Louise Sclafani, OD, FAAO CORNEAL DYSTROPHIES Diplomate, American Academy of Optometry Cornea, AND DEGENERATIONS: Contact Lens and Refractive Technology Associate Professor of Ophthalmology DIAGNOSIS AND TREATMENT University of Chicago 2003-2017 Vice President Professional Affairs, SynergEyes Private Practice Louise A. Sclafani, OD, FAAO SoLo Eye Care and Eyewear Gallery L.L.C. Chicago, IL Clinical Associate Professor AAO Diplomate, Cornea & Contact Lens Illinois College of Optometry CONSULTING/SPEAKER Aerie Pharmaceuticals Alcon Allergan Bausch & Lomb BHVI Cooper Vision EyeprintPRO, Shire, SynergEyes, Johnson& Johnson Vistakon, Zeavision OPTOS, North America Husband, Jeff McClimans GOALS Review the Layers of the Cornea ➢ Differentiate dystrophy vs. degeneration ➢ Tear film 7-11 um ➢ Review Normal vs. abnormal ➢ Epithelium 50 um ➢ Classify the condition by location ➢ Epithelial BM <128 nm ⚫ Layers of the cornea ➢ Bowman 8-14 um ⚫ Central vs. peripheral ➢ Stroma 500 um ➢ Determine appropriate treatment and present in order of complexity ➢ “Dua Layer” 15 um ➢ Similar treatment for various conditions ➢ Descemet 5-10 um ➢ Endothelium 5 um SCLAFANI CORNEAL DYSTROPHY CORNEAL DYSTROPHY ➢ Stromal ➢ Epithelial ➢ Rare conditions ⚫ Lattice Dystrophy ⚫ Map/dot/fingerprint ⚫ Granular Dystrophy ⚫ Meesman’s ➢ Slowly progressive, bilateral, central location ⚫ Avellino Dystrophy ⚫ Macular Dystrophy ➢ Primary involvement of single corneal layer * ➢ Subepithelial/ Bowman’s ⚫ Gelatinous Drop-Like ⚫ Reis-Bücklers Dystrophy (CDB 1) Dystrophy ⚫ Schnyder Crystalline ➢ Variable penetration and severity ⚫ Thiel-Behnke Honeycomb Dystrophy (CDB 2) Dystrophy ⚫ Central Cloudy Dystrophy of ⚫ Subepithelial Mucinous ➢ No associated systemic or ocular disease Francois ⚫ Fleck Dystrophy ➢ Endothelial ➢ No sex predilection. ⚫ Cornea Farinata ⚫ ’ Fuchs dystrophy ⚫ Pre-Descemet’s Dystrophy ➢ Onset by age 20, stabilize by age 40 (except Fuchs) ⚫ CHED—congenital hereditary ⚫ Posterior Amorphous Corneal endothelial dystrophy Dystrophy ➢ Autosomal dominant (50%) ⚫ PPMD—posterior polymorphous dys ⚫ Congenital Hereditary Stromal Dystrophy ⚫ Primary Band Keratopathy SCLAFANI-CORNEA 1 CORNEAL DEGENERATION Corneal Degenerations ➢ Non-familial, late onset From Periphery to Center (arbitrary division) ➢ Asymmetric, unilateral, central or peripheral ➢ Changes to the tissue caused by inflammation, ➢ Arcus senilis ➢ Spheroidal degeneration age, or systemic disease. ➢ Lipid keratopathy ➢ Iron deposition ➢ White limbal girdle of Vogt ➢ Coats’ white ring ➢ Characterized by a deposition of material, a ➢ Senile furrow ➢ Crocodile shagreen thinning of tissue, or vascularization ➢ Terrien’s marginal degeneration ➢ Corneal farinata ➢ Hassall-Henle bodies ➢ Salzmann’s corneal degeneration ➢ Calcific band keratopathy ➢ Corneal keloids ➢ Calcareous degeneration ➢ Corneal amyloid degeneration EPITHELIUM EPITHELIAL BASEMENT ➢ 50 um non-keratinized stratified MEMBRANE “DISORDER” EBMD squamous epithelium ➢ ➢ 5-10 layers central 8-10 peripheral Most common anterior corneal disorder ➢ Superficial layers have microvillae ➢ DYS: inherited, single layer, bilateral that attach tears. ➢ Exfoliation q 5-7 days ➢ DEG: