+

Bala
Notes

for

Ophthalmology
Post Graduate
Examination Fundus

C Balasubramaniam
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 Bala Notes

for

Ophthalmology Post Graduate Examination

Fundus

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iii
 Bala Notes

for

Ophthalmology Post Graduate Examination

Fundus

C Balasubramaniam
MS DNB FRCS (Edinburgh, UK)

Medical Director

Acchutha Eye Care

Erode, Tamil Nadu, India

Chairman

Acchutha Institute of Optometry

Erode, Tamil Nadu, India

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Preface

Following the dire need for an exam oriented book, this 'Notes' was prepared
initially, in 1998, during my ophthalmology post graduation at Regional
Institute of Ophthalmology & Government Ophthalmic Hospital (RIO
GOH), Chennai, to help the post graduates approach the university practical
exams confidently. It was basically a compilation of commonly asked
questions by teachers at RIOGOH during the practical exams.

For the past two decades, this 'Notes’, in its informal & hand written form,
has been used by ophthalmology post graduates for their exams. This book is
an attempt to update the 'Notes', without any change in the original format of
'questions and answers', for the benefit of current and future ophthalmology
post graduates.

Although the examination format keeps changing, I hope, as before, this

'Notes' would still continue to be useful to the postgraduates for their exams.

Please be free to give your feedback, suggestions & corrections if any.

Email: balaophthalnotes@[Link]

C Balasubramaniam

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Acknowledgements

My Teachers and Post Graduate Colleagues (1998 batch)

at

Regional Institute of Ophthalmology &

Government Ophthalmic Hospital,

Chennai, Tamil Nadu, India

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Contents

1. Diabetic Retinopathy

2. Hypertensive Retinopathy

3. Retinal Vein Occlusion

4. Retinal Artery Occlusion

5. Retinal Detachment

6. Central Serous Retinopathy

7. Macular Hole

8. Retinitis Pigmentosa

9. Macular Dystrophy

10. Age Related Macular Degeneration

11. Optic Atrophy

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Diabetic Retinopathy

Diabetic Retinopathy

• What type of DR is more common in Type 1 DM?

Proliferative DR

• What type of DR is more common in Type 2 DM?

Diabetic maculopathy

• What is ETDRS Classification of DR?

Non-proliferative DR
Mild NPDR at least one micro aneurysm
Moderate NPDR severe retinal hemorrhages in at least one quadrant or CWS, venous beading
or IRMA definitely present
Severe NPDR Severe retinal Haemorrhages in 4 quadrants; or venous Beading in 2
Quadrants; or extensive IRMA in 1 quadrant ([Link] rule – Remember HBI)
Very severe NPDR any 2 of the features of severe NPDR

Proliferative DR
High risk

Diabetic maculopathy
CSME

• How was DR classified before ETDRS?

Background
Pre-proliferative
Proliferative
Advanced Diabetic Eye Disease
Diabetic Maculopathy

• What are the clinical findings in BDR?

1. Microaneurysm
2. Dot and blot haemorrhage
3. Hard exudates
4. Retinal edema

• Why is it called Background DR?

Lesions lie within the outer retina and form a ‘background’ to lesions on the retinal surface such as
new vessels

• What is the earliest ophthalmoscopically detectable sign in DR?

Microaneurysm

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Diabetic Retinopathy

• How does microaneurysm occur in DR?

Diabetes – loss of pericytes in vessel wall – saccular out-pouching of capillary walls

• What are the causes of microaneurysms?

1. DR
2. BRVO
3. Retinal telangiectasia

• What are dot and blot haem?

Haem in the outer plexiform and inner nuclear layer – secondary to rupture of microaneurysm

• How to differentiate between microaneurysm and dot-blot haem?

Sometimes might be difficult clinically
FFA - hyper fluorescent spot – microaneurysm
Hypo fluorescent spot - dot-blot haem

• What are hard exudates?

Lipid-laden macrophages

• What are the types of hard exudates?

Circinate, Discrete, Confluent

• What is circinate pattern of Hard exudates?

Ring of hard exudates with a central leaking microaneurysm

• How to differentiate between hard and soft exudates?

Hard exudates Soft exudates (Cotton wool spots)
• white or yellowish- white with distinct • white and fluffy with ill-defined
margin margins
• deep in inner plexiform and inner nuclear • superficial in nerve fibre layer –
layers – does not obscure blood vessels obscures blood vessels
• Lipid laden macrophages • Micro infarcts /Stasis of axoplasmic
flow
• Can coalesce • Never coalesce

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Diabetic Retinopathy

What are the causes for hard exudates?

1. DR
2. Hypertension
3. Choroidal neovascular membrane
4. Retinal vein occlusion
5. Parafoveal telangiectasia
6. Macroaneurysm
7. Capillary haemangioma
8. Coat’s disease
9. Papilledema
10. Radiation retinopathy

• What are the clinical findings in Preproliferative DR?

1. Vascular changes – venous beading / looping / sausage-like segmentation / narrowing of
arterioles
2. Dark blotchy haemorrhage
3. Cotton wool spot
4. IRMA

• What is the pathophysiology of cotton-wool spot?

Occlusion of precapillary arterioles - nerve fibre layer infarcts - axoplasmic flow stasis

• Why is cotton-wool spot found only in the posterior pole?

Since nerve fibre layer is of appreciable thickness only in the posterior pole

• What are the causes for cotton-wool spot?

1. Hypertension
2. Retinal artery / arteriole occlusion
3. SLE
4. AIDS
5. Purscher’s retinopathy
6. Anaemia
7. Leukemia

• What is [Link] rule?

Used in Severe NPDR
Remember HBI
4 quadrants – severe Haemorrhage
2 quadrants – venous Beading
1 quadrant – IRMA

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Diabetic Retinopathy

• What are the types of retinal haemorrhage in DR?

1. Dot & blot
2. Dark blotchy
3. Flame-shaped (esp. if associated with hypertension)
4. Preretinal

• Which is more clinically important – dark blotchy haem or dot-blot haem?

Dark blotchy haem. – indicates retinal ischemia

• What is the most significant predictor of progression to PDR?

Venous loops and beading

• What is IRMA?
Intra Retinal Microvascular Abnormalities – remodeled capillary beds without proliferative changes

• What are the clinical findings in PDR?

1. New vessels on disc (NVD)
2. New vessels elsewhere (NVE)
3. Vitreous haemorrhage

• What is high-risk PDR as per Diabetic Retinopathy Study?

1. NV within one DD of disc and > 1/3 DD
2. Vitreous or preretinal haemorrhage associated with less extensive NVD or with NVE > 1/2
DD associated with preretinal haem

• What are the stages of neovascularisation?

1. Stage of naked vessels
2. Fibrous condensation around the vessel
3. Stage of cicatrisation

• How to differentiate between normal and new vessel?

Pattern – branching dichotomous in normal vessel / loops or retes in new vessel
Location – normal vessel never elevated into vitreous / new vessel can be elevated
Normal vessel supply or drain an area of the retina / new vessel no such role

• How to differentiate between IRMA and NVE?

IRMA NVE
Location Deeper retina Superficial retina
Crossing major blood Does not cross Crosses
vessel
FFA leak No or mild Florid

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Diabetic Retinopathy

• What is the term for any retinopathy with new vessel?

Proliferative retinopathy

• What are the causes for proliferative retinopathy (new vessel in retina)?

1. Diabetes
2. Ischemic CRVO
3. Ischemic BRVO
4. Sickle retinopathy
5. Vasculitis
6. ROP

• How to differentiate between DR and CRVO?

Diabetic Retinopathy CRVO

Onset Gradual Sudden
Laterality Usually bilateral Usually unilateral
Disc edema Absent Present
Veins Tortuosity Absent Present
Hard Exudates Usually present Usually absent
Haemorrhages Less prominent More prominent

• What to suspect if patient has DR BE, but asymmetry with extensive retinal haem. in one eye?
CRVO in that eye

• What to suspect if patient has DR BE, but asymmetry with minimal findings in one eye?
Carotid occlusion on the side of that eye

• How to differentiate between DR and Hypertensive Retinopathy?

Diabetic Retinopathy Hypertensive Retinopathy

Haemorrhages Dot-blot Flame shaped
Microaneurysm Common Uncommon
Retinal arterioles Not necessary except in Narrowed
preprolif.

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Diabetic Retinopathy

• How to differentiate between DR and Ocular Ischemic Syndrome?

Diabetic Retinopathy Ocular Ischemic Syndrome

Laterality Bilateral Unilateral
Haemorrhage Dot-blot Larger
Haemorrhage location Posterior pole Mid-periphery
Exudates Present Absent

• What are the indications for laser in DR?

1. CSME
2. PDR
3. Rubeosis / Angle new vessel

• What is the treatment for PDR?

Pan Retinal laser Photocoagulation (PRP)

• What is the other name for PRP?

Scatter photocoagulation

• What is the goal of laser treatment in PDR?

To cause regression of NV and reduce the risk of vision loss

• How does PRP help in treating PDR?

PRP converts hypoxic area to anoxic area / reduces oxygen demand – decrease in vasogenic factors
– regression of new vessels

• What is the result of Diabetic Retinopathy Study (DRS)?

PRP reduces the risk of ‘severe’ visual loss by 50%

• How is PRP done?

After informed consent
Laser burns 500 micron size / one burn apart / power adjusted to get grey-white burn/ macula
avoided / temporally done 2 disc diameters from the centre of the macula / nasal, superior and
inferior retinal periphery / number 2000-3000 in 2-3 sittings / avoid disc, major vessel, elevated NV
& area of tractional detachment

• Which part of retina should be done in the first sitting of PRP?

Inferior retina – since may not be visible if the patient develops vitreous haemorrhage

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Diabetic Retinopathy

• What laser delivery systems are used for PRP as outpatient procedure?
Slit-lamp
Indirect

• What are the advantages of indirect laser over slit-lamp delivery?

Can be used in
1. hazy media compared to slit-lamp delivery
2. patients who cannot be seated for slit-lamp

• How is the spot size varied during indirect laser?

By varying the distance of the focusing 20D lens from the eye

• What is the risk of doing PRP in eye with macular edema?

Exacerbation of macular edema

• How do you manage if patient has both PDR and macular edema?
CSME + non-high risk PDR – initial macular laser alone followed by PRP
CSME + high-risk PDR – macular and PRP 1st session - both in the same sitting / avoid laser in the
temporal area during PRP 1st sitting to reduce the risk of macular edema

• How long does it take for the laser beneficial effect?

2-4 months

• What are the signs of response to PRP?

1. Regression of new vessels
2. Decrease in venous dilatation
3. Decrease in retinal haem.
4. Disc pallor

• What to do if NV is persistent even after PRP?

1. Consider further laser
2. Make sure the systemic risk factors – blood sugar / BP / lipids / renal status are under control
/ rule out smoking / alcohol use

• What is the indication for repeat laser following PRP?

1. Persistent NV
2. Increasing NVE or NVI
3. New vitreous haem
4. New areas of NV

• What is the name of the laser technique of doing further laser after PRP?
Fill-in PRP

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Diabetic Retinopathy

• Why is it called Fill-in PRP?

Laser is done to fill-in the available retinal spaces between the previous laser scars

• What are the complications of PRP?

1. Pain
2. Peripheral Field loss
3. Central loss due to macular edema
4. Choroidal detachment if PRP extensive
5. CNVM
6. Epiretinal membrane

• What to do if NV is persistent foll. PRP and the media is hazy for further laser?
Anterior Retinal Cryopexy

• What is Anterior Retinal Cryopexy?

Ablation of peripheral / anterior retina with cryotherapy

• What to do if NV is persistent in spite of repeated laser?

• Intravitreal anti-VEGF
• Vitrectomy

• How does anti-VEGF help in treating persistent NV?

Reducing VEGF – decrease stimulation for new vessel formation- regression of NV

• Is anti-VEGF better than PRP for PDR?

DRCR Net
Ranibizumab non-inferior to PRP
Clarity Trial
Aflibercept better visual acuity than PRP group

• What are the advantages and disadvantages of anti-VEGF for PDR compared to PRP?
Anti-VEGF compared to PRP
Advantage
▪ No field loss
Disadvantage
▪ Cost
▪ Patient compliance
▪ Risk of exacerbation of TRD

• What is the current accepted first line of treatment for PDR?

PRP
Anti-VEGF as an adjunct
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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Diabetic Retinopathy

• What are the treatment options for vitreous haemorrhage due to PDR?
PRP (if retinal view is present)
Observe
Vitrectomy if not resolving

• What is the investigation of choice for dense vitreous haemorrhage with no view of retina?
Ultrasound B scan

• What would you look for in the B scan?

Retinal status –rule out TRD; if present, identify if it is involving macula or not
PVD present or not (helps during surgery)
Location of the haemorrhage (intragel /sub-hyaloid)

• What is DRVS?
Diabetic Retinopathy Vitrectomy Study
Vitrectomy beneficial for persistent vit haem
Early vitrectomy beneficial in Type 1 Diabetic

• What are the indications for vitrectomy in DR?

1. Persistent vitreous haemorrhage
2. Tractional retinal detachment involving the macula
3. Combined Rhegmatogenous and Tractional RD
4. Vitreous haemorrhage with rubeosis

• What are the steps in vitrectomy for DR?

1. To clear the blood in the vitreous - vitrectomy
2. To release the tractional forces that pull the retina
3. To remove the scaffolding on which the new vessels grow – membrane peeling
4. To perform laser photocoagulation – endo / indirect laser
5. To tamponade the retina – air / gas / silicone oil

• What are the complications of vitrectomy?

1. Recurrent vitreous haemorrhage
2. Retinal detachment
3. Rubeosis iridis
4. Cataract

• What should be ruled out in the anterior segment in eye with DR?
Rubeosis iridis & Angle New Vessel

• How to look for rubeosis?

Before dilation
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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Diabetic Retinopathy

Examine pupillary margin

Using high magnification

• What are the causes for rubeosis?

DR / Ischemic RVO / Retinal artery occlusion /Ocular ischemic syndrome / Chronic Retinal
Detachment / Chronic uveitis / Intraocular tumor

• What is the treatment for rubeosis iridis / angle neovascularisation?

Urgent PRP to prevent neovascular glaucoma

• What are the causes for visual loss in DR?

1. Macular edema
2. Macular ischemia
3. Tractional retinal detachment involving macula
4. Vitreous haemorrhage
5. Neovascular glaucoma

Diabetic Maculopathy
• What are the types of diabetic maculopathy?
1. Diffuse
2. Focal
3. Ischemic
4. Exudative
5. Mixed

• What is the investigation of choice in diabetic maculopathy?

OCT

• What is the role of OCT in diabetic macular edema?

To rule out foveal involvement
To rule out Vitreo Macular Traction
To quantify the amount of edema
To assess the response to treatment and for follow-up

• When to treat diabetic maculopathy?

Only if CSME present

• What is CSME?
Clinically Significant Macular Edema
Defined as the presence of one or more of the following features.
1. Retinal thickening within 500 μm of the centre of fovea
2. Hard exudates within 500 μm of the fovea, if associated with adjacent retinal thickening.

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Diabetic Retinopathy

3. Retinal thickening that is one disc area (1500 μm) or larger, any part of which is within 1 Disc
Diameter of the centre of the fovea.

• What is the newer classification of Diabetic Macular Edema?

Based on OCT and the location
Centre-involving DME (Corresponding to ETDRS CSME - Criteria 1)
Non-centre involving DME (Corresponding to ETDRS CSME - Criteria 2 & 3)

• How is CSME diagnosed?

With 3 dimensional assessment
Clinically by stereoscopic examination of the macula with 78D or 90D lens or
by stereoscopic fundus photography

• Can CSME be diagnosed by FFA?

No – it is a clinical diagnosis

• Can CSME diagnosed by ‘normal’ fundus photography?

No - only with stereoscopic fundus photography

• What are the 3 patterns of fluid accumulation in OCT in eyes with diabetic macular edema?
1. Diffuse Retinal Thickening (DRT)
2. Cystoid Macular Edema (CME)
3. Sub-retinal fluid (SRF)

• What are the different types of OCT findings in Diabetic Macular Edema?
1. Diffuse type (DRT)
2. Cystoid type (CME)
3. Serous type (SRF)
4. Vitreomacular traction type (VMT)
5. Mixed

• What are the treatment options for CSME?

