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SCLAFANI U pdate on Keratoconus Diagnosis and Treatment Mahdavi MD.

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Presentation on theme: "SCLAFANI U pdate on Keratoconus Diagnosis and Treatment Mahdavi MD."— Presentation transcript:

1 SCLAFANI U pdate on Keratoconus Diagnosis and Treatment Mahdavi MD

2 SCLAFANI “Keratoconus is a clinical term to describe a condition in which the cornea assumes a conical shape because of thinning and protrusion”

3 SCLAFANI Keratoconus History Blurred vision DistortionPhotophobia Monocular polyopia Halos Patient presents with frequent eyeglass changes

4 SCLAFANI KCN HISTORY Non-inflammatory1/ Central 2/3 AR/AD Inheritance Females =Males Presents initially at puberty & progression varies, stability in 30s

5 SCLAFANI “Why don’t we see elderly patients with keratoconus” Do they die youngerNO Do they not visit POSSIBLE Have they CE/PKPPOSSIBLE THEORY BY KRACHMER The eye becomes more rigid as the patient ages and therefore the condition stabilizes The eye becomes more rigid as the patient ages and therefore the condition stabilizes

6 SCLAFANI ASSOCIATED SYSTEMIC CONDITIONS Vernal KC Atopic Dermatitis Down’s Syndrome Floppy Eyelid Syndrome Mitral Valve Prolapse Ehlers-Danlos Syndrome Osteogenesis Imperfecta Lawrence-Moon-Biedl Syndrome Neurofibromatosis Psuedoxanthoma Elasticum

7 SCLAFANI ETIOLOGY OF KCN History of trauma that causes weakness Recurrent trauma due to rubbing from Blepharitis, CL/lids, 53% have atopic dx Blepharitis, CL/lids, 53% have atopic dx Inflammatory component !!! Decrease proteinase inhibitors Decrease proteinase inhibitors Increase collagenase Increase collagenase Premature keratocytic apoptosis Premature keratocytic apoptosis Increase cytokine binding Increase cytokine binding

8 SCLAFANI Basic Science Research KCN have higher # of mitochondrial DNA deletions that leads to decrease oxidative phosphorylation… increase H Causes leakage, damages proteins, and results in oxidative stress Leads to apoptosis, abnormal healing, inflammation.

9 SCLAFANI Basic Science Research Yaron Rabinowitz, MD UCLA KCN have suppressed Aquaporin 5 (AQP5) AQP5 is the water transport gene that is responsible for cell migration and wound healing. Quantitative PCR testing (epithelial cells) could diagnose this

10 SCLAFANI RESEARCH MAY INDICATE NEW THERAPIES KCN is unlikely a single gene defect Chromosome 5, 21 Chromosome 5, 21 Multiple genes in a common pathway Those with the defect may develop KCN naturally or only if exposed to factors that induce oxidative stress: CL over-wear, UV, allergy or refractive surgery TX: Anti-inflammatory, Anti-oxidant

11 SCLAFANI RETINOSCOPY Scissors Reflex Against motion that breaks apart Represents multiple refractive powers within the optic zone

12 SCLAFANI KERATOCONUS-SLIT LAMP FINDINGS FLEISCHER RING abrupt change in curvature 50% VOGT’S STRIAE 1 st Sign 65% STROMAL THINNING STROMAL SCARS ENLARGED CORNEAL NERVES ACUTE HYDROPS 5%

13 SCLAFANI FLEISCHER RING

14 SCLAFANI VOGT’S STRIAE

15 SCLAFANI STROMAL SCARS

16 SCLAFANI ACUTE HYDROPS

17 SCLAFANI EXTERNAL FINDINGS MUNSONS SIGNRIZZUTIS SIGN

18 SCLAFANI Keratoconus- Keratometry Steepening begins infero-temporally and progresses clockwise Steepening begins infero-temporally and progresses clockwise TOPOGRAPHY- more sensitive PLACIDO RINGS- get closer

19 SCLAFANI PLACIDO RING IMAGES Rings that are closer together represent areas of steeper curvature May indicate a tight suture applicable

