Chronic Visual

Disturbance and Visual

Loss
Setareh Ziai
April 2nd, 2009
sziai@ottawahospital.on.ca

QUICK review

Basic Anatomy

Where is the problem?

LMCC Objectives

Pre-retinal:

cornea (dystrophy, scarring, edema)

lens (age-related, traumatic, steroid-induced)

glaucoma
Retinal:

DM (diabetic retinopathy, macular edema)

vascular insufficiency (arterial or venous occlusion)

tumours

macular degeneration
Post-retinal:

anterior to optic chiasm (if optic nerve = monocular)

compressive optic neuropathy (intracranial masses, thyroid eye disease)
toxic/nutritional (nutritional deficiencies, alcohol/tobacco amblyopia)

optic chiasm lesions (pituitary adenoma)

Where is the problem?

Pre-retinal:

cornea (dystrophy, scarring, edema)

lens (age-related, traumatic, steroid-induced)

glaucoma
Retinal:

DM (diabetic retinopathy, macular edema)

vascular insufficiency (arterial or venous occlusion)

tumours

macular degeneration
Post-retinal:

anterior to optic chiasm (if optic nerve = monocular)

compressive optic neuropathy (intracranial masses, thyroid eye disease)
toxic/nutritional (nutritional deficiencies, alcohol/tobacco amblyopia)

optic chiasm lesions (pituitary adenoma)

Diagnosis based on:

- focused ophthalmological history
-

exam: start with gross examination

-

monocular vs. binocular

acute vs. chronic
painful vs. painless
VA
slit lamp biomicroscopy +/- fluorescein
dilated fundus examination

VF testing
fluorescein angiography +/- other tests

 **Remember

for exam:

sometimes, chronic visual loss in ONE eye is

noted incidentally some time later due to
occlusion of normal eye: CHRONIC LOSS
OF VISION CAN PRESENT ACUTELY!!

Corneal Causes
-

dystrophy
- scarring
- edema

The Cornea
- allows light to enter the
eye
- provides most of the eyes
optical power

- 0.5-0.8 mm thick
- transparent due to its
uniformity, avascularity
and deturgescence

Epithelium

Stroma

Endothelium

Corneal Dystrophies
-

rare inherited disorders

progressive, usually bilateral

can affect any of the three layers of the

cornea

affect transparency

age at presentation: first to fourth decades

Corneal Dystrophies
-

divided into:
-

anterior dystrophies:
- epithelium
- may present with recurrent corneal erosions

stromal dystrophies:
- usually present with visual loss

- if very anterior, can cause erosions and pain

-

posterior dystrophies:
- endothelium
- vision loss secondary to edema (endothelial dysfx)

Corneal Scarring
-

multiple causes:
-

trauma

infectious (eg., herpes)

post-surgical

Corneal Edema
-

most often caused by dysfunction of the

corneal endothelium:
-

dystrophy

trauma

infectious (eg., herpes)

post-surgical

Corneal
Transplantation

If the corneal stroma opacifies due to

trauma or infection, or if there is
swelling or an irregularity of the
surface of the cornea, light cannot
properly reach the retina.

In some cases, a cornea from a

deceased donor can be transplanted.

Corneal Transplantation

Lens-Related Causes
(cataract)
age-related
- traumatic
- steroid induced
-

The Lens

- biconvex, avascular,
transparent structure
- sits inside a thin
capsule, attached to the
ciliary body by the
zonules
- provides the
remainder of the eyes
optical power (along
with the cornea)

Lens

 cataracts

are due to the opacification of

this normally clear structure

Age-Related Cataract
-

often affect the nucleus of the lens first:

-

yellowing, followed by a browning of the lens

eventually, liquefaction

causes myopic changes (increased

refractive index of the lens)

Traumatic Cataract
-

most common cause of unilateral cataract

in young individuals

most often caused by direct penetrating

injury to the lens

can also be caused by:

-

concussion

ionizing radiation to ocular tumours

infrared radiation (glassblowers)

Steroid-Induced Cataract
-

both systemic and topical steroids can be

the culprits

posterior part of lens affected first

children may be more susceptible

if lens changes develop, dose should be

reduced to the minimum necessary

early opacities may regress with

discontinuation of therapy

Glaucoma

Glaucoma
disease

of the optic nerve, often

caused by an increase in intraocular
pressure due to poor drainage of
aqueous from the trabecular
meshwork

Glaucoma
if

left untreated, glaucoma can lead to

permanent damage to the optic nerve
and resultant visual field loss
can progress to blindness

