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SECTION 4: RETINAL PATHOLOGY

ANATOMY REVIEW
Vitreous
Attached to retina
Internal limiting membrane by fibrils at 3 points: Ora (strongest) Optic disc
(around ONH) at macula (ring around macula weakest
Retina
Inner limiting membrane
Nerve fibre layer (biggest
BV)
Ganglion cell layer
Inner plexiform layer
Inner nuclear layer
Outer plexiform layer
Outer nuclear layer
External limiting
membrane
Photoreceptor layer
RPE layer
Bruchs membrane
Choroid
1. Inner Retina
Vessels

Function
Histology
Clinical
2. Outer retina
Vessels
Clinical

Inner retina

Sensory retina

Outer retina
(Avascular)

Choroid

All retinal vessels are here


NFL = larger arteries and veins
INL = arterial and venous capillaries
Supplies nutrition to retina
Bipolar, horizontal, amochrine Mueller cells
Specialized glial cells
Smaller vessels in nuclear layer leaks with diabetes

Supplied by vessels from choroid


Avascular
Any bleeding here originates ffrom the choroid
OPL tends to accumulate any fluid in the retina (primarily
interstitial space)

3. RPE Layer
Attachm
ent

Function

Clinical

Firmly attached to Bruchs membrane by tight junctions


Barrier (outer retinal barrier) to prevent leakage of fluid
from choriocapillaris
Attached to photoreceptive layer by loose junctions
Service and maintain receptor cell by phagocytes
Regeneration of pigment
Absorbs light
Acts as blood retinal barrier
With age, RPE is less efficient
Phagocytosis decreases > waste products accumulate >
restored as lipofucin within RPE and Drusen in Bruchs
If outer retinal barrier is compromised, leakage results

4. Bruchs membrane
Def
Part of choroid
The only elastic layer
Function
In young eyes, it is smooth and ensures regular alignment of
RPE
Separates choriocapillaris from retina
Clinical
All systemic diseases affecting collagen and elastic tissue will
affect Bruchs
Layer changes with age and is involved in many disease
processes
Breaks in Bruchs allow for sub-retinal fluid and
neovascularization (from choroid)
Bruchs and RPE are closely related and disease in one usually
affects the other
Compromise to Bruchs can lead to compromise to choroid
layer and RPE
5. Choroid
Vessels

Vortex
veins

Contains choriocapillaris in inner, middle and outer layers


Leads to vortex veins
Choriocapilllaris vessels have fenestrated endothelial cells,
which leak easily, have loose junctions and good permeability
Can be seen with BIO
Looks like spirals or pineapple heads in mid periphery

6. Sclera
- Avascular
- Relies on episcleral and choroidal BV
7. Macula
Vessels
Clinical

No vessels
Nurtured by choriocapillaris
Cherry red spot in CRAO

Sensory
retina

ILM, OPL, ONL and photoreceptors


RPE characteristically elongated and has increased elanin
OPL has xanthophil pigment

COLOURATION OF THE RETINA

Due to combination of pigmentation in vaious ocular layers


Most of colour is due to: RPE layer (pigment) highly vascularized choroid white
sclera

Varations of normal fundus


1. Tessellated or Tigroid Fundus
- More noticeable away from posterior pole towards periphery where fundus
appears more pigmented in streaks running toward periphery
2. Pigmentation of fundus
Association
Related to skin/hair colour of patient
Young, fair patient or albinoids > lack of pigmentation in RPE >
choroidal vasculature may be seen shining through
Darker skin > darkly pigmented fundus
Any area of attempted healing may become more pigmented
or depigmented
Choroidal
Pigmented crescent shaped area on border of optic disc
crescent
Due to retraction of choroid and retina
Often seen in myopes (myopic crescent)
Choroidal
Dark rim is due to accumulation of pigment at aperture
ring
through which optic nerve passes
Scleral ring
white ring found inside of the other formed by exposure of
sclera due to larger choroidal aperture
Zones alpha
varied pigmented zones within the crescent in very early
+ beta
development of glaucoma
Risk indicator
Clinical
Choroidal crescent and scleral crescent presence > suspected
indication for glaucoma

