Optic Neuropathies and Glaucoma

1. Kyle Cheatham, O.D., F.A.A.O. KMK EDUCATIONAL Services, LLC. Optic Neuropathies and Glaucoma FCO Conference: November 4th, 2011

2. Disclosures The content of this COPE Accredited CE activity was prepared independently without input from members of the ophthalmic community. I have no direct financial or proprietary interest in any companies, products or services mentioned in this presentation. The content and format of this course is presented without commercial bias and does not claim superiority of any commercial product or service.

3. Course Objectives Provide a big-picture overview of optic nerve pathology (excavation, edematous, pallid) with an emphasis on glaucoma. Overview glaucoma assessment, differential diagnosis, treatment and management.

4. Guide to Optic Nerve Disease Only three ways that an unhealthy optic nerve can present: Excavated (Typically Glaucoma) Edematous (Disc Edema/Papilledema) Pallid (Primary/Secondary Optic Atrophy) Heartland Eye Consultants

6. Heartland Eye Consultants Optic Nerve Edema

7. Optic Nerve Edema Disc Edema – if not specified otherwise, refers to unilateral disc swelling. Papilledema – refers to bilateral disc swelling. Pathophysiology impacting disc edema is much different than what leads to papilledema. Therefore, the differentials for each significantly differ!

8. Common Causes of Disc Edema Ischemic (AION, NAION) Vascular (DM, CRVO) Compressive (Meningioma, TED, Lymphoma) Inflammatory (Optic neuritis, Meningitis) Other (Hypotony) Heartland Eye Consultants

9. Symptoms of GCA Headache Neck pain Anorexia/weight loss Fatigue Fever Tenderness/sensitivity on the scalp Jaw claudication Vision loss Jaw claudication – arteritis of the maxillary artery causing ischemia of the muscles of mastication. Aching or tiredness of these muscles is brought on by chewing and is relieved by rest Hayreh’s chart of symptoms TAB [No. (%)] Signs and symptoms Positive (106) Negative (257) P Value Headache 59 (55.7) 117 (45.5) 0.84 Anorexia/weight loss 55 (51.9) 84 (32.7) 0.0005 *Jaw Claudication 51 (48.1) 22 (8.6) <0.0001 Malaise 40 (37.7) 78 (30.4) 0.177 Myalgia 31 (29.2) 68 (26.5) 0.606 Fever 28 (26.4) 42 (16.3) 0.040 Abnormal temp artery 21 (19.8) 33 (12.8) 0.105 Scalp tenderness 19 (17.9) 27 (10.5) 0.058 *Neck pain 17 (16.0) 11 (4.3) 0.0003 Anemia 14 (13.2) 31 (12.1) 0.730 Total symptoms 335 513 Average S/S per pt 3.16 1.99 Jaw claudication – arteritis of the maxillary artery causing ischemia of the muscles of mastication. Aching or tiredness of these muscles is brought on by chewing and is relieved by rest Hayreh’s chart of symptoms TAB [No. (%)] Signs and symptoms Positive (106) Negative (257) P Value Headache 59 (55.7) 117 (45.5) 0.84 Anorexia/weight loss 55 (51.9) 84 (32.7) 0.0005 *Jaw Claudication 51 (48.1) 22 (8.6) <0.0001 Malaise 40 (37.7) 78 (30.4) 0.177 Myalgia 31 (29.2) 68 (26.5) 0.606 Fever 28 (26.4) 42 (16.3) 0.040 Abnormal temp artery 21 (19.8) 33 (12.8) 0.105 Scalp tenderness 19 (17.9) 27 (10.5) 0.058 *Neck pain 17 (16.0) 11 (4.3) 0.0003 Anemia 14 (13.2) 31 (12.1) 0.730 Total symptoms 335 513 Average S/S per pt 3.16 1.99

10. Diagnosis of GCA American College of Rheumatology Criteria 3 or more of the following Age of 50 or more at disease onset New onset of localized HA Temporal artery tenderness or decreased pulse Elevated ESR of 50 mm/hr or more Temporal artery biopsy showing necrotizing arteritis Several important things, one being Vision!Several important things, one being Vision!

