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1 © Mark E. Damon - All Rights Reserved Jeopardy! Ocular Effects of Systemic Disease DISCLOSURE STATEMENT Please silence all mobile devices. Nothing to disclose Caroline Pate, OD, FAAO Beth Steele, OD, FAAO © Mark E. Damon - All Rights Reserved JEOPARDY! Ocular Effects of Systemic Disease Caroline Pate, OD, FAAO Beth Steele, OD, FAAO UAB School of Optometry February 6, 2014 No disclosures AOA’s definition of Optometry approved Sept 2012: Doctors of optometry (ODs) are the independent primary health care professionals for the eye. Optometrists examine, diagnose, treat, and manage diseases, injuries, and disorders of the visual system, the eye, and associated structures as well as identify related systemic conditions affecting the eye. © Mark E. Damon - All Rights Reserved © Mark E. Damon - All Rights Reserved Rules: 1 round, lasting ~50 minutes In order to ensure no cheating—handouts were just uploaded online—if you want to play, don’t reference them until the conclusion of the lecture Your i>clicker response device will be used to input your answer (multiple choice). You will be given 15 seconds to submit your answer. Correct answers will “earn” you points related to the dollar value of the category. Incorrect answers will “lose” you money. Everyone has the opportunity to answer at the same time. Keep track of your correct answers (and incorrect). We will use this information to crown the “jeopardy” champion at the conclusion of the lecture. **All rules are subject to the loose interpretation by Drs. Pate and Steele © Mark E. Damon - All Rights Reserved Let’s practice using our clickers! Which “fun fact” below about Atlanta is NOT TRUE? A) There are over 50 streets in Atlanta that use the name “Peachtree”. B) The famous Varsity restaurant serves the highest amount of Coca-Cola in the world. C) The Centennial Olympic Games were held in Atlanta in 2006. D) The Hartsfield-Jackson Atlanta International Airport still holds the title of “worlds busiest airport”. Source: interestingfunfacts.com © Mark E. Damon - All Rights Reserved Let’s Get Physical The Terrifying Truth Hodge Podge $100 $100 $100 $100 $200 $200 $200 $200 $300 $300 $300 $300 $400 $400 $400 $400 Scores Anterior Segment Clues
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Page 1: © Mark E. Damon - All Rights Reserved DISCLOSURE STATEMENT ... · 5 © Mark E. Damon - All Rights Reserved Your patient reports that when he turns his head a certain way, his vision

1

© Mark E. Damon - All Rights Reserved

Jeopardy! Ocular Effects of Systemic Disease

DISCLOSURE STATEMENT

Please silence all mobile devices.

Nothing to disclose

Caroline Pate, OD, FAAOBeth Steele, OD, FAAO

© Mark E. Damon - All Rights Reserved

JEOPARDY!Ocular Effects of Systemic Disease

Caroline Pate, OD, FAAOBeth Steele, OD, FAAO

UAB School of Optometry

February 6, 2014

No disclosures

AOA’s definition of Optometryapproved Sept 2012:

Doctors of optometry (ODs) are the independent primary health care professionals for the eye.

Optometrists examine, diagnose, treat, and manage diseases, injuries, and disorders of the visual system,

the eye, and associated structures as well as identify related systemic conditions affecting the eye.

© Mark E. Damon - All Rights Reserved © Mark E. Damon - All Rights Reserved

Rules:

• 1 round, lasting ~50 minutes

• In order to ensure no cheating—handouts were just uploaded online—if you want to play, don’t reference them until the conclusion of the lecture

• Your i>clicker response device will be used to input your answer (multiple choice). You will be given 15 seconds to submit your answer. Correct answers will “earn” you points related to the dollar value of the category. Incorrect answers will “lose” you money.

• Everyone has the opportunity to answer at the same time.

• Keep track of your correct answers (and incorrect). We will use this information to crown the “jeopardy” champion at the conclusion of the lecture.

• **All rules are subject to the loose interpretation by Drs. Pate and Steele

© Mark E. Damon - All Rights ReservedLet’s practice using our clickers!

Which “fun fact” below about Atlanta is NOT TRUE?

A) There are over 50 streets in Atlanta that use the name “Peachtree”.

