CE in the Southwest

CE in the Southwest

HOSTED BY: PROGRAM LOCATION Westin Galleria Dallas 13340 Dallas Parkway, Dallas, Texas 75240

PROGRAM COURSEMASTERS Sandra Fortenberry, OD, FAAO - UIWRSO Marcus Gonzales, OD, FAAO - UHCO

Saturday, March 30, 2019

7:00 am to 8:00 am Registration/Continental Breakfast/Visit Exhibits

8:00 am to 8:50 am Let’s Go Viral Presented by Roberto Saenz, OD, MS, FAAO COPE ID# 61431-AS 1 DT

8:50 am to 9:45 am DEWS 2: What’s New Presented by Roberto Saenz, OD, MS, FAAO COPE ID# 58563-AS 1 DT

9:45 am to 10:15 am Break & Visit Exhibits

10:15 am to 12:00 pm The Disky Divas Presented by Roberto Saenz, OD, MS, FAAO

COPE ID# 61430-NO CEE Available 2 DT

12:00 pm to 1:00 pm Lunch provided by UHCO and UIW/Visit Exhibits

1:00 pm to 2:40 pm New Horizons: A Look at Future Technologies & Treatments in Eye Care Presented by Christopher Wroten, OD, FAAO

COPE ID# 61582-GO CEE Available 2 DT

2:45 pm to 3:15 pm Break & Visit Exhibits

3:15 pm to 4:05 pm Ophthalmic Ultrasound for the Primary Eyecare Provider Presented by Christopher Wroten, OD, FAAO COPE ID# 61566-PD 1 DT

4:05 pm to 5:00 pm YAG Capsulotomy for the Optometric Physician Presented by Christopher Wroten, OD, FAAO COPE ID# 61564-LP 1 DT

Sunday, March 31, 2019 7:00 am to 8:00 am Registration/Continental Breakfast/Visit Exhibits

8:00 am to 9:40 am Not all glaucoma suspects are created equal Presented by Michael Sullivan-Mee, OD, FAAO

COPE ID# 61349-GL CEE Available 2 DT

9:40 am to 10:20 am Break & Visit Exhibits

10:20 am to 12:00 pm Glaucoma Management Tips for Optimizing Decision-Making Presented by Michael Sullivan-Mee, OD, FAAO

COPE ID# 61410-GL CEE Available 2 DT

12:00 pm to 1:00 pm Lunch provided by UHCO and UIW/Visit Exhibits

1:00 pm to 2:45 pm Ocular & Systemic Differentials of Uveitis Presented by Zanna Kruoch, OD, FAAO

COPE ID# 61422-SD CEE Available 2 DT

2:45 pm to 3:15 pm Break & Visit Exhibits

3:15 pm to 4:05 pm Resident Grand Rounds from Community Clinics Presented by Dominic D'Orazio, OD, and Noor Abushagur, OD COPE ID# 61506-PS 1 DT

4:05 pm to 5:00 pm 2019 Professional Responsibility for Texas Optometrists Presented by Andrew Kemp, OD, FAAO COPE ID# 60707-EJ 1 PR

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NOT ALL GLAUCOMA SUSPECTS ARE CREATED ALIKE

Which glaucoma suspects actually have glaucoma ??

Michael Sullivan-Mee, OD, FAAO

AAO Diplomate, Glaucoma

No conflicts of interest to report

And which glaucoma suspects are actually imposters ??

CESW 2019

COURSE FOCUSPrimary Care Optometry

Glaucoma is the second leading cause of blindness,

a disability that is largely preventable if detected in

early stages

1. Maximize visual function2. Identify vision & life-

threatening conditions

GLAUCOMA EPIDEMIOLOGY

Shaikh Y, Yu F, Coleman AL. Burden of undetected and untreated glaucoma in the United States. Am J Ophthalmol 2014.Varma et al. Prevalence of open angle glaucoma and ocular hypertension in Latinos. The Los Angeles Latino Eye Study. Ophthalmology 2004.

1. Access to care2. Diagnostic failure

U.S. estimates: 50% of cases are undiagnosed

Primary Reasons

Under‐diagnosis primarily occurs in young, old, and those with poor access to healthcare

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GLAUCOMA EPIDEMIOLOGY/ U.S.

• 3.3 million in U.S. with glaucoma in 2000• 3.9 million in U.S. with glaucoma in 2020

(projected)• 4.7 million in U.S. with glaucoma in 2040

(projected)

Tham et al. Global prevalence of glaucoma and projections of glaucoma burden through 2040. Ophthalmology 2014.

Glaucoma prevalence increases with aging

AGING TRENDS

• A man reaching age 65 today can expect to live, on average, until age 84.3.

• A woman turning age 65 today can expect to live, on average, until age 86.6.

• About one out of every four 65-year-olds today will live past age 90

• One out of 10 will live past age 95

According to data compiled by SSA:

Tham et al. Global prevalence of glaucoma and projections of glaucoma burden through 2040. Ophthalmology 2014.

Glaucoma Prevalence Rate by Ancestry

GLAUCOMA EPIDEMIOLOGY

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Keys to raising our glaucoma suspicion flag

IOP = 29,25

My Mom went blind

from glaucoma

Advanced disease is generally straightforward to diagnose

Early glaucoma, however, can be quite challenging to accurately identify

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OPTIMIZED GLAUCOMA DETECTION STRATEGY

1. Neuro-retinal rim characteristics2. Disc size (to interpret cup size)3. Disc hemorrhage4. RNFL integrity 5. Peri-papillary atrophy

OPTIC NERVE HEAD ASSESSMENT


OPTIC NERVE HEAD ASSESSMENT 1. Neuro-retinal rim characteristics2. Disc size (to interpret cup size)3. Disc hemorrhage4. RNFL integrity 5. Peri-papillary atrophy

ISNT rule violation Focal thinning/Notching Vessel bayonneting/Baring

Risk for developing POAG increases with increasing C/D ratio BUT……….

Gordon MO et al. The Ocular Hypertension Treatment Study: Baseline factors that predict the onset of Primary Open-Angle Glaucoma. Arch Ophthalmol 2002;120(6):714-720

Quigley HA et al. Risk factors for the development of glaucomatous visual field loss in ocular hypertension. Arch Ophthalmol 1994;112:644-9

Schulzer M et al. A comparison of treated and untreated glaucoma suspects. Ophthalmology 1991;98:301-7

What about cup/disc ratio ???

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DISC SIZE/CUP SIZE CORRELATION

Garway-Heath D et al. Vertical cup/disc ratio in relation to optic disc size: its value in the assessment of the glaucoma suspect. Br J Ophthalmol 1998.

Larger disc with larger cup

GLAUCOMA NORMAL

Smaller disc with medium cup

DISC SIZE/CUP SIZE CLINICAL IMPACT

a. Gordon MO et al. The Ocular Hypertension Treatment Study: Baseline factors that predict the onset of Primary Open-Angle Glaucoma. Arch Ophthalmol 2002;120(6):714-720

b. Quigley HA et al. Risk factors for the development of glaucomatous visual field loss in ocular hypertension. Arch Ophthalmol 1994.c. Schulzer M et al. A comparison of treated and untreated glaucoma suspects. Ophthalmology 1991;98:301-7

Many studies show that failure to account for disc size is the leading factor in erroneous optic nerve

assessment

HOW TO MEASURE DISC SIZE

Disc size measurement methods: Direct 0-scope, Slit lamp indirect lens (60D), HRT/OCT

Direct O-scope

60 D Volk2.4mm

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CUP/DISC ASYMMETRY: EXPLAINED BY OPTIC DISC SIZE ASYMMETRY

NOT ALL CUPS OF THE SAME SIZE ARE BUILT THE SAME

C/D 0.8Glaucoma

Small discs

Large discs

..

Danesh-Meyer HV et al. Regional Correlation of Structure and Function in Glaucoma, Using the Disc Damage Likelihood Scale, Heidelberg Retina Tomograph, and Visual Fields. Ophthalmology 2006, 603 – 611.

Disc Damage Likelihood Scale

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OPTIC NERVE HEAD ASSESSMENT --applies to clinical exam and photography

Need prospective approach

Can precede NFL, VF and/or rim defects

Usually occur infero- or supero-temporallyOften occur adjacent to existing damage

DISC HEMES

Drance SM, Fairclough M, Butler DM, Kottler MS. The importance of disc hemorrhage in the prognosis of chronic open angle glaucoma. Arch Ophthalmol 1977;95:226‐228.

Leske MC, Heijl A, Hyman L, et al. Predictors of long‐term progression in the early manifest glaucoma trial. Ophthalmology 2007;114:1965‐1972.

Budenz DL, Anderson DR, Feuer WJ, et al. Detection and prognostic significance of optic disc hemorrhages during the Ocular Hypertension Treatment Study. Ophthalmology 2006;113: 2137‐2143.

How many disc hemes clinically missed in OHTS ?

OHTS: 84% of disc hemes missed clinically




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Look for co-location of B-PPA and rim loss, RNFL defect and/or disc heme



OPTIC NERVE HEAD ASSESSMENT Also need to

consider non-optic nerve factors

MAXIMIZING DETECTION OF GLAUCOMA

PAS Strategy

Prospective approach Active index of Suspicion

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PASS

• Demographics• Age• Ethnicity

• Ocular History• Trauma• Surgery• Medications

• Medical History• Hypotension, Migraine, sleep apnea ?• Medications (e.g. steroids)

• Family History/Glaucoma• Blindness/Eyedrops/Surgery ???

75Black male

Yes, bluntNoNone

Postural hypotensionHTN: BB, CCB, diuretic

Yes, blindness

Patient example

??

• Best acuity

• Refractive error

• Confrontation VF’s

• Pupils• Irregularity• APD

OD 20/20; OS 20/60(old macular scar OS)

Mild myopia

Normal

Mild/1+ sluggish OSNo

Patient example

PASS

OS

ANTERIOR SEGMENT• Slit lamp exam

• K-spindle• Iris abnormalities (neo/pxe/TID)• Anterior chamber depth/angles

Patient example

NoNoNormal

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• IOP• Absolute level• Asymmetry• Fluctuation (AGIS/CIGTS)

GAT: 15, 20YesUnknown

Patient example

PASS

Williams A et al. The Value of Intraocular Pressure Asymmetry in Diagnosing Glaucoma. Journal of Glaucoma. 2013

GAT-IOP !

• Optic nerve appearance• Disc size/proportional to cup size• Rim configuration/erosion• RNFL defect• Disc hemorrhage• Peri-papillary atrophy• Asymmetry

Patient exampleLarge/YesISNT failure OU (I<S)NoNoNoNo

PASSPatient example

PASS SUMMARY• 75 yo black/Fam Hx/Low BP • Reduced vision in one eye• Pupils

• Irregularity/trauma• IOP

• Asymmetry *• Optic nerve appearance

• Rim configuration/erosion*

Patient example

Baseline glaucoma workup indicated*

Urgency: Intermed/high

Dx: Glc suspect, high risk*

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• Elevated or asymmetric IOP• Slit lam finding

• Shallow anterior chamber/angle• K-spindle/Iris transillumination defect• Pseudoexfoliation material• Irisneovascularization/mass

• ANYAngleclosureconcern

• Any optic nerve clinical suspicion*

NOT ALL GLAUCOMA SUSPECTS ARE CREATED ALIKE

Summary: Who needs baseline GLAUCOMA workup ?

ANY CRITICAL FINDING CUMULATIVE HAZARD• Combination of minor risk factors

BASELINE GLAUCOMA SUSPECT WORKUP

• GAT-IOP• GONIOSCOPY (4-mirror; compression)• Pachymetry• VF test (24-2 vs FDT-matrix vs HEP vs Octopus)• Optic nerve photography (preferably stereo)• OCT

• RNFL• Disc*• Macula*


• GAT-IOP

Sullivan-Mee M1, Gerhardt G, Halverson KD, Qualls C. Repeatability and reproducibility for intraocular pressure measurement by dynamic contour, ocular response analyzer, and goldmann applanation tonometry. J Glaucoma. 2009 Dec;18(9):666-73

World Glaucoma Association consensus statements:1. Goldmann applanation tonometry is more precise (lowest measurement variability),

compared to other methods.2. Intraobserver variability: 2.5 mmHg (two readings by the same observer

will be within this figure for 95% of subjects)Interobserver variability: ± 4 mmHg (95% confidence limits either

side of mean difference between observers)(In clinical practice these figures may be considerably higher )

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Type of glaucoma is related to glaucoma risk

Gonioscopy needed to identify type of glaucoma

Angle closure/plateau iris Pigment dispersion syndrome Pseudoexfoliation Angle recession Iris neovascularization Neoplasm/Melanoma Inflammation-related

BASELINE GLAUCOMA SUSPECT WORKUP• Pachymetry

World Glaucoma Association consensus statements:1. On average, greater central corneal thickness (CCT) results in overestimation of

intraocular pressure (IOP) as measured by Goldmann applanationtonometry (GAT)……..BUT the extent to which CCT contributes to the measurement error (in relation to other factors) in individual patients under various conditionshas yet to be established.