Prevalent 43%, 25% unilat,> 29y,trauma ➢ Deeper layers (Basilar Columnar ➢ Abnormality of epithelial turnover, maturation, cells) have hemi-desmosomes ⚫ connect the epithelium to and production of BM and adhesion complexes basement membrane which connects to Bowman’s Layer. Thickened BM ultimately weakens the epithelium ➢ First phase of wound healing occurs with and causes recurrent corneal erosion (RCE). migration of existing cells over the wound EBMD SLX of EBMD ➢ The basal cells produce abnormal finger-like ➢ Negative NaFL pattern and instantaneous TBUT projections that bend intra- epithelialy and trap ➢ No Rose Bengal Stain cells/debris that form cysts. ➢ When Microcysts surface and erupt , + NaFL ➢ MAPS : multi-lamination of BM and collagen ➢ Asymptomatic vs. Variable degrees of ➢ DOTS: grey opacities,cysts Blur, diplopia, ➢ FINGERPRINT: photophobia, dryness, reduplication of BM FBS, or pain. AD: TGFBI/BIGH3 gene SCLAFANI-CORNEA 2 EBMD Bandage Contact lens Focus Night/Day BC 8.4/13.8 Plano 20/20+ CW 6 pm TREATMENT FOR EBMD ➢ Indicated if vision or comfort are compromised. ➢ Manage co-existing ocular surface disease ➢ Environment/ diet /bedtime eyeshields ➢ Lubricants ➢ Punctal occlusion ➢ Bandage Contact Lens (BCL) ➢ Surgical: PTK TREATMENT FOR EBMD/ABMD to AT Containing Hyaluronic Acid HA Prevent Recurrent Erosion ⚫ Most common in Europe and Asia ➢ Avoid preservatives or surfactants ⚫ Higher concentration of Sodium Hyaluronate ➢ Electrolytes nourish eye ⚫ Strong ability to bind water ➢ DED benefits from Hypo-osmotic drops ⚫ Visco-elastic properties to stabilize tear layer Counteract the high salt contact of dry eyes Vs. ⚫ Predominant glycos-aminoglycan to appear at Bland ointment help retain fluid on the eyes the wound site and aids in cell proliferation, ➢ Edematous Corneas or Weak junctions that migration and ultimate healing lead to RCE benefit from Hyper-osmotic agents ➢ Sodium Chloride vs. High Oncotic Pressure ⚫ High MW HA = cohesive Muro 128: Solution (2-5%) vs. ointment (5%) ⚫ Low MW HA= dispersive Ung: comfort, > concentration Treat 6 weeks Solution /3-6mo ung ➢ Warm Packs: QID 2-3 weeks/shields SCLAFANI-CORNEA 3 Autologous Serum Drops ➢ Utilizes patient’s own blood serum ➢ Blood is drawn and the serum is spun down and mixed with artificial tears. Devoid of cells and clot factors ➢ Replaces “personal” growth factors ➢ Replaces individualized antibodies ➢ Serum contains growth factors, fibronectin, Vit. A and anti-proteases ➢ Requires blood donation 2-3 times year $150-$300 Hospital/Lieters ➢ Consider 5-10% serum albumin drops qid instead ANTI-INFLAMMATORY EFFECTS OF EFA DEWS II ➢ WOMENS HEALTH STUDY ➢ Published 2005 ➢ N > 32K ➢ Association of Low Dietary intake of ω3 and DED ➢ 30% reduction in risk/1g/day ➢ Elevated Risk DED 15:1 when ω6>ω3 ➢ When balance is off, it is associated with lipid abnormalities ➢ Lacrimal gland preventing apoptosis of the secretory epithelial cells 21 22 Caffeine is a non-selective competetive Superficial Punctate Keratitis of adenosine antagonist, that increases the level of acetylocholine, for the parasympatheic path.. Thygeson (SPKT) Acts on lacrimal gland o increase secretion Protective against