20 SCLAFANI ELEVATION (FLOAT) MAPS Predicts the relative elevation or depression of the cornea (in mm) using a computer generated BEST FIT SPHERE as a reference and fit at the steepest point

21 SCLAFANI ELEVATION MAPS PREDICT Na-FL PATTERN + VALUES- warm colors points higher than sphere = elevation Areas of bearing or touch - VALUES- cool colors points lower than sphere = depression Areas of pooling

22 SCLAFANI PELLUCID MARGINAL Tear meniscus can creates pseudo-PMD

23 SCLAFANI PMD vs. KCN PMD vs. KCN

24 SCLAFANI PSEUDOKERATOCONUS Corneal warpage topography can mimic KC Repeat topography must be performed and a measurable change would indicate pseudo- KC Evaluation of elevation maps at steep zone: Predicts the elevation or depression of the cornea if the best fit sphere was on cornea

25 SCLAFANI POSTERIOR KERATOCONUS

26 SCLAFANI KCN Effects on Vision Tim McMahon, OD 60% reduction in VA is due to curvature, not just high cylinder RGP corrects cylinder however HOA remain COMA May consider reverse geometry CLS Reduced low contrast VA Reads chart slower

27 SCLAFANI COMA Z 3 1 Similar to SA except that it concerns off axis peripheral rays that cause a comet-shaped image deformity to non-axial portions of the image. Minimal Post refractive surgery “Potato chip” due to flap hinge and shows the most dynamic change.

28 SCLAFANI Refractive Surgery Corneal laser refractive surgery: pre-op, enhancement options Phakic IOLs Corneal refractive implants: Intacs Anterior Segment Imaging and Surgery Corneal Imaging and Measurement Iris Imaging and Evaluation Trauma Assessment

29 SCLAFANI Visante Applications Anterior Segment Imaging and Surgery Corneal Imaging and Measurement imaging and evaluation of corneal pathologies penetrating keratoplasty lamellar keratoplasty endothelial keratoplasty keratoconus imaging and assessment anterior segment imaging through opaque corneas

30 SCLAFANI Terrien‘s Marginal Degeneration image courtesy of Dr. M. Packer

31 SCLAFANI Evolution of KCN: Ectasia to Hydrops image courtesy of Prof. G. Baikoff

32 SCLAFANI KCN/Open Angle (ML)

33 SCLAFANI KCN- Thinning (ML)

34 SCLAFANI Indications for Intra-Limbal Lenses KCN RGP dropouts Pellucid Marginal Post-PKP Astigmatic corneas SCL failures: due to neovascularization or poor visual acuity.

35 SCLAFANI Large Diameter Lenses Corneo- Scleral 12.9 mm mm Semi- Scleral 13.6 mm mm Mini- Scleral 15.0 mm-18.0 mm Scleral Bearing, minimum corneal clearance Full Scleral 18.1 mm- > 24+ mm Scleral Bearing, maximum corneal clearance Dyna Intralimbal (Lens Dynamics) Macrolens(C&H)Jupiter (Innovations in Sight) GBL(Con-Cise) Robert Breece, OD

36 SCLAFANI Intra-Limbal Fitting BC is Flatter than expected 4-5mm temporal vs. 4-5mm temporal vs. Average Mid K +.2mm Average Mid K +.2mmGoal Light feather touch Light feather touch.2mm < corneal diameter.2mm < corneal diameter (11.3 OAD).1-.2 mm movement.1-.2 mm movement.2mm edge clearance.2mm edge clearance Menicon Z or Extreme Menicon Z or Extreme Unique ph or Claris Unique ph or Claris

37 SCLAFANI SOFT LENS OPTIONS FOR KERATOCONUS Soft Spheres Soft Torics X-cel Flexlens Tricurve Basecurve Diameter Basecurve Diameter Center Thickness.45dK 13.2 Center Thickness.45dK 13.2 Continental, Gelflex USA, Ocu-Ease (Ocuflex K) Continental, Gelflex USA, Ocu-Ease (Ocuflex K)

38 SCLAFANI Benz 5x material,Glycerol Methacrylate Less dehydration, flexure,better optics Less dehydration, flexure,better optics Fit the normal peripheral cornea & sclera like standard SCL. The central posterior curve provides sagittal depth to touch POSTERIOR: Steep central curve, flatter paracentral peripheral curve all aspheric ANTERIOR: Central optical surface that quickly tapers to maximize 02 Low riding More movement Innovations in Sight SUPER NOVA HydroKone™

