Genetics DISCLOSURES. Exfoliation Syndrome 2/28/2017 NEW TOPICS IN GLAUCOMA DIAGNOSIS AND TREATMENT. New Diagnostic Approaches To Glaucoma

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1 NEW TOPICS IN GLAUCOMA DIAGNOSIS AND TREATMENT G. RICHARD BENNETT, M.S., O.D., F.A.A.O. PROFESSOR AND CHIEF THE GLAUCOMA SERVICE, THE EYE INSTITUTE, SALUS UNIVERSITY 1 Exfoliation Syndrome (aka Glaucoma Capsulare, Pseudoexfoliation Syndrome, PXF, PEX, PXS, XFS) Prevalence: Female > Male Scandinavian Countries (historically) Incidence doubles with each decade of life over age 50 Genetics: (discovered in 2007) Principle genetic risk factor = single nucleotide polymorphisms (SNPs) of the lysyl oxidase-like 1 (LOXL1) gene on chromosome 15q24.1 Within Nordic population, 2 of these SNPs attribute to a 99% higher risk for PXS and PXG DISCLOSURES CONSULTANT/ADVISORY BOARD: ALLERGAN, ALCON LECTURE HONORARIA: ALCON THE CONTENT AND FORMAT OF THIS COURSE IS PRESENTED WITHOUT COMMERCIAL BIAS AND DOES NOT CLAIM SUPERIORITY OF ANY COMMERCIAL PRODUCT OR SERVICE New Diagnostic Approaches To Glaucoma New Thoughts, Tools, and Techniques Genetics LOXL1 Enzymes involved in the cross-linking of collagen and elastin in the extracellular matrix Connective tissue maintenance Actual role in the syndrome still unclear LOXL1 risk genotypes seen in 92% of XFS, but also seen in 74% of normal population Contactin-associated protein-like 2 found in German cohort Potassium channel trafficking Others: lysosomal trafficking regulator, clusterin, adenosine receptors, matrix metalloproteinase (MMP1), glutathione transferase 1

2 A Systemic Disorder Material: Grey-white, fibrillogranular, extracellular, matrix material Protein core surrounded by glycosaminoglycans FXS material deposits around blood vessels of connective tissue Lung, liver, kidney, gall bladder, cerebral meninges Potentially related to cardio, cerebrovascular disease, hearing loss, and Alzheimer s disease* Strong literature evidence that this is an ischemic systemic disorder Ocular Signs of Exfoliative Syndrome Anterior Lens: Central translucent disc c rolled edges Clear mid-zone due to constant rubbing of the pupil Peripheral granular band c multiple radial striations Only detected post-dilation Lens Zonules Accumulation of PEX material Zonular dehiscence Phacodonesis or lens subluxation XFS in the Eye Deposition: anterior lens capsule, zonules, ciliary body, iris, trabeculum, anterior vitreous face, conjunctiva Pseudoexfoliation Syndrome and Cataracts Pre-op (Blue Mountain Eye Study 2013): Contributing to progression of cataracts Due to free radicals and oxidative damage Zonulodialysis -> Phacodonesis Post-op: Poorly dilating pupil Increased incidence of post-op capsular opacification and contraction Increased incidence of spontaneous dislocation of IOL Prolonged intraocular inflammation and corneal decompensation Ocular Signs of Exfoliative Syndrome Cornea: FXS material on the endothelium Pigment deposition Potential for Krukenberg spindle Anterior Chamber: Mild flare breakdown of blood aqueous barrier Iris: FXS material on pupillary margin Sphincter atrophy moth eaten TIDs Patchy iris neovascularization c extensive capillary dropout Exfoliative Syndrome and the Retina Correlation with ocular ischemia XFM found in vortex veins and central retinal veins [Ritch et al. 2010] found correlation with incidences of CRVO with XFS CRVO (but not BRVO) 2