1. Macular laser
2. Intravitreal anti VEGF
3. Intravitreal steroid
4. Vitrectomy if vitreomacular traction +

• When to treat diabetic maculopathy?

CSME

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Diabetic Retinopathy

• What is the first line of treatment for Centre-Involving CSME?

Intra-vitreal anti-VEGF

• What is the treatment for Non-Centre involving CSME?

Laser with or without prior intravitreal anti-VEGF

• What is ETDRS? What are the study results?

Early Treatment of Diabetic Retinopathy Study
Addressed 3 issues
1. Efficacy of laser treatment for macular edema
Laser reduced the risk of ‘moderate’ visual loss by 50%
2. Optimal timing of initiating PRP
PRP should be given for high-risk PDR / can be considered for severe NPDR / not required
for mild / moderate NPDR
3. Role of Aspirin
Aspirin did not alter the rate of progression of DR / no influence on VA / did not increase the
risk of vitreous haemorrhage
No ocular contraindication for Aspirin

• What are the signs of good prognosis in diabetic maculopathy?

1. Circinate ring of hard exudates with recent onset
2. Well-defined leakage
3. Good perifoveal perfusion

• What are the signs of bad prognosis in diabetic maculopathy?

1. Diffuse edema / Multiple leaks
2. Foveal lipid deposition
3. Macular ischemia
4. Cystoid macular edema
5. VA < 6/60
6. Hypertension

• What is CME?
Cystoid Macular Edema

• What are the causes of CME?

1. Diabetic retinopathy
2. Retinal vein occlusion
3. Retinal telangiectasia – PFT/Coat’s
4. Post-cataract surgery
5. Chronic uveitis, esp. pars planitis
6. Epiretinal membrane

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Diabetic Retinopathy

7. Latanoprost
8. Retinitis Pigmentosa
9. CNVM
10. Choroidal tumours
11. Birdshot chorioretinopathy
12. Radiation retinopathy
13. Nicotinic acid maculopathy

• What is the angiographic finding in CME?

Flower-petal appearance- due to radial arrangement of outer plexiform layer

NOTE
CME and CSME are not the same
CME refers to cystoid macular edema due to any cause including DR
CSME refers specifically to the Diabetic Maculopathy satisfying the ETDRS criteria.
An eye with diabetic CSME can have CME

• What are the types of macular laser?

1. Grid Macular laser
2. Focal Macular laser

• What lens is used for macular laser?

Mainster area centralis

• When to do macular grid laser?

If macular edema is clinically significant and diffuse

• How to do macular grid laser?

Laser spot 50-100 micron / power enough to get light grey burn / in the area of diffuse capillary
leakage / in a grid pattern - the shape of C with open end of C facing the fovea

• When to do focal macular laser?

If macular edema is clinically significant and focal

• How to do macular focal laser?

Laser applied to focal leakage points e.g. central microaneurysm in a circinate ring of hard exudates

• How does macula laser work in CSME?

Laser photocoagulation helps to trigger the RPE pump – thereby helping resolution of macula edema

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Diabetic Retinopathy

• Why should argon blue/green laser not be used for macular treatment?
Blue light absorbed by xanthophylls pigment overlying the macula

• What are the complications of macular laser?

1. Initial decrease in vision
2. Inadvertent foveal burn
3. Central / Paracentral field defects
4. Sub retinal fibrosis

• What should the patient not do during macular laser?

Never look at the laser beam directly during treatment – causes foveal burn

• What is the role of intravitreal anti-VEGF?

1. Used in CSME
2. Persistent NV not responding to repeated laser
3. Prior to vitrectomy to reduce the vascularity of the fibro vascular proliferation

• What are the anti-VEGFs used?

Ranibizumab (Lucentis) –monoclonal antibody fragment/ costly / FDA approved
Bevacizumab (Avastin) – monoclonal antibody /cheap, off-label / not approved by FDA
Aflibercept (Eyelea) – receptor-antibody fusion protein / costly / FDA approved / longer action
Pegaptanib (Macugen) – aptamer (not used now)

• How does anti-VEGF act?

Binds and blocks the VEGF receptor

• How does anti-VEGF help in diabetic macular edema?

VEGF – increases capillary permeability – macular edema
Anti-VEGF- blocks VEGF receptors – prevents VEGF mediated capillary permeability & leakage –
reduces macular edema

• What is DRCR Net?

Diabetic Retinopathy Clinical Research Net trial

• What are the results of DRCR Net trial?

Intravitreal anti-VEGF (Ranibizumab) with immediate or deferred macular laser group better VA
than laser alone group
Intravitreal triamcinolone combined with macular laser ‘no better VA’ compared to laser alone but
benefit in pseudophakic eyes

• How is anti-VEGF injection given?

After informed consent,
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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Diabetic Retinopathy

In operation theatre / prior povidone iodine in the eye / topical or subconjuctvial anaesthesia / 3.5mm
from the limbus superiorly / injected into the vitreous cavity / post-injection check for retina artery
pulsation / topical antibiotics

• What is the intravitreal dosage of Avastin?

1.25 – 2.5 mg (0.05-0.1ml)

• What are the risks of intravitreal anti-VEGF?

1. Endophthalmitis
2. Retinal detachment
3. Haemorrhage
4. Systemic risk – hypertension / stroke / myocardial infarction

• What should be the frequency of intravitreal anti-VEGF for DME?

Monthly injections for 3-4 months followed by prn

• What should be assessed during the follow-up after intravitreal anti-VEGF?

Visual acuity
Macular evaluation
OCT

• What is the ideal response to intravitreal anti-VEGF for DME?

Complete resolution of edema (Primary)
Improvement in visual acuity (Secondary)

• What is sub-optimal response to intravitreal anti-VEFG for DME?

50% reduction in excess thickness
BCVA <6/12

• When to switch to a different anti-VEGF?

If early response to anti-VEGF (after 3-4 injections) is sub-optimal

• How long to continue with anti-VEGF?

4-6 months

• When to potentially consider STOPPING anti – VEGF treatment?

Fluid still present in the retina
Lack of anatomical improvement (e.g.: CRT)
Lack of improvement in BCVA
Monthly injections are too burdensome (e.g. poor compliance)
Recent stroke or cardiovascular event

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Diabetic Retinopathy

• Is there a Biomarker to predict whether the patient will NOT respond to anti-VEGF
treatment?
DRIL - Disorganization of Retinal Inner Layer

• What are the factors responsible for patient’s response to anti-VEGF?

Primary factor - Balance of VEGF versus other inflammatory cytokines
Secondary factor - Chronicity of the DME

• What is the other treatment option if the eye is not responding to anti-VEGF?
Intravitreal steroid for refractory DME

• What are the poor prognostic indicators identifiable in OCT?

Sub‐retinal fluid and large cysts
Hyper reflective dots
Partial/total disorganisation of retinal inner layers (DRIL) and photoreceptor disruption
Epiretinal membranes, vitreomacular traction, vitreomacular adhesion.

• What are the intravitreal steroids available?

Triamcinolone acetate
Dexamethasone
Fluocinolone acetonide

• How does intravitreal steroid help in macular edema?

Controls the inflammation-mediated vascular permeability

• What are the risks of intravitreal steroid?

1. Raised IOP
2. Cataract

• What should be ruled out in DR with exudative maculopathy?

Renal disease

• What is the role of FFA in DR?

1. To guide the treatment of DR – identify areas of capillary leakage
2. To evaluate unexplained visual loss – to rule out macular ischemia
3. To identify suspected but clinically obscure retinal neovascularisation – differentiate
between IRMA (no / mild leak) & NV (profuse leak)

• What are the FFA findings in DR?

Microaneurysms – hyper fluorescent spots due to pooling of fluorescein followed by increase in size
and intensity of fluorescence due to leakage
Retinal haem – hypoflourescence due to blockage
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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Diabetic Retinopathy

Hard exudates – hypoflourescence due to blockage

Retinal edema – hyperfluorescence increasing in late stage due to leakage
Retinal ischemia –hypofluorescence due to capillary non-perfusion
IRMA – no / mild leak
NV – increasing hyperfluorescence due to profuse leakage

• What is the role of OCT in diabetic maculopathy?

1. To quantify retinal thickness
2. To monitor macular edema
3. To identify vitreomacular traction

• Is OCT useful to diagnose ischemic maculopathy?

No

• How to diagnose Ischemic Maculopathy?

1. Visual loss not corresponding to macular signs
2. FFA – capillary non-perfusion in macula / FAZ enlargement

• What is the treatment for diabetic maculopathy with vitreoretinal traction?

Vitrectomy

• Which group of oral hypoglycemic agents can cause macular edema?

Glitazones

• What are the positive risk factors for DR?

Duration of diabetes / Blood sugar level / Hypertension / Hyperlipidemia / Renal disease /
Pregnancy/ Anaemia /Obesity / Smoking / Alcohol / Physical inactivity

• What are the protective factors for DR?

1. Myopia > - 5.0 D
2. Glaucoma
3. PVD
4. Optic atrophy
5. CRAO
6. Carotid artery stenosis
7. Chorioretinal scarring

• What is DCCT? What are the results of DCCT?

Diabetes Control and Complications Trial
Result:
Intensive glycaemic control was associated with reduced risk of newly diagnosed retinopathy and
Reduced progression of existing retinopathy in IDDM patients
Reduction of DR by 76% / Diab. Nephropathy by 35-50%/ Diab. Neuropathy by 60%
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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Diabetic Retinopathy

• Is there any ocular risk factor for DR?

Cataract surgery

• What is the pathophysiology of DR?

Microangiopathy
BM thickening / endothelial damage / RBC changes / sticky platelets – micro vascular occlusion –
hypoxia – vasogenic factors – pre-prolife /PDR
Pericyte loss – out pouching / leaky capillaries – haemorrhage / retinal edema / hard exudates

• What are the indications for cataract surgery in a diabetic?

1. Visually significant cataract interfering with patient’s routine activities
2. Cataract preventing adequate fundus examination
3. Cataract interfering with laser treatment

• What is the risk of cataract surgery in a patient with DR?

Worsening of diabetic retinopathy / maculopathy

• How to differentiate between post-cataract surgery CME and diabetic maculopathy?

Disc leak on FFA in post-cataract surgery CME, but not in diabetic maculopathy

• What IOL to be avoided in diabetics esp. with DR?

Silicone IOL

• What are the special considerations for cataract surgery in diabetics?

Preoperative glycaemic control
Poor dilation of pupil
Larger rhexis
Avoid Silicone IOL
Increased risk for infection
Delayed wound healing

• What are the ocular findings in a diabetic?

Orbit – Cellulitis / Mucormycosis
Nerves / EOM – Diabetic neuropathy
Ocular appendages – Blepharitis / Xanthelasma / Abnormal conjunctival vasculature
Cornea...Corneal wrinkles / pigmentation / decreased sensitivity
Iris / TBM – Pigment dispersion/Ectropion uveae/Glaucoma/Rubeosis
Ciliary body – weakness of accommodation
Pupil – sluggish / small / AR pupil / fixed & dilated in III N palsy

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Diabetic Retinopathy

Lens – cataract snowflake / fluctuations in refraction

Vitreous – asteroid hyalosis / vitreous haemorrhage
Optic nerve – AION
Retina – Diabetic retinopathy / increased incidence of Retinal vein occlusion

• What is LASER?
Light Amplification by Stimulated Emission of Radiation

• What are the properties of a laser?

Collimated / Monochromatic / Coherent

• How does laser act on tissues?

Photochemical
◼ Photoradiation (dye laser) – photosensitive (cytotoxic)
◼ Photo ablation (Excimer) – Incision
Thermal effect
◼ Photocoagulation (Argon/ Double Freq. YAG / Diode) – Coagulation & Cautery
◼ Photovaporisation (CO2 laser)
Ionising
◼ Photodisruption (Nd:YAG)

• What are the 3 primary absorbing molecules in ocular tissue?

1. Melanin
2. Macular Xanthophyll
3. Haemoglobin

19
Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Diabetic Retinopathy

20
 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Hypertensive Retinopathy

Hypertensive Retinopathy

• What are the fundus findings suggestive of hypertensive retinopathy?

Flame-shaped retinal haem., arteriovenous changes, cotton-wool spots;
Disc edema and macular star in malignant hypertension

• How do you classify hypertensive retinopathy?

Keith-Wagner- Barker classification
Group 1: Slight constriction of retinal arterioles
Group 2: Group 1 + focal narrowing of retinal arterioles + AV nicking
Group 3: Group 2 + flame-shaped haemorrhages + cotton-wool spots + hard exudates
Group 4: Group 3 + optic disc swelling

Scheie Classification
For Hypertensive Retinopathy
Stage 0: No visible abnormalities
Stage 1: Diffuse arteriolar narrowing
Stage 2: Stage 1 + focal arteriolar constriction
Stage 3: Stage 2 + retinal hemorrhage
Stage 4: Stage 3 + hard exudates + retinal edema+ optic disc swelling

For Arteriosclerosis
Stage 0: Normal
Stage 1: Broadening of arteriolar light reflex
Stage 2: Stage 1 + AV crossing changes
Stage 3: Copper wiring of arterioles
Stage 4: Silver wiring of arterioles

• What are the arteriolar changes in hypertensive retinopathy?

Decrease in the AV ratio
Change in the arteriolar light reflex (appears as copper and/or silver wiring)

• What is the normal AV ratio?

2:3

• What are the AV changes seen in hypertensive retinopathy?

Salus’s sign - Deflection of veins at AV crossings
Bonnet sign – banking of veins distal to AV crossings
Gunn sign – tapering of veins on either side of the AV crossing
Right angled deflection of veins

• Are the first AV crossing changes significant? Why?

No - As the first crossing is in direct continuation with the main vessels of disc

21
 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Hypertensive Retinopathy

• What are the features of retinal blood vessels that differentiate them from other blood
vessels?
Absence of sympathetic nerve supply
Auto regulation of blood flow
Presence of blood retinal barrier

• What are the four phases of hypertensive retinopathy?

Vasoconstrictive phase
Sclerotic phase
Exudative phase
Malignant hypertension

• What are the four circumstances in which hypertensive retinopathy can occur?
Hypertension without sclerosis
Hypertension with involutionary sclerosis
Hypertension with compensatory arteriolar sclerosis
Malignant hypertension

• What are the posterior segment complications of Hypertension?

Hypertensive retinopathy
Retinal vein occlusion
Retinal artery occlusion
Retinal arterial macroaneurysm
Hypertensive choroidopathy
AION (Non-arteritic)

• What is the pathophysiology for the findings in hypertensive retinopathy?

Hypertension affects the inner blood retinal barrier and causes vascular leakage, leading to
haemorrhages and edema
Also causes occlusion of precapillary arterioles, causing cotton-wool spots

• What are the causes for disc edema in hypertension?

Malignant hypertension
CRVO
AION

• How would you differentiate hypertensive retinopathy from diabetic retinopathy?

Hypertensive Diabetic
Retinopathy Retinopathy
Haemorrhages Flame shaped Dot-blot
Microaneurysm Uncommon Common
Retinal arterioles Narrowed Not necessary except
in preprolif.

22
 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Hypertensive Retinopathy

• How will you differentiate between hypertensive retinopathy and retinal vein occlusion?

Hypertensive Retinopathy Retinal vein occlusion

Laterality Bilateral Unilateral usually
Haemorrhages Mild to moderate Extensive
Retinal arteries Narrowed Normal unless secondary to
HTN
Retinal veins Not dilated or tortuous Dilated and tortuous

• What is FIPT?
Focal Intra-retinal Periarticular Transudates - Early sign esp. in Malignant Hypertension
Result of breakdown of the blood-retinal barrier in pre-capillary retinal arterioles due to dilatation
of the arterioles from failure of autoregulation
Commonly mistaken as cotton-wool spot

• What is macular star?

Deposition of hard exudates in the outer plexiform layer of the macula, giving a star-like
appearance

• What would you suspect if the hypertensive retinopathy changes are present in only one
eye?
Carotid artery occlusion on the side of the uninvolved eye

• What are the findings in hypertensive choroidopathy?

Elschnig’s spots – hyper pigmented patches with a margin of hypo pigmentation secondary to
choroidal infarcts
Siegrist’s streaks – linear configurations of hyper pigmentation over choroidal arteries due to
fibrinoid necrosis
Focal RPE detachment
Exudative RD

• Who is at risk of developing hypertensive choroidopathy?