B) The famous Varsity restaurant serves the highest amount of Coca-Cola in the world.

C) The Centennial Olympic Games were held in Atlanta in 2006.

D) The Hartsfield-Jackson Atlanta International Airport still holds the title of “worlds busiest airport”.

Source: interestingfunfacts.com

© Mark E. Damon - All Rights Reserved

Let’s Get Physical

The Terrifying

Truth

Hodge Podge

$100 $100 $100 $100

$200 $200 $200 $200

$300 $300 $300 $300

$400 $400 $400 $400

Scores

Anterior Segment

Clues

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2

© Mark E. Damon - All Rights Reserved

$100$100Answer

Which of the following findings is

outside normal limits? A) Oral temperature of 96.4⁰F

B) Heart Rate of 62 bpm

C) Blood Pressure of 140/90 mmHg

D) Respiration rate of 20 breaths/minute

E) BMI of 23

Which of the following findings is

outside normal limits? A) Oral temperature of 96.4⁰F

B) Heart Rate of 62 bpm

C) Blood Pressure of 140/90 mmHg

D) Respiration rate of 20 breaths/minute

E) BMI of 23

© Mark E. Damon - All Rights Reserved

$100$100

What is C (BP of 140/90)?What is C (BP of 140/90)?

© Mark E. Damon - All Rights Reserved

Infection

Patient Vital Signs

• Temperature – 96.4ͦ - 99.1ͦ

• Blood Pressure – <120/<80

• Respiration Rate – 20 breaths/min

• Heart Rate – 50-90bpm

• Others– Weight/height

BMI<25

– Pain

© Mark E. Damon - All Rights Reserved

Is routine blood pressure part of your daily routine in patient care?

HYPERTENSION

• Over 70 million people in US– 20-25% unaware

• 7.1 million deaths per year– “Silent Killer”

– Stroke, MI, End-stage Renal Disease

© Mark E. Damon - All Rights Reserved

Blood Pressure Classifications and Referral Guidelines

(adapted from the Joint National Committee on Detection, Evaluation, and Treatment of High Blood Pressure – JNC 7, 2003)

Hypotension normal Pre- HTN Stage 1 Stage 2 Critical High Point

Systolic < 90 < 120 120-139 140- 159 ≥160 >180

Diastolic < 60 < 80 80 - 89 90-99 ≥100 >110

Confirm within 2 months

Evaluate or refer to

PCP within 1 month

Evaluate or refer immediately or within 1 week

© Mark E. Damon - All Rights Reserved

What’s new from JNC 8?What’s new from JNC 8?

• Threshold for treatment of BP in ages ≥60– 150/90 vs. 140/90

• Recommendations for initial therapy– Thiazide diuretics

– ACE inh, ARBs, Ca2++ channel blockers

– NOT: β-blockers, α-blockers, loop diuretics

From: 2014 Evidence-Based Guideline for the Management of High Blood Pressure in Adults: Report From the Panel Members Appointed to the Eighth Joint National Committee (JNC 8)

JAMA. 2013;():. doi:10.1001/jama.2013.284427

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© Mark E. Damon - All Rights Reserved

Same BP – 2 different situationsBP 190/112 BP 190/112• Feeling “fine”

• Forgot his medicine today

• Denies H/A, etc

• DFE: crossing changes

• (+) “migraine”since yesterday

• DFE: disc edemaflame heme

Home

© Mark E. Damon - All Rights Reserved

What is:

A) Hemorrhagic strokeB) Ramsay-Hunt syndromeC) Lyme diseaseD) Bell’s palsyE) Epstein Barr virus

$200$200Answer

Cranial nerve testing on the patient below with this condition revealed

involvement of CN VII and VIII.

Cranial nerve testing on the patient below with this condition revealed

involvement of CN VII and VIII.

© Mark E. Damon - All Rights Reserved

$200$200

What is B?

(Ramsay Hunt Syndrome)

What is B?

(Ramsay Hunt Syndrome)

© Mark E. Damon - All Rights Reserved

Ramsay Hunt SyndromeRamsay Hunt Syndrome

• Varicella Zoster Virus reactivation in geniculate ganglion

• Symptoms: pain, hearing loss, dizziness, tinnitus, nausea, vertigo

• Poorer prognosis than Bell’s palsy• 35% completely recover• Recurrences rare

• Treatment • oral antivirals + oral prednisone• Protect the cornea!

