GOALS CORNEAL DYSTROPHIES AND DEGENERATIONS:  Differentiate dystrophy vs. degeneration DIAGNOSIS AND TREATMENT  Normal vs. abnormal  Classify the disease by location  Layers of the Louise A. Sclafani, OD, FAAO  Central vs. peripheral AAO Diplomate, Cornea & Contact  Determine appropriate treatment Associate Professor of University of Chicago Medical Center

Review the Layers of the Cornea  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

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 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, , , 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 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)  : 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  • 35% - 40% of all acute Long duration with remissions Steriods – may pose risk in misdiagnosis • 80% - 90% of viral and exacerbations

 “If antibiotics are not effective, it  CPT Code 87809  Asymptomatic (esp. later) vs. FBS, , must be viral.”  Cost $13 Reimburse $17 photophobia  Treat the symptoms

SCLAFANI-CORNEA 5 Treatment for SPKT MEESMAN’S DYSTROPHY

 Lubricants for comfort  Diagnosed within first year of life  BCL to smooth surface  A “peculiar” substance is produced which thickens BM  Lack of response to systemic or topical AB,  Numerous epithelial vesicles that extend to limbus* debridement/ cautery of tissue  Contain debris,cells,GAG  Good response to steroids however long taper and can  No scarring. Photophobia. Irritation prolong the course or worse  May have slight decrease in VA.  Antivirals ?  CLS are not contraindicated and may be therapeutic  Cyclosporine when rupturing  Reinhard showed 70% suppression with 2%  LISCH : whorl-shaped clusters

RECURRENT CORNEAL EROSION Treatment for RCE  Traumatic erosions  Onset in the am due to  due to thickened BM edema or shearing  Prophylaxis with lubricants/hyperosmotic with poor hemi- effects agents/BCL desmosomal  Symptoms may be more  Treat like a corneal abrasion: heals slower severe than it appears attachments.   Epithelial loss Debridement  May result from surrounded by pooling  Anterior Stromal Puncture incomplete healing and loose ends  PTK with PRK following trauma  ProphylaxisTreatment:  Associated with lubricants/ plugs/BCL EBMD (50%) or Lattice Dystrophy

BANDAGE CONTACT LENSES INDICATIONS FOR BCLS

 To aid in healing by offering protection  ACUTE  CHRONIC  To provide comfort for decompensating corneas with  Traumatic abrasion  Severe dry eye erupting microcysts  Following FB removal  Bells Palsy exposure  To aid in dehydration  RCE  Cicatrical disease  Chemical burns  Nocturnal lag  To produce a more regular refracting surface  Thermal Burns  Conjunctival elevations  To aid in drug delivery  Shield Ulcer that reduce wetting  To reduce inflammation  Whorl Keratopathy

SCLAFANI-CORNEA 6 INDICATIONS FOR BCLS CONTRAINDICATIONS FOR BCL

 SURGICAL RESULT  DISEASE  Non-compliant patient   Thygeson’s Retinal surgery causes  Poor Hygiene epithelial defects  Salzmans

   PRK Granular Dystrophy Socio-economic  Lattice Dystrophy  PTK  High risk for infection  EBMD  Extrusion of Intacs   Bullous Non-consent  Irregular surface from filtering blebs  Band Keratopathy  Piggyback  RGP induce abrasions for ectasias

Faster Recovery with BCL GOALS IN FITTING BCL  HIGH Water = provides    Donnenfeld reported in A.Ophthalmology 1997  CL should have smooth mechanism for surface  Compared patients treated with: dehydration and slower   Pressure patch /AB vs. BCL vs. BCL/Topical NSAID  Minimal ET drug release.  No difference in re-epithelialization time  Wettability  LOW Water = when   Psychometric Analysis: patients prefer BCL/NSAID  High dK evaporation is not  Return to normal activities in1.37 days  High modulus when lid desired.  edema is present  Soak lens in ANTIBIOTIC  Minimal movement to   Full coverage, minimal Caution with preservative toxicity, especially BZK avoid rupture of hemi- movement  Other options: Collagen Shields desmosomal bonds. Complete coverage.  Disposables / EW/low ET