Young patients who develop acute hypertension
Pre-eclampsia, pheochromocytoma, accelerated hypertension

• How will you manage hypertensive retinopathy?

BP check and refer to physician for control of hypertension
Observe
Review in a month

• What is the significance of hypertensive retinopathy from systemic point of view?

Indicates the possibility of other Target Organs Damage (TOD) secondary to hypertension
Associated with an increase risk of stroke even after controlling BP and other vascular risk
factors

23
 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Hypertensive Retinopathy

• Why is it important to do an urgent referral of a patient with malignant hypertension to a

physician?
If untreated, mortality can be 50% within 2 months and almost 90% in 1 year

• What is the visual prognosis in hypertensive retinopathy?

Usually good if the hypertension is controlled

• What are the causes of permanent vision loss in hypertensive retinopathy?

Secondary optic atrophy after prolonged disc edema
Retinal pigmentary changes after exudative RD

• What is the importance of hypertension in a patient with diabetic retinopathy?

Can worsen the diabetic retinopathy even if the blood sugar is controlled

24
Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Hypertensive Retinopathy

25
 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Retinal Vein Occlusion

Branch Retinal Vein Occlusion

• What are the symptoms of branch retinal vein occlusion

Blurred vision
Field loss

• What are the characteristics of haemorhages in BRVO?

Superficial sector of retina along the branch retinal vein
Do not cross the horizontal raphe

• What are the signs of ischemic BRVO?

Cotton-wool spots
Retinal neovascularisation

• How to differentiate between BRVO and Diabetic Retinopathy?

BRVO Diabetic Retinopathy

Laterality Usually Usually bilateral

unilateral

Haemorrhages - Superficial Deep –Dot & Blot

Location

Haemorrhages– Do not cross Cross the horizontal

Distribution the horizontal raphe
raphe

• How to differentiate between BRVO and Hypertensive Retinopathy?

BRVO Hypertensive
Retinopathy

Laterality Usually unilateral Usually bilateral

Vessels Veins dilated post- Narrowed

occlusion arterioles

Haemorrhages-– Sectoral Diffusely

Distribution distributed
Do not cross the
horizontal raphe Cross the
horizontal raphe

26
 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Retinal Vein Occlusion

• What is the aetiopathogenesis of BRVO?

Retinal arterial wall disease – secondary to Hypertension / Arteriosclerosis / DM – Occlusion of the
retinal vein at the level of arteriovenous crossing

• What are the causes of vision loss in Branch Retinal Vein Occlusion?
Macular edema
Macular haemorrhage
Vitreous haemorrhage
Tractional Retinal Detachment involving macula

• What type of field defect is seen in BRVO?

Sector shaped defect with apex at the site of occlusion.

• What type of BRVO is common in diabetics?

Superonasal BRVO

• When does neovascularisation occur in BRVO?

when capillary non-perfusion is more than 5 DD

• What are the FFA findings in BRVO?

Localised areas of capillary non-perfusion
Macular leakage / edema
Collateral
New vessels.

• What is pre-thrombotic sign of Bonnet?

In impending BRVO - presence of oedema and halo around a particular AV crossing where BRVO
is commencing.

• Which vein is the most commonly affected in BRVO and why?

Superotemporal because it has large number of AV crossings.

• What are the signs of old BRVO?

Sheathing of vessels
Collaterals
Microaneurysms
Hard exudates
Cystoid Macular Edema
RPE changes at the macula

• What is the most common cause for BRVO?

Hypertension
27
 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Retinal Vein Occlusion

• What will you suspect if the occlusion does not occur at AV crossings?
Suspect inflammatory cause

• What is the incidence of rubeosis in BRVO?

8% in 3 years

• Which is more common – NVE or NVD?

NVE

• What is the common site of NVE in BRVO?

At the border of ischemic retina drained by the blocked vein

• What are the routine investigations for a patient with BRVO?

Blood pressure check
Blood sugar
Complete / Differential blood count
Platelets
ESR

• What is the indication for FFA in BRVO?

To rule out macular ischemia
To rule out retinal neovascularisation (if clinically not sure)

• What is the risk of developing BRVO in the other eye?

5%

• What is the most common cause for persistent poor vision in BRVO?
Chronic macular edema

• How does OCT help in BRVO?

To identify and quantify macular edema
To monitor the response to treatment

• What are the treatment options for macular edema in BRVO?

Intravitreal anti-VEGF (first-line)
Intravitreal steroid (Dexamethasone implant, triamcinolone)
Laser – Macular grid

• What are the indications for laser treatment in BRVO?

Macular edema
Retinal neovascularisation – Sectoral retinal laser photocoagulation

28
 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Retinal Vein Occlusion

• What are the indications for vitrectomy in BRVO?

Persistent vitreous haemorrhage
Tractional retinal detachment involving macula

Central Retinal Vein Occlusion

• Distant direct ophthalmoscopy

Media - usually clear (if vitreous haemorrhage is present, it will be hazy)
Disc: Size, shape normal
Margins - Clear; if oedematous, blurred margins
Colour – Hyperaemic
Vessels over disc - veins - dilated and tortuous
Haemorrhages - Superficial haemorrhages close to disc and in posterior pole / mid-periphery
Macula – Look for edema especially cystoid
Look for cotton-wool spots

• What is the diagnosis?

Central Retinal Vein Occlusion

• What are the points to say it is CRVO?

Haemorrhages and other findings concentrated around disc and in posterior pole / mid periphery
Disc edema
Tortuous dilated veins in all 4 quadrants

• How to classify Retinal Vein Occlusion?

CRVO - ischaemic or haemorrhagic retinopathy
-nonischemic or venous stasis retinopathy
Hemi CRVO - Ischemic (22%), Non ischemic (78%)
BRVO - Major BRVO close to disc margin
-Macular RVO
-Peripheral tributary vein occlusion

• What are the common causes of CRVO?

Atherosclerosis
Hypertension
Glaucoma
DM
RB injection
29
 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Retinal Vein Occlusion

Trauma
Vasculitis – phlebitis etc.

• What are the causes of CRVO in young adults?

Phlebitis
Hypercoagulability state
Hyperhomocysteinemia
DM/HT
Collagen vascular diseases
Medications –Oral Contraceptives, Isoretinoin (a form of Vit A used for acne)
AIDS
AV malformation
Trauma
Hypermetropia

• What are the causes of Bilateral CRVO?

HT
DM
Hyperviscosity syndromes

• What are the causes for combined CRVO & CRAO?

RB injection
Hypreviscosity syndromes

• How does trauma cause CRVO?

Sudden compression of the eyeball or change in IOP – vessel wall damage by shearing or
compression against the lamina cribrosa

• What ocular disease is most commonly associated with CRVO?

Glaucoma

• What is the importance of IOP in CRVO?

Raised IOP can cause CRVO – IOP might be low in eye with CRVO

• What is the need for treating ocular hypertension with IOP greater than 30 mm Hg?
To reduce the risk of developing glaucoma
To prevent CRVO

• What is the earliest sign in RVO?

Tortuosity of blood vessels

• What are the fundus signs in resolving CRVO?

Resolving haemorrhages / Macular edema

30
 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Retinal Vein Occlusion

• What examination should be done before dilation in a patient with CRVO?

VA
Look for rubeosis - Slit lamp examination of pupillary margin under high magnification
Gonioscopy to rule out angle neovascularisation
IOP
Relative afferent pupillary defect

• What would you check in the other eye in a patient with CRVO?
IOP
Disc cupping
Evidence of DR if patient is diabetic

• What is the risk of developing CRVO in the other eye?

10%

• What are the types of CRVO?

Non-ischemic -75%
Ischemic -25 %

• What is the natural course of non-ischemic CRVO?

Complete resolution - 48%
Partial resolution - 30%
Ischemic 22%

• What is the incidence of rubeosis in ischemic CRVO?

60% in 3-4 months

• How to differentiate between superficial and dot / blot haemorrhages?

Superficial Dot & Blot Age

In nerve fibre layer In Middle compact layer

Colour - Bright red Dark red
Flame-shaped Round with well defined margin

• What is the visual prognosis in CRVO?

Usually good in Non-ischemic CRVO
Usually worse in Ischemic CRVO

• What are the causes of vision loss in Retinal Vein Occlusion?

Macular edema
Macular haemorrhage
Cilioretinal artery occlusion
Neovascular glaucoma

31
 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Retinal Vein Occlusion

• What is the cause for cilioretinal artery occlusion in CRVO?

CRVO – venous stasis – increased backflow pressure in capillary bed – stagnation in cilioretinal
artery – physiological block – retinal ischemia in area supplied by cilioretinal artery

Note: Cilioretinal artery is derived from the choriocapillaries and not central retinal artery – no
autoregulation in cilioretinal artery – cannot pump blood into the high-pressure capillary bed

•
• How to differentiate between non-ischemic and ischemic CRVO?

Non-ischemic Ischemic CRVO

CRVO

Haemorrhages Mild-Moderate Severe

extent

Cotton-wools Absent Present

spot

RAPD Absent Present

Rubeosis Absent Present

Visual acuity Usually good Usually worse

FFA Capillary Present Absent

perfusion

ERG b-wave Normal Decreased

amplitude

• What would you like to do for this patient?

Check VA / Look for rubeosis / IOP / RAPD / Other eye fundus for disc cupping / retinopathy
Investigate to find the systemic risk factors / cause for CRVO

• What is the appearance of the haemorrhages in CRVO?

Tomato splash.

• Where is the site of occlusion in CRVO?

Ischaemia - Lamina cribrosa or just posterior to it
Non ischaemia - further back collateral

• How to differentiate between CRVO & Diabetic Retinopathy?

32
 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Retinal Vein Occlusion

CRVO Diabetic
Retinopathy

Laterality Usually unilateral Usually bilateral

Haemorrhages Both in posterior Mainly in posterior

pole and periphery pole

Hard exudates More common Less common

• How to differentiate between CRVO & Ocular Ischemic Syndrome?

CRVO Ocular Ischemic

Syndrome

Venous Present Absent

tortuosity

Haemorrhages Posterior pole and Midperiphery

periphery

Disc edema Present Absent

• What are the systemic investigations you will do for a patient with CRVO?
Blood pressure check – Hypertension
Blood sugar – DM
Complete blood count with differential - Leukaemia
Platelet –
ESR – Giant Cell Arteritis
Lipid profile – Hyperlipidemia

• What are the special investigations for CRVO in young adult?

Homocysteine levels / PTT / ANA / FTA-ABS/ VDRL / Antiphospholipid antibodies / Serum
protein electrophoresis / Activated Protein- C /

• What is the follow-up protocol for CRVO patients?

Every month for 3 months followed by every 3 months for 6 months, then every 6 months for 2
years

• What is CRVOS?
Central Retinal Vein Occlusion Study

• What are the recommendations of CRVOS?

Macular photocoagulation for macular edema in non-ischemic CRVO shows angiographic

33
 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Retinal Vein Occlusion

improvement but no improvement in visual acuity

Pan Retinal laser Photocoagulation for ischemic CRVO should be done only if rubeosis develops
and causes significant regression of rubeosis
Prophylactive PRP for ischemic CRVO can be considered only in patients with poor compliance

• What are the risk factors for development of rubeosis in ischemic CRVO?
Increased areas of capillary non-perfusion
Extensive intraretinal haemorrhage
Poor Visual Acuity

• What are the signs of resolved / old CRVO?

Optociliary shunt / Disc collateral
Mild retinal venous tortuosity

• What is optociliary shunt?

Collateral vessel at the disc connecting the retinal and ciliary circulations

• What are the causes of optociliary shunt?

CRVO
Chronic glaucoma
Chronic papilloedema
Optic nerve sheath meningioma

• How to differentiate optociliary shunt from new vessel at the disc?

Optociliary shunt – Larger, no leakage in FFA
NVD – leaks profusely in FFA

• What are the treatment options for macular edema in CRVO?

Laser – Macular grid in non-ischemic CRVO
Intravitreal anti-VEGF
Intravitreal steroid

• What is the indication for PRP in CRVO?

Presence of new vessel in iris or angle

• What is the risk of not treating rubeosis in ischemic CRVO?

Neovoascular glaucoma – which can cause painful, irreversibly blind eye

• Is prophylactic PRP routinely recommended in ischemic CRVO without neovascularisation?

No – except patients with poor compliance

• What is 100th day glaucoma?

Neovascular glaucoma secondary to untreated rubeosis following ischemic CRVO commonly
occurs

34
 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Retinal Vein Occlusion

3 months (90-100 days) after RVO onset – hence called 100th day glaucoma

• When to do FFA?
After 4-6 weeks for the haemorrhage to clear
• How does OCT help in CRVO?
To identify and quantify macular edema
To monitor the response to treatment

Hemi RVO

• What are the points for Hemi RVO?

Haemorrhages restricted to only superior or inferior retina respecting the horizontal raphe
Dilated and tortuosity of superior or inferior retinal veins (not both)

• What is Hemi CRVO?

Blockage of superior or inferior hemi-retinal vein

• How to differentiate between hemi RVO and major BRVO?

Hemi RVO Major BRVO
Site of occlusion is within optic nerve Always at AV crossing near the disc

Disc - edema at corresponding part Disc normal

Collaterals on the optic disc or within the Located on the retina away from the disc
optic nerve

• What is the normal development of Central Retinal Vein?

In the 3rd month of intra-uterine life, 2 trunks of Central Retinal Vein present on either side of the
Central Retinal Artery – after birth, one of the trunks disappears
20% show persistence of the dual- trunk

• What is the pathogenesis of Hemi RVO?

Pathogenesis being similar to CRVO.

• How to treat Hemi-RVO?

Treat the cause
Treatment for macular edema –anti-VEGF / intravitreal steroid / laser
Treatment for rubeosis - PRP

35
Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Retinal Vein Occlusion

36
 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus CRAO

Central Retinal Artery Occlusion

Distant direct: Media

Direct: Disc- size, shape margins usually normal

colour – pale if long-standing
vessels over the disc important arteries attenuated .
AV ratio - decreased / altered.
Macula - Cherry red spot.
Vessels - veins - any cattle truck appearance
General retinal background – pale, Retinal emboli might be visible

• What is the first sign of CRAO?

The retina appears white as a result of cloudy swelling caused by intracellular oedema especially at
the posterior pole where the nerve fibre and ganglion all layer are thickest
.
• Why is the retina pale in CRAO?
Due to cloudy swelling of ganglion cell layer.

• How long can the retina withstand ischemia?

90 min - 100 min.

• What is the reason for cherry-red spot in CRAO?

Here the inner retinal layers appear pale and gray. The normal retinal transparency is lost.
Histopathologically, the ganglion cells are swollen and later necrosed.
In the thin foveal area, inner retinal elements are not present and the normal reddish colouration of
choroid remains visible, which is not seen elsewhere → CHERRY RED SPOT.

• When is cherry-red spot absent in CRAO?

1. If associated with cilioretinal artery is present and spared
2. Old CRAO

• What are the causes of cherry - red spot?

1. CRAO
2. Tay - Sachs disease (80%)
3. Sandhoff's disease
4. Generalised gangliosidosis
5. Niemann - Pick disease
6. Farber's lipo granulomatosis
7. Metachromatic leukodystrophy
8. Sea-blue histocyte syndrome
9. Commotio retinae
10. Goldberg - collier syndrome

37
 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus CRAO

• What are the reasons for Cherry-red spot in these storage disorders?
Niemann Pick
Lipid infiltration into peripheral ganglion cells with relatively little ballooning.
Tay-Sach's disease
Peripheral infiltration of lipids in ganglion cells produce peripheral gray to white density, which
contrasts with non-infiltrated fovea.
Metachromatic Leukodystrophy
Sulfatide infiltration into retinal ganglion cells.

• When does cherry - red spot disappear in CRAO?

in few weeks.

• When and why do you get cattle truck appearance?

In acute CRAO
Due to sluggish and segmental blood flow
Also called’ boxcarring’

• How do patients present?

Sudden, painless loss of vision

• What to suspect if VA is No PL with findings similar to CRAO?

Ophthalmic Artery Occlusion

• Why is cilioretinal artery, if present, spared in CRAO? What is the advantage?

Because it is derived from the choroidal circulation and not from the central retinal artery
Central vision spared

• Can cilioretinal artery occlusion occur?

Yes
As isolated or in the event of CRVO or AION

• How does CRVO cause cilioretinal artery occlusion?