DIFFERENTIAL DIAGNOSIS OF RETINAL LESIONS


Pigmentary changes
1. RPE Window Defects RPE hypoplasia
RPE
Single layer with pigment
Def
Lost/absent RPE, causing pallor in that area
Looks like drusen
Morph
Seen as tiny white spots on fundus
Angiogram makes it easy to see
Aetiology
Congenital
Acquired > Due to inflammation or degeneration
Clinical
No associated visual field defects
If it occurs on a wide spreading manner, RPE atrophy and choroidal
vessel atrophy may result
2. RPE hypertrophy
Def
Increase in RPE cell enlargement
Location
More in periphery
Morph
Area looks darker
Regular shape with well-defined borders (like naevus)
Size = 0.25 DD to 8DD
If larger > halo around darker area (Halo naevus)
Tiny collections of hypertrophy= bear track or puppy paw tracks
Aetiology
Usually congenital
Pathogenesi
Benign abnormal elongation of cells in groups or isolation
s
No change in size
Do not become malignant
Clinical
No associated visual field defects
No usually referred unless related to other specific conditions
Atypical congenital hypertrophy puppy paw tracks > strong link with
adenomatous polyps in rectum and colon that develop in
adolescents
80% develop colon cancer by 50y
Polyp: skin lesion that normally has a peduncle
With pigmentation, look for regularity
3. RPE Hyperplasia
Def
Increased number of RPE cells
Aetiology
Secondary to iinflammation, infection or trauma
Typically in retinitis pigmentosa
Morph
Are is irregular in shape, but has defined borders > bone spicule
formation
Not uniform pigmentation

4. Retinal Hole
Def

Morph

Association

Managemen

Clinical

Sensory retina is removed


More orange/red than surrounding area
More vivid than surrounding area
If a white lesion is present, it is due to an RPE window defect
Not usually referred unless related to other specific conditions
Seen with OCT > can see development > lighter/redder than
surrounding area (lacks RPE in that area)
Danger with new holes > can have fluid leakage > progress to full
thickness hole

5. Choroidal Scar
Aetiology
Toxoplasmosis
Result of inflammation or subretinal haemorrhages
Def
Well-defined white area indicative of scar tissue, or absence of
retinal and choroidal ttissue
Morph
Often lesions are big (1-2DD)
Fluffy white region around lesion (Foglight) = ACTIVE (Indicates cells
in vitreous)
6. Choroidal naevus
Presentatio
Can become malignant
n
Morph
Size = 0.25DD to 7DD
Colour = greenish/gray and faded compared to RPE
hyperpigmentation
Feathery but defined borders
Managemen
If larger than 4DD, refer
t
Document shape (H and V) and elevation/flatness
If drusen is observed superficially, it is a good sign
Drusen reflects (lack of absorption of lipofucin)
Tests
With Red Free Filter, choroidal naevi seem to disappear/get less
7. Choroidal melanoma
Clinical
Lipofucin = waste product of RPE layer = metabolic problem
Orange = malignancy (Showing choroiddal blood vessels due to
thinning of RPE
Managemen
Cobalt plaque-ing
t
Radiation therapy
Enucleation

Haemorrhages in the retina and choroid


1. Pre-retinal Haemorrhages Boat/keel shaped haemorrhages
Location
Occurs between vitreous face and ILM or between NFL and ILM
Haemorrhage lies above the retina
Morph
Relatively large = 2-3DD
Bright red
Retinal blood vessels obscured
Typical boat/keel shape occurs with time (Due to gravity/settling of
blood)
Clinical
Transient scotomas present > take long time to absorb (6months)
Haemorrhage resolves very slowly
Aetiology
Usually secondary to systemic diseases
Blood dyscrasia
Diabetes
Hypertension
Leukaemia
2. Nerve fibre haemorrhages
Def
Result of leaking large vessels in NFL
Location
Drance/dric haemorrhage = at ONH (associated with normotensive
glaucoma low tension glaucoma)
Usually occurs at posterior pole, peripapillary areas or arcades
(where nerve fibres are)
Morph
Fairly small (visual field defect often too small to pick up)
Clinical
Visual acuity usually not affected
Resolution fairly quick (4-6 weeks)
Aetiology
Primarily due to systemic disease that affects arteries and veins
Hypertension and venous based disease
Diabetes
Retinal vein occlusion
Papilloedema
Could also be neurologically based
3. Roth Spots
Location

