SECTION 7: RETINAL DEGENERATIONS

PERIPHERAL RETINA
Anatomy
Def
Equator

Short ciliary
nerves and
arteries
Long ciliary
nerves and
arteries

Blood
vessels

Oral bays
Oral teeth

Pars plana

Area of retina anterior to equator extending to ora serrate

Encompasses 40% of ocular fundus
Imaginary line drawn from vortex ampullae (4-6)
Usually one per quad
Looks like octopus or pine-apple, with varying amounts of pigment
Most prominent in vertical meridians
10-20
Run perpendicular to ora
Yellow/white and may have pigment around borders
Most prominent at 3 and 9 o clock
Separates superior and inferior retina
Usually 2
Runs perpendicular to ora
Artery usually runs above ciliary nerve in nasal retina, and below in
temporal retina
Yellow/white in fccolour
Become smaller and less towards periphery
Changes direction as they approach ora, running parallel to it
Ora denotes anterior limit of retina
Nasally is wider and more scalloped
Rounded extensions of pars plana at ora (seeing to non-seeing)
Dentate processes
Extensions of retina between oral bays
Usually white
Anatomical landmark
Chocolate colour
Runs from ora to ciliary processes
60-70

Variations in peripheral retina

Meridional folds
Presentation
Mostly benign, no consequences
Usually bilateral
More common in males
Developmental anomaly present in 25% normal eyes
Def
Permanent fold involving all retinal layers
Typically elevated and with slight alteration to retinal tissue and
glial cells
Considered as posterior extensions of oral teeth
Whiter than surrounding
Location
More in superior nasal quadrant

Meridional complex:
Fold that extends all the way to ciliary processes

Benign peripheral retinal degenerations and variations

Peripheral senile pigmentary degeneration
Morph
Granular pigment
Pigment has reticular/bone spicule appearance
Location
Between ora and equator
Presentation
20% population over 40
Bilateral and benign
Pathophysiol
Caused by breakdown of RPE
ogy
Deposition of pigment granules in sensory retina
Ultimately loss of photoreceptors and sclerosis of choriocapillaris
Implicates possible vascular compromise with aging
Management
Monitor/routine dilation 2 yearly
Paving stone/cobble stone degeneration
Morph
Whitish
Multiple rounded punched out areas of choroid and retinal atrophy
Lesions flat with discrete margins + pigmented edges
Lesions: Several disc diameters in size
Location
Usually inferior nasal and inferior quadrant
Lesions are located between ora and equator
Presentation
Common with increasing age > 40y
Typically benign
Pathophysiol
Results from occlusion of compartments of choriocapillaris
ogy
Causes RPE degeneration and retinal thinning > paler area
Management
Monitor/routine dilation 2 yearly
Cystoid degeneration
Presentation
Occurs in virtually all px over 8
Pathophysiol
Involves cystoid like changes in outer plexiform layer of retina
ogy
Can extend to involve entire sensory retina
If cysts enlarge and coalesce, may form large cystoid spaces > true
splitting/retinoschisis of sensory retina
Can develop inner retinal holes > no threat to developing RD
Clinical
Area of thickened retina DD from ora
appearance
May extend to equator > difficult to see
Looks like honeycomb
Management
Document + routin dilation 2 yearly
If patient has retinoschisis, dilate yearly
Location
Found more in temporal and superior retina

White with pressure

Def

Presentation

Pathophysiol

ogy

Clinical

appearance

Artificially increase pressure > retina will bleach out in 32% px

Benign
Not associated with retinal breaks
Distinct milky white opalescent appearance of peripheral retina
when scleral depression is done
Releasing pressure goes back to normal
White area at depression surrounded by darker area (shadow)