39 SCLAFANI Innovations in Sight SUPER NOVA HydroKone™ Base Curves: 4.1 to 9.3 ( ) Diameters: 12.0 to 17.0(14.8) Paracentral: (8.6) Sphere: to Cylinder: to Axis: 1 to 180 in 1 degree steps Mean K + 1mm Mean K + 1mm Do not use H2O2 due to thickness EXPECT MORE MOVEMENT

40 SynergEyes™ A High Dk Hybrid Material Paragon HDS 100 GP Center Paragon HDS 100 GP Center 27% Water Non Ionic Skirt (Group I) 27% Water Non Ionic Skirt (Group I)Design 14.5 mm over all diameter 14.5 mm over all diameter 8.4 mm rigid center 8.4 mm rigid center 7.8 mm optic zone 7.8 mm optic zone 2-4skirt radii choices for each base curve radius 2-4skirt radii choices for each base curve radius Skirt thickness consistent across full power range Skirt thickness consistent across full power range Engineered edge Engineered edge HyperBond™ junction technology HyperBond™ junction technology 14.5mm 8.4 mm Non-Ionic 27% water Hydrogel Skirt Non-Ionic 27% water Hydrogel Skirt Paragon HDS 100® Rigid Center Paragon HDS 100® Rigid Center

41 SCLAFANI SynergEyes KC 3 skirt curve options for fitting flexibility Spherical Skirt begins at 9.0 mm diameter Prolate ellipsoid base curve FDA Clearance December 2005

42 SCLAFANI SynergEyes KC Diagnostic Set Parameters Aspheric Base Curve PowerFlatSkirtMediumSkirtSteepSkirt 5.7mm (59.00) mm (57.00) mm (55.50) mm (53.50) mm (52.00) mm (50.50) mm (49.00) mm (47.50)

43 SCLAFANI SynergEyes Fitting Lens Movement.2mm to.3mm.2mm to.3mm movement with blink Slight lag in upward gaze Slight lag in upward gaze Free of scleral impingement Free of scleral impingement Free to move on “push up” Free to move on “push up” Free of “edge fluting” Free of “edge fluting”

44 SCLAFANI The Fitting Tips Never prescribe Flatter than Flat KNever prescribe Flatter than Flat K Counter-intuitive:Counter-intuitive: Corneas flatter than 44.25D and larger than 12.0 mm: Steeper Skirt Corneas Steeper 44.25D and smaller than 11.5 mm: Flatter skirt

45 Identical Apical Radius with Different HVID = different sagittal depth 11.0 mm 12.0 mm

46 SCLAFANI UPDATES FOR SYNERGEYES Proprietary materials that has a SiHy skirt and higher Dk GP The GP will have less flexure, will likely discontinue the enhanced profile To reduce peripheral crimping, the skirt curves will be multicurve: bi or aspheric CLEAR KONE : Additional KC lens for more ectopic or decentered peaks with reverse geometry to eliminate steep BC

47 SCLAFANI Vault the cornea yet aligns closer to cornea allowing lower powers Reverse geometry at skirt to allow more tear flow, easier removal

48 SCLAFANI TIPS ON PIGGYBACKS SCL protects from RGP or environment Reduces epithelial damage due to touch Protects from apical nodules Concurrent EBMD High DK, easily replaced= SiHi Soft Modulus molds to highly toric/steep K +SCL to flatten the RGP - SCL to steepen the RGP fit

49 SCLAFANI Intra-Stromal Rings Ring segments are placed into peripheral corneal channels outside the visual axis to correct low to moderate myopia by flattening the cornea without cutting or removing tissue form the central optical zone FDA approval of Intacs in 1999 for low/mod myopia. Recently approved for keratoconus in US July 2004 Principle benefit: delay or eliminate corneal graft Reversible/Removable