Morph

Clinical

Aetiology

Occur in NFL
Flame shaped haemorrhages
Yellow/white centre
If seen in young px > associated with leukaemia (spot is due to
accumulation of WBC)
Hx important
In adult, white spot may be fibrin or cotton wool spot (If close to
large blood vessel, possible infarction)
Diabetes
Hypertension
Connective tissue diseases
Other inflammatory vascular diseases (lupus, anaemia)

4. Intraretinal haemorrhage Dot and Blot haemorrhage


Location
Occurs in OPL, but originates in INL
Often in small blood vessels
They sit underneath bigger blood vessels
Morph
Extremely small
Looks like dots/blots
Clinical
Do no cause scotomas
Do not obscure BV
Resolve over several months (deeper in retina)
Aetiology
Secondary vascular disease causing venous stasis or congestion
Blood dysplasia, slow flow retinopathy, congested
Typically seen in early diabetic retinopathy
5. Subretinal haemorrhage
Location
Between sensory retina and RRPE (from choroid)
Morph
Borders are irregular and feathery
Relatively large
Less darker
Clinical
Very long time to resolve
Do not obscure BV
Causes permanent scotoma and permanent scar
Acuity reduced even with reabsorption
Aetiology
Secondary to degenerative processes or inflammatory processes
Breaks I Bruchs and RPE
Choriocapillaris leakage
Pathogenesi
Haemorrhage occurs above RPE layer with its loose junctions
s
tends to spread sideways and shallow
brighter red compared to sub-RPE haemorrhage
6. Sub-RPE haemorrhage
Location
Between RRPE and Bruchs
Morph
Grey/green colour (under RPE)
Smaller but more raised (tight junctions between Bruchs)
Darker
Clinical
Do not obscure vasculature
Long time to resolve
Permanent scotomas and scarring
Subretinal haemorrhage and sub-RPE haemorrhage may occur
together
Aetiology
Secondary to degenerative process/inflammatory break in Bruchs

Vascular related abnormalities to retina


1. Microaneurysms
Def
Breakdown in BV walls (related to systemic DO, esp diabetes) where
vessels become very permeable and leak
Diabetic nature of blood = thinner, slower, hypoxic
Results in retinal haemorrhages/exudates
Location
Arise from INL
Aetiology
Hypoxia
Morph
50-100 microns
Test
Only visible by fluorescein angiography
2. Macroaneurysms
Test
Easily visible with ophthalmoscope
Fluorescein angiography: Tiny spots showing leakage
Clinical
Isolated and rare
Surrounded by circinate exudate
Seen in association with vasodilation of major arterial branch
Aetiology
Vascular diseases causing venous stasis, where vessels become
extremely leaky
Presentatio
+50y
n
Presenting with systemic hypertension (typically malignant)
Females more than males
Prognosis not good
Tx
Must be referred
3. Exudates
Morph

Presentatio
n
Location
Aetiology
Pathogenesi
s

Tx

Small, yellow, waxy, shiny


Not regular and round > residual like particles
Partial exudates or macular star exudates
Usually bilateral
More in number in one eye
Occur in OPL, but from INL (like dot and blot, and keep company
with dot and blot)
Secondary to chronic partial occlusion of small vessels
Occlusive disease
Leakage of clear fluid from occluded vessels result in lipid deposits
Forms circinate ring around leaking vessel (can be in area around
macula)
Remain for months before being absorbed
Argon laser to decrease leakage
Ant vgf into vitreous to decrease inflammation and leakage

4. Drusen
Morph

Location
Def
Presentatio
n
Pathophys
Clinical
Test

Duller than exudates


Yellowish
Refractile
No specific pattern
Between RPE and Bruchs
Peripheral retina
Consists of accumulation of waste products of RPE metabolism which
is not removed by choriocapillaris, and deposted extra-cellularly
Mostly bilateral and symmetrical