White without pressure

Presentation
In darker pigmented fundi, reported to be seen 10x more often
More common in myopic px
Associated with aging
Pathophysiol
Unusual vitreo-retinal relationship causes disorganization of NFL of
ogy
sensory retinal down to RPE
Clinical
Distinctive white appearance of peripheral retina visible without any
appearance
depression
Generally occurs between ora and equator > may extend
posteriorly to equator
Management
No treatment
Associated with abnormal vitreo-retinal adhesion > can lead to RD
Consider annual exams
Peripheral retinal findings associated with retinal complications
Shaffers sign/tobacco dust
Presence of cells containing golden brown pigment granules in anterior vitreous of
phakic eyes
Suggests retinal breaks
Atrophic retinal holes/tears
Pathophysiolo
Full thickness breaks in retina due to progressive retinal thinning
gy
Due to underlying vascular insufficiency that compromises retina
> breaks in retina
Red appearance > RPE and choroid where retinal tissue is not
longer
Clinical
Seen from pinpoint to 2DD round red lesions
appearance
Atrophic hole surrounded by dark brown pigment > indicates
lesion has been present for 3 months or longer
Complications
Atrophic retinal holes present access for liquefied vitreous to seep
underneath sensory retina
Fluid accumulation between sensory retinal and RPE > sensory RD
10% of atrophic holes can develop this
Cuff of oedema observed (intra-retina oedema) > sub-clinical
sensory RD surrounding lesion
Management
Patient education (because of risk of detachment) explain
symptoms

If pigmented, monitor annually

If cuff of oedema, refer to ophthalmologist

Operculated retinal hole

Pathophysiolo
Due to abnormal vitreo-retinal adhesions
gy
Plug of sensory retinal tissue will detach from RPE
Plug = operculum > attached to vitreous
Clinical
Typically from ora to equator
appearance
Round red holes with free floating plug of retinal tissue attached to
vitreous
Operculum creates shadow on retina > grey colour
Px might complain of floaters when detached
Complication
Oedema around hole
s
Vitreous could get stuck and create sub-clinical detachment
Management
If oedema > refer
No oedema + plug/pigment seen > no referral
Normal
Normal holes caused by chronic atrophy of neuro retina
Lesser chance to develop intra retinal detachment
If there is cystic tuft, more likely to develop RD due to traction
Horseshoe tears (also called flap tears)
Def
Common and important type of full thickness retinal tear (RPE still
attached)
Sx
Flashes of light
Pathophysiolo
Usually due to abnormal vitreo-retinal adhesions and posterior
gy
vitreous detachment
Tear occurs where part of retinal tissue is attached to vitreous
(ffree end) and base to retina
Clinical
Horse shoe shape
presentation
Location NP > if superior, prognosis not good
Complication
Holes more likely to lead to retinal detachment than normal retinal
s
holes > have vigtreal traction to pull on flap of tear
Traction makes it more likely for fluid to pass through tear and
undermine potential space between retina and pigment epithelium
Vitreal haemorrhages commonly occur when horse shoe tears are
produced
Managementt
30% RD
Refer all horse shoe tears to ophthalmologist

Lattice degeneration
Presentation

Pathophysiolo

gy

Location

Morph

Complication
s
Management

Distinctive important retinal degeneration

Oval/elliptical retinal thinning near equator
Common in 8% population
Vitreo-retinal degeneration affecting inner retinal layers and vitreoretinal surface
Result of extreme thinning of retinal tissue
Break is usually linear, flat break or horse shoe tear at posterior
border of lattice degeneration
Usually parallels ora serrate
Lesions occur in any meridian, more common in superior or inferior
Usually 2DD to full quadrant in size and to 1 DD in width
Fine white lines > attenuated and sheathed retinal vessels and
some RPE changes around it
Vitreous liquefies over lattice > strong vitreo-retinal adhesions
surrounding margins of retina > white halo on retinal surface
Small round holes in lesion
Quiescent and stationary for years without progressing to RD
Only lattice > annual DFE
Holes/no symptoms 6/12
If holes/symptoms 3/12
If tears > refer

Snail tracking degeneration

Same as lattice, but instead of fish bone appearance, has whitish trail instead
Vitreo-retinal tufts
Pathophysiolo
gy
Clinical
appearance
Complication
s

