IDIOPATHIC MULTIFOCAL WHITE DOT SYNDROMES Dr George Papanikolaou SHO in Ophthalmology, Dept of Ophthalmology, Singleton Hospital, Swansea

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

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

SERPIGENOUS CHOROIDOPATHY Bilateral, chronic/ recurring, progressive, 40-60y, Poor prognosis VA uni bilateral (asymmetric), Scotomata, Photopsiae Active: Cream coloured,hazy borders, increasing brightness/ optic disccentrifugaly/ 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

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

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

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

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-B7Maculopathy Histo spots (PP, midperiphery)/ linear streaks-equator PPA CNVM (v if macular spots) Clear Vitreous, No PED Positive skin testing/ serology/ CXR Laser/ Surgical