The Cloudy Cornea

1. The Cloudy Cornea Optometry 8570

2. Corneal Dystrophies • Progressive, mostly bilateral, non-inflammatory disorders leading to opacification. • Genetically determined. • Classified into Epithelial, Bowman, Stromal and Endothelial Dystrophies.

3. Corneal Dystrophies Epithelial Dystrophies

4. Epithelial Dystrophies Epithelial basement membrane dystrophies (EBMD). • AKA map-dot dystrophy, fingerprint dystrophy, Cogan microcystic dystrophy. • Atypical to other dystrophies in that they are neither genetically determined and usually not progressive. • A thickened BM and loss of hemidesmosomes leads to the clinical appearance and the 10% of patients that develop recurrent erosions. • Usually in their 20’s.

5. EBMD variants

6. EBMD (Cogan microcystic)

7. EBMD

8. Meesmann Dystrophy • Occurs early in life (1-3 years old) • AD inheritance • Appears as tiny intraepithelial cysts which are more densely packed centrally and more diffuse in the peripheral cornea • Very rare

9. Meesmann Dystrophy

10. Meesmann Dystrophy

11. Corneal Dystrophies Bowman Dystrophies

12. Reis-Bücklers Dystrophy • AD inheritance • Gray to white round opacities in Bowman’s layer. The opacities are more densely packed centrally . • Reduced corneal sensation • Tx is typically with laser keratectomy • A variant is called Thiel-Behnke Dystrophy which shows a different inheritance pattern • Opacities are honeycomb-like.

13. Reis-Bücklersvs.Thiel-Behnke

14. Central Schnyder Dystrophy • AKA Crystalline Dystrophy • Crystalline-like opacities in the central cornea • Deposits of phospholipid & cholesterol • Patient is usually in their 20’s and complains of reduced vision and glare • AD inheritance • Tx with laser keratectomy

15. Crystalline Dystrophy

16. Corneal Dystrophies Stromal Dystrophies

17. Lattice Dystrophy • Several sub-types that manifest at different ages. • Most are AD inheritance • Amyloid deposits that form lattice-like opacities which are more concentrated centrally. Progresses deeper into the stroma and more peripherally. • Tx with PK

18. Lattice – Anterior Stromal Dots

19. Lattice – Early Lines

20. Lattice Forms

21. Stromal haze and use of Retro

22. Granular Dystrophy • Small, well-demarcated, white opacities in the central anterior stroma. • Gradual confluence of the opacities can decrease VA. • AD inheritance • Usually presents with kids with recurrent erosions. • Tx with PK usually by their 40’s

23. Granular Dystrophy

24. Granular Dystrophy - moderate

25. Granular Dystrophy - Advanced

26. Avellino Dystrophy • Similar to granular dystrophy but the opacities are variable from round discs, lines to rings. • Begins slightly later than Granular dystrophy (20’s) but recurrent erosions are rare. • AD inheritance • Tx usually not required but the density and concentration of the opacities could need PK

27. Avellino Dystrophy

28. Corneal Dystrophies Endothelial Dystrophies

29. Fuchs Endothelial Dystrophy • Can be AD but can show sporadic inheritance • More common in women • Begins in the second decade as diffuse central guttata. As the endothelia decompensates and the VA decreases from stromal edema. Pain from ruptured bullae occurs and scarring ensues . • Tx aimed at symptoms.

31. FuchsEpithelial Microcysts and Edema

32. Fuchs Bullae

33. Posterior Polymorphous Dystrophy • Asymptomatic, widely varying appearances. • Isolated areas of endothelial cells that show histological characteristics of epithelial cells. • Present at birth but usually found as an incidental finding during routine eye exams. • Associated with other developmental anomalies (iris membranes, corectopia, ICE-like glaucoma…) . • No Tx. • Rare?

34. Posterior Polymorphous Dystrophy

35. Posterior Polymorphous Dystrophy

36. Posterior Polymorphous Dystrophy

37. Keratoconus

38. Background • An unpredictable progressive condition • Most begin in young adulthood. Some progress and stabilize quickly, others steadily progress to surgery, while still others go through periods of progression and stabilization. • Questionable inheritance • Associated with genetic syndromes (Down’s, Turner’s, Marfan’s and others). • Usually begins in one eye, but usually becomes bilateral.

39. Signs • Irregular astigmatism • Mild: K’s < 48D, Moderate: K’s 48-54D, Severe: > 54D • “Oil droplet” pupillary reflex with direct ophthalmoscopy • Early Slit lamp signs: • Deep stromal lines (Vogt’s stria) • Prominent corneal nerves • Late Slit lamp signs: • Epithelial iron deposits (Fleischer ring) • Stromal scarring • Bulging of lower lid in downgaze (Munson sign)

40. Keratoconus signs

41. Vogtsstriae

42. Acute hydrops and Apical scarring

43. Treatment • Hyperosmotics for acute episodes • Rigid Contact lenses • Epikeratoplasty • Penetrating or deep lamellar keratoplasty

44. The Basics of Penetrating Keratoplasties

45. The Donor Button • Best if harvested within 24 hours after death. • Infants and the very elderly are poor donors. • Should be free of infectious diseases of the CNS or systemic infections i.e. AIDs, syphilis… • Leukemia • Chronic eye disease (uveitis, ocular malignancy…)

46. Prognostic factors to consider Conditions that may negatively affect the outcome of PK • Eyelid abnormalities (i.e. blepharitis, trichiasis, entropion) • Tear film anomalies • Recurrent conjunctival inflammations • Severe stromalvascularization • Anterior synechiae • Uncontrolled glaucoma • Uveitis

47. Penetrating Keratoplasty Basics 1) Donor button is prepared first. a) Slightly larger than the host cornea b) The donor button is trephined from the endothelial side. 2) Viscoelastic injected into host eye. 3) Host cornea is trephined. 4) Donor tissue is attached to host with 10-0 sutures a) interrupted sutures b) running sutures

48. PK interrupted sutures

49. Post-operative Management 1) Topical Steroids Used QID for 2-3 weeks then tapered based upon tissue response. Can be used 1-2 drops per day for many months. 2) Cycloplegics BID for a couple of weeks. 3) Removal of sutures in 9-12 months.

50. Post-operative Complications • Persistent epithelial defects • Protruding sutures → FB sensation / GPC • Wound leak • Flat AC • Iris prolapse • Increased IOP • Recurrence of the initial disease • CME