GOALS CORNEAL DYSTROPHIES AND DEGENERATIONS: Differentiate dystrophy vs. degeneration DIAGNOSIS AND TREATMENT Normal vs. abnormal Classify the disease by location Layers of the cornea Louise A. Sclafani, OD, FAAO Central vs. peripheral AAO Diplomate, Cornea & Contact Lens Determine appropriate treatment Associate Professor of Ophthalmology University of Chicago Medical Center Review the Layers of the Cornea CORNEAL DYSTROPHY Tear film 7-11 um Rare conditions Epithelium 50 um Slowly progressive, bilateral, central location Primary involvement of single corneal layer * Epithelial BM <128 nm Variable penetration and severity Bowman 8-14 um No associated systemic or ocular disease Stroma 500 um No sex predilection. Descemet 5-10 um Onset by age 20, stabilize by age 40 (except Fuchs) Autosomal dominant (50%) Endothelium 5 um CORNEAL DYSTROPHY CORNEAL DEGENERATION Stromal Epithelial Lattice Dystrophy Map/dot/fingerprint Non-familial, late onset Map/dot/fingerprint Granular Dystrophy Non-familial, late onset Meesman’s Avellino Dystrophy Macular Dystrophy Asymmetric, unilateral, central or peripheral Subepithelial/ Bowman’s Gelatinous Drop-Like Reis-Bücklers Dystrophy (CDB 1) Dystrophy Changes to the tissue caused by inflammation, Thiel-Behnke Honeycomb Schnyder Crystalline Dystrophy (CDB 2) Dystrophy age, or systemic disease. Subepithelial Mucinous Central Cloudy Dystrophy of Francois Fleck Dystrophy Characterized by a deposition of material, a Endothelial Cornea Farinata Fuchs’ dystrophy Pre-Descemet’s Dystrophy thinning of tissue, or vascularization CHED—congenital hereditary Posterior Amorphous Corneal endothelial dystrophy Dystrophy PPMD—posterior polymorphous Congenital Hereditary Stromal dystrophy Dystrophy Primary Band Keratopathy SCLAFANI-CORNEA 1 Corneal Degenerations GENETICS Most corneal dystrophies are autosomal dominant: From Periphery to Center (arbitrary division) Heterozygous =only one of the DNA strands effected Homozygous= more severe disease and recurrence in transplanted corneas is more prevalent. arcus senilis spheroidal degeneration lipid keratopathy iron deposition Autosomal recessive: 25% get it white limbal girdle of Vogt Coats’ white ring X linked: only men senile furrow crocodile shagreen Not much has changed in the diagnosis of corneal Terrien’s marginal degeneration corneal farinata disease however our increased understanding of the Hassall-Henle bodies Salzmann’s corneal degeneration genetics has allowed us to classify better. calcific band keratopathy corneal keloids calcareous degeneration corneal amyloid degeneration GENETICS EPITHELIUM Chromosomes 1,5,9,10,12,16,17,20,21 50 um non-keratinized stratified squamous epithelium Long arm of chromosome 5, 5q31 5-10 layers central 8-10 peripheral Gene codes for protein keratoepithelin which is involved in Bowmans and stroma attached to Descemet’s layer Superficial layers have microvillae that attach tears. Gene codes for 683 amino acids Exfoliation q 5-7 days Lattice, Granular, Avellino, Reis-Buckler Dystrophies Deeper layers (Basilar Columnar cells) have hemi- Discovering the pathways may aid in the use of drugs desmosomes to interfere with the deposition of substances connect the epithelium to basement membrane Many chromosomes explain the phenotypic variations. which connects to Bowman’s Layer. Any change of sequence in the amino acid chain can cause variations of the disease EPITHELIAL BASEMENT EBMD MEMBRANE “DISORDER” EBMD The basal cells produce abnormal finger-like Most common anterior corneal disorder projections that bend intra-epithelialy and trap DYS: inherited, single layer, bilateral cells/debris that form cysts. DEG: Prevalent 43%, 25% unilat,> 29y,trauma MAPS : multi-lamination of BM and collagen Abnormality of epithelial turnover, maturation, DOTS: grey opacities,cysts and production of BM and adhesion complexes FINGERPRINT: Thickened BM ultimately weakens the reduplication of BM epithelium and causes recurrent corneal erosion (RCE). SCLAFANI-CORNEA 2 SLX of EBMD TREATMENT FOR EBMD Negative NaFL pattern and instantaneous TBUT Indicated if vision or comfort are compromised. No Rose Bengal Stain No Rose Bengal Stain Manage co-existing ocular surface disease When Microcysts surface and erupt , + NaFL Environment/ diet Asymptomatic vs. Lubricants Variable degrees of Punctal occlusion Blur, diplopia, photophobia, dryness, FBS, or pain. Bandage Contact Lens (BCL) TX: Lubricants, hypertonics Surgical: PTK LUBRICANTS What’s New in Juice Avoid preservatives or surfactants Systane ALCON optive by Allergan Electrolytes nourish eye Hydroxypropyl-guar binds Lubrication to the tear layer and acts Avoid bland ointments: as a gelling agent and