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 • 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, epiphora, 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
CYCLOPLEGIA 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 uveitis, 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 anisometropia/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 cholesterol 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: +/- hyperlipidemia 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, aniridia, 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 astigmatism seen and stromal infiltrate on topography Progresses circumferentially forming an Treat irregular astigmatism with RGPs overhanging edge Adjacent conj. and sclera 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 eyelid 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 Blepharitis 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 Conjunctivitis 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, glaucoma, Dull corneal reflex Blepharospasm or endophthalmitis. 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/ red eye 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 cataract 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 keratoconus 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 Iris 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-SLIT LAMP 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 KERATOGLOBUS
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, optic nerve hypoplasia, ptosis, iron rings, and posterior synechia, 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
SCLAFANI-CORNEA 18