2. Correction nomograms that adjust GAT IOP based solely on CCT are neithervalid nor useful in individual patients.

Ultrasound vs other methods

BASELINE GLAUCOMA SUSPECT WORKUP• Pachymetry: strongest predictor of conversion in OHTS

OHTS results: (Brandt et al, Ophthalmology 2001)Mean CCT in their sample: 573 ±39 µm

(mean CCT in overall population 535-545 µm)

They defined following the following risk groups: (Gordon et al. JAMA Ophthalmology 2002)

Thin <555 µm Higher riskIntermediate 555-588 µm Intermediate riskThick >588 µm Low risk

(Ocular Hypertension Treatment Study)

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• VF test (24-2 vs FDT-matrix vs HEP vs Octopus)• Optic nerve photography (preferably stereo)

• OCT• RNFL• Disc*• Macula*

Optical coherence tomography• Fast• Objective• Quantitative• Repeatable

Anatomic structures of interest• RNFL• Macula• Neuro-retinal rim


• Diagnostic accuracy: expert-level performance• Vessani RM et al. Comparison of quantitative imaging devices and subjective optic nerve

head assessment by general ophthalmologists to differentiate normal from glaucomatous eyes. J Glaucoma 2009.

• Azuara-Blanco A et al. Clinical agreement among glaucoma experts in the detection of glaucomatous changes of the optic disk using simultaneous stereoscopic photographs. Am J Ophthalmol 2003.

• Improves consistency in optic nerve assessment• Almazroa A et al. Agreement among ophthalmologists in marking the optic disc and optic

cup in fundus images. Int Ophthalmol 2016.• Abrams LS, Scott IU, Spaeth GL, Quigley HA, Varma R Agreement among optometrists,

ophthalmologists, and residents in evaluating the optic disc for glaucoma. Ophthalmology 1994 Oct;101(10):1662-7.

WHAT DOES SD-OCT DO FOR US ?

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OCT-RNFL OPTIMIZED GLAUCOMA DETECTION STRATEGY

INTERPRETATION STRATEGY

Asymmetry

1. Data validity: Segmentation Error

2. TSNIT

3. Symmetry

4. Maps/Flags

5. Optic nerve image (next pg)

OCT-RNFL

• Infra-red images from OCT may demonstrate RNFL defects

Maps

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RNFL DATA VALIDITY

• Segmentation error• Anatomic

confounders• Scan quality

• Segmentation error: PPA included in scan

RNFL DATA VALIDITY

• Anatomic confounders: Retinal Edema

RNFL DATA VALIDITY

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75 YO BLACK MALE WITH FAM HX GLC/LOW BP/TRAUMA/REDUCED ACUITY OS

• Gonioscopy: Normal/open OU

• GAT-IOP• 15, 20 (Last visit)• 14,18 (TODAY)

• US pachymetry/CCT: 539, 545

Back to Patient example

CCT interpretation: (Ultrasound)OHTS study cut-offs (555, 588)Mean CCT population ~ 545um

1 SD: 505-585

Patient example

OD OS24-2

CASE 1: GLAUCOMA SUSPECTPatient example

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IS THERE A STRUCTURE/FUNCTION RELATIONSHIP ?

Diagnosis = Glaucoma

CASE 2

CASE 2: GLAUCOMA SUSPECT DUE TO OPTIC NERVE APPEARANCES

• 72 yo Hispanic female

• History: non-contributory

• Visual acuity: • 20/20 OD• 20/25 OS

• Slit lamp exam: • mild cataract OS>OD

• GAT-IOP: 14,12

• No 24-2 VF loss

• Open angles on gonioscopy

• US Pachymetry: 548,555

• Optic nerves: large cups OS>OD• larger discs (2.2)• Suspicious rim OS

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CASE 2

rnfl

Glaucoma suspect: low risk profile based on low IOP, normal gonio/SLE, normal

VF’s and normal/symmetric RNFL

Robust and symmetric

Stable now for 9 years

• 79 yo Caucasian male

• History: HTN/HCTZ


• Slit lamp exam: • Pseudophakia OU

• GAT-IOP: 27,27

• No 24-2 VF loss



CASE 3: OCULAR HYPERTENSION

CASE 3: OCULAR HYPERTENSION

Optic nerves: moderate-sized cupsSmall discs (1.7)Suspicious rim OS

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CASE 3

Put in oct-rnflRobust and symmetric

CASE 3

Put in oct-rnfl

Impression: Ocular HTN with thick pachs, open angles, normal VF’s, open angles, and suspicious optic nerves BUT with normal/symmetric RNFL

PLAN: Defer IOP-lowering treatment; follow-up in 6 months

CASE 4: OPTIC NERVES

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CASE 4

• 64 yo African American male

• History: HTN/DM/COPD• Meds: Metropolol/albuterol


• Slit lamp exam: • minimal NS cataract

• GAT-IOP: 18,19


• US Pachymetry: 531, 529

• Optic nerves: • Moderately large cups OD>OS• larger discs (2.2)• Suspicious rim OD

24-2 VISUAL FIELDS

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17 32

10-2 VISUAL FIELDS

STRUCTURE/FUNCTION CORRESPONDENCE

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OPTIMIZED GLAUCOMA DETECTION STRATEGY MACULA-OCT

OD

OS

94 8


POSTERIOR POLE ASYMMETRY ANALYSIS DIAGNOSTIC ABILITY

50 early POAGVersus

50 Normal

83% sensitivity95% specificityusing threshold value of 5um

Sullivan-Mee et al. American Journalof Ophthalmology 2013

Early POAG

>4 um<78 um>8 um>8 um

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PARTIAL MACULAR THICKNESS STRATEGY


MACULA-OCT

Ganglion cell layer +

Inner plexiform layer

GC-IPL STRATEGY

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1. Diagnostic uncertainty• Early stages/suspects

2. Anomalous optic nerves

3. Enhance efforts to identify

structure/function correspondence

4. Facilitate differential diagnosis

SO WHEN IS MACULAR SCANNING INDICATED FOR GLAUCOMA DIAGNOSTICS

1. Diagnostic uncertainty• Early stages/suspect






High Myopia

xxxx

7 11


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• Corroborate structure/function












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• 71 yo Causian male• History: HTN/DM/CAD• Meds:

Metropolol/HCTZ/insulin


• Slit lamp exam: • 1+ NS cataract OU

• GAT-IOP: 16,15


• US Pachymetry: 561, 565

• Optic nerves: • Moderately large cups OU• Average/large discs (2.2)• Suspicious rim OS (inf)

CASE 5: IS THIS GLAUCOMA ??

IS THIS GLAUCOMA ??OD OS

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IS THIS GLAUCOMA ??

• Superior

• Inferior

• Superior

• Inferior

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MACULAR SCANS CAN HELP IDENTIFY OCCULT RETINAL DISEASE

Sullivan-Mee et el. OVS 2018

1. Quantify amount of rim tissue

2. Identify type of PPA

OPTIC NERVE-OCT

Rim undercall

Rim overcall

Chauhan et al. Enhanced Detection of Open-angle Glaucoma with an Anatomically Accurate Optical Coherence Tomography–Derived Neuroretinal Rim Parameter. Ophthalmology 2013.

CLINICAL ERROROPTIC NERVE-OCT

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EFFECT OF RIM ORIENTATION


MRW versus HRW

Oblique angle leads to marked over-

estimation of rim tissue


Diagnostic Accuracy: MRW vs HRW

Figure 3 The receiver operating characteristic (ROC) curves illustrating the diagnostic performance of retinal nerve fiber layer thickness (RNFLT), Bruch's

membrane opening-horizontal rim width (BMO-HRW), and Bruch's membrane opening-minimum rim width (BMO-MRW)

SPECTRALIS NEURO-RETINAL RIM MODULE

os

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Differentiation of Parapapillary Atrophy

JONAS JB ET AL. MICROSTRUCTURE OF PARAPAPILLARY ATROPHY: BETA ZONE AND GAMMA ZONE. IOVS 2013

Alpha: agingBeta: glaucomaGamma: myopia

PPA zones are associated with:

1. Quantitative evaluation of optic nerve healtha) Can reliably identify healthy impostersb) Can reliably identify optic nerve damage

OPTIMIZED GLAUCOMA DETECTION STRATEGY Value of OCT

CONFIDENCE !!!

2. Permits corroboration of structure/structure and structure/function abnormality

Wants updated spectacle Rx No other ocular/visual complaints

Last eye exam 3 years ago in California No history of eye injury/surgery/disease/trauma Med Hx: borderline DM/HTN; no meds Fam Hx: no glaucoma/blindness

Last case: 60 year old black male reports for routine eye exam

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Exam findings: ◦ Corrected visual acuity: 20/20 OD/OS with

minor myopia◦ Entrance tests: normal◦ External/slit lamp: unremarkable

◦ Goldmann applanation tonometry: 20, 19

◦ Dilated exam findings: CD: 0.5 OD; 0.4 OS; normal macula/poles/periphery OU

Penultimate Case Example: 60 year old black male reports for routine eye exam

1.6mm 1.6mm

Measurement with 60 D Volk lens

OCT-RNFL output report

Asymmetry

1. Segmentation

2. TSNIT

3. Asymmetry

4. Maps

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MAC SCAN

14 012

• Gonioscopy: open/normal OD/OS

• Pachymetry: 545, 548

ADDITIONAL WORKUP24-2 VISUAL FIELDS

1.6mm disc size

Diagnostic keys to the case

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CONCLUSION

Accurate identification of undiagnosed glaucoma requires a prospective, structured approach that incorporates a graduated index of suspicion

OCT is useful not just for aiding glaucoma diagnosis but ALSO for calling out the GS imposters

Optometrists are well-positioned and crucial for reducing glaucoma burden in the coming decades

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GLAUCOMA MANAGEMENT: OPTIMIZING DECISION-MAKING

TIPS FROM THE TRENCHES

Michael Sullivan-Mee, OD, FAAO

AAO Diplomate, Glaucoma

Albuquerque VA Medical Center

CESW 2019No conflicts of interest to report

Many factors must be considered for good quality, consistent decision-making

Mission Glaucoma

CLINICAL SUCCESS IN GLAUCOMA MANAGEMENT

PATIENT THAT IS STABLE

No apparent structural deterioration No progressive loss of rim No progressive loss of RNFL or new RNFL defects No new disc hemorrhages

No progressive visual field loss

Acceptable level of IOP (treated or untreated)

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BUT WHAT IS FAILURE IN GLAUCOMA MANAGEMENT ?

PATIENT THAT IS NOT STABLE

(+) structural deterioration progressive loss of rim tissue

progressive loss of RNFL (exceeding age-related rate)

new clinically evident RNFL defects

new disc hemorrhages

(+) progressive visual field loss

UN-Acceptable level of IOP

According to George Spaeth, MD:1) Prevent disability in our patients.2) If disability already exists, repair the

disability or prevent further disability.

SO WHAT ARE THE GOALS FOR GLAUCOMA CARE ?

All about “Quality of life”

• Maintain visual abilities• Ability to perform and enjoy

household, work, and personal tasks

• Reduction of risk for MVA

• Reduction of risk for falling

Quality of life factors impacting treatment decisions

• Maintain ability to live independently

• Maintain ability to remain physically active and mobile

• Reduce Psychological stress

• Fear of falling• Fear of blindness

• Negative effects of treatment/care• Burden of glaucoma

meds/follow-up visits/disease costs

• Glaucoma medicine side effects

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1. Preventsymptomaticvisionloss2. Limitfurthervisionlossifvisionloss

alreadyexists3.Preventblindness

Glaucoma Goal Summary

Foundation of care is to:

1. SEVERITY OF DISEASE

4. Life expectancy !

2. Rate of disease

progression

To achieve these goals clinically:

3. Level/cause/risk associated with IOP and the methods to treat it

The Essential Factors In Glaucoma

Decision-Making

IOP and type of glaucoma

Rate of progression

Lifeexpectancy

Severity of disease

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LIFE EXPECTANCY DIFFERENCES (RACE/GENDER)

EFFECT OF AGING ON LIFE EXPECTANCY

EFFECT OF AGING ON LIFE EXPECTANCY

• A man reaching age 65 today can expect to live, on average, until age 84.3.

• A woman turning age 65 today can expect to live, on average, until age 86.6.