Altered state of metabolically healthy retinal complex


Huge cause of legal blindness
Fluorescein is taken up earlier and fades in late stages
Hard/Nodular

Morph

Clinical

Morph
Clinical

DDX
Associated
conditions

Small
Localized RPE dysfunction
Definite borders
If area grows or involves Bruchs and groups together, it can become
soft/confluent drusen
Soft/Confluent
Larger
Fluffier borders
Associated with pathological situations
Peripheral = Not a problem
Macular area = problem
Almost looks like cotton wool spots
Difference: no pathology + under retinal vessels
ARMD
Peripheral, retinal degeneration

5. Cotton wool spots


Moph
White
Fluffy borders
Location
In NFL in inner retina
Close to/on top of arteries and veins (wont see in area without
retinal blood vessels)
Obscures BV
Within 3DD of ONH, but never in fovea
Follow nerve fibre striations
Clinical
Implies ischemic micro-vascular disease (lack of O2 in NFL)
Precursors to retinal neovascularisation
Due to infarct, will take a while (2-12 weeks) to resolve
Once resolved, will leave a nerve fibre defect
Isolated CWS in onoe eye = possible inidicator of HIV
Pathophys
Due to occlusion of terminal retinal arterioles leading to infarct of

NFL
Aetiology
secondary to vascular diseases with retinal hypoxia
HIV Diabetes Hypertension SLE BV infarct disease
6. Retinal neovascularisation
Aetiology
Secondary to retinal ischemia or hypoxia (precipitated by stimulus)
Morph
Very fragile and lacey
Location
Between NFL and ILM
Or between ILM and vitreous (very superficial)
Categories
Neovascularisation at the disc (NVD)
Neovascularisation elsewhere (NVE)
Clinical
Vessels tend to leak and haemorrhage
Results in fibrosis and later retinal traction
Main cause of neovascularisation in the eye
VEGF (veso-endothelial growth factor) present
Tx
Anti VEGF injected (anti-inflammatory)
Neo in eye
Retinia disc choroid - iris
7. Intraretinal Microvasular Abnormalities (IRMA)
Location
Occurs in areas of retinal hypoxia (usually deeper part) in intraretinal
vessels (very small vessels)
DDX
Easily mistaken for neovascularisation vessels or collateral, but
DEEPER
Fluorescein angiography > IRMAs leak in late phase of angiogram
Clinical
Poor prognosis
Aetiology
Response to hypoxia
8. Collaterals
Aetiology

Def

Function

DDX
Morph

Joins

Occurs after the event, when continual retinal hypoxia is present


Good vessels (save system)
Shunt systems that form in small areas of pre-existing vessel
network
Drains blood around area of retinal compromise
Shunts blood around occluded artery or vein from one vessel to
another > indicates self-healing
Unlike IRMA, they do not leak during fluorescein angiography
Strong non-fenestrated tight junctions
Tortuous appearance
Small
A-A
10% of A-V
Less V-V

9. Serous retinal pigment detachment


Location
Between RPE and Bruchs
Morph
Small, focal detachment
Distinct borders
Contains clear (serous) fluid
More raised > looks a bit blurry
Test
Use green filter to see nicely > look for demarcated area
10.
Location
Morph

Serous

sensory retinal detachment


Between RPE and sensory retinal layer
Larger in size
Contain clear fluid
Shallow detachment

11.
Choroidal neovascular membrane (CNVM)
Pathophysiol
Due to neovascularisation of choroid
ogy
Underlying blood vessel formation is like a fibrous meshwork and is
obscured
Clinical
Ocular emergency
CNVM tend to grow rapidly and are unpredictable
May recur at later stage
Morph
Greenish-grey membrane
Slight elevation to area involved
Layers
Choroid Bruchs RPE sensory retina
involved
Tendency to underlie macula
Complication
Serous RP detachment
s
Serous sensory retinal detachment
Retinal haemorrhage
DDX
Between serous detachments
Serous are paler, more defined by their definite borders and balloon
out acuity more affected
Use 90D to see
Tx
Urgent referral
Try to determine aetiology and location
Fluorescein angiography and laser treatment indicated
Photocoagulation

