9/15/2013. Naturally-existing Corneal Pathology Forme Fruste Keratoconus Keratoconus Pellucid Marginal Degeneration



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Bill Tullo, OD, FAAO Diplomate Vice-President of Clinical Services TLC Vision 33386-PO 1 Hour Bill Tullo, OD has no financial interests in any of the products or companies discussed in this program Naturally-existing Corneal Pathology Forme Fruste Keratoconus Keratoconus Pellucid Marginal Degeneration Surgically induced Pathology Mechanical Instability Post- LASIK ectasia Post-PRK ectasia Abnormal Collagen fibrils vs biomechanical destabilization 1

Progressive corneal steepening and thinning in the absence of refractive surgery Adolescent onset Progresses over 10-20 years Incidence 1/2,000 in general population Prevalence: 1/430 2% in patients seeking surgical correction of refractive errors About 20% of patients with KC require corneal transplantation KC is the indication for 5000 (15%) corneal transplants performed each year in the US 1 Kennedy RH, et al., A 48-year clinical and epidemiologic study of keratoconus. Am J Ophthalmol. 1986; 101; 267-73 2 Nielsen, K., et al., Acta Ophthalmologica Scand. 23, 2007 3 Nesburn AB, et al., Keratoconus detected by videokeratography in candidates for photorefractive keratectomy. J Ref Surg. 1995;11:194-201 4 2006 EBAA Eye Banking Statistical Report Reduced corneal rigidity in eyes with keratoconus Corneal Hysteresis 2

Reduced likelihood of keratoconus progression with age (physiological cross-linking) 1 Diabetes protects against development of keratoconus glycation 2 Cigarette smoking protects against development of keratoconus unknown toxins in cigarette smoke protects against development of keratoconus 3 1 Malik et al., Biochem. Biophys. Acta, 1992 1 Malik et al., Biochem. Biophys. Acta, 1992 2 Seiler et al., Graefe s Arch Clin Exp Ophthalmol 238:822, 2000 3 Spoeri et al. J Refractive Surg. 2008:24:7: Flap Thickness/Diameter Ablation Depth Irregular topography 3

Glutaraldehyde crosslinking (prosthetic heart valves) Formaldehyde (pathology specimens) Aldehyde sugars (diabetes) UVA-induced crosslinking (dentistry) Absorption 5 365-370 nm 4 3 2 1 0 300 350 400 450 500 550 wavelength (nm) Basics in Cross Linking 1. Riboflavin (vit. B2) + Ultraviolet radiation H 3 C OH O P H 2C OH H C OH H C OH H C OH H 2 C N N O - O NH H 3C N O 2. Production of oxygen radicals (ROS) O - 2 Cross Linking 3. Induction of collagen cross-links -CH 2-CH 2-CH 2-CH = NH-CH 2-CH 2-CH 2-CH 2- collagen fibril collagen fibril 4

Pretreatment 30 Days Post Treatment Studied since 1994 University of Dresden T Seiler, E Spoer, G Wollensak 23 eyes (Maximum K values 48-72D) treated In ALL treated eyes, the progression of Keratoconus stopped 70% reduction of K readings by 2.01 D 1.14D reduction of refractive error 65% improvement in Visual Acuity (3.6 lines Caprorossi et al) 5

Does it really work? Is it really safe? How long does the effect last? Does the epithelium have to be removed? 1 What is the best protocol? 1 Chan et al., J. Refract. Surg. 33:75, 2007) Preop Postop Wollensak, Spoerl, and Seiler, AJO 135:620, 2003. Wollensak, Spoerl, and Seiler, AJO 135:620, 2003. Caporossi A, Mazzotta C, Baiocchi S, Caporossi T. Long-term results of riboflavin ultraviolet a corneal collagen cross-linking for keratoconus in Italy: the Siena eye cross study Am J Ophthalmol. 2010 Apr;149(4):585-93 6

687 Peer-reviewed literature citations 96% of eyes show topographic stability Average flattening 1.7D of max-k Flattening effect reduced max-k > 54D 0.1% riboflavin UVA dosage 3 mw/cm2 for 30 minutes 7

1 drop q2 min x 30 min 8

Riboflavin q 2 minutes Endothelial Cell Loss Crystalline lens opacity Pain Infection Delayed Epithelial healing Retinal damage Stromal Haze 9

Confocal Microscopy Pre op 1 m postop 3 m postop Apoptosis 300 µm deep after CXL Repopulation takes 6 months 6 m postop Courtesy of Dr. Caporossi, 400 microns when using 3.0 mw/cm2 and Dextran based Riboflavin Thicken a thin cornea with hypoosmotic riboflavin not safe 1 Stromal haze peaks at 1 month post-op and decreases significantly between 3 and 12 months 2 1.Hafezi F. Limitation of Collagen Cross-Linking With Hypoosmolar Riboflavin Solution: Failure in an Extremely Thin Cornea. Cornea. 2011 Mar 8. 2. Greenstein SA, Fry KL, Bhatt J, Hersh PS Natural history of corneal haze after collagen crosslinking for keratoconus and corneal ectasia: Scheimpflug and biomicroscopic analysis. J Cataract Refract Surg. 2010 Dec;36(12):2105-14 10

Halts progression of ectatic corneal diseases Decreases corneal curvature and thickness Regularizes corneal surface Improves UCVA and BSCVA Effect lasts indefinitely Offers safe and effective treatment for conditions with no currently available treatment and may avoid 15% of corneal transplants Disability, cost, loss of productivity, CTL International CE Mark since 2006 Distributed internationally United States Investigational Combination product Device: UVA light source Drug: Riboflavin > 27 total FDA studies 13 US studies FDA Grants Riboflavin Orphan Drug Status 10/10 Avedro 7 years of exclusive rights 1/12 FDA Expedited Review Request 11

