Retina And Vitreous

Front 1

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?

Back 1

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

Front 2

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

Back 2

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

Front 3

Anatomy: Retina
-What are the layers of the retina (from innermost to outermost)?

Back 3

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

Front 4

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?

Back 4

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

Front 5

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?

Back 5

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

Front 6

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?

Back 6

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

Front 7

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?

Back 7

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

Front 8

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?

Back 8

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

Front 9

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?

Back 9

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

Front 10

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?

Back 10

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

Front 11

RETINA: Anatomy
-What happens to Bruch's membrane over time?
-What happens to Bruch's membrane in pseudoxanthoma elasticum?

Back 11

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)

Front 12

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?

Back 12

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

Front 13

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?

Back 13

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)

Front 14

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?

Back 14

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

Front 15

RETINA: IVFA
-What is autofluorescence? examples?
-What are 2 causes of hypofluorescence?

Back 15

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

Front 16

RETINA: IVFA
-What are 5 major patterns of hyperfluorescence?
-Describe appearance of each pattern

Back 16

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

Front 17

RETINA: IVFA
-What are side effects of IVFA?
-What happens if dye extravasates into skin during injection?

Back 17

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

Front 18

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.

Back 18

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

Front 19

RETINA: ICG
-What allergies should you be aware of when administering ICG?
-What are contraindications to ICG?

Back 19

ICG
-Use with caution in pts with iodine or shellfish allergy (contains 5% iodide)
-Contraindications: liver disease, use of Metformin

Front 20

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?

Back 20

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

Front 21

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?

Back 21

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

Front 22

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?

Back 22

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

Front 23

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?

Back 23

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

Front 24

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?

Back 24

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

Front 25

RETINA: ERG
-What is a foveal ERG?
-When is it useful?
-What is a multi-focal ERG?
-When is it useful?

Back 25

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)

Front 26

RETINA: ERG
-What is a bright-flash ERG and when is it useful?
-What is a pattern ERG and when is it useful?

Back 26

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

Front 27

RETINA: ERG
-What are 4 uses for ERG?

Back 27

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)

Front 28

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?

Back 28

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

Front 29

RETINA: VEP
-What is a VEP?
-What type of stimulus is preferred when measuring VEPs?
-What areas of visual function do VEPs test?

Back 29

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)

Front 30

RETINA: VEP
-What are 5 indications for VEP?

Back 30

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

Front 31

MACULA: ICSC
-Epidemiology?
-Pathophysiology?
-Symptoms?
-Associations?

Back 31

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

Front 32

MACULA: ICSC
-3 most common IVFA patterns?
-Which is most common and least common?

Back 32

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

Front 33

MACULA: ICSC
-Name 3 other tests that can be used to evaluate ICSC

Back 33

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

Front 34

MACULA: ICSC
-Name 4 diseases in the DDx of ICSC and ways to distinguish between them

Back 34

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

Front 35

MACULA: ICSC
-Prognosis?
-Treatment options?
-Criteria for consideration of laser photocoagulation?

Back 35

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)

Front 36

MACULA: Optic pit maculopathy
-Appearance? Location?
-What is the associated macular pathology? Treatment?

Back 36

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

Front 37

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?

Back 37

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

Front 38

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?

Back 38

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

Front 39

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?

Back 39

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

Front 40

MACULA: Dry AMD
-What are regressed drusen?
-What are calcified drusen?
-Causes of hyperfluorescent lesions on IVFA
-Causes of hypofluorescent lesions on IVFA

Back 40

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)

Front 41

MACULA: Dry AMD
-Name 4 diseases in the DDx of dry AMD
-How does a preferential hyperacuity perimeter work?

Back 41

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

Front 42

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?

Back 42

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
-

Front 43

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

Back 43

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!

Front 44

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?

Back 44

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)

Front 45

MACULA: Wet AMD
-Hallmark lesion? How does it form?
-Sx/signs?
-Gold standard for diagnosis?

