Jim Hannigan
Definition
Corneal Dystrophy
• Bilateral, inherited corneal disorder,
showing no signs of inflammation or
neovascularization
Jim Hannigan OD
Hallmark
• The hallmark of corneal dystrophy is the
primary involvement of a single corneal
layer
Epithelial Basement Membrane
Dystrophy
• Autosomal dominant
• May be asymptomatic
• Possible recurrent corneal erosion
• Morning syndrome
1
2
Anterior Stromal
Puncture
Treatment
• Artificial tears
• Hypertonics
• Treat erosion
• Bandage contact lens
• Debridement
• Stromal puncture
• PTK
A Jackhammer
A Dremel Tool
A Dremel Tool
Use surgical spear to define limits of defective
epithelial attachment – be ready for a surprise!
FINAL STEP
Diamond
burr to
remove
defective
epithelium
DON’T
WIMP OUT
HERE!!!
Polish the basement
membrane and
contour wound edge
If performed correctly, treatment
is near 100% effective
3
Reimbursement
Superficial Keratectomy
Prognosis
65600
Stromal puncture for
recurrent erosion (NOTE:
best fit code)
$$$
Medicare
Our Fee
• Good short term
• Guarded long term
• CL risk 3+, 1+ if treated
$338.42
$450.00
Pearl # 1
• The dystrophies involving the epithelium
and anterior stroma tend to present with
recurrent epithelial erosions, while those
involving the deeper cornea usually
present with bilateral loss of visual acuity
Pearl # 2
• Recurrent corneal erosion complications
can be predicted with relative accuracy by
checking Bell’
Bell’s Phenomenon
Meesman’s Dystrophy
• Rare autosomal dominant
• Present in first years of life
• Best seen with retro
4
Treatment
Prognosis
• Treat symptoms if necessary
• Superficial keratectomy
• Lamellar corneal transplant
• PTK
• Excellent
• CL risk 1+
Reis-Buckler’s Dystrophy
• Autosomal dominant
• Progressive, but appears early in life
• Painful recurrent erosions common
5
Treatment
Prognosis
• Erosion therapy
• Superficial lamellar keratectomy
• PTK
• PKP
• Guarded
• CL risk 2+
Granular Dystrophy
• Autosomal dominant
• Presents in first decade
• Symptomatic in middle age
• Visual acuity gradually reduces…
reduces….rarely
worse than 20/200
6
7
Treatment
• Usually none
• If VA decreases, PKP
Prognosis
• Good
• CL risk 3+
Lattice Dystrophy
• 4 known types:
• 1) classic form w/ no systemic
involvement
• 2) less common, associated with systemic
amyloidosis
• 3) rare form, adult onset
• 4) rare form, adult onset
• Autosomal dominant
• Corneal erosions common
8
9
10
Treatment
Prognosis
• Treat erosions
• If VA decreases, PKP
• Guarded
• CL risk 2+
The BIGH3
Avellino Dystrophy
• A transforming growth factor betabeta-
• Also called Granular type 2
• Granular and lattice dystrophy combo
• Autosomal dominant
• Possible erosions
inducing gene…
gene…..located on chromosome
5
• Lattice type 1, 3, and 4
• Granular type 1 and 2
• ReisReis- Bucklers
11
Treatment
Prognosis
• Treat erosions
• If VA decreases, PKP
• Guarded
• CL risk 2+
12
Macular Dystrophy
• Autosomal recessive
• Recurrent erosions common
• Appears in 1st decade
• Vision decreases by age 30
• Corneal guttata with secondary erosions a
late occurrence
Treatment
• Treat erosions
• If VA decreases, PKP
13
Prognosis
• Guarded
• CL risk 2+
Central Crystalline Dystrophy
• Autosomal dominant
• YellowYellow-white crystals, 1st-2nd decade
• Dense arcus ring, 3rd-4th decade
• Vision mildly affected
14
Treatment
• Systemic treatment
• WorkWork-up includes fasting serum
cholesterol and triglyceride levels
• Ocular signs not reversible
Prognosis
• Good
• CL risk 2+
Posterior Polymorphous Dystrophy
• Autosomal dominant
• Usually asymptomatic
• Congenital or early onset
• Rare VA decrease…
decrease…..20/30
• Some risk of glaucoma
15
Treatment
Prognosis
• Hypertonics for edema
• Annual pressure check for glaucoma
• Good
• CL risk 2+
16
Congenital Hereditary Endothelial
Dystrophy (CHED)
Type 1
Congenital Hereditary Endothelial
Dystrophy (CHED)
Type 2
• Autosomal recessive
• Present at birth
• NonNon-progressive
• Nystagmus
• Autosomal dominant
• Presents during childhood
• Slow progressive
• No Nystagmus
• Pain and photophobia
Treatment
• Refer to pediatric specialist
• PKP
• Genetic counseling
17
Prognosis
Fuch’s Endothelial Dystrophy
poor
• Type 1…………
1…………poor
guarded
• Type 2…………
2…………guarded
• CL risk 4+
• Autosomal dominant
• Rarely seen before age 50
• Affects women more than men
• Bilateral, but not always symetrical
• Edema, bullous keratopathy
• Morning syndrome
18
Treatment
• Hot packs for edema
• Hypertonics
• Blow dryer
• Glaucoma meds
• (-) Rx for am if needed
• Mydriatic (allows light around central
clouding)
Treatment con’t
• Treat ruptured corneal bullae
• Bandage contact lens
• PKP
• Genetic counseling
• 3-6 month f/u to check edema and IOP
Prognosis
• Guarded in peripheral cases
• CL risk 1+ with treatment
19