3 Conversion into Glaucoma: XF Glaucoma XFS is the most common cause of secondary open angle glaucoma Males > Females Although Females > Males in XFS Diagnosis usually in 7 th decade of life Usually unilateral in presentation Risk factors: Cumulative risk for conversion 5% at 5 years 15% at 10 years Unilateral XF GLC and XFS in fellow eye : 50% in 5 years Unilateral XF GLC and NO PEX in fellow eye : low risk No apparent association between angle characteristics and severity In Scandanavia, this accounts for 50% of open angle glaucoma New Research: The Latitude Effect [Stein, Pasquale, et al. 2011] Purpose: Determine the risk of exfoliation syndrome based on geographic and climatic risk factors Retrospective study of 626,901 patients spanning 47 states seen between the years of Results: Northern tier residence (above 42 N) was associated with increased hazard Southern tier residence (below 37 N) was associated with decreased hazard *** Excluding whites did not change these associations*** Pseudophakic Exfoliative Glaucoma XF material can still deposit on an IOL Characteristic pattern as seen on a phakic lens Deposition in areas exposed to the highest aqueous humor flow XF material can develop on the IOL surface 4-10 yrs post CE in patients with XFS The Latitude Effect Pseudophakic Exfoliative Glaucoma The Latitude Effect Climate Findings: Change in Temps Hazard of PXS 1 increase in July high Decreased by 9% temp 1 increase in Jan low Decreased by 3% temp Addl sunny day annually Increased by 1.5% Conclusion: Ambient temperatures and sun exposure may be environmental triggers for XFS 3

4 The Lattitude Effect Possible Explanation Nucleation reaction is prone to occur at lower temperatures. Anterior chamber and lens may be susceptible to ambient temperatures. Reaction also related to UV exposure Pasquale et al. current project: Solar Exposure Questionnaire Lifeguards: 3x more risk Ski Instructors: 8x more risk Differential Diagnosis: Pigmentary Dispersion Syndrome Confused because: Both have material found in the angle and anterior chamber Potential for Krukenberg spindle Difference: Age of onset: PDS usually in 30-40s Transillumination defects Pupillary border of iris Appearance of anterior lens after dilation Zonular changes GONIOSCOPY Goniolens Options Angle Documentation Pigment Dispersion Syndrome GONIOSCOPY Posner 4-Mirror Lens Sussman 4-Mirror Lens Single Mirror Lens Goldmann 3- Mirror Lens 4

5 Spaeth System Level of iris insertion: A anterior to trabecular meshwork B anterior to posterior limit of trabecular meshwork C posterior to scleral spur D into the mid ciliary body face (anterior CB band visible) E posterior CB (wide band of CB band visible) Angle width estimate in degrees from line tangential to the trabecular meshwork to line tangential to the iris surface one third of the way from the periphery (ranges from 0 40 degrees) Curvature of iris: f flat configuration, no significant forward or backward arching of iris b bowed or forward bowing (convex) curve c concave or posterior bowing curve P plateau iris configuration Pigmentation: 0 (no pigment) to 4 (heavy pigmentation) Gonioscopy Perform when suspicious for narrow angle Occludable angles: posterior, usually pigmented TM is seen for less than 90 of the angle circumference or if the angle width is less than 20 Perform gonioscopy in a dark room smallest square of light for a slit beam, so as to avoid stimulating the pupillary light reflex 5

6 Appositional Gonioscopy Differentiates appositional closure from synechial closure Gentle pressure on the cornea with goniolens pushes back the iris and reveals whether the angle can be opened any further synechial closure is present if unable to open can also help break acute attacks by forcing fluid into the periphery and opening areas of appositional closure Angle from synechial closure will not open Neovascularization of the Angle DYNAMIC (COMPRESSION OR APPOSITIONAL) GONIOSCOPY Useful in differentiating pupillary block in angle-closure glaucoma IMAGING MODALITIES supplement gonioscopy detailed images of structures and quantitative measurements useful in primary angle closure but can also help detect secondary cases of angle closure such as ciliary body masses or anterior rotation no widely agreed upon quantitative measurement cutoffs 6

