Lecture 8 - Vascular Disorders and CME (part 1) Flashcards

1
Q

where is the fluid located in cystoid macular edema (CME)?

A

intraretinal in and between muller glia cells, occasionally beneath neurosensory retina (SRF) in outer plexiform layer/Henles layer = forms a petalloid or flower-petal pattern

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2
Q

what does CME look like on FA?

A

multiple small perifoveal leaks from altered capillary permeability and late pooling in petaloid pattern, disc staining

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3
Q

what does CME look like on OCT?

A

diffuse retinal thickening with cystic areas of low reflectivity more prominently in the inner nuclear layer and outer plexiform layers (honeycomb-like cystoid spaces)

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4
Q

what is the CME etiology?

A

diabetic retinopathy, CRVO, BRVO, uveitis (pars planitis), RP, ocular surgery/laser, prostaglandin analogues, CNVM, RD, choroidal hemangioma, Niacin supplement

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5
Q

when does CME happen with cataract surgery?

A

peak 6-10 weeks post-op with spontaneous resolution 95% during uncomplicated surgeries within 6 months

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6
Q

what causes CME to happen post cataract surgery?

A

post-op inflammation, surgical complications (vitreous loss or iris prolapse), possible photic effects from microscope

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7
Q

what is the treatment for CME?

A

high rate of spontaneous resolution, combination steroid + NSAID, triamcinolone acetonide injection, systemic acetazolamide (chronic/RP), anti-VEGF (DM), YAG for vitreous adhesions

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8
Q

what are the retinal findings in Coats disease?

A

vascular dilations (retinal telangiectasia), ectatic arterioles, microaneurysms, venous dilations (phlebectasias), exudates and fusiform capillary dilations (exudative RD)

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9
Q

what 3 things can patients present with Coats disease?

A
decreased VA (43%), strabismus (23%) and leukocoria (20%)
rarely a painful red eye with NVG
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10
Q

who typically presents with coats disease?

A

males, unilateral, 80% before age 20 and 66% before age 10 (more severe/acute progressive if under age 4), linked to chromosome 4

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11
Q

what is the treatment for coats disease?

A

photocoagulation, cryotherapy and in severe cases RD surgery

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12
Q

what is parafoveal (juxtafoveal) retinal telangiectasia?

A

focal retinal gliosis and telangiectasia of the capillary bed, VA loss occurs from capillary incompetence and exudation, there are refractile changes in advanced cases

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13
Q

what are the 3 types of parafoveal (juxtafoveal) retinal telangiectasia?

A

type 1 = unilateral, congenital, or acquired, type 2 = bilateral, type 3 = bilateral with retinal capillary obstruction

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14
Q

how does parafoveal (juxtafoveal) retinal telangiectasia look on FA?

A

the telangiectasic vessels leak, microaneurysms close to the macula and they are connected with small lines

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15
Q

what is seen in type 1 parafoveal (juxtafoveal) retinal telangiectasia?

A

aneurysmal telangiectasia, usually males/variant of coats disease, circinate exudation, and leber military aneurysms

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16
Q

what is seen in type 2 parafoveal (juxtafoveal) retinal telangiectasia? (most common)

A

perifoveal telangiectasia, bilateral, M = F, macular thickening, gray appearance, cystic fovea, temporal fovea, later intralesional RPE migration, CNVM, 1/3 abnormal GTT

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17
Q

what is seen in type 3 parafoveal (juxtafoveal) retinal telangiectasia?

A

progressive vision loss from the obliteration of the perifoveal capillaries

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18
Q

what is the treatment for parafoveal (juxtafoveal) retinal telangiectasia?

A

type 1 = photocoagulation and types 2/3 = anti-VEGF

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19
Q

what are some differential diagnoses for parafoveal (juxtafoveal) retinal telangiectasia?

A

BRVO, DR, CME (chronic), radiation retinopathy, carotid artery disease

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20
Q

where do arterial macroaneurysms occur?

A

2nd order arterioles, usually at an A-V junction, temporal arcades

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21
Q

who typically gets arterial macroaneurysms?

A

over age 70, HTN and can follow a CRVO

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22
Q

what causes the vision loss in arterial macroaneurysms?

A

embolic/thrombotic occlusion of the endarteriole (white infarct), sub-ILM hemorrhage or intraretinal, subretinal or vitreous hemorrhage (red infarct), capillary telangiectasia, macular edema and circinate exudation

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23
Q

what does the FA look like with arterial macroaneurysms?

A

hypofluorescence = blockage + hyperfluorescence = pooling in aneurysm

24
Q

what is the treatment for arterial macroaneurysms?

A

treat if vision threatening = mild laser adjacent to aneurysms

25
Q

what are the 3 phakomatoses?

A

retinal angiomatosis (arterial), congenital retinal arteriovenous malformations (arterial + venous) and retinal cavernous hemangioma (venous)

26
Q

what is a retinal angiomatosis?

A

spherical orange-red capillary hemangioblastoma - fed by dilated tortuous artery and drained by engorged vein (subretinal or epiretinal lesions)

27
Q

what are the complications of a retinal angiomatosis?

A

serous RD, macular exudation, vitreous hemorrhage, TRD, possible NVD/NVI, NVE

28
Q

what causes a retinal angiomatosis?

A

acquired or hereditary (AD)
von hippel lesions = retinal and optic disc lesions
von hippel-lindau lesions = retinal angiomatosis + CNS and visceral lesions

29
Q

what can cause death with retinal angiomatosis?

