corneal dystrophies and degeneration

1. ‫الرحيم‬ ‫الرحمن‬ ‫هللا‬ ‫بسم‬
‫تبصرون‬ ‫أفال‬ ‫أنفسكم‬ ‫وفي‬
‫العظيم‬ ‫هللا‬ ‫صدق‬

2. Samhaa Mohammed Abd Elmoneim
Zagazig Ophthalmologic Hospital

3. Corneal diseases
1. Congenital
2. Traumatic
3. Inflammatory (infective, immune related)
4. Degeneration/Dystrophies
5. Neoplastic
Sailing through cornea
Sailing through eye
Sailing through body

4. Corneal dystrophies and degeneration

5. Dystrophy Degeneration
Laterality Bilateral Uni/ bilateral
Symmetry Symmetrical Assymmetrical
Age Early (young, adult) Late (eldery)
F/H Positive Negative
Site Central Peripheral
Vascularization Negative Positive

6. Corneal Degeneration
1. Arcus senilis
2. Vogt limbal girdle
3. Corneal farinata
4. Corocodile shagreen
5. Lipid keratopathy
6. Band keratopathy
7. Spheroidal degeneration
8. Salzmann nodular degeneration
9. Gutatta ?
Age related
Deposits
Site
Shape
Cause
Colour
Clear zone

7. Corneal Degeneration
1. Arcus senilis (generotoxon, arcus lipoides):
• The most common corneal opacity.
• In younger age (arcus juvenilis) … Dyslipidemia.
C/P:
• Stromal lipid deposit
• Starts superior and inferior (1mm in width)… V>H.
• Clear zone from limbus, whitish.
• Furrow
Work up:
• Lipid profile in younger age.

8. Arcus senilis
Clear zone
(furrow in slit
beam)

9. Corneal Degeneration
2. Vogt limbal girdle:
• Over 40 y, common in females.
C/P:
• Limbal cresenteric bands at 3, 9 o’clock.
• Chalk-like flecks with irregular central extension.
• Type 1 clear zone, with holes (swiss cheese
appearance)
• Type 2 no clear zone, without holes, more common.
DD:
• Band keratopathy

11. Corneal Degeneration
3. Corneal farinata:
C/P:
• Bilateral, central flour-like opacities in deep
stroma.
• Affected VA.

12. Corneal Degeneration
4. Corocodile shagreen:
• Polygonal white polygonal opacities seperated by
clear zone.
• Visually insignificant.
Anterior corocodile shagreen:
• Anterior 2/3 of stroma.
Posterior corocodile shagreen:
• Posterior 1/3 of stroma.
• Francois dystrophy ??!!

14. Corneal Degeneration
5. Lipid keratopathy:
• Visually insignificant.
2ry1ry
More commonRareIncidence
Previous scar (HSV,
HZV)
Hypercholesterolemia
(cholesterol, phospholipid)
Cause and deposits
YesNoVascularization
• Decrease
inflamation
• BL.V (cautery,
photocoagulation)
• KP
• Cause
• Reduce inflamation
• KP
TTT

17. Corneal Degeneration
6. Band keratopathy:
• Calcium deposits in BM, epithelium, ant stroma.
• Chalk-like horizontal band with holes .. Expand
centrally
• Clear zone from limbus.
• Late stage (elevated, nodular) … Corrugated surface &
epithelial defect.
Causes :
• Ocular: Silicon Filled Eye (SFE), chronic ant uveitis,
phthisis bulbi.
• Systemic: hyperuricemia, ch renal failure, increase
serum Ca.
• Other: age related, hereditary as icthyosis.

18. Corneal Degeneration
6. Band keratopathy:
TTT:
• Underlying condition
• If decreased VA … (chelation with EDTA, excimer, diamond
burr, lamellar KP)
• EDTA: Ethylene DiamineTetraacidic Acid
EDTA chelation:(can be repeated)
1. Calcification and epith removal using blade NO,
15.
2. Cotton tipped soaked in EDTA 2% for 20 min.
3. CL application.

21. Corneal Degeneration
7. Sheroidal degeneration:
• Protenious material in ant stroma and BM.
• UV exposue ---- Male predominance.
• Amber color irregular lesion, peripheral--- central.
• Anterior stroma, conjunctiva.
• No clear zone
TTT:
• Protection against UV rays.
• Superficial keratectomy , ALK.

23. Corneal Degeneration
8. Salzmann nodular degeneration:
• Hyaline material anterior to BM.
• Chronic irritation and inflamation (dry eye,
trachoma, blepharitis)
• Whitish, blue grey color elevated rounded or
elongated lesion.
TTT:
• Treat cause … lubricant.
• Superficial keratectomy (diamond burr , PTK, ALK).