FDA Approved vs. STD of CARE INFECTION PROPHYLAXIS Erthromycin ung or  Cooper Vision Permalens  Acuvue Therapeutic Bacitracin ung q 2-4h or  58% H20 dK 28  71% H20 dK 34   Polytrim gtt +/- qid  Bausch & Lomb Plano-T Acuvue Advance/Oasys AND  38% H20, dK 9.2  47% H20 dK 60/103   CIBA Focus Night &Day  B&L Soflens Tobramycin +/- qid  24% H20, dK 140 OR  66% H20 dK 32  CIBA CSI-FW   Cooper ProCLear Ciloxan/ Ocuflox qid  38% H20, dK 13  Zymar/ Vigamox qid  B & L Purevision Compatibles  36% H20 dK 101  62% H20 dK 34  Submerge BCL  DOSAGE & TOXICITY

SCLAFANI-CORNEA 7 PAIN MANAGEMENT FOLLOW-UP CARE FOR BCL

 ORAL MEDS  24 Hours  5 % Homatropine  Motrin 400-600 mg AND (no PG)  May note 25-50% improved  1-2 % Cyclopentolate  Acetominophen 500-1000 mg (no  If improvement q 2-5 days  Scopalamine ETOH ETOH   Monitor high risk patients daily BID  Utram (non-narcotic) 50mg TID  CL wearers  NSAIDS  Lortab (Schdule III narcotic)   Decreases pain by 40% Hydrocodone with acetaminophen HSV, immuno-compr, DM  Acular PF (Keterolac) 2.5/500, 5/500, 7.5/500  Monocular, children  Voltaren (Diclofenac)  Central or Large abrasion  Ocufen  LUBRICATION  Do not remove BCL too early-  Nevanac(Nepafenac)  Non-preserved AT wait 5-7 days until after it appears  Xibrom  AT w/ Hyaluronic acid  to be resolved- late phase healing Q3-4 h D/C after 48 hours to • Blink, Aquify prevent 20% healing time  • RX 5% BID for AC rxn If condition worsens or no improvement, consider referral for tarsorrhaphy or conjunctival flap

Nat'l Abrasion Patching Study Group

ANTERIOR STROMA MICROPUNCTURE CORNEAL DEBRIDEMENT

 Disturb Bowman’s Layer to promote tighter adhesion  Soften epithelium and stimulate cornea to produce functional BM 1-2 gtt topical anesthetic complexes complexes q 15-30 seconds for 2-3 minutes  Topical anesthetic and a 27g cannula: use forceps to  Use cotton swab, spatula, spud bend needle to avoid puncture or jewelers forceps  Closely spaced (.5mm)  Remove flaps by pulling edges toward center punctures damaged/adjacent  Don’t pull directly up or out  Anterior Stroma :100-150 u   Apply tangential force Remove flaps down to tight,  Avoid Visual axis since minimal scarring can occur firm edges.  RR 40%  Tx abrasion (>50-100%)  Recurrence Rate 18%

PREVENTION OF RCE Alodox

 Patients with RCE show a chronic increased level of  20 mg Doxycyline Hyclate metallo-proteinase enzymes (MMP 2&9) which may dissolve the basement membrane and fibrils due to  Sub-antimicrobial dosage inadequate neutralization.  (<50mg)  Treatment is to inhibit metallo-proteinases  Enzyme modulation of inflammation  Doxycycline: oral, 50mg BID   2 months treatment time. Reduced MMP 70%. By OCuSOFT  Topical Steroids  Kit comes with lid scrub foam  Pred Forte, FML, Lotemax, TID,2-3 weeks   No recurrences in 21 months. Claims to be a more potent  Dursan and Plugfelder. “Treatment of Recalcitrant RCE with inhibitors of matrix metalloproteinase –9.” American Journal of Ophthal. 2001,132:8-13 collagenase inhibitor than minocycline and therefore less SE  Long term use

SCLAFANI-CORNEA 8 Bowman’s Layer REIS-BUCKLER DYSTROPHY

 Acellular modified layer of anterior stroma  Bilateral, symmetric, AD, by age 5  Type 1 collagen fibers randomly arranged  Bowman’s layer is obliterated and replaced with  Pores for corneal nerves to pass randomly arranged  Fxn? Not found in many species with good vision and regular collagen that thickens. normal epithelial-stroma junctions.  Linear, ring-like or “Honey comb”  Not replaced and when damaged, causes significant  Marked VA loss due to superficial stromal haze or opacification which effects VA topographical changes from elevation of tissue  Painful if recurrent erosions occur.  TX: PKP or LK around age 50 but may recur in grafts