50 SCLAFANI Intacs Studies By Wachler and et al. 74 keratoconus eyes has insertion of intacs with F/U of 9 months 74 keratoconus eyes has insertion of intacs with F/U of 9 months 45% gain ≥ 2 lines BCVA (worst pre-opt) 51% had no effective changes 4% loss ≥ 2 lines of BCVA

51 SCLAFANI Single intrastromal corneal implant favored for paracentral cones Colin Chan, MD and Boxer Wachler,MD Compared 20 eyes (double) vs. 17 (single) Single used.25mm segment/ Double.25&.35 All had paracentral/peripheral cones Significantly better outcomes in change in cylinder, K values, UBVA, BCVA Single 2-3 line gain, Double 1 line gain

52 SCLAFANI Complications of Intacs for KCN UndercorrectionOvercorrection Neovascularization toward the Incision Migration of One segment toward the Wound Extrusion Stromal deposit Flap wrinkling (intracorneal inlays) Epithelial ingrowth Residual refractive error Complication rate ranges from 5-30%

53 SCLAFANI Biomechanical Effect of Combined Riboflavin-UVA The cross-linking in KCN is abnormal Too elastic and the biomechanical resistance is 50% Loss of Bowman’s Kristen Fry, OD

54 SCLAFANI Biomechanical Effect of Combined Riboflavin-UVA GOAL: Increase cross-linking Increase diameter 12% Anterior 12% Anterior 5% Posterior 5% Posterior

55 SCLAFANI C3-R Mechanism Riboflavin.1% UVA 370nm Corneal Collagen Crosslinking Biomechanical Stiffness Stability

56 SCLAFANI Theo Seiler, MD Initial workAJO, % reduction in max K by 2D (N=23) Increase in rigidity by 329% Increase in spacing (1nm) between the collagen molecules leads to increase diameter with no effect on transparency (150nm) Increased resistance to enzymatic digestion Has been shown to be effective for iatrogenic ectasia in animals. Bed < 400 um, severe endothelial damage

57 SCLAFANI PROCESS OF C3R-TX Topical Anesthetic Epithelium is scraped Acts as diffusion barrier, potential damage Acts as diffusion barrier, potential damage.1% Riboflavin drops q 5 min throughout Protects the endothelium, lens, retina Protects the endothelium, lens, retina Increases absorption into stroma Increases absorption into stroma 30 min. radiation 370 nm UVA –3mW/cm 3 Post-op FQ and pain relief Depth goes to 300 um therefore must have 400 um pachymetry to protect endothelium

58 SCLAFANI Studies by Eberhard Spoerl, PhD Immediate Evidence of increased x linking: Resistance to swelling and stretching utilizing Reicherts air pulse deforms cornea and measures area of deformation. Increases anchoring and reduces bulge Cellular Process 24h-12 weeks Leads to apoptosis of keratocytes with late migration of keratoblasts that result in flattening 5 yrs, N = 60, BCVA >1.4 lines K flat 2.87 D

59 SCLAFANI Studies by Aldo Caporossi, MD University of Sienna, Italy Suggests using it early in the disease to freeze tissue and prevent further ectasia Scrapes the epithelium prior to procedure. 12 eyes followed for 3months in All showed improved UCVA, BCVA, and reduced steepness- One side effect was transient stromal edema

60 SCLAFANI POTENTIAL USES OF C3R Post-Lasik ectasia Prevent KCN regression/scars Post CK-to enforce result Post-CRT- to enforce result? epithelial Boxer Wachler, MD has shown this to be an effective treatment when combined with Intacts for KCN

61 SCLAFANI References 1.Colin J, Simonpoli-Velou S. The Management of Keratoconus with Intrastomal Corneal Rings. International Ophthalmology Clinics. 43(3):65-80, Summer Kaiser P, Friedman N, et. al. The Massachusetts Eye and Ear Infirmary Illustrated Manual of Ophthalmology. Ed Kunimoto D, Kanitkar K, et al. The Wills Eye Manual. Fourth Edition. Lippincott Williams & Wilkins Roque M, Limbonsiong R, et. al. Myopia, Intracorneal Rings. August 14, Wachler B, Chandra N, et. al. Intacs for Keratoconus. American Academy of Ophthalmology Weissman B, Yeung K, et al. Keratoconus. Jan 29,

62 SCLAFANI Thank you


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