• Varicella Zoster Virus reactivation in geniculate ganglion

• Symptoms: pain, hearing loss, dizziness, tinnitus, nausea, vertigo

• Poorer prognosis than Bell’s palsy• 35% completely recover• Recurrences rare

• Treatment • oral antivirals + oral prednisone• Protect the cornea!

© Mark E. Damon - All Rights Reserved

Updated Clinical Practice Guidelines: Bell’s Palsy

• Recommends prescribing oral steroids within 72 hours of symptom onset for patients with Bell’s palsy 16 years and older

• Recommends offering oral antiviral therapy in addition to oral steroids within 72 hours of symptom onset

• Other recommendations:– Inclusion of eye care for impaired lid closure

– Careful history and physical exam to rule out other causes

– Against routine laboratory testing and imaging for new onset Bell’s palsy

Baugh R, Basura G, Ishii L, et al. Clinical practice guideline: Bell’s Palsy. Otolaryngol Head Neck Surg 2013; 149(suppl 3) S1-S27.

© Mark E. Damon - All Rights ReservedSummary of Cranial Nerve Functions and Testing(Adapted from Muchnick, B. Clinical Medicine in Optometric Practice, 2nd ed.)

Summary of Cranial Nerve Functions and Testing(Adapted from Muchnick, B. Clinical Medicine in Optometric Practice, 2nd ed.)

Cranial Nerve Test

I – Olfactory Identify odors

II - Optic Visual acuity, visual field, color, nerve head

III - Oculomotor Physiologic “H” and near point response

IV – Trochlear Physiologic “H”

V - Trigeminal Corneal reflex; clench jaw/palpateLight touch comparison

VI - Abducens Physiologic “H”

VII - Facial Smile, puff cheeks, wrinkle forehead, pry open closed lids

VIII - Vestibulocochlear Rinne test for hearing, Weber test for balance

IX - Glossopharyngeal Gag reflex

X - Vagus Gag reflex

XI – Accessory Shrug, head turn against resistance

XII - Hypoglossal Tongue deviation Home

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© Mark E. Damon - All Rights Reserved

Which of the following is consistent with the diagnosis of Diabetes

Mellitus?

$300$300 Answer

A) HbA1c of 6.9%

B) Random plasma glucose of 180 mg/dl

C) Fasting plasma glucose of 100 mg/dl

D) OGTT 2 hour post-load glucose of 140mg/dl

A) HbA1c of 6.9%

B) Random plasma glucose of 180 mg/dl

C) Fasting plasma glucose of 100 mg/dl

D) OGTT 2 hour post-load glucose of 140mg/dl

© Mark E. Damon - All Rights Reserved

$300$300

What is A (HbA1c of 6.9%)?What is A (HbA1c of 6.9%)?

© Mark E. Damon - All Rights Reserved

Current ADA Diagnostic Criteria for DM

• HbA1c ≥ 6.5%

• Random plasma glucose ≥ 200mg/dL + symptoms (polyuria, thirst, wt loss, blurred vision)

• Fasting plasma glucose ≥ 126mg/dL

• OGTT 2 hour post-load glucose ≥ 200mg/dL

American Diabetes Association. Standards of Medical Care in Diabetes 2014.

© Mark E. Damon - All Rights Reserved

The Diabetes Epidemic…

• 86% of Type 1 diabetics40% of Type 2 diabetics

• 1 in 3 children born today will develop diabetes in their lifetime

• 1/3 to 1/2 of diabetic patients do not receive an annual eye examination

• By 2050, the number of patients with diabetic retinopathy will triple

have clinically evident diabetic

retinopathy

Hazin R, Barazi MK, Summerfield M. Challenges to establishing nationwide diabetic retinopathy screening programs. Curr Opin Ophthalmology 2011; 22: 174-179.

