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Opt 631 Exam 1 Corneal Dystrophies
Dr. Yudcovitch Lecture 2
43
Medical
Graduate
09/06/2011

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Term
What is the genetic nature of corneal dystrophies?
Definition
autosomal dominant; therefore family members will often also be afflicted
Term
What are the three most common corneal dystrophies?
Definition
EBMD

Fuch's

Keratoconus
Term
What is Epithelial Basement Membrane Dystrophy
Definition
subtle 'fingerprint' or 'maplike' breakdown of epithelium
Term
What are the SE of EBMD?
Definition
mild: irritation in morning

moderate: glare, chronic irritation

Severe: reduction in VA, quite painful. Spontaneous epithelial erosions
Term
Is TBUT normal in EBMD?
Definition
No
Term
What is the potential worst case SE of severe EBMD?
Definition
Can lead to permanent damage to cornea
Term
What is the mechanism of a spontaneous epithelial erosion?
Definition
Hemidesmosomes break allowing epithelium to peel off of stroma/bowmans
Term
What is the tx for mild EBMD?
Definition
hypertonic 5% NaCl at night

lubricate
Term
What is the tx for moderate EBMD?
Definition
hypertonic NaCl sol'n; prophylactic AB; homatropine
Term
What is the tx for severe EBMD?
Definition
Debride loose epithelium

hypertonic sol'n

bandage CL

prophylactic AB

May need anterior stromal puncture
Term
What is anterior stromal puncture?
Definition
using a 30ga needle to 'spot weld' the epithelium back onto the stroma for spontaneous epithelial erosions
Term
Is there a cure for EBMD?
Definition
No, can only be managed
Term
What is Meesman's Juvenile Epithelial Dystrophy?
Definition
cysts or vesicles in epithelium; a juvenile variant of EBMD.
Term
What are the Bowman's layer dystrophies?
Definition
Reis-Buckler's and Anterior Mosaic dystrophy
Term
What is Reis-Buckler dystrophy?
Definition
irregular opaque pattern or 'fishnet' pattern.
Term
What are the SE of Reis-Buckler's dystrophy?
Definition
recurrent epithelial erosion, stromal opacification
Term
What is the tx for Reis-Buckler's dystrophy?
Definition
keratoplasty, low vision lenses, erosion tx
Term
What is Anterior Mosaic Dystrophy?
Definition
gray polygonal opacities in central cornea
Term
What are the three Anterior stromal dystrophies?
Definition
Granular, Lattice, and Crystalline dystrophy
Term
What is granular dystrophy?
Definition
looks like salt grains in corneal stroma
Term
What are the SE of granular dystrophy?
Definition
reduced VA's with age
Term
What is the tx of granular dystrophy?
Definition
keratoplasty, LV aids
Term
What is Lattice Dystrophy?
Definition
refractile lines in anterior stroma, in severe cases can appear like ground glass
Term
What are the SE of lattice dystrophy?
Definition
RCE
Term
What is the tx of lattice dystrophy?
Definition
keratoplasty generally by age 40

RCE tx

LV aids
Term
What is a common DDX of lattice dystrophy?
Definition
fungal infection
Term
What is crystalline dystrophy?
Definition
white to yellow 'glitter' in a ring around central cornea
Term
What is crystalline dystrophy often associated with?
Definition
Hypercholesterolemia w/ arcus
Term
What is xanthelasma?
Definition
Fat deposits in the eyelids, indicates high cholesterol, often seen with crystalline dystrophy
Term
What is the tx for crystalline dystrophy?
Definition
visually asymptomatic, refer for CV workup to check lipid levels
Term
What is macular dystrophy?
Definition
diffuse clouding of cornea due to elevated sugar levels
Term
What is the tx for macular dystrophy?
Definition
keratoplasty, LV aids
Term
What are the SE of macular dystrophy?
Definition
reduced VA's by age 30
Term
What is keratoconus?
Definition
Central thinning of cornea, increased refractive power of cornea, thinning and bulging seen
Term
What is the mechanism of keratoconus?
Definition
Unknown, but an enzymal cascade --> toxicity is suspected
Term
What is the prevalence of keratoconus?
Definition
1/2000 people
Term
What are the SE of keratoconus?
Definition
Severe refractive error, high cylinder.

Cornea may rupture easily
Term
What are Vogt' striae?
Definition
looks like cat scratches on back of cornea, seen in keratonus
Term
What is hydrops?
Definition
posterior corneal break leading to excess fluid in cornea. NO Tx available.
Term
What are the tx of keratoconus?
Definition
anti-edema tx

keratoplasty

refractive correction
Term
What is commonly seen in retinoscopy on keratoconus pt's?
Definition
dewdrop' or 'oil droplet' in retroillumination
Term
What is an intrastromal corneal ring?
Definition
an implanted ring that stabilizes the stroma to treat keratoconus
Term
What is ectasia?
Definition
protrustion of cornea
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