Retinal Vascular Disease

Don Simpson, O.D.

St. Louis VA Medical Center

WELCOME TO MID-TOWN 915 N GRAND BLVD2 BLOCKS N OF THE FOX THEATER

St. Louis VAMC - John Cochran Division

St. Louis VA Rotation

2 divisions John Cochran – mid town Jefferson Barracks- south county Equal time both divisions

Case # 1

71 yo male pt presents for routine eye exam No remarkable findings until Fundus

examination Med hx remarkable for htn, ateriosclerosis Rt eye reveals following:

Diagnosis

Retinal embolus- Hollenhorst plaque What now?

Return to history

Anterior circulation TIA SX ? Amaurosis fugax Unilateral motor disturbance Unilateral numbness, tingling Slurred speech Momentary confusion 50-75% of stroke patients have TIAs

Return to examination

Additional tests Auscultation of carotid arteries What if normal? What if bruit?

Management

? TIA sx present TIA sx absent

No TIA Sx

Primary care provider, Neurology Antiplatelet meds if on none, ASA contraindications 81 mg qd Carotid ultrasound Echocardiogram

TIA SX

Urgent care Primary care provider Neurology ER

St Louis VA Neurology

Heading of Neuro consult work sheet at St. Louis VA “ TRANSIENT ISCHEMIC ATTACK IS A MEDICAL EMERGENCY. If TIA is suspected , please refer the patient to the ER or page the Neuro resident on call for an immediate assessment. TIA should also mean: TAKE IMMEDIATE ACTION “

Additional signs carotid insufficiency

Hypo-perfusion retinopathy Ocular ischemic syndrome

Hypo-perfusion retinopathy

Peripheral retinal hemorrhages associated with decreased retinal artery perfusion pressure

Ocular ischemic syndrome

NVI Retinal neovascularization Neovascular glaucoma

Surgical management carotid disease

Carotid angiogram Gold standard to evaluate stenosis Invasive procedure Carotid endarterectomy

Cardiac sources of emboli

Mitral valve disease Arrhythmias- a fib , vent tach Valve replacement thrombi Sbe

Retinal Emboli and Stroke

Beaver Dam study- population based Looked at risk of CVA with retinal emboli Results published in Archives of Ophth Vol

117; Aug 99. [1063-68]

Findings in Beaver Dam Study

Emboli prevalence 1.3% [3.1%>75yo] Emboli not present at follow up 90% of the

time With emboli 3x greater risk of fatal CVA in 8

years than if no emboli present

TMVL & HTIA

Transient monocular vision loss Hemispheric TIA Stroke risk lower if only tmvl v htia sx based

on large trials NASCET, ECST 3yr risk CVA with med tx 10% tmvl 3yr risk CVA with med tx 20% htia Why risk different?

6 RISK FACTORS FOR STROKE IN TMVL

MALE 75yo or > Hx htia or stroke Hx intermittent claudication Internal carotid stenosis of 80-94% Absence of collateral vessels on angiogram 3 of these risk factors with TMVL carotid

endarterectomy beneficial

ASYMPTOMATIC HOLLENHORST PLAQUE

No evidence to suggest that carotid endarterectomy is of benefit

Prevalence of Stroke by Age and Sex

NHANES: 1999-2002

Source: CDC/NCHS and NHLBI.

1.1

3.1

6.6

11.5

0.41.2

12.0

0.3 0.82.1

3.0

6.3

0

2

4

6

8

10

12

14

20-34 35-44 45-54 55-64 65-74 75+

Ages

Per

cen

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op

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tio

n

Men Women

`

Estimated Direct and Indirect Costs of Cardiovascular Diseases and StrokeUnited States: 2005

Source: Heart Disease and Stroke Statistics – 2005 Update.

254.8

142.1

56.8 59.727.9

393.5

050

100150200250300350400450

Hea

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se

Cor

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Dis

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Str

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VD

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Bil

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Percentage Breakdown of Deaths From Cardiovascular DiseasesUnited States:2002 Preliminary

Source: CDC/NCHS.

18%

6%

5%

4%0%0%

13%

53%

Coronary Heart Disease

Stroke

Congestive Heart Failure

High Blood Pressure

Diseases of the Arteries

Rheumatic Fever/RheumaticHeart Disease

Congenital CardiovascularDefects

Other

Case 2

Sudden vision loss right eye few days duration

65 yo male Ocular hx unremarkable Med hx hypertension, diabetes

Examination

Best corrected vision - 10/400 Right afferent pupillary defect

DIAGNOSIS

CRVO 2 types Ischemic vs non ischemic likely ischemic

Ischemic CRVO

VA less than 20/200 APD Numerous CWS > 10 disc areas of capillary nonpefusion 30% of all CRVO- 50-60% develop NVG NVG 3-4 months [90 day glaucoma]

Nonischemic CRVO

VA usually better than 20/200 or better No APD Few cotton wool spots May progress to ischemic CRVO

Uncommon Etiologies of CRVO

Polycythemia Plasma cell dyscrasias

Polycythemia

Increased RBC and blood volume Polycythemia vera- idiopathic Secondary polycythemia- erythrocytosis Erythrocytosis can be due to hypoxia or

condition causing increased stimulating factor

Plasma Cell Dyscrasias

Multiple myeloma Waldenstrom’s macroglobulinemia Malignant production of immunoglobulins Increased serum viscosity

