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Maculopathies
Macular pathology
| Question | Answer |
|---|---|
| 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 |
| 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 |
| Idiopathic central serous chorioretinopathy: demographics | 20-30 year old<br>usually white males<br>type A personality<br>higher incidence with oral steroid use |
| Idiopathic central serous chorioretinopathy: NaFl angiography results | classic smokestack<br>NaFl moves upward due to convection |
| Idiopathic central serous chorioretinopathy: Treatment | resolves spontaneously in 3-4 months<br>monitor every 6-8 weeks until resolution |
| 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 |
| Macular hole: etiology and mechanism | vitreous shrinks with age<br>this creates foveal traction<br>traction pulls macula and creates a hole |
| 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 |
| Macular hole: treatment | vitrectomy to relieve traction |
| 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 |
| Epiretinal membrane a.k.a. | cellophane retinopathy<br>surface wrinkling retinopathy |
| 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 |
| 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 |
| 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 |
| Wet AMD: signs | confluent, large drusen<br>metamorphopsia from leaky vessels under retina<br>Signet's ring sign (grey/green, neo) |
| What is the most common retinal degeneration? | Retinitis pigmentosa |
| Most common type of photoreceptor dystrophies<br>rod-cone or rods only or cones only? | rod-cone |
| Retinitis pigmentosa: mode of inheritance: | autosomal dominant (22%)<br>autosomal recessive (16%)<br>X-linked (9%)<br>remainder are "simplex" (unknown inheritance) |
| 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 |
| 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)<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 |
| Congenital Stationary Night Blindness: mode of inheritance | X-linked recessive |
| Congenital Stationary Night Blindness: signs | normal looking fundus<br>highly myopic<br>ERG is negative (like CRAO and X-linked retinoschisis) |
| 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 |
| 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 |
| 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"<br><b>EOG is normal!</b> |
| 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<br>starts as fine drusen or confluent plaque<br>progresses to exudative maculopathy with geographic atrophy and scars |
| North Carolina Macular Dystrophy defined | macular coloboma<br>non-progressive<br> |
| 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 |
Created by:
lastscout
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