7 Evaluating Anterior Chamber Depth Average Depth: 3.15 mm Shallows by 0.01 mm per year Less than 2.5 mm is at risk for angle closure Measuring: A Scan OCT UBM Ultrasound biomicroscopy (UBM) B scan ultrasound high-resolution cross-sectional images of the anterior segment of the eye to the anterior vitreous helpful for evaluating plateau iris and other CB pathology Disadvantages water bath immersion specialized equipment skilled technician to operate relatively costly and time consuming A-SCAN ULTRASONOGRAPHY Narrow anterior chamber angles may be measured with A-scan ultrasonography Relative risk of angle-closure can be assessed 75% of ACG occurs in chambers < 1.5mm ACG very rare in chambers > 2.5mm That Very Closed Open Angle The Gonioscopically Challenged Doctor Anterior segment OCT (AS-OCT) diode light source highly detailed images of the cornea, angle region, and anterior CB similar to those seen with UBM AS-OCT is unable to image structures posterior to the iris plane well because of posterior pigmented iris shadowing and scleral light scattering. noncontact exam the patient can be imaged in an upright position avoiding positional lens changes all four quadrants can be scanned at once get undisturbed images in the dark CASE Patient treated for years for Chronic Open-Angle Glaucoma IOPs elevated to the low 30 s Progressive visual field loss Never evaluated with a gonioprism! 7

8 Angle Closure High Resolution Image Pachymetry Measurement of Central Corneal Thickness (CCT) found to be important in OHTS Average CCT among OHT patients was found to be 570 microns: >588 microns was considered thick Thin corneas (< 555 microns) were a significant risk factor in conversion Thin corneas give a low Goldmann measurement- underestimates true IOP 8

9 CCT ASSESSMENT Has become standard Equipment has become widely available DGH was used in OHTS Consider potential effect of LASIK on IOP findings Reichert ocular response analyser (ORA) The Ocular Response Analyzer (ORA) is a new instrument that measures the corneal biomechanical response (corneal hysteresis, CH) to rapid indentation by an air jet. Cirrus HD-OCT anterior segment image of the cornea provides pachymetry readings using a caliper tool to measure central corneal thickness. Anterior Segment Imaging 560 μm in cornea Goldmann Tonometry: The Gold Standard Corneal Hysteresis Corneal biomechanical and physical properties, such as corneal hysteresis and CCT, are highly correlated and associated with VF progression. As corneal hysteresis may describe corneal properties more completely than thickness alone, it may be a parameter that is better associated with progression Goldmann Tonometry Remains the standard of care Consider corneal hysteresis and CCT Other modalities may be less influenced by CH and CCT 9

10 Pascal Dynamic Contour Tonometer The Tono-Pen XL cannot be used as a substitute for GAT in the management of patients with glaucoma or OHT. Tono-Pen XL The dynamic contour tonometer (DCT; Pascal tonometer) is a novel tonometer designed to measure intraocular pressure (IOP) independent of corneal properties. Tono-Pen XL Pascal dynamic contour tonometer A concave contact surface with a radius of curvature of 10.5 mm creates a distribution of forces between the central contour matching area of the tip and the cornea that equals the forces generated by the internal pressure of the eye. Readings are higher than with Goldmann applanation tonometry Appears to be less influenced by CCT May be useful S/P LASIK Tono-Pen XL 10

11 Cirrus HD-OCT Spectral domain OCT technology Capable of volumetric (3D) & high definition line scanning of the retina Similar to NFL polarimetry, OCT is used to determine the NFL thickness in the peripapillary region Compared to ultrasonography using laser light instead of acoustic waves Cirrus Optic Nerve Head Analysis Automatically identifies the optic disc and cup boundaries. Is generated using the dense data in the Optic Disc 200x200 data cube and a proprietary ZEISS algorithm. Is designed to precisely measure the neuro-retinal rim while accounting for tilted discs, disruptions to the RPE and other challenging pathology. How Does OCT Work? Cirrus RNFL and ONH Reference Mirror Beam Splitter Report shows RNFL and ONH for both eyes, using the Optic Disc Cube 200x200 scan Light Source Detector Cirrus RNFL Analysis OPTIC DISC CUBE SCAN The 6mm x 6mm cube is captured with 200 A-scans per B-scan, 200 B-scans. Cirrus RNFL and ONH Analysis Elements OCT en face fundus image shows boundaries of the cup and disc and the RNFL calculation circle. The integrated RNFL thickness deviation map shows deviation from normal RNFL thickness map also displays cup and disc mask CALCULATION CIRCLE AutoCenter function automatically centers the 1.73mm radius peripapillary calculation circle around the disc for precise placement and repeatable registration. Optic Nerve Head calculations are presented in a combined report with RNFL thickness data. Key parameters are compared to normative data and displayed in table format 11