• About one out of every four 65-year-olds today will live past age 90

• One out of 10 will live past age 95

According to data compiled by SSA:

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Likelihood of living to be 90

years old (%)

60%

30%

0%Does not smoke Smokes SmokesNot overweight Sedentary SedentaryNot diabetic DiabeticNot hypertensive HypertensiveExercises Overweight

54%

30%

4%

Courtesy: George Spaeth

• Seat of the pants• Take into account standard clinical history

• Family history

• General health status

• Healthy behaviors

• Perception of health: one question survey

• Calculators• SSA has a calculator on the web (gender and age only)• Other calculators can be found on an internet search

SO HOW DO WE CLINICALLY DETERMINE LIFE EXPECTANCY ?

Estimated Physiologic Age of Elderly Patients Adjusted for Their Self-Reported Health Status*

Welch, H. G. et. al. Ann Intern Med 1996;124:577-584

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HTTPS://WWW.MYABARIS.COM/TOOLS/LIFE-EXPECTANCY-CALCULATOR-HOW-

LONG-WILL-I-LIVE/

LIFE EXPECTANCY CALCULATOR

• The ABARIS life expectancy calculator is based on a

statistical regression of more than 400,000 data samples. The data was collected by the National Institute of Health and AARP.

https://www.myabaris.com/tools/life-expectancy-calculator-how-long-will-i-live/

AgeGenderRaceHeightWeight

EducationMarital statusRetired ?Annual Income

Exercise habitsGeneral healthDM statusAlcohol habitsSmoking habits

Email required

LIFE EXPECTANCY

Ultimately, every case needs an assessment of life expectancy

All personal health information, health behaviors and family history/family

member longevity should be considered in this effort so that treatment/management

decisions can be optimally titrated

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Decision-Making


Rate of progression

Lifeexpectancy

Severity of disease

Severe disease at presentation isconsistent risk

factor associatedwith glaucoma-related blindnes

STAGING OF GLAUCOMA DISEASE

• Several staging systems have been developed

• Most systems are based on functional (VF) status

• Some efforts directed at a combined index (VF + OCT-RNFL)

Medeiros FA et al. A combined index of structure and function for staging glaucomatous disease. Arch Ophthalmol. 2012.

Hirasawa K et al. A modified glaucoma staging system based on visual field index. Graefes Arch Clin Exp Ophthalmol. 2013.

MG M et al. Comparison of visual field severity classification systems for glaucoma.J Glaucoma. 2012.

Mills RP et al. Categorizing the stage of glaucoma from pre-diagnosis to end-stage disease. Am J Ophthalmol. 2006.

Bayer A et al. Validity of a new disk grading scale for estimating glaucomatous damage: correlation with visual field damage. Am J Ophthalmol 2002

ICD-10 STAGE DEFINITIONS

Optic nerve abnormalities consistent with

glaucoma • AND glaucomatous visual field abnormalities in BOTH hemifields • AND/OR loss within 5 degrees of fixation in at least one hemifield.


glaucoma • AND glaucomatous visual field abnormalities in ONE hemifield

and • NOT within 5 degrees of fixation

Optic Nerve abnormalities consistent with

glaucoma • but NO visual field abnormalities on any visual field test

(except SWAP or FDT)

Mild stage

Moderate stage

Advanced stage Mild stage ??

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Mild stage

Moderate stage

Advanced stage

Moderate stage ??

MD: -11.96 PSD 14.35

Put severe arc that spares fxnhere










Mild stage

Moderate stage

Advanced stage

Advanced stage ??

MD: -5.89 PSD 4.74

MOST COMMON METHOD: HPA

Hodapp E, Parrish RK II, Anderson DR. Clinical decisions in glaucoma.St Louis: The CV Mosby Co; 1993. pp. 52–61

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HPA METHOD

Hodapp E, Parrish RK II, Anderson DR. Clinical decisions in glaucoma. St Louis: The CV Mosby Co; 1993. pp. 52–61

VF STAGE MD(mean defect)

Early > -6.00

Moderate -6.00 to -12.00

Severe < -12.00



Advanced stage ??

Fxn pt=27 dB

STAGING: COMBINING STRUCTURE AND

FUNCTION

To increase staging precision, optic nerve status and VF function should both be considered within disease staging process.

Worse of the two should usually be used as stage of disease for making glaucoma decisions *

Global RNFL: 95, 71

WHAT WE KNOW REGARDING STAGING WITH OCT RNFL

RNFL floor

• 50 um Spectralis• 57 um Cirrus• 65 um RTVue

Below 75um, VF and RNFL correlated

IOVS 2015

Asymmetry

Global RNFL: 95, 71

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MD -4.33; PSD 6.32

EXAMPLES OF STAGING

Global RNFL: 90, 75

MODERATEMILD

MD -3.65; PSD 4.25; Fxn pt 14 dB

VCDR: 0.75; 0.55; 2.0 discsinf thin OU; (+) B-PPA)

24-2

Global RNFL: 67, 80

Moderate stage ??Advanced stage

HOW DOES OTHER VISUAL LOSS AFFECT

STAGING?

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MACULAR THICKNESS CHANGE MAP

So, how do we incorporate this information into the glaucoma risk assessment ?

Level of Risk


Decision-Making


Rate of progression

Lifeexpectancy

Severity of disease

RATE OF PROGRESSION

Functional progression Micro-Structural progression

Macro-Structural progression

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MACRO-STRUCTURAL PROGRESSION

DETERMINING PROGRESSIVE FIELD LOSS

Heijl A et al. A comparison of visual field progression criteria of 3 major glaucoma trials in EMGT patients. Ophthalmology 2008.

Bengtsson B, Heijl A. A visual field index for calculation of glaucoma rate of progression. Am J Ophthalmol 2008.

•Methods

• Event = reach “threshold” change

from baseline (e.g. GPA program)

• Trend = rate of change over time

PROGRESSION EVENT OS

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MODE OF VISUAL FIELD TESTING

HVF 24-2 offers best option for monitoring

established glaucoma

• SAP (standard automated perimetry)

• Sita, 24-2 pattern, achromatic

Sample PA et al. Identifying glaucomatous vision loss with visual-function-specific perimetry in the diagnostic

innovations in glaucoma study. IOVS 2006.

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• Timely determination of rate of change

• Numbers of tests to detect change event

• Effect of poor reliability

Frequency of Visual field testing

Pertinent factors

TIME PERIOD (YEARS) REQUIRED TO DETECT VARIOUS RATESOF MD CHANGE IN VISUAL FIELDS WITH LOW, MODERATE AND HIGH DEGREES OF VARIABILITY WITH ONE, TWO AND THREE

EXAMS PER YEAR

Chauhan BC et al. Practical recommendations for measuring rates of visual field change in glaucoma. Br J Ophthalmol 2008.

Progression rate Variability

(dB/year) Low Moderate High

1 VF exam ‐0.5 9 13 19

per ‐1.0 6 9 13

yr ‐2.0 5 6 9

2 VF exams ‐0.5 4.5 6.5 8.5

per ‐1.0 3 4.5 6.5

yr ‐2.0 2.5 3 4.5

3 VF exams ‐0.5 3 4.3 6.3

per ‐1.0 2 3 4.3

yr ‐2.0 1.7 2 3

Loss of 10 dB before you determine

there is catastrophic rate

PROGRESSION TREND

1.4 x 20 yrs = 28 %

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CONSIDERATIONS WITH RATE OF PROGRESSION IN ADVANCED DISEASE

OCT-RNFL PROGRESSION

Evidence: RNFL progression

Are there validated criteria to define

CLINICALLY SIGNIFICANT

OCT-RNFL progression in

an individual patient ?

NO…..but by using what is known along with corroborative analysis,

serial OCT-RNFL data can be a useful clinical tool

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STABLE RNFL OVER TIME

OCT RNFL and Guided Progression Analysis (GPA)

CONSIDERATIONS FOR IDENTIFYING TRUE PROGRESSION

• Data validity

• Scan quality

• Segmentation

• Centration

• Anatomical variation including pathology

• Age-related RNFL decline

• Baseline thickness effect

• Floor effect

• Axial length/magnification effects

• Frequency of testing

• Statistical vs. clinical significance

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Case 4: Glaucoma Progression ?

THE AGING RNFL

• Cross-sectional studies

• Mean loss of average RNFL thickness: -0.16 to -0.44

• Longitudinal studies

• Mean loss of average RNFL thickness:

-0.45 to -0.60• 95% CI ~ 3µm per year

Leung et al. Ophthalmology 2012; Wessel et al. IOVS 2013

Age impact not accounted for in machine outputs--Time trend compared against zero/flat slope--But slope is not zero as there is age-related loss

Guided Progression Analysis

THE AGING RNFL

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BASELINE RNFL THICKNESS

• Thicker RNFL changes more over time

• Thinner RNFL changes less over time

Harwerth RS et al. Age-related losses of retinal ganglion cells and axons. IOVS 2008.Leung CK et al. Retinal nerve fiber layer imaging with spectral-domain optical coherence

tomography: a prospective analysis of age-related loss. Ophthalmology 2012

Axial length indirectly related to

RNFL thickness which may

make detection of RNFL

change over time in high

myopia more difficult

Floor effect needs to be considered

BASELINE RNFL THICKNESS

Patient example

In early disease, RNFL decreases while VF remains

stable/normal

FLIP SIDE

Abe RY et al. The Relative Odds of Progressing by Structural and Functional Tests in Glaucoma. IOVS 2016

Patient exampleRNFL change: -2.28 um/yearVF mean sensitivity: -0.25 db/year

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What is clinical threshold for change ?

1. Significant event-related RNFL change ~ 5um

2. Significant rate of change ? …..depends

Clinically significant RNFL rate of change

Leung et al. Ophthalmology 2012; Wessel et al. IOVS 2013

Miki et al. Rates of retinal nerve fiber layer thinning in glaucoma suspect eyes. Ophthalmology 2014

EFFECT OF TESTING FREQUENCY ON DETECTION OF CHANGE

24-36 months

Example Rate: Loss of global RNFL of 3.1 um per year

Example Rate: Loss of global RNFL of 3.1 um per year

12-18 months

EFFECT OF TESTING FREQUENCY ON DETECTION OF CHANGE

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What about sectoral RNFL rates of change• Variability is essentially twice as great with sector

versus global measures

• Rate of change appears to differ by sector

Feuer W et al. Topographic differences in age-related changes in the RNFL of normal eyes measured with Stratus OCT. J Glaucoma 2011

Global IT Inferior IN Nasal SN Superior ST Temporal

POAG ‐1.11* ‐3.02* ‐2.29* ‐1.49 ‐0.35 ‐0.45 ‐1.00* ‐1.40* ‐0.94NML ‐0.46 ‐1.55 ‐1.07 ‐0.58 ‐0.06 0.18 ‐0.05 ‐0.33 ‐0.30

* Significantly different p<0.05; Ruezga et al. AAO 2014

POAG

NML

Global and Sectoral rate of RNFL loss over time: POAG vs NML (µm/yr)

1. Rate associated with development of field loss

Global RNFL loss ~ 2 um or more per year• Miki et al. Rates of retinal nerve fiber layer thinning in

glaucoma suspect eyes. Ophthalmology 2014

2. Event-related RNFL change ~ 5um (repeatable)

Summary: What is clinically significant RNFL change

Interpretation should consider:– Data quality

– Concurrent visual field change

– Concurrent structural change (disc/macula)

– Level of IOP

– Age and Life expectancy

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What about Macular Thickness Progression ?

Zhang et al. Comparison of Glaucoma Progression Detection by Optical Coherence Tomography and Visual Field. Am J Ophthalmol 2017;184:63-74.


Decision-Making


Rate of progression

Lifeexpectancy

Severity of disease

Level/cause/risk associated with IOP and the methods to treat it

Case Example

Age: 45Occupation: Engineer

s/p LASIK at age 35--moderate myopia

1. “Vision seems off”2. Trouble serving a

volleyball (loses it during serve)

3. No discomfort/pain

Wants enhancement !

1422

OD

Diagnosis: Advanced primary angle closure glaucoma

(with missed opportunity)

OS OS

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Current factors of case decision making

Age: 45Good health

Current visual status VA normal OD/OS VF: severe loss OS

Treatment goals Maintain current

visual status Keep IOP low Healthy 45 year old/monocular/high risk !

OD OS

Disease progression rate To be determined

So is PACG something to worry about ?

PACG blinds disproportionately vs. OAG: 3-10x more people blind from PACG vs. OAG

IOP tends to be relatively high in angle closure

PACG is usually asymptomatic (up to 85% or more of cases )

Asymptomatic presentations often lead to late diagnosis and treatment

So is PACG something to worry about ?

PACG blinds disproportionately vs. OAG: 3-10x more people blind from PACG vs. OAG

IOP tends to be relatively high in angle closure

PACG is usually asymptomatic (up to 85% or more of cases )

Asymptomatic presentations often lead to late diagnosis and treatment

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Type of glaucoma is related to glaucoma risk

SLE/gonio needed to identify 2’ glaucoma

Angle closure/plateau iris Pigment dispersion syndrome Pseudoexfoliation Angle recession Iris neovascularization Neoplasm/Melanoma Inflammation-related

HOW IS IOP RISK ASSESSED/CONSIDERED ?

