This document summarizes various corneal dystrophies and degenerations. It describes corneal dystrophy as a group of genetically determined, bilateral corneal diseases classified by the affected corneal layer. Various epithelial, stromal, endothelial, and mixed dystrophies are outlined. Age-related degenerations like arcus senilis and Hassall-Henle bodies are also discussed. Pathological degenerations involving lipid, amyloid, or calcium deposits and conditions like keratoconus, Salzmann's nodular degeneration, and pellucid marginal degeneration are summarized. Treatment options for different dystrophies and degenerations include contact lenses, corneal cross-linking, corneal ring insertion, corneal transplantation, and chelation therapy.

1. CORNEAL DYSTROPHY
and
DEGENERATION

2. CORNEAL DYSTROPHY
 Group of corneal diseases that are:
genetically determined
classified with respect to the corneal layer affected
bilateral

3. Classification
Epithelial and Subepithelial Dystrophies
Epithelial-Stromal TGFBI Dystrophies
Stromal Dystrophies
Endothelial Dystrophies

4. Epithelial and Subepithelial
Dystrophies
1) Epithelial basement membrane dystrophy
2) Meesmann epithelial corneal dystrophy
3) Lisch epithelial corneal dystrophy
4) Gelatinous drop like corneal dystrophy
5) Epithelial recurrent erosion dystrophies
6) Subepithelial mucinous corneal dystrophy

5. Epithelial basement membrane dystrophy

6. LISCH EPITHELIAL DYSTROPHY

7. MACULAR DYSTROPHY GRANULAR DYSTROPHY

8. Lattice stromal dystrophy

9. Keratoconus

10. **Greek words: kerato = cornea
**conus = cone-shaped
**Is a non-inflammatory condition of the cornea in which there is progressive
central thinning of the cornea changing it from dome-shaped to cone-
shaped. Causing vision to become blurred and distorted.

11.  Cornea with keratoconus.
Note the steeper curvature

12. Pathophysiology
All layers of the cornea are believed to be affected by KC,
most notable features are :
1. Thinning of the corneal stroma.
2. Ruptures in the Bowman layer.
3. Deposition of iron in the basal epithelial cells, forming the
Fleischer ring.
4. Breaks in and folds close to the Descemet membrane
result in acute hydrops and striae, respectively.

13. ** Etiology :
Sporadic 90%: Imbalance of enzymes within the
cornea ; leads to collagen defect . This imbalance
makes the cornea more susceptible to oxidative
damage from compounds called free radicals,
causing it to weaken and bulge forward.
Heredity
Eye rubbing as in case of allergic conjunctivitis.
Contact lenses wear
Hormonal change
Collagen systemic disease

14. ** Symptoms :
 Start in puberty (in the teens) and may progress for the
next 10 to 20 years. Is a progressive disease
 Frequent prescription changes in glasses and contact
lenses.
 Usually bilateral involvement but asymmetrical.
 Nearsightedness.
 Astigmatism.
 Blurred vision and distored vision - even when wearing
glasses and contact lenses

15. • The Classic Signs Of Keratoconus
Slit lamp
Fleischer's ring (an iron
colored ring surrounding
the cone
resulted from iron
deposition on the basal
epithelial layer )

16. Vogt's striae (longitudinal stress lines caused by
corneal thinning)
 Apical scarring (scarring at the apex of the cone).

17. *Corneal thinning:
In advanced cases, the thinning of
the central cornea can be seen on
examination

18. * Munson's sign:
 It’s an angulation of the lower lid during inferior gaze due to corneal
protrusion

19. **Treatment :
(To improve vision & to stop progression)
1) Hard contact lens for best vision but has poor tolerance.
2) crosslinking of the stromal collagen
3)Corneal ring - Intacs
4) Cornea transplant

20. CORNEAL DEGENERATION
 Non-familial, late onset
 Asymmetric, unilateral, central or peripheral
 Changes to the tissue caused by inflammation, age, or
systemic disease.
 Characterized by a deposition of material, a thinning of tissue,
or vascularization

21. CLASSIFICATIONS
 A. Depending upon locations
I. Axial corneal degeneration
1.
2.
3.
4.
5.
Fatty degeneration
Hyaline degeneration
Amyloidosis
Calcific degeneration(Band keratopathy)
Salzmann’s nodular degeneration

