome -X-linked recessive |
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CONGENITAL: Night vision abnormalities
-List names of congenital night vision abnormalities with normal and abnormal fundi -What is CSNB? -What are the 3 genetic subtypes of CSNB? |
Night vision (rod) abnormalities
-Normal fundi: CSNB -Abnormal fundi: fundus albipunctatus, Oguchi disease, enhanced S-cone syndrome -CSNB: lifelong stable abnormality of scotopic vision (vision at which rods alone operate) -3 genetic subtypes of CSNB: 1) x-linked (most common); 2) auto dominant (French Nougaret), 3) auto recessive |
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CONGENITAL: Night vision abnormalities
-What are most cases of decreased VA in CSNB associated with? -What is the typical fundus appearance in CSNB? -Most common ERG pattern? -What systemic diseases have a similar ERG pattern? -Distinguish between the complete and incomplete forms of CSNB? |
Night vision (rod) abnormalities - CSNB
-Most cases of reduced vision are assoc w/ significant MYOPIA -Normal fundus appearance -ERG: negative pattern (the maximal dark adapted response shows large A wave but absent/reduced B wave) -Negative ERG also seen in Duchenne muscular dystrophy and systemic malignant melanoma -Complete type: very poor rod function -Incomplete type: still have some rod function but an elevated dark adaptation threshold |
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CONGENITAL: Night vision abnormalities
-Are there abnormalities in rhodopsin generation in CSNB? -Presumed pathophysiology? |
Night vision (rod) abnormalities - CSNB
-Both amount and rate of rhodopsin generation are normal following a bright light bleach --> this is not the problem in CSNB -Presumed pathophysiology: communication failure b/w proximal end of photoreceptor and bipolar cell |
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CONGENITAL: Night vision abnormalities
-Pathophysiology of fundus albipunctatus? -Typical color vision and VA of pts w/ this disease? -Clinical appearance? -DDx? |
Night vision (rod) abnormalities - fundus albipunctatus
-Pathophysiology: slow recovery of normal rhodopsin levels after intense light exposure (may take hours) --> pts are symptomatically night blind with minimal rod ERG until they have spent several hours in a dark environment -GOOD color vision and VA -DFE: yellow-whitish dots in posterior pole (EXCEPT fovea) -Ddx: retinitis punctata albicans (variant of RP, severely depressed ERG, narrowed vessels), fleck retina of Kandori (large flecks, less severe night vision impairment) |
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CONGENITAL: Night vision abnormalities
-Pathophysiology of Oguchi disease? -What is the Mizuo-Nakamura phenomenon? -Pathophysiology of enhanced S-cone syndrome? -ERG and DFE findings in this disease? |
Night vision (rod) abnormalities
-Oguchi disease: slow dark adaptation, defect in retinal circuitry (not in visual pigments) -Mizuo-Nakamura phenomenon: yellowish iridescent sheen after light exposure, disappears after dark adaptation -Enhanced S-cone syndrome: photopic ERG responses resemble scotopic ones, recessive, lack rod function & have poor red-green cone function -ERG: behaves like a greatly magnified blue-cone signal -DFE: ring of RPE degeneration seen in region of vascular arcades |
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HEREDITARY: ERG types
-Photopic ERG? -Scotopic ERG? -Bright flash ERG? -30-Hz flicker ERG? -What % of total photopic responses are contributed by the macula? -What cells do the A wave and the B wave correspond to? |
ERG types
-Photopic: performed in light-adapted state, measure cone function -Scotopic: performed in dark-adapted state, uses dim flash of light below cone threshold, measure rod function -Bright flash: bright flash stimulus used during dark-adapted state --> measures both rod and cone function -30-Hz flicker: measures cone function -Macula contributes 10-15% of total photopic ERG response (therefore, ERG does not correlate w/ macular health and VA) -A wave: negative waveform, represents photoreceptors -B wave: positive/upgoing waveform, represents bipolar and Muller cells |