© Mark E. Damon - All Rights Reserved

In-office Glucometry

Blood glucose reading in ~5 seconds

82962QW Glucometry (with at-

home device)

Average reimbursement $3.42

© Mark E. Damon - All Rights Reserved

In-office HbA1c Testing

A1C Now+® (pts Diagnostics) 99% lab accuracyResults in 5 minuteswww.a1cnow.com Available in sets of 10 or 20

83037 QWHemoglobin; glycosolated (A1c) by

device cleared by FDA for home use

Average reimbursement $13.00-18.00

Home

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© Mark E. Damon - All Rights Reserved

Your patient reports that when he turns his head a certain way, his vision “goes out” in the right eye. OD – mid-peripheral retinal hemes 4 quadrants

OS – clear Which of the following methods of evaluation would be

most useful in his/her management?

$400$400 Answer

A) Look for an SVP

B) Watzke-Allen

C) Carotid Artery Auscultation

D) OCT

E) VEP

A) Look for an SVP

B) Watzke-Allen

C) Carotid Artery Auscultation

D) OCT

E) VEP

© Mark E. Damon - All Rights Reserved

$400$400

What is C (Carotid Artery Auscultation)?

What is C (Carotid Artery Auscultation)?

© Mark E. Damon - All Rights Reserved © Mark E. Damon - All Rights Reserved

© Mark E. Damon - All Rights Reserved

• Bruit : ≥50% stenosis

• ≥70% blockage before ocular manifestations– 90% occlusion=50%

decrease in CRA perfusion pressure

• 5 year mortality rate – 40% Home

© Mark E. Damon - All Rights Reserved

This “syndrome” is characterized by abdominal obesity, dyslipidemia,

elevated blood pressure, and insulin resistance.

$100$100Answer

A) Metabolic SyndromeB) Diabesity SyndromeC) Millenial SyndromeD) McDonalds Syndrome

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© Mark E. Damon - All Rights Reserved

What is A?

(Metabolic Syndrome)

$100$100© Mark E. Damon - All Rights Reserved

© Mark E. Damon - All Rights Reserved

Large Waist Size For men: 40 inches or largerFor women: 35 inches or

larger

Cholesterol: High Triglycerides

Either150 mg/dL or higher

orUsing a cholesterol medicine

Cholesterol: Low Good Cholesterol (HDL)

EitherFor men: Less than 40 mg/dL

For women: Less than 50 mg/dL

orUsing a cholesterol medicine

High Blood Pressure

EitherHaving blood pressure of 135/85 mm Hg or greater

orUsing a high blood pressure

medicine

Blood Sugar: High Fasting Glucose Level 100 mg/dL or higher

3 or more are diagnostic of Metabolic Syndrome:

Large Waist Size For men: 40 inches or largerFor women: 35 inches or larger

Cholesterol: High Triglycerides 150 mg/dL or higher

Cholesterol: Low Good Cholesterol (HDL)For men: Less than 40 mg/dL

For women: Less than 50 mg/dL

High Blood Pressure 135/85 mm Hg or greater

Blood SugarFasting Glucose: 100 mg/dL or higher

© Mark E. Damon - All Rights Reserved

Obesity Rates in US

© Mark E. Damon - All Rights ReservedPrevalence* of Self-Reported Obesity Among U.S. Adults by State and Territory, BRFSS, 2013

*Prevalence estimates reflect BRFSS methodological changes started in 2011. These estimates should not be compared to prevalence estimates before 2011.

CA

MT

ID

NV

UT

AZNM

WY

WA

OR

CO

NE

ND

SD

TX

OK

KS

IA

MN

AR

MO

LA

MI

IN

KY

ILOH

TN

MS AL

WI

PA

WV

SC

VA

NC

GA

FL

NY

VT

ME

HI

AK

NH

MA

RI

CTNJ

DE

MD

DC

PRGUAM

15%–<20% 20%–<25% 25%–<30% 30%–<35% ≥35%

Source: CDC Behavioral Risk Factor Surveillance System

© Mark E. Damon - All Rights Reserved

Ideal Body Weight and BMI

Hamwi Formula for Ideal Body Weight:

For men: 105 lb for the first 5 ft; 5 lb for each inch over 5 ftFor women: 95 lb for the first 5 ft; 4 lb for each inch over 5 ft

Body Mass Index= Weight (lbs) / [Height (in)2] x 703

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© Mark E. Damon - All Rights Reserved

Prediabetes

A1C: 5.7% – 6.4%

Fasting BS: 100 – 125 mg/dl

OGTT: 140 -199 mg/dl

Upwards of 20% of patients with “prediabetes” may show signs of NPDR

Chen et al. Eye (2012) 26, 816–820 Home

© Mark E. Damon - All Rights Reserved

Transmission from patient to patient of which of the following pathogens has

been linked to Goldmann ApplanationTonometry?

