define dystrophy:

1. define dystrophy: • primary tissue changes of a genetic nature which occur in a previously normal tissue

2. define degeneration: • any tissue change occurring in a previously normal tissue

3. define congenital anomaly: • gross morphological tissue alterations that manifest at birth

4. name 10 “characteristics” of a corneal dystrophy • bilateral • symmetrical • central • avascular • inherited • early onset (1st or 2nd decade) • slowly progressive • unrelated to systemic disease • non-inflammatory • usually 1 cornea layer

5. name 10 characteristics of a corneal degeneration • unilateral • asymmetric • peripheral • vascularized • not inherited • mid-life or later • progressive • often associated with systemic disease • variable inflammation • usually multiple cornea layers involved

6. name 3 anterior corneal dystrophies • EBMD • Reis-Buckler’s dystrophy • Meesmann’s dystrophy

7. name some stromal cornea dystrophies • lattice dystrophy • granular dystrophy • macular dystrophy • central crystalline (Schneider’s dystrophy)

8. name some endothelial dystrophies • Fuch’s • posterior polymorphous dystrophy (PPD) • congenital hereditary endothelial dystrophy (CHED)

9. name some corneal degenerations • depositions: • arcus • band keratopathy (Ca) • Salzmann’s nodular degeneration • spheroidal • amyloid • Coat’s ring • systemic medications • keratoconus • peripheral degenerations/marginal thinning

10. posterior polymorphous dystrophy (PPD) pearls • mesodermaldysgenesis syndrome • autosomal dominant • bilateral • male = female • Descemet’s changes lead to epithelialization of endothelium • may appear as vesicles arranged in a linear or grouped pattern, usually as broad bands with irregular, scalloped edged • may cause iridocorneal adhesions and/or decentered pupil (corectopia)

11. congenital hereditary endothelial dystrophy (CHED) clinical pearls: • bilateral corneal edema with normal diameter, normal IOP, and no guttata • autosomal recessive: • present at birth • +nystagmus • pain and photophobia uncommon • autosomal dominant: • first seen in childhood • slowly progressive • pain/tearing/photophobia common

12. keratoconus clinical pearls • usually bilateral • usually asymmetric • slowly progressive irregular astigmatism • contrary to Dr. Crane’s claim, keratoconic patients may see improvement with a pinhole • Vogt’s striae: vertical tension lines in cornea • irregular K mires/ret reflex • corneal hydrops

13. “AMAnDA LEAH” • keratoconus associated etiologies • atopy • Marfan’s syndrome • Addison’s disease • Down’s syndrome • amyloidosis • Leber’scongenital amaurosis • Ehler’sDanlos syndrome • Apert’s syndrome • hypothyroidism

14. dellen clinical pearls • painless, oval thinning • secondary to corneal drying and stromal dehydration adjacent to abnormal conjunctival or corneal elevation • epithelium usually intact • palliative treatment

15. furrow degeneration clinical pearls • painless corneal thinning just peripheral to arcus • no vascular infiltration or inflammation • thinning not progressive; perforation rare • no treatment needed

16. collagen vascular disease melt clinical pearls • Etiologies: • RA, SLE, polarteritisnodosa, Wegner’s granulamatosis, relapsing polychondritis • unilateral or bilateral • peripheral thinning with possible infiltrates • may involve entire cornea; may perforate • may be first sign of systemic disease