3. Introduction
deterioration and decrease in function
aging, inflammation, or environmental insult
broad spectrum of ocular abnormalities
4. 4Features Degeneration Dystrophy
Onset Presents later in life,
associated with aging
Present early in life,
hereditary
Laterality Unilateral Bilateral; May
be asymmetric
Bilateral & symmetric
Family history Uncommon Common
Vascularization Common Uncommon
Location often peripherally
located
Centrally located
Progression Progression can be very
slow or rapid
Progression usually
slow
5. Classification
No any exact classification
A. According to location
1. central corneal degeneration
2.peripheral corneal degeneration
B.According to ETIOLOGY
1.Involutional corneal degeneration
2.Noninvolutional corneal degeneration
18. Band is wider in vertical than horizontal meridian
A lucid interval is present between arcus and limbus
This clear is about 0.3 mm wide, known as clear
interval of vogt
19. 19
Prevalence- increase with age
Incidence;
- 60% in 50-60 years
-100% in >80 years
Men >women.
Prevalence increase -postmenopausal period
Black males have the highest incidence
Arcus is almost always bilateral
20. 20 Histopathology
Lipid is first deposited at Descemet's membrane and subsequently
at Bowman's layer
Stromal accumulations of
-cholesterol esters
-triglycerides
-phospholipids
Vascular in origin(Lipid material leaks from limbal capillaries, but
central flow is limited
21. Systemic association:
may be associated with dyslipidemia in under 50 years (arcus
juvenilis)
Young patients who have arcus also have an increased risk for
type IIa dyslipoproteinemia but a decreased risk for type IV(type
2&3)
Occasionally congenital anomaly involving only a sector of
peripheral cornea
22. Clinical significance
< 40 years - increased risk of coronary artery disease and should be evaluated for
hyperlipoproteinemia
Diseases causing a rise in β-lipoproteins (nephrotic syndrome,
hypothyroidism, increased cholesterol intake, obstructive jaundice, and diabetic
ketoacidosis)
lecithin cholesterol acyltransferase (LCAT) deficiency&tangier dz.
Unilateral – carotid artery occlusion
22
25. VOGT'S WHITE LIMBAL GIRDLE
Type I
mild, early form of calcific band keratopathy
chalklike opacities & scattered clear holes like swiss cheese
Separated from sclera by peripheral clear zone called lucid interval
degenerative change of anterior limiting membrane
27. VOGT'S WHITE LIMBAL GIRDLE
Type II
more common type
white flecklike needlelike deposits
lacks a peripheral clear zone between the arc and the limbus
consists of fine, white radial lines
located nasally more often than temporally.
common in >45yrs,female
Incidence increase with age -55% at 40-60 years
28. Histopathology
Lesion is subepithelial and may have overlying epithelial
atrophy
Destruction and calcification of Bowman's layer (type 1)
Epithelial elastotic degeneration (type 2)
28
29. 3.Senile corneal furrow degeneration
• Thinning of cornea in older peple in lucid interval of corneal arcus
• No tendency to perforate
• epithelium is intact
• Rare, true thinning with no inflammation, vascularization, or induced corneal
astigmatism
30. 4.Furrow degeneration associated with
systemic disease
Focal or extensive ring type epithelial defect & sterile
ulceration near limbus
Accompany systemic dz.
