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PPO > post midterm week 1 > Flashcards

post midterm week 1 Flashcards

1
Q

what characterizes distrophies?

A

hereditary/genetic
bilateral
symmetric

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2
Q

macular distrophies are classified based on what

A

mode of transmission
age of onset
central vs peripheral

cone vs rod
symptoms
diagnostic testing
histology

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3
Q

what are the risk factors associated with macular distrophies

A

family history / genetics

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4
Q

electro retinopathy (ERG) represents the activity of what

A

photo receptors and bipolar region

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5
Q

electro oculography reflects the activity of what

A

RPE and the photoreceptors (diffuse or wide spread disease of the RPE is needed to affect the EOG response significantly)

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6
Q

what does hyper fluorescence vs hypo represent on fundus photography

A 
hyper = increased metabolic activity which = injeopardy/abnormal
hypo = dead
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7
Q

what is the treatment for distrophies

A

just supportive really.

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8
Q

what is the inheritance pattern for progressive cone distrophy / cone rod distrophy

A

sproadic can be ad ar or xl

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9
Q

when does cone rod distrophy present

A

presents in 1st to 3rd decade

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10
Q

what can you see clinically with progressive cone distrophy and what is the VA

A

photophobia and nystagmus in late stage

end stage = 20/200 vision

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11
Q

in congenital monochromatism or complete rod chromatism what is the inheritance pattern

A

AR

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12
Q

in congenital monochromatism or complete rod chromatism what is the visual acuity and other clinical signs.
is ERG or EOG affected?

A 
VA = 20/200 (because this is best acuity with rods)
macula usually appears normal
congenital nystagmus and photophobia
ERG phototopic abnormal.
color vision totally absent
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13
Q

what is the inheritance pattern in congenital monochromatism or incomplete rod chromatism

A

AR or XL

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14
Q

what is the inheritance pattern in congenital monochromatism or incomplete rod chromatism.
what is VA. what other clinical signs

A 
VA is 20/40 - 20/80
macula is normal
nystagmus and photophobia may be present
ERG photopic abnormal
some color vision may be present
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15
Q

what is cone monochromatism? (what is the inheritance, what is VA, what do you see clinically)

A 
inheritance is uncertain.
VA is normal 20/20 -20/30
macula normal
nystagmus and photophobia absent
ERG and color vision are normal
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16
Q

what is dominant cystoid maculaEdema(inheritance, when does it start, etc.)

A

inheritance is AD
presents in first to second decade
with gradual impairment of central vision
DOES NOT respond to systemic acetazolamide

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17
Q

what do you see on EOG, ERG, FA

and what is the prognosis of dominant cystoid maculaedema

A

ERG, EOG are normal
FA = flower pedal pattern of leakage at the fovia
prognosis is poor due to risk of geographic attrophy

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18
Q

what is stargardt disease (inheritance, presentation)

A

inheritance = AR
presents in 1st to 2nd decade
vision impairment may be out of proportion to macular changes

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19
Q

what other signs of the fovia might you see with stargardt disease

A

normal or nonspecific modeling.
oval snail slime or beaten bronze fovial appearance. may be surrounded by yellow white flecks. may be geographic atrophy. maybe bullseye.

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20
Q

what do diagnostic tests reveal with stargardt disease

A

ERG is normal
EOG is abnormal in advanced cases
Colorvision defects
FA = dark cortoid due to lipofuscin deposits in the RPE
maybe window defect at macula due to geographic atrophy

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21
Q

what is prognosis for stargardt

A

poor

once VA drops to 20/40 there is a rapid decrease until stabilizes at 20/600

22
Q

what is juvenile best disease (inheritance and presentation)

A

inheritance = ad
presents 3-15 years. avg age 6.
VA may be good for many years.
Atrophic stage usually occurs after age 40.

23
Q

What signs will you see in the macula with best disease?

A

lypofuscin in the RPE and subRPE.

maybe RPE changes and photoreceptor loss.

24
Q

what is stage 0 or previtelliform of best disease

A

subnormal EOG

asymptomatic child with normal fundus

25
Q

what is stage 1 of best disease

A

pigment modeling at macula

26
Q

what is stage 2 of best disease/ vitelliform

A

develops in 1st to 2nd decade.
characterized by sunny side up macular degeneration
with subretinal lypofuscin
VA is normal or slight decrease

27
Q

what is stage 3 /pseudohypopyon of best disease

A

occurs when part of the lesion becomes absorbed

sometimes this doesnt affect vision

28
Q

what is stage 4 of best diease (vitelliruptive)

A

egg yolk begins to break up/scrambled egg.