$200$200Answer

A) Hepatitis C

B) HIV

C) Herpes

D) MRSA

E) Adenovirus

A) Hepatitis C

B) HIV

C) Herpes

D) MRSA

E) Adenovirus

© Mark E. Damon - All Rights Reserved

$200$200

What is E (Adenovirus) ? What is E (Adenovirus) ?

© Mark E. Damon - All Rights Reserved

© Mark E. Damon - All Rights Reserved

• Centers for Disease Control and Prevention

• Guideline for Disinfection and Sterilization in Healthcare Facilities, 2008– Update from 1985– 158 pages (1035

references)• 2 pages

specifically dedicated to tonometers

© Mark E. Damon - All Rights Reserved

Disinfection based on Spaulding Classification

Body Contact Disinfection Requirement

FDA Device Class

Sterile body cavity or blood present

Sterilization Critical

Mucous membrane or non-intact skin

High level Semi-critical

Intact skin Low level Non-critical

CDC, Guideline for Disinfection and Sterilization in Healthcare Facilities, 2008

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© Mark E. Damon - All Rights Reserved

Tonometer probes

• Clean probe

• High level disinfection with soak in 3% H2O2 or 1:10 bleach• Do not leave in solution

for > 1 hour!

• Rinse thoroughly

• Dry and store

Home

© Mark E. Damon - All Rights Reserved

$300$300 Answer

The ADA recommends that pregnant patients

with pre-existing diabetic retinopathy

should receive a DFE:A) During the 1st trimester

B) During the 2nd trimester

C) During the 3rd trimester

D) Once per trimester

E) After delivery, since dilating drops are contraindicated in pregnancy

The ADA recommends that pregnant patients

with pre-existing diabetic retinopathy

should receive a DFE:A) During the 1st trimester

B) During the 2nd trimester

C) During the 3rd trimester

D) Once per trimester

E) After delivery, since dilating drops are contraindicated in pregnancy

© Mark E. Damon - All Rights Reserved

$300$300

What is a D (once per trimester)?What is a D (once per trimester)?

© Mark E. Damon - All Rights Reserved

Pregnancy and Diabetic Retinopathy

Pregnancy and Diabetic Retinopathy

• Main risk factor for DM worsening during pregnancy is baselineseverity of diabetic retinopathy

• 2.5x increased risk of worsening of retinopathy

• Recommend HbA1c <6% in pregnant patients with pre-existing Type I or II DM

• Main risk factor for DM worsening during pregnancy is baselineseverity of diabetic retinopathy

• 2.5x increased risk of worsening of retinopathy

• Recommend HbA1c <6% in pregnant patients with pre-existing Type I or II DM

© Mark E. Damon - All Rights Reserved

Gestational DiabetesGestational Diabetes• 5-10% of pregnancies

• Diagnosed 2nd-3rd trimester

• Glucose tolerance typically returns to normal 6 weeks post-partum

• Due to short/temporary duration GDM does not typically lead to development of diabetic retinopathy

• Patients with a hx of GDM have a 35-60% chance of developing Type II DM in next 10-20 years

• 5-10% of pregnancies

• Diagnosed 2nd-3rd trimester

• Glucose tolerance typically returns to normal 6 weeks post-partum

• Due to short/temporary duration GDM does not typically lead to development of diabetic retinopathy

• Patients with a hx of GDM have a 35-60% chance of developing Type II DM in next 10-20 years

Home

© Mark E. Damon - All Rights Reserved

$400$400 Answer

A 72 yo male presents with complaints of blurred vision that resolved after about 30 minutes.

After further questioning, you conclude your patient has had a TIA. What is this patient’s risk

of suffering a stroke within the next 5 years?

A) 5%

B) 10%

C) 40%

D) 75%

A 72 yo male presents with complaints of blurred vision that resolved after about 30 minutes.