Aetiology
ARMD
Presumed
ocular
histoplasmos
is

Pathological
myopia
Angioid
Streaks

Argon

Krypton

Yag

Visuodyme

Primary cause of CNVM in older population


Primary cause in younger population
Inflammatory condition causing damage to RPE and Bruchs
If occurring close to macula, increases risk of CNVM
Histospots (triad) in mid periphery
Macular involvement
Cystoid macular oedema
Optic nerve involvement
Progressive myopia (> 12-15D
Causes retina to stretch, including Bruchs (especially at fovea)
Fuchs spot and lacquer cracks also observed
Systemic problem affecting Bruchs
Associated with following conditions:
Pseudoxanthala
Females 40-50y
sma elasticum
Plucked chicken look due to premature aging of
skin under neck and stomach
Sick cell
anaemia
Pagets disease
Involve calcific changes in Bruchs
Involved in bonoe deformity
Ehlers-Danlos
Hyperelasticity
syndrome
Circus people
Trauma/choroid
Associated with younger patients
al ruptre
Idiopathic and
1. Choroidal naevus
other
2. Excessive photocoagulation
3. Drusen of ONH
4. Chorioretinis
5. Best disease
6. Rubella retinopathy
7. Choroidal melanoma and haemangioma
8. Serpiginous choroidopathy
Laser Tx
Used in foveal avascular zone
Xanthophyl absorbs wavelength and creates more scarring by
burning tissue
Softer laser
Not absorbed by pigment
Used if CNVM is firther out
Idea is to preserve existing vision
Not used here
Involves much higher intensity for shorter duration
Removal of opacification of posterior capsule after cataract surgery
Idea is to decrease oxygen need and to keep neovascularisation
from spreading
Patient is injected with specific photo labile chemical

congeals within appropriate area on laser activation


effectively stops leakage

Anti-VEGF
The Macula
A. Anatomy Review
Posterior
Size: 3 to 4DD
pole
Area is defined where ganglion cells are greater than one cell layer
in thickness
Normally extends from temporal edge of ONH, bounded by vascular
arcades
Macula
Size: 5mm
Affects central 5deg of vision
Region bounded by Xanthophyll and contains more than 1 layer of
ganglion cells
Unique part of retina due to elongated RPE cells
Avascular
Choroid underneath is extremely vascular and appears darker
Central pit accentuates foveal slopes
Fovea
Depression in inner retinal surface at centre of macula
Forms foveal reflex due to concave mirror effect (Loss of reflex is
significant)
Affects central 1-deg of vision
Size: 1.5mm
Foveal
Important landmark in laser Tx
avascular
Extends to 1/3DD in size
zone
Darker, and RPE is taller with presence of xanthophyll
300 microns devoid of capillaries
Foveola
Forms central floor of fovea
Diameter = 0.35mm
Contains no ganglion cells
Only cones and their nuclei
Very thin
Central depression = umbo
B. Details of anatomy of macula
Retinal
Only outer layers present: ILM OPL ONL ELM Photoreceptors
Layers
(outer seg) RPE layer
OPL has huge cystic spaces (carries receptor cell axons) > increase
in extracellular space at macula
Function of RPE layer is to keep this area relatively dry > pumps out
Fe and water from subretinal space
Pathology > fluid tends to accumulate
NFL of Henle = radial arrangement of axons > forms stellate pattern
of fluid and exudate if RPE is compromised
RPE
RPE-Bruchs very tightly attached and affects each other
Bruchs
If integrity is lost, waste products from photoreceptor cells collects
choriocapilla
(RPE cant phagocytose it)

