Covering the Cornea from A(BMD) to Z(oster) Justin Schweitzer OD, FAAO Vance Thompson Vision Sioux Falls, South Dakota

Transcription

1 Covering the Cornea from A(BMD) to Z(oster) Justin Schweitzer OD, FAAO Vance Thompson Vision Sioux Falls, South Dakota

2 Glaukos Bausch and Lomb Alcon Allergan Bio- Tissue Reichert Tearscience

3 Corneal Dystrophies Bilateral Genetically Determined Classified by the corneal layer they affect Not caused by inflammation or neovascularization Usually not associated with systemic disease Molecular Science is questioning traditional thought

4 Epithelial Basement Membrane Dystrophy Equivalent to ABMD, Map- Dot- Line, & Cogans Autosomal Dominant Could be an age- related degeneration?? - prevalence increases with age

5 EBMD Symptoms: FB sensation Blurred vision from irregular astigmatism Pain secondary to RCE s - 10% of EBMD patients experience RCE s - 50% of patients w/ RCE s have ABMD

6 Clinical findings: Chalky patches Intraepithelial microcysts Fine lines typically in the central 2/3 rd of the cornea Combination of any of the above (maps, dots or fingerprints)

7 Goal of Treatment in EBMD

8 Treatment of RCE Initial conservative approach Hyperosmotics (ointment or solution) Bandage contact lenses Lubricants

9 Treatment of RCE Severe or recurrent episodes Oral Doxy (50mg bid) w/wo a topical steroid Azasite (? MGD component) Stromal puncture w/wo Amniotic membrane other

10 PTK

11 Amniotic Membrane Grafts (AMG) Biotissue- Prokera, Amniograft, & Amnioguard IOP Ophthalmics- Ambiodisk

12 AMG Actions 1. Reduces inflammation 2. Inhibits scarring 3. Inhibits angiogenesis 4. Promotes epithelialization 5. Possesses anti- microbial properties 6. Restoration of lost corneal thickness*

13 Indications/Conditions for use Acute corneal trauma Chemical or thermal burn Non- healing epithelial defects (herpes, diabetes) Neurotrophic corneal ulcers Filamentary Keratitis Severe Dry Eye Syndrome Recurring epithelial defects High risk keratoplastys Superficial keratectomy Tube shunt/bleb exposure Pterygium removal Kenyon, K. R., & Lam, H. (2013, June 1). Amniotic Membrane: Themes and Variations. Ophthalmology Management, 1-6.

14 Meesmann s Dystrophy Multitude of epithelial/intraepi cysts Autosomal dominant, present at birth Epithelial layer, bilateral Epi cysts can ruptureà RCE s Friedman, N., Kaiser, P., & Pineda, R. (2009). The Massachusetts Eye and Ear Infirmary Illustrated Manual of Ophthalmology(3rdrd ed., p. 219). N.p.: Elsevier- Health Sciences.

15 Reis- Buckler Corneal Dystrophy Abnormal deposition of collagen replacing Bowman s Gray lesions in honeycomb shape Autosomal dominant RCE s at young age Friedman, N., Kaiser, P., & Pineda, R. (2009). The Massachusetts Eye and Ear Infirmary Illustrated Manual of Ophthalmology(3rdrd ed., p. 220).: Elsevier- Health Sciences.

16 Macular Dystrophy (Groenous Type II) Diffuse haze w/gray- white flecks extending to limbus Autosomal Recessive Abnormal mucopolysaccharides in Stroma With age: Extends into deeper stromal layers Stromal hazy occurs Progressive loss of vision Photophobia and ocular discomfort Emily, Birkholz, Nasreen Syed, and Michael Wagoner. "Epithelial-Stromal and Stromal Corneal Dystrophies:." A Clinicopathologic Review, 17 Aug Accessed 3 Nov

17 Granular Dystrophy (Groenouw Type I) #1 stromal dystrophy Autosomal dominant Snowflake granules of hyaline in VA from subepithelial scarring or dense stromal deposits. RCE s are common