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HEREDITARY: Diffuse photoreceptor dystrophies
-What does it mean when a dystrophy is "rod-cone" or "cone-rod"? -What are differences in visual fields in each type? |
Diffuse photoreceptor dystrophies
-Rod predominant disease: "rod-cone" --> contracted VF, large spaces b/w small and larger isopters, partial to full ring scotomata in midequatorial region w/ small central island of vision -Cone predominant disease: "cone-rod" --> isopters are closer to each other (like onion rings), ring scotoma are closer to fixation **Note: in pure cone dystrophies, VF generally are full althouhg central scotomata are common |
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HEREDITARY: RP
-What are the characteristics of RP? -Clinical findings? |
RP
-Group of hereditary disorders that diffusely involve photoreceptor and RPE function --> progressive VF loss and abnormal ERG -Clinical: arteriolar narrowing, waxy pallor of disc, bone spicule pigmentary changes (if only RPE atrophy w/o pigment changes --> RP sine pigmento) -Macula: loss of foveal reflex and irregular vitreoretinal interface |
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HEREDITARY: RP
-ERG findings? -Which photoreceptor is predominantly affected: rods or cones? |
RP
-ERG: loss of or marked reduction of BOTH rod and cone signals (rod predominates) w/ reduction of BOTH A and B waves (because photoreceptors primarily involved) -Later in course, ERG undetectable |
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HEREDITARY: RP
-Describe clinical features of the regional variants of RP, including sectorial RP -Why is UV-light protection and antioxidant use recommended in these patients? |
RP
-Regional distribution --> sharp demarcation between affected and unaffected parts of retina; tend to be slowly progressive OR nonprogressive -Sectorial RP: 1-2 sectors of fundus involved, symmetric -Possibly related to light toxicity |
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HEREDITARY: RP
-What % of RP cases are accounted for by autosomal dominant, autosomal recessive and x-linked inheritance? -Name some genes that have been implicated in RP |
RP - genetics
-Autosomal dominant: 10-20% of cases -Autosomal recessive: 20% of cases -X-linked: 10% of cases (up to 25% in England) -No family history: up to 40% of US cases -Genes: rhodopsin (visual pigment in rods that mediates night vision), RDS/peripherin gene (peripherin is a protein in hte peripheral aspect of rod & cone photoreceptor discs) |
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HEREDITARY: RP
-Definition of Leber congenital amaurosis? -Inheritance? -Clinical findings? -ERG? |
RP - Leber congenital amaurosis
-Definition: group of RP pts w/ infantile to early childhood onset of disease, BOTH rods and cones affected -Autosomal recessive -Clinical: reduced vision from birth, wandering nystagmus, undetectable or severely impaired rod AND cone ERG responses, may later develop pigmentary changes in retina -Oculodigital reflex: rubbing/poking eyes -Normal intelligence **Note: ERG response is normally small in first few months of life --> should repeat later on to confirm |
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HEREDITARY: RP
-General prognosis for vision in RP? -Offspring of pt at risk? -Risk of deafness? -Do nutrient/vitamin supplements help? Does light modify the disease? |
RP
-Disease tends to be chronic, degenerative problem and majority of pts do well for decades -Offspring not at immediate risk unless pt has autosomal dominant disease -Risk of deafness is low if pt not born with congenital deafness (Usher syndrome presents w/ congenital deafness) -Vitamin supplements, light protection does not affect RP (no clinical evidence) |
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HEREDITARY: Cone dystrophies
-How to distinguish from congenital color blindness? -Onset? -ERG? -Visual fields? |
Cone dystrophies
-In color blindness, there are color deficits for specific colors but NO associated retinal degeneration, no progression of disease -Onset in teenage years or later adult life (can also develop rod involvement later in life) -Abnormal/nonrecordable photopic ERG, normal scotopic ERG -Normal peripheral VF |