Homocystinemia

Elevated homocystine levels associated with atherosclerosis and CRVO

Normal homocystine metabolism, but elevated levels of the amino acid

Neovascular glaucoma

Response to ischemia Difficult to manage Intractable pain/ enucleation

Clinical Management and Natural History of CRVO Arch Ophth Vol 115, Apr 97, 486-91 Prognostic value of initial visual acuity 20/40 or better- likely good outcome 20/50-20/200- variable prognosis 20/200 or worse- poor prognosis, likely

ischemic, high risk NVI, ANV 56% of <20/200 had NVI and ANV at one

month

Follow up CRVO based on Initial Visual Acuity >20/40 q 1-2 months for 6 months 20/50-200 q 1-2 months for 6 months <20/200 q 1 month for 6 months

CRVO Treatment

PRP if ischemic Intravitreal triamcinolone injection as tx for

non-ischemic occlusion, likely not effective for ischemic occlusion

Vision Limiting Complications of BVO Macular edema- 1/3 spontaneously regain

vision Macular nonperfusion, no vision improvement Vitreous heme- NVE, NVD

Clinical Features of BVO

NV if capillary nonperfusion > 5 disc areas NVE/D most likely 6-12 months up to 3 years Close f/u until heme resolves

PC Criterion BVO Study

FA proven macular edema No residual heme in center of fovea 3-18 month duration No DM retinopathy VA less than 20/40 Grid tx to leaking area- no closer than FAZ

Management of BVO if VA<20/40

Watch for 3-6 months FA, ME vs macular nonperfusion Grid PC if <20/40 / IVK More than 5 disc areas of retina involved FA

watch for NV

I can’t see out of my right eye

Sudden vision loss rt eye BCVA= 20/ LP; 20/20 Rt afferent pupillary defect Med hx aterioro sclerosis, htn, type 2

diabetes Slex and iop normal Fundus examination OS normal Rt eye

Right fundus

diagnois CRAO Other questions Duration < 24 hr management

Duration < 24 hr

AC paracentisis IV diamox Ocular massage Inspiration of high concentration of oxygen

and carbon dioxide

Additional Hx

Physical sx Important questions Stat Lab tests? ESR, C-RP Essential condition to rule in / rule out GCA What tx? Steroids / bx later Why critical ? Bilateral blindness

Giant Cell Arteritis

Fever, malaise, weight loss, scalp tenderness Need STAT ESR&C-RP in evaluating CRAO Temporal artery biopsy PO steroids Risk of bilateral blindness within hours if no

treatment

Disposition after GCA ruled out

Work up for other associated systemic conditions, emergent

Need medical work up for associated systemic conditions

Internest, cardiology, neurology

Systemic Conditions Associated with CRAO Carotid stenosis Cardiac valvular disease, MVP, Rheumatic Coagulopathies, sickle cell disease, platelet and

factor abnormality Optic nerve drusen Elevated intraocular pressure Collagen vascular disease, SLE, Giant cell arteritis

Clinical Features of CRAO

APD Cherry red spot in macula due to nerve fiber

layer thinning at fovea Acutely arteries are attenuated Emboli visible 20% of cases NVG 15-20% of cases Need systemic work up

CRAO Treatment

AC paracentisis if <24 hours old IV diamox Ocular massage Inspiration of high concentration of oxygen

and carbon dioxide

There is a curtain in my vision

Phone call/ walk in visit Initial differential Happened less than 3 hours ago Sudden loss of vision superior field left eye 64 yo male hx hypertension

Examination findings

Best corrected vision 20/20;20/40 Perrla – no afferent defect Confrontation field superior defect OS SLEx and IOP unrmarkable Fundus examination

Diagnosis

? Branch retinal artery occlusion Emergent/invasive measures for vision

preservation ?Y/N Next Urgent / emergent medical evaluation? Y/N Work up for source of embolus

BRAO Features

90% on temporal vessels Permanent field defect 80% cases final VA >20/40 Similar etiologies as CRAO Ocular therapeutic measures generally not

undertaken

There is a dark spot in my vision

Inferior field left eye Present for 2 weeks Normal vision OU Normal fundus examination OD

Management

Diagnosis Retinal arterial macroaneurysm Additional tests FA Prognosis

Management of Macroaneurysms

FA helpful in dx, ddx Spontaneous resolution in 12 months Observation if no macular involvement Photo-coagulation in some cases with

macular involvment

Retinal Arterial Macroaneurysms

Distinct entity from Coats, Lebers, Eales Temporal retina within 1st 3 bifurcations M:F ratio 1:2 usually < 60 years old 75% have HTN, atherosclerosis

Sickle cell disease

Patients of African descent Hemoglobinopathy, abnl amino acid Sickle shaped RBC trapped in small vessels-

hypoxia, necrosis Different genotypes, least severe systemically

has most ocular complications and vice versa

Ocular Findings in Sickle Cell

Comma shaped conj capillaried Iris atrophy, synechia Salmon patch retinal hemorrhages Black sunbursts in retina Peripheral retinal sea fans,

neovascularization Scatter PC to reduce ischemia

Stages of Sickle Cell Retinopathy

1. Peripheral arterial occlusions

2. Peripheral arterio-venous anastomosis

3. Neovascularization

4. Vitreous hemorrhage

5. Retinal detachment