18 Granular Dystrophy- Type 2/Avellino Dystrophy Combination of Granular opacities with Lattice lines/opacities.

19 Lattice Dystrophy (Type I) Autosomal Dominant Amyloid deposits Lattice fence appearance

20 Schnyder Corneal Dystrophy Small, needle- shaped crystals that are either white or polychromatic Epithelium remains normal Stromal and may extend into deeper Stroma Autosomal dominant Big Mac. In Mcdonalds. Retrieved from

21 Treatment DALK (Deep Anterior Lamellar Keratoplasty) Penetrating Keratoplasty Granular PTK

22 Surgical Treatments Penetrating Keratoplasty (PKP or PK) Deep Anterior Lamellar Keratectomy (DALK)

23 Corneal Grafts are increasing in number Registry study of total # corneal grafts per year from Number of grafts Anterior Lamellar grafts Endothelial grafts Penetrating grafts Year Coster, D. J., Lowe, M. T., Keane, M. C., & Williams, K. A. (2015, May). A Comparison of Lamellar and Penetrating Keratoplasty Outcomes. American Academy of Ophthalmology, 121(5),

24 Penetrating Keratoplasty (PK)/(PKP) A circular button- shaped full- thickness section of cornea is removed using a trephine or a laser. A matching button is removed from the donor cornea. The new donor cornea is sewn to the host cornea with sutures.

25 23 years Archives of Ophthalmology 2011 Jun; 129 (6): Epub 2011 Feb 83%

26 Here s a patient who Comes into your office because she can t see 57 y/o female Complains of blurry vision worse in the morning.. Gets better Vision Like looking through water Do I have a cataract? Endothelial Dystrophy Friedman, N., Kaiser, P., & Pineda, R. (2009). The Massachusetts Eye and Ear Infirmary Illustrated Manual of Ophthalmology(3rdrd ed., p. 223).: Elsevier- Health Sciences.

27 Endothelial pump cells atrophy leaving spaces/clumps (guttata) and eventual corneal edema Pleomorphism & polymegathism Autosomal dominant endothelial layer Fuch s Dystrophy

28 Posterior Polymorphous Dystrophy Assymetric patches of vesicles with no pattern Gray patches on endothelium Deep stromal edema Associated with PAS and glaucoma

29 ECC

30 DSEK Descemet s Stripping Endothelial Keratoplasty (DSEK/DSAEK) Removes a. Descemet s b. Endothelium Insert a. Post. Stroma b. Descemet s c. Endothelium

31 DMEK Descemet s Membrane Endothelial Keratoplasty Removes a.descemet s b.endothelium Insert a.descemet s b.endothelium

32 DMEK vs. DSEK- Acuity logmar pre- operative logmar bcva 3- month logmar 6- month logmar bcva bcva logmar 0=20/20 logmar 0.3=20/40 logmar 0.6=20/80 DMEK DSEK Tourtas, T., Laaser, K., Bachmann, B., Cursiefen, C., & Kruse, F. (2012, June). Descemet Membrane Endothelial Keratoplasty Versus Descemet Stripping Automated Endothelial Keratoplasty. American Journal of Ophthalmology, 153(6),

33 DMEK vs. DSEK- ECD DMEK DSEK ECD pre- op ECD 3 months PO ECD 6 months PO Tourtas, T., Laaser, K., Bachmann, B., Cursiefen, C., & Kruse, F. (2012, June). Descemet Membrane Endothelial Keratoplasty Versus Descemet Stripping Automated Endothelial Keratoplasty. American Journal of Ophthalmology, 153(6),

34 DMEK vs. DSEK- Graft Adhesion 70 DMEK vs 62 DSEK 10.00% 8.6% 3.2% DMEK DSEK 0.00% Rebubble rate 1. Hamzaoglu, E. C., Straiko, M. D., Mayko, Z. M., Sales, C. S., & Terry, M. A. (2015, August 11). The First 100 Eyes of Standardized Descemet Stripping Automated Endothelial Keratoplasty versus Standardized Descemet Membrane Endothelial Keratoplasty at One Institution. Ophthalmology.

