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