Centre for Eye Health/UNSW

Overall topics to be covered

 Review the visual pathways and the associated
characteristics of visual field loss
Clinical nuggets – visual pathway  Application of key principles in clinical practice
– Visual fields, optic nerve head, RNFL, Ganglion Cell Analysis
assessment • Clinical challenges
Professor Michael Kalloniatis • Optic atrophies
Director, Centre for Eye Health • Post-chiasmal lesions
School of Optometry and Vision Science – (may include retrograde degeneration at the ON/RNFL level)
University of New South Wales
 Five participant polls #1-5 during the lecture
Polls are anonymous, please participate
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Postretinal Pathways - the significance

Postretinal Pathways of fibre crossing at the chiasm

 Visual pathway Partial f

decussation
N
at chiasm
f

More crossed than

uncrossed pupil fibers

Left eye Right eye

f f

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https://cim.ucdmc.ucdavis.edu/eyerelease/interface/topframe.htm
Review ganglion cell axon paths to optic nerve

Anatomical
assessment of retinal
ganglion cells
• Neuroretinal rim
• Retinal nerve fiber layer
(RNFL) using OCT
• Ganglion Cell Analysis
UT (GCA) using OCT
UN
M
LN
LT

Macular disease

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Review ganglion cell axon paths to optic nerve Review ganglion cell axon paths to optic nerve

Close to chiasm
Vertical Raphe
Cross

UT UN

M
UT UT
UN UN LT LN
Horizontal Raphe M M
LN LN
LT LT

Altitudinal loss
Arcuate loss Post-chiasmal loss

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Pituitary macroadenoma Pituitary tumours: asymmetric VF change

Not hormonally active; No headaches; Mild pre-proliferative diabetic retinopathy;
Only subtle visual disturbance noticed; VA OD 6/6 OS 6/12 - 6/24 (gaze dependent)

An initiative of Guide Dogs NSW/ACT and The University of New South Wales Do not expect ‘textbook’ symmetry inHerse,
VFClinloss
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Exp Optom 2014
Herse, Clin Exp Optom 2014

Temporal lobe Temporal lobe

 Tumors, iatrogenically induced, AV malformations
 Meyer’s loop involvement produce superior, incongruous,  Tumors, iatrogenically induced, AV malformations
homonymous defects  Meyer’s loop involvement produce superior, incongruous,
– “Pie in the sky” homonymous defects
– “Pie in the sky”
 Nonspecific homonymous hemianopia
 Temporal lobe seizure activity, including olfactory and formed
visual hallucinations
 Dominant hemisphere cause language disturbance
 Depending upon size of lesion, peripheral motor dysfunction

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Parietal lobe
OS OD
 Superior fibers involved first hence
– “Pie on the floor”
 Usually vascular lesions
 Associated neuro-ophthalmic changes
– Agnosias
– Apraxia http://www.ukoptometry.co.uk
– Dominant hemisphere
• Gerstmann syndrome: acalculia, agraphia, finger agnosia, and left-right confusion
– OKN nystagmus inability to side of lesion (if damage near visual radiations)
– Conjugate movements of the eyes to the side opposite the lesion on forced
lid closure
– Inattention (nondominant parietal lobe lesions)

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Horton & Hoyt, Arch Ophthal. 109:861, 1991

Lesions to selective brain regions cause

specific functional defects

REM: VF loss with

lesions here
Cortical damage and
visual field loss
REM: VF would be bilateral
except temporal crescent
Color

Facial recognition
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Facial expresion
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Right eye Left eye

or retina Key Points #1

RAPD
 Key questions to ask yourself
– Does the retinal and optic nerve look normal?
– Is there a RAPD?
– Is vision anomaly monocular or binocular?
– Are there other associated signs/symptoms?
– Is the vertical or horizontal midline in visual fields followed?
– Does the patient display anomalies consistent with higher
visual areas?
– Are flashing lights achromatic or chromatic?

