15 - Retina/Choroid3 Flashcards

1
Q

Histoplasmosis

  • epidemiology
  • pathophys
A

Ohio-Mississippi river valley, chicken poo (e.g. farmer), rare in AAs

Infection by Histoplasma capsulatum, FUNGUS that grows in soil, material contaminated with bird/bat droppings

  • recurrences (30%) through pre-existing HISTO SPOTS
  • if spots are present in disc/macular area, chance of symptomatic recurrence is ~20% in 3 years
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2
Q

Histoplasmosis

-signs/symp

A

Signs: BILATERAL CHOROIDITIS

  • clinical TRIAD: PERIPAPILLARY ATROPHY, MULTIFOCAL PERIPHERAL LESIONS, MACULOPATHY (incl cnvm (late stage, 20-45))
  • vitreous is always clear (vs toxo headlights in fog)

Symp: asymptomatic unless maculopathy develops (metamorphopsia)

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3
Q

Toxo vs histo

  • laterality
  • lesion(s)
  • vitreous
  • causitive organism
A

T: one eye, one lesion, cloudy vitreous, parasite

H: bilateral, multiple lesions, clear vitreous, fungus

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4
Q

Pathological/degenerative/high myopia

  • epidemiology
  • pathophys
  • symp
A

Genetic predisposition, females, young adulthood, 2% pop

RE > -6D spherical equivalent
Axial length >/= 26.5mm

Asymptomatic; decr vision, metamorphopsia

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5
Q
Pathological/degenerative/high myopia
-signs
—macular/posterior pole (5)
—peripheral (4)
—non-retinal (3)
A

M/PP:

  • posterior staphyloma (hallmark) = post bulging of WEAK SCLERA
  • oblique optic nerve insertion
  • FUCHS SPOTS = focal hyperpig secondary to scarring/cnvm
  • LACQUER CRACKS
  • macular holes/ERMs

Peripheral: think DEGENERATIONS

  • LATTICE degen = thinned retina
  • SNAIL-TRACK degen (recall PPMD (cornea) is train track lesions)
  • PAVINGSTONE degen
  • retinal breaks/detachments

Non-ret:

  • premature cataracts (PSC > NS)
  • extensive syneresis
  • PVD
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6
Q

Pathological/degenerative/high myopia

-lacquer cracks

A

5%, esp young males
Fine, YELLOW, irregular spokes from the macula
Represent large BREAKS IN BRUCHS

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7
Q

Pathological/degenerative/high myopia

-pavingstone degeneration

A

aka Cobblestone
Discrete, CIRCULAR, areas of YELLOW-WHITE chorioretinal atrophy in periphery
No clincal importance

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8
Q

Epiretinal membrane

  • epidemiology
  • pathophys
A

aka macular pucker (advanced)
Females, incr with age

Often idiopathic, may be from PVDs (#1), retinal breaks, cataracts, other intraocular surgeries/trauma
Result from GLIAL CELL* PROLIFERATION on the ILM
*from posterior hyaloid or inside the retina

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9
Q

Epiretinal membrane

-signs/symp

A

Signs:

  • mild: fine, glistening membrane (cellophane maculopathy)
  • advanced: thick, gray-white membranes with assoc retinal folds (macular pucker)

Symp: asymptomatic; decr vision and/or metamorphopsia

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10
Q

Macular hole

  • epidemiology
  • pathophys
A

Aging (50-70), women

From POSTERIOR VITREOUS TRACTION on macula
IDIOPATHIC/senile (#1), trauma, surgery, CME, inflammation
BILATERAL ONSET 25-30%
-risk of development in fellow eye varies (none if PVD occurred)

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11
Q

Macular hole

-signs/staging/symp

A

Signs: ROUND, RED, WELL-DELINEATED SPOT

  • stage 1: YELLOW spot/ring at fovea, impending hole
  • stage 2: round, small, full-thickness hole with PSEUDO-OPERCULUM
  • stage 3: large, full-thickness hole with OPERCULUM, (+)WATZKE-A*
  • stage 4: stage 3 + PVD
  • positve watzke-allen = complete break in middle of thin line

Symp: decr vision and/or metamorphopsia
-full-thickness = 20/200 or worse

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12
Q

Macular photostress test

A

Determine BCVA prior to starting
Hold bright light @2cm from eye, look at for 10sec
Measure time it takes for pt to read one line less than his/her BCVA
Normal: <60sec

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13
Q

Choroidal folds

  • what/app
  • common causes
A

Waves within choroid/bruchs/RPE secondary to mechanical stress on/within choroid
-alternating light/dark striations within fundus

Tumor, post scleritis, choroidal detach/inflamm, orbital pseudotumor, thyroid-related ophth, orbital myositis, hypotony, intracranial HTN

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14
Q

Albinism

  • pathophys
  • signs/symp
A

UNDERDEVELOPED IRIS, CORNEA, ONH, FOVEA
Group of disorders, mutation in genes responsible for MELANIN production

Signs: hypopigmentation, optic nerve/FOVEAL HYPOPLASIA (poor vision), MICROCORNEA, nystagmus, strabismus, mis-routing of temporal nerve fibers thru chiasm (most fibers cross), HIGH HYPEROPIA + ASTIG