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HEREDITARY: Cone dystrophies
-Clinical presentation? -Classic fundus finding? |
Cone dystrophies
-Clinical: progressive loss of VA and color discrimination, hemeralopia (day blindness), photophobia -DFE: bull's eye maculopathy! (classic) -Other DFE findings: temporal optic atrophy, tapetal retinal reflexes (greenish/golden sheen), retinal atrophy |
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HEREDITARY: Cone-rod dystrophies
-Definition? -Visual fields? |
Cone-rod dystrophies
-Cone-isolated ERG is proportionately worse than rod-isolated ERG (although BOTH are abnormal!) -May be less severe form of Leber congenital amaurosis -VF: expanding central scotoma over time --> leads to significant visual disability |
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HEREDITARY: Macular dystrophies
-Definition of Stargardt disease? -Inheritance? -Gene? -Clinical appearance? -IVFA? -Pathophysiologic basis for IVFA? |
Macular dystrophies - Stargardt disease
-Most common juvenile macular dystrophy -Common cause of central vision loss in adults < 50 yo -Autosomal recessive -ABCA4 gene --> ATP binding casette transporter expressed by rod outer segments -Clinical: foveal atrophy (bull's eye pattern) surrounded by discrete pisciform flecks at RPE level -IVFA: "dark choroid" (against which retinal circulation is highlighted) --> in 80% of pts with disease! -Pathophysiology of dark choroid: accumulation of lipofuscin throughout the RPE (blocks underlying choroidal hyperfluorescence) |
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HEREDITARY: Macular dystrophies
-DDx of bull's eye maculopathy? |
Macular dystrophies - DDx of bull's eye maculopathy
-Stargardt disease -Cone dystrophy -Chloroquine toxicity -Chronic macular hole -AMD -Central areolar choroidal dystrophy -Ceroid lipofuscinosis -Olivopontocerebellar atrophy |
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HEREDITARY: Macular dystrophies
-Inheritance of Best disease? -Gene? -Clinical appearance? -What % of pts develop CNV? -ERG/EOG findings? |
Macular dystrophies - Best disease
-Autosomal dominant -VMD2 gene (bestrophin protein) --> novel transmembrane chloride channel -Clinical: yellow, yolk-like macular lesion in childhood --> breaks down to leave mottled geographic atrophy -Pts maintain good vision despite appearance -20% of pts develop CNV (cause of vision loss) -ERG: normal -EOG: always abnormal --> severe loss of light response (Arden light-dark ratio < 1.5, often near 1.1) |
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HEREDITARY: Adult onset vitelliform lesions
-Gene? -Clinical appearance? -Age of onset? -EOG? -Ddx? |
Adult onset vitelliform lesions
-RDS/peripherin gene -Clinical: bilateral round/oval, yellow subfoveal lesions, 1/3 DD size with central pigmented spot --> looks like Best disease -Onset during 4th-6th decades -Asymptomatic vs mild blurring/metamorphopsia -EOG tends to be normal -Ddx: vitelliform exudative macular detachment 2/2 numerous basal laminar (cuticular) drusen OR drusenoid RPE detachment (2/2 large central coalescence of drusen) --> mimic vitelliform lesions |
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HEREDITARY: Macular dystrophies
-What is the clinical appearance of familial (dominant) drusen? -What are basal laminar (cuticular) drusen? -ERG/EOG findings? -risk of AMD? -VA? |
Macular dystrophies - Familial (dominant) drusen
-Clinical: numerous drusen of varying size, extend beyond vascular arcades and nasal to disc; younger aged pts -Basal laminar/cuticular drusen: drusen that look like numerous tiny dots -ERG/EOG normal, VA good as long as drusen are extrafoveal -Higher than normal risk of AMD with aging! |
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HEREDITARY: Macular dystrophies
-When do pattern dystrophies typically appear? -Inheritance? -Name some patterns that can be seen -Gene? -VA? EOG? |
Macular dystrophies - pattern dystrophies
-Appear in midlife -Autosomal dominant -Patterns: vitelliform, butterfly, reticular, fundus pulverulentus (coarse pigment mottling) -RDS/peripherin -May be asx or slightly blurry VA/metamorphopsia -EOG borderline or slightly reduced (consistent w/ diffuse RPE disorder) |