35 Risk of Transplant Rejection 14.0% 12.0% 10.0% 8.0% Risk of Transplant Rejection at 1 Year and Probability at 2 Years 9% 12% 6.0% 4.0% 2.0% 0.0% 0.7% 1% 13 month F- U Probability at 2 years DMEK DSEK Anshu, A., Price, M., & Price, F. (2012, March). Risk of Corneal Transplant Rejection Significantly Reduced with Descemet's Membrane Endothelial Keratoplasty. Ophthalmology, 119(3),

36 HSV Keratitis

37 HSV Keratitis Features Unilateral presentation always suspicious for HSV Iritis with high IOP always suspicious for HSV After 2 nd episode, 70-80% had another recurrence within 10 years Bilateral involvement or prolonged HSV suggests comorbid disease (immunodeficiency or immunosuppression)

38 Epithelial Corneal vesicles Dendritic Ulcer Geographic Ulcer Marginal Ulcer Stromal Inflitration Vascularization Haze and scarring Ulcer/no ulcer

39 Endothelitis Area of corneal edema No epi involvment pseudo- guttae and Descemet s folds th.uiowa.edu/eyef orum/cases/160- hsv.htm Neurotrophic Ulcerated Results from altered corneal innervation and decreased tear production

40 HSV Keratitis Treatment Acyclovir (Zovirax) 400 mg 5 x daily for 7-10 days Valacyclovir (Valtrex) 500 mg 2 x daily for 7-10 days Famciclovir (Famvir) 250 mg 2 x daily for 7-10 days Trifluridine ophthalmic solution 1% (Viroptic) 1 drop 9 x a day for 7 days; can decrease to 5 x a day after 7 days if ulcer not healed. Ganciclovir ophthalmic gel 0.15% (Zirgan) 1 drop 5 x a day until ulcer heals followed by 1 drop 3 x a day for 7 days.

41 HSV Keratitis Prophylaxis Why? 1. Multiple recurrences of HSV keratitis 2. Recurrent inflammation with scar/vascularization 3. Post- keratoplasty performed for HSV reasons 4. Postoperatively in patients with history of HSV undergoing any type of ocular surgery 5. In patients with a history of ocular HSV during immunosuppressive treatment

42 HSV Keratitis Prophylaxis Acyclovir (Zovirax) 400 mg 2 x daily for 1 year Valacyclovir (Valtrex) 500 mg 1 x daily for 1 year Famciclovir (Famvir) 250 mg 2 x daily for 1 year

43 Treatment Principles Treat epithelial disease 1 st and stromal 2nd When using steroids use either therapeutic or prophylactic dose of orals to prevent reoccurrence In stromal cases that are controlled taper steroid gradually. Patient may never be able to get off in stromal disease and prophylactic orals may be required indefinitely.

44 Herpes Zoster: Shingles treatment

45 HZV Features HZV is the etiologic agent of both varicella (chickenpox) and reactivation (shingles) Can erupt anywhere on the body (15% involve the ophthalmic division of the CN 5) Clinical Manifestation Phases Pre- eruptive Phase Acute Eruptive Phase Chronic Phase (PHN)

46 HZV Features HZV typically happens once in life (30% of adults) Recurrent episodes are atypical must consider a sinister etiology A workup for occult malignancy or other reduced cell mediated immunity concerns

47 Post Herpetic Neuralgia Neuropathic pain syndrome that persists beyond 90 days or develops after lesions have resolved Most frequent and debilitating complication of HZV Treatment: oral anti- virals, cool compresses, analgesics, amitriptyline, gabapentin, nerve block

