Retina 6 - Macula diseases Flashcards

1
Q

Retina fact sheet :-

A

• 120million rods
- 5/6th in perifoveal region
- 1/6th in peripheral retina
- Absent fovea
•6.3-6.8million cones
- Mainly in fovea
- some further than 10 degrees from fixation

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2
Q

Layers of retina factsheet:-

A

•RPE is blood retinal barrier
- Prevents blood from seeping into retina
•Bruchs membrane is first layer of choroid
RPE or bruchs membrane not working together can result in pathologies

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3
Q

Age related macular degeneration factsheet:-

A

• Leading cause of blindness in over 50s in western world
•20% of >75 yr olds
•Degenerative disorder of retina, progressive in nature
•Classified into dry and wet(neovascular)

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4
Q

AMD Categories (5 in total)

A

• No apparent ageing changes
- No drusen + No amd pigment abnormalities
•Normal ageing changes
- Only druplets + NO Amd pigment abnormalities
•Early AMD
- Medium drusen (63-135um) + NO Amd Pigment abnormalities
•Intermediate AMD
- Large drusen (>125um) + ANY Amd pigment abnormalities
• Late AMD
- Neovascular AMD and/or Any geographic atrophy

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5
Q

ARMD Risk factors?

A

•Age
•Family History
•Smoking
•Ethnicity (white Caucasians high risk)
•Gender (female>male)
•Complement factor H gene
•Diet
- Macular Carotenoids, spinach, kale, broccoli, oily fish

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6
Q

Armd - Pathophysiology

A

•Drusen - Extracelluar deposits between RPE and Bruchs membrane (immune mediated and metabolic by products from RPE)
•Increased number and size of drusen correlated with pigmantory abnormalities
•RPE stops working properly with eventual loss of RPE
•Breakdown of Blood retinal barrier
•Blood vessels from Bruchs grow into retina - causing wet ARMD

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7
Q

ARMD - Symptoms

A

•Reduced vision (depending on wet/dry can alter)
• Distortion
•Scotoma (in central vision)
• Can present with sudden loss of vision

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8
Q

Assessment

A

• VA
•Refraction
•Cataract test
•Amsler
•Diluted fundal examination
•OCT if available

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9
Q

Treatment of ARMD?

A

•Dry ARMD - None
- Give lifestyle advice - stop smoking, healthy eating, UV protection, vitamins supplements (only if advanced) to prevent progression
- Blind/partial sight registration
•Wet ARMD
- ANTI-vegF injections
- Laser : Used for Px if polypoidal choroidal vasculopathy
- Surgery : If large sub macular haemorrhage (7-10days)

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10
Q

Anti-Vascular endothelium growth factor (Anti-VEGF) and why is it used?

A

•Reduces vascular permeability
•Halt progression of pathological neovascularisation
•Reduce sub retinal fluid (SRF), intraretinal fluid (IRF) and haemorrhage

•Aim of treatment is to stabilise vision
•Injections given to eye on monthly basis

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11
Q

Anti-VEGF - indication in ARMD

A

•NICE
-The best corrected VA between 6/12 and 6/96
- No permanent damage to central fovea
- Lesions size is less than or equal to 12 disc diameters in greatest linear dimension
- There is evidence of recent presumed disease progression
• SMC:
- No material difference

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12
Q

Who to refer with ARMD?

A

• Reduced VA
• Macular haemorrhage with pigemantory changes/drusen
•SRF or IRF with oct (Sub retinal fluid/infra retinal fluid)
•New distortion
•Sudden loss of vision

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13
Q

What do you include in referral of ARMD?

A

• VA
•Refraction
•Lens status (Cataract)
•Symptoms, include duration
•Signs - describe what you see at the macula
•Include colour photos/OCT if possible

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14
Q

Who NOT to refer ARMD?

A

•ASymptomatic patients or VA> 6/9 with drusen, atrophy or pigmentary changes with no evidence of fluid
• Patients already in the system
•Patients with macular scars who have previously been discharged
•Patients who dont want to come

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15
Q

What are the four types of retinal vascular disorders?

A

•Diabetic retinopathy/maculopathy
•Retinal vein occlusion
•Retinal artery occlusion
•Hypertensive retinopathy

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16
Q

What risk factors associated with Diabetic retinopathy?

A

• Commonest cause of blindness in working age population
•Risk factors include :
- Poorly controlled diabetes
- Hypertension
- Obesity

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17
Q

Pathophysiology of Diabetic retinopathy?