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HEREDITARY: Choroidal dystrophies
-How can choroideremia be distinguished from RP? -Inheritance? -Clinical features? -Gene? |
Choroidal dystrophies - Choroideremia
-How to distinguish from RP: marked atrophy of both choroid and RPE, normal retinal vessels, no optic atrophy -X-linked recessive rod-cone dystrophy (otherwise meets criteria for RP) -CHM gene --> basic RPE defect -Clinical: night blindness, slow progressive VF loss over 3-5 decades |
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HEREDITARY: Macular dystrophies
-Pathophysiology of choroideremia? -IVFA appearance? -ERG? -Findings in disease carriers? -VA? |
Macular dystrophies - choroideremia
-Pathophysiology: diffuse, progressive degeneration of RPE and choriocapillaris (first looks like pigment mottling in macula, anterior equatorial region) --> degenerates to confluent scalloped areas of RPE and choriocapillaris loss w/ preservation of larger choroidal vessels -IVFA: scalloped areas of missing choriocapillaris (hypofluorescent) next to brightly fluorescent areas of patent choriocapillaris -ERG normal early --> extinguished by midlife -Carriers: patchy subretinal black RPE mottling, normal ERG and VA -VA prognosis: good VA for 4-5 decades |
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HEREDITARY: Macular dystrophies
-Inheritance of Sorsby macular dystrophy? -Age of onset? -Clinical manifestations? -Early sign of disease? -Gene? |
Macular dystrophies - Sorsby macular dystrophy
-Autosomal dominant -TIMP3 gene (protein for extracellular matrix remodeling) -Starts around age 40 -Clinical: bilateral subfoveal CNV --> evolve into atrophic areas w/ pigment clumping around central atrophic zone ("pseudo-inflammatory" appearance) -Early sign: numerous fine drusen-like deposits beneath posterior pole RPE |
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HEREDITARY: Choroidal dystrophies
-Inheritance of gyrate atrophy? -gene? -Laboratory findings? -DFE findings? -How to distinguish from choroideremia? -Symptoms/signs? -Mgmt? |
Choroidal dystrophies - Gyrate atrophy
-Autosomal recessive -OAT (ornithine aminotransferase) gene defect -Labs: 10x elevation of plasma ornithine (toxic to choroid/RPE); confirm dx by measuring serum or plasma ornithine levels -DFE: generalized hyperpigmented fundus w/ lobular loss of RPE/choroid --> hyperpigmentation allows distinguish from choroideremia -Sx/signs: night blindnes in first decade, progressive VF/VA loss later -Mgmt: dietary restriction of arginine or vit B6 administration |
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HEREDITARY: Choroidal dystrophies
-Name the 2 central choroidal dystrophies -Inheritance of both? -Shared characteristics? |
Choroidal dystrophies - central choroidal dystrophies
-2 most important types: central areolar & North Caroline macular dystrophy -Both are autosomal dominant -Shared features: demarcated RPE/choriocapillaris atrophy within macula, normal full-field ERG --> must distinguish from acquired disease (i.e., toxo)! |
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HEREDITARY: Choroidal dystrophies
-Clinical appearance of central areolar choroidal dystrophy? -Clinical appearance of North Carolina macular dystrophy? |
Choroidal dystrophies - central choroidal dystrophies
-Central areolar: nonspecific RPE mottling in macula in younger pt --> develops to round/oval area of GA (sharply demarcated), RDS/Peripherin gene -North Carolina: begins in infancy, cluster of yellow-white lesions at RPE in macula --> increase in number and confluence --> becomes atrophic (can appear staphylomatous/excavated) |
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HEREDITARY: Inner retinal dystrophies
-What is the definition of retinoschisis? -What are the 3 forms of retinoschisis? |
Inner retinal dystrophies - Retinoschisis
-Retinoschisis = splitting of neurosensory retina -3 forms: 1) degenerative peripheral form (not inherited); 2) congenital x-linked recessive; 3) secondary form |
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HEREDITARY: Inner retinal dystrophies
