5/31/21

DISCLOSURES ENDOTHELIOPATHIES

NOPE, THAT’S NOT JUST DRY EYE: PRIMARY SECONDARY OTHER CORNEAL DISEASES • Corneal guttata • Contact wear • Fuchs dystrophy • Surgical procedures

• Posterior Polymorphous Dystrophy (PPD) • Age related

Cecelia Koetting, OD FAAO • Congenital hereditary endothelial dystrophy • Iatrogenic (im munodeficiency) (CHED) • induced Virginia Eye Consultants • Iridocorneal endothelial syndrome (ICE) • Ocular inflammation Norfolk, VA

1 2 3

OTHER CORNEAL CORNEAL FUNCTION

• • Central cloudy dystrophy of Francois • Pellucid marginal degeneration • Thiel-Behnke • Shields the eye from germs, dust, other harmful matter • Lattice Dystrophy • Ocular Bullous pemphigoid WHY IS THE CORNEA IMPORTANT? • Contributes between 65-75% refracting power to the eye • Recurrent corneal erosion (RCE) • SJS • Filters out some of the most harmful UV wavelengths • Granular corneal dystrophy • • Reis-Bucklers corneal dystrophy • • Schnyder corneal dystrophy • HSV/HZO • Congenital Stromal corneal dystrophy • • Fleck corneal dystrophy • Burns/Scars • Macular corneal dystrophy • Perforations • Posterior amorphous corneal dystrophy • Vascularized cornea

4 5 6

CORNEAL ANATOMY CORNEA

Epithelium Bowmans Layer • Cornea is a transparent, avascular structure consisting of 6 layers • A- Anterior Epithelium: non-keratinized stratified squamous epithelium; cells migrate from BRIEF ANATOMY REVIEW Stroma basal layer upward and periphery to center • B- Bowmans Membrane: condensed stroma- structural • C- Stroma: connective tissue layer, type 1 collagen. Keratocytes (fibroblasts); anterior compact, posterior is spongy Endotheilum • D- Descemet’s Membrane: barrier Descemets • D- Duas Layer Layer • E- Endothelium: actively pumps water and ions from stroma to produce corneal Dua’s Membrane dehydration and keep transparency. These cells do not regenerate if damage and corneal decompensation will result (white and cloudy)

7 8 9

1 5/31/21

EPITHELIUM EBMD

EPITHELIAL BASEMENT MEMBRANE • 5-7 cells thick • Abnormal production of epithelial basement membrane • Approximately 50 um • Extends to epithelium

DYSTROPHY • Peripheral cornea continuous with and • Causes multiple basement membrane layers • 0.5-1mm band of limbal cells at corneoscleral junction • Trapped epithelial cells form Cogan microcyts • Degenerative AKA ABMD, Map Dot dystrophy, Fingerprint Dystrophy or • Occasionally autosomal dominant Cogan Microcycstic dystrophy • Tran fo rm in g growth factor beta-induced gene (TGFBI) on chromosome 5q31

10 11 12

SYMPTOMS CLINICAL FINDINGS TREATMENT

• Commonly asymptomatic • Corneal staining, NaFl • Controlling ongoing ocular surface disease • Recurrent corneal errosions • Map like lines • Decrease the inflammation and MMP9

• Distortion to vision • Microcycts • Muro 128 or Sodium Chloride solution or ointment • • Monocular shadows or Finger print like epithelial lesions • Freshkote • Increased halos or glare • Retroillumination can help to highlight these findings • Surgical • Lamellar Keratoplasty

• Debridement of epithelium with a diamond burr polishing or excimer laser phototherapeutic keratectomy (PTK)

13 14 15

RECURRENT CORNEAL EROSION RCE TREATMENT

• Typically triggered by an injury to the cornea • Lubrication SALZMANNS NODULAR • Most common fingernail • Hypertonic drops and ointment • Poor healing causes weak links between the hemidesmosomes in the basement • Pressure patching DEGENERATION membrane • Topical corticosteroids

• Corneal swelling occurs during sleep 2/2 decreased oxygen exchange with the cornea • Oral doxycycline • Swelling causes the poorly bound tissue to break and results in an epithelial defect or • Bandage soft contact lens area of localized loose epithelium • Anterior stomal micropuncture • Epithelial debridement or Lam-K • With diamond burr or PTK

16 17 18

2 5/31/21

ETIOLOGY SYMPTOMS PRESENTATION

• Women>Men • Often asymptomatic • Single or multiple white, grayish or bluish elevated nodules anywhere on the • History of chronic keratopathy • May affect vtision if paracentral or central cornea surface

• Interstitial • Foreign body sensation • Longstanding nodules may have iron pigment deposits at base • Vernal

• Keratoconjunctivitis sicca • Phlyctenulosis

• Trach o m a

19 20 21

TREATMENT ETIOLOGY

• Liberal lubrication • Chronic ocular inflammation • Familial • Control dryness and inflammatory BAND KERATOPATHY • Silicone oil in the eye • Idiopathic • Superficial keratectomy with blade or PTK • Hypercalcemia or hyperphosphatemia • Chronic exposure to toxic vapor or