11. Diagnosis Physical exam Laboratory tests Erythrocyte Sedimentation Rate (ESR) C-reactive Protein (CRP) Complete Blood Count (CBC) Temporal artery biopsy Gold standard ESR normal in 20% of patients Biopsy - Since the blood vessels are involved in a patchy pattern, there may be unaffected areas on the vessel and the biopsy might have been taken from these parts. So, a negative result does not definitely rule out the diagnosis. ESR normal in 20% of patients Biopsy - Since the blood vessels are involved in a patchy pattern, there may be unaffected areas on the vessel and the biopsy might have been taken from these parts. So, a negative result does not definitely rule out the diagnosis.

12. GCA or NAION? If we take 1000 swollen ONH’s caused by an ION in the general population, epidemiological studies show that 90% of them will be NAION, 10% GCA. Of the NAION patients, studies also show 90% of them will have a C/D of = 0.3. GCA happens to any C/D size so we can assume half will be = 0.3 and half = 0.4. But the ONH is swollen? Studies also show < 1% of patients have a C/D ratio that is = 0.2 difference between the two eyes….use contralateral eye as an estimate.If we take 1000 swollen ONH’s caused by an ION in the general population, epidemiological studies show that 90% of them will be NAION, 10% GCA. Of the NAION patients, studies also show 90% of them will have a C/D of = 0.3. GCA happens to any C/D size so we can assume half will be = 0.3 and half = 0.4. But the ONH is swollen? Studies also show < 1% of patients have a C/D ratio that is = 0.2 difference between the two eyes….use contralateral eye as an estimate.

13. ESR and CRP Positive predictive value = 0.775 ¾ of the time tests will correctly indentify GCA ¼ of the time will be falsely positive *negative predictive value = 0.993 ESR, CRP, C/D = 0.3 Positive predictive value = 0.658 2/3 of the time will correctly indentify GCA 1/3 of the time will be falsely positive ESR, CRP, C/D = 0.4 Positive predictive value = 0.946 Diagnosis of GCA If we use sensitivity and specificity of ESR and CRP in GCA patients from Hayreh’s study, and plug them into our population of 1000 patients with swollen ONH’s from ION, we can determine PPV for blood work alone. Just by adding C/D ratio information into this population, we can modify the PPV. If we use sensitivity and specificity of ESR and CRP in GCA patients from Hayreh’s study, and plug them into our population of 1000 patients with swollen ONH’s from ION, we can determine PPV for blood work alone. Just by adding C/D ratio information into this population, we can modify the PPV.

14. Atrophic Pathology Includes Excavated and Pallid sources: Excavated (loss of neuroretinal rim), classically caused by glaucoma  Pallor: Primary and Secondary Optic Atrophy. Heartland Eye Consultants

15. Primary Optic Atrophy (POA) Key Distinction: In POA, the optic nerve goes from healthy to pale WITHOUT any intermediary stage of edema. Trauma Toxic/Nutritional Retrograde/Orthograde degeneration Hereditary (Leber’s Optic Neuropathy) Heartland Eye Consultants

16. Secondary Optic Atrophy (SOA) Key Distinction: In SOA, the optic nerve goes from healthy to EDEMATOUS and then becomes PALE. Causes include any of the edematous sources discussed previously in the lecture. Heartland Eye Consultants

17. Secondary Optic Atrophy from Sphenoid Wing Meningioma

18. Summary Points The ONH can appear healthy, excavated, edematous or pale. Excavated nerves result from glaucoma. Edematous nerves will become pale, if longstanding. The causes of disc edema and papilledema vary significantly (pre-chiasmal, post-chiasmal). Pale nerves are most commonly the result of longstanding edema (secondary optic atrophy) but can go straight from healthy to pale as a result of primary optic atrophy sources. Heartland Eye Consultants

19. Summary Points Excavated (glaucoma) Edematous Papilledema (pseudotumor, HTN, meningitis, brain tumor) Disc edema (ischemic, inflammatory, vascular, compressive) Pallid Primary Optic Atrophy (toxic/nutritional, ortho/retro) Secondary Optic Atrophy (see list of edematous causes) Heartland Eye Consultants

