IDIOPATHIC MULTIFOCAL WHITE DOT SYNDROMES

Dr George Papanikolaou

SHO in Ophthalmology,

Dept of Ophthalmology,

Singleton Hospital,

Swansea

MULTIPLE EVANESCENT WHITE DOT SYNDROMEMEWDS

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

1. Creamy white placoid lesions at PP (RPE level)

2. 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

1. Active: Cream coloured,hazy borders, increasing brightness/ optic disccentrifugaly/ May start ANYWHERE/ Skip- satellite lesions/ Border inactive scars (DD APMPPE)/ Vascular predilection

2. Anterior uveitis/ vitritis

3. 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

CNVMNo systemic associationsRx: steroids, azathioprine, cyclosporine/ laser

BIRDSHOT CHORIORETINOPATHY/ BSR

Bilateral, women, middle age, guarded prognosisRemissions- exacerbations

HLA-A29 ( HIGHEST RR of any disease~200x)A29.2/ S-antigen/ CD8+/ Choroid + ret. vessels

Floaters, VA- nyctalopia- photopsia- colour vision

1. Creamy –yellow ovoid deep spots/ centrifugal/ nasal-inferior retina

2. Vitritis/ vasculitis/ narrowing of vessels3. CMO/ disc oedema4. Chronic: well defined atrophy/ NO PIGMENT

FFA: early: normal/ ‘Quenching’ of vessels

late: leakage (intraretinal, disc)ICG: hypo-, fuzzy choroidal vessels: ActivityRx: steroids/ immunosuppressants

PUNCTATE INNER CHOROIDOPATHYPIC

Bilateral, young myopic women, guarded prognosis

Scotomata/ photopsia/ Blurred vision

1. Small spots (same age),PP/ same as MCP

2. SRD

3. 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

1. Multiple, deep, yellow-grey lesions

2. Vitritis (all)/ ant. uveitis 50% (DD POHS)

3. Chronic: atrophy+pigmentation

FFA: early: hypo- late: fuzzy leaking

ICG: hypofl. spots- peripapillary

Recurrences (common)

CMO, Subretinal Fibrosis, CNVMRx: Steroids/ Imunosuppressants/ Laser

PRESUMED OCULAR HISTOPLASMOSIS SYNDROMEPOHS

H. capsulatum/ Missisipi- Ohio30-40yGood prognosis/ 2-10% ocular lesions in endemic areas 1/1000 maculopathy HLA DR2, HLA-B7Maculopathy

1. Histo spots (PP, midperiphery)/ linear streaks-equator

2. PPA

3. CNVM (v if macular spots)

4. Clear Vitreous, No PED

Positive skin testing/ serology/ CXR

Laser/ Surgical