Venous stasis cause functional block of the cilioretinal artery as it is supplied by the
choriocapillaries, which lack autoregulation like central retinal artery to overcome this.

NOTE:
The presence of cilioretinal artery is an advantage in CRAO but a disadvantage in CRVO!

• What is the normal blood supply to the retina?

Outer retina from ciliary arteries through the choriocapillaries
Inner retinal from the central retinal artery

38
 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus CRAO

• What are the causes for haemorrhage in CRAO?

Recanalisation
Associated CRVO
Anastomosis

• When you press on the globe if arterial pulsation is seen what does it indicate?
In some cases, the obstruction is not complete in which event the arteries remain small but can be
made to pulsate vigorously on pressing upon the globe and usually, with an increased beading
effect.

• What is the pressure in CRA? What is the relationship with brachial artery pressure?
80/40 mm Hg
2/3 of systole pressure and 1/2 of diastole pressure of brachial artery pressure.

• What are the signs of old CRAO?

Disc pallor
Retinal vessel attenuation
Grossly decreased vision

• What are the causes for CRAO?

Embolus – from carotid or cardiac
Thrombus
Collagen Vascular Disorders
Hypercoagulation disorders
Giant Cell Arteritis
Trauma

• What are the causes of bilateral CRAO?

1-2% is bilateral
• Cardiovascular diseases
• Giant cell arteritis
• Other vascular inflammations

• What are the causes of combined CRAO & CRVO?

RB injection causes combine CRA & CRV obstruction presumably as a result of injection within
the optic nerve stealth and/or direct damage to the CRA and vein.
Young HT patients
Vasculitis.
• How do you investigate?
BP
Blood tests- TC/DC/ESR / Blood sugar / Lipid profile
Carotid Doppler
Cardiac evaluation

39
 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus CRAO

• What are the types of emboli?

Carotid Source – Calcific / Cholesterol / Fibrinoplatelet
Cardiac source – Calcific / Vegetations / Thrombus / Myxomatous

• What is the most common source of emboli?

Carotid – Atheromatous plaque

• Why is retina easily affected by carotid source emboli?

Since ophthalmic artery is the first branch from the internal carotid artery

• What are Hollenhorst plaques?

refractile yellow–white cholesterol plaques ,

• How to identify the type of emboli based on the colour?

Yellow-white: Cholesterol
White: Calcific
Greyish: Fibrin-platelet

• What is the role of Doppler study in CRAO?

Detect both ulcerations and stenotic lesions
Measures the rate of flow of blood.

• What is the treatment for CRAO?

Ocular: If less than 24 hrs duration – can try ocular massage / oral Acetazolamide / Paracentesis
Systemic: Urgent review with physician to find the source of embolus / management of systemic
vascular risk factors like BP / Blood sugar / Lipid profile / carotid and cardiac evaluation for source
of emboli – this is important to reduce the risk of stroke
Anti-platelet therapy
Carotid endarterectomy if significant stenosis present

• What is the rationale behind ocular massage / Paracentesis / Acetazolamide?

cause lowering of IOP, which leads to vasodilation and increased retinal blood flow causing
dislodgement of embolus

• How to do the ocular massage?

Pressure for 10-15 seconds, followed by sudden release.
Intermittent pressure given for 10 minutes

• What is Carbogen inhalation? How does it help?

5% CO2 + 95% O2
Oxygen diffuses through choroid and reduces ischemia
CO2, on the other hand, acts as retinal vasodilator that can produce increased blood flow PO2 at
retina.

40
 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus CRAO

• How does anterior chamber paracentesis help?

Causes sudden decrease in IOP, which can increase the perfusion pressure behind the obstruction,
thereby dislodging the obstructing embolus.

• How is anterior chamber paracentesis done?

Usually performed at the slit lamp / under microscope with topical anesthesia
With 27 G needle attached to a syringe / inserted into the AC through the corneal stroma / directed
away from the pupil / kept over the iris / not towards the pupil to avoid lens touch

• What is the incidence of rubeosis in CRAO?

5-20%

• What is the cause for low incidence of rubeosis in CRAO?

Acute CRAO causes rapid inner layer retinal death, thus preventing the formation of angiogenic
factor

• What is Amaurosis fugax?

characterized by transient painless monocular loss of vision

• What is the significance of amaurosis fugax?

Should have urgent review with physician (similar to CRAO) for detailed evaluation and
management of vascular risk factors to reduce the risk of stroke

• How to differentiate from BRAO?

BRAO: Escorial whitening corresponding to the blocked branch retinal artery; vision not severely
affected; Absent cherry red-spot
CRAO: Four quadrant whitening; vision grossly affected; Cherry red spot present

• How to differentiate between CRAO & OAO?

Features CRAO Ophthalmic Artery Occlusion

V/A Typically CFCF to HM Usually no light perception
Cherry-red spot Present in acute stage Absent

Retinal opacification Mild to moderate Moderate to severs

Pigmentary changes Absent Present

Optic atrophy Mild pallor Marked pallor

FFA Delay in the arm-retina Delay in both choroidal filling and arm-
time but normal retina filling time
choroidal filling
ERG b wave diminished Both a and b waves diminished

41
Bala Notes for Ophthalmology Post-Graduate Examination: Fundus CRAO

42
 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Retinal Detachment

Retinal Detachment
Distant Direct Ophthalmoscope - Grey reflex

Fundus – Usually well seen with high plus power – due to detached retina

Media - clear / Hazy - depending on the case comment on it

Disc - visible or not and then describe about it.

If visible usually normal unless it is a myopic disc

Describe the detachment

Retina appearance – grayish elevated retina

Extent - If it is not total, tell about the maximal fluid level (in relation to the clock hrs).

Vessels over the detached retina - appear tortuous, darker, and wavy.

Macula – on / off

Characteristics of the detached retina

Clock hrs, vessels over detached retina - configuration – convex, concave bullous
shallow, Mobility, shifting of fluid.

Look for holes in the peripheral retina.

If retinal break is present –type / extent / location in clock hours /

anter. or poster. to equator.

Look for signs of longstanding RD - PVR.

Diagnosis – Retinal Detachment Type / Fresh or old / Partial or Total /

with or without PVR

Etiology – Rhegmatogenous / Tractional / Exudative

• What is RD?
It is the separation of the sensory retina from RPE

• Why is it a misnomer?
It is a misnomer because the retina as a whole is not involved, but a cleavage occurs
between the two primitive retinal layers.

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Retinal Detachment

• Why it occurs at that level?

Between these two layers there is embryologically the potential space of optic
vesicle

• What are the factors that keep the retina normally attached?
Acid Mucopolysaccharide - biological glue between RPE & sensory retina
RPE cell sheaths - contain a layer of actin which forms adhesion
Microvilli of RPE - Muller cells
Hydrostatic pressure - Active pump is present between the RPE & sensory retina,
which pumps out the SRF present here. So the hydrostatic pressure in the vitreous is
greater than this potential space, so the vitreous flattens up against the retina.

• Why do you get grey reflex in detached retina?

In attached retina, normally, the colour of the retina is due to the underlying
choroidal vasculature
In RD, retina is away from the underlying choroidal vasculature and light is reflected
from the detached retina, hence the grey reflex

• Why can't you differentiate between arteries and veins in detached retina?
Loss of contrast

• What are the symptoms of RD?

Floaters, Flashes of light, Failing vision and Field defect.

• Which has a localizing value – Floater or Flash?

Floater

• Why do you get flashes of light?

As the posterior vitreous pulls away from the retina during syneresis, the retina may
be stimulated to produce flashing lights
Symptom of vitreoretinal traction

• What is a retinal break?

Full thickness defect in the sensory retina.

• What are the types of retinal break?

Retinal hole – usually develops in atrophic retina – not associated with vitreoretinal
traction.
Retinal tear - develops in normal / degenerated retina – always due to vitreoretinal
traction

• What are the types of retinal tears?

Horse-shoe / U shaped tear, Giant Retinal Tear, Operculated tear

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Retinal Detachment

• What is Giant Retinal Tear?

Retinal tear >90 degrees or 3 clock hrs in extent

• What is operculated tear?

Retinal tear with overlying avulsed retinal tissue (operculum)

• What is retinal Dialysis?

Retinal disinsertion from the ora serrata
Upper Temporal tears are dangerous - macula is likely to be involved early in the
event of RD

• What are the causes of retinal dialysis?

Blunt trauma / Congenital

• How does trauma cause retinal dialysis?

Blunt trauma – axial compression and equatorial expansion of the eye – vitreous
traction along the posterior aspect of the vitreous base in the periphery – dialysis

• Where do you get pseudohole?

White without pressure – normal reddish retina within the WWOP resembles hole
Macular Epiretinal Membrane with a central defect mimics a macular hole

• What is PVD?
Detachment of the posterior hyaloid from the retina

• What are the areas where the posterior hyaloid is firmly attached?
[Link]
2. Disc
3. Retinal blood vessels
4. Ora serrata
5. Posterior edge of lattice degeneration

• What are the causes for PVD?

1. Old age – syneresis of the vitreous
2. Trauma
3. Myopia
4. Cataract surgery, esp. with vitreous disturbance
5. Post YAG laser Capsulotomy
6. Inflammation

• What is the mechanism of PVD in old age?

Old age – liquefaction of the vitreous – defect in the thinned posterior hyaloid –
liquefied vitreous enters the subhyaloid space – separates the posterior hyaloid from
the retinal ILM – PVD
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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Retinal Detachment

• How does acute PVD cause RD?

During PVD – if vitreoretinal attachment strong+/-underlying weak retinal areas like
lattice – traction on the retina – pulls the retinal tissue – retinal tear – liquefied
vitreous enters through the retinal tear into subretinal space- RD

• What percentage of PVD causes retinal tear?

10%

• Can PVD cause vitreous haemorrhage without retinal tear?

Yes – it can sometimes just cause avulsion of the retinal blood vessel without tearing
the retina – causing vitreous haemorrhage

• What is the pathogenesis of atrophic hole related RD?

Atrophy of the retina –retinal thinning – esp. with lattice – retinal hole – overlying
vitreous enters through the retinal hole into subretinal space – RD
NOTE: RD occurs without PVD

• How will you know whether the hole is anterior or posterior to equator?
In relation to vortex veins

• What is the position of the vortex veins?

clock position (1,4,7,5).

• How to identify the different landmarks?

Equator –14mm from the limbus
Ora serrata - 6-8 mm
Parsplana – 3 -3.5 mm

• Which type of retinal break is more dangerous – hole or tear?

Retinal tear more dangerous than retinal hole –Tear-related RD develops & spreads
fast compared to hole-related RD

• Which type of retinal tear is more significant - Operculated or U / Horse-shoe

Tear?
Horse-shoe / U tear – vitreoretinal traction is still active – increased risk of RD
In operculated tear – vitreoretinal traction has been released with the avulsion of the
retinal tissue

• Which break is dangerous in relation to the location?

Superior breaks are more dangerous than inferior: due to gravity SRF is likely to
spread more quickly.
Temporal breaks are more dangerous: early involvement of the macula

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Retinal Detachment

• What is Lincoff's rule?

The probable location of a primary retinal break can be predicted by studying the
shape of RD.
1. A shallow interior RD in which the SRF is slightly higher on the temporal side
points to a 1 break on that side.
2. A 1 break located at 6 O' clock will cause an inferior RD with equal fluid levels.
3. In a bullous inferior RD, the 1 break usually lies above the horizontal meridian,
especially in aphakic eyes.
4. If the 1 break is located in upper nasal quadrant, the SRF will revolve around
optic disc and then rise on temporal side until it is level with 1 break.
5. A subtotal RD with a superior wedge of attached retina points to a 1 break
located in the periphery nearest its highest border.
6. When the SRF crosses the vertical midline above, the 1 break is near to 12 O'
clock, the lower edge of the RD corresponding to the side of the break.

• What are the ‘malignant’ retinal degenerations?

Those which predispose to RD.
a. Lattice degeneration
b. Snail track
c. Acquired retinoschisis
d. White with and without pressure
e. Diffuse chorioretinal atrophy
f. Paravascular vitreoretinal attachments
g. Meridional folds
h. Pigment clumps

• What are the Benign vitreoretinal degenerations which are not usually
predisposing to RD?
1. Microcystoid degeneration
2. Snowflakes
3. Oral pigmentary degeneration
4. Paving stone degeneration
5. Honey comb degeneration
6. Drusen

• What is the difference between White with pressure (WWP) and White without
pressure (WWOP)?
White with pressure - translucent grey appearance of retina, induced by indenting
sclera.
White without pressure - same appearance without scleral indentation.

• Which is more dangerous? Why?

White-without-pressure
Giant tears occ. develop along posterior border of an area of `white without pressure'.
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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Retinal Detachment

If white without pressure is found in the fellow eye of a patient with a spontaneous
giant retinal tear, prophylactic retinopexy should be performed

• What is Lattice degeneration?

usually bilateral
6-8% Normal population
40% - of eyes undergoing repair for RD
Important cause of RD in young myope (>-3D)
Appear early in life - well established by second decade.
Limbus - parallel, cigar shaped area of retinal thinning with associated vitreous
traction in the retinal periphery.
Width -0.5 - 2 mm
length -1.5mm to more than liquid
Early -frost-like appearance (fine yellow white slapping)
Late -White crisscross pattern due to sclerosis of retinal vessels within the
lesion.
Common - near the 6 or 12 O'clock position
Temporal retinal periphery
Halfway between ora & equator
FFA - Nonperfusion of retinal vessels within the lesion; Clumped pigment blocks
choroidal fluorescence.
Pathogenesis -Unknown
Ischemia in the area of lattice degeneration.
Prophylactic Rx - Aim - to increased adhesion of RPE
a. Peripheral photocoagulation
b. Transconjunctival Cryopexy

• In which quadrant, hole is common?

Nonmyopes –Inferotemporal
Myopes –Supertemporal

• In aphakia, which quadrant is common?

Upper nasal breaks are more common. Since zonules are shorter & stronger in that
region.

• What are the reasons for increased incidence of RD in ICCE compared to

ECCE?
The extraction of lens deprived the vitreous of its support at the level of patellar
fossa. Vitreous projects partially into the AC and exert greater traction at the
vitreous base.
Rupture of anterior hyaloid membrane - incarceration of vitreous in the surgical
wound
Endophthalmodonesis is greater after ICCE in due to the loss of lens – zonular
barrier

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Retinal Detachment

Hyaluronic acid concentration after ICCE is much less than after ECCE. This
serves the function of shock absorber of the vitreous; its loss might make the eye
more subject to trauma during normal ocular movements/trauma.

• Why RD is common in myopia?

Increased incidence of
Atrophic Retinal holes due to retinal degeneration esp. Lattice degeneration
Retinal tear secondary to vitreous traction especially at the posterior border of the
lattice, where the vitreoretinal adhesion is dense

• What is Schaffer's sign?

Pigment (macrophages) cells in the anterior vitreous in a patient complaining of
sudden photopsia and floaters are strongly suggestive of retinal tear
Pigments are released from the exposed RPE of the retinal tear

• What is Schwartz Syndrome?

RD associated with increased I.O.P. - uveitis is the mechanism.

• What is the significance in knowing about the macula condition?

Prognosis
Positioning
When to do the surgery

• Why the other eye has to be evaluated?

To look for any peripheral retinal degenerations / retinal breaks / detachment

• Why scleral indentation is important?

To enhance visualisation of the peripheral retina anterior to the equator and to
perform a kinetic evaluation of the retina. The contrast between the choroid and
sensory retina is enhanced.

• When to avoid scleral indentation?

Immediate post-op
Perforating injury
Scleral thinning / staphyloma

• What is the colour coding for fundus drawing?

Attached retina- Solid red
Detached retina- Blue outline / Solid blue
Ora serrata - Blue forward dentate processes
Disc - Red
Macula - Red if attached
Blue if detached
Any opacities in the media – Green

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Retinal Detachment

Vitreous traction - Green

Hole - Blue outline, dark red centre
Operculum - Solid green for operculum; solid red outlined on blue for hole
Lattice - Blue outline with blue hatching / red background
Sheathing -Yellow over red or blue line
Exudates -Yellow solid,
Cotton wool -Yellow outline
Retinal Hge - Red
Red vitreous Hge - Red & Green
"White" vitreous Hge – Green
Retinal edema - Blue shading over solid red, no outline
CME - Blue circles, red cross - attached retina

• What is the significance of duration of RD?