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Poll #1 - Patient #13: 77 yo male with a history of stroke (15 yrs

ago). Visual field stable over a ~13 yr period (left partial
quadratanopia)

Applying these principles in

clinical practice

Clinical challenges

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Poll #1 - Patient #13: 77 yo male with a history of stroke (15 yrs Poll #1 - Patient #13: 77 yo male with a history of stroke (15 yrs
ago). Visual field stable over a ~13 yr period (left partial ago). Visual field stable over a ~13 yr period (left partial
quadratanopia) quadratanopia)
Poll #1: Which of the following is most correct relating Poll #1: Which of the following is most correct relating to the superior
to the superior quadratic visual field loss? quadratic visual field loss?

a. The visual field loss is largely congruous a. The visual field loss is largely congruous
b. The lesion is most likely on the left side of cerebral cortex (expect right sided lesion)
b. The lesion is most likely on the left side of cerebral cortex
c. The lesion is most likely at the chiasm (not bi temporal)
c. The lesion is most likely at the chiasm
d. A left RAPD will likely be present (not a complete cut and thus congruous nature of
d. A left RAPD will likely be present quadrantanopia suggests post LGN)

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Poll #1: A 77 yo male with a history of stroke (15 yrs ago). Visual Poll #2: 33 yo female; family history of glaucoma:
field stable over a ~13 yr period (left partial quadratanopia) IOPs 14mm Hg OU, AC quiet, normal CCT
Look carefully at the optic nerve heads

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Poll #2: 33 yo female; family history of glaucoma: Poll #2: 33 yo female; family history of glaucoma:
IOPs 14mm Hg OU, AC quiet, normal CCT IOPs 14mm Hg OU, AC quiet, normal CCT
Look carefully at the optic nerve heads Look carefully at the optic nerve heads

Poll #2: Which of the following is incorrect? Poll #2: Which of the following is incorrect?
a. The right optic nerve heads appear slightly larger compared to the left a. The right optic nerve heads appear slightly larger compared to the left (true)
b. Artery:Vein ratio is within normal limits b. Artery:Vein ratio is within normal limits (true)
c. The notch is strongly suggestive of optic neuropathy c. The notch is strongly suggestive of optic neuropathy
d. The neuroretinal rim appears
An initiative of Guide healthy
Dogs NSW/ACT and Thein both ofoptic
University nerve
New South Walesheads d. The neuroretinal
An initiative rim appears
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Dogs NSW/ACT both opticofnerve
University heads
New South Wales(true)

Poll #2: 33 yo female; family history of glaucoma: The challenge #2: 33 yo female; family history of glaucoma:
IOPs 14mm Hg OU, AC quiet, normal CCT IOPs 14mm Hg OU, AC quiet, normal CCT

Split bundle
(OU)

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Optic atrophy (OA)

 Optic atrophy
– Morphologic description of the endpoint of any disease that

Applying these principles in causes RGC axon degeneration

• Neuroretinal rim atrophy or pale appearance of ONH

clinical practice  Categories

1. Primary OA
2. Secondary OA
Optic atrophy 3. Consecutive OA
4. Glaucoma
5. Retrograde degeneration OA

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Visual field loss secondary

1. Primary Optic atrophy (OA) to optic neuritis
 Primary optic atrophy: occurs without ONH swelling
preceding the atrophy
– Compressive
– Retro-bulbar neuritis (optic neuritis)
– Hereditary optic neuropathy
– Toxic & nutritional neuropathies
– ONH drusen
– Trauma

Savino & Danesh-Meyer 2001 (Neuro-ophthalmology)

Wills Eye Hospital Atlas of Clinical Ophthalmology

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From: Visual Field Profile of Optic Neuritis: A Final Follow-up Report From the Optic Neuritis
Treatment Trial From Baseline Through 15 Years

Arch Ophthalmol. 2010;128(3):330-337.

