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Pigmentations

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Dystrophy's  

  • Bilateral
  • Congenital
  • Rare
  • Slowly progressive and stabilize by age 20-30
  • No infiltrates,
  • No thinning
  • No neovascularization
  • Usually central location,
  • Usually involves only one corneal layer


Epithelium

Map-dot-fingerprint

  • Epithelial basement disorders (EBMD)
  • Basal epithelial cells secretes basement membrane which attaches the epithelium to the stroma
  • Takes 6-8 weeks to reform
  • Migration of cysts to surface leads to RCE (AM syndrome)
  • Projections from the basal cells trap cells and debris forming microcysts  (Seen as dots)
  • Intraepithelial changes lead to negative stain and reduced acuity
  • Maps, dots, fingerprints
  • Hold lids open and tell pt to shut eyes
  • Positive EBMD and negative bells phenomena predicts 75% chance of spontaneous erosion
  • 50% of recurrent erosions have EBMD
  • Preventative therapy
  • Sicca worse in PM  EBMD worse in AM
  • Autosomal dominate?
  • Small number are congenital dystrophy most age related
  • Cyst like vesicle's in epithelium
  • Ridges


Bowmans membrane

 Anterior moasic dystrophy

  • Crocodile shagreen
  • Central gray polygonal opacities with a clear zone around
  • Blanches with limbal pressure
  • No problem

  Ring dystrophy

  • Reis-Bucklers
  •  “Fish net” swirls stromal haze
  • Painful reccurent erosion
  • Less problem after age 30

Anterior stroma


Central crystalline dystrophy
(Schnyder's)

  • Central crystals during first 2 decades
  • Dense arcus during next 2 decades
  • Associated with high cholesterol (Refer for blood panel)

Granular dystrophy (Groenouw’s)

  • First decade
  • Discrete white translucent hard cornflake or powdery dots
  • Need  keratoplasty

  Anterior stromal line dystrophy (Lattice) (Biber's)

  • Re-occurent erosions
  • Autosomal dominant
  •  X-crossed lines
  • Late teen or post surgical


Full stroma


Fleck dystrophy
(Francois-Neetens)

  • Gray or white ring by age 2
  • Extends to periphery
  • No problem

  Macular dystrophy (Groenouw's)

  • Diffuse ground glass haze with white or grey opacities
  • Extends to periphery
  • Autosomal recessive
  • Late corneal guttata and erosions
  • Rare but devastating

Deep stroma


 Central cloudy dystrophy
(Francois's)

  • Fuzzy grey areas deep in stroma
  • No problem

Posterior amorphous dystrophy

  • Gray sheet in first decade to periphery
  • No problem but may reduce acuity to 20/40

Pre-Decemet’s dystrophy (Farinata)

  • Deep punctate or filimentary opacities
  • No problem


Endothelial 

Congenital hereditary endothelial dystrophy

  • Recessive form congenital with nystagmus
  • Dominate form no nystagmus
  • Diffuse ground glass edema
  • 2-3 times corneal swelling 
  • Erosions
  • May extend to periphery
  •  “Orange peel” effect in decemets


  Late endothiel dystrophy (Fuch’s)

  • Progressively more guttata of endothelium
  • Stromal edema  (Wet “ground glass” bedewing)
  • Much more common in post-menopausal females
  • Appears to affect several layers
  • Thickening of decemets
  • Stromal edema
  • Endothelium cells enlarge, lose hexagonal shape and thin
  • AM erosions
  • 10 min. hairdryer
  • Soft lenses for bullous keratopathy
  • Low minus RX for AM

  Posterior polymorphous dystrophy (Schlicting's)

  • Grouped vesicle's forming “Swiss cheese” pattern
  • Gray lines in decemets forming “Railroad tracks”
  • Watch IOP
  • May reduce acuity slightly

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Copyright © 1997, 1998, 1999, 2000, 2001, 2002, 2003, 2004, 2005, 2006 Don Steensma, O.D., F.A.A.O.
Last modified: May 1, 2006