Prognosis
Preop - preparation of patient
Selection of type of surgical procedures

• What is the cause for PVR in RD?

Ischemia - proliferation of fibroblasts new vessels - contraction of myofibroblast.

• What is the common cause for Pseudophakic RD?

Intra-operative vitreous disturbance

• What are the signs of old RD?

Retinal thinning - takes 12 months to form
Intraretinal cysts - develops in outer plexiform layer
Sub retinal demarcation line - 3 months to develop
Sub retinal gliosis
High water mark
PVR

• How is PVR graded?

Grade A: Vitreous haze, pigment clumps, pigment clusters on inferior retina
Grade B: Wrinkling of inner retinal surface, retinal stiffness, vessel tortuosity,
rolled & irregular edge of retinal break; decreased mobility of vitreous
Grade C: Posterior
Full-thickness retinal folds or sub retinal strands posterior to
equator - Focal, Diffuse, Sub retinal
Anterior
Full-thickness retinal folds or sub retinal strands posterior to equator
Circumferential, Anterior
• What is shifting fluid?
Classical sign of Exudative RD

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Retinal Detachment

• How is it tested?
Size and extent of the RD are noted in sitting and lying position
In Exudative RD – in sitting position, RD is more inferiorly with fluid shifted down
due to gravity; in supine position, RD is mainly in the posterior fluid
In Rhegmatogenous RD – not much variation of the size and extent of the RD both
in sitting and supine position

No. Features Rhegmatogenous Exudative Tractional

1. Break Always No No

2. Surface Convex-may be Convex Dentate or scalloped edges.

bullous Concave surface.

3. Fluid Rarely shift Shifts especially No shifting, sub retinal

from a tumour or fluid appears sucked into
Haradas disease; space under pulled-up
often dependent retina.

4. Height Bullous from superior Often not high. Generally not high
break shallow from The only type that
inferior break never may touch lens
touches lens

5. FFA No leakage or pooling Leaking and pooling No leakage or pooling

window defect in long- of fluorescein in window defect possible.
standing detachment SRF

6. Natural Progresses or remains Waxes and waxes Generally progresses, may

history detached does not according to cause, develop break and become
resolve spontaneously may resolve rhegmatogenous
spontaneously

7. Treatment Surgical Medical-treat Observe if macula not

underlying cause; involved
photocoagulation to Treat the associated
leaking source (e.g.) vascular retinopathy
hemangioma Vitrectomy if macula
involved

• What are the causes of Exudative Retinal Detachment?

I. Inflammatory: Haradas disease
Peripheral uveitis (peripheral exudative detachment)
Excessive Cryopexy
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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Retinal Detachment

Extensive photocoagulation
Choroidal effusion
II. Systemic: Renal failure
Hypertension
Dysproteinemias & macroglobulinemia
III. RPE defect: RPE detachment
CSR
IV. Sub retinal neovascularisation:
Senile disciform macular degeneration. Presumed ocular histoplasmosis, Angioid
streaks, choroidal rupture.
V. Tumours: Malignant
Malignant melanoma
Metastasis
Retinoblastoma
Benign - Haemangioma of choroid
Angiomatosis retinae
Coat's

• What are the features of tumour detachment?

more solid, less tremulous, retina over the tumour is more pigmented and
degenerated, new vessels forming an abnormal vascular pattern, SLE &
transillumination will show the tumour; tension is raised.

• How to confirm the tumour?

USG - detecting the tumours.
Diagnostic aspiration of sub retinal fluid will reveal the malignant cells.

• What is retinopexy?
Procedure done to create chorioretinal adhesion to seal a retinal break

• What are the methods by which retinopexy / chorioretinal adhesions can be

produced?
1. Laser Photocoagulation
2. Cryotherapy
3. Diathermy (not used now)

• How is laser retinopexy done?

Laser burns applied in 2 – 3 rows around the retinal break as confluent burns

• How does Cryopexy work?

Aseptic chorioretinal inflammation produced -so that stronger bond is produced
between sensory retina and RPE and choroid – chorioretinal adhesion
Liquid N2 / CO2 - Temp. between - 40C and - 100C.

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Retinal Detachment

• What is the principle of cryo?

Passing a gas under high pressure through a narrow opening causes very low
emperature
CRYOPROBE (Temp - 60C)
Bring the RPE as close as possible to the break
Commence freezing
Applied around the retinal break

• What are the errors & Complications of Cryopexy?

1. Failure of cryo to appear
2. Excessive cryotherapy - postoperative vitritis / exudative RD due to pigments
fall out
3. Insufficient cryotherapy
4. Premature removal of cryoprobe - choroidal haemorrhage, scleral rupture
5. Extraocular haemorrhage

• What are the indications for scleral buckle?

Fresh RD with superior and anterior breaks

• What is the management for fresh RD?

See the hole
Seal the hole
Scleral buckling

• What is Gonnin’s principle?

Hole - > RD
See the hole
Seal the hole
Encirclage
SRF drainage

• What is the purpose of scleral buckling?

1. To close retinal breaks by apposing the RPE to sensory retina
2. To release vitreoretinal traction.

• What are the four basic ‘S’ steps in scleral buckling?

See the retinal break – Localisation
Seal the retinal break - Cryopexy
Settle the retina - SRF drainage
Support the retinal break - Scleral buckle

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Retinal Detachment

• What is false localization? Why does this happen?

In bullous RD, during Indirect Ophthalmoscopy, scleral indentation area which
appears corresponding to the retinal break is actually not the corresponding area
when the retina is attached – this is due to a parallax – will result in false area being
cryo’ed

• How to avoid false-localisation in bullous RD?

By doing D-ACE technique

• What is D-ACE technique?

Drainage of Sub-retinal Fluid done first
Air injected intravitreally –
Cryopexy - to retinal break
Explant –

• What are scleral explants / implants?

Explant -Material sutured directly onto sclera to create a buckle
Implant -Material placed within the sclera to create a buckle (not done now)

• What are the types of explants?

1. Radial - Placed at right angles to limbus
Indications
Large U shaped tears - particularly when fish mouthy is anticipated
Posterior breaks - Sutures are easier to insert

2. Segmental circumferential - This is placed circumferentially with the limbus to

create a segmental buckle
Indications
Multiple breaks - located in one or two quadrants anterior breaks, wide
breaks - dialysis / grant tears

3. Encircling circumferential - placed around the entire circumference of the globe to

create a 360-degree buckle
Indications
Breaks involving 3 or more quadrant
Lattice degeneration involving 3 or more quadrant
Extensive RD without detectable breaks < esp. in aphakia
A broad buckle is used in an attempt to seal breaks anterior to equator
PVR grade C
Failed local procedures
Inexperienced surgeon

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Retinal Detachment

• What material is used for scleral explant?

Silicone
Soft silicone (silastic) sponge - can be used for both radial and circumference
Hard silicone straps - used only for 360 buckling (encircling band)
Hard silicone tyre - to supplement an encircling buckle

• What is the disadvantage of silicone sponge compared to silicone tyre?

Infection – as the spores can act as nidus for microorganisms

• What are the indications of SRF drainage?

1. Glaucoma, if pressure rises above systolic level when the buckle is placed.
2. A highly elevated hole for which localisation may be inaccurate and for which
several days may be needed for the hole to settle on the buckle.
3. Mottled and hypo pigmented retinal pigment epithelium.
4. Inferior hole,
5. Severe vitreous traction
6. Long standing RD
7. Immobile retina

• What is the site of SRF drainage?

Usually at the region of highest retinal elevation / away from the macula / retinal
break

• What is the advantage of SRF drainage?

Provides immediate contact between the sensory retina and RPE with flattening of
the fovea.

• How will you assess / monitor I.O.P. while RD surgery?

By checking Central Retinal Artery pulsation at the disc.
a. If spontaneous pulsation present
Central Retinal Arterial Pressure. is between diastolic and systolic blood pressures
& requires no specific action.
b. If spontaneous pulsation absent
Central Retinal Arterial Pressure is either below diastolic or above systolic blood
pressure.
o If pulsation is induced by pressing the globe - Central Retinal Arterial
Pressure is less than Diastolic.
o If not induced by pressure - Central Retinal Arterial Pressure > systolic.
Hence, slacken the sutures and massage the Globe for 2 minutes and then
tighten the sutures very slowly. If CRA is still occluded, then either drain the SRE or
perform a paracentesis

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Retinal Detachment

• What are the complications of SRF drainage?

1. Choroidal Hge [Link] incarceration
2. Ocular Hypotony 6. Vitreous prolapse
3. Dry tap 7. Damage to LPCA & nerves
4. Iatrogenic break 8. Bacterial endophthalmitis

• What are the complications and errors of intravitreal air injection?

1. Loss of visualisation 4. Damage to retina
2. Excessive elevation of I.O.P. 5. Haemorrhage
3. Damage to lens 6. Bacterial endophthalmitis

• What are the causes for failure of scleral buckling surgery?

Preoperative
In hazy media, visualisation of the peripheral retina may be very difficult and retinal
breaks likely to be missed

Operative
Buckle failure - inadequate size
Fish-mouthing - incorrect position
Inadequate buckle height
Missed break

Late postoperative
1. Post op. development of PVR
2. Reopening of retinal break
3. New break

• What are the complications of RD surgery?

Early complications
1. Acute orbital cellulitis
2. Anterior segment ischemia
3. Anterior uveitis
4. Vitritis - as a result of excessive cryo
5. Choroidal detachment
6.  IOP
7. Bacterial endophthalmitis

Late complications

o Explant exposure / infection / migration

o Cellophane maculopathy / Macular pucker
o EOM imbalance
o Changes in refraction

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Retinal Detachment

• What is fish mouthing?

This refers to a tendency of retinal tears (typically large `U' shaped equatorial
tears located superiorly in a bullous RD) to open widely following scleral
buckling and drainage of SRF.
Fish mouthing of a large retinal tear may result in failure to reattach the retina.

• How to prevent fish-mouthing?

By using a radial buckle
1. Add extra radially orientated buckling material.
2. Inject air into vitreous cavity and position the patient postoperatively so that the
bubble is closing the tear.

• What are the indications for vitrectomy in RD?

1. RD associated with very large, posterior breaks and severe PVR
2. Tractional RD which involves threatens the fovea

• What are the steps in vitrectomy for rhegmatogenous RD?

Complete vitrectomy (with PVD induction)
Membrane peeling if PVR present
Internal SRF drainage
Retinal attachment with fluid-air exchange
Retinopexy with laser / cryo to seal the break
Internal tamponade with Gas (SF6 or C3F8) or Silicone oil
Closure of sclerotomies

• What are the gases used for internal tamponade?

SF6- Sulphur hexafluoride
C2F6 – Perfluoroethane
C3F8 – Perfluoropropane (commonly used)

• What is the isoexpansile concentration of gases used during vitrectomy?

20 % for SF6
16% for C2F6
14 % for C3F8

• What is the duration for spontaneous absorption of various gases in the post-
operative period?
Air: 5-7 days
SF6: 2 weeks
C2F6: 4-5 weeks
C3F8: 6-8 weeks

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Retinal Detachment

• What is the difference between gas and silicone oil used for internal
tamponade?

Gas Silicone Oil

Internal For short-term (2-8 weeks) For long term (3-6 months)
Tamponade
duration
Surface Tension 30 times more Less compared to Gas
Buoyancy Higher Less compared to Gas
Air travel in the Not advisable Can travel by air
immediate post-op
Need for another No - since the gas is Yes – another surgery
surgery spontaneously absorbed required to remove the
silicone oil (since in long-
term, silicone oil is
retinotoxic)

• What are the commonly used viscosities of silicone oil?

1000 and 5000 centistokes

• What are the complications of long-term use of silicone oil?

Oil emulsification
Band keratopathy
Increased IOP
Retinotoxicity

• What is inverse hypopyon?

Emulsified oil bubbles float up and get accumulated in the superior portion of AC
and give an appearance of hypopyon

• What is the site of iridectomy if silicone oil is injected in an aphakic eye?

Inferiorly – since the silicone oil bubbles tend to float up

• What are Perfluorcarbon Liquids (PFCL)?

Heavy liquid
Used only as an intra-operative tool – to manipulate or flatten the retina
Removed at the end of the surgery

• What is Pneumatic Retinopexy?

Injection of gas bubble into the vitreous cavity for attaching the retina

• What are the indications for Pneumatic Retinopexy?

Subclinical RD involving 2-3 clock hours due to superior, small and anterior break

• What gas is normally used?

100% SF6
For pneumatic retinopexy, expansile 100 % gas is used
For internal tamponade foll. vitrectomy, isoexpansile lower gas concentration used

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Retinal Detachment

• What is the expansion timing of each gas?

100 % SF6: expands approximately 2 times over 1-2 days
100% C2F6: expands approximately 3 times over 1-2 days
100 % C3F8: expands approximately 4 times over 3-4 days

• What are the steps in Pneumatic Retinopexy?

Retinal cryopexy – to seal the break
Injection of Gas bubble
Paracentesis – if the IOP is high
Positioning of the patient

• What are the two characteristics of gas that help in Pneumatic Retinopexy?
Surface tension
Buoyancy

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Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Retinal Detachment

60
 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus CSR

Central Serous Retinopathy

• What is CSR?
An apparently spontaneous edema develops in the macular area, frequently of obscure
etiology usually self limiting but often recurrent.

• What are the types of CSR?

a.94% fluid under the neurosensory retina
b.3% RPE alone is detached
c. Intermediate 3% - both are elevated

• What do you mean by metamorphopsia and micropsia?

Metamorphopsia means distortion of objects - due to alteration in the arrangement of cones.
Micropsia means objects appearing small - due to spreading apart of foveal cones by fluid

• How will you demonstrate metamorphopsia clinically?

Amsler grid test

• What type of field defect & refractory error will the patient have?
Central positive scotoma - Amsler grid test
Increased hyperopia.

• What is the pathogenesis of CSR?

Interruption of RPE barrier results in free diffusion of substances in both retinochoroid and
chorioretinal direction.
In CSR, the impaired RPE cells begin to secrete large amounts of ions in the chorioretinal
direction so that choroidal fluid is attracted into this area.

• What are the risk factors for CSR?

Raised cortisol level
Stress
Type A personality

• What are the fundus findings in CSR?

• Elevated retina at the macula
• ring reflex at the edge of the CSR
• yellowish- white sub-retinal spots

• What are the stages of CSR?

Pathologically:
Stage I Localised serous detachment (partially affection RPE)
Stage II Widespread serous detachment extensive neurosensory detachment.
Stage III Stage of resolution begins in a few weeks times & complete without major
complications or severe loss of vision unless the fovea is affected.
This serous fluid absorbs, leaving a few retinal precipitates.

• What are the complications of CSR?

1. Geographic atrophy of PE and choriocapillaries
2. Invasion of sub pigment epithelial space by new vessels
3. Fibrovascular scarring
4. Tearing of RPE
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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus CSR

• What are the signs of chronic CSR?

Macular atrophy / pigmentary disturbances / small drusen like white or yellow spots /
cystoid edema at macula / lamellar hole if ILM ruptures.

• What is Amsler Grid Test?

Evaluates the 10 degree of visual field surrounding fixation.
Primarily used for screening of macular disease and also in the diagnosis of subtle optic
nerve lesion
10 cm squares divided into smaller 5mm squares.
Viewed at 33 cm
Each small square subtends an angle of 1 degree.
The patient should wear reading spectacles and cover one eye. He is asked to look directly
at the center dot with the uncovered eye and report any distortion, wavy lines, blurred areas
or blank spots anywhere on the grid. Pt. should draw lines (Macular dazzling test).

• What is Photostress (Macular Dazzling) test?

Principle: persistence of after image; after image will persist for a longer time in CSR.
To differentiate between retinal (macular) and post retinal (optic nerve) disease
Eye is exposed to light for 10 seconds
Time taken for acuity to return to within one line of pre-bleach acuity
Normal: 50-60 sec
Macular pathology: 1.5-3 min

• What will you ‘look for’ in the disc in an eye with CSR?
Optic disc pit

• What are the FFA findings in CSR?

Preretinal phase
Patchy choroidal filling, but no persisting filling defect / Small area of masking
corresponding to the serous RD.

Late transit & last phase

Spot leak or Inkblot

Smokestack appearance leak.

When there is an extensive neurosensory separation, the early spot usually leads to an
elongated shaped leak. The dye on entry to the sub retinal space travels upwards due to the
difference in osmolarity of the serious fluid
.
Mushroom or umbrella - When the top part collapses after reaching a certain height.