Retinal dialysis
Presentation

Location

Pathophysiolo

gy
Clinical

appearance
Tx

Accumulation of proliferated glial cells on retinal surface

Causes retinal degeneration and subsequent vitreous detachment
Greyish-white pieces of tissue at vitreo-retinal interface
Usually located between equator and ora
Often found nasally
RD/tears occur with vitreous liquification (syneresis)

Usually associated with trauma

Can occur spontaneously in younger px
Bilateral in inferior temporal quads
Mostly in superior nasal quadrants
Retinal tear that occurs near ora
Slow progressing RD
Refer all retinal dialysis
Needs proper Tx, especially if near posterior pole

Acquired retinoschisis
Def
Not true RD > only half way
Presentation
70% bilateral
Occurs with increasing age in 4-8% population
Usually in inferior temporal quadrant
Pathophysiolo
Splitting of sensory retina: outer plexiform and inner nuclear layers
gy
May develop inner layer breaks > clear and oval (light pink)
May develop outer layer holes/breaks > darker pink and larger >
increased risk for detachment
Sign
Absolute visual field defect
Types
Flat (typical)
Bullous (reticular)
Flat retinoschisis
Def
Represents advanced cystoid degeneration
Not associated with retinal holes
Bullous retinoschisis
Morph
Thin transparent ballooning forward of retinal tissue
Posterior may be serrated but typically no pigmented line
Pathophysiolo
Surface taut and doesnt move with EM
gy
Cavity in retinoschisis filled with hyaluronic acid (viscous > taut)
Presentation
Progression rare
11% with retinoschisis develop RD
Retinal detachment
Aet
Bond between sensory RPE and sensory retina is weak
Bond between sensory RPE and Bruchs is strong
Types
Non-rhegmatogenous RD
Rhegmatogenous
Signs of fresh
1. Relative APD (if extensive)
RD
2. IOP decrease by 5mmHg
3. Mild uveitis in anterior chamber
4. Posterior chamber retinal breaks (contrast in colour)
5. Can appear convex, opaque, corrugated and mobile undulates
Signs of old
1. Retinal thinning
RD
2. Secondary retinal cysts
3. Subretinal demarcation lines (high water marks)
4. Limiting lines of RD
5. Choroidal adhesions
Symptoms
1. Flashing lights
2. Floaters
3. Later on > field defects
Tx
1. Argon laser
2. Cryotherapy (liquid nitrogen)
3. Scleral buckle

Retinal break
RD
PVD

Retinoschisis
Choroidal
detachment
Vitreoretinal
traction

Rhegmatogenous RD
50% caused by tears associated with vitreal traction
30% caused by lattice
20% caused by dialysis or holes
Occur in 8-12 phakics perr 10 000
Aphakes, 2-5% develop RD > 50% o these within/after 1yr of post
surgery
Non-rhegmatogenous RD
Less common and still connected to rest of retina
No loose end to retinal break
Sign of space occupying lesion (tumour)
Tractional and exudatie
Sensory retinal pulled away from RPE by contracting vitreoretinal
membranes
Associated with diabetic retinopathy
Penetrating ocular trauma leading to fibroblastic proliferation of
vitreous > traction on retina
Caused by sub-retinal DO which damage RPE > allow fluid derived
from choroid into sub-retinal space
Aetiologies:
a. Choroidal tumours
b. Inflammation (posterior scleritis)
c. Following RD surgery
d. Following PRP or proliferative retinopathies
Definitions
Full thickness defect in sensory retina
Separation of sensory retina from RPE by subretinal fluid
Separation of cortical vitreous from ILM of sensory retina posterior
to vitreous base
Splitting of sensory retina into 2 layers
Effusion of fluid into suprachoroidal space

Force exerted on retina by structures originating in vvitreous

Aet

Epidemiology

Def

Forms
Tractional

Exudative

Other retinal degenerations Pigmentary degenerations