Retinal Vascular Disease

1. Retinal Vascular Disease Kelli Shaon, O.D.

2. Central Vein Occlusion (CRVO) • Unilateral – “Blood & Thunder” • Signs: • Hemorrhages in all 4 quadrants – to the periphery • Dilated tortuous veins • Optic disc edema • Variable macular edema (CME common) • Etiology: • Thrombosis of CRV at the lamina cribosa • Strong association with systemic disease (HTN, DM, blood dyscrasias (including polycythemia) and glaucoma • Rarely associated with localized optic nerve compression (orbital mass)

3. Central Vein Occlusion (CRVO)

4. Non-ischemic CRVO • Non-ischemic CRVO: • Most common type, presenting in ~75% of the cases – may progress to ischemic state • Signs of non-ischemic CRVO: • moderately decreased vision (VA better than 20/200) • (-) APD • Hemorrhages in all 4 quadrants, retinal edema, possible macular and disc edema • Dilated tortuous veins in all 4 quadrants • Less than 10 DD area of capillary non-perfusion (best determined by FA)

5. Non-ischemic CRVO • The top picture is a fundus photograph • The bottom picture is an angiogram of an eye with non-ischemic CRVO. • All the capillary bed fills very well, and there are a few scattered retinal hemorrhages and engorged retinal veins.

6. Ischemic CRVO • Ischemic CRVO: • Signs: • Dramatically decreased vision (usually VA 20/200 or worse) • (+) APD • Hemorrhages in all 4 quadrants, retinal edema, extensive macular and disc edema, (+) presence of many CWS • Dilated tortuous veins in all 4 quadrants • Silver-wired or sheathed arteries • 10 DD area or more of capillary non-perfusion (best determined by FA)

7. Ischemic CRVO • The top picture is a fundus photograph • The bottom picture is an angiogram of an eye with ischemic CRVO. • There is almost total non-perfusion of the retinal capillary bed, indicating that this is an ischemic CRVO • ***NOTE: If there are extensive retinal hemorrhages, it is almost impossible to evaluate capillary non-perfusion accurately, because of the masking effect by the blood

8. Management of CRVO • Follow-up for a non-ischemic CRVO: • Every 4 weeks for minimum of 6 months to watch for conversion to ischemic and to monitor for neovascularization • Follow-up for ischemic CRVO: • Every 3 to 4 weeks for minimum of 6 months to watch for neovascularization (more specifically 90 day neovascular glaucoma)

9. Treatment Methods • Panretinal photocoagulation (PRP): • Used to reduce the amount of retinal hypoxia due to the large area of non-perfusion • According to the Central Vein Occlusion Study (CVOS), prophylactic PRP treatment does not prevent development of iris neovascularization, therefore, it is safe to wait until neovascularization develops before applying PRP • Nd: YAG laser: (Prophylactic treatment) • Used to rupture Bruch’s membrane and a retinal vein, which creates an anastamosis between the choroid & the retinal vessels • ↓ amount of time it takes to reduce retinal ischemia by waiting for the growth of new collaterals

10. 90 day Neovascular Glaucoma • The diagnosis of a CRVO indicates an increased chance of fast developing neovascularization of the iris and/or the angle • NVI and NVA is more common in an ischemic CRVO • Incidence of 90 day glaucoma: • non-ischemic CRVO - 1% • ischemic CRVO - 47%

11. Branch Retinal Vein Occlusion (BRVO) • Thought to be the 2nd most common retinal vascular disease (2nd to Diabetic retinopathy) • Occurs at arteriovenous crossing (hardened artery compresses vein) • Most common quadrant affected – Superior-temporal (Also most Vision affecting because above the macula) • If occurs at the nerve then 2 quadrant are affected (hemi-retinal vein occlusion)

12. Branch Retinal Vein Occlusion (BRVO)

13. Branch Retinal Vein Occlusion (BRVO) • Also has strong association with systemic disease (HTN, DM, blood dyscrasias) • Open angle Glaucoma is often associated • Often occurs in 50-60 yo with no gender or racial predilection