Ring shaped leak

when the serous detachment is very localised involving the foveal area above. As the
Xanthophyll pigment in the fovea continues to mask the background florescence, the leak
assumes a ring shape.

Punctate staining - RPE degeneration following resolution.

Leakage point can be single / multiple

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus CSR

• What is the prognosis?

As a general rule, spontaneous resolution with good prognosis but relapses at intervals of
months or years are common and each attack adversely affects the final visual outlook.

• What are the factors for Good prognosis in CSR?

1. Age less than 55 years
2. Detachment of less than 1 disc diameter
3. Noninvolvement of fovea

• What is the treatment for CSR?

Reassurance that usually resolves spontaneously
Avoid Stress

• What is the indication for laser treatment in CSR?

1. Recurrent
2. Bilateral
3. persistent for 4 months
4. Occupational requirement for early visual recovery

• Where to do laser?
To the site of leakage.

• What is the advantage of laser?

It hastens the symptomatic relief by achieving a speedier resolution of the serious
detachment.
It reduces the chance of recurrence

• Will it affect the final visual outcome?

No

• What is the mechanism of action of laser in CSR?

The coagulation beam destroys the cluster of diseased RPE cells, thus stopping the
secretion of fluid beneath the neurosensory retina.
The resulting scar helps to transport fluid back into the choriocapillaries, allows fluid
movement in a retinochoroidal direction. as prophylactic - Since 80% of leakage points of
recurrent disease occur in the immediate vicinity of first degree leakage point.

• How is laser done?

2 or three burns / at the leakage site / 200 micron spot size / duration of 0.2 second and a
low power intensity to product minimal greying of RPE

• What is the rate of recurrence?

30-50% chance of recurrence within 1 year

• What are the drugs causing macular edema?

Benzalkonium chloride, Carmustine (BCNU), Docetaxel, Paclitaxel, Fingolimod,
Glitazones, Niacin, Prostaglandin analogue, Tamoxifen, Timolol

• What is the reason for CSR  in pregnancy?

Due to raised cortisol levels

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus CSR

• Is there a role for anti-VEGF in CSR?

No- except in secondary CNVM

64
 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Macular Hole

Macular Hole

• What are the clinical signs of full-thickness macular hole?

Central, Punched-out, Red Spot in the Macula
1/3-2/3 disc diameter in size
Surrounded by gray halo
Yellow dots in the macular hole
Overlying operculum

• What clinical test would you do to confirm macular hole?

Watzke-Allen test

• How is it done? What are the test results and their significance?
Watzke- Allen test – slit beam focused at the macula with 90 D or 78 D lens / patient asked to
describe if the slit light is Continuous, Broken or Distorted
Full thickness - Broken slit with a central gap
Partial thickness macular hole – Kinked / distorted in the centre but no discontinuity / gap in the slit
light
No Macular hole – Continuous with no distortion or discontinuity / gap

• What are the causes for true macular hole?

Idiopathic / Age-related
Trauma
High myopia
Solar retinopathy

• Is there any sex predilection for age-related macular hole?

Females more commonly affected

• What do patients complain of?

Decreased vision / Central scotoma / Distortion
Initially asymptomatic if unilateral

• What vision would you expect in a patient with macular hole?

Depends on the size, location and stage of macular hole;
Rough estimate
Stage 1 – 6/9
Stage 2 - 6/12
Stage 3 – 6/18 to 6/36
Stage 4 - 6/60

• How would you confirm central scotoma clinically?

By Amsler Chart Monitoring

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Macular Hole

• Is defect in Amsler chart specific for macular hole?

No – noted in other macular pathologies like edema, scar, haem., atrophy

• Why is macula prone for hole formation?

Since fovea is thin / avascular / devoid of support

• What is the mechanism of macular hole formation?

Anteroposterior / Oblique vitreous traction
Tangential traction
Involutional changes in the retinal layers

• What are the stages of macular hole based on clinical findings?

Gass Staging
Stage 1 a– Impending hole (Foveal pseudocyst)
Loss of foveal depression with Yellow Spot in the centre of fovea

Stage 1 b – Occult hole

Loss of foveal depression with Yellow Ring in the centre of fovea

Stage 2 – Small full-thickness hole (less than or equal to 400 microns)

Enlargement of the foveal ring

Stage 3 – Full thickness hole (> 400 microns) with cuff of SRF, incomplete PVD
Round, punched out area / gray halo at the edge / yellow deposits in the base of the hole

Stage 4 - Full thickness hole with cuff of SRF, with complete PVD (Weiss ring present)

• What is Stage 0 Macular hole?

OCT diagnosis based on oblique foveal vitreoretinal traction before the appearance of clinical
changes.

• What are the histological changes in each stage of macular hole?

Stage 1a: the inner retinal layers detach from the underlying photoreceptor layer, often with
the formation of a cyst-like schisis cavity

Stage 1b: With loss of structural support, the photoreceptor layer commonly undergoes
centrifugal displacement

Stage 2: A dehiscence is present in the inner retina with persistent vitreofoveolar adhesion

Stage 3: An operculum consist primarily of glial tissue and condensed vitreous cortex,
(though 40% contain photoreceptor elements) para foveal attachment of the vitreous
cortex still present
Stage 4: Complete PVD – Vitreous detached in parafoveal area and also at the disc

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Macular Hole

• What is the reason for yellow spot in Stage 1 macular hole?

Due to increased visibility of Xanthophyll pigment

• Is the yellow spot pathognomonic of macular hole?

No

• What are the causes of foveal yellow spot?

Stage 1 Macular hole
Adult vitelliform macular dystrophy
Solar retinopathy
Laser pointer retinopathy
CMO

• What is macular microhole?

Small full thickness foveal retinal defect – less than 150 microns

• Is the operculum in macular hole a true or pseudo retinal operculum?

Pseudo-operculum as it contains predominantly glial tissue and condensed vitreous cortex

• What is the investigation of choice for macular hole?

Optical Coherence Tomography

• How does OCT help in macular hole?

To diagnose and confirm macular hole
To classify the stage of macular holes
To measure the size of the hole and help in giving the prognosis
In the follow-up of macular hole – for progression

• What is Vitreomacular Traction Disease?

Abnormalities of focal or broad vitreoretinal adhesions in the presence of detaching or detached
posterior vitreous

• What are the 3 recognised categories of vitreomacular traction diseases?

Vitreo Macular Adhesion (VMA)
Vitreo Macular Traction (VMT)
Macular Hole

• What is IVTS Classification?

International Vitreomacular Traction Study Group classification of VMA, VMT and macular hole
based on OCT findings
VMA – Vitreomacular adhesion
VMT- Vitreomacular Traction

67
 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Macular Hole

Small (<250 microns) or medium (250-400 microns) FTMH with VMT

Medium or large FTMH with VMT
Small, medium or large FTMH without VMT

• What is the chance of other eye developing macular hole?

10%

• What would you advise to identify macular hole in early stage in the uninvolved eye?
Home Amsler chart monitoring

• If the other eye already has complete PVD, will it still develop macular hole?
Very unlikely

• Can macular hole resolve spontaneously?

Yes – Stage 1 and sometimes Stage 2 holes

• Can age-related macular hole cause retinal detachment?

Usually not

• Which type of macular hole is at risk of developing RD?

Myopic macular hole

• What is lamellar macular hole?

Partial thickness macular hole

• Which layer is involved in lamellar hole?

Inner retinal layer; outer layer intact

• How to differentiate between full thickness and partial thickness macular hole?
Full Thickness Macular Lamellar Macular Hole
Hole
Colour Red spot Not red
Surrounding Gray Halo Present Absent
Watzke-Allen test Central gap in the slit Central thinning (no gap) in
the slit
OCT Defect in both outer and Defect only in the inner
inner retina retina

• What are the causes for lamellar macular hole?

Cystoid Macular Edema
Epic-retinal membrane
Vitreomacular traction

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Macular Hole

• Does surgery help in the treatment of lamellar macular hole?

No

• Can lamellar hole progress to full-thickness macular hole?

Very rare

• What is pseudo-macular hole?

Clinical appearance in the fundus looks like macular hole, but there is no actual macular hole

• What are the causes for pseudo macular hole?

Epiretinal membrane
Macular haemorrhage

• How to differentiate pseudo-macular hole from true macular hole?

By Watzke-Allen test and OCT
True macular hole – Watzke-Allen positive; OCT shows defect in the retinal layers
Pseudo-macular hole- Watzke –Allen negative; OCT shows intact retinal layers

• Is FFA required for macular hole?

No – OCT is the main diagnostic test for macular hole management

• What are the FFA findings in macular hole?

Early foveal hyperfluorescence due to window-defect with no evidence of leakage in the late phase.

• What is the treatment of macular hole?

Stage I : Observe – can resolve spontaneously
Stage II-IV: Vitrectomy Surgery

• What are the steps in vitrectomy surgery for macular hole?

Core vitrectomy and PVD Induction (to relieve the antero posterior traction)
Internal Limiting Membrane Peeling (to relieve the tangential traction)
Internal tamponade – gas / silicon oil (for hole closure)

• What are the 3 thin, transparent tissues in vitreoretinal interface?

Posterior hyaloid membrane
Epiretinal membrane
Internal limiting membrane

• How to identify them during surgery?

Using vital dyes and drugs
Posterior vitreous - Triamcinolone acetate
ERM - Trypan blue (does not stain ILM)
ILM- Methylene blue, ICG

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Macular Hole

• What are the complications of macular hole surgery?

Cataract formation and progression
Iatrogenic retinal break
Retinal detachment
Hole non-closure / late reopening
Endophthalmitis

• What are the types of macular hole closure following surgery?

Type 1 – Macular hole closure without neurosensory defect
Type 2 – Macular hole closure with neurosensory defect

• Is there a pharmacological treatment option for macular hole?

Yes – recently pharmacologic vitreolysis tried

• What is pharmacologic vitreolysis?

Intravitreal injection of Ocriplasmin for macular holes associated with VMT
Degrades the macromolecular vitreous attachment complex and relieves the vitreoretinal traction

• What is Ocriplasmin?
Recombinant protease which acts against fibronectin and laminin, found in the vitreoretinal
interface

70
 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Retinitis Pigmentosa

Retinitis Pigmentosa

• What are the fundus findings suggestive of RP?

The clinical triad of arteriolar narrowing, waxy pallor of the disc and bone-spicule pigmentation

• What is RP?
Hereditary diffuse retinal dystrophy predominantly affecting the rods

• How is it inherited?
Autosomal Dominant – most common, best prognosis
Autosomal Recessive
X-linked Recessive – least common, most severe
Sporadic

• How common is RP?

1in 4000 - 5000

• What is the earliest symptom of RP?

Delayed dark adaptation

• What do you ask the patient to elicit the history of delayed dark adaptation?
Difficulty to identify objects while entering a dark room
Difficult to identify the seat in theatres

• Why do you get pigmentary changes in RP?

Due to photoreceptors' degeneration, atrophy in the outer retina and pigment epithelium, and
RPE cells migrating into the retina

• Is bone-spicule pigmentation pathognomonic of RP?

No

• What are the other conditions that can cause bone-spicule pigmentation?
Syphilitic Chorioretinitis
Chloroquine retinopathy
Thioridazine retinopathy
Cancer-related retinopathy

• What are the macular changes in RP?

CME, Atrophic and Cellophane maculopathy

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Retinitis Pigmentosa

• What are the ocular associations in RP?

Refractive error (Myopia in X linked disease, Hyperopia in autosomal dominant disease)
Keratoconus
Open-angle glaucoma
Posterior sub-capsular cataract
Intermediate uveitis
Vitreous detachment
Optic nerve head drusen

• What are the types of atypical RP?

Retinitis Pigmentosa sine pigmento - clinical features of typical RP without no pigmentary changes
Sector RP – pigmentation in one quadrant (nasal) or one half (inferior)
Pericentral RP – pigmentation along the arcades
RP with exudative vasculopathy

• What is Retinitis Punctata Albescens (RPA)?

Variant of RP
Seen in children
Characterised by small white spots in the fundus without pigmentary changes

• What is the fundus finding in female carrier of X-linked recessive type?

Normal or golden metallic tapetal reflex and pigmentary changes / salt-and-pepper fundus

• What are the systemic associations with RP?

Usher syndrome – deafness
Refsum disease
Bassen-Kornzweig syndrome
Kearns-Sayre syndrome – mitochondrial cytopathy
Bardet-Biedel syndrome

• What are the conditions associated with RP and deafness?

Usher’s syndrome, Refsum’s disease, Cockayne’s syndrome and Hallgren’s syndrome

• What are the conditions associated with RP and mental retardation?

Lawrence-Moon-Bardet-Biedl syndrome, Cockayne’s syndrome, Hallgren’s syndrome

• What is Usher syndrome?

Autosomal recessive inheritance
RP before puberty with Labrynthine Deafness

• What is Refsum disease?

Phytanic acid accumulation due to phytanic acid 2-hydroxylase deficiency
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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Retinitis Pigmentosa

Ocular findings: salt and pepper retinopathy, prominent corneal nerves, miosis, cataract
Systemic: Polyneuropathy, cerebellar ataxia, deafness, anosmia, and cardiomyopathy, icthyosis,
and cytoalbuminous inversion (elevated CSF protein in the absence of pleocytosis)
Treatment with plasmapheresis and phytanic-acid free diet

• What is Laurence-Moon-Bardet-Biedl syndrome?

RP, bull’s eye maculopathy with obesity, hypogenitalism, mental deficiency and polydactyly

• What is Bassen-Kornzweig syndrome?

Due to betalipoprotein deficiency
Retinal pigmentation larger than in RP and not restricted to the equatorial area, Ophthalmoplegia,
Ptosis
Treatment with Vit E

• What is Cockayne’s syndrome?

RP with progressive infantile deafness, dwarfism, mental retardation, nystagmus and ataxia

• What is Hallgren’s syndrome?

RP with vestibulo-cerebellar ataxia, congenital deafness and mental deficiency
• What are the typical field defects in RP?
Mid-peripheral scotomas - Ring scotoma – Constriction of peripheral field – Tubular field

• What is the cause for ring scotoma?

Due to involvement of equatorial / mid-peripheral retina

• What is the most important investigation for RP?

ERG – used to confirm the diagnosis, assess the severity and progression of RP

• What are the typical ERG findings in RP?

Initially reduced scotopic rod and combined responses
Later reduced photopic response

• Can ERG changes occur early in the disease before objective changes in the fundus?
Yes

• How is ERG done?

Active electrode embedded in a contact lens placed on the patient’s cornea
Reference electrode on the patient’s forehead
Potential between the two electrodes amplified and recorded
Done in light-adapted and dark-adapted conditions

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Retinitis Pigmentosa

• Describe the normal ERG wave

Initial negative a-wave - arising from the photoreceptors
Positive b-wave – due to Muller cells in bipolar cell region,
c- wave – from RPE

• What are the components of b-wave?

b1 –represents both rod and cone
b2 – represents predominantly cone

• What are the 5 recordings in ERG?

Scotopic ERG
Rod response
Combined
Oscillatory potential
Photopic ERG
Cone response
Cone flicker

• How will you differentiate between RP and Congenital Stationary Night blindness with
ERG?
Electronegative b wave in CSNB, not in RP

• What are the retinal conditions with electronegative b wave in ERG?

CSNB
X-linked Retinoschisis
Inner Retinal dystrophy
Batten disease

• What are the 3 main groups of retinal dystrophies diagnosed on ERG?

Rod-Cone dystrophy (RP): Scotopic reduced amplitude
Cone-Rod dystrophy (Cone Dystrophy): Photopic reduced amplitude
Inner retinal dysfunction: (CSNB): electronegative b wave

• What is dark-adaptometry?
Measurement of dark adaptation using Goldmann-Weekes adaptometer
Complimentary to ERG as it is a focal test
Measures the absolute thresholds of cone and rod sensitivity
Useful in evaluating night-blindness and also cone dysfunction syndromes
Represented by a bipartite sensitivity curve – initial rapid cone function segment and a slower rod
function segment

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Retinitis Pigmentosa

• What are the treatment options tried for RP?