ris
Bruchs

Innermost part of choroid


Firly adhered to RPE layer = barrier (Blood-retinal)
Consists of collagen and elastic tissue (5 layers) > any systemic
diseases affecting these tissues affect Bruchs
Choriocapilla
Supplies outer retina
ris
Centre of macula depends on choriocapillaris > Open network of
fenestrations in endothelial cells as well as density in numbers
C. Theories to explain vulnerability/susceptibility of macula to pathology
Hemodynam
If RPE and Bruchs break down > complicates the influx of blood
ic Stress
supply and related nutrition from choriocapillaris
Theory
H2O cannot be pumped from spaces
Fluid like detachment results
Watershed
Decrease in oxygen-blood perfusion to choriocapillaris > decreased
Theory
nutrition
Ischaemic response > macula will respond differently > macula is
extremely sensitive
D. Clinical examination of the macula
1
VA
Distortion or decrease in VA
Even if VA is okay, retina may not be smooth anymore >
distorted image
If suspected, follow up with Amsler
2
Amsler
Monocular
ADV: Easy test to dispense for home therapy
DDV: Poor px compliance
3
Photostress
Monocular with distance Rx (Test better eye first, then other eye
test
> compare)
One line above best VA projected
Bright light shone onto fovea for 10sec
Recovery time measured (how long it takes for px to read line
10-20sec recovery is okay)
Measures function of photoreceptor layer (synthesis of visual
pigment > integrity of macula)
Only for macular problems; not optic nerve disease
Dry exudates give better function than wet
4
Pupil APD
Marcus Gunn pupil
Positive = significant lesion to papillo-macular bundle
5
Fundus eval
Binocular ophthalmoscopy better than monocular
78/90D lens BIO Goldmann 3 mirror central lens
Compare posterior poles of eyes
6
Fluorescein
Valuable in understanding physiology of retinal and choroidal
Angiograph
circulation
y
Must know what to look for
Important in treatment prior to laser therapy
7
Indocyanine
Newish
green
Less allergic response

angiograph
y
Fundus
photograph
y

Colour
vision

10

Contrast
sensitivity

Better to evaluate choroidal circulation

Dx
Documentation
Objective monitoring
Good test for acquired colour defects
Blue/Yellow loss indicates macular disease
Tritan defect = diabetes with oedema
Red/Green defects = ONH disease
Impression of quality of vision
Higher frequency CS decrease in certain macular pathologies

Diseases of the outer retina


A. Age Related Macular Degeneration (ARMD)
Epidemiolog
Leading cause of legal blindness in +60y
y
10% = 6/60
Risk factors
Age (30% of 75y will have acuity loss due to macular degen)
Cigarette (causes constriction of BV)
Blue-eyed
UV exposure
Familial Hx
Arteriosclerotic vascular disease
Many unknown factors
Clinical
Dry or non-exudative or atrophic form (most common)
presentation
Wet or exudative form
Dry ARMD (nodular)
Epidemiolog
80-90% of ARMD
y
S+S
Mild to severely affected VA
Bilateral but asymmetrical
Decreases gradually
Affects central vision
Clinical
RPE changes
appearance
RPE function compromised > cannot clear waste > more
disrupted
RPE dropout/dumping results
Normally, areas are well circumscribed and typically goes with
hyperplasia
First pigmentary changes > mottling defects in RPE or focal
areas
Thickening of Bruchs > look for uniform colour
Drusen
Metabolic waste from photoreceptor layer
Contain lipofucin and cell debris = hard drusen
Never disappear and get progressively more, but takes time
May coalesce > major non-functional areas
Geographic atrophy (SCOTOMA on Amsler)
Management
Low vision aids > telescopes (improve remaining functional vision)
Dietary supplements > may slow down degen process
(controversial)
Zn (min daily allowance) Vit E Anti-oxidants (B-carotene)
May prevent release of free-radicals > maintain integrity of fine
vasculature for longer
Ocuvit (expensive)
Wet ARMD (Exudative discharge)
S+S
Central vision affected
VA lost quickly and severely
Clinical
Degeneration of RPE
appearance
Thickening of basement membrane