12 Cirrus RNFL and ONH Analysis Elements GANGLION CELL ANALYSIS RNFL Peripapillary Thickness profile, OU compared to normative data Neuro-retinal Rim Thickness profile, OU - compared to normative data RNFL Quadrant and Clock Hour average thickness, OD and OS - compared to normative data Cirrus GPA Analysis Using the Cirrus HD-OCT, we can identify progression in RNFL loss through event analysis and trend analysis. GANGLION CELL ANALYSIS Event analysis assesses changes that are beyond an expected variability at certain points compared to normative data. If a patient falls outside this area, it is identified as progression. Trend analysis looks at the rate of change over time, using linear regression to determine whether or not the trend is outside the expected rate of RNFL loss. GLANGLION CELL INJURY GANGLION CELL ANALYSIS 12

13 Cirrus GPA Analysis RNFL Thickness Maps provide a topographical display of RNFL for each exam. RNFL Thickness Change Maps demonstrate change in RNFL between exams. Up to 6 progression maps are compared to baseline. Areas of statistically significant change are color-coded yellow when first noted and then red when the change is sustained over consecutive visits. THE FUTURE OF LASER IMAGING Broad applications in measurement of enlargement of the cup and change in the thickness of RNFL Guided Progression Analysis (GPA) DRANCE HEMORRHAGES Optic Disc Hemorrhages observed in glaucoma perhaps noticed particularly in NTG linked to progression of disease not adequately controlled GUIDED PROGRESSION ANALYSIS (GPA) 13

14 Drance Hemorrhage? Drance Hemorrhages Glaucomatous Optic Disc Hemorrhages characteristically in the axon bundles therefore, splinter-shaped and superficial crossing the disc boundary, or over a peripapillary crescent or halo or within the disc, not flame-shaped Definition of Glaucomatous ODH Flame- or splinter-shaped hemorrhage radially oriented and perpendicular to disc margin OHTS DATA Data from the Ocular Hypertension Treatment Study (OHTS) used to see: what makes disc hemorrhage likely? how predictive are they of the future course (conversion to glaucoma) in OHT? Definition of Glaucomatous ODH Flame- or splintershaped hemorrhage Not associated with disease other than glaucoma Vascular occlusive disease Diabetic retinopathy Ischemic optic neuropathy X Results: Cumulative Incidence of Disc Hemorrhage prior to POAG Months Since Randomization

15 Cumulative Incidence of Disc Hemorrhage prior to POAG endpoint 12 months between disc photos Disc hemorrhages may come and disappear between photographs Therefore previous graph almost certainly underestimates the true incidence of ODH Summary: Why do you get Drance Hemorrhages? OHTS participants who developed ODHs Similar to those who develop POAG including older age, thinner corneas, and larger vertical C/D, higher PSD EXCEPT THAT.. ODH not less frequent with lower baseline IOP assignment to treatment Drance Hemorrhages To our surprise Tx IOP lowering medicine did not reduce risk of ODH. Q: What does this mean? A: We don t know ODH marks inadequate IOP lowering ODH marks pathogenic mechanism mechanism is still there but it no longer causes harm ODH had lower incidence than POAG and analysis has insufficient power Summary: What if you get ODH? ODH is an additional independent risk factor for developing POAG But ODH is not synonymous with developing POAG 87% of OHT participants with ODH did NOT develop POAG endpoint during follow-up period 153 eyes of 168 who developed POAG did so without prior ODH ODH prediction of POAG in multivariate analysis Conclusions Hazard ratio 3.7 [ ] After adjusted for: Treatment Group Age Vertical cup/disc ratio Pattern standard deviation (visual field) Baseline IOP Central corneal thickness ODH are difficult to find, but a diligent search is worthwhile, because they represent one of the risk factors to be taken into account when making clinical decisions. 15