Diet rich in Vit A and Lutein
Vitamin E in Bassen-Kornzweig syndrome
Plasmapheresis and phytanic acid- free diet in Refsum’s disease
Oral Acetazolamide if cystoid macular edema present
Retinal chips
Gene therapy
Low vision aids
Rehabilitation
Genetic counselling

75
 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Macular Dystrophy

Macular Dystrophy

• What are the fundus findings suggestive of Stargardt’s?

Foveal mottling
Oval lesion at the macula – ‘snail-slime’ or ‘beaten bronze’
Yellow-white flecks
Late stage – RPE and choriocapillaries atrophy

• What is the age of presentation of Stargardt’s macular dystrophy?

10-20 years

• What is the inheritance?

Autosomal recessive

• Is Stargardt’s different from Fundus Flavimaculatus?

Both variants of the same disorder
Stargardt’s – early presentation in 1st or 2nd decade
Fundus Flavimaculatus – late presentation in 4th or 5th decade

• Why do you get flecks in Fundus Flavimaculatus?

RPE atrophy secondary to lipofuscin-like substance

• What are the FFA findings in Stargardts?

Silent choroid / Midnight fundus

• Is ‘silent choroid’ pathognomonic of Stargardt’s disease?

No, but a useful FFA finding in favour of Stargardt’s

• Why do you get Silent Choroid in Stargardt’s?

Lipofuscin in the RPE causes blockage of choroidal fluorescence

• How to differentiate between fundus Flavimaculatus and drusen?

Fundus Flavimaculatus Drusen
Yellow-white lesion Flecks Spots
FFA Not all lesions hyper All lesions hyper fluoresce
fluoresce

76
 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Macular Dystrophy

Best’s Dystrophy

• What are the stages of Best’s dystrophy?

Previtelliform – no fundus finding / abnormal ERG
Vitelliform – Egg-yolk / sunny-side up macula
Pseudohypopyon – lesion partly absorbed
Vitelliruptive – ‘scrambled egg’ appearance
Endstage – scar / atrophy / CNVM

• Why do you get egg-yolk macula in Best’s disease?

Due to accumulation of yellow lipofuscin granules between RPE and sensory retina in macula

• What is the inheritance pattern?

Autosomal dominant

• What gene is responsible for Best’s disease?

Bestrophin 1 gene – chromosome 11q12-13

• What is Bestrophinopathy?
Collective term for a variety of ocular diseases with a broad phenotypic spectrum mutation of
Bestrophin gene

• What are the five major categories of Best’s Retinopathy?

Best Vitelliform Macular Dystrophy (BVMD)
Adult-onset Vitelliform Macular Dystrophy (AVMD)
Autosomal dominant vitreo retino choroidopathy (ADVIRC)
Retinitis Pigmentosa 50 (RP50)
Autosomal recessive Best’s retinopathy (ARB)

• Which layer of retina does the Best’s disease affect?

RPE

• What is the most important test to confirm Best’s disease?

Electro Oculo Gram (EOG)

• How is EOG done?

Electrodes placed on the skin near the medial and lateral canthi
Patient asked to move the eye on either side
When the eye moves, the cornea makes the nearest electrode positive with respect to the other
The potential difference between the two electrodes amplified and recorded
Test is done in both light-adapted and dark-adapted tests

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Macular Dystrophy

• What does EOG measure?

Standing potential between the electrically positive cornea and the electrically negative back of the
eye
Reflects the activity of the RPE and photoreceptors

• What is this standing potential?

Voltage at the RPE, generated due to the different ionic permeability characteristics of the junctions
at the apical and basal membranes of RPE

• What is light peak and dark trough?

Light peak -Maximum height of the potential in the light
Dark trough – Minimal height of the potential in the dark

• What is Arden ratio?

Light peak by dark trough ratio
Normal value 1.85 or above

• What are the characteristic EOG findings in Best’s disease?

Loss of the light response
Arden ratio less than 1.5 and often near 1.1

• What will be the EOG finding in Best disease gene carrier but with normal fundus?
Abnormal even if the fundus is normal

• What is the ERG finding in Best’s disease?

Normal ERG

• What is the term used if ERG is normal and EOG abnormal?

ERG-EOG dissociation

• What are the other causes for ERG-EOG dissociation?

Diffuse fundus Flavimaculatus
Pattern dystrophy (Butterfly dystrophy)

• What is the role of FFA in Best’s disease?

To rule out CNVM

• What are the OCT findings in Best’s disease?

To localise the vitelliform lesion to the sub retinal space
To demonstrate thickening o the cone outer segments
To evaluate fluid associated with CNVM

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Macular Dystrophy

• What are the atypical presentations of Best’s disease?

Multifocal vitelliform lesions
Normal fundus and reduced EOG in family members manifesting incomplete penetrance
Unilateral vitelliform lesion with bilateral reduced EOG

• What is the treatment for Best’s disease?

No medical or surgical treatment
Observe
If CNVM present, laser photocoagulation for extra-foveal CNVM and anti-VEGF for subfoveal
CNVM

• What is the visual prognosis in Best’s disease/

Usually good – Retain good vision until sixth decade

• What is adult vitelliform dystrophy?

Lesions similar to Best’s disease, but occurs in adults
Also called Foveomacular dystrophy

• How will you differentiate adult vitelliform dystrophy from Best’s vitelliform dystrophy?
EOG – normal in adult vitelliform and abnormal in Best’s vitelliform dystrophy

79
 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus ARMD

Age-Related Macular Degeneration

• What are the findings suggestive of ARMD?

Hard Drusen, RPE atrophy, pigmentary changes in macular area, old age, bilaterality suggestive of
Dry ARMD
Soft Drusen, Greyish membrane, Haemorrhage, edema in macular area, old age, bilaterality
suggestive of Wet ARMD

• What are the other names for dry and wet ARMD?
Non-Neovascular, Non-exudative or Atrophic ARMD for Dry ARMD
Neovascular or Exudative ARMD for Wet ARMD

• What will be the patients complain of?

Blurring of central vision
Distortion

• How will the patient with advanced ARMD see?

By eccentric viewing
Will look away from the object of interest to bring the object to the peripheral field of vision

• Can a patient with ARMD become completely blind?

No – the peripheral vision will be retained

• Who are all at risk for developing ARMD?

Older individual
Whites > Hispanics and Asians>African Americans
Positive family history
Females
Men with higher waist-to-hip ratio
Patients with Hypertension, hypercholesterolemia, cardiovascular disease, elevated levels of C-
reactive protein and other inflammatory markers
Cigarette smokers
Hyperopes
Patients with Light iris color.

• What are the normal ageing changes in the macula?

Predominantly affects the outer retina, Retinal pigment epithelium (RPE), Bruch membrane, and
choriocapillaries
Many clinically undetected
Photoreceptors: reduced in density and distribution
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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus ARMD

Retinal Pigment Epithelium

o loss of melanin granules, formation of lipofuscin granules, and accumulation of residual
bodies
Bruch’s membrane: Basal laminar/linear deposits accumulate
Choriocapillaries: Progressive involutional changes

• What is the defining lesion of dry / non-neovascular ARMD?

Drusen

• What is the defining lesion of wet / neovascular ARMD?

Choroidal Neovascular Membrane (CNVM)

• What is drusen?
Abnormal thickening of inner aspect of Bruch’s member appearing as
Round, dull yellow lesion in outer retina, usually seen in the posterior pole

• What is the reason for the thickening of Bruch’s membrane?

Due to extracellular deposition of abnormal material (lipid and protein) in Bruch’s membrane,
derived from RPE

• What is the anatomical location of the deposition of abnormal material in drusen?

Between basal lamina of the RPE and inner collagenous layer of Bruch’s membrane

• What are the 5 layers of Bruch’s membrane?

Basement membrane of the RPE
Inner collage nous layer
Middle elastic layer
Outer collage nous layer and
Basement membrane of choriocapillaries endothelium

• What is the source of lipofuscin in RPE?

A by-product of incomplete digestion of outer segments of photoreceptors

• What are the types of drusen?

Based on the boundaries of drusen
Hard drusen –distinct margins
Soft drusen –indistinct margins
Confluent – contiguous boundaries between drusen

Based on the size

Small – less than 64 microns
Intermediate – 64-125 microns
Large – greater than 125 microns
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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus ARMD

• What is Druplet?
Another name for small drusen less than 64 microns

• Which is more significant – hard or soft drusen?

Soft drusen
Can coalesce over time to form a large drusenoid RPE detachment,
Can be a precursor for CNVM

• What is the effect / fate of drusen in the long-term?

Can cause overlying RPE atrophy
Coalesce and cause drusenoid RPE detachment
Can be a risk factor for CNVM
Calcify
Regress

• What are the causes for visual loss in ARMD?

Geographic atrophy in Dry ARMD
CNVM in Wet ARMD

• What is AREDS classification of AMD?

No AMD - no or few small drusen ((63–124 µm in diameter), or mild RPE abnormalities

Early AMD - a combination of multiple small drusen, few intermediate drusen (63–124 µm in
diameter), or mild RPE abnormalities.

Intermediate AMD - any of the following features

numerous intermediate drusen 125 µm in diameter
At least one large drusen > 125 microns
Geographic atrophy not involving the center of the fovea

Advanced AMD - one or more of the following (in the absence of other causes) in one eye
Geographic atrophy of the RPE involving the foveal center
Neovascular maculopathy

• What are the risk factors for progression of ARMD?

Increasing number, size and coalescence of drusen and increased RPE pigmentation are risk factors
for development of geographic atrophy or CNVM

• Can drusen occur in less than 50 years of age?

Yes

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus ARMD

• What is the difference between Focal atrophy and Geographical atrophy?

Focal atrophy: non-contiguous areas of pigment mottling or frank depigmentation.
Geographic atrophy (GA) of the RPE: if areas of focal atrophy become contiguous and have a
diameter greater than 175 μm

• Why is it called Geographic atrophy?

Since it looks like a map

• What are the OCT findings in dry ARMD?

Drusen
Appear as sub-RPE nodular elevations or even small RPE detachments with a notable absence
of intraretinal and sub retinal fluid
Reticular pseudodrusen are identified above the RPE and beneath the inner segment ellipsoid
layer and are graded according to their degree of elevation

RPE Atrophy
Progressive loss of RPE, the overlying inner segment ellipsoid, and the photoreceptor layers

Focal RPE hyper pigmentation

Hyper reflective outer retinal foci

• How does autofluorescence help in dry ARMD?

Used to monitor the disease progression by measuring the areas of GA, which are densely
hypoautofluorescent;
Reticular pseudodrusen: the location of reticular pseudodrusen above the RPE

• What will be the FFA findings in Dry ARMD?

Drusen
Small hard drusen: hyperfluorescein early stage because of a window defect
Larger soft and confluent drusen and drusenoid PEDs: slowly and homogenously stain late
because of pooling of the fluorescein dye in the sub-PED compartment.

RPE Atrophy
Causes window defects – early hyperfluorescence with decrease in intensity in late phase

RPE hyper pigmentation

Causes blocked choroidal fluorescence

• What would you advise a patient with dry ARMD?

Low vision aid
Diet rich with anti-oxidants and vitamins
Anti-oxidant and mineral supplements
Home Amsler chart monitoring
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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus ARMD

• How is Amsler chart testing done?

Keep the Amsler chart at reading distance
Focus on the central dot
3 questions to be answered
Are all the four corners seen?
Are all the small squares seen or is there an area of scotoma where the squares cannot be seen?
Are all vertical and horizontal lines straight or distorted in any area?
Tests the central 10 degrees of field

• What is the purpose of Home Amsler chart monitoring?

To suspect the development of CNVM

• What is AREDS Study?

Age Related Eye Disease Study

Aim
To assess the clinical course, prognosis and risk factors for both ARMD and Cataract
To evaluate the effect of high doses of vitamin C, vitamin E, beta-carotene and zinc on the
progression of AMD and cataract

Result
Showed that high levels of anti-oxidants and Zinc
- can reduce the risk of progression of ARMD
- had no significant effect on the development or progression of cataract

• What did the AREDS formulation contain?

Vitamin C - 500 milligrams
Vitamin E - 400 IU
Beta-carotene 15 mg
Zinc – 80 mg as Zinc oxide
Copper – 2 mg as cupric oxide

• What was the reason for adding copper in AREDS formulation?

Since zinc in high doses can inhibit copper absorption at the RBC level, causing copper-deficiency
anaemia

• What is the risk of using supplement with beta carotene in smokers?

Beta carotene can increase the risk of lung cancer

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus ARMD

• What is AREDS 2?
AREDS 2 study
Aim
To study whether adding Omega 3 fatty acids and lutein and zeaxanthin (instead of beta
carotene) further reduced the risk of progression to advanced ARMD
To test the effects of eliminating beta carotene and reducing the zinc dose from the AREDS
formulation

Result
Adding omega-3 fatty acids had no effect on the formulation
Lutein and zeaxanthin together appeared to be a safe and effective alternative to beta-carotene
Lower zinc oxide doses (25mg) did not significantly increase the risk of advanced AMD,
although a trend to more protection from advanced AMD was noted with higher zinc oxide
doses (80mg)

• What do the newer formulations contain?

Vitamin C - 500 milligrams
Vitamin E - 400 IU
Copper – 2 mg as cupric oxide
Zinc -80 mg as Zinc oxide - 80 mg
Lutein 10 mg and Zeaxanthin 2 mg
No beta carotene

• Can drusen be treated with laser? If so, is it useful?

Laser treatment has been tried for drusen
It can cause resolution or reduction of drusen, but does not affect the natural course of vision loss or
CNVM
Not done much now for drusen

• What is the tell-tale sign of CNVM during fundus evaluation?

Subtle grey-green discolouration of the fovea
Can be seen as slight retinal elevation with fundus biomicroscopy

• When would you suspect the conversion of dry ARMD to wet ARMD?
Sudden distortion or decrease in central vision
Presence of grey-greenish membrane, edema, exudates at the edge of RPE atrophy, haemorrhage

NOTE
A patient with dry ARMD presenting with sudden distortion should be investigated (OCT & FFA)
to rule out the development of CNVM even if the fundus examination did not reveal any obvious
sign of CNVM

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus ARMD

• What is the single most important basic underlying prerequisite for the development of
CNVM of any cause?
Any disturbance of Bruch’s membrane

• What is the pathophysiology of CNVM in general?

Disturbance in Bruch’s membrane- predisposes to new vessel formation from choriocapillaries
throughout the outer aspect of Bruch’s membrane - accompanied by fibroblast – Fibrovascular
proliferation along the inner aspect of the Bruch’s membrane – disrupt and destroy the normal
architecture of the choriocapillaries, Bruch’s membrane and the RPE – later the photoreceptors and
outer retina

• What causes the disturbances in Bruch’s membrane in ARMD?

Drusen

• How is CNVM classified anatomically based on its level of origin?

Type 1 – Sub-RPE
Type 2 – Sub-retinal (between RPE and neurosensory retina)
Type 3 – Intra-retinal (arises from the deep capillary plexus in retina and grows towards RPE)
Previously called RAP (Retinal Angiomatous Proliferation)

• What are the complications of wet ARMD?

RPE Detachment
Sub-RPE or sub-Retinal haemorrhage
RPE rip/tear
Exudative / haemorrhagic retinal detachment
Disciform scarring
• What are the causes for CNVM other than ARMD
High Myopia
Angioid Streaks
Choroidal Rupture
Ocular Histoplasmosis syndrome
Laser Photocoagulation
Posterior Uveitis
Idiopathic

• Is retinal pigment epithelial detachment (PED) indicative of Wet ARMD?

Not necessarily – PED can occur without CNVM; as age-related change

• What is Retinal pigment epithelial detachment (PED)?

Separation of the RPE from Bruch's membrane
Due to accumulation of drusenoid material, serous fluid and blood, presence of fibrovascular
tissue or a combination in the sub-RPE space

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus ARMD

• What are the types of PED?

Based on the type of material / fluid in the sub-RPE space
Drusenoid PED (drusen)
Serous PED (serous fluid)
Haemorrhagic PED (blood)
Fibrovascular (occult CNVM)

• What can happen to PED?

Can flatten and resolve spontaneously
Progress to Sensory detachment of retina
Become fibrovascular PED with the development of Occult CNVM
RPE tear

• What is the FFA finding in PED?

Well-defined area of early hyperfluorescence, which increases in intensity, but not in the extent, in
late phase

• How do you identify PED clinically on fundus examination?

Seen as sharply circumscribed dome-shaped elevated lesion

• What investigations would you do to rule out CNVM?