Young patients with good history expected to progress if untreated Age 35 yr Kmax 56 D Pachymetry > 400 microns Health History Non-smoker/Non-diabetic Corneal signs Scissoring or Thinning No or few Vogt s striae No or little scarring Keratoconus/Ectasia History Rapidly progressive disease At least 3 months of topographic history preferred Multicenter (100 sites), randomized Keratoconus and Ectasia after Refractive Surgery > 12 yrs old and evidence of KC on topography Minimum Pachymetry > 375 microns 3 treatment groups Epi-removal 15 mw/cm 2 x 8 minutes Epi-removal 30 mw/cm 2 x 4 minutes Epi-removal - 45 mw/cm 2 x 2 min 40 sec Barrier to riboflavin penetration of stroma Epithelium Stroma 12

Epithelium-OFF 0.1% Riboflavin with 20% Dextran VibeX Rapid - 0.1% riboflavin with hydroxypropyl methylcellulose Reduced corneal thinning allow treatment of corneas as thin as 325 microns Epithelium-ON (Transepithelial) Paracel BAC opens epithelial junctions VibeX Xtra - 0.1% riboflavin Mechanical Disruption Daya Disruptor Chemical Disruption BAK Tetracaine EDTA 20% Alcohol Permeability Enhancers Cellulose or sodium phosphate Iontophoresis 1. Apply topical anesthetic to the cornea. 2. Insert lid speculum using standard clinical techniques. 3. Apply 2-4 4 drops of ParaCel to coat the cornea. Apply an additional drop every 90 secs for a total soak of 4 mins. 4. Rinse cornea completely with VibeX XtraTM. 5. Apply sufficient VibeX Xtra to coat the cornea and repeat this procedure every 90 secs for a total of 6 mins. 6. Initiate UV treatment using the KXL SystemTM for 2 mins 40 secs at 45mW/cm2, applying 1-2 drops of BSS as needed during irradiation. 7. Rinse cornea completely with BSS. 8. Remove speculum using standard clinical techniques. 13

Riboflavin 1% with gum cellulose Q2 min: From local compounding pharmacies Topical Tetracaine with BAK provided Q2 to 5 min Corneal Protector sponge used 60-80 minutes of Riboflavin drops required Corneal Protector Sponge William Trattler, MD Benefits: Faster visual recovery & less pain Reduced risk of pain & haze Reduced risk of infection & slow re-epithelialization Very good clinical results Even in keratoconus patients in their 50 s and 60 s Downside: Longer procedure (30-50 min longer) Insufficient Riboflavin penetration can lead to UVA over-exposure and damage to ocular structures. Can not combine with simultaneous topo-guided PRK Effects of both treatments are synergistic Increased K flattening Increased BCVA Increased UCVA Kamburoglu G, Ertan A: Intacs Implantation with Sequential CXL Treatment in Postoperative LASIK Ectasia. J Refractive Surg. 2008:24:7:S726-S729 Chan CC, Sharma M, Wachler BS: Effect of inferior-segment Intac with and without C3-R on keratoconus. J Cataract Refract Surg. 2007;33:75-80. 14

Simultaneous CXL & PRK more effective than sequential CXL & PRK PRK before CXL better than PRK after CXL Kanellopoulos AJ, Binder PS. Management of Corneal Ectasia After LASIK with Combined, Same-Day, Topography-Guided Partial Transepithelial PRK and Collagen Cross-Linking: The Athens Protocol. J Refract Surg. 2010 Nov 5:1-9. Kanellopoulos AJ Comparison of sequential vs same-day simultaneous collagen cross-linking and topography-guided PRK for treatment of keratoconus. J Refract Surg. 2009 Sep;25(9):S812-8 Topographically Guided Transepithelial PRK Treat 70% corneal astigmatism 0.02% Mitomycin-C 20 seconds 0.1% riboflavin sodium phosphate x 10 minutes 370nm 3.0 mw/cm 2 x 30 minutes Use of modified CXL protocol for LASIK patients All LASIK patients vs. at risk Thin corneas Irregular topography High myopia Stabilize hyperopic LVC? 15

1. After ablation, sufficient VibeX Xtra is applied to coat the stromal bed 2. Following a 90 second soak, VibeX Xtra is thoroughly flushed from the eye using BSS 3. The flap is carefully replaced, and the cornea is irradiated with the KXL device at 45mW/cm2 for 90 seconds Poorer prognosis Faster progression Increased probability of PK Higher risk of PK rejection MUST DIAGNOSE KERATOCONUS AS EARLY AS POSSIBLE!! Early Keratoconus progressive topography or tomography minimal reduced BCVA CXL as soon as Dx confirmed Moderate Keratoconus progressive topography or tomography moderate reduced BCVA Consider CXL / Intacs Advanced Keratoconus pachymetry less than 400 microns Intacs if stable with CL refit PK as last resort 16

Transepithelial CXL High Irradiance CXL Oxygen Enhanced CXL Accelerated CXL with Pulsed Illumination Intrastromal CXL LASIK Xtra & PRK Xtra Topographic Guided - CXL Alternative Agent CXL Selective CXL Wait till ectasia progresses before CXL Older patients don t benefit from CXL Insurance will pay for CXL soon The epithelium must be removed for CXL CXL effect is temporary CXL not approved because it s dangerous Epithelium has to be removed for CXL The Mission : Comprehensive KC Education for ECP s Resource for all KC Patient s Needs Provide Comprehensive Care for KC Patients in Conjunction with Affiliated ECP s Advancement of Knowledge re-kc Through Clinical Research Leading to Ongoing Improvement in the Care of KC Patients 17

THANKS! 18