Presented by Yasmine Sameh el Sharnouby Ophthalmology Specialist

RetinochoridopathiesThey are collectionof disorders of unknown cause with characteristic clinicalpresentations involving inflammation of choroid, choriocapillaris, RPE and sensory retina Syn white spot syndromes, inflammatory multifocal , chorioretinopathies , primary idiopathic inflammatory choriocapillaropathies.

A-acute posterior multifocal placoid pigment epitheliopathy : (APMPPE) 2nd -3rd decade male = female bilateral (fellow eye is affected several days-few weeks after first eye affected) Presentation: ?? prodrome of viral illness , associated with HLA B7,HLA DR2 mild myelomeningeal encephalitis sudden painless loss of vision, central and paracentral scotoma and photopsia

Signs: Minimal ant uveitis, Mild vitritis multiple, cream cloured plaque like lesions, homogenous, below retina, 1 disc diameter , 2-6 weeks it fades away leaving pigment with geographic alteration Diagnosis, ,FA shows early hypoflourescence, late hyperflourescence due to staining ,

y ICG is superior to FA in demonstrating the non-

perfusion of the choriocapillarisy Rx none , CST has been suggested with no evidence of

speeding up recovery , VA may regained to 20/40 in cases with macular involvement , severe central visual loss y Good prognosis

Acute retinal pigment epithelitis (ARPE)2nd 4th decade, unilateral vision loss, absence of ocular inflammation, , 2-6 dots of honey comb pattern of hypopigmented surrounded by hyperpigmented lesions NO Rx excellent prognosis

Birdshot retinochoroidoretinopathy: Chronic Recurrent Idiopathic Bilateral 3rd -6th decade females, 95% are positive to HLA-A29

Presentation: Impaired central vision nactylopia, decrease colour vision peripheral and central photopsias, Floaters

Signs: Moderate vitritis Retinal vaculitis Multifocal depigmented cream coloured patches, scattered allover the posterior pole Old inactive lesions consist of well delineated atrophic spots (with no hyperpigmentation) disc oedema, CME (maybe present)

Diagnosis:FA: early hypoflourescence and late mild hyperflourescence disc staining due to leakage retinal vascular staining, CME(maybe present)

Prognosis and treatment Chronic diseae with remissions and relapses Respond poorly to CST and NSAIDs and immunosuppresives, Periocular CST may help in Rx of CME and vitrous inflammatory cells dose 2-5 mg/kg/day (has been found to stabilize development of new retinal lesions) Some patients may complicate by optic atrophy and cystoid macular degeneration

Multiple evanescent white dot syndrome: ( MEWDS) Idiopathic Unilateral if bilateral affection is asymmetrical, Self limiting Females 80% , 2nd-5th decade may preceeded by flu like illness

Presentation : sudden onset Decreased vision or paracentral scotoma Maybe photopsia mainly in the temporal field

Signs: Mild afferent pupillary defect Mild vitritis multiple small Ill defined, outer retinal ,inner choroidal white dots, in the posterior pole, macula is granular in the acute phase Optic disc odema and enlarged blind spot maybe present ERG, EOG abnormal,

y FA:

Punctate hyperfluorescence which may form a wreath like pattern

Early and late hyperfluorescence of the white dots Diffuse (patchy) late staining at the level of RPE

ICG: Multiple hypofluorescence more than that appear clinically or on FA

Course and prognosis: Resolves within 2-6 weeks, requires no Rx , excellent prognosis Blind spot enlargement may take several months

NB: A- MEWDS has been reported wit acute macular neuroretinopathy(AMN) which is a rare disorder with a paracentral scotoma and a reddish brown wedge shaped lesion in the macula B- also has been reported with syndrome of prolonged enlargement of blind spot. In which pt complain of scotoma that is noted on Visual field testing treatment occurs within several weeks C- also has been reported with acute zonal occult outer retinopathy (AZOOR): Which is loss of retinal function in one or several areas , with clinical examination it shows large scotmata, photopsias,mild vitritis later retinal degeneration and RPE pigmentary changes.

