Update on Keratoconus Diagnosis and Treatment Mahdavi MD
- Slides: 62
Update on Keratoconus Diagnosis and Treatment Mahdavi MD SCLAFANI
“Keratoconus is a clinical term to describe a condition in which the cornea assumes a conical shape because of thinning and protrusion” SCLAFANI
Keratoconus History Blurred vision Distortion Photophobia Monocular polyopia Halos Patient presents with frequent eyeglass changes SCLAFANI
KCN HISTORY Non-inflammatory 1/2000 -5000 Central 2/3 AR/AD Inheritance Females =Males Presents initially at puberty & progression varies, stability in 30 s SCLAFANI
“Why don’t we see elderly patients with keratoconus” Do they die younger NO Do they not visit POSSIBLE Have they CE/PKP POSSIBLE THEORY BY KRACHMER n The eye becomes more rigid as the patient ages and therefore the condition stabilizes 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 SCLAFANI
ETIOLOGY OF KCN History of trauma that causes weakness Recurrent trauma due to rubbing from n Blepharitis, CL/lids, 53% have atopic dx Inflammatory component !!! n n Decrease proteinase inhibitors Increase collagenase Premature keratocytic apoptosis Increase cytokine binding SCLAFANI
Basic Science Research KCN have higher # of mitochondrial DNA deletions that leads to decrease oxidative phosphorylation… increase H 202 Causes leakage, damages proteins, and results in oxidative stress Leads to apoptosis, abnormal healing, inflammation. SCLAFANI
Basic Science Research Yaron Rabinowitz, MD UCLA KCN have suppressed Aquaporin 5 (AQP 5) AQP 5 is the water transport gene that is responsible for cell migration and wound healing. Quantitative PCR testing (epithelial cells) could diagnose this SCLAFANI
RESEARCH MAY INDICATE NEW THERAPIES KCN is unlikely a single gene defect n 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 SCLAFANI
RETINOSCOPY Scissors Reflex Against motion that breaks apart Represents multiple refractive powers within the optic zone 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% SCLAFANI
FLEISCHER RING SCLAFANI
VOGT’S STRIAE SCLAFANI
STROMAL SCARS SCLAFANI
ACUTE HYDROPS SCLAFANI
EXTERNAL FINDINGS MUNSONS SIGN RIZZUTIS SIGN SCLAFANI
Keratoconus- Keratometry Steepening begins infero-temporally and progresses clockwise TOPOGRAPHY- more sensitive PLACIDO RINGS- get closer SCLAFANI
PLACIDO RING IMAGES Rings that are closer together represent areas of steeper curvature May indicate a tight suture applicable 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 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 SCLAFANI Areas of pooling
PELLUCID MARGINAL Tear meniscus can creates pseudo-PMD SCLAFANI
PMD vs. KCN 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 SCLAFANI
POSTERIOR KERATOCONUS SCLAFANI
KCN Effects on Vision Tim Mc. Mahon, 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 SCLAFANI
COMA Z 31 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. SCLAFANI
Refractive Surgery n Corneal laser refractive surgery: pre-op, enhancement options n Phakic IOLs n Corneal refractive implants: Intacs Anterior Segment Imaging and Surgery n Corneal Imaging and Measurement n Iris Imaging and Evaluation n Trauma Assessment 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 SCLAFANI
Terrien‘s Marginal Degeneration SCLAFANI image courtesy of Dr. M. Packer
Evolution of KCN: Ectasia to Hydrops SCLAFANI image courtesy of Prof. G. Baikoff
KCN/Open Angle (ML) SCLAFANI
KCN- Thinning (ML) SCLAFANI
Indications for Intra-Limbal Lenses KCN RGP dropouts Pellucid Marginal Post-PKP Astigmatic corneas SCL failures: due to neovascularization or poor visual acuity. SCLAFANI
Large Diameter Lenses Dyna Intralimbal (Lens Dynamics) Macrolens (C&H) Jupiter (Innovations in Sight) GBL (Con-Cise) Corneo- Scleral 12. 9 mm- 13. 5 mm Semi- Scleral 13. 6 mm- 14. 9 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 SCLAFANI Robert Breece, OD
Intra-Limbal Fitting BC is Flatter than expected n n K @ 4 -5 mm temporal vs. Average Mid K +. 2 mm Goal n n n Light feather touch. 2 mm < corneal diameter (11. 3 OAD). 1 -. 2 mm movement. 2 mm edge clearance Menicon Z or Extreme Unique ph or Claris SCLAFANI
SOFT LENS OPTIONS FOR KERATOCONUS Soft Spheres Soft Torics X-cel Flexlens Tricurve n n n Basecurve 6. 0 - 9. 9 Diameter 10. 0 -15. 0 Center Thickness. 45 d. K 13. 2 Continental, Gelflex USA, Ocu-Ease (Ocuflex K) SCLAFANI
SUPER NOVA Hydro. Kone™ Innovations in Sight Benz 5 x material, Glycerol Methacrylate n Less dehydration, flexure, better optics Fit the normal peripheral cornea & sclera like standard SCL. The central posterior curve provides sagittal depth to touch SCLAFANI 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 Hydro. Kone™ Base Curves: 4. 1 to 9. 3 (5. 3 -8. 5) Diameters: 12. 0 to 17. 0 (14. 8) Paracentral: 8. 0 -9. 2 (8. 6) Sphere: +50. 00 to -75. 00 Cylinder: -0. 25 to -50. 00 Axis: 1 to 180 in 1 degree steps Mean K + 1 mm Do not use H 2 O 2 due to thickness EXPECT MORE MOVEMENT SCLAFANI
