Macular pathology

Question Answer
Idiopathic central serous chorioretinopathy: signs and symptoms pincushion metamorphopsia
decreased color vision
relative central scotoma
no pain or inflammation
hyperopic shift
RPE mottling
yellow-white subretinal deposits
Idiopathic central serous chorioretinopathy: etiology not edema, just a leak
accumulation of fluid under macula results in separation of photoreceptor outer segments from RPE
results in loss of foveal reflex
Idiopathic central serous chorioretinopathy: demographics 20-30 year old
usually white males
type A personality
higher incidence with oral steroid use
Idiopathic central serous chorioretinopathy: NaFl angiography results classic smokestack
NaFl moves upward due to convection
Idiopathic central serous chorioretinopathy: Treatment resolves spontaneously in 3-4 months
monitor every 6-8 weeks until resolution
Macular hole: signs and symptoms abrupt loss of central VA
central scotoma
macula appears redder than normal with surrounding ring of detached retinal tissue
hyperfluorescent window defect with NaFl angiography
Macular hole: etiology and mechanism vitreous shrinks with age
this creates foveal traction
traction pulls macula and creates a hole
Macular hole: stages Stage 1 - Tractional foveal detachment
Stage 2 - Partial thickness hole
Stage 3 - Full thickness hole
Stage 4 - Full thickness hole with vitreous separation
Macular hole: treatment vitrectomy to relieve traction
Epiretinal membrane: signs and symptoms can be asymptomatic
may have metamorphopsia
may have severe visual impairment
uneven, reflective surface
acuity is too good to be a macular hole
Epiretinal membrane a.k.a. cellophane retinopathy
surface wrinkling retinopathy
AMD: risk factors age (>60 years)
fair skin
light iris
exposure to sunlight
family history
AMD: Characteristics of Dry AMD Dry AMD:
80% of cases are dry
drusen (macula, Bruch's, or RPE)
etiology: photoreceptor atrophy
show-through/window defect in NaFl angiography
Wet AMD: etiology VEGF released
abnormal vessel growth under retina
leakage, bleeding, scarring
serous retinal detachment
hemorrhagic retinal detachment
soft drusen give new vessels a place to grow
Wet AMD: signs confluent, large drusen
metamorphopsia from leaky vessels under retina
Signet's ring sign (grey/green, neo)
What is the most common retinal degeneration? Retinitis pigmentosa
Most common type of photoreceptor dystrophies
rod-cone or rods only or cones only?
Retinitis pigmentosa: mode of inheritance: autosomal dominant (22%)
autosomal recessive (16%)
X-linked (9%)
remainder are "simplex" (unknown inheritance)
Retinitis pigmentosa: signs and symptoms night blindness
tunnel vision
ring scotoma
bilateral, slowly progressive vision loss
bony spicules
arteriolar attenuation
waxy pallor of ONH
Usher's Syndrome defined: Retinitis pigmentosa with associated hearing loss
Usher's Syndrome: mode of inheritance autosomal recessive
Bardet-Biedl Syndrome: signs polydactyly (not in Lawrence-Moon)
severe VA/VF loss by middle age
mental retardation
retinitis pigmentosa
salt and pepper fundus
Congenital Stationary Night Blindness: mode of inheritance X-linked recessive
Congenital Stationary Night Blindness: signs normal looking fundus
highly myopic
ERG is negative (like CRAO and X-linked retinoschisis)
Stargardt Macular Dystrophy: signs beaten bronze appearance
pisciform lesions (looks like scales) around macula at level of RPE
central vision loss (BVA 20/40 - 20/200)
teen or young adult
Stargardt Macular Dystrophy: diagnostic tests Sita Standard 10-2
normal color vision until later stage
NaFl Angiography shows dark choroid at posterior pole
ERG normal or minimal loss
Stargardt Macular Dystrophy: mode of inheritance autosomal recesive
Most common inherited macular dystrophy: Stargardt's
Best's Disease a.k.a. vitelliform macular dystrophy
Best's Disease vs. Adult Vitelliform Macular Dystrophy: smaller "egg yolks"
EOG is normal!
Familial Drusen vs. Age-related drusen Familial drusen stretches to macula and nasal to optic disc
Which macular dystrophy affects Mueller cells? Dominant Cystoid Macular Edema
Sorsby's Macular Dystrophy: mechanism lipid deposits between Bruch's and RPE
starts as fine drusen or confluent plaque
progresses to exudative maculopathy with geographic atrophy and scars
North Carolina Macular Dystrophy defined macular coloboma
Best's Disease: signs large, yellow, yolk-like, bilateral lesions at macula
appears during childhood
EOG: Arden ratio lower than 1.5 (as opposed to normal ~1.8)
EOG differentiates Best's from Stargardt

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