Back 45

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

Front 46

MACULA: Wet AMD
-What are 2 angiographic patterns of CNV? Describe differences between each

Back 46

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

Front 47

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

Back 47

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

Front 48

MACULA: Wet AMD
-Describe difference between "classic" and "occult" CNV vs "poorly defined" and "well defined" CNV
-Why is this distinction important?

Back 48

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)

Front 49

MACULA: Wet AMD
-Name 6 conditions that mimic wet AMD

Back 49

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)

Front 50

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?

Back 50

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

Front 51

MACULA: Wet AMD
-How does PDT work?
-Findings of TAP study?
-Findings of VIP study?
-What are the FDA-approved indications of PDT?

Back 51

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

Front 52

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?

Back 52

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

Front 53

MACULA: Wet AMD
-What is pegaptanib?
-VISION trial results?

Back 53

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

Front 54

MACULA: Wet AMD
-What is ranibizumab?
-MARINA trial results?
-ANCHOR trial results?

Back 54

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

Front 55

MACULA: Wet AMD
-What is bevacizumab?
-What 2 upcoming studies are looking at combination treatment? What treatments are being evaluated by each?

Back 55

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

Front 56

MACULA: OHS
-Epidemiology?
-Disease transmission?
-4 clinical signs of OHS?
-Sx?

Back 56

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!

Front 57

MACULA: OHS
-What did MPS show re: laser treatment of OHS?
-Which type of CNV does not benefit from laser?
-VOH study results?

Back 57

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)

Front 58

MACULA: Angioid streaks
-What are angioid streaks?
-IVFA appearance?
-What is the main vision-limiting effect?

Back 58

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

Front 59

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?

Back 59

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

Front 60

MACULA: Pathologic myopia
-What % of population is myopic? pathologically so?
-How is high vs pathologic myopia classified?
-Fundus findings?

Back 60

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

Front 61

MACULA: Pathologic myopia
-Risk of CNV?
-VIP Pathologic Myopia trial results?
-What is expanding RPE atrophy?

Back 61

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)

Front 62

MACULA: ERM
-What is an ERM? Cellular composition? Location?
-Difference b/w idiopathic and secondary ERM?

Back 62

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

Front 63

MACULA: ERM
-Clinical appearance? (early vs late appearance, vascular changes, macular edema)

Back 63

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

Front 64

MACULA: ERM
-Treatment?
-Critera for surgery?

Back 64

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

Front 65

MACULA: Vitreomacular traction syndrome
-How does VMT occur?
-What happens to the macula in VMT?

Back 65

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

Front 66

MACULA: Macular holes
-Epidemiology?
-Stage 0?
-Stage 1? Prognosis?
-Stage 2?
-Stage 3? typical VA?
-Stage 4? How differentiated from stage 3?

Back 66

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)

Front 67

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?

Back 67

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

Front 68

MACULA: Valsalva retinopathy
-Pathophysiology?
-Location of heme?
-Prognosis?
-DDx?

Back 68

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

Front 69

MACULA: Purtscher retinopathy
-Causes of Purtscher and Purtscher-like retinopathy?
-Fundus appearance?
-IVFA?
-Consequences for vision?
-Pathophysiology?

Back 69

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)

Front 70

MACULA: Terson syndrome
-What is the clinical appearance and pathophysiology of Terson syndrome?
-Visual consequence?

Back 70

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

Front 71

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?

Back 71

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)

Front 72

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?

Back 72

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

Front 73

VASCULAR: HTN
-What patient population gets HTNsive choroidopathy?
-What are the 2 characteristic choroidal lesions?
-IVFA appearance?

Back 73

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

Front 74

VASCULAR: HTN
-What are characteristics of HTNsive optic neuropathy?
-Does HTNsive optic neuropathy depend more on severity or chronicity of HTN?

Back 74

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

Front 75

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?