-What is the phenotype of x-linked retinoschisis? -IVFA? -Where does the splitting occur in x-linked vs peripheral (degenerative) retinoschisis? |
Inner retinal dystrophies - X-linked retinoschisis
-Phenotype: foveal schisis (small cystoid spaces in fovea w/ radial striae in central macula), VA 20/200 over time -IVFA: NO leakage in cystic areas! -Splitting occurs within nerve fiber layer (in degenerative retinoschisis, splitting occurs in deeper layers of retina) - |
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HEREDITARY: Inner retinal dystrophies
-What are complications of retinoschisis? -ERG findings? -Gene implicated? What is the function of this gene? |
Inner retinal dystrophes - x-linked retinoschisis
-Complications: VH (broken vessels in area of schisis), tractional RD, increased vulnerability to trauma/mechanical injury -ERG: A wave is normal, B wave attenuated (negative waveform) -Gene: retinoschism --> localizes to all retinal neurons, essential for Muller cell health |
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HEREDITARY: Goldmann-Favre syndrome
-What is another name for this disease? -Clinical findings? -ERG? -Inheritance? |
Godmann-Favre syndrome
-AKA enhanced S-cone (or blue-cone) syndrome -Features: night blindness, increased sensitivity to blue light, pigmentary retinopathy, optically empty vitreous, varying peripheral - midperipheral VF loss -Macula: sheen-like yellow round lesions along the arcades with diffuse retinal degeneration -ERG: no response to low-intensity stimuli that normally activate rods (but slow response to high intensity stimuli) -Autosomal recessive |
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SYSTEMIC: Infantile/early childhood-onset
-What is complicated LCA? -What systemic disorders may mimic LCA? -What are ways to distinguish these diseases from primary LCA? |
Infantile/early childhood-onset syndromes
-Complicated LCA = LCA assoc w/ systemic disease -LCA-mimicking systemic diseases: Batten disease, Refsum disease, Zellweger, neonatal adrenoleukodystrophy -How to distinguish from primary LCA: presence of seizures, deterioration in mental status/school performance |
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SYSTEMIC: Bardet-Biedl complex of disease
-Features (incl retinal)? -Causative gene? -Pathophysiology? |
Bardet-Biedl complex
-Systemic features: obesity, polydactyly, hypogonadism, MR -Retina: pigment mottling and atrophy WITHOUT bone spicule changes -Multigenic (at least 12 genes) -Pathophys: defect in microtubule-based intracellular transport processes |
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SYSTEMIC: Usher syndrome
-What is Usher syndrome? -What are the 2 types? -Inheritance? |
Usher syndrome
-RP + congenital sensorineural hearing loss -Stable over time -Autosomal recessive -Type 1 (profound) & type 2 (partial hearing loss) |
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SYSTEMIC: Usher syndrome
-Pathophysiology? -Prevalence? -Other genetic conditions w/ hearing loss + pigmentary retinopathy? |
Usher syndrome
-Pathophys: defect in dynamic protein complex present in inner ear hair cells & retinal photoreceptors -Prevalence: 3/100,000 -Other causes of RP + hearing loss: Alport, Refsum, Hurler |
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SYSTEMIC: Duchenne muscular dystrophy
-What is the ERG abnormality? -What other eye disease has same ERG abnormality? -Eye findings in Duchenne patients? -Gene? |
Duchenne muscular dystrophy
-Negative ERG (normal A wave, reduced B wave) --> same as congenital stationary night blindness -Duchenne patients do NOT have night blindness (normal vision) -Dystrophin gene mutation |
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SYSTEMIC: Renal disease
-What is familial juvenile nephronophthisis? (inheritance, features) -Name 4 other diseases assoc w/ renal dysfunction and pigmentary retinopathy |
Renal disease
-Familial juvenile nephronophthisis: AR, juvenile-onset renal failure & pigmentary retinopathy -Other etiologies of pigmentary retinopathy w/ renal disease: Bardet-Biedl, Alport, Alstrom, type II membranoproliferative glomerulonephritis |
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SYSTEMIC: GI disease