After further questioning, you conclude your patient has had a TIA. What is this patient’s risk

of suffering a stroke within the next 5 years?

A) 5%

B) 10%

C) 40%

D) 75%

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© Mark E. Damon - All Rights Reserved

$400$400

What is C (40%)? What is C (40%)?

© Mark E. Damon - All Rights Reserved

Risk of stroke after TIA• Consider your risk factors in patients with TIA and act

F.A.S.T!– Starting medical treatment within 24 hours of a TIA ↓ the risk

of stroke within 3 mos by 80%

• ABCD2 rule : ≥3 points = emergency – Age>60 (1 pt)

– BP ≥140/90 on first assessment after TIA (1 pt)

– Clinical features of TIA (unilateral weakness=2 pts or speech impairment w/o weakness=1 pt)

– Duration of TIA (≥60 minutes=2 pts; 10-59 minutes=1 pt)

– Diabetes (1 pt)

Johnston WC, et al. Lancet. 2007; 369:  283‐292.

© Mark E. Damon - All Rights Reserved

Management of intra-arteriolar plaque

• Symptoms?– Often transient – plaques are pliable

– Correlated with degree of occlusion?

– Predictive of future events?

• Antiplatelets? Blood thinners? – Eliquis (apixaban)

• Doppler

• EKG/Angiography

11% with symptoms had significant occlusion Wakefield, et al

22% w/o symptoms had 30-60% occlusion Dunlap, et al

Home

© Mark E. Damon - All Rights Reserved

The anterior segment finding pictured below can be associated with what systemic

disease?

$100$100Answer

A) Lupus

B) Sjogren’s Syndrome

C) Fabry Disease

D) Wilson’s Disease

A) Lupus

B) Sjogren’s Syndrome

C) Fabry Disease

D) Wilson’s DiseasePhoto courtesy of Melanie Sivley, OD

© Mark E. Damon - All Rights Reserved

$100$100

What is C (Fabry’s Disease)? What is C (Fabry’s Disease)?

© Mark E. Damon - All Rights Reserved

Photos courtesy of Melanie Sivley, OD

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© Mark E. Damon - All Rights Reserved

Fabry Disease• Inherited disorder caused by a lysosomal

enzyme (alpha-galactosidase A) deficiency

• Leads to insufficient breakdown of lipids throughout the body (eyes, kidneys, heart)

• Non-ocular manifestations include:– Progressive and/or unexplained chronic kidney

disease

– Premature cardiac disease and/or stroke

– GI difficulties

– Angiokeratomas (reddish-purple skin lesions that do not blanch with pressure) Home

© Mark E. Damon - All Rights Reserved

A 66 year old overweight male presents complaining of droopy upper eyelids and eye irritation/burning for several months. Lids are

easily everted. What condition would you suspect?

$200$200Answer

A) Graves Disease

B) Obstructive Sleep Apnea

C) Myasthenia Gravis

A) Atopic dermatitis

A) Graves Disease

B) Obstructive Sleep Apnea

C) Myasthenia Gravis

A) Atopic dermatitis

© Mark E. Damon - All Rights Reserved

$200$200

What is B (Obstructive Sleep Apnea)?

What is B (Obstructive Sleep Apnea)?

© Mark E. Damon - All Rights Reserved

Impaired blood flow in OSA

• Systemic complications:– HTN

– Type 2 DM

– Congestive heart failure

– Coronary artery disease

– Atrial fibrillation

– Stroke

© Mark E. Damon - All Rights Reserved

Ocular Complications of OSA• Floppy eyelid

syndrome

• Keratoconus

• Non-arteritic anterior ischemic optic neuropathy

• Glaucoma (NTG, OAG)

• Central serous chorioretinopathy

• CRVOHome

© Mark E. Damon - All Rights Reserved

Along with ptosis, this polychromatic cataract is a classic finding in which of the

following systemic conditions?

$300$300Answer

A) Wilson’s Disease

B) Myotonic Dystrophy

C) Myasthenia Gravis

D) Diabetes

A) Wilson’s Disease

B) Myotonic Dystrophy

C) Myasthenia Gravis

D) Diabetes

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© Mark E. Damon - All Rights Reserved

$100$100

What is B (myotonic dystrophy)? What is B (myotonic dystrophy)?