Back 75

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)

Front 76

VASCULAR: DR
-What is the presumed pathogenesis of DR?
-What are the 3 categories of conditions associated w/ vision loss in DR?

Back 76

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

Front 77

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?

Back 77

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)

Front 78

VASCULAR: DR
-What is the difference b/w focal & diffuse macular edema?

Back 78

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

Front 79

VASCULAR: DR
-What were the findings of EDTRS?
-What was the primary outcome in EDTRS?
-What are 3 definitions of CSME?

Back 79

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

Front 80

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?

Back 80

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

Front 81

VASCULAR: DR
-Diabetic macular ischemia: clinical exam and IVFA findings?
-What is indicative of vision loss in diabetic macular ischemia?

Back 81

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

Front 82

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?

Back 82

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

Front 83

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?

Back 83

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!

Front 84

VASCULAR: DR
-What type of patient population was used and what were the main findings of the DCCT and UKPDS?

Back 84

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**

Front 85

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?

Back 85

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

Front 86

VASCULAR: DR
-What are some side effects of PRP?
-In what pattern should PRP be performed?

Back 86

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

Front 87

VASCULAR: DR
-What was the purpose and findings of the DRS?
-Definition of high risk PDR?

Back 87

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

Front 88

VASCULAR: DR
-What is the appearance of concerning and non-concerning NVI?
-What are the 2 main sequelae of PDR that require surgical mgmt?

Back 88

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

Front 89

VASCULAR: DR
-What was the purpose and findings of the DRVS?
-What is the mechanism of a tractional RD in DR?

Back 89

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

Front 90

VASCULAR: DR
-5 most common indications for PPV in DR?

Back 90

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

Front 91

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?

Back 91

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

Front 92

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?

Back 92

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

Front 93

VASCULAR: Sickle cell retinopathy
-What is the pathophysiologic change in sickle cell hemoglobin?
-Which hemoglobinopathies are most frequently assoc with retinopathy?
-Pathophysiology of retinopathy?

Back 93

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

Front 94

VASCULAR: Sickle cell retinopathy
-What are the 3 most common findings of nonproliferative sickle cell retinopathy?
-Pathophysiology?

Back 94

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)

Front 95

VASCULAR: Sickle cell retinopathy
-Describe the 5 pathogenic stages of proliferative sickle cell retinopathy
-What are 2 differences b/w PDR and PSR?

Back 95

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)

Front 96

VASCULAR: Sickle cell retinopathy
-What are 3 other ocular abnormalities in SS or SC disease?

Back 96

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)

Front 97

VASCULAR: Sickle cell retinopathy
-Mgmt of black pts w/ hyphema?

Back 97

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)

Front 98

VASCULAR: Sickle cell retinopathy
-How should laser be used in mgmt of PSR?

Back 98

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

Front 99

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?

Back 99

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

Front 100

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?

Back 100

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

Front 101

VASCULAR: ROP
-What are criteria for conclusion of ROP exams?
-What is the criteria for prethreshold disease?

Back 101

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

Front 102

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?

Back 102

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

Front 103

VASCULAR: ROP
-What are the 2 distinct processes by which vascularization occurs? Where does each process occur in the retina?

Back 103

ROP
-Vasculogenesis: de novo formation of new vessels --> zone I disease
-Angiogenesis: formation of new vessels by budding from existing vessels --> zone II disease

Front 104

VASCULAR: ROP
-What is the presumed pathophysiology of ROP?
-What factors aside from high O2 exposure may increase risk of ROP?

Back 104

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

Front 105

VASCULAR: ROP
-Define the 5 stages of ROP

Back 105

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

Front 106

VASCULAR: ROP
-Define the 3 zones of ROP
-Which zones have the most favorable prognosis?

Back 106

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

Front 107

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?

Back 107

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

Front 108

VASCULAR: ROP
-What % of eyes have spontaneous regression?
-What is the appearance of regressing ROP?