16 SURGICAL MANAGEMENT OF GLAUCOMA ALT Spot vs. SLT Spot Size ALT SLT LPI MIGS FILTRATION SURGERY SETONS Courtesy M. Berlin, M.D. Selective Laser Trabeculoplasty (SLT) SLT vs. ALT Q-Switched frequency doubled (532 nm) Nd:YAG Laser Selective targeting of pigmented (TM) cells No structural or coagulative damage to the TMless destructive than ALT Cytokines released that attract macrophages Courtesy M. Berlin, M.D. Human TM (organ system) ALT 50 um spot 360 o with non-overlapping spots R. Noecker, T. Kamm 16

17 Human TM (organ system) SLT 400 um spot, 0.8 mj/pulse Laser Iridotomy: Indications Primary angle closure glaucoma Secondary pupil block glaucoma Fellow eye in primary angle closure High risk asymptomatic narrow angles Nanophthalmos Long term miotic therapy with narrow angles R. Noecker, T. Kamm Pigmentary glaucoma (controversial) Laser Iridotomy IRIDOTOMIES 17

18 MIGS: MICROINVASIVE GLAUCOMA SURGERY BLEB-RELATED ENDOPHTHALMITIS Hypopyon, Vitritis GLAUCOMA FILTRATION SURGERY Trabeculectomy involves 8 separate incisions Conjunctiva Sub-Tenon s space Tension insertion at limbus Episclera Scleral flap edges Scleral flap base Sclerectomy Iridectomy MIGS: MICROINVASIVE GLAUCOMA SURGERY MIGS Viscocanalostomy Canaloplasty Transciliary Filtration (Fistulization) Trabectome istent XEN Gel Stent Glaucoma Implant Hydrus Microstent (Investigational) CyPass Micro-Stent (Investigational) Penetrating Filtering Surgery - Trabeculectomy istent Trabecular Micro- Bypass Stent Micro stent implanted in Schlemm s canal 18

19 istent: Heparin-Coated Nonferromagnetic Surgical Grade Titanium The stent is a soft, permanent, subconjunctival implant that shunts fluid from the anterior chamber to the subconjunctival space. istent: Placed in Schlemm s Canal at Lower Nasal Quadrant XEN Gel Stent for Glaucoma Approximately 6-mm long and the width of a human hair, the stent comes preloaded in a disposable Xen injector and is implanted through a small, self-sealing corneal incision. Visible on Gonioscopy GLAUCOMA GRAND ROUNDS 19

20 CASE 1 Color vision normal with AO-HRR pseudoisochromatic plates O.U. Desaturation to color targets O.D. Exophthalmometry normal O.U. Positive maintenance, pursuit, saccadic, vergence, oculocephalic, and ocular kinetic nystagmus (OKN) systems were intact PHYSICAL EXAMINATION 57-YEAR OLD ASIAN MALE No visual complaints S/P repair herniated disc Other review of systems negative No allergies to medications Negative family history Negative social history Presented taking 0.5% Timolol b.i.d. O.U. Tonometry: O.D. 10 mm Hg O.S. 11 mm Hg Review of prior records and subsequent diurnal examination show IOPs have consistently been measured from 10 to 15 mm Hg PHYSICAL EXAMINATION PHYSICAL EXAMINATION Entering Visual Acuity: O.D. 20/25 O.S. 20/20 No improvement with manifest refraction Pupils: 1+ RAPD O.D. SLE: Unremarkable O.U. Gonioscopy: D-40-f configuration with 1+ pigment observed O.U. OPHTHALMOSCOPY AND FUNDUS EVALUATION Clear media O.U. Sharp disc margins + Foveal reflex Healthy peripheral retina Intact retinal nerve fiber layer O.U. 20

21 Right nerve showed a large cup with shelving of the superior neuroretinal rim Left cup was smaller with an intact neuroretinal rim OPHTHALMOSCOPY OPHTHALMOSCOPY 30-2 Humphrey Visual Fields showed a dense inferior arcuate bundle defect breaking out of the blind spot O.D. The left visual field was normal and reliable THRESHOLD VISUAL FIELDS 21