VA for sure drops

29
Q

what is stage 5 of best disease or atropic

A

when yellow material disappears and RPE atrophy remains

VA can deteriorate more.

30
Q

what is stage 6 of best disease

A

choroidal neo develops

leads to wider subretinal fiber scar

31
Q

what will diagnostic tests show with best disease

A

ERG = normal
EOG is abnormal always
possible colorvision defects
fa affected

32
Q

what is the prognosis of best disease

A

good until 5th decade. complications may lead to legal blindness

33
Q

what is adult vitellaform fovia macular distrophy (inheritance, presentation, signs)

A

inheritance is probably ad
presents in fourth to sixth decade with mild metamorphopsia
macula is roudn slightly elevated yellow subfovial lesions about 1/3 dist diameter in size

34
Q

what do you see on diagnostic test on adult vitellaform fovia macular distrophy

A

ERG and EOG usually normal

mild triton color vision defect

35
Q

what is the prognosis of adult vitellaform fovia macular distrophy

A

good in the majority of cases

36
Q

what is sorsey pseudoinflammatory macular distrophy (inheritance, prsentation, signs)

A

inheritance is ad
presents in 5th decade with bilateral exudative maculopathy
yellow white drusen posits along arcades
nasal to the disk in mid periphery
CNV and exudative maculopathy and subretinal scarring.

37
Q

in sorsey pseudoinflammatory macular distrophy what do diagnostic tests reveal and what is the prognosis

A

ERG may be abnormal in late disease
prognosis is poor due to maculopathy
may lose vision by 7th decade

38
Q

what is butterfly macular distrophy(inheritance, presentation, signs)

A

inher = ad
presents in 2nd to 5th decade
signs = yellow fovial pigment that loks like a butterfly

39
Q

butterfly macular distrophy, what do the diagnostic tests reveal and what is the prognosis

A

ERG = normal
EOG = abnormal
FA = hypo
prognosis is excellent

40
Q

what are the inheritance patterns of RP

A 
AD = common, best prognosis
AR = less common, intermediate prognosis
XL = least common, most severe
41
Q

when does RP usually present

A

during the 3rd decade, but possibly sooner.

42
Q

what fundus changes do you see in RP

A

arteriorlar narrowing. mid peripheral bone spicules, waxy disk palor. tessellated fundus maculopathy (atrophic cellophane or CME)

43
Q

what do you see with diagnostic tests for RP

A

ERG = reduced
EOG = abnormal
colorvision is normal
fa is diffuse hyper, small areas of hypo

44
Q

what are some ocular associations of RP

A

most common = posterior sub cataracts

open angle glaucoma
myopia
keratoconus
vitreous changes
optic disk drusen
45
Q

give three examples of atypical RP

A

sector RP
perycentral RP
RP with exudative vasculopathy

46
Q

what are three subtypes of congenital stationary nightblindness with a normal fundus

A

ad congenital
ad stationary without myopia
AR or exo congenital with myopia

47
Q

give two examples of congenital stationary nightblindness that present with abnormal fundus

A

oguchi disease
a change in fundus color during dark adaptation
fundus albipunctatus spares the fovia
ERG and EOG may be abnormal but then revert back to normal after dark adaptation

48
Q

what is fundus flavimaculatus

A

variation of stargardts but macula is not affected
AR inheritance
maybe asymptomatic
presents in 2nd to 3rd decade

49
Q

what are some clinical signs of fundus flavimaculatus

A

yellow white flecks in posterior pole and midperiphery.

uncommon, but can get geographic attrophy in some cases.

50
Q

what diagnostic tests are affected in fundus flavimaculatus and what is the prognosis

A 
ERG: photopic, possibly slightly abnormal
EOG: abnormal
colorvision normal
FA = generalized dark chortoroid
prognosis is good
51
Q

what is familiar / dominant drusen (inheritance patterns, what diagnostic tests are abnormal)

A 
inheritance = ad
diagnostic tests:
ERG = normal
EOG= abnormal in advanced cases
FA = hyper spots
52
Q

what are the clinical features of mild , moderate, and advanced familiar or dominant drusen

A

mild=few small hard drusen in macula, usually in 3rd decade
moderate=large soft drusen, possibly mild impairment of VA.
advanced=uncommon after 5th decade with CNV or geographic atrophy.

PPO (10 decks)

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