ANTERIOR MOSAIC BAND KERATOPATHY DEGENERATION

 Dystrophy or Degeneration  Deposition of Calcium salts in Bowman’s layer  AKA: Anterior Crocodile Shagreen  Located interpalpebral region  Breaks in Bowman’s that appear like central grey  History of , renal failure, prolonged use of polygonal opacities with clear spaces. miotics, syphillis, interstitial  Blanches with limbal pressure. keratitis,hyperparathyroidism  Asymptomatic  TX: Chelation with EDTA 1%  TX: Therapeutic CL

Treatment: Cosmetic Contact Lens SALZMANN’S NODULAR DEGENERATION

 Black Underprint: color is applied to a dark  Bluish, superficial nodular elevations background to mask and make a scar more  Inflammatory/Non-inflammatory event that exposes the uniform This darkens and mutes the overlaid cornea and results in excess COLLAGEN plaques that color. replace Bowmans  Post-chronic-keratitis  Store in glass vials  Asymptomatic to very painful and sight threatening   53% H20 to maintain dye depending on location and severity  TX: BCL/AB/NSAID, PTK,PKP

SCLAFANI-CORNEA 9 STROMAL DYSTROPHYS Name of Dystrophy Name of Deposition  90 % of corneal thickness Pathology Stain  22%: Comprised of collagenous lamellae (type 1) interspersed with keratocytes and ground substance(proteoglycans, glycoproteins, serum)  Marilyn Monroe Always  GAGS: affect hydration, thickness, transparency  Gets Her Man  78%: rest is water.  Los Angeles County  Abnormal Substance found within the cells or  Southern California Ocean fibrils that have distinct histological-stains S C O

MACULAR DYSTROPHY GRANULAR DYSTROPHY

 Clouding due to build-up of mucopolysaccharides  Central, anterior to mid-stromal deposits of Hyaline  Begins centrally & superficially then extends limbus to limbus  AD thru all layers   Thinning without clear spaces Discreet white spots (translucent) to powdery rings  Primary involvement of the endothelium: guttata*  Clear areas between lesions in early years  Begins in 1st decade of life: aggressive causing early & severe  Erosions can break thru BM. VA loss  Autosomal dominant w/ complete penetrance*  Predominant in Virginia area  Granular / Hyaline/ Masson Trichrome  Autosomal recessive*  TX: PKP  Macular / Mucopolysaccharide / Alcian Blue stain

Granular Dystrophy GRANULAR DYSTROPHY TREATMENT  Pinhole effect may maintain VA (20/20) until the lesions 70 YO AAF 25 YO WF coalesce and reduce VA=20/200.  1992 VA 20/50  C/O /haze  PKP was only treatment and recurrences were  1997: 20/80 & RCE  RE +7.50 – 2.00 x 010 20/30 common  PKP vs. PTK  LE +.25 – 2.25 x 170 20/30  SRX pre: +1.00  CL FIT  Present treatment includes PTK and BCL: K: 42.00/41.00  DIL +3.50 8.08/11.2 20/25 +  Smooth epithelial surface to treat monocular diplopia  SP 2 mo: +8.75  PV pl -1.75 x 180  Pain management following PTK or erosions  Refit OD at 4 months pg K: 36.75/ 37.75 Refit OD at 4 months pg  Induced anisometropia  Hydrasoft Options  SP 6 mo: +6.50  Spectacle distortions of high plus lens  +8.75 -2.00 x 010 20/25 !!!  TX: Acuvue +7.00

SCLAFANI-CORNEA 10 LATTICE DYSTROPHY TYPES OF LATTICE DYSTROPHY

 Branching refractile filaments  TYPE 1 of AMYLOID Poor VA by age 40-60  Symptoms occur early in life, age 20-30, AD  TYPE 2  RCE are common Merotoja syndrome  Resultant scars and late intervening haze can blur VA Bilateral Facial palsy, skin thickens,  Lines thicken with age & penetrate deeper layers Depressed brows, prominent  Autosomal Dom/ Recessive VA loss >65y  TX: PKP  TYPE 3  Lattice / Amyloid / Congo Red Floppy ears, protruding lips, Auto-R Lger deposits, mid stroma, no RCE VA loss > 60 y

