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IDIOPATHIC MULTIFOCAL WHITE DOT SYNDROMES

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

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IDIOPATHIC MULTIFOCAL WHITE DOT SYNDROMES

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

  2. 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

  3. 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

  4. 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

  5. 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

  6. 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

  7. 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

  8. 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

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