22. II. PERIPHERAL DEGENERATION
1.
2.
3.
4.
5.
6.
7.
Arcus senilis
Vogt’s white limbal girdle
Hassa-Henle bodies
Terrien’s marginal degeneration
Morren’s ulcer
pellucid marginal degeneration
Furrow degeneration(senile marginal
degeneration)

23. B. DEPENDING UPON ETIOLOGY
A] Age related degeneration:-
Arcus senilis, Vogt’s white limbal girdle,
Hassal-Henle bodies,Mosaic degeneration.
B] Pathological degeneration:-
Fatty degeneration, Amyloidosis, Calcific
degeneration, Salzmann’s degeneration,
Furrow degeneration, Spheroid
degeneration, Pellucid marginal
degeneration, Terrien’s marginal
degeneration

24. AGE RELATED CORNEAL
DEGENERATIONS

25. ARCUS SENILIS
 Arcus senilis refers to
an annular lipid
infiltration of corneal
periphery.
 Sometimes, similarly
changes may or may
not be associated
with hyperlipidemia.

26. CLINICAL FEATURES



The arcus starts in the
superior and inferior
quadrants and then
progresses
circumferentially to form
a ring which is about 1
mm wide.
Peripheral border of this
ring opacity is sharp
while central border is
diffuse
This ring of opacity is
separated from the
limbus by a clear zone.

27. HASSAL-HENLE BODIES
 Hassal-henle bodies are
drop like excrescences of
hyaline materal projectng
into the anterior chamber
around the corneal
periphery.
 These form the
commonest senile change
seen in the cornea.
 In pathological conditions
they become larger and
invade the central area and
the condition is called
CORNEAL GUTTATA

28. CROCODILE SHAGREEN:
 Asymptomatic
 Greyish white polygonal
stromal opacities separated by
relatively clear spaces
 Frequently involves anterior to
thirds of the stroma
occasionally posterior one third.

29. PATHOLOGICAL CORNEAL DEGENERATION

30. FATTY DEGENERATIONS
 It is characterized by
whitish or yellowish
deposits.
 Initially fat deposits are
intracellular but some
becomes extracellular
with necrosis of stromal
cells.
 Lipid keratopathy can be
primary or secondary

31. • Primary : characterized by white or yellowish
often with crystalline element, stromal deposits
consisting of cholesterol , fats and phospholipids
and not associated with vascularization.
• Secondary : common associated with previous
ocular injury or disease resulted in corneal
vascularization.
Most common cause herpes simplex and zoster.

32. Treatment : primarily medical control of underlying
inflammatory disease:
 Photocoagulation or needle cautery of feeding vessels.
 Penetrating keratoplasty in advanced but quiescent
disease.

33. HYALINE DEGENERATION
 Primary Hyaline
degeneration association
with granular dystrophy .
 Secondary Hyaline
degeneration is unilateral and
associated with various types
of corneal disease including
old keratitis, long-standing
Glaucoma, trachomatous
pannus.
 It may be complicated by
recurrent corneal erosion.

34. TREATMENT
 Treatment of the condition when it causes
visual disturbance is keratoplasty

35. AMYLOID DEGENERATION
 Amyloid degeneration of
cornea is characterized
by deposition of Amyloid
material underneath
epithelium.
 It is very rare condition
and occurs in primary
(in a healthy cornea)
and secondary forms (in
a disease cornea).

36. Band shape keratopathy Calcific degeneration (BSK)
CALCIFIC DEGENERATION
(BAND SHAPE KERATOPATHY )
 Band shape
keratopathy (BSK) is
essentially a
degenerative change
associated with
deposition of calcium
salts in Bowman’s
membrane, most
superficial part of
stroma and in deeper
layers of epithelium.

38. ETIOLOGY
 Ocular disease complicated by band keratopathy
include chronic uveitis in adults, children with
still’s disease, phthisis bulbi, chronic Glaucoma,
chronic keratitis and ocular trauma.
 Age related BSK is common and affects
otherwise healthy cornea.
 Metabolic conditions rarely associate with BSK
included hypercalcaemia

39. CLINICAL FEATURES
 It typically presents as
a band shaped opacity
In the interpalpebral
zone with a clear
interval between the
ends of the band and
the limbus.
 The condition begins
at the periphery and
gradually progresses
towards the centre.