Glaucoma
by

definition, glaucoma is a trimodal

disease, characterized by:

increased IOP
optic nerve changes
visual field changes

Goldmann Applanation Tonometer

Glaucoma
classification:

primary: open-angle, angle-closure

secondary: inflammatory, traumatic,
neovascular, steroid-induced etc
congenital

Risk Factors for Glaucoma

age
african-american
high

IOP
family history
myopia

heritage

Symptoms of Glaucoma
often

asymptomatic
with late disease, constriction of
peripheral, and later central visual field
with very high IOP, can have blurry
vision and halos around lights

Glaucoma: Optic Nerve

Changes
increased

cup:disc ratio
thinning of neural rim
progressive loss of nerve fiber layer
flame hemorrhages on disc

Primary Open Angle

Glaucoma
most

common (90%)
usually bilateral (can be asymmetric)
prevalence increases with age
angle is open, eye is quiet
increased resistance to aqueous drainage
at the level of the trabecular meshwork is
thought to be the main pathophysiologic
feature

Treatment options
goal

is to stabilize the IOP to protect

the optic nerve against further damage
options:

drops
laser
surgery

Glaucoma - Medications
mechanism

of action:

decrease aqueous production:

beta blockers: timolol
alpha agonists: brimonidine
carbonic anhydrase inhibitors: diamox

increase aqueous outflow:

miotics: pilocarpine
epinephrine
prostaglandin analogs: latanoprost

Glaucoma - Lasers
usually

when medical management

fails

ALT (argon laser trabeculoplasty), SLT

(selective laser trabeculoplasty): for open
angle glaucomas
peripheral iridotomy: for angle-closure
glaucomas

high

success rate

Glaucoma - Surgery
usually

when medical management and

laser treatments fail

trabeculectomy: sub-conjunctival shunt of

aqueous
drainage devices (valves)
cyclodestruction: last resort destruction
of ciliary body

Where is the problem?

Pre-retinal:

cornea (dystrophy, scarring, edema)

lens (age-related, traumatic, steroid-induced)

glaucoma
Retinal:

DM (diabetic retinopathy, macular edema)

vascular insufficiency (arterial or venous occlusion)

tumours

macular degeneration
Post-retinal:

anterior to optic chiasm (if optic nerve = monocular)

compressive optic neuropathy (intracranial masses, thyroid eye disease)
toxic/nutritional (nutritional deficiencies, alcohol/tobacco amblyopia)

optic chiasm lesions (pituitary adenoma)

THE RETINA
- neural tissue lining
the inside of the eye
- converts the visual
image into a
neurochemical
message and sends it
to the brain
- is made up of 10
anatomic layers

Diabetes
diabetic retinopathy
- diabetic macular edema
-

Diabetic Retinopathy
microangiopathy
affects pre-capillary arterioles, capillaries
and post-capillary venules
features of:
microvascular occlusion
leakage

clinically, can be divided into:

background DR (nonproliferative)
preproliferative DR
proliferative DR

Diabetic Retinopathy: Epidemiology

239 million people by 2010
doubling in prevalence since 1994
diabetes will affect:
28 million in western Europe
18.9 million in North America
138.2 million in Asia
1.3 million in Australasia

#1 cause of blindness in patients 20-64 yrs

prevalence increases with duration of diabetes and

patient age
rare to find DR in children < 10 yrs, regardless of duration
risk of developing DR increases after puberty

Epidemiology
Wisconsin Epidemiologic Study of Diabetic Retinopathy
Between 1979-1980
1210 patients with Type 1
1780 patients with Type 2
predominantly white population

After 20 yrs, DR present in:

99% of Type 1
60% of Type 2

WESDR: Frequency of retinopathy in

subjects with type 1diabetes

WESDR: Frequency of retinopathy in

subjects with type 2 diabetes

Diabetic Retinopathy: Risk Factors

duration of diabetes: most important risk
factor
poor metabolic control
pregnancy: can be associated with rapid
progression
HTN
nephropathy
smoking
obesity
hyperlipidemia

Classification of Diabetic Retinopathy

Classified into 2 stages

Nonproliferative Diabetic Retinopathy (NPDR)

early stage
also known as background DR (BDR)
further categorized based upon extent of DR
mild, moderate, severe, very severe