Symp: photophobia, reduced VA

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15
Q

Retinitis pigmentosa

  • epidemiology
  • pathophys
A

Most common RETINAL dystrophy
Sporadic or AD (usually inherited)
Assoc systemic condn = Usher’s (AR, hearing loss)
Avg age dx 9-19yo

Group of condns, progressive loss of PR/RPE function
Rods damaged&raquo_space;> cones
Tremendous variability in presentation

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16
Q

Retinitis pigmentosa

-signs/symp

A

Signs:

  • TRIAD: bone-spicule pigmentation, arteriolar attenuation, waxy optic disc pallor
  • PSC CATARACTS, optic DISC DRUSEN (VF loss), MACULAR CHANGES (atrophy, CME, ERM), keratoconus, myopia
  • REDUCED SCOTOPIC ERG (early stages)

Symp: NYCTALOPIA (#1), PERIPHERAL VISION LOSS, by age 30 over 75% symptomatic

17
Q

Stargardt’s disease

  • epidemiology
  • pathophys
A

Most common hereditary MACULAR dystrophy
Onset 1st-2nd decade (esp 6-20yo)
AUTO RECESSIVE inheritance, no sex predilection

18
Q

Stargardt’s disease

-pathophys

A

Most often due to mutation in ABCA4 transmembrane protein - responsible for moving all-transAL from disc lumen to cytoplasm
-this leads to toxic accumm of all-transAL in the discs -> degeneration of PRs/RPE

19
Q

Stargardt’s disease

-vs fundus flavimaculatus

A

FF and SD are considered variants of the same disorder

FF: dx is reserved for pts WITHOUT macular dystrophy signs, often presents LATER in life (4th-5th decade vs 6-20yo), commonly ASYMPTOMATIC

20
Q

Stargardt’s disease

-signs/symp

A

Signs:

  • early: bilateral YELLOW FLECKS in PISCIFORM configuration, non-specific RPE MOTTLING of macula, normal ERG
  • late: classic “BEATEN BRONZE” macula (“BULL’S EYE”) and “SALT AND PEPPER” pigmentary changes, abnormal ERG

Symp: rapid vision loss and color vision abnormalities

  • level of vision loss is often OUT OF PROPORTION* with fundus app in early stages; typically 20/200 by third decade, stable/slowly progressive thereafter
  • early SD is the “acanthamoeba of the retina”
21
Q

Choroideremia

  • epidemiology
  • pathophys
A

XL-RECESSIVE, males, 1st decade

Deficiency in rab geranyl-geranyl transferase - enzyme utilized in membrane metabolism -> atrophy of choroid

22
Q

Choroideremia

-signs/symp

A

Signs: progresive, bilateral, diffuse atophy of RPE/choriocapillaris -> exposure of underlying sclera
-macula often spared until late

Symp: night-blindness, peripheral vision loss

  • most have good vision until 50-60yo
  • in females the condn is benign, non-progressive
23
Q

Ddx for night-blindness (3)

A

Gyrate atrophy
Choroideremia
Retinitis pigmentosa

24
Q

Cone dystrophy

  • epidemiology
  • signs/symp
A

Onset 1st-3rd decade, AD inheritance

Signs:

  • early: normal fundus app + low VA, abnormal photopic ERG
  • late: CENTRAL GEOGRAPHIC ACTROPHY, BULL’S EYE MAC, VESSEL ATTENUATION, TEMPORAL DISC PALLOR, FINE NYSTAGMUS, severe deutan-tritan color defect, abnormal photopic ERG, THINNED PR LAYER ON OCT

Symp: SLOWLY PROGRESSIVE DECR CENTRAL VISION, severe photophobia and color vision loss
-poor prognosis: 20/400 by 40yo

25
Q

Ddx for bull’s eye maculopathy (3)

A

Stargardt’s
Progressive cone dystrophy
Hydroxy/chloroquine or Thioridazine toxicity

26
Q

Best’s disease

  • epidemiology
  • pathophys
A

Uncommon, AD inheritance, presents early childhood (5-10yo)

Abnormal accum of lipofuscin in the RPE

27
Q

Best’s disease

  • signs/symp
  • stages
A

Signs: BILATERAL, yellow, round, SUBFOVEAL “egg-yolk” lesion
-will have normal ERG but abnorm EOG, even prior to vision loss/fundus signs

Symp: majority are detected with little/no pt symptoms (75% better than 20/40), eventually complain of decr vision

Stages:

1) pre-vitelliform = abnormal EOG (arden <1.8), normal fundus, fam hx
2) vitelliform = 2 eggs, usually ages 3-15
3) pseudohypopyon = entire lesion absorbed, little/no effect on vision
4) vitelliruptive = breaks up -> “scrambled egg” app, MILD VISION LOSS
5) end-stage* = MOD-SEVERE VISION LOSS due to CNV, hemorrhage, atrophy, and/or macular scarring
* Best’s is best to have until ~50yo

28
Q

Adult foveomacular vitelliform dystrophy

A

Presents in pts 30-50
Signs similar to Best’s
Overall prognosis better - minimal metamorphopsia, mild acuity loss, normal EOG and ERG, slight tritan color defect