22 TREATMENT PLAN D/C timolol (which he had been taking for several years without effect on IOPs) IOPs remained low and stable Currently being followed with serial visual fields, stereo disc photography, IOPs, and OCT imaging Currently on no medications Guided Progression Analysis (GPA) Statistical analysis of subsequent visual fields (overview and change analysis) showed stable visual fields O.U. THRESHOLD VISUAL FIELDS Guided Progression Analysis (GPA) 22

23 Follow-Up Evaluation 01/2017 IOPS: OD 11 OS 10 This patient has remained stable for 25 years off topical medication Careful observation is still required Monitor with visual fields, OCT, tonometry, fundus photography and IOPS x 6 months PHYSICAL EXAMINATION Visual Acuity: OD 20/20 OS 20/20 Refraction: OD x174 OS x174 Pupils: Reactive with trace RAPD OS Bright Illumination: OD 4.25 OS 3.75 Dim Illumination OD 5.75 OS 4.75 Lids demonstrate a 2 mm ptosis OS PHYSICAL EXAMINATION + Aproclonidine test Anhidrosis left side of face CASE 2 Horner (Horner s) Syndrome: Interruption of sympathetic nerve supply Anhidrosis, miosis and partial ptosis 49-YEAR OLD WHITE MALE Longstanding history of right hyper deviation Hypercholesterolemia Other review of systems negative No allergies to medications Negative family history Negative social history Presented with CC of things sometimes being more out of focus THRESHOLD VISUAL FIELDS 2/2011 Threshold Visual Fields (24-2 Program): OD Superior Nasal Step OS Superior Arcuate Defect With Superior Nasal Step 23

24 THRESHOLD VISUAL FIELDS 2/2011 FOLLOW-UP EVALUATION Neuro-eye Group ordered extensive testing including imaging, carotid duplex test, cardiac work-up, and comprehensive laboratory testing MRI of brain, orbits and neck were all normal including lung apices Carotid ultrasound normal All other testing was unremarkable and the patient referred for treatment of presumed normal tension glaucoma (NTG) PHYSICAL EXAMINATION 2/2011 OD 551 microns OS 556 microns IOPs: OD 16 OS 17 Note: IOPs never higher than 18 OD and 17 OS with multiple readings over 18 years Color Vision: OD 6/14 OS 5/14 using Ishihara Plates Fundus: OD.55/.6 OS.6/.65 with pallor of the neuroretinal rims. Pallor and cupping increased from photos taken in 1993 FOLLOW-UP EVALUATION 2012 Neuro-eye group ruled/out non-glaucomatous optic neuropathy and referred for glaucoma management Treatment was initiated with Travatan-Z qhs OU with a target IOP of 10 mmhg OU Treatment was increased by adding Simbrinza tid OU and target IOPs were achieved OCT showed significant RNFL thinning OS>OD ONH examination showed progressive pallor and cupping OS>OD ONH Photographs 2/2011 OCT RNFL ANALYSIS 10/

25 ONH PHOTOGRAPHS 10/2012 WHY NO BETA-BLOCKERS? Significant concerns re: very low pulse and blood pressure In addition, there are theoretical concerns with respect to beta-blockers such as timolol and perfusion to the ONH The patient was maintained on TravatanZ and Simbrinza S/P SLTs IOPs were found to be OD 08 and OS 09 in 8/2015 The patient was again referred to Neuro-eye Services to R/O non-glaucomatous optic neuropathy FOLLOW-UP EVALUATION Although target IOPs were achieved, there appeared to be progressive ONH and visual field changes observed OCT RNFL Analysis showed thin RNFL OS>OD Pupils were reactive but appeared to demonstrate a reversal of Horner Syndrome and fundus examination showed progressive ONH cupping OS>OD Blood pressure was very low at 110/60 and pulse was low at 40 bpm SLT laser treatment was performed to attempt to maintain IOPs as low as possible in addition to maximum medical therapy PHYSICAL EXAMINATION 8/2015 Neuro-eye found no new complaints and reports excellent compliance with his topical medication Currently taking Lipitor for hypercholesterolemia Runs regularly and reports excellent health Blood Pressure 115/64 ras and Pulse 44 bpm Visual Acuity: OD 20/20- OS 20/20- IOPs: OD 11 OS 10 OCT RNFL Analysis 3/14 PHYSICAL EXAMINATION 8/2015 Pupils: Reactive but + RAPD OS Bright Illumination: OD 4.0 OS 4.5 Dim Illumination: OD 4.5 OS Aproclonidine test Horner Syndrome reversal!! What does that mean??? ONH evaluation showed a C/D ratio of OD.75/.8 and OS.9/.95 OCT shows marked RNFL thinning OS>OD 25

