Macular pathology

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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 unilateral 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 metamorphopsia 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 HTN smoking arteriosclerosis 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? rod-cone 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 hypogonadism obesity paraplegia 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 bilateral symmetrical 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 non-progressive 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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