OCT and FFA

• What is the advantage of OCT compared to FFA?

Non-invasive

• How is OCT useful in Wet ARMD?

To detect, classify the type of CNVM and monitor the response to treatment

• What are the OCT findings in Wet ARMD?

Type 1 CNVM:
Elevation of the RPE and PEDs
Sub retinal hyper reflective material (SHRM) found between the sensory retina and RPE in eyes
with CNVM

Type 2 CNVM
Appears as a hyper reflective band or plaque between RPE and sensory retina, with associated
sub retinal and/or intraretinal fluid

Type 3 CNVM
Hyper reflective foci arising from the deep capillary plexus of the retina, with or without
associated CME and PED.
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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus ARMD

• How does FFA help?

Helps to detect, identify the location and type of CNVM based on the leakage pattern

• What will be the FFA finding in CNVM?

Early hyperfluorescence in choroidal phase with increase in hyperfluorescence in late phase due to
leakage

• What are the types of CNVM based on FFA findings?

Based on the pattern of fluorescein leakage
Classic
Predominantly Classic – more than 50 % of the lesion is classic
Minimally Classic – less than50 % of the lesion is classic
Occult

Based on location in relation to fovea, classic CNVM classified into

Extra-foveal – more than 200 microns from the centre of fovea
Juxta-foveal – less than 200 microns from, but not involving, the centre of fovea
Sub-foveal – under the centre of the fovea

• How to differentiate between classic and occult CNVM?

Classic CNVM – well-defined lacy pattern
Occult CNVM – ill-defined

• What is the new imaging modality to identify the structural details of CNVM?
OCT Angiogram

• What is the main treatment for Wet ARMD?

Intravitreal anti-VEGF

• What are the different types of anti-VEGF?

Bevacizumab – monoclonal antibody
Ranabizumab – monoclonal antibody fragment
Aflibercept – receptor-antibody fusion protein
Pegaptanib – aptamer (not used now)

• What is the ‘Treat and Extent’ strategy for intravitreal anti-VEFG in CNVM?
Initial monthly injection for 3 months followed by injections in increasing inverval till the CNVM
becomes inactive –no fluid in OCT, no leak in FFA

• What is the role of laser for CNVM?

Can be used for extrafoveal classic CNVM
Not used much with the advent of intravitreal anti-VEGF injections
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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus ARMD

• What are the disadvantages of laser treatment for CNVM?

Increased recurrence of CNVM
Cannot improve the vision
Risk of causing vision loss, especially with sub-foveal CNVM

• Prior to the availability of anti-VEGF, what was the treatment option for sub-foveal CNVM?
PDT

• What is PDT? How is it done?

Photodynamic therapy (PDT)
IV infusion of Verteporfin dye – a photosensitser when exposed to light becomes toxic to targeted
cells after 15 minutes, laser applied
Selective destruction of CNVM

• What are the side-effects of PDT?

Dye related back pain
Allergy at the local injection site

• What is PCV?
Polypoidal choroidal vasculopathy
Initially called posterior uveal bleeding syndrome
A variant of CNV (type 1)
Multiple, recurrent serosanguineous RPE detachments
Network of polyps associated with feeder vessels that adhere to the RPE monolayer of the
Fibrovascular PED in a “string-of-pearls” configuration
Less responsive to anti-VEGF

• What is the investigation of choice for diagnosing PCV?

Indocyanine Angiography (ICGA)

• How is ICG done?

Indocyanine green dye is injected intravenously
Near -infra red light used for the imaging

• What are the advantages of ICG over FFA?

Penetrates the melanin and xanthophyll
Binds to serum proteins and hence, retained within the choriocapillaries, which are impermeable to
larger protein molecules
Hence, useful for deeper choroidal vascular pathology and in eyes with media opacity

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus ARMD

• What is the treatment for PCV?

Anti-VEGF + PDT superior to anti-VEGF alone

• Does cataract surgery worsen ARMD?

No

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Bala Notes for Ophthalmology Post-Graduate Examination: Fundus ARMD

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Optic Atrophy

Optic Atrophy

• What is optic atrophy?

Condition wherein the optic nerve is degenerated final common morphologic endpoint of
any disease process that causes axon degeneration in the retinogeniculate (retina to
lateral geniculate body) pathway.

• Is optic atrophy a diagnosis?

A sign of end-stage optic nerve damage and not a diagnosis in itself

• Can you get optic atrophy in post Lateral Geniculate Body (LGB) lesions involving
visual pathway?
Post-LGB (Optic radiation & Visual Cortex) lesions do not cause optic atrophy

• What is the importance of the sex of the patient with optic atrophy?
Males – Leber’s Hereditary Optic Neuropathy,
Females – Multiple sclerosis (Demyelination / optic neuritis);
Benign Intracranial Hypertension (Secondary optic atrophy)

• What is the importance of personal history in a patient with optic atrophy?

Alcohol and Tobacco use can cause Toxic Optic Neuropathy

• What are the tests for optic nerve function?

Visual Acuity, Colour Vision, Contrast sensitivity, Pupil (RAPD), Disc evaluation, Visual
Fields, VEP, ERG

• What is the reason for the yellow-pink colour of the optic disc?
Total internal reflection of light from the ophthalmoscope through the axonal fibres and
then from the disc surface capillaries

• Why does the disc appear pale in optic atrophy?

Possible explanations
Degenerated axons lose the ability to allow the light to pass through and hence, no total
internal reflection from the disc surface capillaries
Loss of pial capillaries, which supply the optic disc

• What are the causes for “pseudo disc pallor”?

Using a brighter ophthalmoscope than usual
Viewing the disc in a pseudophakic eye

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Optic Atrophy

• What are the causes for non-pathological disc pallor?

Axial myopia
Myelinated nerve fibres
Optic disc pit
Tilted disc
Disc drusen

• What is Kestenbaum index?

Number of capillaries counted on the disc
Around 10: Normal
Less than 6: Disc atrophy
More than 12: Disc hyperaemia

• What are the causes of unilateral optic atrophy?

Optic neuritis, Trauma, Tumours

• What are the causes of bilateral optic atrophy?

Toxic ON, Nutritional ON, Hereditary ON, Post-papilledema, bilateral optic neuritis

• What is the time taken for optic atrophy to manifest following the onset of the optic
nerve disease / insult?
Trauma – 2 weeks
AION – 2 months
Papilloedema – 6-9 months

• What are the histopathological changes in optic atrophy?

Cup deepening with baring of lamina cribrosa
Myelin and axonal loss
Glial cell proliferation
Subarachnoid space widening with redundant dura
Severed end bulbous axonal swellings (Cajal end bulbs) of severed end in nerve transaction

• What are the types of optic atrophy?

Based on
Ophthalmoscopy findings
Primary
Secondary
Consecutive
Glaucomatous (Cavernous)

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Optic Atrophy

Aetiology

Hereditary
Consecutive
Vascular
Toxic or Drug-induced
Metabolic
Demyelination
Pressure atrophy
Post-inflammatory
Traumatic Optic Neuropathy

Pathology

Anterograde degeneration (Wallerian)

Retrograde degeneration

• What is Anterograde degeneration (Wallerian degeneration)?

Degeneration begins in the retina and proceeds toward the lateral geniculate body
(e.g., toxic retinopathy, chronic simple glaucoma)
Larger axons disintegrate more rapidly than smaller axons.

• What is Retrograde degeneration?

Degeneration starts from the proximal portion of the axon and proceeds toward the optic
disc (e.g. optic nerve compression by intracranial tumor)

• What is the ‘common saying’ for Retrobulbar neuritis?

Patient sees Nothing

Doctor sees Nothing (No ocular finding except for RAPD)

NOTE: Similar ‘saying’ also applies to Amblyopic Patients.

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Optic Atrophy

Ophthalmoscopic Primary Secondary Consecutive Glaucomatous Temporal

Classification (Cavernous) Pallor
Causes Pituitary Secondary to Consecutive Increased IOP Toxic ON
tumour, prior disc to retinal Vascular Nutritional
Optic swelling pathology ON
Nerve (Papillitis, e.g.
tumour, Papilloedema, Retinitis
Traumatic AION) Pigmentosa,
ON CRAO
Pathology Nerve Excessive No glial
fibres proliferation proliferation;
degenerate of the glial Hyaluronic
in an tissue acid
orderly accumulation
manner
and
replaced
by
columns
of glial
cells
ONH No Obscuration Normal cup Increased No
Architecture alteration of lamina Cupping of alteration
cribrosa disc, NRR
thinning,
Lamina
cribrosa pores
seen,
Bayonnetting
and
nasalisation of
vessels,
Peripapillary
halo and
atrophy
Disc colour Chalky Dirty grey Waxy pallor Increased CD Pallor
white ratio; NRR only in
thinning temporal
area
Disc margin Well- Ill-defined Well- Well-defined Well-
defined defined defined
Retinal vessels Normal Normal Attenuation Normal Normal

• What does RAPD indicate?

Unilateral or asymmetric afferent sensory defect
Usually seen in Optic nerve dysfunction or gross retinal pathology (usually retinal
ischemia)

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Optic Atrophy

• How do you identify Relative Afferent Pupillary Defect?

Swinging-flash light test

• How is swinging flash light test done?

Bright light used to shine the pupil in one eye and then swung to the other eye, Repeated 3-
5 times.
Normally, pupil constricts when light is shone. If the pupil is dilated when light is shone, it
indicates RAPD

• What is the other name for pupil with RAPD?

Marcus-Gunn pupil

• Can you identify RAPD if one pupil is already dilated and non-reactive?
Can identify RAPD by looking only at the undilated eye’s pupillary reaction - consensual

• How is RAPD clinically graded?

Trace: Initial constriction, but greater escape to a larger intermediate size than when the
light is swung back to normal eye
Grade 1-2+: No changes in initial pupillary size, followed by dilation of the pupils
Grade 3-4+: Immediate dilation of the pupil, instead of normal initial constriction

• How to quantify RAPD objectively?

Using Neutral Density Filter

• What is Pulfrich phenomenon?

In optic nerve damage, the transmission of impulses to the occipital cortex is delayed.
In patients with unilateral or markedly asymmetric optic neuropathy, when an oscillating
small target in a frontal plane is viewed binocularly, the target appears to move in an
elliptic path rather than in a to-and-fro path

• What is contrast sensitivity test?

Measures the ability to perceive slight changes in luminance between regions that are not
separated by definite borders
Sensitive test for optic nerve function.

• What are the contrast sensitivity charts used in clinical practice?

Pelli Robson contrast sensitivity chart, Cambridge low-contrast grating test or Arden
gratings.

• What are the causes for compressive optic neuropathy?

Optic nerve tumours (gliomas or meningiomas)
Orbital masses
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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Optic Atrophy

Pituitary tumours, Craniopharyngiomas, Meningiomas

Internal Carotid Artery and Ophthalmic Artery Aneurysms

• What are the signs of compressive optic neuropathy?

Decreased vision
Field defect based on the location of the lesion
Initially mild disc edema, later optic atrophy
Optociliary shunts
Proptosis

• What is optociliary shunt?

Collateral vessels on the disc that shunt blood from the retinal to the choroidal venous
circulation;
Present normally but enlarge and become visible only when there is a compressive
obstruction to venous drainage by a tumour compressing the optic nerve

• What are the causes of optociliary shunt?

Orbital or intracranial tumour compressing the optic nerve
Optic nerve gliomas
CRVO
Chronic papilloedema
POAG

• What causes “bow-tie pallor” of optic disc?

Chasmal Compressive lesion

• What is Nutritional Optic Neuropathy?

Optic nerve dysfunction due to deficiency of nutrients (B Complex vitamins, Folic acid)
essential for normal functioning of nerve fibres

• What is the treatment for Nutritional Optic Neuropathy?

Vitamin B12, Folic acid, Protein supplements

• What is Toxic Optic Neuropathy?

Optic nerve damage due to toxin

• What are the common causes for Toxic Optic Neuropathy?

Alcohol especially Methanol, Tobacco, Ethambutol, Isoniazid,

• What is Mitochondrial Optic Neuropathy (MON)?

Characterised by mitochondrial dysfunction of the optic nerve

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Optic Atrophy

• How do you classify MON?

Congenital MON
Leber Hereditary Optic Neuropathy (LHON)
Autosomal Dominant Optic Neuropathy (ADON)

Acquired MON
Toxic Optic Neuropathy
Nutritional Optic Neuropathy

• Why are Toxic and Nutritional Optic Neuropathy called acquired mitochondrial
neuropathies?
Toxins and nutritional deficiency affect mitochondrial oxidative phosphorylation, causing
mitochondrial dysfunction in the optic nerve, especially papillomacular bundle

• Why is papillomacular bundle affected in Toxic Neuropathy?

Papillomacular bundle is more susceptible to toxin damage

• What are the signs of Toxic and Nutritional Optic Neuropathy

Since both toxic and nutritional optic neuropathy cause optic nerve dysfunction by similar
mechanism, clinical signs are also similar
Decreased vision, optic disc pallor, bilateral, central or centrocaecal scotoma, colour vision
affected

• What are the causes of Cavernous optic atrophy?

Glaucoma
AION
Methyl alcohol poisoning

• What are the types of field defects seen in optic atrophy?

Altitudinal– AION
Constriction of peripheral fields – Glaucoma
Centrocaecal - Optic Neuritis, Optic Nerve Tumours, Compressive optic neuropathy, Toxic
& Nutritional Optic Neuropathy,
Bitemporal hemianopia – Chiasmal

• What are the ERG findings in optic neuropathy?

Subnormal: Potential less than 0.08 microvolts; seen in toxic neuropathy
Negative: a preserved a-wave but absent b-wave.
May be seen in arteritic AION or central retinal artery occlusion.
Extinguished ERG: seen in complete optic atrophy.
N95:P50 ratio in pattern ERG is low in optic neuropathy and normal in maculopathy

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus Optic Atrophy

• What are the VEP findings in optic neuropathy?

Increased latency period
Reduction of amplitude and waveform changes in compressive optic neuropathy

• What are the investigations you would advise if the cause for optic atrophy is
unknown even after complete clinical examination?
Blood glucose, BP, CVS Examination, Carotid Doppler Ultrasound,
Venereal Disease Research Laboratory (VDRL)/ Treponema pallidum hemagglutination
(TPHA) tests l Serum vitamin B-12 levels l Anti-nuclear antibody levels l Sarcoid work-
up l Homocysteine levels
Antiphospholipid antibodies, ELISA for toxoplasmosis, rubella, cytomegalovirus, herpes
simplex virus (TORCH panel)

• What is the treatment for optic atrophy?

Treatment of cause to prevent further worsening
No proven treatment to reverse optic atrophy
Low vision aids for rehabilitation

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 Bala Notes for Ophthalmology Post-Graduate Examination: Fundus References

References

• The Will’s Eye Manual, 7th Edition

• Kanski Clinical Ophthalmology: A systematic approach - 8th Edition

• AAO – Retina & Vitreous. 2018-2019 Basic and Clinical Science Course

• Hypertensive Retinopathy - Stat Pearls - NCBI Bookshelf

[Link] › books › NBK525980 by P Modi - 2019

• Optic Atrophy: Major Review - Devendra et al.

[Link]
 Key Features

* An update of the popular "Bala Notes", which has been used in its
informal and handwritten form, by many Ophthalmology Post Graduates for their exams for the
past two decades since 1998.

* Revision aid in question and answer format

* Questions arranged based on the examiner's thought process during the

examination

* Helps postgraduates approach the university exams confidently

Dr C Balasubramaniam
Graduated from Madras Medical College (MMC); did postgraduation in Ophthalmology (MS)
from Regional Institute of Ophthalmology & Government Ophthalmic Hospital (RIO GOH),
Chennai1995-98; cleared FRCS at Edinburgh, UK 1998 and DNB 1999; underwent Vitreo Retinal
Fellowship at Sankara Nethralaya, Chennai and Anterior Segment Fellowship at Cheltenham, UK.

Was awarded Prof. Sathiavakesan Medal for being First in university examination at RIO GOH
and the Best Outgoing Postgraduate at RIO GOH, Chennai and Dr VT Joshi Medal for the Best
Outgoing Fellow at Sankara Nethralaya.

Has been involved in post graduate education for the past two decades both in India and the UK.

Currently Medical Director, Acchutha Eye Care and Chairman Acchutha Institute of Optometry,
Erode, Tamil Nadu.