MACULAR DISEASE

Dr Russell J Watkins

Macular Disease ARMD SRNVM Angioid streaks Central serous

chorioretinopathy Cystoid macular oedema Macular holes

Epiretinal membranes Choroidal folds Myopic maculopathy Traumatic maculopathy Hereditary macular

dystrophies

Macular Disease Symptoms

VA↓ (especially NV) Central scotoma ↑Hypermetropia Metamorphopsia Micropsia Macropsia Red-green colour defects

Macular Disease Assessment

DVA & NVA Colour vision Photostress recovery test Amsler chart Maddox rod Visual fields Fundus evaluation - slit lamp BIO Other electrophysiological & psychophysical

tests Ophthalmologist will perform FFA

ARMD Leading cause of blindness in western world ~30% of all blind registrations in the UK Bilateral though asymmetrical Features

Drusen RPE tears

Dry ARMD Exudative ARMD

Drusen White-yellow lesions at posterior pole Occur frequently in over-60s Hyaline material deposited in Bruch’s membrane Probably due to ↓RPE phagocytosis 2 morphologies

Large & fluffy (soft) Small & discrete (hard)

Drusen Pathology

Atrophy of RPE & photoreceptor outer segments Thickening & hydrophobicity of Bruch’s membrane Macrophage invasion of drusen; vascularisation

Significance May reflect kidney disease in under-50s In ARMD, with hyperpigmented large confluent soft

drusen, high risk of progression to SRNVM (9% pa)

RPE Tears RPE tears associated with occult SRNVM RPE tears can → acute severe visual loss - some

may retain good VA in spite of subfoveal defect Round, asymmetrical elevations of RPE

associated with RPE tears ±intraretinal haemorrhage, exudate, serous elevation of the retina

High risk of second eye visual loss (37% in first year) as a result of RPE tear or SRNVM

Dry ARMD Choroidal sclerosis Large areas of well-circumscribed RPE atrophy,

neurosensory retinal atrophy & choriocapillaris atrophy

Prominence of choroidal vessels

Exudative ARMD RPE detachment (PED)

Serous, demarcated yellow-orange, dome-shaped elevation of RPE

Confluent drusen can produce RPE elevation Some have occult SRNVM

• Notching of PED• Uneven elevation of PED• Radial chorioretinal folds surrounding PED• Intraretinal exudate• Intraretinal & subretinal blood• Serous retinal elevation

Exudative ARMD SRNVM

Metamorphopsia ±acute visual loss Round oval green-grey lesion PED - serous, haemorrhagic or solid (drusen) Serous retinal elevation Intra- & subretinal haemorrhage, occasional

intravitreal Intraretinal exudate Later disciform scarring

Disciform Degeneration Haemorrhage from SRNVM → circumscribed

scarring at macula SRNVM proliferate due to abnormalities of

Choriocapillaris Bruch’s membrane RPE Outer retinal layers

SRNVM - it’s not just ARMD Congenital or hereditary

Rubella retinopathy Best’s disease Cone dystrophy Retinitis pigmentosa

Vascular Coat’s disease Central or branch retinal vein occlusion

SRNVM Inflammatory

POHS Birdshot chorioretinopathy AMPPE Serpiginous choroidopathy VKH Chronic uveitis Toxoplasmosis Toxocariasis

SRNVM Traumatic

Choroidal rupture Photocoagulation RD surgery

Neoplastic Choroidal naevus Choroidal melanoma Choroidal haemagioma Choroidal metastases

SRNVM Degenerative

ARMD Myopia (Fuch’s spot) Angioid streaks Serpiginous choroidopathy Optic nerve drusen

Management of ARMD Optometrist’s role

Optimal correction and LVAs BD8 SRNVM may be amenable to macular laser

photocoagulation Extrafoveal membrane if >200µm from fovea Subfoveal membrane, after FFA assessment,

well-defined borders <3.5 disc areas in size Treatment benefits only apparent some years

after treatment

Management of ARMD New treatments

Thalidomide - now proven to be of no benefit Radiotherapy - ditto Visudyne (verteporfin) - exudative ARMD only

• IV injection of verteporfin (selectively accumulates in the new vessels)

• Activation by non-thermal laser stimulation (→ free radicals) results in cessation of growth of new vessels