$300$300© Mark E. Damon - All Rights Reserved

Myotonic Dystrophy• Most common adult form of

muscular dystrophy– 1 in 8000 patients

– often presents 2nd-3rd decade

– Autosomal dominant inheritance• Genetic mutation in the protein

kinase gene (DMPK) located on chromosome 19q13.3

• Characterized by myotonia (slow relaxation of muscles after contraction), muscle weakness, and progressive muscle atrophy

© Mark E. Damon - All Rights Reserved

Myotonic Dystrophy• Ocular complications

accompany all cases of myotonic dystrophy– Polychromatic “Christmas Tree”

lenticular opacities (100%)May be cortical, sutural, or subcapsular in location

– Bilateral ptosis (80%)

– Lagopthalmos

– Extraocular muscle involvement (XP, CI)

– Hypotony

– Macular pigment disruption (25%) Home

© Mark E. Damon - All Rights Reserved

As pictured below, iris flocculi may rarely be associated with which of the following?

A) Aortic dissectionB) SarcoidosisC) LymphomaD) Crohn’s disease

$400$400 Answer

© Mark E. Damon - All Rights Reserved

$400$400

What is A?

Aortic dissection

What is A?

Aortic dissection

© Mark E. Damon - All Rights Reserved

Lewis RA, Merin LM. Iris flocculus and familial aortic dissection. Arch Ophthalmol 1995; 113(10):1330-1.

Iris Flocculi

• Multiple pigment epithelial cysts extending up to 360 degrees around pupillary margin

• Mostly congenital

• Generally no systemic manifestations

• Association with familial aortic dissection in adults– Due to connective tissue weakness

– Linked to mutation in smooth muscle ACTA2

Home

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© Mark E. Damon - All Rights Reserved

$100$100Answer

Based on the American Academy of Ophthalmology 2011 recommendations, screening for Chloroquine and Hydroxychloroquine Maculopathy should include all of the following EXCEPT:

A) Best visual acuity

B) Dilated ophthalmoscopic examination of macular and perimacular tissues

C) 10-2 visual field testing

D) Spectral domain OCT paramacular scan

E) Amsler grid

Based on the American Academy of Ophthalmology 2011 recommendations, screening for Chloroquine and Hydroxychloroquine Maculopathy should include all of the following EXCEPT:

A) Best visual acuity

B) Dilated ophthalmoscopic examination of macular and perimacular tissues

C) 10-2 visual field testing

D) Spectral domain OCT paramacular scan

E) Amsler grid

© Mark E. Damon - All Rights Reserved

$100$100

What is E (Amsler Grid)?What is E (Amsler Grid)?

© Mark E. Damon - All Rights Reserved

American Academy of Ophthalmology Revised Recommendations on Screening for

Chloroquine and Hydroxychloroquine Retinopathy Marmor MF, et al. Ophth Feb 2011.

• Risk of toxicity increases sharply towards 1% after 5-7 years of use, or cumulative dose of 1000 g HCQ

• After initial baseline exam, screenings should occur annually after 5 years (or more regularly if unusual risk)

• Screening should include:– Regular exams with dilated fundus

examination– 10-2 visual fields– Objective testing

• Spectral Domain OCT• Fundus autofluorescence• mfERG

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Plaquenil -- What to look for on OCT…

• Outer retina– Loss of IS/OS line (PIL); thinning of PR layer

– Thickening of outer band of RPE

• Inner retina– Parafoveal thinning of GCL, IPL

– 1.0mm (but not 0.5mm) from foveal center

Marmor MF, et al. Ophthalmology. AAO Revised Recommendations on Screening for Chloroquine and Hydroxychloroquine Retinopathy. Feb 2011.

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But WAIT!! • 10% of patients with a ring scotoma

do NOT show damage with SD-OCT!

Marmor MF, Melles RB. Ophthalmology. 2014 Jan 15. pii: S0161-6420(13)01174-3. doi: 10.1016/j.ophtha.2013.12.002. Disparity between Visual Fields and Optical Coherence Tomography in Hydroxychloroquine Retinopathy.