2. Secondary Optic Atrophy (OA)
 Secondary optic atrophy: occurs secondary to long-standing
swelling of the ONH
– Chronic papilloedema
– Anterior ischaemic optic neuropathy (AION)
– Papillitis (most common form of optic neuritis in children but also present in
adults)

• No ONH fundus autofluorescence (exclude ONH drusen)

• Critical to differentiate this from other causes of ONH crowding
– See Chiang et al Clin Exp Optom 2015
Table Title:
Frequency Distribution of Visual Field Classifications in the Fellow Eyea

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71 yo male with long-standing VF loss in OD (noticed after

cardiac surgery 35 yrs ago). VA 6/12 OD, 6/75 OS. Classic VF loss in AION
RAPD OD

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3. Consecutive Optic Atrophy (OA)

 Consecutive optic atrophy: occurs due to diseases of the
inner retina or retinal blood supply
– Retinitis pigmentosa (rod-cone) or cone-rod dystrophies
– Vasculitis
– Retinal necrosis/neuroretinitis
– Excessive photocoagulation
– Vascular (arterial or venous occlusive disease)

Longstanding AION (OD): if bilateral and no RAPD,

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consider bilateral cortical lesions

53 yo female first Dx with RP 26 yrs ago. LP OU; small temporal

islands detected with Goldmann; ERG no core or rod responses; Glaucoma
parents 1st cousins
 Most common optic nerve disease?
 An optic neuropathy
– chronic destruction of ganglion cells
– characteristic atrophy (cupping)
 Functional loss
– Typical “distinctive” visual field
defects involving the nerve fiber bundles

Advanced RP OU
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Cirrus OCT – Optic atrophy (glaucoma) Glaucoma:

VF loss

Note optic disc OCT results

cupping

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Poll #3: Identify the most correct response.

Poll #3: Describe the visual field loss in this 62 yo male Poll
a. #3: Describe
The patient likelythe
hasvisual field
LE optic loss in this
neuropathy 62 yo visual
and central malefield loss
b. The patient likely has RE optic neuropathy and central visual field loss
c. The patient has unusable visual field results
d. A temporal lobe lesion at Meyer’s loop should be considered

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Poll #3: Identify the most correct response.

Poll
a. #3: Describe
The patient likelythe
hasvisual field
LE optic loss in this
neuropathy and62 yo male
central visual field loss Poll #3: Describe the visual field loss in this 62 yo male
b. The patient likely has RE optic neuropathy and central visual field loss (the neuropathy is
affecting the LE)
c. The patient has unusable visual field results (can forgive fixation errors if central VF is affected)
d. A temporal lobe lesion at Meyer’s loop should be considered (would expect a pie in the sky)

1. Forgive fixation errors

2. Care in assessing pattern deviation vs grey scale

Possible ONH
changes

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Be aware of retrograde RNFL degeneration/ONH changes ± patent ONH disease

Key points #2
 May get optic atrophy without ONH swelling
– Compressive lesions, retrobulbar inflammation, retrograde degeneration,
trauma, toxicity, neutritional
 Long-term ONH swelling leads to axonal loss (secondary OA)
Applying these principles in
 Many retinal conditions lead to secondary neuronal loss and glial
remodelling (consecutive OA)
clinical practice
 Hereditary optic atrophy Dx of exclusion
 Optic neuritis is predominantly a binocular disease Post-chiasmal lesions
 Be aware of key characteristics of VF loss
– Arcuate; observance of vertical of horizontal midline
(retrograde degeneration of ON/RNFL)

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5. Post-chiasmal lesions Poll #4: 69 yo male; birth defect causing paralysis on right side;
had a TIA 8 yrs ago (? Stroke). IOPs 16mm Hg OU, AC quiet,
(may include retrograde degeneration ON/RNFL) normal CCT Describe the optic nerves?

