Skip to main content

Table 2 Summary of the Clinical Features of the Corneal Dystrophies

From: Corneal dystrophies

  Disease onset Visual acuity Clinical appearance of cornea on slit-lamp biomicroscropy
SUPERFICIAL CORNEAL DYSTROPHIES    
MECD Early childhood Vision rarely blurred Multiple distinct epithelial vesicles
RBCD Childhood Progressive visual impairment Confluent irregular geographic opacities in Bowman layer and superficial stroma
TBCD First/second decade Progressive visual impairment Subepithelial honeycomb opacities in central superficial cornea
GDCD First/second decade Marked visual impairment Subepithelial nodular deposits and late staining of fluorescein
LECD Childhood Sometimes impaired Epithelial opacities in different patterns
ERED First decade Sometimes impaired Epithelial erosions
SMCD First decade Progressive loss of vision Subepithelial opacities
CORNEAL STROMAL DYSTROPHIES    
MCD Usually childhood Eventually severe visual impairment Thinner than normal cornea with diffuse corneal haze with irregular shaped whitish opacities
GCD type I Childhood Progressive visual impairment Well-defined granules that sometimes resemble crushed bread crumbs within a crystal clear cornea
GCD type II First/second decade Progressive impairment of vision Variable shaped opacities in a clear superficial mid stroma of the cornea. Lattice lines sometimes appear in deeper cornea
LCD type I and variants First decade Progressive visual impairment Delicate branching interwoven linear opacities occur in association with ovoid dots
LCD type II Third/fourth decade Vision usually normal until sixth decade Corneal opacities forming lattice lines are found mainly in the peripheral cornea
FCD At birth Normal small discrete dandruff or ring shaped, fleck like opacities
SCD Early in life Progressive decrease in visual acuity Central corneal haze or subepithelial crystals
CSCD Before birth Moderate to severe visual loss Diffuse corneal clouding with flake-like opacities throughout stroma
PACD Infancy or childhood Mildly affected Diffuse sheet-like opacities especially in posterior corneal stroma
POSTERIOR DYSTROPHIES    
FECD Variable but usually fourth decade or later Progressive visual impairment Diffuse thickening of Descemet membrane with excrescences (guttae). Endothelial cells sparse and atrophic
PPCD Early childhood Rarely progressive visual impairment Variable shaped abnormalities of the corneal endothelium
CHED1 Occasionally at birth, but usually in first/second decade Blurred vision that worsens in the morning Thickened cornea with diffuse clouding with occasional focal gray spots
CHED2 At birth Blurred vision Thickened cornea with diffuse clouding with occasional focal gray spots
XECD At birth Blurred vision common in males Cloudy cornea (only in males) with moon crater-like endothelial cells