A

•Diabetic retinal microangiopathy
- Caused by Pericyte loss
- Basment membrane thickening
-Endothelial proliferation
This results in:-
• Increased vascular permeability
- Causes leakage resulting in OEDEMA (Maculopathy)
- Ischaemia
•Vascular occlusion due to BM thickening/proliferation
- Causes Ischaemia

All of this results in NEOVASCULARISATION

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18
Q

Diabetic retinopathy signs

A

•Micro-aneurysms
•Haemorrhages
•Exudates
•Cotton wool spots
•Venous bleeding
•IRMA
•New vessels

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19
Q

3 classifications of Diabetic retinopathy:

A

•Background DR
- 1 to 5 MA
- Retinal haemorrhages
- Venous loop
- Any exudates
- Any Cotton wool spots
• Pre-proflierative DR (Signs of ischaemia)
- Venous beading
- Blot haemorrhages
- IRMA’s
• Proliferative DR
- Stable pre-retinal fibrosis
- Neovascularisation at disc or elsewhere
- New fibrosis/Vit heam
- New Traction RD

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20
Q

Retinal vein occlusion different types:

A

•Branch retinal vein occlusion
•Central retinal vein occlusion
•Hemi Retinal vein occlusion
- Can present with or without cystoid macular oedema
- Atherosclerotic blockage of retinal vein

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21
Q

Retinal vein occlusion - Risk factors

A

All “Vascularpathic”
• Age
• Hypertension
• Hyperlipidaemia
• Diabetes
• Smoking

Less common : Infection/inflammation
REFER ALL RVO TO GP FOR BLOODS AND BP CHECK

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22
Q

Retinal vein occlusion - Symptoms

A

•Sudden painless loss of vision
•Central distortion
•Asymptomatic (if branch and unilateral)

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23
Q

Retinal vein occlusion - Signs

A

• Haemorrhages
• Cotton wool spots (ischaemia)
• Tortous blood vessels
•Disc swelling
• May have RAPD (if ischaemic)
• Exudates ( if long standing)
• CMO - Central macular oedema

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24
Q

Treatment for vein occlusion?

A

•Control of risk factors, smoking
• Monitor for complications
• Observation
•Anti-vegF injections
•Laser

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25
Q

3 categories of Retinal artery occlusion:

A

• Branch retinal artery occlusion
• Central retinal artery occlusion
• Cilloretinal artery occlusion

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26
Q

Retinal artery occlusion Causes:

A

• Atherosclerosis
• Carotid embolism (cholesterol/calcium/fibrin)
• Giant cell arteritis
•Other Haematological conditions

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27
Q

Retinal artery occlusion Symptoms

A

•Sudden painless loss of vision
•Scotoma/shadow
•Field loss
•Asymptomatic (less common with artery than vein)

28
Q

Retinal artery occlusion : Signs/symptoms

A

Signs:
•RAPD
•Retinal pallor in distribution of blockage (pale)
•”Cherry red spot” (Central retinal artery occlusion)
•Thinning of retinal arteries

Symptoms:
Reduced VA
•Visual field loss

29
Q

Retinal artery occlusion - Treatment

A

•Breathing into a paper bag
• IOP lowering
- Digital masage to “move embolus”
- Diamox or paracentesis (reduces pressure drugs)
•Stroke clinic referral as early sign stroke

30
Q

What is Hypertensive retinopathy?

A

•Microvascular damage from elevated blood pressure
•Primary response of arterioles is vasoconstriction
•Inner blood-retinal barrier is disrupted in localised areas with increased vascular permeability

31
Q

Hypertensive retinopathy grading scale and referral?

A

• Grade 1 - Mild generalised arterial narrowing
• Grade 2 - Focal narrowing, AV nipping, copper wiring
• Grade 3 - Grade 2 + Haemorrhages (dot, blot and flame), exudates, cotton wool spots, “macular star”
• Grade 4 - Optic disc swelling
Patients may complain or headaches/intermittent loss of vision
- Refer to GP for BP check
- Urgent referral to HES if grade 4

32
Q

Central serous Retinopathy - CSR character:

A

• Central serous chorioretinopathy
•idiopathic disease with localised serous retinal detachment
•Falls within pachychoroid disease spectrum
• Typically affects young male, Type A personality

33
Q

Choroidal serous retinopathy - Risk factors

A

•Steroid use
•Pregnancy
•Stress
•Cushings disease (too much steroid)
•Sleep apnoea

Rarely can be associated with optic disc pit

34
Q

Choroidal serous retinopathy - Pathophysiology

A

•Not clearly understood
• Serous leakage from choroidal vessels
• Could be related to hydrostatic pressures