 Retinal nerve fiber layer loss and associated ONH changed

due to RGC loss due to post-LGN lesions
(temporal/parietal/cortical)
 Retrograde (trans-synaptic) degeneration: relatively new concept
– The visualisation of retrograde trans-synaptic degeneration
secondary to stroke depends upon:
• Time post insult
• Brain location
• Size of insult & size of GCA imaging Jindahra et al 2012; Park et al 2013

– Retrograde degeneration secondary to Multiple Sclerosis (MS)

lesions post-LGN
Klistorner et al 2014; Huang-Link et al 2014

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Poll #4: 69 yo male; birth defect causing paralysis on right side; Poll #4: 69 yo male; birth defect causing paralysis on right side;
had a TIA 8 yrs ago (? Stroke). IOPs 16mm Hg OU, AC quiet, had a TIA 8 yrs ago (? Stroke). IOPs 16mm Hg OU, AC quiet,
normal CCT Describe the optic nerves? normal CCT Describe the optic nerves?

Poll #4: Which of the following is incorrect? Poll #4: Which of the following is incorrect?
a. The optic nerve heads appear slightly asymmetric in overall appearance (Left ONH
a. The optic nerve heads appear slightly asymmetric in overall appearance shows superior and inferotemporal anomalies)
b. Moderate beta zone atrophy exists OU b. Moderate beta zone atrophy exists OU (true – has both alpha & beta OU)
c. The LE has an abnormal neuroretinal rim infero-temporal and superiorly c. The LE has an abnormal neuroretinal rim infero-temporal and superiorly (true)
d. The neuroretinal
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An initiative of appears
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Dogs NSW/ACT and Thein both of
University optic nerve
New South Walesheads

Poll #4: 69 yo male; birth defect causing paralysis on right side; Poll #4: 69 yo male; birth defect causing paralysis on right side;
had a TIA 8 yrs ago (? Stroke). IOPs 16mm Hg OU, AC quiet, had a TIA 8 yrs ago (? Stroke). IOPs 16mm Hg OU, AC quiet,
normal CCT normal CCT

GCA - GCL+IPL

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Poll #5: 57 yo male;. IOPs 25mm Hg OU, AC quiet, 470um CCT; Poll #5: 57 yo male;. IOPs 25mm Hg OU, AC quiet, 470um CCT;
gonio open angles no secondary glaucoma. Slightly smaller than average gonio open angles no secondary glaucoma. Slightly smaller than average
ONH size ONH size

Poll #5: Which of the following is incorrect?

a. If corrected for CCT, the IOP would be higher
b. The left rim appears irregular and pale
c. The right optic nerve head has subtle thinning of neuroretinal rim
superiorly/superiortemporally
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d. The left optic nerve head is slightly smaller than the right

Poll #5: 57 yo male;. IOPs 25mm Hg OU, AC quiet, 470um CCT; Poll #5: 57 yo male;. IOPs 25mm Hg OU, AC quiet, 470um CCT;
gonio open angles no secondary glaucoma. Slightly smaller than average gonio open angles no secondary glaucoma
ONH size

Poll #5: Which of the following is incorrect?

a. If corrected for CCT, the IOP would be higher (CCT is well below normal thickness and true
IOP will be higher)
b. The left rim appears irregular and pale
c. The right optic nerve head has subtle thinning of neuroretinal rim
superiorly/superiortemporally (true)
d. An nerve
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(true) An initiative of Guide Dogs NSW/ACT and The University of New South Wales

Poll #5: 57 yo male;. IOPs 25mm Hg OU, AC quiet, 470um CCT;

gonio open angles no secondary glaucoma

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1. Congruous GCA change

2. GCA obey vertical midline
3. Visual discordance of VF/GCA with
RNFL analysis (anatomically correct)

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1. Incongruous GCA change

2. GCA disobey vertical midline
3. Largely concordance of VF/GCA with GCA and RNFL change
RNFL analysis will depend where in
the normative data
range the patient started
Retrograde
degeneration
– Time after lesion
– Size of lesion
– Location of lesion

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Right eye Left eye

or retina Key Points #3

 Carefully assess pupils (RAPD)
 Assess the symmetry in visual field defect and
ensure suitable testing is undertaken (central vs
peripheral)
 Interpret imaging results (RNFL and GCA) in
conjunction with visual fields
 Consider retrograde degeneration (trans-synaptic
degeneration)
 In diagnosing glaucoma - has there been progression?

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Thank you for your attention

End of lecture

12
Right eye Left eye

or retina

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