• Level/elevation of IOP*• IOP symmetry*• Type of Glaucoma/

cause of IOP elevation*• Fluctuation ?

* Requires thorough & timely SLE/GONIO

Williams AL et al. The value of intraocular pressure asymmetry in diagnosting glaucoma. J Glaucoma 2013

WHAT IS AN ACCEPTABLE LEVEL OF IOP ?

• This estimate based on : • Untreated high IOP• Estimated IOP volatility• Disease severity

• Other visual morbidity• Progression rate• Life expectancy

Level of IOP that does not permit the development of symptomatic vision loss

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INITIAL TARGET GUIDANCE FROM CIGTS VS EMGT

Both trials examined treatment in early glaucoma

EMGT used fixed tx regimen (betaxolol and ALT) Average IOP reduction with tx = 22%

CIGTS medical treatment group had strict IOP targets, based on a formula that involved untxIOP and visual field status;

Average medical IOP reduction with tx = 35% Surgery group reduction =46%

In EMGT, 45% of treated subjects had VF progressionIn CIGTS, 0% had visual field progression

BEYOND TARGET IOP, ACHIEVING AN ACCEPTABLE LEVEL OF IOP REQUIRES ADHERENCE TO THE

TREATMENT REGIMEN

• Factors to consider with regard to medical adherence: • Tolerance to topical agents/ocular surface health• Instillation techniques• Regimen complexity• Medicine Costs• Comprehension of disease• Medical co-morbidity

Both under-treatment and lack of adherence have been consistently identified as risks

for glaucoma-related blindness

WHAT IF IOP IS ACCEPTABLE BUT THE DISEASE CONTINUES TO

PROGRESS ?

• Expected IOP fluctuation• Measurement confounders

• Steroid treatments • BP meds (b-blocker)• Dehydratrion

• Nocturnal IOP/BP pressure changes• Sleep posture issue• Harmful activities (eye rubbing/ valsalva/ yoga ?• Irregular med adherence and/or instillation

technique• Inaccurate IOP measurements• Progression rate/life expectancy/disease stage

These factors should be considered

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• 60 yo Caucasian male

• History: Sleep apnea


• Slit lamp exam: • Normal OU

• GAT-IOP: Mean 16,15 (tx)• Other visit: 18, 18

• Open/Normal angles on gonioscopy


• Dilated exam: normal retinal health except mild/mod ERM OS>OD

• Optic nerves: next slide

FINAL CASE

OD

FINAL CASE

OS

FINAL CASE: OD

ERMThin

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FINAL CASE: OS

ERMThin

FINAL CASE: 24-2

FINAL CASE: 10-2

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• Life expectancy

• Disease Stage

• Rate of disease change

• IOP risk

FINAL ASSESSMENT

Overall level of risk and aggressiveness of treatment/management: HIGH

• 20-30 years

• OD Advanced

• OS Advanced • based on structure and VF loss

• ?? FAST ??

• LOW ?? High IOP to date: 18/18

• Life expectancy

• Disease Stage

• Rate of disease change

• IOP risk

FUTURE CASE ASSESSMENT (GOAL)

• 5-10 years

• OD Advanced

• OS Advanced • based on structure and VF loss

• Minimal to none

• LOW: IOP consistently 8-12 s/p trab

Overall level of risk and aggressiveness of treatment/management: medium


Decision-Making


Rate of progression

Lifeexpectancy

Severity of disease

CONCLUSION

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Zanna Kruoch, OD, FAAO, ABO DiplomateAssistant ProfessorUniversity of Houston, College of Optometry

› I have received honoraria from:– Essilor Custom Contact Lens Specialist

› Assistant Professor – University of Houston, College of Optometry

› Opinions from this lecture are my own

› Identify key elements in an examination to aid in the diagnosis of uveitis.

› When to utilize further history and/or laboratory testing to aid in the diagnosis of uveitis.

› Be able to build a list of systemic differentials associated with uveitis.

The Fundamentals of Building the Ocular Differentials

CC: “red, painful eye"

› CC: red, painful eye

› HPI: – Right eye– Onset 4 days ago– Gradually getting worse– Constant– 1st episode started 2 years ago. Occurs every 3-4 months. Only

right eye.– Associated symptoms: photophobia, pain on the right side of the

head– No discharge– Mild head relief with Tylenol BID po

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› POH:– Unremarkable

› PMH:– Hypothyroidism, primary

› Systemic Medications:– Levothyroxine

› ALL:– Penicillin

› Intraocular Pressures – OD 38 mmHg– OS 14 mmHg– Time: 8:39 am

› VA (sc)– OD 20/20– OS 20/25, PH 20/20

› EOMs– Unrestricted OD, OS

› CF– FTFC OD, OS

› PERRL (-) APD– Marked photophobia

Determining the ocular differentials

DIFFERENTIALS?

Infectious Conjunctivitis / Keratitis

Staph. Hypersensitivity / Sterile Ulcer

Dry Eyes

Corneal Abrasion

Recurrent Corneal Erosions

Scleritis

Uveitis

DRAWBOARD• Age/Race/Gender:

• 41 yro:• Collagen-vascular auto-

immune?• MS?

• Hispanic:• Secondary inflammation from

DM?• Female

• Adnexa / External:• No lid lesions evident• No lid edema• Hyperemia, no evident discharge• No heavy flaking or madarosis

DIFFERENTIALS?

Infectious Conjunctivitis / KeratitisStaph. Hypersensitivity / Sterile Ulcer

Dry EyesCorneal Abrasion

Recurrent Corneal ErosionsScleritisUveitis

DRAWBOARD• CC: red and painful

• Inflammation• Corneal pain receptors

• HPI:• Acute unilateral red eye:

• Infectious i.e. viral or bacterial?• Hx of recurrence:

• Chlamydia? Episcleritis or iritis? • Photophobia:

• CB spasm• Corneal defect causing spasms?

• Head pain:• GCA?• Scleritis?

• Lack of discharge:• Less likely to be infectious

DIFFERENTIALS?Infectious Conjunctivitis / Keratitis

Staph. Hypersensitivity / Sterile UlcerDry Eyes

Corneal AbrasionRecurrent Corneal Erosions

ScleritisUveitis

Adult Inclusion ConjunctivitisEpiscleritis

Giant Cell Arteritis

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DIFFERENTIALS?Infectious Conjunctivitis / Keratitis

Staph. Hypersensitivity / Sterile UlcerDry Eyes

Corneal AbrasionRecurrent Corneal Erosions

ScleritisUveitis

Adult Inclusion ConjunctivitisEpiscleritis

Giant Cell ArteritisNeovascular Glaucoma

DRAWBOARD• PMH / Med:

• Hypothyroidism / Levothyroxine

• Prelims:• Good acuities: No significant media

opacity• Normal EOMs, lack of pain. • Observed photophobia on direct

and consensual of OD• IOPs:

• Asymmetry• High pressure• Outflow issue

• External exam:• Significant injection• Lack of chemosis

DIFFERENTIALS?

Infectious Conjunctivitis / Keratitis

Staph Hypersensitivity / Sterile Ulcer

Dry Eyes

Corneal Abrasion

InfectiousConjunctivitis / Keratitis

Clinical Findings

Consistent with DDxAcute UnilateralHyperemiaPhotophobia

Not Consistent with DDx

(-) Lid edemaDecent vision(-) DischargeRecurrence

Findings to Confirm DDx

If keratitis, corneal inflammation should be present.

Staph Hypersensitivity / Sterile Ulcer

Clinical Findings

Consistent with DDx HyperemiaPhotophobia


Normal lidsAcute presentation


Evidence of prior / present inflammation particularly inferior.

Dry Eye Clinical Findings

Consistent with DDxHypothyroidismHyperemiaPhotophobia


Diffuse hyperemiaNo discharge


Present of MGD, (+) LG stain, low tear volume.

Corneal Abrasion Clinical Findings

Consistent with DDx Pain


(-) TraumaRecurrence(-) discharge

Findings to confirm DDx

Epithelial defectPositive/negative NaFl stain

DIFFERENTIALS?

Recurrent Corneal Erosions

Scleritis

Uveitis

Adult Inclusion Conjunctivitis

Recurrent Corneal Erosions Clinical Findings

Consistent with DDx PainRecurrence


Diffuse hyperemia(-) DischargeNo prior hx of corneal injury


Epithelial defectPositive or negative NaFl

Scleritis Clinical Findings

Consistent with DDxEye painHead painRecurrence


No associated systemiccondition


Presence of deep scleral vascularization and inflammation

Uveitis Clinical Findings

Consistent with DDxOcular / Head painPhotophobia (+ Pupil Testing)Recurrence

Not Consistent with DDx High pressure?

Findings to Confirm DDx Anterior chamber reaction

Adult Inclusion Conjunctivitis Clinical Findings

Consistent with DDx Acute unilateral hyperemiaRecurrence

Not Consistent with DDx (-) discharge


Presence of conjunctival follicles





Presence of conjunctival follicles

Episcleritis Clinical Findings

Consistent with DDx HyperemiaRecurrence

Not Consistent with DDx Presence of pain

Findings to Confirm DDx Superficial hyperemia

Giant Cell Arteritis Clinical Findings

Consistent with DDx Pain on side of head


AgeGood vision

Findings to Confirm DDx Elevated, chalky nerve

NeovascularGlaucoma Clinical Findings

Consistent with DDxAcute unilateralHyperemiaAsymmetric, high pressure

Not Consistent with DDx No known reason for ischemia


Neovascularization and/or evidence of ischemia

DIFFERENTIALS?

Episcleritis

Giant Cell Arteritis

Neovascular Glaucoma

TOP DIFFERENTIALS:

Uveitis

Adult Inclusion Conjunctivitis

Neovascular Glaucoma

Uveitis Clinical Findings

Consistent with DDxOcular / Head painPhotophobia (+ Pupil Testing)Recurrence

Not Consistent with DDx High pressure?

Findings to Confirm DDx Anterior chamber reaction




Findings to confirm DDx Presence of conjunctival follicles

Neovascular Glaucoma Clinical Findings

Consistent with DDxAcute unilateralHyperemiaAsymmetric, high pressure

Not Consistent with DDx No known reason for ischemia

Findings to Confirm DDx Neovascularization and/or evidence of ischemia

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OD OS

normal ADNEXA normal

normal LIDS/LASHES normal

3+ injection temporally, 2+ diffuse injection elsewhere,

(-) folliclesCONJUNCTIVA white and quiet

4-5 scattered fine KPs diffusely, 1 pigmented fine

KPCORNEA clear, (-) KPs

3+ cells, 1+ flare A/C (-) C/F

flat, brown, (-) nodules, (-) NVI IRIS flat, brown, (-) nodules

clear LENS clear

clear, (-) cells VITREOUS clear, (-) cells

› Assessment:1. Non-granulomatous anterior uveitis OD.

› History of recurrence.

› Plan:1. In-office instillation of 1 gtt Scopolamine 0.25% and 1 gtt

Pred Forte OD. Start Pred Forte 1% q2h OD during waking hours. RTC 2 days for f/u, IOP ck, and DFE.

› Inflammation of the uveal tissue – iris, ciliary body, choroid

› Symptoms include pain, photophobia, decreased vision.

› Two broad types:– Nongranulomatous

› Fine KPs› Koeppe nodules

– Granulomatous› Mutton fat keratic precipitates› Busacca nodules

› Anterior chamber flare, resulting from extra protein in the aqueous, is usually present.

– 0 = None– 1+ = Faint– 2+ = Moderate (iris and lens detail clear)– 3+ = Marked (iris and lens detail hazy)– 4+ = Intense (fibrin or plastic aqueous)

› Cells, the hallmark of iritis, are present in the aqueous. They should be graded by severity under high-magnification slit lamp examination in a 1 X 3-mm field of light

– 0 < 1– 0.5 = 1-5 cells– 1+ = 6-15 cells– 2+ = 16-25 cells– 3+ = 26-50 cells– 4+ = More than 50 cells

› WBCs that collects on the back of the endothelium– Fresh KPs – round, white, regular in shape– Old KPs- irregular in shape, pigmented

› Sign of chronic inflammation

› Types:– Fine KPs – typically non-granulomatous– Mutton fat KPs – typically granulomatous– Stellate KPs – part of Fuch’s Heterochromic

Iridocyclitis

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› Nevus Nodules– Lisch

› melanocytic hamartomas› present in Neurofibromatosis 1

– Mammallations › Regular, elevated vitiliform

nodules regularly spaced on hyperpigmented iris

› Unilateral› Seen in association with ocular

melanocytosis– 1 in 400 with ocular

melanocytosis will develop uveal melanoma

› Can be associated with high IOP

› Inflammatory Nodules– Koeppe

› In nongranulomatous and granulomatous

› At pupillary margin– Busacca

› Almost always granulomatous› At mid-periphery

1. Is the disease acute or chronic?2. Inflammation granulomatous or non-granulomatous?3. Is the disease unilateral or bilateral?4. Where is the inflammation located?5. What is the demographics of the patient?6. What associated symptoms/signs does this patient

have?

