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©2003, Remo Susanna Jr, MD

Optic Nerve Head Changes In Glaucoma

By

PROF.DR. ALI KHALIFA

AL AZHAR UNIVERSITY

CAIRO, EGYPT

How to Prevent Blindness in Glaucoma “Training Course for Trainers”

Remo Susanna Jr, MD University of São Paulo - Brazil

Paris

19 – 21 September

2003

©2003, Remo Susanna Jr, MD

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Hamilton Eye Center University of San Diego, California

February 14, 2004

How to Look at the Optic Nerve

Remo Susanna Jr, MD University of São Paulo - Brazil

©2003, Remo Susanna Jr, MD

©2003, Remo Susanna Jr, MD

WORKSHOP Prof. Remo Susanna Jr

Sintra, 21 de Fevereiro de 2004 Portugal

Caeser Park – Penha Longa

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©2003, Remo Susanna Jr, MD

Sintra – Portugal February 2004

©2003, Remo Susanna Jr, MD

©2003, Remo Susanna Jr, MD

Latin America Glaucoma Satellite Symposium March 13, 2004

How to Prevent Blindness in Glaucoma Remo Susanna Jr, MD

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©2003, Remo Susanna Jr, MD

Latin America Glaucoma Satellite Meeting March 2004

Brazil Argentina Chile

Colombia Equador Mexico Peru Venezuela

©2003, Remo Susanna Jr, MD

Optic Nerve Head Changes In Glaucoma

Jedda

SAUDI ARABIA

2008

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OPTIC NERVE HEAD CHANGES IN

GLAUCOMA (Remo Susanna)

presented by

PROF. DR. ALI KHALIFA AL AZHAR UNIVERSITY

EGYPTIAN SOCIETY FOR THE GLAUCOMAS

2007-2012

©2003, Remo Susanna Jr, MD

Why Do People Go Blind From Glaucoma?

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©2003, Remo Susanna Jr, MD

The Probability of Blindness From Open-Angle Glaucoma

• 20 years of follow-up: 1965-1985

• Timolol, pilocarpine, epinephrine, acetazolamide, phospholine iodide, etc

• Glaucoma: 54% blind in one eye 22% blind in both eyes

• Ocular hypertension (OHT): 14% blind in one eye 4% blind in both eyes

Hattenhauer, et al. Ophthalmology. 1998.

©2003, Remo Susanna Jr, MD

Patients Blind From Glaucoma

• 25 (27%) blind at the first visit

• 84 (73%) blind under treatment

– 42.5% no compliance

– 57.5% good compliance

Glaucoma Is Underdiagnosed

Grant and Burke. Ophthalmology. 1982.

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©2003, Remo Susanna Jr, MD

1Friedman, et al. AGS. 2003. 2Mitchell, et al. Ophthalmology. 1996.

Glaucoma Diagnosis

• In 2000, 2.2 million Americans were diagnosed with glaucoma1

• 50% of individuals with glaucoma are undiagnosed2

• Many are undiagnosed despite having an eye exam

©2003, Remo Susanna Jr, MD

Glaucoma Diagnosis in Clinical Practice

• Intraocular pressure (IOP)

• Optic nerve head (ONH) assessment

• Standard visual fields (VFs)

• Retinal nerve fiber layer (RNFL)

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©2003, Remo Susanna Jr, MD

1Doughty and Zaman. Surv Ophthalmol. 2000. 2Liu, et al. Invest Ophthalmol Vis Sci. 2003.

Limitations of IOP

• Corneal thickness affects accuracy1

– Thin Cornea–IOP underestimated

– Thick Cornea–IOP overestimated

• IOP fluctuates2

• IOP damage threshold varies

– OHT

– Normal tension glaucoma (NTG)

©2003, Remo Susanna Jr, MD

Limitations of Visual Fields

• Subjective

• High variability

• Poor sensitivity

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First VF abnormal (GHT outside normal limits)

Second VF normal (GHT within normal limits)

Third VF abnormal (GHT outside normal limits)

Visual Fields: Highly Variable

• OHTS

– VF* abnormality not replicated 86% of the time1

*VF defect defined as GHT outside normal limits, CPSD <.05%, or both. 1Keltner, et al. Arch Ophthalmol. 2000.

©2003, Remo Susanna Jr, MD

1Quigley, et al. Arch Ophthalmol. 1982. 2Quigley, et al. Am J Ophthalmol. 1989.