20. Glaucoma Definitions Glaucoma is a complex disease with variable ocular signs and one underlying theme: PROGRESSIVE optic neuropathy categorized by EROSION of neuroretinal rim tissue. The optic nerve undergoes progressive, irreversible damage and, in most cases, is a chronic disease. Glaucoma is ocular DEMENTIA, INFLAMMATION, and ISCHEMIA leading to ganglion cell death with subsequent RNFL death and coincident functional loss, even compromise to the cortex.

21. Glaucoma Definitions Glaucoma is a complex disease with variable ocular signs and one underlying theme: PROGRESSIVE optic neuropathy categorized by EROSION of neuroretinal rim tissue. The optic nerve undergoes progressive, irreversible damage and, in most cases, is a chronic disease. Glaucoma is ocular DEMENTIA, INFLAMMATION, and ISCHEMIA leading to ganglion cell death with subsequent RNFL death and coincident functional loss, even compromise to the cortex.

22. Types of Glaucoma OPEN ANGLE GLAUCOMA Primary open angle glaucoma Secondary open angle glaucoma (PXF, PDG) Normal Tension Glaucoma INFLAMMATORY GLAUCOMA (Glaucomatocyclitic crisis, Fuch’s) ANGLE CLOSURE GLAUCOMA Primary angle closure glaucoma Secondary angle closure glaucoma (NVG, Uveitic glaucoma) ANGLE RECESSION GLAUCOMA

23. Heartland Eye Consultants

24. Which of the following glaucoma medications decreases IOP by increasing outflow through the uveoscleral meshwork? Pilocarpine Prostaglandins Carbonic anhydrase inhibitors Beta Blockers

25. Which of the following tests is LEAST likely to be abnormal in advanced primary open angle glaucoma? Red cap test Brightness comparison test APD test Photostress test

26. Which of the following is FALSE regarding normal tension glaucoma? Raynaud’s syndrome and migraines may be more prevalent Drance hemorrhages are only seen in this type of glaucoma Visual field loss tends to be more central Females are more susceptible

27. Which of the following statements is FALSE regarding the ocular hypertension treatment trial? The presence of drance hemorrhages increase the risk of POAG. Thinner corneas increase the risk of POAG development. The conclusion of the trial was that all patients with pressures over 21 should be treated The risk of developing POAG in the untreated group after 5 years was 9.6%

28. Which of the following statements is FALSE regarding angle closure glaucoma? It can be caused by plateau iris syndrome. It can be caused by pupillary block. It can be caused by 360 degrees of posterior synechiae. Acute angle closure is more common than chronic angle closure..

29. What is the term for adhesion between the iris and trabecular meshwork? Posterior synechiae Peripheral anterior synechiae Iris bombe

30. Which of the following statements is FALSE? A small disc with a large cup is more concerning than a large disc with a large cup. Glaucoma patients can have an APD even though glaucoma tends to be a bilateral disease because it is often asymmetric. Bean pot appearance (bayoneting) is a risk factor for some forms of optic neuropathy, but not glaucoma. Patients with large cupping or optic nerve asymmetry have an increased risk of glaucoma.

31. Which of the following structures is most anterior in the angle?  Iris Trabecular meshwork Ciliary body Schwalbe’s line

32. Which of the following statements is FALSE regarding visual field testing?  Static Automatic Perimetry (SAP) is a threshold test Short Wavelength Automated Perimetry (SWAP) can detect damage before SAP would detect. Frequency Doubling Technology (FDT) is a quick (90-seconds) screening test. On Humphrey SAP testing, total deviation plot is more helpful than pattern deviation for evaluating glaucoma.

33. Which of the following medications increases uveoscleral outflow and decreases aqueous production?  Pilocarpine Prostaglandins Carbonic anhydrase inhibitors Alpha-2 agonists

34. E-MAIL: KMKBOARDCERTIFICATION@ GMAIL.COM Questions?