Punctuate inner choroiditis(PIC) idiopathic inflammation to the choroid , young females 3rd decade , Myopic Bilateral

Presentation: Bilateral acute diminution of central vision Maybe associated with photopsias Signs: NO AC or vitreous inflammation Lesion are small, multiple, fuzzy borders in the posterior pole,at the level of inner choroid FA: block fluorescence early with late staining,

Complication: Choroidal neovascular membrane serous retinal detachment Rx CNV: CNV extrafoveal : focal laser photocoagulation Subfoveal : removal by surgery or photodynamic therapy Serous RD CST systemic or periocular RD:

Serpiginous choroidopathy: Chronic Recurrent Bilateral 4th -6th decade Male =female ??HLA_B7

Presentation: Unilateral blurring of central vision Metamorphopsia due to macular involvement Fellow eye is usually affected after a short period of time Signs: Mid vitrits and mild anterior uveitis Lesions are Grey white to yellow at the level of RPE and inner choroid They first appear around the disc then spread in serpentine manner towards macula and peripheral fundus , inactive lesions become atrophic Disease takes remissions and relapses

FA: Active lesions show early hypoflourescence due to non perfusion of the choriocapillaries then hyperflourescence due to staining ICG: active lesions will show marked hypoflourescence throughout all phases

Treatment : y No definitive treatment y Suggestive triple therapy : to prolong remission y (Steroids+ azathioprine+cyclosporine) y Subtenon steroids for macular involvement y Laser photocoagulation at the borders of the lesion has been unsuccesful in stopping the progression

y Multifocal choroiditis with panuveitis:

Bilateral Chronic Recurrent Frequently assymetric 3rd-4th decades Myopic females

Presentation:Blurred central vision Maybe associated with photopsia and floaters 50% has anterior uveitis and vitritis Bilateral multiple, rounded or oval yellowish lesions (maybe arranged in clumps or linear streaks ) Located at the level of RPE and choriocapillaries *enlarged blind spot, disc oedema and CME may be present In active lesions will have punched out margins and pigmented borders resembling POHS

FA Active lesions :Early hypofluorescence late hyperfluorescence due to staining Old lesions show RPE window defects ICG: Hypofluorescence of old and active lesions ERG: May remain normal until advanced retinal atrophy

Treatment : Systemic and periocular steroids effective when administered early Many pts may require immunosuppression CNV : laser and PDT

Progressive subretinal fibrosis and uveitis syndrome: chronic Bilateral Females Myopic Presentation : Gradual unilateral blurred vision ,eventually other eye becomes affected

y Signs:

Mild anterior uveitis and vitritis Yellow indistinct subretinal lesions that coalesce together in a dirty yellow mounds at posterior pole Normal retinal and choroidal filling ,early mottled hyperflourescence and RPE window defect with late hyperflourescence of the edges of the lesion Treatment : poor response to therapy . Little success after use of CST and immunosuppressive drugs

age APMPEE ARPE birdsot MEWDS PIC serpiginous MCP SFU 2nd-3rd 2nd-4th 3rd-6th 2nd-5th 3rd 4th-6th 3rd-4th 2nd-4th

Sex = = F>M F>M F = F>M F

laterality Bilateral Unilateral Bilateral Unilateral Bilateral Bilateral Bilateral Bilateral

lesionAPMPEE multiple, cream cloured plaque like lesions, homogenous, below retina, Honey comb pattern

Flourecin angioEarly: hypo Late hyper

RxNONE, steroids

ARPE

Hypo surrounded by hyper

none

birdsot

Multifocal depigmented cream scattered allover Old inactive atrophic spots no hyperpigmentation) White dot,macula granular,wreath small, multiple, fuzzy borders in the posterior pole Serpentine manner starting from the disc multiple, rounded or oval yellowish lesions Located at the level

Early hypo,late hyper Disc staining, CME

CST

MEWDS

wreath

NONE

PIC

block fluorescence early with late staining

Rx of CNV &RD

serpiginous

Triple therapy

MCP

Early hypo then hyper

?CST,immunosuppressive

Thank you