CENTRAL CRYSTALLINE AVELLINO DYSTROPHY DYSTROPHY OF SNYDER  Avellino, Italy  Deposits of crystals in anterior stroma  Premature peripheral arcus  Typical granular dystrophy with axial anterior Premature peripheral arcus  Vision is generally good stromal haze and mid-stroma discreet linear  Usually normal serum lipid profile: +/- opacities.  Expressivity is variable  Congo red  B120 gene on chromosome 1 is responsible for lipid metabolism and transport  Snyder / Cholesterol /Oil

POSTERIOR AMORPHOUS DYSTROPHY WHITE LIMBAL GIRDLE OF VOGT

 Rare, autosomal dominant  Effects > 50% population between 40-60  Gray opacities in the posterior stroma that may extend  With/Without clear zone to the limbus, central corneal thinning  Represents subepithelial degeneration and sometimes   Flattening of the curvature and induced hyperopia calcium deposition  Prominent Schwalbes line  Does not affect visual acuity  Pathology shows focal areas of endothelial disruption  Located in the horizontal meridian  Slow progression and may not threaten vision

SCLAFANI-CORNEA 11 POSTERIOR EMBRYOTOXIN ARCUS SENILIS

 Extremely prominent ring of Schwalbe  Effects >60% population between 40-60 years  Peripheral lipid deposition  Eye is normal but may be associated with Peripheral lipid deposition  Located anterior to Descemet’s layer and in Bowman’s correctopia, , or corneal conditions that layer are part of systemic syndromes  Juvenile form usually represents hyper-lipidemia  Be suspicious of carotid disease if this is present to a greater degree in one eye.

FURROW DEGENERATION DELLEN

 Peripheral thinning in the elderly  Peripheral 50% thinning of one or more layers  Lucid interval of Arcus  Runs along the limbus parallel to area of swelling  No inflammation  Limbal elevation causes dryness which leads to  Vision unaffected further excavation  CL, lid disease, OSD

TERRIENS MARGINAL DEGENERATION Peripheral Ulcerative Keratitis

 INTACT epithelium with progressive thinning  A painful, chronic, crescent-shaped peripheral  Non-inflammatory ulcer  Supero-nasal location  Adjacent epithelial defect  Attacks young men (3:1)  Produces AR or oblique seen and stromal infiltrate on topography  Progresses circumferentially forming an  Treat irregular astigmatism with RGPs overhanging edge  Adjacent conj. and are erythematous and inflammed.

SCLAFANI-CORNEA 12 RHEUMATOID ARTHRITIS ALTERNATIVES TO BCL

 Marginal Furrows  TARSORRHAPHY   Surgically close the palpebral fisssure by suturing the Usually Bilateral superior and inferior lids at the lateral aspect  Due to decreased tear production  STAMLER LID SPLINT  Limbal vascular compromise  Adhesive on one side with enough rigidity on the other to hold the in the closed position  TX: Lubricants, BCL, Tarsorraphy  Allow for use of meds and examination  Alternative treatments

® ANTI-INFLAMMATORY THERAPY Restasis® Allergan ANTI-INFLAMMATORY THERAPY (Cyclosporine Ophthalmic Emulsion 0.05%)  CYCLOSPORINE  Indicated for patients who do OFF LABEL not get relief with USES  .05% and .1% compresses and lubricants compresses and lubricants  EBMD  Restores tear production  Reduction in artificial tear use  Thygesons Dx  Increases goblet cells  Increase in goblet cell density   Excellent safety profile Excellent safety profile  Refractive Surgery  Decrease in corneal staining  BID dosing BID dosing  Graft vs.Host in BMT  Mild stinging  Improved Schirmer test scores Mild stinging  Viral  Improved visual  Chronic Uveitis

MOOREN’S ULCER DEGENERATION Mechanism of Ulceration Process

 Peripheral Ulcerative Keratitis PUK  trauma or infxn or systemic disease  Idiopathic or related to  Nl Corneal Antigens Altered Corneal antigens autoimmune disease, trauma, surgery.  Association to Crohn’s disease and Hepatitis C  corneal melting cellular and  Symptoms are mild* to severe  humoral  neutrophil immune rxn  degranulation or  keratocyte collagenase complement  stimulation activation

SCLAFANI-CORNEA 13 Treatment of Mooren’s Ulcer Peripheral Ulcerative Keratitis Work-up