35
Q

Central serous retinopathy - Symptoms

A

•Central blurring of vision
• Distortion
• Scotoma
•Hyperopic shift due to “lifted retina”

36
Q

Central serous retinopathy- Signs

A

•Pale orange raised area
• May have pigment changes
• Sub retinal fluid on OCT

37
Q

Treatment of Central serous retinopathy

A

•Observation
- Usually resolve spontaneously within 6/12
• May have relapsing remitting course - chronic CSR
• Can develop secondary choroidal neovascularisation

•Treatment
- Stop steroids; depends what for (system condition?)
- Eplenarone; diuretic, reduces fluid in body
- Laser: chronic CSR

38
Q

What is Cystoid macular Oedema and it’s associated diseases?

A

•Affects outer plexiform and inner nuclear layers
•Cystic intra-retinal fluid at the macula
•Not stand alone diagnosis
•MANY ASSOCIATIONS
- Inflammatory (eg post op/uveitis)
- Vascular e.g diabetes or RVO
- Choroidal vascular disease
- Toxic e.g drug induced
- Retinal dystrophy e.g retinitis pigmentosa
- Systemic disease e.g chronic renal failure

39
Q

Degenerative Myopia character:

A

•May also be called pathological myopia
•Myopia <6D is physiological
•Myopia >6D is high myopia
•Myopia >6D and continues increasing is called pathological myopia
- Due to increasing axial length
- “The next pandemic”(myopia itself)
In east Asia, affects 80-90% of school leavers

40
Q

Degenerative myopia - risk factors/associations

A

•Excessive near work
•Genetic factors

•Systemic diseases
- Down syndrome
- Prematurity
- Albinism

41
Q

Degenerative myopia - symptoms

A

•Progressive change in refraction
•Reduced vision
•Distortion
•Photopsia

42
Q

Degenerative myopia. - signs

A

•Pale, tessellated fundus
•Tilted disc
•Atrophy
•Laquer cracks (breaks in bruchs membrane)
•Haemorrhage (foster fuchs spot at fovea)
• Lattice degeneration
• Early PVD

43
Q

Degenerative myopia - complications

A

•Increasing axial length
•Secondary choroidal neovascular membrane
•Macular hole
•Retinal tears/detachment
•Retinoschisis

44
Q

Treatment of degenerative myopia

A

•Prophylactic treatment
- Outdoor play: 1hr per day children
- Myopia control contact lenses
- Atropine
•No treatment for degeneration/atrophy
•Anti-VegF injections for secondary CNV

45
Q

When to refer degenerative myopia

A

•Progressive/sudden loss of vision
•New haemorrhage
•Any signs of retinal tear/detachment

46
Q

What are Hereditary dystrophies?

A

•Inherited conditions - many genes now identified
•Disorders affecting RPE-photoreceptor and choriocapilaris complex
- Any component of the complex can lead to disease

•Separated into retinopathies and maculopathies
•Diagnosed clinically with electro diagnostic testing

47
Q

How are Hereditary dystrophies Classified?

A

•Location
- Generalised: Rod cone dystrophy or cone rod dystrophy
- Central : Macula only
•Progression
- Stationary (non progressive/
- Progressive
• Cellular
- Photoreceptors
-RPE
- choroid

48
Q

Retinitis pigmentosa (RP) Character:

A

•Most common inherited retinal disorder
•Incidence is 1 in 3-4,000
•Large group of disorders
•Variable inheritance - Sporadic, dominant, recessive, X-Linked
•Many genes identified
•Severity of disease depends on inheritance and gene
•Progressive condition - initially involves rods but eventually cones leading to total blindness

49
Q

Retinitis pigmentosa - Systemic associations

A

• ~ 25% associated with systemic disease
•Ushers syndrome
•Bardet biedl syndrome
•Kearns sayre syndrome
•Refsum disease
•Muchopolysachoroidosis

50
Q

Retinitis Pigmentosa - Signs and symptoms

A

•Symptoms
- Nyctalopia (loss of night vision)
- Reduced visual field
- Reduced VA (later stages)

•Signs
- Mid-periphery bony spicules
- Pale disc
- Arteriolar attenuation
- Cataract
- Myopia
- Primary open angle glaucoma
- CMO

51
Q

Retinitis pigmentosa - Treatment

A

•Supportive
•Low vision assessment
•Blind registration

•Cataract surgery
•Dorzolamice for cystoid macular oedema

•Genetic treatment
- Genetic counselling
- RPE45 gene therapy now available

52
Q

Stargardt’s disease - What is it?