-FAP features, inheritance, screening? |
GI disease: familial adenomatous polyposis
-Eye findings: bilateral multiple pigmented lesions similar to CHRPE -Autosomal dominant -Retinal lesions are marker for family members at risk for colonic polyps w/ malignant potential |
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SYSTEMIC: Dermatologic disease
-What is ichthyosis? -What are systemic diseases assoc w/ skin and retinal findings? -What are features of incontinentia pigmenti (inheritance, clinical, retina)? |
Dermatologic diseases
-Ichthyosis: abnl scaling, dryness, tightness of skin -Diseases w/ skin and retinal findings: Refsum, Sjogren-Larsson, incontinentia pigmenti -Incontinentia pigmenti: x-linked, triphasic dermatopathy & disease of eyes, teeth, CNS -Retinal findings: pigmentary changes, peripheralr etinal avascularity --> cicatricial RD |
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SYSTEMIC: Paraneoplastic retinopathy
-Pathophysiology of CAR & MAR? -Retinal antigens implicated in these diseases? |
Paraneoplastic retinopathy
-Pathophys: tumors express protein antigens that cross-react w/ retinal proteins --> antibodies react to retina --> progressive retinal degeneration -Anti-retinal antibodies made to: recoverin, alpha-enolase, arrestin, transducin, neurofilament --> pt may have multiple antibodies |
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SYSTEMIC: Paraneoplastic retinopathy
-What are features of CAR 2/2 antirecoverin antibodies? -What are features of CAR 2/2 antienolase antibodies? |
Paraneoplastic retinopathy: CAR
-Antirecoverin antibodies: rapidly progressive VF loss, both rods and cones affected, reduced A & B waves on ERG, arterial narrowing but no pigment changes early on -Antienolase antibodies: slow progression of central VF loss, cone dysfunction, eventual optic disc pallor |
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SYSTEMIC: Paraneoplastic retinopathy
-Features of MAR? -ERG of MAR? -What are antibodies directed towards in MAR? |
Paraneoplastic retinopathy: MAR
-Features: vision loss, night blindness -ERG: negative waveform (similar to CSNB) -Antibodies directed towards undefined retinal bipolar cell antigens |
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SYSTEMIC: Paraneoplastic retinopathy
-When should this dx be suspected? -What is CME in RP pts strongly assoc with? |
Paraneoplastic retinopathy
-Suspect in pt with late-onset, rapidly progressive retinal dysfunction --> consider autoimmune retinopathy or occult malignancy -CME in RP pts is strongly assoc w/ presence of antiretinal antibodies |
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SYSTEMIC: Albinism
-Pathophysiology? -What are the 2 forms of albinism? -What are the 2 types of ocular involvement in albinism that can occur in either form of albinism? |
Albinism
-Pathophys: reduced/absent synthesis of melanin -Oculocutaneous: affects eyes, skin, hair follicles -Ocular: only eyes affected -Pattern 1: congenitally poor VA + nystagmus (true albinism) -Pattern 2: normal/minimally reduced VA without nystagmus (albinoidism) |
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SYSTEMIC: Albinism
-What features do all forms of albinism share? -What is one clinical feature that distingiushes albinism from albinoidism? |
Albinism
-Both patterns have: photophobia, iris TI defects, hypopigmented fundi -True albinism: hypoplastic fovea, no luteal pigment in fovea, no pit/reflex (normal in albinoidism) |
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SYSTEMIC: Albinism
-How is ocular albinism transmitted? -How is oculocutaneous albinism inherited? -How does increase in pigment affect visual prognosis n pts w/ albinism? |
Albinism
-Ocular: x-linked recessive -Oculocutaneous: autosomal recessive (tyrosinase negative vs tyrosinase positives) -If pt develops greater pigmentation over time, better visual prognosis |
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SYSTEMIC: Albinism
-What are the 2 forms of potentially lethal oculocutaneous albinism? |
Albinism
-Chediak-Higashi: increased susceptibility to infections -Hermansky-Pudlak: platelet defect --> early bruising/bleeding; Puerto Rican origin |