Common Posterior Pole Disorders

1. Common Posterior Pole Disorders Kelli Shaon, O.D.

2. Macular Degeneration

3. Age Related Macular Degeneration • Leading cause of legal blindness in those >65 years of age • More common in Caucasians, much less common in African-American • More common in Cigarette smokers, those w/ significant light (sun) exposure, and family history (sibling or parental), & associated systemic disorders (HTN, Hyperlipidemia, and DM) • Presents in 2 forms: • Dry (non-exudative) – 90% • Wet (exudative) – 10%

4. Dry (Non-exudative) ARMD • Signs: • Hard drusen* – • Round, discrete, punctate yellowish deposits • Not diagnostic of ARMD but need to monitor • Soft drusen - • Larger, indistinict, paler yellow deposits (can become confluent) – more likely to ↓ VA • RPE clumping (mottling, changes) • Breakdown of RPE • Geographic atrophy • Gradual, progressive loss of RPE and choriocapillaris (visible choroidal vessels on examination)

5. Dry (Non-exudative) ARMD • Hard drusen • Fine, discrete yellow • Soft drusen • Confluent, larger, pale

6. Dry (Non-exudative) ARMD • RPE mottling: • RPE distruption • Geographic atrophy: • RPE & choriocapillaris destruction (visible choroidal vessels)

7. Dry (Non-exudative) ARMD • Clinical Pearls: • 90% of all ARMD*** • Bilateral, but can be asymmetrical • Vision can vary from 20/20 (with Drusen &/or RPE changes only to <20/200 from geographic atrophy)

8. Dry (Non-exudative) ARMD • Management: • Should be monitored for progression to exudative (wet) form • Follow-up time will vary based on severity • An ↑ in the # of Soft, confluent drusen ↑ the risk of progression to wet – f/u should be sooner • Home Amsler grid are most common way for pt. To monitor for changes (metamorphopsia) • Stop smoking • Vitamin supplement (AREDS formula) • Vitamins A (beta-carotene), C, E, zinc, & copper • NO Beta-carotene (Vit. A) for smokers (or those who have smoked in past 10-15 years) – Ocuvite w/ Lutein is altern.

9. Wet (Exudative) ARMD • Signs: • Grayish-green discoloration in macular (CNVM) • New vessel growth that develops & grows through a disruption in Bruch’s membrane • Sub-retinal hemorrhage • Deeper, darker red colored hemorrhage often associated w/ exudates • RPE detachment or sensory retinal detachment • Detachment of RPE or sensory retinal in macula • Disciform scar • Fibrovascular scarring secondary to old leakage from a ruptured/leakage in a CNVM

10. Macular Photocoagulation Study (MPS): • Eyes with soft drusen – 30% risk of CNVM • Eyes w/o large, soft drusen & w/o pigment abnormalities – had 10% risk of CNVM • Patients with CNVM in 1 eye have a 58-73% chance of developing CNVM in fellow eye in 5 years if… • 2 or more large drusen & focal hyperpigmentation of the macula

11. Choroidal Neovascular Membrane • IVFA: • Top - Classic CNVM • Bottom - Occult CNVM

12. Wet (Exudative) ARMD • Top – Pigment epithelium detachment (PED) • Bottom – Disciform scar

13. Wet (Exudative) ARMD • Management/Treatment: • Laser photocoagulation • MPS study demonstrated that severe vision loss can be reduced in patients w/ extrafoveal wet ARMD • MPS showed 10-15% of CNVM are treatable w/ Argon photocoagulation • Photodynamic therapy (PDT) – Vertiporfen (Visudyne) • Light-activated drug (approved for ARMD, Histoplasmosis, & Pathological myopia) • Sub-macular surgery • Removal of CNVM • Macular Translocation • Movement of macula to functioning part of retina • New treatments – • Macugen - VEGF-I injection  q 6 weeks – FDA approved • Lucentis - VEGF-I injection  q 4weeks • Retanne - deriv. of corticosteroid, juxtascleral q 6 monhts

14. Additional Causes of CNVM

15. Additional Causes of CNVM • Choroidal rupture • Unilateral • Etiology: Blunt Force Trauma • Sign: Circumferential breaks in Bruch’s

16. Additional Causes of CNVM • Myopic Degeneration • Bilateral Involvement • Etiology: Pathological (High) Myopia – • Defined as at least 6 diopters of axial myopia that has created thinning of the RPE and choroid • Signs: Lacquer cracks, Fuch’s spots, staphylomas, &/or choroidal atrophy

17. Additional Causes of CNVM • Angioid Streaks • Bilateral involvment • Breaks in bruch’s membrane that are thickened & calcified – peau de’ orange appearance • Most associated systemic dz: • Pseudoxanthoma elasticum (PXE)** • Paget’s disease • Sickle cell disease

18. Additional Causes of CNVM • Histoplasmosis (POHS) • Bilateral • Etiology: Fungus commonly found in soil (Histoplasma capsulatum) • Often from bird & bat feces • Concentrated along Ohio-Mississippi river valley • Fundus findings: Classic Triad • Punched-out lesions (histo spots) • Peripapillary atrophy (PPA) • CNVM