 Control of underlying systemic disease  Investigation for Mooren’s-like Ulcer  Anti- collagenolytic – Topical or oral tetracycline  Thorough Medical Hx  Topical steroids & Oral Steroids- 60-100 mg/day  Corneal Cultures  With cycloplegia / topical antibiotic / BCL  CBC w/ diff, platelet count, ESR, RF,ANA,  Topical or Systemic Immuno-suppressives ANCA, circulating immune complexes, LFT’s,  Topical Cyclosporin 1% QID or 2% BID/ Methotrexate VDRL and FTA-ABS, BUN and creatinine, serum  Conjunctival resection protein electrophoresis, urinalysis, CXR  Lamellar Keratoplasty: Doughnut shaped with donor  Additional testing based on ROS and physical sclera, conjunctiva and peripheral cornea. exam  Healing rate of 95.6% Recurrence Rate of 25.6%

SUMMARY OF SURGICAL OPTIONS Stem Cell Deficiency

 Penetrating keratoplasty PKP  Defects in renewal and  SYMPTOMS: Full thickness exchange repair causes invasion of  Blur conjunctival epithelial   Lamellar keratoplasty LKP Photophobia cells onto the cornea  Pain Exchange epithelium/partial stroma  SIGNS:  Tearing Less risk for rejection, ,  Dull corneal reflex  Blepharospasm or .  Ingrowth of thickened  recurrent epithelial  Doughnut shaped:replace sclera and limbal stem cells fibrovascular pannus breakdown in the case of peripheral marginal disease.  Keratitis  chronic inflammation w/  Epikeratophakia  Scarring Donor lenticule to flatten cornea  Calcification

FUCH’s SUPERFICIAL FUCH’s SUPERFICIAL MARGINAL KERATITIS MARGINAL KERATITIS  Affects middle aged adults Chronic disease leads to  Histopathologic studies shown corneas to be thinned  It is characterized by periods progressive 20-25% in the periphery. of relapses and remissions of irritation and redness. circumferential peripheral  Inflammatory cells in the cornea consisted of mostly  Begins as superficial corneal thinning with lymphocytes and PMN but also mast cells & basophils. marginal keratitis that vascularized  BV leaking lipids advances non-uniformly pseudopterygia growing sparing the central cornea.  These studies suggest no clear cut etiology of the over these areas. These studies suggest no clear cut etiology of the  Advancing keratitis is disease. demarcated from the central cornea by a gray line.  Active keratitis is accompanied by stromal infiltrates

SCLAFANI-CORNEA 14 TREATMENT FUCH’s SUPERFICIAL Normal Changes to the Endothelium MARGINAL KERATITIS  A.T., topical steroids, and topical antibiotics during  Descemet’s layer thickens from 3-17u acute exacerbations  There is a decrease in the # of endothelial cells  Topical Cyclosporine 1% BID There is a decrease in the # of endothelial cells 2  Fit RGP contact lenses (Improve Vision)  from 3500 cells/mm to 1200  Lamellar Keratoplasty (reports of recurrence in the  this single layer spreads out: lacks mitosis graft)  High density mitochondria : 90% pump  Combined superficial keratectomy with a conjunctival  Lenses produce reversible polymegathism autograft (Kotecha and Raber) Method used to retard recurrent psuedopterygium formation

Abnormal Changes to the Endothelium FUCH’S DYSTROPHY

 Endothelial cells become  Bilateral, asymmetric, Guttata represent clear, more irregular begins in 5th or 6th vesicular endothelial decade secretions that project  Cells secrete collagen  More predominant in into the potential space towards Descemet’s causing multilamination = guttata More predominant in women (3x) between the  This breaks down the barrier function and results in endothelium and  Initially pigment dusting stromal and epithelial edema Descemet’s l  Non-symptomatic

FUCH’S DYTROPHY STAGE 2 FUCH’S DYTROPHY STAGE 3

 Guttata interrupt the normal pumping mechanism =  Edema is reduced but subepithelial connective edema tissue grows and causes reduced vision.  Edema begins around Descemet’s and Bowman’s  Patient is comfortable due to reduced corneal layers and then spreads the entire thickness. sensitivity.  Pts experience glare/hazy VA  Elevated IOP, peripheral neovascularization,  Bullae appear: they reduce vision and cause pain and corneal erosions. when they rupture, especially in am

SCLAFANI-CORNEA 15 FUCH’S DYSTROPHY TREATMENT DSEK: Descemet Stripping Endothelial Keratoplasty

