corneal degeneration

1. Corneal degeneration
Dr.Rakshya Basnet
1st year resident
NAMS,LEI

2. contents
 Introduction
 Classification
 Peripheral corneal degenerations
 Central/ diffuse corneal degenerations
 Summary
2

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

6. C.According to corneal layers
1.Epithelial& subepithelial degenerations
2.Stromal degenerations
3.Endothelial degenerations

7. Classification
Peripheral degenerations
1. Corneal arcus
2. White limbal girdle of Vogt
3. Idiopathic furrow degeneration
4. Post irradiation thinning
5. Terrien’s marginal degeneration
6. Furrow degeneration associated
with systemic disease
Central or diffuse degenerations
1. Iron Lines
2. Coat’s White Ring
3. Lipid Degeneration
4. Amyloid Degeneration
5. Spheroid Degeneration/Climatic
Droplet Keratopathy, keratinoid
Degeneration
6. Band Keratopathy
7. Salzmann’s Nodular Degeneration
8. Corneal Keloid

8. Classification
Epithelial and sub epithelial degenerations

9. COAT’S WHITE RING

10. STROMAL DEGENERATION

11. SALZMANN NODULES IN PARACENTRAL CORNEA LIPID KERATOPATHY SECONDARY TO CORNEAL
VASCULARISATION
AMYLOID DEGENERATION CORNEAL KELOID

12. CORNEA FARINATAVOGT LIMBAL GIRDLE TYPE 2
CROCODILE
SHAGREEN

13. TERRIEN MARGINAL DEGENERATION

14.  Endothelial degenerations
Iridocorneal endothelial syndrome
Peripheral corneal guttae
Melanin pigmentation
IRIDOCORNEAL ENDOTHELIAL SYNDROME

15. • Depending on etiology:
a)Age related:
• Arcus senilis,
• Vogt’s white limble girdle
Hassel- Henle bodies
b) Pathological Degeneration:
• Fatty degeneration
• Hyaline degeneration
• Amyloidosis
• Calcific Degeneration
• Salzmann’s nodular degeneration
• Terrien’s marginal degeneration
• Pellucid marginal degeneration

16. A.Peripheral corneal degenerations

17. 1.Arcus senilis(gerontoxon,arcus lipoides)
• most common peripheral corneal
degenerations

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

23. 2.VOGT'S WHITE LIMBAL GIRDLE

24. Limbal Girdle (of Vogt)
Type 1
Type I

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

26. Type 2
Type 2

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

31. 5.Post irradiation thinning
Non-inflammatory corneal excavation at limbus
May occur after high local doses of B-radiation

32. 6.Terrien marginal degeneration:
• Terrien disease is an uncommon idiopathic slowly progressive
thinning of peripheral cornea
•

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

35. types35
quiescent
More common
Older patients
Asymptomatic
Inflammatory
Younger age
Recurrent inflammation(episcleritis, scleritis)

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

38. treatment
 large astigmatism
 impending perforation
 perforation
lamellar or eccentric
penetrating grafts
38

39. B.Central or diffuse degeneration

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

47. 4.Spheroidal degeneration
1. Climatic droplet keratopathy
2. Bietti's nodular corneal degeneration
3. Labrador keratopathy
4. Corneal elastosis
5. Fisherman's keratitis
6. Keratinoid corneal degeneration
7. Chronic actinic keratopathy
8. Eskimo’s corneal degeneration
9. Proteinaceous corneal degeneration
47

48. Clear to yellow-gold spherules oily appearing are seen in
subepithelium,bowman layer or superficial stroma
0.1 to 0.4 mm.
48

49. Spheroidal degeneration
Type 1 Type 2 Type 3
49

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

55. Etiologies
 Idiopathic
 corneal inflammatory disease, especially meibomian gland
dysfunction
 Vernal keratoconjunctivitis
 Interstitial keratitis
 Contact lens wear
 Dry eye
 keratoconus
55

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

57. treatment
 Lubrication – epithelial erosions
 Superficial keratectomy
 Lamellar or penetrating keratoplasty
57

58. 6.calcific Band keratopathy
Deposition of calcium salts in the Bowman layer, epithelial
basement membrane and anterior stroma
58

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

60. etiology
Ocular
• Chronic ocular disease esp.
inflammatory
• Silicone oil in anterior chamber
• Chronic exposure to mercurial vapors or
mercurial preservatives
Others
• Age-related
• Metabolic- hypercalcemia
-elevated serum phosphous level
• Hereditary
60

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

62. investigations
 Serum calcium
 Phosphorus
 Uric acid
 Renal function measurements
 Parathyroid hormone (PTH)
 Angiotensin-converting enzyme (ACE)
62

63. treatment
 Topical EDTA 0.05 molar concentration after removal of
epithelium
 PTK
63

64. 7.Corneal keloids
White nodules may extend deep into stroma
Appear following trauma, surgery, or
inflammatory processes
64

65. Histopathology
 Fibroblastic proliferation(myofibroblast) intermixed with
hyalinized collagen bundles
 Bowman's layer may be fragmented or absent
65

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.
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