26 OCT RNFL ANALYSIS 8/2015 TREATMENT PLAN Neuro-eye work-up was unremarkable except for very low blood pressure and pulse. Second opinion Neuro-eye opinion at Wills Eye Hospital was in agreement with our findings and treatment plan Two cardiology opinions were obtained with our suggestion of implanting a pacemaker Low perfusion pressure seems to be the only remaining modifiable risk factor Maximum medical therapy was maintained and IOPs (8/2016) were OD 10 OS 09 Threshold Visual Fields 10/2015 Follow-Up Evaluation 02/2017 IOPs increased to OD 18 and OS 17 (Repeatable) Surgical Trabeculectomy with MMC scheduled for 03/2017 Sleep study in New York- still considering pacemaker To be continued! Guided Progression Analysis GPA) 10/2015 CASE 3 26

27 67-YEAR-OLD WHITE MALE Hx: Referred from Primary Care Module for elevated IOP OS Originally from Canada and worked as a ski instructor No ocular or visual complaints Negative family Hx PHYSICAL EXAMINATION Slit-Lamp Examination: White, flaky material on the anterior border of the iris and on the anterior surface of the anterior lens capsule OS>>OD. Only a few flakes were present OD A clear middle zone was present OS with material present centrally and in the periphery Positive iris transillumination defects were present OS>>OD giving a moth eaten appearance to the pupillary margin. PHYSICAL EXAMINATION Entering VA: OD 20/20-3 OS 20/25-1 Pupils: PERRL but sluggish with + RAPD OS EOMs: Smooth and Full Gonioscopy (Sussman): OD D-40-f OS D-40-f Marked pigment deposition on the trabecular meshwork and Schwalbe s Line OS>OD PHYSICAL EXAMINATION TA: OD 22 mmhg OS 38 mmhg Ophthalmoscopy: OD.45/.45 with intact rim 360 OS.85/.85 with loss of the inferior neuroretinal rim CCT: OD 570 microns OS 565 microns 27

28 DIAGNOSIS Advanced Exfoliation Glaucoma OS (PXG)with borderline IOPs OD THRESHOLD VISUAL FIELDS MEDICAL MANAGEMENT Threshold Visual Field testing revealed essentially normal fields in the OD (borderline GHT) The OS showed profound loss of the superior hemifield Rx: TravatanZ qhs OS monocular trial FOLLOW-UP Follow-up at 3 weeks showed IOP decreased to 17 mmhg OS OD increased to 23 mmhg Rx: Travatan qhs OU Follow-up at 4 weeks demonstrated IOPs of : OD 14 mmhg OS 15 mmhg Careful observation x 3 months 28

29 FOLLOW-UP 2/2015 Regular follow-up evaluations have demonstrated stable visual fields and optic nerve head appearance. IOPs were elevated to OD 18 mmhg OS 24 mmhg on current regimen of Travatan Z and Azopt Combigan bid OU was added 2/2015 CHANDLER AND GRANT It does not require any genius to treat this disease. All one needs is a knowledge of the basic principles of diagnosis and treatment and of their correct application to the individual case. FOLLOW-UP 6/2015 THANK YOU!! Reports excellent compliance with medications IOPs were improved at OD 13 mmhg OS 17 mmhg on current regimen of Travatan Z, Azopt, and Combigan but target IOPs were not achieved OS SLT laser was performed 7/2015 OS and 8/2015 OD FOLLOW-UP Regular follow-up evaluations have demonstrated stable IOPs, OCT, visual fields, and optic nerve head appearance. Normal corneal hysteresis OU (OD 11.1 OS 10.9) Last examination (02/17) showed IOPs of: OD 09 mmhg OS 12 mmhg on current regimen of Travatan Z, Combigan and Azopt S/P SLT OU Do we need incisional surgery?? 29