-rheumatoid arthritis
-wegner’s granulomatosis
-polyarteritis nodosa
- relapsing polychondritis
-SLE
-collagen vascular disease
33. Signs
Begins superiorly,spreads circumferentially, rarely central cornea
inferior limbus
Central wall is steep & peripheral wall slopes gradually
Epithelium remains intact,& fine vascular pannus traverses area of
stromal thinning
Rupture in Descemet membrane can result in interlamellar fluid
even corneal cyst
Perforation may rarely occur either spontaneously or following
blunt trauma
34. • Begins superonasally
• Fine punctate opacities in the anterior
stroma
• Superficial vascularization
• Gutter forms between the opacity and
limbus
• Stroma progressively thins
• Overlying epithelium remains
intact
34
36. Gradual visual deterioration occurs as a result of increasing corneal
astigmatism
Fuchs superficial marginal keratitis(children young adults)
37. Histopathology
Fibrillar degeneration of collagen
Epithelium may be normal, thick, or thinned
Bowman's layer is fragmented or absent
Breaks in Descemet's membrane may be seen in thinned area
37
40. 1.Iron lines
Chronic abnormalities of tear flow
Hudson-stahli line: Normal aging
Ferry’s line: Adjacent to filtering bleb
Stocker’s line: Head of pterygium
Fleischer’s ring: Base of Keratoconus
41. 2.Coats’ White Ring
Small corneal opacity usually
located in an area that
previously harbored
foreignbody
42. Coats’ White Ring
<1 mm ring,circle or oval granular ring in s/l,grey-white
dots
Represent iron containing fibrotic remnants of metallic FB
The condition causes no symptoms & require no therapy
43. 3.Lipid degeneration
primary secondary
More common
Occurs in vascularized cornea
No prior history of:
Trauma
Family history of similar conditions
Corneal vascularization
No known disorders of lipid metabolism
43
44. Lipid degeneration
Accumulation of yellow or cream colored crystalline
material in corneal stroma which may be thick or thin
Lipid keratopathy may be peripheral, central, or diffuse
45. Increased vascular permeability of
limbal vessels
Release of fatty material into the stroma
from dying cells
cholesterol, triglycerides, and
phospholipids
(extracellular space)
45
46. treatment
Only If –
For cosmesis and decrease vision
Argon laser treatment with or without fluorescein
photodynamic therapy with verteporfin
Subconjunctival & topical bevacizumab
50. Etiologies
Ultraviolet radiation
Microtrauma (sand, dust,
wind, and drying)
Type 1 ,3
Secondary degeneration is caused by multiple disease entities
Associated with corneal neovascularization
50
51. Clinical grading
Grade I – fine shiny droplets present only peripherally without symptoms
Grade II – central cornea is involved: vision ≥ 20/100 (6/30)
Grade III – there are large corneal nodules: vision ≤ 20/200 (6/60).
51
52. pathogenesis
• Secreted by corneal and conjunctival fibroblasts
• Interaction between UV light and plasma proteins within
stroma has been proposed to result in abnormal deposits
52
53. treatment
Aphakic/pseudophakic/clear lens
---excimer laser PTK
cataractous condition
a)cataract extraction with or without addressing opacity
b) combined penetrating keratoplasty &cataract extraction
c) combined lamellar keratoplasty &cataract extraction
d)PTK
54. 5.Salzmann's nodular degeneration
• Superficial stromal opacities
pannus
• Unilateral/ bilateral
• Female>male
54
• Elevated
Blue–grey nodular lesions
• Round or elongated
• Separated by clear zone
56. Histopathology
Dense collagen plaques with hyalinization are located
between epithelium and Bowman's layer
Bowman's layer is absent under lesion
Overlying epithelium may be atrophic or absent
56
59. 59
Gray white and chalky
Begins at corneal periphery in 3 and 9 o’clock positions
Centrally in cases of chronic ocular inflammation
Periphery- sharply demarcated edge separated from
limbus by a lucent zone
Lucent holes are scattered throughout the opacity and
represent penetrating corneal nerves
61. Histopathology
Fine basophilic granules (first at the level of Bowman's layer)
Calcium deposits (intra/ extracellular)
Hyaline-like material is deposited in subepithelial tissue around calcific
depositions
61
66. CROCODILE SHAGREEN
Mosaic pattern resembling cobblestone or crocodile skin
Usually bilateral
Gray to white ‘cracked ice’ opacities with
central lucent zones
66
67. 67
Anterior May be seen as a senile change
Keratoconus patients with hard
contact lenses
Trauma, band keratopathy, hypotony,
juvenile X-linked megalocornea
Posterior Age-related degeneration
68. CORNEA FARINATA
Incidental finding
Asymptomatic
Very fine, dust-like dots (white or gray)
central stroma (just anterior to Descemet's
membrane)
idiopathic
68
69. Dellen / Fuchs’ dimples
Saucer-like depressions in corneal surface
Commonly adjacent to elevated areas
May last only 24 to 48 hours
Most commonly in temporal peripheral cornea (adjacent to a
paralimbal elevation)
69
70. Cornea verticillata
Lysosomal deposits in epithelium (amiodarone)
Whorl- like pattern
Other drugs- chloroquine, chlormazipine, indomethacin
Usually dose not result in reduction of vision
70
71. summary
Secondary deterioration or deposition in cornea, distinct from dystrophies
Occurs in later life
Dose not reduce visual acquity
Not inherited
May be age-related ( white limbal girdle, arcus senilis, crocodile shagreen,
cornea farinata)
May be post inflammatory ( Salzmann nodular degeneration, corneal keloid,
lipid keratopathy, calcific band keratopathy)
Idiopathic (furrow degeneration, Terrien marginal degeneration)
71
72. references
Krachmer, Mannis, Holland. Cornea- fundamentals, diagnosis and
management. 2nd edition . Vol 1
AAO. External disease and cornea. 2013-2014
Kanski Clinical ophthalmology. 8th edition
Albert and jacobiec’s principles and practice of ophthalmology. 3rd edition
. Vol 1.
