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IDIOPATHIC MULTIFOCAL WHITE DOT SYNDROMES
Dr George Papanikolaou SHO in Ophthalmology, Dept of Ophthalmology, Singleton Hospital, Swansea
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MULTIPLE EVANESCENT WHITE DOT SYNDROME
MEWDS Unilateral, Young myopic women, idiopathic, Excellent prognosis Acute unilateral VA, photopsia, scotomata/ Viral prodrome Numerous migratory white dots (faint, PP, mid-periphery) Foveal granularity- Pathognomonic- PERSISTENT Mild papillitis/ vitritis/ disc oedema/ vasculitis FFA: early punctate hyperfluorescence/ late: staining ICG: typical multiple hypofluorescent spots throughout fundus Hypofluorescence around optic nerve VF: BS, disproportionate to clinical findings/ PERSISTENT Recovery: 3-10/52, , no scarring
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ACUTE POSTERIOR MULTIFOCAL PLACOID PIGMENT EPITHELIOPATHY/ APMPPE
Bilateral, young healthy adults, good prognosis HLA-B7 ,DR2 / Influenza-like prodrome 50% VA uni- bilateral in a few days Creamy white placoid lesions at PP (RPE level) Mild vitritis/ vessel sheathing/ disc oedema FFA: early: dense hypofluorescence late: staining Inactive: window defect ICG: Hypofluorescence (active+healed) Systemic microvasculopathy (Skin, kidney, CNS) Recovery: within 1/12, depigmentation and clumping
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SERPIGENOUS CHOROIDOPATHY
Bilateral, chronic/ recurring, progressive, 40-60y, Poor prognosis VA uni bilateral (asymmetric), Scotomata, Photopsiae Active: Cream coloured,hazy borders, increasing brightness/ optic disccentrifugaly/ May start ANYWHERE/ Skip- satellite lesions/ Border inactive scars (DD APMPPE)/ Vascular predilection Anterior uveitis/ vitritis Inactive: choroidal atrophy (large chor. vessels) FFA: early: central hypo+ hyper rim late: hyper leaks into the lesion (spotty staining) Healed: visible large choroidal vessels CNVM No systemic associations Rx: steroids, azathioprine, cyclosporine/ laser
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BIRDSHOT CHORIORETINOPATHY/ BSR
Bilateral, women, middle age, guarded prognosis Remissions- exacerbations HLA-A29 ( HIGHEST RR of any disease~200x) A29.2/ S-antigen/ CD8+/ Choroid + ret. vessels Floaters, VA- nyctalopia- photopsia- colour vision Creamy –yellow ovoid deep spots/ centrifugal/ nasal-inferior retina Vitritis/ vasculitis/ narrowing of vessels CMO/ disc oedema Chronic: well defined atrophy/ NO PIGMENT FFA: early: normal/ ‘Quenching’ of vessels late: leakage (intraretinal, disc) ICG: hypo-, fuzzy choroidal vessels: Activity Rx: steroids/ immunosuppressants
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PUNCTATE INNER CHOROIDOPATHY
PIC Bilateral, young myopic women, guarded prognosis Scotomata/ photopsia/ Blurred vision Small spots (same age),PP/ same as MCP SRD NO uveitis/vitritis DD from POHS Weeks to recover Residual scars (with pigment) CNVM
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MULTIFOCAL CHOROIDITIS WITH PANUVEITIS
MCP Uni bi, myopic, women, middle age, fair prognosis Subacute blurring/ photopsiea/ scotomata/ floaters/ photophobia/ pain Multiple, deep, yellow-grey lesions Vitritis (all)/ ant. uveitis 50% (DD POHS) Chronic: atrophy+pigmentation FFA: early: hypo- late: fuzzy leaking ICG: hypofl. spots- peripapillary Recurrences (common) CMO, Subretinal Fibrosis, CNVM Rx: Steroids/ Imunosuppressants/ Laser
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PRESUMED OCULAR HISTOPLASMOSIS SYNDROME
POHS H. capsulatum/ Missisipi- Ohio 30-40y Good prognosis/ 2-10% ocular lesions in endemic areas 1/1000 maculopathy HLA DR2, HLA-B7Maculopathy Histo spots (PP, midperiphery)/ linear streaks-equator PPA CNVM (v if macular spots) Clear Vitreous, No PED Positive skin testing/ serology/ CXR Laser/ Surgical
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