14. Treatment/Management of BRVO • Monitor every 1-2 months, then every 3-12 months (depending on severity) • Check blood pressure & r/o other systemic causes • Treatment IOP, if glaucoma is suspected • Branch Vein Occlusion Study (BVOS) – • If chronic macular edema (for 3-6 months) with reduced VA <20/40, without macular non-perfusion, Grid laser photocoagulation is indicated • If neovascularization, Sector PRP is often used • Laser treatment also shown to reduced likelihood of VH

15. Central Retinal Artery Occlusion (CRAO) • Unilateral – • Signs: • Opacification or whitening of the retina • Cherry red spot (in center of macula) • (+) APD • Narrowed retinal arteries • Occasionally will see emboli • Amarosis fugax (possible history) • Etiology: • Embolus or Thrombosis (carotid or cardiac plaque) • Giant cell arteritis (GCA) • Collagen-vascular disease • Hypercoaguable disorders (blood dyscrasias) • Elevated IOP

16. Central Retinal Artery Occlusion (CRAO)

17. CRAO management (if <24 hours) • Ocular massage (digital massage vs. fundus contact lens) • Anterior chamber paracentesis (hole through cornea to ↓ aqueous) -↓ IOP • IV or PO Diamox to lower IOP • Hyperventilation (inspiration of high amounts of O2 and CO2) – “Brown bag breathing”

18. CRAO management • Work-up: • STAT ESR & C-Reactive Protein to r/o GCA • CBC w/ differential • Consider other collagen-vascular workup • Carotid duplex • To r/o Hollenhorst plaque – highly refractile plaque, most often seen at arteriole bifurcation • Cardiac evaluation (Cardiac echo - ECG) • To r/o cardiac platelet emboli – dull gray-whitish palque • Follow-up: • Follow-up every 3-6 months • Watch for neovascularization

19. Branch Retinal Artery Occlusion (BRAO) • Unilateral – (most often temporal arteries) • Signs: • Opacification or whitening of a portion of the retina • Narrowed retinal arteries • Occasionally will see emboli • VF defect in associated w/ affected retina • Etiology: similar to CRAO • Management: Similar to CRAO

20. BRAO w/ Hollenhorst plaque

21. Hypertensive Retinopathy • Grade 1: mild arteriolar attenuation with broadening of ALR and vein concealment • Grade 2: More severe arteriolar constriction, also associated with deflection of veins at AV crossings (ie. Crossing changes) • Grade 3: Characterized by copper-wiring of arterioles & right angle deflection of veins. Flame hems, CWS, and hard exudates are also often found. • Grade 4: Consist of all Grade 3 changes + silver wiring of arterioles & disc swelling (Malignant Hypertension) Kanski J. Clinical Ophthalmology, 4th ed.

22. Hypertensive Retinopathy • Increased ALR • Arteriole attenuation • A-V crossing changes

23. Sickle Cell Retinopathy • Often bilateral (may be asymmetric) • Most dominantly affects African-Americans • Signs: • Peripheral retinal neovascularization – “Sea fans” • Sclerosis of peripheral retinal vessels • Abnormal, dull gray peripheral fundus • Tortuoisty of veins • Black midperipheral fundus lesions (black sunbursts) • Intraretinal & subretianl hemes (Salmon patches) • Refractile (iridescent) intraretinal deposits • Angioid streaks • Vitreous hemes • *** Dark w/o pressure – can be associated with SC

24. Ocular Ischemic Syndrome (OIS) • Associated with Carotid Occlusive Disease • Signs: • Dilated retinal veins • Arterial attenuation • Mid-peripheral retinal hemorrhages *** • Iris neovascularization &/or Retinal Neo • Mild Cells & flare (mild anterior uveitis) • Management: • Carotid Duplex (doppler) & notify PCP • Follow-up based on severity of ocular signs

25. Ocular Ischemic Syndrome (OIS)

26. Diabetic Retinopathy • Two main types: • Non-proliferative • Proliferative • See Diabetes and the Eye Handout for Details