Proliferative Diabetic Retinopathy (PDR)

more advanced stage

***Macular edema
May be present at any stage of DR

NPDR
typically asymptomatic
fluctuating visual acuity:
fluctuating blood sugar
decreased visual acuity:
CSME
macular ischemia

review these patients annually

Mild NPDR

Moderate NPDR

Severe NPDR

Proliferative Diabetic Retinopathy

more likely to become
symptomatic than early
NPDR
may have decreased
vision, sudden vision loss,
floaters, cobwebs, flashes,
dull eye ache

PDR can also affect visual

function by affecting the
macula with resulting
macular ischemia and/or
edema

Proliferative DR
affects 5-10% of the diabetic population
neovascularization is the hallmark
NVD: neovascularization of the disc
NVE: neovascularization elsewhere
new vessels are not only extremely fragile
(intraretinal or vitreous hemorrhage), but
often associated with fibrous proliferation,
leading to an increased risk of tractional retinal
detachment

Advanced PDR

Tractional retinal
detachment
resulting from
contraction of
the fibrovascular
proliferative
tissue on the
retina

Panretinal Photocoagulation for High-risk

PDR

goal is to induce
involution (or at
least arrest) of new
vessels by creating
areas of retinal
ischemia
1200-3000 burns
4 sessions

Vitrectomy for Vitreous

Hemorrhage / TRD

Diabetic Macular Edema (DME)

retinal edema threatening or involving the
macula
diagnosis is made by slit-lamp exam,
confirmed by fluorescein angiography and/or
OCT

important observations include:

location of retinal thickening relative to the
fovea
presence and location of exudates

DME and CSME

Treatment of CSME

argon laser application

intravitreal steroid injection

intravitreal anti-VEGF injection

pars plana vitrectomy

Ophthalmological Follow-Up

Diabetic Screening

Type 1 diabetics:
Dilated funduscopic exam (DFE) 5 yrs after diagnosis
Newly diagnosed patients with Type 1 diabetes rarely
have retinopathy during the first 5 yrs

Type 2 diabetics:
Type 2 diabetics typically diagnosed yrs after initial
onset
DFE at the time of diagnosis
Significant portion of newly diagnosed Type 2
diabetics have established DR at the time of
diagnosis

Vascular Insufficiency
arterial occlusions (CRAO, BRAO)
- venous occlusions (CRVO, BRVO)
-

CRAO

CRAO
most

of the retina is supplied by the

central retinal artery (branch of the
ophthalmic artery, which is the first branch
of the ICA)
if this supply is interrupted (embolus,
thrombosis, inflammation, vasculitis or
compression), the retina becomes
ischemic
irreversible damage occurs after
approximately 90 minutes

CRAO
presentation is with sudden and
profound loss of vision
RAPD is present
orange reflex from the choroid stands
out at the fovea, and contrasts with the
surrounding pale retina (cherry-red
spot)
must r/o temporal arteritis

CRAO
most commonly the result of
atherosclerosis (thrombosis) but may
also be caused by calcific emboli
often in older patients, with a hx of
arteriosclerosis
may have had a hx of amaurosis fugax
(transient visual loss)

CRAO
OPHTHALMOLOGIC EMERGENCY!!
treatment:
decrease IOP
paracentesis
ocular massage
goal: to send the embolus distally
**remember to r/o giant cell arteritis! (ESR, CRP, plt)
poor prognosis: 60% 20/400

BRAO

BRAO
sudden and profound altitudinal or
sectoral visual field loss
similar causes as CRAO
identify and treat associated medical
conditions (HTN, DM,
hypercholesterolemia, smoking,
vasculitis etc)

BRAO
retinal cloudiness in ischemic area
+/- visible embolus
also has a poor prognosis, unless the
obstruction can be dislodged within a
few hours

CRVO

CRVO
thrombosis

of the central retinal vein

sudden loss of vision in affected eye
severity of symptoms varies
non-ischemic: 75%
Ischemic

most characteristic finding: retinal

hemorrhages

CRVO
underlying associations
advancing age
systemic conditions: HTN, DM, smoking,
obesity, hyperlipidemia
glaucoma
inflammatory diseases: sarcoidosis, Behcet
disease
thrombophilic disorders:
hyperhomocysteinaemia, antiphospholipid
antibody syndrome

CRVO
Treatment:
treat associated medical conditions
decrease IOP if elevated
pan-retinal photocoagulation
(laser) if:
neovascularization (iris, angle,
retina)
especially if ischemic CRVO

BRVO

BRVO
thrombosis

of a branch of the central

retinal vein
visual loss depends on the amount of
macular drainage compromised by the
occlusion (peripheral occlusions may be
asymptomatic)
characteristic findings in one sector of the
retina:
dilatation and tortuosity of veins
retinal hemorrhages
retinal/macular edema