27. Corneal Dystrophies
1. Epithelial
2. Bowman membrane
3. Stromal
4. Endothelial

28. Corneal Dystrophies
1. Epithelial (Cogan, Mesmann, Lisch..)
2. BM (Reis Buckler, Theil Benckle,
schynder crystalline)
3. Stromal (lattice, granular, macular)
4. Endothelial (Fuchs, CHED, PPED)

29. Corneal Dystrophies general tips
• Bilateral.
• symmetrical.
• C/P: earlier in age than degeneration.
• All are AD except macular dystrophy AR.
• Marlyin Monoro Always Get Her Man at
Los Anglos City.
1.Macular … Mucopolysacharide … Alcian blue.
2.Granular … Hyaline … Masson Trichome.
3.Lattice … Amyloid … Congo red.

30. Corneal dystrophies
1. Epithelial dystrophies:
a. cogan basement membrane dystrophy:
• Most common dystrophy, AD.
• Mis Dx due to variable appearance.
• Thichened basement membrane (fibrillary protein
between BM and basement membrane)

31. Corneal dystrophies
1. Epithelial dystrophies:
a. cogan basement membrane dystrophy:
C/P:
• 2nd decade
• Asymptomatic
• Recurrent corneal erosion
• Variable (dot like, microcysts, whroled fingerprint,
subepithelial map-like)
• Retroillumination, sclerotic scatter.
TTT: treat RCE

34. Corneal dystrophies
1. Epithelial dystrophies:
b. Meesmann eithelial dystrophy:
AD
C/P:
• Asymptomatic.
• Ocular irritation in 1st few months of life.
• Intraepithelial cyst, not reach limbus.
• Decease sensation, thinning.
TTT: lubricant usually enough.

37. Corneal dystrophies
1. Epithelial dystrophies:
c. others:
Lisch dystrophy Gelatinous drop-like

38. Corneal dystrophies
2. Bowman membrane dystrophies:
a. Reis –Bucklers (type 3 granular dystrophy:
• Fibrous tissue in BM, AD.
C/P:
• 2nd decade.
• Severe RCE
• Decease sensation, affected VA ( scarring).
TTT: RCE, excimer laser keratectomy.

40. Corneal dystrophies
2. Bowman membrane dystrophies:
b. Thiel Behnkle (honeycomb -shaped):
• Fibrous tissue in BM, AD.
C/P:
• End of 1st decade.
• Honey comb-shaped subepith. opacities.
TTT: usually not requires.

43. Corneal dystrophies
2. Bowman membrane dystrophies:
c. Schynder central crystalline:
• Lipid metabolism disorder. High serum cholesterol.
C/P:
• 2nd decade.
• Central, oval subepithelial opacities.
• At 3rd decade… diffuse haze, prominent corneal
arcus.
• TT: ttt hyperlipidemia, excimer keratectomy.

45. DD OF Crystalline keratopathy
1. Infectios crystalline keratopathy
(streptococcus viridans).

48. DD OF Crystalline keratopathy
1. Infectios crystalline keratopathy
(streptococcus viridans).
2. Schynder crystalline BM dystrophy (lipid).
3. Fabry disease, Bietti macular and
crystalline dystrophy (idiopathic).
4. Cystinosis, multiple myeloma (proteins).
5. Band keratopathy, argyrosis, chrysiasis
(minerals).
6. Topical cipro, Amiodarone, Tamoxifen,
phenothiazine (drugs).

49. Corneal dystrophies
3. Stromal dystrophies:
a. lattice type 1 :
• AD, Amyloid deposition in stroma, Congo red stain.
C/P:
• RCE at 1st decade before other signs.
• Central, refractile linear opacities.
• Decrease sensation
• TT: DALK, PKP.

52. Corneal dystrophies
3. Stromal dystrophies:
b. Lattice type 2 (Meretoja) :
• Amyloid deposition in stroma, Cogno red stain.
C/P:
• Decrease sensation.
• Central, peripheral refractile linear opacities.
• Systemic: peripheral neuropathy, mask like facies.
• TT: DALK, PKP later in life.

53. Corneal dystrophies
3. Stromal dystrophies:
c. Granular type 1 :
• AD, Hyaline deposition in stroma, Masson trichome
stain.
C/P:
• Photophobia, glare.
• Discrete well delineated white central corneal
opacities (sugar granules, bread crumbs, glass
splinter).
• Clear intervening spaces, spared limbus
• TT: DALK, PKP later in life.

55. Corneal dystrophies
3. Stromal dystrophies:
d. Granular type 2 (Avellino) :
• AD, Hyaline and amyloid deposition in stroma.
C/P:
• Photophobia, glare.
• Discrete superficial annular and deep linear
opacities.
• TT: usually not required

57. Corneal dystrophies
3. Stromal dystrophies:
e. Macular:
• AR, Mcopolysacharide in stroma, Alcian blue stain.
C/P:
• Decrease vision, RCE.
• poorly delineated white stromal opacities central
and peripheral.
• Stromal odema and haze.
• Decrease sensation.
• TT: PKP, DALK.