Risk

Drusen/basal lamina deposits


Px with more confluent/soft drusen more prone to developing wet
ARMD

B. NNB CNVM formation refer to earlier notes


Test
Confirm with fluorescein angiogram
Amsler is good home self monitor > compliance not good > force px
to come back
Clinical signs
If present in one eye, high risk to other
Blood in macular area arises from subretinal vascular supply or
retinal vessesl > no underlying retinal vascular disease = CNVM
sign
Complication
Disciform scar > permanently damages big area after long time >
scotoma
Tx
Urgent referral
C. Idiopathic Central Serous Choroidopathy (ICSC) Refer to section 3 choroidopathic
degenerations
Clinical signs
Occurs only in macula (very occasionally may be extra-foveal)
No CNVM formation
Risk
Females 10x more than males
20-45y
High emotional stress
Hx of headaches
Type A personality (yuppy type overworked young achiever
emotionally stressed)
Clinical
Sudden decrease in VA from 6/6 or 6/5 to 6/60
presentation
Unilateral (depending on how long fluid leakage has been there
Tests
Refractive correction: Hyperopic shift
With PH or plus lenses, VA increases dramatically
Colour vision desaturation
Amslers grid: metamorphopsia micropsia central scotoma
Clinical
90D/
Small elevation
appearance
Goldmann
Subtle mottling
Ophthalmosc
Absent foveal reflex
ope
Lesion
1-4DD
Elevated
Round/oval
Borders outlined by glistening reflex
Fluorescein
Smoke-stack appearance
angiography
Indicates seepage of dye through subretinal space
at first, and then outwards
Mx and Tx
Slow recovery (1 week 1 month)
95% recover to 6/9 or better (vision not same > colours duller)
5% never recover > Some retinal specialists may drain OR photocoagulation (argon laser)

Diseases of the inner retina


A. Epiretinal membrane
Test
Direct ophthalmoscopy
Seen best with red-free filter > irregular light reflex/sheen observed
Synonyms
Pre-retinal membrane
Cellophane maculopathy
Pre-retinal gliosis (glial cell proliferation)
Surface wrinkling retinopathy
Mcular pucker
Epidemiolog
50-65y males and females
y
Pathophysiol
Glial cell proliferation and subsequent membrane formation at
ogy
vitreo-retinal interface
Later on, becomes more obvious when membrane contracts
(puckers) like cellophane > tiny folds in epi-membrane seen
Pseudo-macular hole seen due to membrane defects
Causes
Idiopathic
RD surgery
Peripheral photo-coagulation
Trauma (Break in ILM)
Ocular inflammation
Clinical
May or may not affect VA (6/36 worst)
presentation
Gradual loss
Amsler: metamorphopsia
Management
Educate px
Usually only 1-2 lines reduction in acuity
VA 6/36 > some retinal surgeons will peel off membrane > 50%
success rate + recurs
Complication
Cystoid macular oedema
B. Macular hole
Pathophys

Clinical

Epidemiolog
y
Causes

Foveal region is thin, avascular and lacks support > susceptible to


hole formation
Loss in retinal tissue in circular area = 1/4DD
Sometimes, macular cyst precedes macular hole (VA is better)
If observed in one eye, 10% chance it will occur in the other
Bright/deep red circular area
Sometimes oedematous slight whitish cupping around hole
(punched-out lesion)
Women 50+
Trauma
Idiopathic
Myopia (high myopes with staphylomas > macular hole > RD)
Solar retinopathy
Small lamellar hole or cyst develops 2 weeks after sun exposure
Observed in young blacks > tribal custom of gazing at sun

Looks like macular burn


DDx
Pseudo macular hole
Macular cyst
Mx
Good low vision candidate
C. Cystoid Macular oedema See section 3
Cause
Extra accumulation of fluid in outer plexiform (Henles layer) and
inner nuclear layers centred about foveola
This is a clinical sign only and not a diagnosis
Associated
Aphakes
with
Intra-ocular surgery (Irvine-Gass syndrome)
Retinal vascular disease (background diabetic retinopathy, RVO)
Trauma
Drug use (prostaglandins and adrenaline 2% - glaucoma tx)
70% occurrence in RP patients
Idiopathic
Clinical
Not easily observed
presentation
Fovea looks bumpy and yellowish with cystic development
Decreased foveal light reflex
Petaloid macular appearance
VA 6/6 6/60
Recover 3 9 months
Short term and innocuous
If long standing > permanent vision loss
Test
Best detected with Fluorescein angiography > hyper fluorescence
Amsler: may or may not record metamorphopsia
Mx
Progress should be checked and documented
Little penetration of drugs > aggressive NSAID
Argon laser sometimes used to remove fluid
Complication
Epiretinal membrane
s
Macular hole (lammmellar)
Permanent decreased acuity