BRVO
obstruction often at arterio-venous
crossings: arteries and veins share
adventitial sheath thickening of the
arteriole (arteriosclerosis) compresses the
vein, eventually causing an occlusion
often associated with:
hypertension (75%)
diabetes (10%)

BRVO
prognosis: depends on amt of venous
drainage involved by the occlusion and
severity of macular ischemia: within 6 mos,
about 50% of eyes have a VA of 20/30 or
better
main complications:
chronic macular edema
neovascularization

laser photocoagulation may be helpful in

above cases

Retinal Tumours

 ocular

tumours:

ciliary body:
melanoma

choroid:
melanoma
hemangioma
metastases

primary ocular lymphoma

retina and optic nerve:
retinoblastoma
astrocytoma
hemangioma

Choroidal Melanoma
most

common primary intraocular

tumour in adults
presentation usually in 6th decade:

asymptomatic vs. visual field defect and/or

decreased visual acuity

signs:
raised, usually pigmented lesion visible at
the back of the eye
may be associated with retinal detachment
optic nerve may be involved

Choroidal Melanoma
treatment:

consider size, location, activity of tumour,

state of fellow eye, general health/age of pt,
pts wishes/fears
brachytherapy
external radiotherapy
transpupillary thermotherapy
local resection
enucleation
exenteration
palliative (may include chemo)

Choroidal Metastases
with

choroidal melanoma, dont forget

general medical investigations!

mets TO the choroid:

most frequently from bronchus in both sexes
and the breast in women, rarely kidney or GI

CXR, rectal exam, mammography

mets FROM the choroid:

liver

hepatic u/s, GGT, ALP

lungs (rarely affected before liver)

CXR

Choroidal Metastases
usually

present with visual impairment

only IF tumour is near the macula
signs:

fast-growing, creamy coloured lesion

most often in posterior pole
usually not very elevated (infiltrates laterally)

Choroidal Metastases
treatment:

observe: if asxic or receiving systemic chemo

radiation: external beam or brachy
transpupillary thermotherapy
systemic therapy for the primary
enucleation: for painful blind eye

prognosis

is poor

median survival: 8-12 mos for all pts, 15-17

mos for those with breast ca

Retinoblastoma
most

common malignant tumour of the eye

in childhood (1:20 000)
mean age of presentation: 8 mos if
inherited, 25 mos if sporadic

60% present with leukocoria (white pupillary

reflex)
strabismus (20%)
occasionally: painful, red eye
if inherited: often bilateral

Retinoblastoma
malignant

transformation of primitive
retinal cells before their final differentiation

can

be caused by germinal mutations (can

be passed on to the next generation), or
can be sporadic (66% of cases)

Retinoblastoma
this

is a clinical diagnosis, but CSF and

bone marrow should be examined to check
for metastatic disease if ON involved or if
there is evidence of extraocular extension
rx:

small: cryotherapy, photocoagulation

medium: brachytherapy, external beam, chemo
large/advanced cases: chemoreduction + local
treatment, enucleation
metastatic disease: chemo (intrathecal if cells
in CSF)

Retinoblastoma
prognosis:

depends on extent of disease at diagnosis

overall mortality ~ 5-15%
~ 50% of children with the germinal mutation
will eventually develop a second primary
tumour (eg., osteosarcoma of the femur or
pinealoblastoma)

Macular Degeneration

Macula
1.5

mm in diameter
central vision: BEST VISUAL ACUITY
colour vision
progressive destruction of the macular
area:

MACULAR DEGENERATION

Macular Degeneration
most

common cause of irreversible

visual loss in the developed world
exists in two forms:

non-exudative (dry) macular

degeneration
exudative (wet) macular
degeneration

Non-exudative Macular
Degeneration
lipid

products arising from

photoreceptor outer segments are
found under retina

can be seen with ophthalmoscope!

called drusen

Exudative Macular
Degeneration
new

vessels from the choroid grow into

the sub-retinal space; form a subretinal neovascular membrane

subsequent

hemorrhage into the subretinal space or even through the retina

into the vitreous is associated with
profound loss of vision

Macular Degeneration
symptoms:

since fovea is responsible for fine visual

resolution, any disruption will cause
severe visual impairment
blurry/reduced vision
distorted vision (metamorphopsia)
reduction (micropsia) or enlargement
(macropsia) of objects
VF loss (scotoma)

Macular Degeneration
rx:

non-exudative (usually slowly

progressive):
no actual medical treatment
use low vision aids
high dose antioxidants MAY be
beneficial (eg., vitalux)

Macular Degeneration
rx:

exudative (can be rapidly progressive and

devastating):
intravitreal injections of anti-VEGF
factors: bevacizumab, ranibizumab
photodynamic therapy (injection of
photosensitizer into systemic circulation
followed immediately by laser targeting
new vessels in macular area)
combination of above treatments

Where is the problem?