• Known as “Photodynamic Therapy”• With Rx, 33% lose vision; without Rx, 66% lose

vision

Angioid Streaks Defects in collagen & elastin of Bruch’s

membrane → 2° changes in RPE & choriocapillaris Dark, irregular lines radiating from ONH which

end abruptly posterior to the equator May interlink around ONH May cause SRNVM which may bleed Usually bilateral 50% related to systemic disorders of

connective tissue

Angioid Streaks Associations

Pseudoxanthoma elasticum Ehlers-Danlos syndrome Marfan’s syndrome Paget’s disease Sickle cell anaemia & thalassaemia Lead poisoning Acromegaly

Central Serous Chorioretinopathy

Unilateral; M>F (?pregnancy); 20-45yrs Often “obsessive personality” Myopia>hypermetropia Pathogenesis

Breakdown of BRB Fluid accumulates in subretinal space Sometimes associated with RPE detachment

Symptoms Blurred central vision Metamorphopsia & micropsia

Central Serous Chorioretinopathy

Signs VA 6/6-6/36; often improves with a +1D lens Positive central or paracentral scotoma Red desaturation Small serous sensory RD of macula Sometimes associated with an optic pit

FFA Characteristic “smokestack” or “inkblot”

appearance in late venous phase

Central Serous Chorioretinopathy

Prognosis 90% spontaneously resolve 40% recur

Treatment Usually conservative Occasionally photocoagulation indicated

• Residual visual defecit after recurrence• Duration >6m• Only eye

Cystoid Macular Oedema Henle’s layer is unsupported around macula

allowing extracellular fluid to accumulate Source of leak is macular capillaries Forms “flower-petal” arrangement as a result of

radiation of cone fibres May develop intraretinal cysts

Causes of CMO Retina

Diabetic & hypertensive retinopathy CRVO/BRVO Macroaneurysms & telangiectasia Tumours Retinitis pigmentosa Retinitis & vasculitis Irvine-Gass syndrome

Causes of CMO Vitreous

Preretinal membrane formation Vitritis

Choroid Tumours esp. haemangioma Subretinal neovascularisation Longstanding uveitis

Macular Hole Features

Full thickness retinal hole Cuff of surrounding subretinal fluid Deposits at base

Causes Idiopathic (focal contraction of posterior

hyaloid face) Myopia Trauma

May respond to vitrectomy & gas tamponade

Epiretinal Membranes Contraction produces macular traction Early (cellophaning)

Often no visual symptoms Translucent sheen Retinal striae Slight retinal traction

Late (macular pucker) Metamorphopsia Opaque membrane visible Macular oedema

Epiretinal Membranes Causes

Idiopathic RD surgery Photocoagulation (esp. excessive PRP) Cryotherapy CRVO/BRVO Diabetic retinopathy Trauma Longstanding chorioretinitis

May be amenable to membrane peeling but full visual recovery unlikely

Choroidal Folds Causes

Hypermetropia

Ocular disease• Scleral buckling, scleritis, choroidal masses,

ocular hypotony, ocular trauma, papilloedema

Orbital disease• TED, tumours, cellulitis

Myopic Degeneration In very large eyes, retina & choriocapillaris are

thinned Myopic macular degeneration results Risk of complications correlated with axial length

Myopic Degeneration Early findings

PPA, thinned macular RPE, tilting of the optic disc

Late findings Lacquer cracks (breaks in Bruch’s membrane),

posterior staphyloma, macular haemorrhages, SRNVM & macular atrophy

Forrester-Fuch’s spots are localised RPE proliferations

Peripheral & macular holes predispose to RD

Traumatic Maculopathy Commotio retinae

Contre-coup injury Berlin’s oedema if lesion close to macula Opacification of the retina - NOT oedema (in spite of

name) - rather it is disruption of the photoreceptors Good prognosis if RPE intact

Choroidal rupture Occurs if sufficient force to expand eye Choriocapillaris, Bruch’s membrane & RPE split Defect usually concentric with optic disc & accompanied

by subretinal haemorrhage

Traumatic Maculopathy Purtscher’s retinopathy

Seen after crush injuries esp. to chest Caused by activation of coagulation cascade Cotton wool spots & superficial retinal

haemorrhages are evident Guarded prognosis (OA associated)

Solar retinopathy Phototoxicity NOT thermal injury (free radical

release) Photoreceptor membrane is damaged

Hereditary Macular Dystrophies Stargardt’s disease Fundus albipunctatus Best’s vitelliform dystrophy Pattern dystrophies Cone dystrophy