59. Corneal dystrophies
4. Endothelial dystrophies:
a. Fuchs endothelial dystrophy:
• Female middle to old age, sporadic, AD
C/P:
• Blurred vision more in the morning.
• Corneal odema… bullous keratopathy
• Specular reflection shows excrescence on endothelium(guttata),
beaten bronze, hummered silver appearance.
• POAG.
• Late scarring and vascularization.
• TTT: conservative (NACL, hair dryer, decrease IOP)
rupture bullae (CL, cyclo, lubricant, ant stromal
puncture)
DMEK, triple procedure.

66. Corneal dystrophies
4. Endothelial dystrophies:
a. Fuchs endothelial dystrophy:
Investigation:
• specular microscopy

72. Corneal dystrophies
4. Endothelial dystrophies:
b. Posterior Polymorphs Endothelial
Dystrophy (PPED):
• AD.
C/P:
• since birth.
• Subtle vesicular band-like endothelial lesions.
• ASSICIATION: Alport dis, glaucoma, iris
abnormalities.
• TT: not required

74. Corneal dystrophies
4. Endothelial dystrophies:
c. Congenital Hereditary Endothelial
Dystrophy (CHED):
• DM, endothelium focal or diffuse thickening
C/P:
TT: endothelial or penetrating kp
CHED 2CHED 1
ARADInheritance
At birth1st yearOnset
lessPhotophobia, epiphoraC/P
MoreLessNystagmus

78. Test yourself

88. STUMPED

94. Dystrophy Degeneration
Laterality Bilateral Uni/ bilateral
Age Early (young, adult) Late (eldery)
Symmetry Symmetrical Assymmetrical
F/H Positive Negative
Site Central Peripheral
Vascularization Negative Positive

95. Corneal Dystrophies
1. Epithelial (Cogan, Mesmann, Lisch..)
2. BM (Reis Buckler, Theil Benckle,
schynder crystalline)
3. Stromal (lattice, granular, macular)
4. Endothelial (Fuchs, CHED, PPED)

96. Corneal Dystrophies general tips
• Bilateral.
• symmetrical.
• C/P: earlier in age than degeneration.
• All are AD except macular dystrophy AR.
• Marlyin Monoro Always Get Her Man at
Los Anglos City.
1.Macular … Mucopolysacharide … Alcian blue.
2.Granular … Hyaline … Masson Trichome.
3.Lattice … Amyloid … congo red.

97. Corneal Degeneration
1. Arcus senilis
2. Vogt limbal girdle
3. Corneal farinata
4. Corocodile shagreen
5. Lipid keratopathy
6. Band keratopathy
7. Spheroidal degeneration
8. Salzmann nodular degeneration
9. Gutatta ?
Age related
Deposits
Site
Shape
Cause
Colour
Clear zone

98. Corneal diseases
1. Congenital
2. Traumatic
3. Inflammatory (infective, immune related)
4. Degeneration/Dystrophies
5. Neoplastic
Sailing through cornea
Sailing through eye
Sailing through body

99. Cornea and globe congenital anomalies
1. Microcornea (horizontal diameter <9mm in age <2y, <10mm
in age >2y), (RE, ACG, cat, ON hypoplasiia, coloboma, aniridia)
2. Microphthalmos (complex, ocular dysgenesis)
3. Nanophthalmos, simple microphthalmos (small, normal
structure, thick choroid, RE, ACG, cataract surgery?)
4. Anophthalmos (globe absence)
5. Megalocornea (bilateral, XLR male, diameter >13mm, RE and
sublaxation)
6. Sclerocornea (bilateral, no demarcation, cornea plana)
7. Cornea plana (flatter than normal, RE)
8. Keratectasia (intrauterine infection, scar) Systemic
consultation

101. Metabolic disorders
1. Cystinosis (AR, cysteine deposition, pediatric renal
failure)
2. Mucopolysacharidosis MPS (GAG, pigmentary
retinopathy, optic atrophy, facial coarseness, cardiac,
skeletal)
3. Wilson (copper, deccease cerruloplasmin, kayser fleischer
ring, sunflower cat, liver basal ganglia, Gonio,
penecillamine)
4. Fabry (X linked, decrease alpha galactosidase, vortex
keratopathy, spoke shaped post cat, angiokeratoma,
cardiac, GIT, renal)
Systemic
consultation

103. Corneal diseases
Samhaa Mohammed
Best wishes

104. Now, lets go for sailing in Sea
not in Cornea
Thanks ALLAH
Thank you