Back 108

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

Front 109

VASCULAR: BRVO
-Exam findings in BRVO?
-Most common location?
-If not at typical location, what dx should be considered?
-4 risk factors for BRVO?

Back 109

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

Front 110

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?

Back 110

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

Front 111

VASCULAR: BRVO
-What is an indication for scatter laser in BRVO according to the BVOS?

Back 111

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)

Front 112

VASCULAR: CRVO
-Describe fundus appearance, IVFA appearance and clinical exam findings of nonischemic CRVO

Back 112

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

Front 113

VASCULAR: BRVO
-What are 2 indications for laser in BRVO? What study provided the basis for these criteria?
-What areas are targeted by laser?

Back 113

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

Front 114

VASCULAR: CRVO
-Describe fundus appearance, IVFA appearance and other clinical exam findings of ischemic CRVO

Back 114

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

Front 115

VASCULAR: CRVO
-Pathophysiology?

Back 115

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)

Front 116

VASCULAR: CRVO
-Most common age of presentation?
-3 most common systemic associations?
-Other meds, systemic conditions assoc with CRVO?

Back 116

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

Front 117

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?

Back 117

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)

Front 118

VASCULAR: Carotid occlusive disease retinopathy
-What is one method of differentiating between CRVO and this entity?

Back 118

Carotid artery occlusive disease
-Ophthalmodynamometry: CRVO shows normal CRA pressure but carotid disedase will show low CRA pressures

Front 119

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?

Back 119

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

Front 120

VASCULAR: BRAO
-Acute clinical appearance?
-Chronic appearance?
-What are 3 types of emboli?

Back 120

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)

Front 121

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?

Back 121

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)

Front 122

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?

Back 122

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

Front 123

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?

Back 123

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

Front 124

VASCULAR: Ocular ischemic syndrome
-Pathophysiology?
-Presentation?
-IVFA findings?

Back 124

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

Front 125

VASCULAR: Ocular ischemic syndrome
-ERG findings?
-5 year mortality?
-Most frequent cause of death?
-Mgmt for NVI?
-Definitive treatment?

Back 125

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

Front 126

VASCULAR: Vasculitis
-Early fundus findings?
-Which vessels involved?
-What is Eales disease?
-What is IRVAN syndrome?

Back 126

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

Front 127

VASCULAR: CME
-Pathophysiology?
-Which retinal layers are involved? Which cells?
-IVFA pattern?

Back 127

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**

Front 128

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?

Back 128

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

Front 129

VASCULAR: Coats' disease
-Clinical appearance?
-IVFA appearance?
-Pathophysiology?

Back 129

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)

Front 130

VASCULAR: Coats' disease
-Systemic associations?
-Inheritance?
-Epidemiology?
-Disease course?

Back 130

Coats' disease
-Sporadic
-Progresses over time w/ increased exudation
-85% of pts are males
-No associated systemic vascular abnormalities

Front 131

VASCULAR: Coats' disease
-Mgmt?
-DDx?

Back 131

Coats' disease
-DDx: familial exudative vitreoretinopathy, ROP, von Hippel disease, facioscapulohumeral muscular dystrophy
-Mgmt: laser, cryo or RD surgery

Front 132

VASCULAR: Juxtafoveal retinal telangiectasias
-Clinical appearance?
-Main cause of visual loss?
-Pathophysiology?

Back 132

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)

Front 133

VASCULAR: Juxtafoveal retinal telangiectasias
-What are features of Groups 1, 2 & 3? (laterality, clinical appearance, associated abnormalities, mgmt)

Back 133

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)

Front 134

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?

Back 134

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

Front 135

VASCULAR: Retinal arterial macroaneurysm
-Frequency of recurrence?
-Mgmt?

Back 135

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

Front 136

VASCULAR: Capillary hemangioblastomas
-Clinical appearance?
-Laterality?
-Pathophysiology?
-Inheritance?