19. Common Macular Disorders

20. Central Serous Chorioretinopathy • Unilateral, serous detachment of macula • Most commonly affected: • Type-A personalities • Male>>Female (10:1) • Usually between 20-60 yo • Common signs: • Metamorphopsia • “Smokestack” on FA - classic • Management: • Monitor • Lifestyle changes • NSAIDs (??) • Laser photocoagulation (if fail to improve)

21. Cystoid Macular Edema (CME) • Fluid accumulation in the OPL & INL of retina (centered in fovea) • Associations: • Post CE (Irvin-Gass Syndrome) • Medications: • Niacin, Xalatan (Prostoglandins), Epinephrine • Signs: • Reduced vision • Metamorphopsia • Treatments: • Topical NSAID (Acular QID) • Non-generic Pred Forte 1% QID • Possible Grid Photocoagulation

22. Epiretinal Membrane (ERM) • AKA: cellophane maculopathy; Macular pucker • Associated w/ a break in ILM & cellular proliferation on the surface (creates surface wrinkling) • Early stages = glistening area of retina • Later stages ↑ contraction & density of ERM ( ↑ wrinkling of retina & tortuosity of vessels • Treatment: • Early stages: Monitor w/ Amsler • Late stages: Membrane peel

23. Macular Hole • Etiology:??, thought to be tugging at macular due to PVD • Stages: • Stage 1: Foveal detachment (20/25-20/70) • A: Yellow spot in fovea • B: Yellow ring in Fovea • Stage 2: Early Hole formation (20/25-20/100) • Stage 3: Fully developed hole w/ vitreoretinal separation but NO PVD • 97% will have (+) Watzke- Allen • Stage 4: Full-thickness macular hole w/ PVD (only 10-20%

24. Macular Hole • Treatment: • Stage 1: Get Retina Consult –However, 50% of stage will spontaneously regress • Stage 2 & 3: Also get Retina consult, vitrectomy may help • Stage 4 – Monitor ? • If macular hole in 1 eye & PVD in other eye, little risk of fellow getting macular hole • If macular hole in 1 eye & No PVD in other eye 28-44% chance of fellow eye developing macular hole • Before you get a consult - Can your pt handle surgery? • face down position for 7 days

25. Macula Pseudo-hole • Looks similar to macular hole, • Differences: • Vision should be good (most 20/20) • (-) Watzke-Allen • It is actually a ERM that develops a hole in the membrane over the macula

26. Retina/Vitreous Inflammation

27. Toxoplasmosis • Infection by the protozoan Toxoplasma gondii • found in cat feces (intestinal phase), cells of the host & in tissue of raw, under cooked meat (extra-intestinal phase) • Most common posterior uveitis in the US • Signs: • Yellow-white retinal lesions (satellite lesions), often associated with old chorioretinal scars • Focal vitreous inflammation is also possible • Treatment/Management: • Order serum anti-Toxoplasma antibody titer – should be (+) • Disease is usually self-limited in immuno-competent patient (Treat the AC uveitis if present, then & monitor) • Systemic Tx is used if immuno-compromised patient or if macula is threatened – Drugs are toxic, work w/ PCP to Tx

28. Toxoplasmosis • Findings: • (+) Group of 3 CR scars • (+) Non-pigmented raised lesion, possible Satellite lesion • (+) Vitreous Strands

29. Toxocariasis • Infection by nematode larvae • Most commonly of Toxocara canis or Toxocara cati • Signs: • Fibrous band often radiating from white retinal mass • Leukocoria can be present • Treatment: • Retina consult for photocoagulation or transvitreal removal of worm • Possible oral antihelmonthic drugs (controversial)

30. Sarcoidosis • Background: • Systemic granulomas disease • Most common involvement includes the eyes, hilar adenopathy w/ associated breathing difficulties, enlargement of parotid gland, facial nerve palsy, tender skin nodules, & arthritis • Much more common in African-Americans than Caucasians • Female > Male • An average of 40% of indiv. w/ sarcoidosis will have eye signs

31. Sarcoidosis • Ocular signs: • Granulomatous Anterior Uveitis** • LARGE Mutton-fat KPs • Koeppe & Busacca nodules or the iris • Possible residual or current PAS (peripheral anterior synchiae) • Sheathing of peripheral retinal veins – “candle-wax drippings”** • String of pearls vitreous opacities • Lacrimal gland infiltation w/ KCS following (severe dry eyes)* • Resultant Cataracts • Resultant Glaucoma (inflammation related)

32. Sarcoidosis • Diagnosis: • Chest X-ray – (+) bilateral hilar adenopathy • Serum ACE – elevated 60-90% of patients • May want to order PPD – to r/o out TB • Management: • Anterior uveitis: Cycloplegic + Pred Forte 1% • Posterior uveitis: systemic &/or periocular steroids (Obtain retina or ophthalmology consult) – Topical steroids will NOT be effective enough for posterior involvement

33. Sarcoidosis

34. AIDS associated Retinopathy • Common findings: • CWS*** • DDx: Interferon Retinopathy – from Hepatitis C treatment • Flame hemes • Roth spots • DDx: leukemia, Bacterial endocarditis, DM

35. AIDS associated Retinopathy • CMV Retinitis: “Cheese & Sauce” • Infiltrative necrosis • White intraretinal lesions • Intraretinal hemmorhages • Exudative retinal detachments

36. The End