Dystrophies Flashcards
What are the best slit lamp examining techqunies to see MDF?
- Sclerotic scatter 2. retroillumination 3. Broad tangential beam
What happens to the cornea in Meesmanns?
Slightly thinned and sensation may be reduced.
Which dystrophy is X linked with gray, band shaped feathery lesions in whorled patterns with densely crowded clear microcysts?
Lisch (pain free)
Which dystrophy has painful recurrent epithelial erosions with geographic or honeycomb gray white reticular opacification in the central cornea?
Reis-Bucklers (sawtooth Bowman’s layer)
How do you diferentie Reis-Bucklers from Thiel-Behnke dystrophy?
RB - rod-shaped bodies; TB - has curly fibers
Which is the most common stromal dystrophy?
Granular
What are the 4 stains for Amyloid (Lattice)?
- Congo red
- Crystal violet
- Thioflavine T
- Masson trichrome stain
What dystrophy stain bright red with rod-shaped bodies immersed in an amorphous matrix?
Granular
Which dystrophy involves the corneal periphery and endothelium?
Macular
What are the 2 recessive corneal dystrophies?
- Maclar 2. Gelatinous droplike
Which stromal dystrophy reoccurs in PK grafts?
Lattice (also gelatinous droplike and reis bucklers)
Schnyder crystalline corneal dystrophy stains with what and has what systemic d/o?
Oil red O stains the phospholipids red; Hyperlipopreteima and hperlipidemia; decreased corneal sesnsation.
Which dystrophy has a extreme asymmetric presentation with decreased corneal sensation, limbal mermaids, keratoconus, punctate cortical lens changes, pseudoxanthoma elasticum, and atopy?
FLECK
What are the genetics of Pellucid Marginal Degeneration?
Uncommon, nonherediatry, bilateral
Where is the thinning greatest in Keratoglobus?
In the PERIPHERY
Which 2 lysosomal storage diseases (mucopolysaccharidosis) have mainly corneal involvement?
- Sheie 2. Hurlers
Which dystrophy has peripheral, sparkling, yellow-white spots at the corneal limbus in the superficial stroma that may fade with time?
Bieetti crystalline corneoretinal dystrophy
Which dystrophy has photophobia with crystals in the corneal stroma and TM and can cause death in the infantile form but adults form life expectancy is normal?
Cystinosis
List 6 causes of ENLARGED corneal nerves
- MEN IIB
- Phytanic acid storage disease (Refsum syndrome)
- Hansen disease
- Riley-Day syndrome
- NF
- Acanthamoeba perineuritis
List 5 causes for MORE VISIBLE corneal nerves?
- Keratoconus
- Ichthyosis
- Fuchs corneal dystrophy
- Corneal edema
5 Congential GL
What are the 11 causes of corneal crystals?
- Macular dystrophy
- Granular dystrophy
- Lattice dystrophy
- Schynder’s dystrophy
- Beitti’s dystrophy
- Ciloxin
- Strep viridans
- Dieffanbachia plant
- cystinosis
- Multiple myeloma
- MONOCLONAL GAMAOPATHY
Fleck dystrophy is inherited?
AD - highly asymmetric and possibly U/L
Which stromal dystrophy presents the earliest?
Macular - followed by lattice then granular.
Lisch dystrophy affects what part of the cornea, what inheritance, and what makes it distinctive?
X-linked DOMINANT; epithelial; densely crowded epithelial microcysts-band shaped in whorled feathery pattern