Back 136

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

Front 137

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?

Back 137

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!

Front 138

VASCULAR: Congenital AVM
-What is a racemose angioma?
-Laterality? Inheritance?
-IVFA?
-What is Wyburn-Mason syndrome?

Back 138

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

Front 139

VASCULAR: Retinal cavernous hemangioma
-Inheritance?
-Clinical appearance?
-IVFA?

Back 139

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!

Front 140

VASCULAR: Retinal cavernous hemangioma
-Mgmt?

Back 140

Retinal cavernous hemangioma
-Asymptomatic --> no treatment unless recurrent VH develops (use laser, cryo)

Front 141

VASCULAR: Radiation retinopathy
-What disease does it most resemble?
-Timeline b/w exposure & onset, degree of exposure required?
-Mgmt?

Back 141

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

Front 142

CHOROID: BDUMP
-Clinical appearance?
-Important systemic association?
-IVFA appearance?

Back 142

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

Front 143

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?

Back 143

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

Front 144

CHOROID: Perfusion abnormalities
-Name 3 other disorders that can cause retinal/choroidal vascular occlusion
-Name 3 iatrogenic causes of choroidal vascular occlusion

Back 144

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

Front 145

CHOROID: Choroidal hemangioma
-Clinical appearance?
-ICG pattern?
-Visual consequences?

Back 145

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

Front 146

CHOROID: Choroidal hemangioma
-Sturge-Weber: how is associated hemangioma different from choroidal hemangioma?
-Mgmt of choroidal hemangioma?

Back 146

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

Front 147

CHOROID: Uveal effusion syndrome
-Pathophysiology?
-Associated eye conditions?
-Consequeces of disease?
-IVFA?
-Mgmt?

Back 147

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

Front 148

INFLAMMATION: APMPPE
-Epidemiology?
-Laterality?
-Clinical appearance (level of retina, appearance of lesion)?
-Pathophysiology?
-IVFA?
-Prognosis, treatment?
-Ddx?

Back 148

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

Front 149

INFLAMMATION: Serpiginous choroidopathy
-Clinical appearance?
-Symptoms?
-IVFA?
-Prognosis, treatment?

Back 149

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

Front 150

INFLAMMATION: MEWDS
-Clinical appearance?
-Laterality?
-Epidemiology?
-IVFA?
-VF?
-ERG?
-Mgmt?
-Ddx?

Back 150

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)

Front 151

INFLAMMATION: Birdshot choroidopathy
-Clinical presentation?
-Sx?
-Epidemiology?
-ERG?
-IVFA?
-Prognosis, treatment?

Back 151

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

Front 152

INFLAMMATION: Multifocal choroiditis and panuveitis syndrome
-Clinical?
-Sx?
-Epi?
-Ddx?
-Mgmt?
-Prognosis?

Back 152

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)

Front 153

INFLAMMATION: Punctate inner choroidopathy
-Clinical?
-Sx?
-Epi?
-IVFA?
-Ddx?
-Prognosis?

Back 153

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

Front 154

INFLAMMATION: Acute zonal occult outer retinopathy
-Clinical?
-Sx?
-IVFA?
-ERG?
-Epi?
-Ddx?
-Mgmt?

Back 154

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

Front 155

INFLAMMATION: Vasculitis - Bechet's
-Classic triad of sx?
-Pathophysiology?
-Epidemiology, genetics?
-Ocular manifestations?
-Mgmt?
-Prognosis?

Back 155

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

Front 156

INFLAMMATION: Vasculitis - SLE
-Clinical?
-Pathophysiology?
-Epidemiology?
-Mgmt?

Back 156

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

Front 157

INFLAMMATION: Intermediate uveitis - Pars planitis
-Ddx of intermediate uveitis?
Pars planitis...
-Clinical appearance?
-IVFA?
-Epidemiology?
-Mgmt?