Visual Fields: Poor Sensitivity

• 50% of optic nerve fibers lost prior to VF defect1,2

• By the time there is a 5 dB loss, there is a corresponding 25% loss of RGCs2

A Large Number of RGCs Are Lost Prior to Detectable VF Abnormalities

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©2003, Remo Susanna Jr, MD

• Early glaucoma can be missed if a clinician relies mainly on the visual field and IOP

• Glaucoma is an optic neuropathy

• The assessment of ONH and RNFL are needed for early glaucoma diagnosis

©2003, Remo Susanna Jr, MD

Glaucoma Damage Detection

Optic Disc

SAP

RNFL

Early

Advanced

Structural Losses

Functional Losses

Blindness Alarm

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©2003, Remo Susanna Jr, MD

Five Rules for the Assessment of the Optic Nerve Head in

Glaucoma

Remo Susanna Jr, MD

©2003, Remo Susanna Jr, MD

This course was developed by:

Remo Susanna Jr, MD University of San Paulo, Brazil

Felipe Medeiros, MD

Robert N. Weinreb, MD Hamilton Glaucoma Center

University of California, San Diego

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Prof. Ali Khalifa

Prof .Remo Susanna

©2003, Remo Susanna Jr, MD

1. Look first at the rim (and not at the cup)

2. Look at the peripapillary region

3. Look at the retinal nerve fiber layer

4. Look at the lamina cribrosa

5. Look for optic disc hemorrhage

Five Rules for the Assessment of the Optic Nerve Head in Glaucoma

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©2003, Remo Susanna Jr, MD

Rule #1 Identify the Rim: Look at the Scleral Ring and Cup Border

©2003, Remo Susanna Jr, MD

Rim width = distance between

the scleral ring and the location

where the vessel is bent (cup border)

Scleral ring

Cup border

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©2003, Remo Susanna Jr, MD

Rim width = distance between

the scleral ring and the location

where the vessel is bent (cup border)

Scleral ring

Kinking

of the vessel (Cup border)

I – S – N – T rule

©2003, Remo Susanna Jr, MD

Clinical Importance of Glaucomatous Signs

Weak

Moderate

Strong

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©2003, Remo Susanna Jr, MD

The Color of the Rim

©2003, Remo Susanna Jr, MD

Cup = Pallor

Normal Disc

If cup = pallor—normal

If pallor > cup—neurological disorder or glaucoma with very high IOP

If cup > pallor—glaucoma

Schwartz. Arch Ophthalmol. 1973.

©2003, Remo Susanna Jr, MD

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©2003, Remo Susanna Jr, MD

Non-Glaucomatous

Optic Neuropathy

To

tal p

allo

r Pale rim Cup

Pallor > Cup

©2003, Remo Susanna Jr, MD

Pallor > Cup

Non-Glaucomatous Neuropathy

Cup

Diffuse Pallor

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©2003, Remo Susanna Jr, MD

Pallor

Cup

Cup > Pallor

Glaucoma

Location of kinking

of the vessel

©2003, Remo Susanna Jr, MD

Rule #2 Look at the Peripapillary Region

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©2003, Remo Susanna Jr, MD

Peripapillary Halo

α

β

Jonas, et al. Invest Ophthalmol Vis Sci. 2000.

Alpha Zone

Beta Zone

Sensitivity 20% to 30%

Specificity 80%

360º–normotensive senil-sclerotic glaucoma

©2003, Remo Susanna Jr, MD

α

β

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©2003, Remo Susanna Jr, MD

Rule #3 Look at the Retinal Nerve

Fiber Layer

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RNFL Red-Free Photographs

Bright

striations

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Hoyt sign

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Localized RNFL Loss

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©2003, Remo Susanna Jr, MD

Localized RNFL Loss

Hoyt, et al. Invest Ophthalmol. 1973.

Better seen with red-free light

©2003, Remo Susanna Jr, MD

©2003, Remo Susanna Jr, MD

Localized RNFL Loss

Localized RNFL loss – wedge-shaped dark area

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©2003, Remo Susanna Jr, MD

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©2003, Remo Susanna Jr, MD

Diffuse RNFL Loss

Bright

striations Diffuse loss

of striations

Diffuse RNFL loss – diffuse loss of the striate pattern +

increased visibility of retinal vessel borders

©2003, Remo Susanna Jr, MD

Diffuse RNFL Loss

Normal RNFL

RNFL reflex

Striations – brightness

Visibility of peripapillary retinal vessels

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©2003, Remo Susanna Jr, MD

Rule #4 Look at The Lamina Cribrosa

©2003, Remo Susanna Jr, MD

Striated pattern (Susanna) Susanna R Jr. Can J Ophthalmol. 1983.

Aquired optic pit (Radius) Radius RL, et al. Br J Ophthalmol. 1978.