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$200$200Answer

A) Factor V Leiden

B) Anemia

C) Occupation requiringheavy lifting

D) Lyme Disease

A) Factor V Leiden

B) Anemia

C) Occupation requiringheavy lifting

D) Lyme Disease

Which of the following is least likely to be a contributing factor for the retinal

hemorrhage pictured?

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Roth spot???

What is D (Lyme Disease)? What is D (Lyme Disease)?

Lots of reasons for retinal hemes….

$200$200© Mark E. Damon - All Rights Reserved

Factor V Leiden??? What’s that?!!

• Factor V – clotting protein– genetic mutation: ↑clotting in

veins • Caucasians of European

descent• Often undiagnosed,

however….• Complications

– Miscarriage and clots in pregnancy

– deep vein thrombosis– pulmonary embolisms – CRVO

Fegan CD et al, Eye (2002) Home

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A)

Your patient below has developed a CNVM and subretinal hemorrhage. Subtle angioid streaks are seen radiating from the disc. Which of the following systemic etiologies most commonly presents with angioid streaks?

A) Pseudoxanthoma ElasticumB) Ehler’s DanlosC) Paget’s DiseaseD) Sickle Cell Anemia

$300$300 Answer© Mark E. Damon - All Rights Reserved

$300$300

What is A (PXE)What is A (PXE)

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Systemic disorders associated with Angioid Streaks

• Aging• Pseudoxanthoma elasticum (85%)• Sickle cell hemoglobinopathies (1-2%)• Acquired hemolytic anemia• Hyperphosphatemia• Ehlers-Danlos syndrome• Neurofibromatosis• Sturge-Weber syndrome• Hypertensive disorders• Dwarfism• Internal carotid aneurysm• Thrombocytopenia• Facial hemangioma• Systemic lupus erythematosus• Retinitis albuminuria• Generalized vascular disease

• Diabetes mellitus• Hemochromatosis• Paget’s disease (8-15%)• Hypercalcinosis• Acromegaly• Lead poisoning• Senile elastosis• Tuberous sclerosis• Diffuse lipomatosis• Marfan syndrome• Pituitary tumor• Epilepsy• Trauma• Exsanguination• Post-inflammation• Gardner’s syndrome

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Angioid Streaks

• Usually peripapillary– 27% remain around disc; 73% radiate outward

• Typically bilateral• 50-100 micron width, more sinuous course than bv• Typically develop between the 2nd and 5th decade of

life• 50-65% of cases associated with systemic disorder• Asymptomatic early on• Up to 70% of pxs with angioid streaks develop some

vision threatening complication secondary to CNV, choroidal rupture, or foveal involvement Home

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$400$400Answer

Your 84 year old female patient presents with complaints of headache and sudden vision loss. ESR and CRP are markedly elevated, and temporal artery biopsy is positive. What is the likelihood, if left untreated that this patient will suffer vision

loss in the fellow eye?

Your 84 year old female patient presents with complaints of headache and sudden vision loss. ESR and CRP are markedly elevated, and temporal artery biopsy is positive. What is the likelihood, if left untreated that this patient will suffer vision

loss in the fellow eye?

A) 100%

B) 90%

C) 50%

D) 25%

E) <10%

A) 100%

B) 90%

C) 50%

D) 25%

E) <10%

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$400$400What is B (90%)?

Giant Cell Arteritis•Systemic inflammatory vasculitis

– Less common causes which can lead to AAION include polyarteritis nodosa, SLE and herpes zoster

– polymyalgia rheumatica – up to 30% will have GCA

•>50 years of age

•Other ocular manifestations: – Retinal artery occlusion—presenting in 10%– CN III, IV or VI palsy

•Increased risk for stroke, MI

What is B (90%)?

Giant Cell Arteritis•Systemic inflammatory vasculitis

– Less common causes which can lead to AAION include polyarteritis nodosa, SLE and herpes zoster

– polymyalgia rheumatica – up to 30% will have GCA

•>50 years of age

•Other ocular manifestations: – Retinal artery occlusion—presenting in 10%– CN III, IV or VI palsy

•Increased risk for stroke, MI

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Check your score!!$2000+ You are a Systemic Disease SUPERSTAR!

$1000-$1999 Great job!

0-$999 Better luck next time.

<0 The next systemic dz class is at 1:30.