48 HZV Treatment Acyclovir (Zovirax) 800 mg 5 x daily for 7-10 days Valacyclovir (Valtrex) 1000 mg every 8 hours for 7-10 days Famciclovir (Famvir) 500 mg every 8 hours for 7-10 days

49 HZV Treatment Acyclovir (Zovirax) 800 mg 5 x daily for 7-10 days? Valacyclovir (Valtrex) 1000 mg every 8 hours for 7-10 days Famciclovir (Famvir) 500 mg every 8 hours for 7-10 days Oral Corticosteroids

50 Herpes Zoster Ophthalmicus Involves the ophthalmic division of the 5 th CN Ocular complications Conjunctivitis Uveitis Acute retinal necrosis (rare) Cranial nerve palsies Keratitis - Pseudodendrites Optic Neuropathy

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52 Corneal Degenerations

53 Corneal Degenerations Non- familial, late onset Asymmetric, unilateral, central or peripheral Tissue changes caused by inflammation, age, or systemic disease Characterized by a deposition of material, thining of tissue, or vascularization.

54 Keratoconus Ectatic corneal degeneration: Tends to be bilateral, Maybe asymmetric, and Generally manifests in the 2nd or 3rd decade. Tends to progress over 7-8 years and then stabilizes

55 Keratoconus: Clinical Findings SLE findings include: Central corneal thinning, Fleischer s ring, Corneal scarring Vertical striae (Vogt s lines). Common refractive or topographic effects include: irregular astigmatism and poor best- corrected visual acuity with specs

56 Treatment PKP DALK Intacs

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59 Cross- linking Procedure Corneal epithelium is debrided, as is done in PRK Riboflavin eye drops (vitamin B2) are applied to the cornea. The riboflavin film is maintained with reapplication of drops at ~2 minute intervals until the stroma is saturated (20 minutes) UVA light (365nm) is shined on the eye for 30 minutes (conventional technique) A bandage lens is placed. Follow- up care is similar to PRK.

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61 Completed Phase III Study Keratoconus *LOCF = Last Observation Carried Forward

62 KCN- Acute Hydrops Symptoms: Sudden decrease in best correctd vision Foreign body sensation or pain Rupture in Descemet s Membrane self- limiting in 8-10 weeks once endo cells regenerate across the rupture external- disease /news/print/ocular- surgery- news/ %7B73f675bc- a8d4-45b1-85a fd5f91%7d/ minor- complications- can- occur- with- collagen- cross- linking

63 KCN- Acute Hydrops Treatment Hyperosmotics??? Antibiotics - to prevent secondary infections Steroids - to help with inflammation PKP if significant scarring limits correctable vision.

64 Pellucid Marginal Degeneration Bilateral corneal disorder thinning of the inferior, peripheral cornea Thinning begins about 1-2 mm above the inferior limbus and Is separated by an area of uninvolved, normal cornea between the thin zone and the limbus Manifests between the ages of 20-40

65 Pellucid Marginal Degeneration

66 Pellucid Marginal Degeneration Symptoms are visual secondary to irregular astigmatism Thinning is free of vascularization or lipid infiltration which differentiates this from Terriens Butterfly wing- like pattern or kissing dives

67 Salzmann s Nodular Degeneration Elevated bluish- white superficial nodules Fifth decade of life or later? Localized corneal inflammation

68 Salzmann s Nodular Degeneration PTK or Superficial keratectomy Aggressive DES Treatment

69 Terrien s Marginal Degeneration Rare, bilateral, asymmetric disease Peripheral cornea, usually superior undergoes lipid deposition, vascularization, and stromal thinning. Corneal perforation is possible

70 Terrien s Marginal Degeneration Eyes are typically not injected Little pain, photophobia, or AC reaction increase in regular and irregular astigmatism are common, but usually asymptomatic to the patient.

71 Terrien s Marginal Degeneration If asymptomatic, no treatment If patient suffers from injection or irritation steriods will resolve Refractive treatments specs Specialty CL s PKP last resort Treatment

72 Thank You!