Pre-retinal:

cornea (dystrophy, scarring, edema)

lens (age-related, traumatic, steroid-induced)

glaucoma
Retinal:

DM (diabetic retinopathy, macular edema)

vascular insufficiency (arterial or venous occlusion)

tumours

macular degeneration
Post-retinal:

anterior to optic chiasm (if optic nerve = monocular)

compressive optic neuropathy (intracranial masses, thyroid eye disease)
toxic/nutritional (nutritional deficiencies, alcohol/tobacco amblyopia)

optic chiasm lesions (pituitary adenoma)

OPTIC NERVE
1.2

million cells

80 % visual fibres
20 % pupillary fibres
carries

visual
information from
the eye to the brain

OPTIC CHIASM
crossover
above

of nasal fibers

the pituitary

internal

carotids are just

lateral
from

optic chiasm:

optic

tract to the
lateral geniculate body
optic

radiation to the
primary visual cortex

Anterior to Optic
Chiasm
compressive optic neuropathies
- toxic/nutritional optic neuropathies
-

Compressive Optic
Neuropathies
INTRACRANIAL

MASSES:

optic nerve glioma

typically affects young women, end of first decade
associated with NF-1

optic nerve sheath meningioma

most frequent in middle-aged women
unilateral, gradual visual impairment

any other orbital or chiasmal tumour

compressing any part of the optic nerve

THYROID

EYE DISEASE

Thyroid Eye Disease

may

occur in the absence of biochemical

evidence of thyroid dysfx
autoimmune reaction (IgG Abs) causing:

inflammation of EOMs: pleiomorphic cellular

infiltration associated with increased secretion
of GAGs and osmotic imbibition of water
muscles can become up to 8 times their original
size!!

no

relation to severity of thyroid dysfx!

Thyroid Eye Disease

main

findings: (not all are always present!)

soft tissue involvement

lid retraction
proptosis
optic neuropathy
restrictive myopathy

Thyroid Eye Disease

vision

loss from:
exposure keratopathy
due to severe proptosis resulting in incomplete lid
closure chronically exposed cornea corneal
ulceration & exposure keratopathy

optic neuropathy
affects 5% of pts
compression of ON or its blood supply by
congested (enlarged) EOMs
can lead to severe, permanent visual impairment
rx with steroids, surgery if needed

Toxic/Nutritional Optic
Neuropathies
nutritional

deficiencies
alcohol-tobacco amblyopia

Nutritional Deficiencies

pts with extremely poor diets, often in association

with alcohol-tobacco amblyopia
usually due to B12 deficiency in combination with
cyanide toxicity
symmetrical VF loss
if early, can be treated with high-dose vitamins
and restoration of well-balanced diet
eventually leads to optic atrophy and permanent
vision loss

Alcohol-Tobacco Amblyopia

affects heavy drinkers, cigar and pipe smokers: deficient in

protein and the B vitamins
symptoms: insidious, bilateral, progressive visual
impairment + dyschromatopsia
signs: symmetrical VF defect, may have pale (or normal)
discs
rx: 1000 units of hydroxocobalamin qweekly X 10 wks +
multivitamins + well-balanced diet
px:
good in early cases if comply with rx
advanced cases: optic atrophy and permanent visual
loss

Optic Chiasm Lesions

-

pituitary adenoma

Pituitary Adenoma
presentation

usually in early adult life or

middle age
symptoms:

h/a
visual symptoms: very gradual onset (often
not noticed by pt until very well-established)
VF defect: usually, bitemporal hemianopia, worst in
the superior field, and extending inferiorly
colour desaturation across vertical midline
optic atrophy: in 50% of cases with field defects
caused by pituitary lesions

Pituitary Adenoma
investigations:

MRI: coronal, axial and sagittal sections before

and after gadolinium injection
CT: demonstrates enlargement or erosion of
the sella
endocrinological investigation: PRL, FSH, TSH,
GH

Pituitary Adenoma
treatment

options:

observation
medical: dopamine agonists (bromocriptine)
surgery
radiotherapy: often used as an adjunct
gamma knife stereotactic radiotherapy

Visual Field Defects

Merci