Back 157

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

Front 158

INFLAMMATION: Panuveitis - Sarcoid
-What % of sarcoid pts have ocular manifestations?
-Anterior and posterior segment manifestations?

Back 158

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

Front 159

INFLAMMATION: Panuveitis - VKH
-Epidemiology, genetics?
-Skin, neurologic signs?
-What is Harada disease?
-IVFA?
-Mgmt?

Back 159

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

Front 160

INFLAMMATION: Panuveitis - VKH
-Describe the 4 stages of disease
-What is Sigiura sign?
-What is a "sunset glow" fundus?
-What are "Dalen Fuchs" nodules?

Back 160

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

Front 161

INFLAMMATION: Panuveitis - Sympathetic ophthalmia
-How to distinguish from VKH?
-Incidence?
-Clinical appearance?
-Prognosis, treatment?

Back 161

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

Front 162

INFLAMMATION: Infiltrative uveitis - intraocular lymphoma
-Clinical appearance?
-Epidemiology?
-Distinctive finding on exam?
-Confirmation of Dx?
-Prognosis, mgmt?

Back 162

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

Front 163

INFLAMMATION: CMV retinitis
-Patient population?
-Clinical appearance?
-Mgmt? (IV vs intravitreal)
-Complications?

Back 163

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)

Front 164

INFLAMMATION: Necrotizing herpetic retinitis - ARN
-Organisms?
-Clinical presentation?
-Complications?
-Laterality?
-Mgmt?
-When is risk of RD highest?

Back 164

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

Front 165

INFLAMMATION: Necrotizing herpetic retinitis - PORN
-Patient population?
-How to distinguish from ARN?

Back 165

PORN
-Immunocompromised pts
-Compared to ARN: more rapid progression, no vitritis, sparing of retinal vessels

Front 166

INFLAMMATION: Endogenous bacterial endophthalmitis
-Clinical appearance?
-Organisms?
-Most common associated etiologies?
-Mgmt?

Back 166

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

Front 167

INFLAMMATION: Candidal endophthalmitis
-Risk factors?
-Clinical appearance?
-Mgmt?
-Prognosis?

Back 167

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

Front 168

INFLAMMATION: Aspergillus endophthalmitis
-Pt population?
-Clinical?
-Mgmt?
-Prognosis?

Back 168

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

Front 169

INFLAMMATION: Tuberculosis
-Most common site of intraocular Tb?
-Clinical findings?
-Mgmt?

Back 169

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)

Front 170

INFLAMMATION: Syphilitic chorioretinitis
-Which stage of syphilis does this occur in?
-Testing?
-Characteristic finding?
-Mgmt?

Back 170

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

Front 171

INFLAMMATION: Cat-scratch disease
-Organism, mechanism of infection?
-Systemic findings?
-What is Parinaud oculoglandular syndrome?
-Typical retinal findings?
-Mgmt, prognosis?

Back 171

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

Front 172

INFLAMMATION: Toxoplasmic chorioretinitis
-Epidemiology?
-Type of organism?
-Features of congenital toxo?

Back 172

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

Front 173

INFLAMMATION: Toxoplasmic chorioretinitis
-Clinical presentation?
-What entity does toxo resemble in immunosuppressed pts?
-Mgmt?

Back 173

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

Front 174

INFLAMMATION: Toxocariasis
-Epi?
-Organism, mechanism of infection?
-Ocular manifestation?
-Systemic manifestations?
-Mgmt?

Back 174

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)

Front 175

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?

Back 175

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

Front 176

INFLAMMATION: DUSN
-Epidemiology?
-Causative organisms?
-Pathophysiology?
-Acute vs end-stage manifestations?
-Mgmt?

Back 176

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!

Front 177

CONGENITAL: Color vision abnormalities
-Define: trichromatism, dichromatism, protanopia, deuteranopia, tritanopia

Back 177

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)

Front 178

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?