Faster visual recovery  Hypertonic solutions to draw fluid out Less astigmatism created since there are no sutures  Sodium Chloride Eye is much stronger and more resistant to injury since  Muro 128 (2% or 5%) solution, 5% ointment-PF only the diseased tissue rather  Fresh Kote than the entire cornea is replaced  BCL to aid in comfort for ruptured bullae Surgery time is quicker Chance of rejection is reduced  Lubricants for comfort Procedure can be combined with surgery  Lower IOP VA 20/30-20/40  Conjunctival flap 1-2 D Hyperopic Shift, thicker  Corneal transplant to restore vision/ DSEK

POSTERIOR POLYMORPHOUS POSTERIOR POLYMORPHOUS DYSTROPHY- PPMD DYSTROPHY- PPMD  Isolated to coalescent vesicles that intervene between  Can be present at birth normal endothelial cells.  Wide variety of expression  Areas of normal or thickened Descemet’s membrane  Non-symptomatic representing a collagenase material representing a collagenase material  Grouped vesicles cause blur  These vesicles can lead to stromal edema.  Stromal edema  Association with  Correctopia and irido-corneal adhesions resulting in glaucoma if they enter TM

Visante Applications Anterior Segment Imaging and Surgery

 Refractive Surgery Corneal Imaging and Measurement  Corneal laser refractive surgery: pre-op, enhancement  imaging and evaluation of corneal pathologies options  penetrating keratoplasty  Phakic IOLs   Corneal refractive implants: Intacs  lamellar keratoplasty  Anterior Segment Imaging and Surgery  endothelial keratoplasty  Corneal Imaging and Measurement  keratoconus imaging and assessment  Imaging and Evaluation Iris Imaging and Evaluation  anterior segment imaging through opaque corneas  Trauma Assessment

SCLAFANI-CORNEA 16 KERATOCONUS Keratoconus- Keratometry

 Initially, mires get small and then there is a “Keratoconus is a clinical lack of parallelism term to describe a  condition in which the Expand perimeters by use of +1.25 SPH and cornea assumes a add 7 D to your reading conical shape because conical shape because  of thinning and Steepening begins infero-temporally and protrusion” progresses clockwise  TOPOGRAPHY- more sensitive  PLACIDO RINGS- get closer

RETINOSCOPY PSEUDOKERATOCONUS

 Scissors Reflex  Against motion that  Corneal warpage topography can mimic KC breaks apart   Represents multiple Repeat topography must be performed and a refractive powers within measurable change would indicate pseudo-KC the optic zone  Evaluation of elevation maps at steep zone:  Predicts the elevation or depression of the cornea if the best fit sphere was on cornea

KERATOCONUS- FINDINGS PELLUCID MARGINAL DEGENERATION

 FLEISCHER RING  20-40yo, no gender preference, slow  VOGT’S STRIAE progression  STROMAL THINNING  Thinning occurs below the steep curvature  STROMAL SCARS  Stromal thinning is concentric to the lower limbus  SWIRL-LIKE PATTERN and runs from 4-8:00, 1-2mm wide  ENLARGED CORNEAL  Clear, epithelialized, and non-vascularized. NERVES  Absence of lipid: ddx from Moorens or Terriens   ACUTE HYDROPS  Vertical stress lines and hydrops can occur  BEER- BELLY CORNEA-

SCLAFANI-CORNEA 17 PROGNOSIS FOR PELLUCID

 Lens fitting is difficult due to inferior apex  A diffuse thinning of the cornea to 1/3-1/5 the normal thickness  Central rings show AR/ Inferior rings show WR  It is noted early in life and progression is minimal  Fitting flat causes bearing and on K (steep)  Associated with Ehlers-Danlos Syndrome and Leber’s causes too much seal off Congenital Amaurosis  Larger lenses needed due to low positioning/  Acute hydrops glare  CAREFUL MONITORING-  Poor SX Candidate

POSTERIOR KERATOCONUS POSTERIOR KERATOCONUS

 Rare developmental defect  Associated Ocular Disease  Focal indentations of the posterior cornea with  Lens abnormalities, choroidal or retinal sclerosis, PPMD, overlying stromal scarring retinal coloboma, hypoplasia, , iron rings, and posterior ,  Anterior curve not effected  Systemic Associations  Descemets’s membrane and endothelium are always  Mental retardation, webbed neck, hypertelerism, short present but may be abnormal in the area of thinning stature, superior placed lateral canthi, genitourinary abnormalilites

Thank you

Louise Sclafani, OD, FAAO [email protected] 773-702-6953

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