› Juvenile Rheumatoid Arthritis› Birdshot Choroidopathy› Serpiginous Choriodopathy› Tuberculosis Uveitis› Postsurgical Uveitis

– P. acnes, fungal

› Intraocular lymphoma› Sympathetic Ophthalmia› Multifocal Choroiditis› Sarcoidosis

CHRONIC› Majority of anterior cases:

– Idiopathic– Ankylosing Spondylitis– Reiter’s Syndrome– Fuch’s Heterochromic Iridocyclitis

› Vogt-Koyanagi-Harada Syndrome› White-dot Syndromes› Acute retinal necrosis› Postsurgical bacterial infection› Trauma

ACUTE› Sarcoidosis› Sympathetic ophthalmia› Uveitis associated with MS› Lens-induced uveitis› Intraocular foreign body› VKH syndrome› Syphilis› Tuberculosis› Other infectious agents

› Sarcoidosis› Lyme disease› Fuchs heterochromic

iridocyclitis, rarely› Juvenile idiopathic arthritis› Tubulointerstitial nephritis and

uveitis syndrome (TINU)

BILATERAL› Idiopathic

› HLA B27– Ankylosing spondylitis– Reactive arthritis– Inflammatory bowel disease– Psoriasis

› Behcet’s

› Sarcoidosis

› Trauma

› Intraocular foreign body

› Infectious– HSV, HZV, CMV– Parasitic– Metastatic endophthalmitis

› Acute retinal necrosis

UNILATERAL› Toxoplasmosis

› Masquerade Syndromes

› Syphilis

› HSV

› CMV

› Sarcoidosis

› Candidiasis

› Mengingococcus

› Tuberculosis

› Toxocariasis

› VKH

› Sympathetic Ophthalmia

› Serpiginous choriodopathy

› Birdshot choriodopathy

POSTERIOR› Idiopathic

› Ankylosing spondylitis

› Reiter’s syndrome

› Inflammatory bowel disease

› Psoriatic arthritis

› Behcet’s disease

› HLA-B27-associated disease

› JRA

› Fuch’s Heterochromic Iridocyclitis

› Sarcoidosis

› Syphilis

› Glaucomatocyclitic crisis

› Masquerade Syndromes

ANTERIOR

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Age Diagnostic Considerations

< 5 JRA, Toxocariasis, Postviral neuroretinitis, Retinoblastoma, Juvenile xanthogranuloma, Leukemia

5-15 JRA, pars planitis, toxocariasis, postviral neuroretinitis, sarcoidosis, leukemia

16-25 Pars planitis, Ankylosis spondylitis, idiopathic, toxoplasmosis, sarcoidosis, acute retinal necrosis

25-45 Ankylosing spondylitis, idiopathic, Fuch’s heterochromiciridocyclitis, toxoplasmosis, Behcet’s, retinal vasculitis, sarcoidosis, White-dot syndromes, VKH, AIDS, Syphilis, Serpiginous choriodopathy

45-65 Birdshot choroidopathy, idiopathic, Behcet’s, Serpiginouschoroidopathy, acute retinal necrosis

> 65 Idopathic, Serpiginous choroidopathy, Masquerade Syndromes

Factor Disease Risks

Female Pauciarticular juvenile rheumatoid arthritis, chronic anterior uveitis

Male Ankylosing spondylitis, sympathetic ophthalmiaAmerican black Sarcoidosis

Native American VKH syndrome

Midwestern American Presumed ocular histoplasmosis

Japanese VKH syndrome, Behcet’s

Mediterranean ancestry Behcet’s

Central American Cysticerosis, onchocerciasisSouth American Cysticerosis, Toxoplasmosis

West African onchocerciasisIV drug user Fungal endophthalmitis, AIDs

Promiscuous sexual activity AIDs, syphilis

Frequent hiking in wooded areas Lyme disease

Symptom or Sign Possible Associated ConditionsHeadache VKH, SarcoidosisParesthesia,weakness

MS, Behcet’s, steroid myopathy

Vitiligo, poliosis VKHErythema nodosum Behcet’s, SarcoidosisSkin nodules Sarcoidosis, onchocerciasisAlopecia VKHSkin rash Behcet’s, sarcoidosis, viral exanthem, syphilis, herpes zoster,

psoriasis, Lyme diseaseOral ulcers Behcet’s, inflammatory bowel diseaseGenital ulcers Behcet’s, Reiter’s syndrome, STDsSalivary/Lacrimal gland swelling

Sarcoidosis, lymphoma

Arthritis Behcet’s, Reiter’s syndrome, sarcoidosis, JRA, rheumatoid arthritis, lyme disease, inflammatory bowel disease, Wegener’s granulomatosus, systemic lupus erythematosus, other connective tissue disease

Tissue Presentation

Conjunctiva Marked ciliary flush

Sclera Scleritis

Cornea Presence of keratic precipitates, corneal edema

Anterior chamber Cells, flare, or hypopyon

Iris Atrophy of iris, heterochromia, posterior synechiae

Lens Cataracts (particularly PSC)

Vitreous Presence of cells

› Medical management: Squash that inflammation!– Aggressive steroid therapy

› Pred Forte vs. Durezol– Cycloplegic agent

› Management of posterior synechiae:– Phenylephrine 10% / 2.5%– Surgical

› Closely follow these patients:– 1-2 days after initiating therapy DFE– Consider blood work if recurrence and/or bilateral.

Tests Conditions/CommentsCBC with diff Underlying systemic conditionAngiotension-converting enzyme (ACE)

Sarcoidosis

ANA SLE, other rheumatic diseaseANCA Wegener’s granulomatosis, polyarteritis nodosaAntiviral Ab Viral infectionESR Underlying systemic disease; to check for presence of

inflammationHLA typing Specific HLA typesRF Rheumatoid arthritisVDRL/FTA-ABS SyphilisChest X-Ray Tuberculosis, SarcoidosisPurified protein derivative (PPD)

Tuberculosis

Sacroiliac x-ray Ankylosing spondylitis

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The Exercise of Building the Systemic Differentials for Uveitis

CC: Red eye

– Associated symptoms: › Foreign body sensation› “film over eye”› discomfort› extreme pain only once

– 8/10 pain, sharp, no pain today

› CC: 49 yro BF c/o of red eye

› HPI: – Left eye– Onset 2 months – Noticed it upon

awakening– Worse in the morning– Constant– 1st episode

› POH:– Unremarkable

› PMH:– Hypertension

› Systemic Medications:– Advil– Centrum– Lisinopril– Vitamin B-12– Vitamin B-6

› ALL:– NKDA

› Intraocular Pressures – OD 17 mmHg– OS 36 mmHg– Time: 11:17 AM

› VA (sc)– OD 20/20– OS 10/80, NIPH



› PERRL (-) APD

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OD OS



white and quiet CONJUNCTIVA 1+ injection

Clear, (-) KPs CORNEA

Intraepithelial bullae, localized cornea edema, (+) MF KPs inferior, (+) fine

KPs centrally

(-) C/F A/C(+) C/F – however difficult

to visualize due to presence of k-edema

flat, brown, (-) nodules IRIS (+) posterior synechiae @ 10, 11, and 1 o’clock

clear LENS clear


OD OS

Clear, (-) cells VITREOUS

0.35 R C/D

distinct DISC

pink, healthy RIM

(+) FR, flat MACULA

2/3 VESSELS

RED REFLEXHAZY VIEWS

› Assessment:1. Granulomatous anterior uveitis OS.

› 1st episode

› Plan:1. In-office instillation of 1 gtt Homatropine 5%, 1 gtt Durezol,

and 1 gtt Azopt OS. Start Durezol QID OS, HomatropineBID, and Azopt BID OS. Return 3 days for follow-up and fundus evaluation. Uveitis survey given to patient to complete.

› Series of questions to help identify systemic cause of uveitis

› When to administer survey:– Nonresponsive to treatment– Granulomatous anterior uveitis– Recurrence of uveitis– Posterior uveitis

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› Pros:– Detailed and easy to understand for patients– Inclusive– Useful in deciding what bloodwork to order

› Cost effective

› Cons:– Symptoms that may be late in the course of the disease; not

very useful– May need to repeat

› Several online available› Uveitis.org offers an example:

– http://www.uveitis.org/uveitis-questionnaire

› Email me for our clinic’s uveitis survey– [email protected]

› Prior illnesses:– Night sweats– Headaches– Sinus problems– White patches of hair or skin– Loss of hair

› Social Hx:– Own a dog– Cigarette use– BCP use

› Prior conditions:– HTN– Herpes (cold sores)– Chickenpox– Measles– Mumps– Allergies

Utilizing the uveitis survey to extract differential diagnoses.

DIFFERENTIALS:Toxocariasis

HSVSarcoidosis

VKH Breaking down the case to extract differentials.

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HSVVKH

SarcoidosisBehcet’s Disease

Acute Retinal NecrosisBirdshot Chorioretinitis

Serpiginous Retinopathy


HSVVKH





HSVVKH




› Lyme Disease› Coccidiodomycosis› Leprosy› Toxoplasmosis› Brucellosis› Post surgical

inflammation› Necrotizing

herpetic retinopathies– Acute retinal

necrosis (ARN)– Progressive Outer

Retinal Necrosis (PORN)

› Sarcoidosis› Sympathetic

ophthalmia› Uveitis associated

with MS› Intraocular foreign

body› Vogt-Koyanagi-

Harada syndrome› Syphilis› Tuberculosis› HSV, VZV, CMV, EBV› Toxocara canis


HSVVKH





HSVVKH


Acute Retinal NecrosisBirdshot Chorioretintis


› Subtypes:– HSV-1: infection in or around mouth– HSV-2: infection of genital or anal area

› HSV-1:– More likely to cause ocular complications than HSV-2– Highly contagious– Commonly acquired during childhood and remains latent and

asymptomatic– Symptoms:

› Mildly painful mouth sores› In immunocompromised patients, severe symptoms can occur with

frequent recurrence

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› Most common manifestation: HSV Keratitis– Granulomatous inflammation from Descemets to Bowmans in

stromal disease– T-cell mediated immune response with HSV antigens located in

deep stroma and endothelium– Can affect all layers of the cornea

› HSV Epithelial Keratitis is the most common herpetic infection› HSV Stromal Keratitis is the most common recurrent herpetic infection

› HSV-associated Uveitis:– Typically unilateral– Associated with acute ocular hypertension– Can be nongranulomatous or granulomatous inflammation– Residual damage can lead to iris atrophy

› Chronic granulomatous inflammation that can affect several organs

› Gold standard of diagnosis is the presence of non-caseating granulomata on tissue biopsy– Ancillary testing can be obtained if biopsy is not possible

› Ocular involvement can occur before systemic disease in up to 30% of cases

› Bimodal age distribution of ocular sarcoidosis– 20 to 30 yro– 50 to 60 yro

› African Americans are more likely to develop ocular involvement than Caucasians

› Females more likely than males to develop ocular involvement

› Most common:– Uveitis– Dry Eye– Conjunctival Nodules

› Less common:– Multifocal choroiditis– Retinal vasculitis:

› “Candle-wax drippings” exudates› Perivascular sheathing

https://openi.nlm.nih.gov/imgs/512/140/2855661/PMC2855661_MEAJO-16-202-g028.pnghttp://www.clevelandclinicmeded.com/medicalpubs/diseasemanagement/pulmonary/sarcoidosis/#figure9

› Ocular involvement:– Decrease vision or visual field defect– Visual hallucinations– Papilledema– EOM defects– Pupillary defects

› Other neurological involvement:– Cranial neuropathy: optic and facial most commonly affected– Encephalopathy– Vasculopathy– Peripheral neuropathy– Meningitis– Hydrocephalus

› Ocular Sarcoidosis can affect any structure of the eye and its adnexa.

› Uveitis is one of the most common findings.

› Race, age, chronic history

› HSV typically presents with corneal inflammation

› Uveitis can occur in HSV but not as likely

› Lack of iris atrophy

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Tests Conditions/CommentsCBC with diff Underlying systemic condition

Angiotension-converting enzyme (ACE) Sarcoidosis

ANA SLE, other rheumatic diseaseANCA Wegener’s granulomatosis, polyarteritis nodosaAntiviral Ab Viral infectionESR Underlying systemic disease; to check for presence of

inflammationHLA typing Specific HLA typesRF Rheumatoid arthritisVDRL/FTA-ABS SyphilisChest X-Ray Tuberculosis, SarcoidosisPurified protein derivative (PPD) Tuberculosis

Sacroiliac x-ray Ankylosing spondylitis

Lysozome Sarcoidosis

› Common:– Chest X-Ray for presence bilateral hilar lymphadenopathy– Elevated serum levels of ACE and/or lysozyme

› Other:– Chest CT if clear CXR– Abnormal liver enzymes– Negative tuberculin in BCG vaccinated patients

› So does this mean this is NOT Sarcoidosis?