A

• Commonest macular dystrophy (RPE is retinal common)
•ABCA4 gene mutation
•Cone-rod dystrophy, central loss of vision progressing to peripheral

•Accumulation of lipofuscin in the RPE (prevents nutrients transfer)
•Presents in childhood or adolescence
•Characterised by retinal flecks

53
Q

Best’s macular dystrophy

A

•2nd commonest macular dystrophy
• Best’s=Juvenile form of the disease (different from adult onset vitelliform)
•BEST1 gene mutation
• Involves macula only, not peripheral retina
•Usually bilateral

54
Q

What are the 5 stages of bests vitelliform macular dystrophy

A

•Progressive disease presenting in childhood
•5 Stages total:
- Stage 1: Pre-vitelliform = electrophysiology changes with normal fundus
- Stage 2: Vitelliform = “sunny side up” egg yolk appearance
- Stage 3: Pseudohypopyon = yellow material settles inferiorly within lesion
- Stage 4: Vitelliruptuve = “scrambled egg” appearance
- Stage 5: Atrophic stage = central atrophy, risk of secondary CNV

55
Q

Chloroquine/Hydroxychloroquine toxicity - what is it?

A

•Chloroquine is an antimalarial drug
•Hydroxychloroquine is used as an immunosuppression
•Have an affinity for pigment “melanotropic”
•Accumulative in the RPE
•Bulls eye maculopathy
•VF defects

56
Q

Drug induced crystalline maculopathy - what is it and what causes it?

A

•Commonest drug is Tamoxifen (breast cancer)
- Fine crystals form on inner retinal layers
- May lead to macular cysts and loss of vision
•Other drugs
- Canthaxanthin (Tanning - increases pigment)
- Nitrogurantoin (Urinary tract infection drug)

•Other causes
- Bietti crystalline dystrophy
- Cystinosis (corneal and macular crystals)
- Gyrate atrophy
- Sjorgen-larsson syndrome

57
Q

Epiretinal membrane - What is it?

A

•Fibrocellular film that develops surface of retina
•Contraction causes wrinkling on surface
•May develop
- Loss of foveal contour
- Irregular retinal surface
- Pseudocysts/CMO formation (called Psydo due to not actually a cyst, just a pulling away of retina looking like a cyst)

58
Q

Epiretinal membrane - Causes

A

•Idiopathic
•Inflammation
•Infection
•Post op
•Post laser
•Vascular disease
Always treat cause first, before the membrane to see if it goes away!

59
Q

Epiretinal membrane - Signs and symptoms

A

•Reduced vision
•Distortion
•Asymptomatic
- Only refer if symptomatic

60
Q

Angioid streaks

A

•Cracks in bruch’s membrane due to calcification and thickening
•Usually radiate from disc
• 50% have systemic association
- PEPSI:
Psuedoxanthoma elasticum
Ehlers danlos syndrome
Paget disease
Sickle cell disease (and other haemopathies
Idiopathic

61
Q

Choroidal folds - what is it

A

•Indulations of choroid - can affect all layers
•Due to compressive stress on the choroid
•Orbital/scleral/choroidal congestion
•Usually asymptomatic
•Need to have careful ocular examination
•Scan to exclude orbital pathology
• Treatment of underlying cause

62
Q

Choroidal folds - Causes

A

•Causes:
- Idiopathic
- Hypermetropia
- Papilloedema
- Orbital disease
- Ocular disease

63
Q

Urgent referral list :

A

•WET ARMD
•Vascular causes with sudden loss of vision (RAO/RVO/Hypertensive retinopathy)
•Any secondary CNV

64
Q

Routine referral list:

A

•Dry ARMD
•Diabetic retinopathy/maculopathy that fits the DRS referral criterion
•CSR
•ERM only if vision affected
•Hereditary dystrophies
•Drug toxicities

65
Q

Do not refer list:

A

•Asymptomatic ERM/VMT/Dry ARMD - Monitor
•Diabetic maculopathy (M2 from DRS) with VA >6/7.5
•Patients already in the system/active treatment
•Patients already registered as blind/discharged - Consider LVA referral if necessary
• Patients who don’t want to come!

66
Q

What is included in referral - always?

A

•VA
•Refraction
•Symptoms including duration
•Describe fundus findings - include photo and/or OCT if possible
•Always include lens status - phakic/pseudophakic. If phakic is the lens clear or is there cataract
•IOP