Back 178

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)

Front 179

CONGENITAL: Color vision abnormalities
-Define achromatopsia
-What are the 2 forms of achromatopsia?
-Clinical presentation (triad)?
-ERG findings?

Back 179

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

Front 180

CONGENITAL: Color vision abnormalities
-Pathophysiology of rod monochromatism?
-Inheritance?
-Fundus findings?
-How to distinguish b/w rod monochromatism & albinism?

Back 180

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

Front 181

CONGENITAL: Color vision abnormalities
-Pathophysiology of blue-cone monochromatism?
-Inheritance?

Back 181

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

Front 182

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?

Back 182

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

Front 183

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?

Back 183

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

Front 184

CONGENITAL: Night vision abnormalities
-Are there abnormalities in rhodopsin generation in CSNB?
-Presumed pathophysiology?

Back 184

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

Front 185

CONGENITAL: Night vision abnormalities
-Pathophysiology of fundus albipunctatus?
-Typical color vision and VA of pts w/ this disease?
-Clinical appearance?
-DDx?

Back 185

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)

Front 186

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?

Back 186

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

Front 187

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?

Back 187

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

Front 188

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?

Back 188

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

Front 189

HEREDITARY: RP
-What are the characteristics of RP?
-Clinical findings?

Back 189

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

Front 190

HEREDITARY: RP
-ERG findings?
-Which photoreceptor is predominantly affected: rods or cones?

Back 190

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

Front 191

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?

Back 191

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

Front 192

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

Back 192

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)

Front 193

HEREDITARY: RP
-Definition of Leber congenital amaurosis?
-Inheritance?
-Clinical findings?
-ERG?

Back 193

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

Front 194

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?

Back 194

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)

Front 195

HEREDITARY: Cone dystrophies
-How to distinguish from congenital color blindness?
-Onset?
-ERG?
-Visual fields?

Back 195

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

Front 196

HEREDITARY: Cone dystrophies
-Clinical presentation?
-Classic fundus finding?

Back 196

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

Front 197

HEREDITARY: Cone-rod dystrophies
-Definition?
-Visual fields?

Back 197

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

Front 198

HEREDITARY: Macular dystrophies
-Definition of Stargardt disease?
-Inheritance?
-Gene?
-Clinical appearance?
-IVFA?
-Pathophysiologic basis for IVFA?

Back 198

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)

Front 199

HEREDITARY: Macular dystrophies
-DDx of bull's eye maculopathy?

Back 199

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

Front 200

HEREDITARY: Macular dystrophies
-Inheritance of Best disease?
-Gene?
-Clinical appearance?
-What % of pts develop CNV?
-ERG/EOG findings?

Back 200

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)

Front 201

HEREDITARY: Adult onset vitelliform lesions
-Gene?
-Clinical appearance?
-Age of onset?
-EOG?
-Ddx?

Back 201

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

Front 202

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?

Back 202

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!

Front 203

HEREDITARY: Macular dystrophies
-When do pattern dystrophies typically appear?
-Inheritance?
-Name some patterns that can be seen
-Gene?
-VA?
EOG?

Back 203

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)

Front 204

HEREDITARY: Choroidal dystrophies
-How can choroideremia be distinguished from RP?
-Inheritance?
-Clinical features?
-Gene?

Back 204

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

Front 205

HEREDITARY: Macular dystrophies
-Pathophysiology of choroideremia?
-IVFA appearance?
-ERG?
-Findings in disease carriers?
-VA?

Back 205

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

Front 206

HEREDITARY: Macular dystrophies
-Inheritance of Sorsby macular dystrophy?
-Age of onset?
-Clinical manifestations?
-Early sign of disease?
-Gene?

Back 206

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

Front 207

HEREDITARY: Choroidal dystrophies
-Inheritance of gyrate atrophy?
-gene?
-Laboratory findings?
-DFE findings?
-How to distinguish from choroideremia?
-Symptoms/signs?
-Mgmt?