› Up to 10% can present with clear CXRs

› Further testing needed

Requested Results

CBC with diff Normal including liver enzymes

ESR Not performed

ACE/Lysozyme Not performed

CXR Clear

PPD Not performed

FTA-ABS/RPR Not performed

› OS:– Significant drop in VA to CF– Development of iris bombe, glaucoma, cataract

› Never was able to obtain clear view of retina– Referred to county hospital for CE, retina evaluation

› BCVA 20/250 s/p CE

› OD:– Developed granulomatous uveitis– Followed similar course as OS– Continued care with OMD at county hospital

› High suspicion of underlying condition of Sarcoidosis– Pending results

CC: Flashes & Floaters

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› CC: flashes and floaters

› HPI: – OS > OD– Persistent flashes OS started 2 months ago, OD has occurred

“every now and then”– Associated symptoms:

› Constant “cloudy vision OS” which started before flashes› Experiencing vertigo with flashes and cloudy vision

› POH:– Retinal tear s/p laser OS – 2 years prior

› PMH:– Adult Rheumatoid Arthritis– HIV: per pt “well controlled” although unknown CD4 and viral load

› Systemic Medications:– Aleve– Meloxicam– Stribild– Tylenol

› ALL:– NKDA

› Intraocular Pressures – OD 17 mmHg– OS 11 mmHg– Time 1:10 pm

› VA (sc)– OD 20/20– OS 20/30, NIPH



› PERRL (-) APD

OD OS



white and quiet CONJUNCTIVA white and quiet

2+ Mutton fat KPs CORNEA 3+ Mutton Fat KPs

1+ C/F A/C 1+ C/F

flat, brown, (-) nodules IRIS flat, brown, (-) nodules

1+ NS LENS clear


OD OS

0.35 / 0.35 CD 0.35/0.35

Distinct 360 DISC Distinct 360

Pink and healthy RIM Pink and healthy

Flat, even pigmentation MACULA Flat, even pigmentation

2/3 VESSELS 2/3

(-)holes/tears/RDs PERIPHERY superior nasal retinal scarring

*slightly obscured views from KPs

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› Assessment:– Bilateral anterior granulomatous uveitis

› Plan:– Start Omnipred 8x/day, Cyclopentolate QID OU.– Uveitis survey given.– RTC 1 week. Building the systemic differentials list.

DIFFERENTIALS:TuberculosisSarcoidosis

SyphilisHSV/VZV/CMV/EBV

VKH

› Current/Prior Conditions:– Anemia– Hepatitis– HTN– Pneumonia– Herpes– Chickenpox– Shingles– Measles– AIDS/HIV– Rheumatoid Arthritis



VKHAIDS/HIV

Rheumatoid Arthritis

› Prior symptoms:– Night sweats– Fatigue– Poor appetite– Tingling/numbness of ears– Hearing/ringing of ears– Painfully cold fingers– Sore in the

throat/sneezing/sinus problems



VKHAIDS/HIV

Rheumatoid Arthritis

› Symptoms:– Stiff joints– Stiff lower back– Back pain while sleeping

› Social Hx:– Owned dogs– Bisexual/homosexual relations



VKHAIDS/HIV

Rheumatoid ArthritisAnkylosing Spondylitis

Toxocariasis

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VKHAIDS/HIV


Toxocariasis

› Inflammatory polyarthritis characterized by inflammation of the joint, leading to synovial joint destruction– Extremities– Cervical spine

› Females > Males

› Can start at any age but most common ages of 40-60

› 25% of RA patients will have ocular involvement

› Greater tendency to affect anterior vs. posterior segment– Most common:

› Dry eyes› Episcleritis / Scleritis:

– May extend to uvea causing uveitis› Peripheral ulcerative keratitis

– Less common:› Choroiditis› Retinal vasculitis (sub-type of uveitis)› Macular edema› Retinal detachments

› Scleritis: – Uncommon inflammation of sclera usually resulting in extreme pain– Scleritis prevalence of 4-10% in RA patients– RA-related scleritis makes up 1/3 of all scleritis cases– Anterior scleritis is much more common than posterior (90% vs 10%)

› Classified base on:– Distribution:

› Diffuse affecting one or more quadrants› Nodular with tenderness and scleral thickening

– Destruction: › Necrotizing › Non-necrotizing

› Uveitis:– Not typically associated with adult RA– Present concurrently with scleritis

http://emedsa.org.au/EDHandbook/eyes/redeye/Scleritis2.jpg

› Retrovirus largely affecting helper CD4+ T cells resulting in severe immunosuppression

› Initially homosexual/bisexual activity accounted for most transmission until early 1990s; now heterosexual activity is the major route of transmission– IV drug abuse– Perinatal transmission– Needle stick injury

› Can affect almost every tissue of the eye– Most common:

› Dry eye› Retinal microvasculopathy› CMV retinitis

– CD4+ count drops below 200 ocular involvement› CMV retinitis likely when CD4+ count below 50 - 100

› CMV Retinitis most frequent ocular infection in HIV – If uveitis present typically mild non-granulomatous uveitis – Clinical presentation:

› Slowly progression of retinal whitening spreading along the vasculature with hemorrhages

› Necrosis leads to thin atrophic retina leading to higher risk of RDs› Optic nerve may be involved› Low grade vitritis

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› Immune Recovery Uveitis– Uveitis that occurs in pts with CMV retinitis on HAART therapy– Thought of as a boost in the immune system from HAART

therapy – Typically presents with vitritis– Directly related to the severity of the CMV retinitis

› Granulomatous anterior uveitis responded well treatment with taper over first 8 weeks– BCVA OS 20/20 by end of week treatment– Clear of anterior chamber reaction and resolution of KPs

› The following labs were requested:– CBC with diff– RF / ANA– CD4+ / viral load – VDRL/RPR– HLA-B27

Orders Results Interpretation

CBC with diff

• Elevated creatinine• Decreased eGFR• Elevated globulin• Decreased

albumin/globulin ratio• Elevated sed rate

Kidney dysfunctionChronic inflammationPotential Multiple MyelomaHIV/AIDS

CD4+ 429 cells/uL Uncontrolled HIV/AIDSViral load 12260 copies/mL Uncontrolled HIV/AIDSCXR Clear Unlikely TB or SarcoidosisHLA-B27 Not performed n/aVDRL/RPR Not performed n/aQuantiferon-TB Gold Negative Unlikely TBESR Elevated Presence of inflammation

ANA Screening Negative Unlikely SLE, Scleroderma, other mix connective tissue

› 6 week f/u:– Results of lab– Pt admits being off HIV medications for several months but

by 6 week f/u with us, back on HIV meds x 3 weeks

› Over course of 5 months:– Pt treated for rebounding anterior uveitis– Responded well to treatment but tendency to rebound

during taper

› CC: – Floaters OS– Physician directed f/u for recurrent uveitis OU

› HPI: – Floaters:

› Sudden increase for several hours with one large floater remaining, some photopsia, noticed 1 week ago. Mild “fog” in superior vision.

– Recurrent uveitis OU:› Taper OD Durezol 1 gtt every other day; OS Durezol QID x 2 weeks.

› VA cc: – OD 20/20– OS 20/20

› IOP: @ 9:00 am– OD 17 mmHg– OS 13 mmHg

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OD OS




clear CORNEA clear

(-) C/F A/C (-) C/F


1+ NS LENS clear

OD OS

Few floaters VITREOUSExtensive amount of floaters noted inferior

(-) cells

0.35 / 0.35 CD 0.35/0.35




2/3 VESSELS 2/3


› Interpretation/Report:– No detachment OD, OS– Vitreous opacifications

noted OS

› Assessment:– Granulomatous anterior uveitis OD, OS. Stable.– Vitreous degeneration OS > OD.

› Plan:– Continue taper: Pred Forte OD, Durezol OS.– Counseled pt about risk of RD and educated on symptoms.

Advised pt to return in 4 weeks for repeat DFE.

› CC: Physician directed f/u – 1 month from last visit– Floaters OS– Recurrent uveitis OS

› HPI: – Floaters:

› Worsening of floaters and vision› Flashes noted superiorly in vision

– Recurrent uveitis OU:› Compliant with taper: Omnipred QD OD, Durezol QD OS› No pain

› VA cc: – OD 20/20– OS 20/200 NIPH (drop from 20/20 from last visit)

› IOP: @ 9:08 am– OD 14 mmHg– OS 14 mmHg

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OD OS




clear CORNEA clear

(-) C/F A/C 1+ C/F


1+ NS LENS clear

Mild amount of floaters VITREOUSExtensive amount of

floaters noted inferior;2+ cells in vitreous

OD OS (HAZY VIEWS)

0.35 / 0.35 CD 0.35/0.35




2/3 VESSELS 2/3

(-)holes/tears/RDs PERIPHERY

Superior nasal retinal scarring

• Retinal elevation inferotemporal with significant vitreous

floaters overlying area• Retinal whitening

superotemporal, questionable vasculitis

› B-scan I&R:– Retinal thickening and

elevation noted inferior OS– Moderate reflectivity in

vitreous cavity consistent with vitritis OS

› OCT-Macula OS:– Poor quality score– Real-time feed showed no

apparently macular elevation

› Assessment:– Panuveitis OS.– Granulomatous anterior uveitis OD. Stable.

› Plan:– Immediate referral to retinal specialist for evaluation and

treatment.– Continue taper Omnipred QD x 1 week. Continue care with

retinal specialist.

› OS: VA 20/200› Hx:

– Re-started HIV treatment few months earlier

– RA stable

› Dx:– Granulomatous Panuveitis OS

SIX MONTH PRESENTATIONINITIAL PRESENTATION› OS: VA 20/20› Hx:

– Poorly controlled HIV – RA

› Dx: – Anterior granulomatous uveitis

OS > OD– Difficulties with rebound and

recurrence

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› Adult RA:– If uveitis present, typically presents with associated scleritis.

› HIV:– Are retinal findings consistent with CMV retinitis?

› No:– CD4+ count of our patient at 429– Whereas CMV retinitis tends to occur when CD4+ count falls below 50

› CMV retinitis presents with hemorrhages or granular appearance along with uveitis

– Typically non-granulomatous anterior uveitis



Necrotizing herpetic retinopathies: Acute retinal necrosis (ARN) Progressive Outer Retinal Necrosis (PORN)

VKHAIDS/HIV


Toxocariasis

› Auto-immune disease characterized by bilateral uveitis– HLA-DR4– Viral triggers

› Higher incidence in Asians, native Americans, Hispanics, Middle Easterners

› Associated with meningeal, auditory, integumentary manifestations– Neurological and auditory occurs prior to uveitis– Integumentary tends to occur after uveitis

› Prodromal:– 1-2 weeks with flu-like symptoms

› Acute:– Acute bilateral posterior uveitis– Non-granulomatous nature first two weeks– Dec VA

› Chronic: – Signs of depigmentation of uveal tissue

› “Sunset glow fundus” depigmentation of the choroid is the most common sign› Nummular chorioretinal scarring

– Signs of depigmentation of integumentary:› Poliosis, alopecia, vitiligo

› Chronic recurrent:– Mild panuveitis with recurrent episodes of anterior uveitis– Recurrence of uveitis changes from nongranulomatous to granulomatous

› Herpetic etiology– VZV most common– HSV second most common

› Generally in immune-competent individuals

› Clinical Presentation:– One or more areas of retinal necrosis

with distinct borders in peripheral retina

› Circumferential spread– Inflammatory reaction of anterior or

vitreous chamber– Rapid progression of disease in

absence of treatment– Occlusive vasculopathy

http://imagebank.asrs.org/Content/imagebank/Image18-ARN-Lecture.jpg/image-full;max$643,0.ImageHandler

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› Etiology:– Typically VZV– Other herpetic etiologies have been reported

(CMV, HSV)

› Immunocompromised individuals– HIV patients with CD4+ count of < 50 cells/ul

› Clinical Presentation:– Minimal to no inflammation of aqueous or vitreous

humors– Absence of vascular inflammation– Multifocal or confluent areas of retinal

opacification in peripheral retina– Necrosis creates atrophic, thin areas in the retina

leading to retinal detachments

› Poor prognosis due to difficulties responding to antiviral therapy

http://www.medscape.com/viewarticle/777098_4

› For:– Immunocompromised individual– Areas of retinal whitening

› Against:– Significant aqueous and vitreous

humor inflammation– Suspicious of retinal vasculitis

PORN› For:

– Presence of vitritis– Areas of retinal whitening

› Against:– Pt is immunocompromised

ARN

› Initial reports:– Assessment: Highly suspicious for PORN due to HIV status– Plan:

› Vitrectomy with biopsy› Tx of intravitreal Foscarnet, oral Valtrex› Laboratory titers for Toxoplasmosis, Syphilis

› Later reports:– Positive for Syphilis– Assessment: Syphilitic Retinitis

› Refer for LP› Initiate treatment for Syphilis

› Originally known as luetic disease

› Veneral disease caused by spirochete Treponema pallidum

› Can be acquired or congenital

› High rate of co-infection of HIV and syphilis

› Tertiary:– Affects cardiovascular

system and CNS months to years later

– Categories:› Gummatous syphilis:

– Granulomatous lesions usually at liver, bone, and testes

– Ulcerate then fibrotic› Cardiovascular syphilis:

– 10 years after initial infection– Aneurysm formation

› Neurosyphilis:– Various parts of CNS affected– Argyll-Robertson pupil

› Primary: – Painless chancre at site of

transmission– Highly contagious

› Secondary: – Fever and malaise– Generalized rash especially

at palms and soles

› Latent:– Clinically undetectable– Some will go into tertiary

syphilis while others remain asymptomatic

› Can occur at any stage of syphilis

› All ocular structures can be affected

› Ocular complications include:– Conjunctivitis– Episcleritis / scleritis– Interstitial keratitis– Uveitis (most common finding)– Iris roseolae (dilated iris vessels)– Elevated IOP– Chorioretinitis / retinitis

https://www.reviewofophthalmology.com/article/how-to-recognize-ocular-syphilishttps://www.aao.org/eyenet/article/be-on-lookout-ocular-syphilis

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› Anterior Uveitis: – Granulomatous– Isolated anterior uveitis is uncommon

› One study suggested granulomatous anterior uveitis tends to occur in HIV positive patients who are also affected by Syphilis

› Posterior Uveitis:– Most common ocular manifestation– Posterior involvement usually includes:

› Chorioretinitis› Retinitis› Vasculitis› Vitritis› Panuveitis

› Posterior Placoid Chorioretinitis:– Infection of RPE in macular or

peripapillary region– Characterized by ill-defined yellow

placoid confluent lesion – Vitritis, vasculitis, papillitis and serous

detachment may be present

› Retinitis / Chorioretinitis:– “Ground glass” retinitis with vasculitis– Patchy earlier on then progressed to

confluent– Choroidal involvement typically

multifocal, punctate lesions – Responds well to penicillin treatment

unlike ARN or PORN

http://imagebank.asrs.org/Content/imagebank/Sifilis-Caso01Color01.jpg/image-full;max$643,0.ImageHandlerhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC2694006/

https://link.springer.com/chapter/10.1007/978-3-319-23416-8_4#citeass

› 1st Dx: Granulomatous anterior uveitis– R/O RA:

› Lack of scleritis– R/O uncontrolled HIV:

› Anterior uveitis rare› If present, non-granulomatous

› 2nd Dx: Panuveitis– Consider HIV again?

› Vitreous and retina involvement now– R/O ARN:

› Typically in immunocompetent patient– R/O PORN:

› Tends to occur when CD4+ falls below 50› Presence of significant anterior/vitreous

inflammation not consistent with PORN› Choroid affected› Retinal vasculitis present

http://www.retinalphysician.com/archive/2013/October/images/OMD_Oct_A09_Fig01.jpg

› 3rd Dx: Syphilitic Chorioretinitis– Areas of retinal whitening

indicating necrosis– “Ground glass”

appearance with small area of multifocal punctate lesion

– GRANULOMATOUS anterior inflammation

– Posterior inflammation

CC: Pain and redness

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› CC: Pain and redness

› HPI: – OD– Started 1 week prior– Upon awakening– Saw an optometrist and was told he had a viral infection.

He was sick 2 weeks before symptoms began.

› POH: unremarkable

› PMH: unremarkable

› Medication: none

› ALL: NKDA

› Intraocular Pressures – OD 29 mmHg– OS 22 mmHg– Time 1:10 pm

› VA (sc)– OD 20/20– OS 20/20



› PERRL (-) APD

OD OS



4+ Diffuse InjectionMild chemosis

FolliclesCONJUNCTIVA Follicles

clear CORNEA clear

3+ cells / 1+ flare A/C (-) C/F


clear LENS clear


OD OS

0.25/0.25 CD 0.25/0.25




2/3 VESSELS 2/3


› Viral Conjunctivitis:– Symptoms x 1 week OD– Hx of URI– Diffuse injection– (+) Follicles

› Uveitis:– Symptoms– Diffuse injection– Presence of cells & flare

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› Assessment:– Non-granulomatous anterior uveitis OD. – 1st episode.

› Plan:– In-office instillation 1 gtt each of Scopolamine 0.25% and

Pred Forte 1%. – Rx’d Pred Forte q2h OD and Homatropine 5% BID OD. – RTC 1 week.

› CC: Physician directed f/u for non-granulomatous anterior uveitis OD.

› HPI:– Mostly compliant with Pred Forte q2h; missed a few drops.– Reports that the eye was “very bad” until Sunday but now

feeling better. – Did not get Homatropine.

› VA: sc– OD 20/20– OS 20/20

› Pupils:– OD 2+ direct response, OS 4+ direct response– No APD

› IOPs: @ 3:17 pm– OD 21 mmHG– OS 18 mmHg

OD OS


normal LIDS/LASHES normal1+ diffuse injection

Follicles CONJUNCTIVA Follicles

Diffuse amount of KPs CORNEA clear

2-3+ cells / 2+ flare A/C (-) C/F


Diffuse amount of pigment on anterior surface LENS clear

1+ vitritis VITREOUS clear, (-) cells

OD (HAZY VIEWS) OS

0.25/0.25 CD 0.25/0.25




2/3 VESSELS 2/3


› Assessment:– Non-granulomatous anterior uveitis with vitritis OD. – Worsening of condition.

› Plan:– Same day referral for special testing:

› B-scan› Photos

– Change medication to Durezol QID.– Uveitis survey.

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› B-Scan:– Unremarkable

› Photos:– Unremarkable

› Social Hx:– Lived in Mexico– Owned a dog– Eaten raw meat or uncooked

sausage– Bisexual/homosexual activity

› Prior conditions:– Chickenpox– Shingles

› Prior symptoms:– Stiff lower back

DIFFERENTIALS:HistoplasmosisToxocariasis

ToxoplasmosisSyphilis

VZVAnkylosing Spondylitis

DIFFERENTIALS:HistoplasmosisToxocariasis

ToxoplasmosisSyphilis

VZVAnkylosing Spondylitis

Orders Results Interpretation

CBC with diff Normal results Unlikely to be infectious

PPD Negative Unlikely TuberculosisHLA-B27 Positive Possible SpondylarthropathiesVDRL/RPR Negative Unlikely SyphilisESR Elevated Presence of inflammation

ANA Screening Negative Unlikely SLE, Scleroderma, other mix connective tissue

RF Negative Unlikely Rheumatoid Arthritis

› Conditions linked to HLA-B27:– Ankylosing Spondylitis– Reactive Arthritis (Reiter Syndrome)– Crohn’s Disease– Ulcerative Colitis– Psoriasis

› Typically ANA and RF negative

› Chronic condition that affects several organs, generally the sacroiliac joints

› Young white males– 2nd decade of life

› Symptoms:– Lower back pain– Symptoms worse in the morning or without activity– Improvement with exercise

2/13/2019

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› Greater anterior segment involvement vs. posterior segment

› Presents most commonly as uveitis:– Anterior, non-granulomatous– Greater tendency to have more inflammation with ocular

surgeries– Greater severity relative to idiopathic uveitis

› Treatment:– More resistant to traditional therapies– Require more aggressive steroids – Consult with PCP for medical management

› Understanding the key elements to make a diagnosis of uveitis– CC & History– Clinical signs

› Hallmark› Other subtle signs

› Utilize additional tests to aid in the systemic cause of uveitis– Survey– Laboratory testing

› Create the systemic differentials– Utilize the history and clinical signs– Pull from the uveitis survey– Use laboratory testing (and repeat testing) to confirm your

suspicion.

› Be flexible!– The course of the condition may change.– Your differentials may change.

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3. Wensing B, de Groot-Mijnes, JDF, Rothova A. Necrotizing and nonnecrotizing variants of herpetic uveitis with posterior segment involvement. Arch Ophthalmol (2011)129:403-409.

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12. Murray PI, Rauz S. The eye and inflammatory rheumatic diseases: The eye and rheumatoid arthritis, ankylosing spondylitis, psoriatic arthritis. Best Practice & Research Clinical Rheumatology 30 (2016): 802-825.

13. Hughes EH, Guzowsky M, Simunovic MP et al. Syphilitic retinitis and uveitis in HIV-positive adults. Clinical and Experimental Ophthalmology 2010; 38: 851-856.

14. Ittner EA, Bhakhri R, Newman T. Necrotizing herpetic retinopathies: a review and progressive outer retinal necrosis case report. Clin ExpOptom 2016; 99:24-29.

15. Nussenblat RB, Lane HC. Human immunodeficiency virus disease: changing patterns of intraocular inflammation. Am J Ophthalmol. 1998 Mar;125(3):374-82.

16. Whitcup SM1, Fortin E, Lindblad AS et al. Discontinuation of anticytomegalovirus therapy in patients with HIV infection and cytomegalovirus retinitis. JAMA. 1999 Nov 3;282(17):1633-7.

17. Bansal R, Gupta V, Gupta A. Current approach in the diagnosis and management of panuveitis. Indian J. Ophthalmol. 2010 Jan-Feb; 58(1):45-54.

18. Verma S, Hughes JD, Mabey D et al. Symptomatic anterior uveitis in HIV-positive patients. International Journal of STD & AIDS. 1999;10:2680274.

19. Amaratunge BC, Camuglia JE, Hall AJ. Syphilitic uveitis: A review of clinical manifestations and treatment outcomes of syphilitic uveitis in human immunodeficiency virus-positive and negative patients. Clinical and Experimental Ophthalmology 2010; 38: 68-74.

20. Androudi S, Dastiridou A, Symeonidis C et al. Retinal vasculitis in rheumatic disease: an unseen burden. Clin Rheumatol (2013)32:7-13.

21. Chen J, Lee L. Posterior placoid chorioretinitis: an unusual ocular manifestations of syphilis. Clinical Opthalmology 2008:2(3)669-673.

22. Liping D, Aize K, c, Peizeng Y. Vogt-Koyanagi-Harada disease: Novel insights into pathophysiology, diagnosis and treatment. Progress in Retinal and Eye Research 52 (2016) 84-111.

23. Haug S.J., Cunningham E.T. (2017) Syphilitic Uveitis. In: Chee SP., Khairallah M. (eds) Emerging Infectious Uveitis. Springer, Cham

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Resident Grand Rounds from Community Clinics

Dominic D’Orazio, OD Noor Abushagur, OD

Ocular Disease Residents of DFW

BENIGN OR MALIGNANT? THAT IS THE QUESTIONDominic D’Orazio ODOcular Disease Resident at Cedar Springs Eye Clinic, Dallas, TX

Case:

46 y/o African American female

Hx of DMII

Presents for annual comprehensive and evaluation for possible ocular complications from DMII

Pertinent Findings

VA(sc): 20/20 OD and OS

Confrontation fields: FTFC

Pupils: PERRLA (-) APD

Elevated melanocytic nevus on right central brow (reported as longstanding)

Pertinent Findings: Posterior

Gray/black growth on superior optic nerve OS

Approximately 1 disc diameter in size

No other significant posterior findings

Diagnosed as an ONH Melanocytoma

Photodocumentation as baseline

Scheduled for 6 month follow up

Pertinent Findings: Posterior

October 2011

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Follow-up

1 year later

Lesion still described as 1 DD in size

Photodocumented

Continued 6 month follow up regimen

Follow-up

October 2012

Follow-up

Two years since initial diagnosis

Photodocumentation of notable change in size

Now measured at approximately 1 DD horizontally, 1.5 DD vertically

Due to change, moved next follow up to 3 months

Follow-up

October 2013

Follow-up Continued follow up, patient was returned to 6 month

intervals

Patient presented three full years after initial diagnosis

Photodocumentation

OCT ordered to measure thickness/height of lesion

Lesion has more than doubled based on initial presentation

Patient referred to Parkland ophthalmology for B-Scan imaging and consultation

Lost to follow up for 2 years

Follow-up

October 2014

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Return to Clinic (2018)

Patient presents to clinic for annual exam for DMII

Patient was seen at UT Southwestern every 6 months from 2014-2016

Was told she could now be followed annually—sooner PRN

BCVA 20/20 OD and OS

Confrontation fields: FTFC

Pupils: PERRLA (-) APD


Fundoscopy revealed a large, evenly pigmented, deep black, elevated lesion obscuring the view of the entire optic nerve OS.