72
Notas del editor
Range from clinical curiosities to sight-threatening anomalies
Inflammatory and systemic association
Noninvolutional corneal degeneration-less commonly seen and related to specific local and systemic condition
This classification is arbitrary as many condition such as spheroidal degeneration and band keratopathy can be found in both location.
Whitish ring of peripheral cornea ….has hazy white appearance,sharp outer border & indistinct central border;it is denser superiorly & inferiorly separated from limbus by a clear zone…STROMAL.. Involutional change modified by genetic factors
It begins superiorly and inferiorly, gradually spreading to involve entire corneal periphery but becoming densest and widest superiorly
In women a similar pattern is seen, but with a delay of about 10 years
Limited by a functional barrier to flow of large molecules in cornea, which keeps deposits in their peripheral location
It frequently occurs without any predisposing systemic condition in elderly individuals,, In older patients, including those with diabetes, arcus does not correlate with mortality .Men with arcus juvenilis have a 4 fold increase risk of mortality from CHD therefore isa useful clinical indication for the need for lipid and cvs exam
Abnormalities in blood lipids may be concomitant in younger patients displaying corneal arcus or Schnyder’s crystalline corneal dystropy
Vogt describe limbal girdle – white, arc-like crecentric opacities in cornea central to limbus in the 3 and 9 o'clock positions..not associated with inflamm,notcvascularized,doesn’t progress..mistaken for arcus…..STROMAL
Vogt's limbal girdle type II is made up of hyperelastotic &hyaline deposits peripheral to Bowman's membrane with sometimes particles of calcium…These findings are similar to those seen in pinguecula &pterygium
requires no therapy, but it should be considered when cataract incisions are made in these patients
Peripheral inflammatory condition, bilateral,,, Most common in those between 20 and 40 years,,, M:F= 3:1
Flattening of peripheral thinned cornea, with steepening of corneal surface approx.90 deg.away from midpoint of thinned area-this pattern results in against the rule astigmatism…………………fuchs-rare,progressive thinning without epithelial ulceration can lead to perforation
Surgery if
--perforation imminent
--marked astigmatism
Crescent shaped lamellar or full thickness corneoscleral patch grafts decrease against the rule astigmatism upto 2o yrs
Annular lamellar keratoplasty grafts in severe cases of 360 degree marginal degenerations
Related to abnormalities of tear pooling due to surface irregularities
Primary-rare, occurs spontaneously,usually bilateral,no evidence of antecedent infection,inflamm,corneal damage,secondary-prolonged infamm with scarring &corneal vascularization(herpes simplex or zoster keratitis,trachoma).. Histopathologically, material consists of intra- and extracellular lipids, similar to those of arcus
Golden brown translucent spheroid like deposits…….the composition is not lipid despite its “oil droplet appearance”..the condition is related to elastotic degeneration of collagen as in pingueculae
. They measure from 0.1 to 0.4 mm. In the early stages of type 1, they appear at the limbus in the interpalpebral zone at 3 and 9 o’clock. In type 2 the spherules may be diffuse or begin centrally. The conjunctival form also occurs interpalpebrally in the 3 and 9 o’clock positions.
May be progressive as long as a patient remains exposed to causative factors
Hyaline-like material are found in corneal stroma, Bowman's layer, and subepithelium…Lesions appear to develop from extracellular material deposited on collagen fibrils secreted by
Noninflammatory & creates multiple …May be associated with recurrent erosion..present in central and paracentral cornea & at ends of vessels of pannus
Simple stripping of focal nodules by….if decrease vision secondary to irregular astigmatism
But sometimes deposition of urates in cornea which are brown cand cause band keratopathy..associated with gout n hyperurecemia
3 to 9 o clock position, later coalese to form horizontal band of dense calcific plaques in interpalpral zone of cornea
Chronic anterior uveitis, interstitial keratitis,severe superficial keratitis,Phthisis bulbi,,…phenylmercuric nitrate preservative in opthal solutions..mercury causes changes in corneal collagen that results in deposition of calcium
Ethylene diamine tetra acetetic acid…then superficial keratectomy performed by stripping calcific scales with forceps…if residual opacity after EDTA,PTK done
White,superficial sometimes protuberant glistening corneal masses found either central or periphery that eventually involve entire corneal surface…may resemble nodule in Salzmann degeneration….can be congenital like Lowe syndrome or can be primary
Anterior crocodile shagreen is at level of bowman’s layer….posterior crocodile shagreen in deep stroma near Descemet membrane
Dot shaped or comma shaped opacities which are best seen in retroillumination…these deposits may consist of lipofuscin,a degenerative pigment that appears in aging cells