Synerg. Eyes™ A High Dk Hybrid Material n n Paragon HDS 100 GP Center 27% Water Non Ionic Skirt (Group I) Paragon HDS 100® Rigid Center Design n n n 14. 5 mm over all diameter 8. 4 mm rigid center 7. 8 mm optic zone 2 -4 skirt radii choices for each base curve radius Skirt thickness consistent across full power range Engineered edge Hyper. Bond™ junction technology SCLAFANI 8. 4 mm 14. 5 mm Non-Ionic 27% water Hydrogel Skirt
Synerg. Eyes KC Prolate ellipsoid base curve Spherical Skirt begins at 9. 0 mm diameter 3 skirt curve options for fitting flexibility FDA Clearance December 2005 SCLAFANI
Synerg. Eyes KC Diagnostic Set Parameters Aspheric Base Curve Power Flat Skirt 5. 7 mm (59. 00) -14. 00 8. 5 8. 2 7. 9 5. 9 mm (57. 00) -14. 00 8. 5 8. 2 7. 9 6. 1 mm (55. 50) -12. 00 8. 5 8. 2 7. 9 6. 3 mm (53. 50) -10. 00 8. 8 8. 5 8. 2 6. 5 mm (52. 00) -8. 00 8. 8 8. 5 8. 2 6. 7 mm (50. 50) -6. 00 8. 8 8. 5 8. 2 6. 9 mm (49. 00) -5. 00 8. 8 8. 5 8. 2 7. 1 mm (47. 50) -4. 00 9. 1 8. 8 8. 5 SCLAFANI Medium Steep Skirt
Synerg. Eyes Lens Movement n n n . 2 mm to. 3 mm movement with blink Slight lag in upward gaze Free of scleral impingement Free to move on “push up” Free of “edge fluting” SCLAFANI Fitting
The Fitting Tips • Never prescribe Flatter than Flat K • Counter-intuitive: Corneas flatter than 44. 25 D and larger than 12. 0 mm: Steeper Skirt Corneas Steeper 44. 25 D and smaller than 11. 5 mm: Flatter skirt SCLAFANI
Identical Apical Radius with Different HVID = different sagittal depth 3. 60 2. 96 11. 0 mm SCLAFANI 12. 0 mm
UPDATES FOR SYNERGEYES Proprietary materials that has a Si. Hy 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 SCLAFANI
Vault the cornea yet aligns closer to cornea allowing lower powers Reverse geometry at skirt to allow more tear flow, easier removal 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= Si. Hi Soft Modulus molds to highly toric/steep K +SCL to flatten the RGP - SCL to steepen the RGP fit 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 SCLAFANI
Intacs Studies By Wachler and et al. n 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 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. 25 mm 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 SCLAFANI
Complications of Intacs for KCN Undercorrection Overcorrection 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% 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 SCLAFANI
Biomechanical Effect of Combined Riboflavin-UVA GOAL: Increase cross-linking Increase diameter n n 12% Anterior 5% Posterior SCLAFANI
C 3 -R Mechanism UVA 370 nm Riboflavin. 1% Corneal Collagen Crosslinking Biomechanical Stiffness Stability SCLAFANI
Theo Seiler, MD Initial work AJO, 2003 70% reduction in max K by 2 D (N=23) Increase in rigidity by 329% Increase in spacing (1 nm) between the collagen molecules leads to increase diameter with no effect on transparency (150 nm) Increased resistance to enzymatic digestion Has been shown to be effective for iatrogenic ectasia in animals. Bed < 400 um, severe endothelial damage SCLAFANI
PROCESS OF C 3 R-TX Topical Anesthetic Epithelium is scraped n Acts as diffusion barrier, potential damage . 1% Riboflavin drops q 5 min throughout n n Protects the endothelium, lens, retina Increases absorption into stroma 30 min. radiation 370 nm UVA – 3 m. W/cm 3 Post-op FQ and pain relief Depth goes to 300 um therefore must have 400 um pachymetry to protect endothelium SCLAFANI
Studies by Eberhard Spoerl, Ph. D 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 24 h-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 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 3 months in 2004. All showed improved UCVA, BCVA, and reduced steepness. One side effect was transient stromal edema SCLAFANI
POTENTIAL USES OF C 3 R 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 SCLAFANI
References 1. Colin J, Simonpoli-Velou S. The Management of Keratoconus with Intrastomal Corneal Rings. International Ophthalmology Clinics. 43(3): 65 -80, Summer 2003. 2. Kaiser P, Friedman N, et. al. The Massachusetts Eye and Ear Infirmary Illustrated Manual of Ophthalmology. Ed. 2. 2004. 3. Kunimoto D, Kanitkar K, et al. The Wills Eye Manual. Fourth Edition. Lippincott Williams & Wilkins 2004. 4. Roque M, Limbonsiong R, et. al. Myopia, Intracorneal Rings. August 14, 2002. www. emedicine. com/oph/topic 665. htm 5. Wachler B, Chandra N, et. al. Intacs for Keratoconus. American Academy of Ophthalmology. 2003. 1031 -1039. 6. Weissman B, Yeung K, et al. Keratoconus. Jan 29, 2005 www. emedicine. com/oph/topic 104. htm SCLAFANI
Thank you SCLAFANI
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