Retina Dystrophy - Investigation

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Retinal Dystrophy Investigation and Management : 

Retinal Dystrophy Investigation and Management Dr Gyanendra Lamichhane Dr.R.N.Byanju Dr.I.Kansakar Lumbini Eye Institute ,Bhairahawa, Nepal

Investigation : 

Investigation Refraction Visual field testing Dark adaptation testing Color vision tests Electroretinography(ERG) Electro-oculography(EOG) FFA

Retinitis pigmentosa : 

Retinitis pigmentosa Visual field testing Midperipheral scotoma develop early in the course of RP Ring scotoma Small central tunnel visual acuity Most RP become legally blind  field constriction

Dark adaptation testing : 

Dark adaptation testing Goldmann-Weekers dark adaptometer Delayed cone-rod break Elevated final threshold

Electroretinography : 

Electroretinography Quite sensitive to photoreceptor impairment Diagnosis in early stage of RP Rate of progression Differential-Diagnosis Reduction rod and cone signal (rod dominate) a and b-wave are reduced b-wave prolong time & diminish amplitude

Management & treatment : 

Management & treatment Current tt not highly effective Slow disease progression & extent the vision Counseling to Pt to understand disease & its genetic Follow-up: 1-2 years Glasses Cataract surgery

Contd... : 

Contd... Vitamin A (15,000IU)daily can slow progression RP by about 2% per year Acetazolamide:for CME 250mg- daily,increased to 500mg if no effective Docosahexaenoic acid Neurotrophic factors

contd : 

contd Disproved treatment strategy placental implantation along sclera ENCAD(daily periocular & intramuscular injections of mushroom RNA extract) treatment in Russia vasodilator drugs and Cuban treatment, which includes vasodilator, hyperbaric oxygen and surgical insertion periorbital fat into the subchoroidal space

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Cone-rod dystrophy : 

Cone-rod dystrophy Reduced color vision V/F:initially full (V4e target),restricted with I4e target ERG:cone ampl reduced (30Hz flicker & photopic single flash) Dark adaptation is normal Good prognosis for vision into later age

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Macular & RPE dystrophy : 

Macular & RPE dystrophy Stargardt disease Investigation FFA: dark or silent choroid ERG:N in early stage EOG:reduced Delayed dark adaptation

Cont... : 

Cont... VA ranges between 20/50 and 20/200 Most pt retain VA between 20/70-20/100 Low vision aids No known treatment

Best’s disease & viteliform dystrophy : 

Best’s disease & viteliform dystrophy EOG:Loss of light response, light/dark ratio less than 1.5,and near 1.1 ERG:reduced c wave , or normal VA usually is good when the yolk remain intact,and vision drop  scarring VA decrease to 6/60 range No treatment

Diffuse drusen : 

Diffuse drusen FFA:well defined hyperfluorescent ERG & EOG are normal VF:central scotoma Dark adaptation is normal No known effective treatment Laser treatment (CNV) Central vision usually is good

Pattern dystrophy : 

Pattern dystrophy ERG is normal EOG is subnormal FFA Visual acuity usually is good

Cone dystrophy : 

Cone dystrophy Color vision :dyschromatopsia Full-field ERG:diminution photopic b-wave & decrease ampl of 30Hz flicker dark-adapted rod response:normal Focal macular ERG:low ampl, abnormal foveal:parafoveal ratio EOG:abnormal V/F:central scotoma

Management of macular degeneration : 

Management of macular degeneration Low vision aids magnifiers & high add glasses for near enlarging reading material vocational counseling hand-held pocket telescope wear sunglasses F/U genetic counseling

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. Thank you

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