Osmoprotection hypo-osmotic and retain fluid binds to the hydrophobic Safely drawn into the Hyperosmotic agents ocular surface epithelial cells below the surface to osmotically Muro 128: Solution (2-5%) vs.ointment (5%) Mild to moderate dry eye, protect against hypertonic RGP wearers protect against hypertonic Ung: comfort, > concentration stress Treat 6 weeks Soln/3-6mo ung Warm Packs: QID 2-3 weeks What’s New in Juice What’s New in Juice Bausch & Lomb Soothe XP Bausch & Lomb Soothe blink contacts by AMO Blink Tears by AMO aka Soothe, Alimera Science Glycerin (0.6%) - Lubricant Sodium Hyaluronate Higher concentration of contains “Restoryl” Propylene Glycol (0.6%) – Compounds Sodium Hyaluronate Lubricant Emolient for Moderate to Naturally occurring Visco-elastic properties Advanced dry eye substance OcuPure® Sodium Light Mineral Oil 1% Visco-elastic properties= Chlorite Preservative Mineral Oil 4.5% Viscosity • Dissipating with light Restores & prevents tear loss Predominant glycos- hypotonic (around 170 Blur upon instillation aminoglycan to appear at mOsm) to help counteract Bedtime and am dosing wound site. the hypertonic stress OK with Contact lenses isotonic 286 mOsm OK with Contact lenses demulcent agent (PEG Aquify by CIBA 400) SCLAFANI-CORNEA 3 Not Available yet… What’s New in Juice FreshKote Bausch and Lomb RX only Artificial Tear* Patents, concentration, monitor Hycosan® is a sterile, preservative- and phosphate Treats all 3 layers free moisturising solution containing the natural Amisol Clear restores lipid ingredient .1 %sodium hyaluronate, sorbitol, a citrate 2% polyvinyl pyrrolidone and polyvinyl alcohol improve both aqueous and buffer and water. mucin layer Polixetonium preservative with low toxicity, anti-microbial, anti- Economy and ease of multi-dose bottle= green! fungal,& facilitates wetting 1 click = 1 drop (300 per) High oncotic pressure Reduces edema, establishes epithelial integrity and may prevent damage 3-6 x per day, OK with CLS Focus Laboratories LACRISERT ALTERNATIVE DROPS N-ACETYLCARNOSINE “inactive ingredient” Visual Ocuity™ A Professional Product from Longevity Science® Can-C, International Anti-ageing Systems, UK HPMC 0.3% and Glycerin 1.0% Anti-oxidant compound combined with CMC Carnosine penetrates the lipid membrane of the lens to reduce opacification Improves VA/glare PUNCTAL OCCLUSION: Autologous Serum Drops PUNCTAL OCCLUSION: THE IDEAL PLUG Utilizes patient’s own blood serum Easy and comfortable to insert Blood is drawn and the serum is spun down and mixed Negligible corneal contact, no sensation with artificial tears. Devoid of cells and clot factors Visible upon inspection only Replaces individualized growth factors Reversible:easy to remove by a professional Replaces individualized antibodies Reversible:easy to remove by a professional Serum contains growth factors, fibronectin, Vit. A and Inert material with no allergic response anti-proteases Effective in the treatment of dry eye Requires blood donation 2-3 times year $150-$300 Responsibility = Consent Hospital/Lieters Increase contact time of natural or artificial tears on the Consider 5-10% serum albumin drops qid instead eye. SCLAFANI-CORNEA 4 Superficial Punctate Keratitis of SPKT Thygeson (SPKT) Chronic, usually bilateral disorder characterized by NaFl/RB staining and elevated central focal epithelial lesions and no stromal during active disease process involvement. Each lesion comprised of multiple lesions Fine or dense/ Single or Multi Change position over time Avg of 15-20 lesions (1 to 50) Conjunctiva: usually not inflamed unless during the Corneal sensation not effected although occasional developmental stage:1-2 wks hypoaesthesia Conjunctiva is not inflamed* Etiology of SPKT Unknown Etiology of SPKT Unknown Possibly Viral due to latency, recurrence, lesion appearance, R/O etiology of duration PCR testing proved that it is NOT HSV 1 or 2, VZV or Punctate epithelial erosions PEE vs. adenovirus Still investigating HPV since both have minimal inflammation Sub-epithelial infiltrates SEI vs. Prolonged SPKT Associated with Salzmans Nodular Deg. Superficial Punctate keratitis SPK Suggested association with eczema, urticaria, asthma HLA-DW3 and DR3 association: glutten sensitive, DM2, Lupus, Graves “Pink EYE” SPKT STANDARD TESTS Other bacterial infections, such No testing done – expensive, as Strep, use a confirmation test. Mean age 29 (2 to 70) time consuming • FDA Cleared Diagnosis based on hx/exam • CLIA Waived Usually Bilateral Misdiagnosis ~ 50% of cases • 10 minute results Most often, treated empirically • Detects all 51 serotypes of Favor the central visual axis Antibiotics – between 95%-99% of adenovirus all cases