• To p ic a l m ito m y c in C • substances • Lamellar keratoplasty • Chronic renal failure

22 23 24

SIGNS AND SYMPTOMS TREATMENT

• Central vision can be effected • Mild cases • Severe cases • Epithelial defects • Observation • Chelation with disodium ethylenedi- aminetetraacetic acid #% FUCHS DYSTROPHY • Peripheral interpalpebral plaque of calcium deposit • Lubricants • • Treat underlying cause Superficial kereatectomy with excimer laser • Begins nasal and temporal extending centrally PTK or blade

25 26 27

3 5/31/21

FUCHS SYMPTOMS TREATMENT

• Wo me n >me n • Asymptomatic in early or mild cases • Hair dryer • Inheritance Autosomal dominant, can be recessive • Decreased and poor quality of vision increases with severity • Hypertonic saline drops during day and ointment at night • Symptomatic after 5th or 6th decade • Significant decrease with edema • NaCl, Muro 128 • Corneal guttata that progress to cause corneal edema • Guttata • Worse vision upon waking in the morning improving over several hours • DSEK • Tiny central excrescences of a thickened Descemet membrane • Servere if bullae develop and rupture • DMEK • Specular microscopy • Penetrating keratoplasty (PK) • Polymegethism • Pleomorphism

• Dark areas

• Reduced number of endothelial cell

28 29 30

ROLE OF LIMBAL STEM CELLS

• Regenerate the entire corneal epithelium • The limbal stem cells also helps to prevent the conjunctival epithelial cells from migrating onto the corneal surface LIMBAL STEM CELL DEFICIENCY • Produces the basal cell layer of the epithelium • Then basal cells migrate toward the center of the cornea

• As move toward center also move up to become wing cells and eventually upwards to become surface cells

• Then shed into the tear film • Turnover of the epithelium cells is approximately 7 days

31 32 33

LIMBAL STEM CELL DEFICIENCY LSCD CAUSES

The Limbus Cornea • When limbal stem cells begin to struggle and poorly function, the epithelial • Acquired Conjunctiva cell layer and its reproduction becomes compromised • Trau m a • Ocular surgeries • Loss or deficiency of stem cells in the limbus which are vital for re-population of the corneal epithelium and to the barrier function of the limbus • Chemical injury • Radiation • Once limbal stem cells are damaged the epithelium will be replaced by conjunctival goblet cells • Contact lens • Mitomycin C, glaucoma drops, preservative sensitivity Mature Cornea Epithelium • Thermal injury • Inflammatory Stem Cells • Autoimmune

34 35 36

4 5/31/21

INFLAMMATORY CAUSES AUTOIMMUNE CAUSES LSCD CAUSES

• DED • Sjogrens Syndrome • Congenital • Sjogrens Syndrome • • Rosacea/MGD • Stevens Johnson syndrome • Allergic • Mucous membrane pemphigoid • Autoimmune Polyglandular Syndrome • Vernal keratoconjunctivitis • Keratitis, Icthyosis, and Deafness Syndrome • Atopic Disease • Chronic limbitis • Bullous Keratopathy

• Neurotrophic keratopathy from trigeminal neuralgia • Diabetes mellitus

• Herpes simplex/Herpes Zoster

37 38 39

SIGNS AND SYMPTOMS SIGNS AND SYMPTOMS SEVERE LSCD

• Signs • Stippled superficial punctate late flourescein staining

• Varying degree of ocular signs depending on severity and level of corneal • Stained cells are more elongated and pill shaped than in SPK from DED • Conjunctivalization conjunctivalization • Early LSCD staining more concentrated peripheral near lim bal area • Corneal surface stains abnormally because the conjunctival epithelium is more • permeable to the stain than true corneal epithelium Symptoms • More progressed LSCD staining spread central and becomes more diffuse • Decreased vision • Natural migration of epithelial cells • More prone to recurrent or non-healing epithelial defects • • Whorl like pattern staining • Stromal scarring or melting • • Te a r in g • Ingrowth of opaque epithelium Expect more pain and vision loss

• Blepharospasm • Superficial neovascularization

• Recurrent pain

40 41 42

IS IT LSCD OR DED? NON-SURGICAL TREATMENT NON-SURGICAL TREATMENT

• Treating underlying systemic causes • Autoimmune control • Chronic DED can lead to or exacerbate LSCD • Remove traumatic or toxic insults that may be the cause • Improve tear film and control inflammation • Not mutually exclusive • Discontinue contact lens wear • Vitamin A ointment QHS

• Possible refit in scleral • To p ic a l s te ro id s • Bandage CL? • Compounded Preservative Free option

• Discontinue or switch topical medications • To p ic a l c y c lo s p o r in e • Glaucoma medications • Preservative free AT

• Preservative sensitivity • Punctal Plugs

• BAK

43 44 45

5 5/31/21

Non-Surgical Treatment THANK YOU!

• Amniotic membrane • Dehydrated vs cryopreserved QUESTIONS? • Amniotic membrane drops

• Can be costly and not covered by insurance currently

• Serum Tears

• Can be costly and inconvenient

• Oxervate

• Neurotrophic keratitis

46 47 48

6