Lamina Cribrosa

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©2003, Remo Susanna Jr, MD

Remember That a Normal Large Disc Has a Large Cup

©2003, Remo Susanna Jr, MD

Cup = Pallor

ISNT rule obeyed

Large disc Large cup

Normal Disc

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©2003, Remo Susanna Jr, MD

Be Cautious With Myopic Discs

©2003, Remo Susanna Jr, MD

Cup Size

Scleral ring

Cup border

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©2003, Remo Susanna Jr, MD

Rule #5 Look for Optic Disc Hemorrhage

©2003, Remo Susanna Jr, MD

Flame-shaped

hemorrhage

• Normally disappear after 2 to 6 months

• Indicative of glaucoma progression

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©2003, Remo Susanna Jr, MD

Detection of disc hemorrhages requires careful optic disc examination

©2003, Remo Susanna Jr, MD

Detecting Glaucoma

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©2003, Remo Susanna Jr, MD

ISNT Rule Is Not Obeyed

Glaucoma

©2003, Remo Susanna Jr, MD

Glaucoma – Small Disc

Peripapillary Halo

Narrowing of Retinal Arteries

Generalized RNFL

Large Cup for a Small Disc

©2003, Remo Susanna Jr, MD

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©2003, Remo Susanna Jr, MD

Small aperture (5 degree)

of Welch-Allen direct

ophthalmoscope

Normal size disc Small disc

Large disc

©2003, Remo Susanna Jr, MD

Optic Disc Size

Measurement of optic disc size:

• Biomicroscopy:

– Volk lens

– Measure size of slit beam

• Correction factors:

– Volk 60D – x 1.0

– Volk 78D – x 1.1

– Volk 90D – x 1.3

Average vertical diameter: 1.8 mm

Average horizontal diameter: 1.7 mm

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©2003, Remo Susanna Jr, MD

Optic Disc Size

Small Medium Large

Size of the cup varies with size of the disc

Identify Small and Large Optic Discs Small Discs: Average Diameter < 1.5 mm Large Discs: Average Diameter > 2.2 mm

©2003, Remo Susanna Jr, MD Read and Spaeth. Trans Am Acad Ophthalmol Otolaryngol. 1974.

Hemorrhage

Nasal Cupping

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©2003, Remo Susanna Jr, MD

Notch

RNFL defect Peripapillary

atrophy (β zone)

Hitchings and Spaeth. Br J Ophthalmol. 1976.

Notch

©2003, Remo Susanna Jr, MD

Notching

Glaucoma

RNFL loss

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©2003, Remo Susanna Jr, MD Armaly. Arch Ophthalmol. 1967.

Cup/Disc Ratio Asymmetry 0.2

©2003, Remo Susanna Jr, MD

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©2003, Remo Susanna Jr, MD

Detecting Progression (Compare Photographs During

Follow-Up)

©2003, Remo Susanna Jr, MD

10 Years

Generalized Rim Thinning

Varma R, et al. Am J Ophthalmol. 1992.

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©2003, Remo Susanna Jr, MD 4 Years

Enlargement of Localized RNFL Defect

Progressive Rim Thinning

©2003, Remo Susanna Jr, MD Uchida, et al. Ophthalmology. 1998.

5 Years

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©2003, Remo Susanna Jr, MD

5 Months

©2003, Remo Susanna Jr, MD

Corresponding VF defects

(previous slide)

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©2003, Remo Susanna Jr, MD 10 Years

©2003, Remo Susanna Jr, MD

Optic Nerve Head Evaluation Test II

20 seconds/Test

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©2003, Remo Susanna Jr, MD

Small disc

Small cup

RNFL reflex

Normal

Test 1

©2003, Remo Susanna Jr, MD

©2003, Remo Susanna Jr, MD Test 2

Disc hemorrhage ISNT rule not obeyed

Glaucoma

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Large disc

Large cup

ISNT rule obeyed

Normal RNFL

Pallor = Cup

Normal

Test 3

©2003, Remo Susanna Jr, MD

©2003, Remo Susanna Jr, MD

Notching superiorly

Beta-zone

Superior and inferior RNFL drop-out

ISNT rule not obeyed

Glaucoma

Test 4

©2003, Remo Susanna Jr, MD

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©2003, Remo Susanna Jr, MD Test 5

Beta-zone

Notching superiorly and inferiorly

Hemorrhage

Glaucoma

©2003, Remo Susanna Jr, MD

Inferior notching

Inferior beta-zone

Inferior RNFL defect

Glaucoma

Test 6

©2003, Remo Susanna Jr, MD

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©2003, Remo Susanna Jr, MD

ISNT rule not obeyed

Inferior peripapillary halo

Inferior notching

Nasal cupping

Glaucoma

Test 7

©2003, Remo Susanna Jr, MD

©2003, Remo Susanna Jr, MD Test 8

ISNT rule obeyed

Whitish RNFL reflex

Normal disc

©2003, Remo Susanna Jr, MD

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Small disc

Beta-zone

Large cup for a small disc

Nasal cupping

Glaucoma

Test 9

©2003, Remo Susanna Jr, MD

©2003, Remo Susanna Jr, MD Test 10

ISNT rule not obeyed

Inferior peripapillary halo

Inferior notching

Nasal cupping

Glaucoma

©2003, Remo Susanna Jr, MD

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©2003, Remo Susanna Jr, MD

Thank You