40. TREATMENT
• Chelation is simple and effective for mild cases.
• Corneal epithelium overlying the opacity and a solid layer of
calcification are first scraped of with forceps and scalpel
blade.
• The cornea is then rubbed with cotton tipped applicator
dipped in solution of EDTA 1.5-3.0 % for 15-20 minutes.
• Other modes : diamond burr , excimer laser keratectomy and
lamellar keratoplasty.

41. SALZMANN’S NODULAR DEGENERATION
 Salzmann’s nodular
degeneration SND is a
slowly progressive
condition in which gray-
white to bluish nodules
measuring 1-3 mm are
seen anterior to
Bowman’s layer of the
cornea.
 They are classically
round and located in
the mid-periphery.
SND is classically round and
located in the mid-peripher

43. PATHOGENESIS
 In Salzmann's nodular degeneration raised
hyaline plaque are deposited between
epithelium and bowman’s membrane
 There is associated destruction of
bowman’s membrane and the adjacent
stroma

44. ETIOLOGY
 It can occur in any form of chronic
corneal irritation or inflammation such as
trachoma, dry eye, chronic blepharitis
and chronic allergic keratoconjunctivitis.
 The condition occurs more commonly in
women and is usually unilateral

45. CLINICAL FEATURES
 Patient may experience discomfort due to
loss of epithelium from the surface of the
nodules impinge on the central zone.
 Signs :
• superficial stromal opacities progressing to
elevated whitish or blue grey nodular lesions
that may be round or elongated.
• Base of the nodule may be associated with
pannus or epithelial iron deposits.

46. TREATMENT
 To improve visual acuity and to decrease
the irregular astigmatism a superficial
keratectomy was recommended to remove
the Salzmann’s nodules.
 Treatment is essential by keratoplasty

47. TERRIEN’S MARGINAL DEGENERATION
 Terrien’s Marginal
Degeneration is
non-ulcerative
thinning of the
marginal cornea.

48. ETIOLOGY
 Predominantly affects males usually
after 40 years.
 Mostly involves superior peripheral
cornea.
 Initial lesions asymptomatic corneal
opacities separated from limbus by
clear zone.
 Lesion progresses slowly with
thinning and superficial
vascularization.

49. COMPLICATION
Complication such as perforation (due to
mild trauma) and pseudopterygium may
develop.

50. TREATMENT
Early refractive treatment includes:
 spectacles (polycarbonate),
 CL an option though difficult to fit due
to irregular astigmatism (RGP over
piggyback),
 And when vision uncorrectable surgical
intervention includes PK.

51. SPHEROID DEGENERATION
Climatic droplet keratopathy
/Labradorkeratopathy/corneal
elastosis.
Etiology:
• Typically occurs in men
who work outdoors.
• Has also been related to
exposure to UV rays or
ageing or cornel disease

52. 

53. CLINICAL FEATURES
 In this condition amber-coloured spheroidal
granules accumulate at the level of
bowman’s membrane and anterior stroma in
the interpalpebral zone.
 In marked degeneration the vision is
affected

54. TREATMENT
 Treatment in advance cases is by corneal
transplantation

55. PELLUCID MARGINAL CORNEAL
DEGENERATION
 Acute hydrops maybe seen in the area of
inferior thinning.
 Commonly manifests b/w ages of 20-40 with
no apparent hereditary transmission and
equal gender distribution

56. PELLUCID MARGINAL CORNEAL
DEGENERATION

57. SIGNS
 Inferior corneal thinning
 Severely reduced uncorrected visual acuity
that typically cannot be improved with
spherocylinder lens
 Practically normal pinhole visual acuity

58. DIAGNOSTIC PROCEDURES
 Corneal topography
 Pachymetry: Used to measure for inferior
corneal thinning, which is a reversal of the
typical pattern in which the cornea thickens
from centre to periphery
 Orbscan: Shows a classic "kissing birds"
appearance with PMD

59. TREATMENT
 Because of extremely abnormal corneal
topography, the treatment of PMD is difficult.
 Therapeutic options are limited by the
degree of corneal protrusion.
 A recent study has found that 88% of PMD
cases were managed nonsurgically with
spectacles (36%) or contacts (52%),
whereas 12% underwent penetrating
keratoplasty.

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