Significant change from previous visits

Ordered OCT

Scheduled 1 week follow up for HVF 30-2


July 2018

Optos Image OSOctober 2011 July 2018

OCT

Oct. 2014: 1.22mm July 2018: 1.35mm

HVF 30-2

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OCT Angiography

An alternative to IV indocyanine green angiography and fluorescein angiography

“Uses laser light reflectance of the surface of moving red blood cells to accurately depict vessels through different segmented areas of the eye”

May eliminate the need in certain cases for these intravascular dyes

OCT Disc Angiography: July 2018

Is this dangerous? What is an Optic Nerve Melanocytoma?

Optic Nerve Melanocytoma

“Melanocytoma” coined in 1965 lecture by Dr. Zimmerman

Determined lesion was benign

Challenged by Cogan

proposed “magnocellular nevus of optic nerve”

According to Shields, “hyperpigmented magnocellular nevus of the optic nerve (HMOD)” is more accurate


Now defined as a variant of the melanocytic nevus

Dark brown/deep black color

Mean age of diagnosis – 52

No racial predilection (different from choroidal melanoma)

Slightly more common in females (62% of 115 cases from Wills Eye Hospital series)

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Clinical Features

Unilateral

Sometimes multifocal

Rarely affects visual acuity

Retinal exudation into fovea or tumor necrosis

Induced APD (even with 20/20 eyes!) – 26%

Can cause visual field defects

Can be associated with ocular melanocytosis (8%)

What is an Optic Nerve Melanocytoma?

Shields, Jerry A., et al. "Melanocytoma of the optic disk: a review." Survey of ophthalmology 51.2 (2006): 99.

https://www.slideshare.net/TVRSchool/zografos-tumors-and-pseudotumors-of-the-optic-disc

Differential Diagnosis

1. Optic Nerve Melanocytoma

2. Choroidal Nevus

3. Choroidal Melanoma

4. Combined Hamartoma of the Retina and RPE

5. Hyperplasia of peripapillary RPE

6. Adenoma of the RPE

7. Metastatic Melanoma to the optic disc

Differential Diagnosis

1. Optic Nerve Melanocytoma

2. Choroidal Nevus

3. Choroidal Melanoma

4. Combined Hamartoma of the Retina and RPE

5. Hyperplasia of peripapillary RPE

6. Adenoma of the RPE

7. Metastatic Melanoma to the optic disc

Associated gliosis

Associated with previous trauma/infection

Differentiated with biopsy, can have adjacent exudation

Located adjacent to, not over ONH, limited to choroid

Growth rate, symptoms, size, location

Previous diagnosis of cancer, growth rate

Combined Hamartoma of the RPE

Eye Rounds Reference

Juxtapapillary Adenoma

https://www.retinalphysician.com/archive/2005/May/fig06_shieldsrpe.jpg

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*Hyperplasia of the RPE

Usually associated with

Previous trauma/inflammation

https://www.researchgate.net/figure/Peripapillary-congenital-hypertrophy-of-the-retinal-pigment-epithelium-showing-additional_fig10_262794509

Choroidal Nevus

https://imagebank.asrs.org/file/26490/choroidal-nevus

Choroidal Melanoma

https://imagebank.asrs.org/file/6283/choroidal-melanoma

Choroidal Melanoma

To Find Small Ocular Melanoma, Use Helpful Hints Daily T: Thickness >2mm

F: Fluid subretinally

S: Symptoms

O: Orange pigment present

M: Margins w/in 3 mm of optic disc

U: Ultrasound

H: Hollow

H: Halo absent

D: Drusen Absent


Malignancy Risk Factors:

Thickness >1.5mm

VA is affected (symptoms)

https://imagebank.asrs.org/Content/imagebank/MelanocytomaMPK.jpg/image-full;max$643,0.ImageHandler

Follow-up and Management

Fundus photos

OCT to measure thickness

Fluorescein and indocyanine green angiography to detect presence of feeder vessels

B-Scan ultrasonography

Fundus autofluorescence

OCT-A may offer some utility as far as tracking vascular changes of the lesion

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Risk for malignant transformation – 1-2% (Shields) VA affected?

Thickness?

Risk for malignant transformation of choroidal melanoma – below 1% (Singh et al.) Yet we often scrutinize these…

Gene Expression Profiling (GEP) BAP1 gene

SF3B1 and EIF1AX genes


Change has been documented as late as 33 years after initial diagnosis (Shukla et al.)

Monitor every 12 months (sooner PRN)

Fundus photos

OCT

VA

Consider referral again if considerable change

References

1.) Shields, Jerry A., et al. “Melanocytoma of the Optic Disk: A Review.” Survey of Ophthalmology, vol. 51, no. 2, Mar. 2006, pp. 93–104.

2.) Reidy, James J., et al. “Melanocytoma: Nomenclature, Pathogenesis, Natural History and Treatment.” Survey of Ophthalmology, vol. 29, no. 5, 1985, pp. 319–327.

3.) Friedman, Neil J., et al. The Massachusetts Eye and Ear Infirmary Illustrated Manual of Ophthalmology. (475-476, 503) Elsevier, 2009.

4.)Singh, Arun D., et al. “Estimating the Risk of Malignant Transformation of a Choroidal Nevus.” Ophthalmology, vol. 112, no. 10, 24 June 2005, pp. 1784–1789.

5.) “Clinical Ophthalmology: a Systematic Approach.” Clinical Ophthalmology: a Systematic Approach, by Jack J. Kanski and Brad Bowling, Elsevier, 2012, pp. 496–506.

6.) Shields, Carol L., et al. “Clinical Factors in the Identification of Small Choroidal Melanoma.” Canadian Journal of Ophthalmology / Journal Canadien D'Ophtalmologie, vol. 39, no. 4, 2004, pp. 351–357.

7.)Shields, Carol L., et al. “Melanoma of the Eye: Revealing Hidden Secrets, One at a Time.” Clinics in Dermatology, vol. 33, no. 2, 2015, pp. 183–196.

8.) Shields, Jerry A.., et al. “Total Blindness From Presumed Optic Nerve Melanocytoma.” American Journal of Ophthalmology, vol. 139, no. 6, 19 Nov. 2004, pp. 1113–1114.

9.) Shukla, Shripaad Y., et al. “Transformation of Optic Disc Melanocytoma Into Melanoma Over 33 Years.” Archives of Ophthalmology, vol. 130, no. 10, 1 Oct. 2012, pp. 1344–1347.

10.) Meyer, David, et al. “Malignant Transformation of an Optic Disk Melanocytoma.” American Journal of Ophthalmology, vol. 127, no. 6, 5 Feb. 1999, pp. 710–714.

11.) Apinyawasisuk, Supanut, et al. “Clinical and Spectral-Domain Optical Coherence Tomography Appearance of Optic Disc Melanocytoma: A New Classification and Differentiation from Pigmented Choroidal Lesions.” Ocular Oncology and Pathology, vol. 3, no. 2, 2016, pp. 142–148.

Questions?

It’s Not Rare if it’s in Your Chair - Plaquenil

Noor Abushagur, OD

Ocular Disease Resident at Community Eye Clinic Fort Worth

A little about me…

2/1/2019

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Case #1

59 year old Hispanic female

CC: diabetic exam

Medical Hx: DM, RA, HTN

Medications: unknown arthritis med unknown HTN medication, unknown DM medication

History of Chloroquine use 250mg/day from 2008-2013

BCVA: 20/25 OD/OS

Lens: Moderate combined cataract OU

Macula: bulls eye appearance OU

CASE #1 – Photos

CASE #1 – Macula OCT9/11/2018 Community Eye Center

OSODMacula Thickness OU: Macular Cube 512x128

Signal Strength:

Exam Time:

Exam Date:

Technician:

Gender:

DOB:

ID:

Name:

6/10

12:13 PM

9/11/2018

czmadmin,

Female

2/8/1959

20516

Corrales, Lucia

12:15 PM

7/10

OD OS

Serial Number: 5000-2740 5000-2740

OS ILM-RPE ThicknessOD ILM-RPE Thickness

Fovea: 264, 59 Fovea: 256, 62

OD OCT Fundus

OS ILM-RPE Thickness MapOD ILM-RPE Thickness Map

OD Horizontal B-Scan OS Horizontal B-Scan

OS OCT Fundus

BScan: 59 62BScan:

Doctor's Signature ExamRoom3SW Ver: 7.0.1.290Copyright 2013Carl Zeiss Meditec, IncAll Rights Reserved

Page 1 of 1

Comments

CASE #1 – Visual Field

Case #1 – Assessment & Plan

1. Type II DM without complications, OU

2. Chloroquine Toxicity, OU

3. Combined cataract, OU

4. Hyperopia, presbyopia, OU

Case #2

63 year old African female

CC: reports blurred vision at distance and near (referred from CAN for cataract eval)

Medical Hx: HTN, arthritis, malaria

Medications: amlodipine, iron supplement, Tylenol

Hx of Chloroquine use for malaria x many years

BCVA:

OD: 20/60++

OS: 20/250

Lens: severe combined cataract OS>>OD

Macula: bulls eye appearance

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CASE #2 Case #2

Case #2 – OCT Macula Case #2 – Visual Fields

Case #2 – Assessment and Plan

1. Combined Cataract, OU

2. Chloroquine Toxicity, OU

3. Hyperopia, astigmatism, presbyopia, OU

Plaquenil (hydroxychloroquine)

Uses:Rheumatoid arthritis

Lupus

Sjogren’s

Mechanism: DMARD (disease modifying anti-

rheumatic drug)

Interferes with cell communication

Binds to melanin

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Advantages

Cost

Minimal side effects: nausea and diarrhea

Visual changes are seen rarely

Effects on cholesterol

Hydroxychloroquine (HCQ) may lower LDL levels!!

May also reduce total cholesterol levels

Prescribing Guidelines

Old daily dosage (based on ideal weight)

New recommendation – Use real weight

Very thin patients in particular are at increased risk when dose is calculated by ideal weight (as previously recommended)1

Toxicity based on cumulative dose is no longer a thing!

Toxicity Mechanism is not well understood

Binding to melanin in the RPE may serve to concentrate the agents and contribute to, or prolong their toxic effects1

The primary damage is to the photoreceptors, and as the ONL degenerates, there is secondary disruption of the RPE.1

The macular localization of the disease suggests that light absorption or possibly cone metabolism may play a role, but that is speculation.1

Toxicity Signs

VA usually not affected

CME may develop

Retinopathy can progress after drug is discontinued

Chloroquine (and sometimes HCQ) can cause corneal verticillata

Reversible

No association with retinopathy

Toxicity Stats

Overall statistical risk for retinal toxicity is 7.5%

This varies greatly, depending on daily dose / duration of use

In patients taking <5mg/kg/day HCQ:

Less than 1% risk in first 5 years

Less than 2% risk in first 10 years

20% chance after 20 years!

Who’s At Risk1. Daily dose and duration of use

Dosage >5mg/kg

2. Renal diseaseCQ and HCQ are cleared through kidneys

3. Concurrent Tamoxifen use5x increased risk

4. Retinal and macular disease Adding a retinal toxin to an already diseased retina

Interferes with accuracy of testing for maculopathy

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Who’s At Risk

In Asian patients, toxicity is distributed in a more peripheral pattern along the arcades!

Caucasian: parafoveal

AA and Hispanics: “predominantly” parafoveal

2016 AAO Guidelines1

Screening for toxicity:

Baseline screening

Fundus photo (color/FA), SD-OCT*

5 years**

Yearly (after 5 years of initiating therapy)

Screening Tests 1. Threshold visual field

10-2 for non-Asian patients, 24-2 or 30-2 for Asian patients

Most frequent region for early damage is inferior temporal

2. Spectral-Domain OCT

Parafoveal thinning of PIL?

3. mfERG

Generates local ERG responses across the posterior pole

4. FAF

Hyperautofluorescence: early PR damage

Hypoautofluorescence: late RPE loss

Tests not recommended

Fundus exam / photography

PR damage detected much sooner with other tests

Time Domain OCT

Insufficient resolution

Fluorescein Angio

Can recognize late RPE changes

Full field ERG

Global test of retinal function, shows late toxicity

Amsler Grid

Color Vision

EOG

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References

1. Marmor, M. F. (2016). Recommendations on Screening for Chloroquine and Hydroxychloroquine Retinopathy - 2016. Retrieved from https://www.aao.org/clinical-statement/revised-recommendations-on-screening-chloroquine-h.Academy ppt

2. Harrison, W. (n.d.). Update on Plaquenil Testing: 2016 Guidelines and Beyond. Lecture.

3. Bowling, B. (2016). Kanski’s Clinical Ophthalmology: A systematic approach. Saunders. Questions?

Documents

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