A

cerebellar hemangioblastoma and renal cell carcinoma

30
Q

what is the management for a retinal angiomatosis?

A

imaging to rule out CNS/visceral lesions, early diagnosis is key, wide-angle FA, photocoagulation, PDT with verteporfin and cryotherapy

31
Q

what is a congenital retinal arteriovenous malformation?

A

a developmental anomaly - no intervening capillary bed exists (racemose angioma), single or complex anastomotic system, unilateral, non-hereditary, retina or optic nerve involved

32
Q

what does a congenital retinal arteriovenous malformation look like on FA?

A

no leakage - hyperfluorescence of entire tumor

33
Q

what is a retinal cavernous hemangioma?

A

grape-like clusters of thin-walled saccular angiomatous lesions inner retina or optic nerve head, sporadic, AD inheritence

34
Q

what does a retinal cavernous hemangioma look like on FA?

A

blood flow is stagnant, slow/delayed filling, plasma-erythrocyte layering, no leakage, no RD/SRF/exudates

35
Q

what is the treatment for retinal cavernous hemangioma?

A

usually asymptomatic but can bleed from vitreous traction - treat if there is bleeding only (laser or cryotherapy)

36
Q

what causes radiation retinopathy?

A

ionizing radiation (external beam or plaque) - delayed onset (18m), slowly progressive, microangiopathy (similar to DM), exposure to doses 30-35 grays

37
Q

what are the retinal findings in radiation retinopathy?

A

CWS, retinal hemorrhages, microaneurysms, perivascular sheathing, capillary telangiectasias, macular edema, and disc edema, capillary non-perfusion on FA (NVE, NVD, NVI)

38
Q

what are some other ocular complications of radiation retinopathy?

A

optic atrophy, CRVO/CRAO, CNVM, NVG, TRD

39
Q

what is the treatment for radiation retinopathy?

A

macular edema = PRP or intravitreal injection of trimcinolone acetonide or anti-VEGF

40
Q

what is the primary cause of VA loss in patients with radiation retinopathy?

A

edema + ischemia (not the CME)

41
Q

what is the pathogenesis of sickle cell disease?

A

a base substitution in the B-globin gene results in the amnio acid replacement GAG to GTG (glutamic acid by Valine) - the sickle cell hemoglobin is deoxygenated and it can block blood flow in microcirculation

42
Q

which type of sickle cell causes the most ocular complications? systemic?

A
ocular = SC and SThal
systemic = SS (most  common)
43
Q

what causes retinopathy from sickle cell disease?

A

peripheral arteriolar occlusion leading to capillary non-perfusion - neovascularization at the border between perfused and non-perfused retina

44
Q

what are the non-proliferative signs of sickle cell?

A

conjunctival coma sign, iris atrophy, salmon patch - halo - iridescent spots, black sunburst, arterial silver wiring, foveal depression sign, optic nerve sign and angioid streaks

45
Q

what is the conjunctival coma sign in non-proliferative sickle cell?

A

dark red coma/corkskrew from vascular occlusion (SS»SC), usually in lower bulbar conjunctiva, vasodilation makes them disappear and vasoconstriction (phenylephrine) increases them

46
Q

what happens with the salmon patch in non-proliferating sickle cell?

A

pre-retinal or superficial 1DD vessel blow-out from ischemia - halo develops and changes from red to orange to salmon - residual simple with ILM reflecting at margin with refractile granules of hemosiderin-laden macrophages (may have schisis cavity ILM and neurosensory retina)

47
Q

what is the black sunburst sign in non-proliferative sickle cell?

A

intraretinal RPE hypertrophy, hyperplasia and pigment migration, may have iridescent spots, speculated appearance and perivascular location, may develop CNVM

48
Q

what is the arteriole silver wiring sign in non-proliferative sickle cell?

A

more common in HbSS - also seen in CRVO/BRVO, HTN

49
Q

what is the foveal depression sign in non-proliferative sickle cell?

A

increased FAZ, degenerative ischemic thinning of the inner retina (also seen in albinism)

50
Q

what is the optic nerve sign in non-proliferative sickle cell?

A

small red dots or linear/Y shaped configuration, pre-capillary arterioles plugged with sickled erythrocytes

51
Q

when are angioid streaks seen in non-proliferative sickle cell?

A

6% in SS and even seen in AS

52
Q

what are the proliferative stages of sickle cell?

A

1 = peripheral arteriole occlusions + peripheral nonperfusion, 2 = peripheral arteriovenular anastomoses, 3 = preretinal “sea fan” neo, 4 = vitreous hemorrhage, 5 = tractional RD

53
Q

how is proliferative sickle cell different than PDR?

A

neo PDR begins post-equatorially and PSR is more peripheral with a sea fan appearance

54
Q

what is the management for sickle cell?

A

all african americans should be screened for sickle cell trait (traumatic hyphema), ischemic optic neuropathy may result from high IOP, early AC washout with hyphema with increased IOP, cautious with CAIs (worsen sickling) and treat NVE with light laser to asvascular retina

55
Q

in sickle cell, which part of the retina do you use scatter photocoagulation?

A

all avascular areas - all the neo and capillary drop out

56
Q

when is surgery indicated in sickle cell?

A

non-clearing vitreous hemorrhage, RD, retinal tears typically at the base of the sea fan precipitated by PRP