Back 207

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

Front 208

HEREDITARY: Choroidal dystrophies
-Name the 2 central choroidal dystrophies
-Inheritance of both?
-Shared characteristics?

Back 208

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)!

Front 209

HEREDITARY: Choroidal dystrophies
-Clinical appearance of central areolar choroidal dystrophy?
-Clinical appearance of North Carolina macular dystrophy?

Back 209

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)

Front 210

HEREDITARY: Inner retinal dystrophies
-What is the definition of retinoschisis?
-What are the 3 forms of retinoschisis?

Back 210

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

Front 211

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?

Back 211

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)
-

Front 212

HEREDITARY: Inner retinal dystrophies
-What are complications of retinoschisis?
-ERG findings?
-Gene implicated? What is the function of this gene?

Back 212

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

Front 213

HEREDITARY: Goldmann-Favre syndrome
-What is another name for this disease?
-Clinical findings?
-ERG?
-Inheritance?

Back 213

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?

Back 214

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

Front 215

SYSTEMIC: Bardet-Biedl complex of disease
-Features (incl retinal)?
-Causative gene?
-Pathophysiology?

Back 215

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?

Back 216

Usher syndrome
-RP + congenital sensorineural hearing loss
-Stable over time
-Autosomal recessive
-Type 1 (profound) & type 2 (partial hearing loss)

Front 217

SYSTEMIC: Usher syndrome
-Pathophysiology?
-Prevalence?
-Other genetic conditions w/ hearing loss + pigmentary retinopathy?

Back 217

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

Front 218

SYSTEMIC: Duchenne muscular dystrophy
-What is the ERG abnormality?
-What other eye disease has same ERG abnormality?
-Eye findings in Duchenne patients?
-Gene?

Back 218

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

Front 219

SYSTEMIC: Renal disease
-What is familial juvenile nephronophthisis? (inheritance, features)
-Name 4 other diseases assoc w/ renal dysfunction and pigmentary retinopathy

Back 219

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

Front 220

SYSTEMIC: GI disease
-FAP features, inheritance, screening?

Back 220

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

Front 221

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)?

Back 221

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

Front 222

SYSTEMIC: Paraneoplastic retinopathy
-Pathophysiology of CAR & MAR?
-Retinal antigens implicated in these diseases?

Back 222

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

Front 223

SYSTEMIC: Paraneoplastic retinopathy
-What are features of CAR 2/2 antirecoverin antibodies?
-What are features of CAR 2/2 antienolase antibodies?

Back 223

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

Front 224

SYSTEMIC: Paraneoplastic retinopathy
-Features of MAR?
-ERG of MAR?
-What are antibodies directed towards in MAR?

Back 224

Paraneoplastic retinopathy: MAR
-Features: vision loss, night blindness
-ERG: negative waveform (similar to CSNB)
-Antibodies directed towards undefined retinal bipolar cell antigens

Front 225

SYSTEMIC: Paraneoplastic retinopathy
-When should this dx be suspected?
-What is CME in RP pts strongly assoc with?

Back 225

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

Front 226

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?

Back 226

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?

Back 227

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)

Front 228

SYSTEMIC: Albinism
-How is ocular albinism transmitted?
-How is oculocutaneous albinism inherited?
-How does increase in pigment affect visual prognosis n pts w/ albinism?

Back 228

Albinism
-Ocular: x-linked recessive
-Oculocutaneous: autosomal recessive (tyrosinase negative vs tyrosinase positives)
-If pt develops greater pigmentation over time, better visual prognosis

Front 229

SYSTEMIC: Albinism
-What are the 2 forms of potentially lethal oculocutaneous albinism?

Back 229

Albinism
-Chediak-Higashi: increased susceptibility to infections
-Hermansky-Pudlak: platelet defect --> early bruising/bleeding; Puerto Rican origin