MRSA ➢ Chronic, usually bilateral disorder characterized by focal epithelial lesions favoring central visual axis ➢ Mean age 29 (2 to 70) ➢ Long duration with remissions and exacerbations ➢ Asymptomatic (esp. later) vs. FBS, epiphora, photophobia ➢ Corneal sensation not effected although occasional hypo- aesthesia… r/o HSV SCLAFANI-CORNEA 4 Lesions are round and show SPKT negative staining ➢ Average of 15-20 lesions ⚫ (1 to 50) ➢ Lesions have +NaFl/RB staining and are elevated during active disease process ➢ Each lesion comprised of multiple lesions ➢ Change position over time ➢ No stromal involvement ➢ Conjunctiva is not inflamed* Conjunctiva: usually not inflamed unless RPS Adeno Detector™ Rapid Pathogen Screen during the developmental stage:1-2 wks Test Procedure Collecting the Sample Dab the sampling pad inside the lower eyelid (palpebral conjunctiva) 4 – 6 times. Allow the sampling pad to rest against the conjunctiva (membrane on inside of the eyelid) for an additional 3 seconds to ensure saturation of the sampling pad with eye fluid. Etiology of SPKT Unknown Treatment for SPKT ➢ Possibly Viral due to latency, recurrence, ➢ Lubricants for comfort lesion appearance, duration ➢ BCL to smooth surface ➢ PCR testing proved that it is NOT HSV 1, HSV 2, VZV or Adenovirus ➢ Good response to steroids however needs long taper and can prolong the ➢ Still investigating HPV since both have minimal inflammation course or worse. Confident DDX… ➢ Prolonged SPKT Associated with Salzmans ➢ Antivirals ? Nodular Degeneration ➢ Cyclosporine ➢ Suggested association with eczema, Reinhard showed 70% suppression with 2% urticaria, asthma ➢ Lack of response to systemic or ➢ HLA-DW3 and DR3 association: topical AB, debridement/ cautery of gluten sensitive, DM2, Lupus, Graves tissue SCLAFANI-CORNEA 5 5 CHARACTERISTICS OF THYGESONS MEESMAN’S DYSTROPHY 1. Chronic, usually bilateral disorder characterized ➢ Diagnosed within first year of life ➢ A “peculiar” substance is by central focal epithelial lesions and no produced which thickens the BM. stromal involvement ➢ Numerous epithelial vesicles that extend to limbus* 2. Long duration with remissions/exacerbations ⚫ Contain debris,cells,GAG 3. Eventual Healing without scars ➢ No scarring. Photophobia. Irritation ➢ May have slight decrease in VA. 4. Lack or response to AB treatment ➢ CLS are not contraindicated and 5. Striking response to steroid TX may be therapeutic when rupturing ➢ LISCH : whorl-shaped clusters MEESMAN’S DYSTROPHY RECURRENT CORNEAL EROSION ➢ Traumatic erosions due to thickened BM with poor hemi-desmosomal attachments. ➢ May result from incomplete healing following trauma ➢ Associated with EBMD CL INDUCED (50%) or Stromal MICROCYSTS Dystrophies esp.Lattice RECURRENT CORNEAL EROSION Treatment for RCE ➢ Onset in the am due to ➢ ACTIVE edema or shearing effects ➢ Aggressive corneal abrasion TX = BCL heals slower ➢ Symptoms may be more ➢ PROPHYLAXIS severe than it appears ⚫ Lubricants/ Hyperosmotic agents ➢ Epithelial loss surrounded by pooling and loose ends ⚫ Long-term BCL, Amniotic Tissue, MMP inhibitors ➢ SURGICAL ➢ Debridement ➢ Anterior Stromal Puncture ➢ PTK with PRK SCLAFANI-CORNEA 6 TREATMENT OF RCE