ophthalmological sub

1. Corneal Degeneration &
Deposition Clinical Approach
Dr. Niwar Ameen
Duhok Eye Hospital
2018

2. Contents
• Introduction
• Definitions
• Difference
• Corneal degenerations
• References

3. Introduction
• Degenerations of the ocular surface may result from
physiologic changes associated with
1. Aging process
2. Related to a specific disease
3. Follow chronic environmental insults to the eye, such as
exposure to (UV) light.
• They may be unilateral or bilateral
• They uncommonly exhibit an inheritance pattern.

4. Definitions
• Degeneration refers to decomposition and deterioration of
tissue elements and functions.
• Dystrophies are defined as bilateral, symmetric, inherited
conditions that appear to have little or no relationship to
environmental or systemic factors.

5. Difference
Degeneration
1. Opacity often peripherally
located
2. May be asymmetric
3. Present later in life
associated with aging
4. Progression can be very
slow or rapid
Dystrophy
1. Opacity often centrally
located
2. May be bilateral and
symmetric
3. Present early in life,
hereditary
4. Progression is usually slow

6. Degenerative Changes
Age-Related (Involutional) Changes
• Normal involutional (age-related) changes that occur in the cornea
include:
1. Gradual flattening along the vertical meridian resulting in against-the-rule
astigmatic drift.
2. Mild thinning of the central cornea.
3. Increase in the refractive index with a corresponding decrease in
transparency.
4. The Descemet membrane thickens over time 3µm to 10µm at adult.
5. Gradual decrease in endothelial cell density that begins in the second
decade of life from 4000 cells/mm²at birth to 2500-3000cells/mm².
6. The cornea becomes more rigid with age, presumably due to increased
crosslinking between collagen fibers within the corneal stroma

7. Epithelial and Subepithelial
Degenerations

8. Coats white ring
• A small (1 mm or less in diameter) circle or oval-
shaped area of discrete gray-white dots is sometimes
seen in the superficial stroma.
• Referred to as Coats white ring, it represents iron-
containing fibrotic remnants of a metallic foreign
body.
• Once these lesions mature and are free of any
associated inflammation, they do not change.
• Therapy with corticosteroids or other anti-
inflammatory agents is not indicated.

9. Coats white ring

10. Spheroidal degeneration
• The condition has been reported under different names, including climatic
droplet keratopathy, Bietti nodular dystrophy, and Labrador keratopathy.
• It is characterized by the appearance in the cornea, and sometimes in the
conjunctiva, of translucent, golden brown, spheroidlike deposits in the
subepithelium, Bowman layer, or superficial stroma
• Primary SD, the deposits are bilateral and initially located in the nasal and
temporal cornea. With age, they can extend onto the conjunctiva in the
interpalpebral zone. It is not related to coexisting ocular disease.
• Secondary SD is associated with ocular injury or inflammation. The
deposits aggregate near the area of corneal scarring or vascularization.
• Histology, extracellular, proteinaceous, hyaline deposits with
characteristics of elastotic degeneration.
• The composition is not lipid, despite its "oil droplet" appearance.
• Rx; medical treatment is of no value, superficial keratectomy or
phototherapeutic keratectomy (PTK)for central cases.
• Recurrence after conjunctival resection is common.

11. Spheroidal degeneration

12. Iron deposition
• Most iron lines are related to abnormalities of tear pooling
due to surface irregularities
• Often, they can be seen only by using red-free or cobalt blue
illumination before instilling fluorescein.
• Iron lines and associations:
1. Fleischer ring in early mild cases of Keratoconus.
2. Hudson-Stahli line associated with keratorefractive surgery.
3. Stocker line in pterygium confined to epithelium
4. Ferry line in filtering bleb anteriorly, confined to epithelium
5. Blood staining in some cases of hyphema

14. Hudson-Stahli line Stocker line

15. Calcific band keratopathy
• Calcific band keratopathy is a degeneration of the superficial cornea that
involves mainly Bowman layer.
• The condition can be idiopathic, but the main known causes are:
1. Chronic ocular disease such as uveitis in children, interstitial keratitis,
severe superficial keratitis, and phthisis bulbi.
2. Hypercalcemia caused by hyperparathyroidism, vitamin D toxicity, milk-
alkali syndrome, sarcoidosis, or other systemic disorders.
3. Hereditary transmission
4. Renal failure, elevated serum phosphorus level with normal serum
calcium.
5. Chronic exposure to mercurial vapors or to mercurial preservatives.
6. Silicone oil instillation in an aphakic eye.

16. • Calcific band keratopathy begins as fine, dustlike, basophilic deposits in
the Bowman layer.
• These changes are usually first seen peripherally in the 3- and 9-o'clock
positions.
• A lucid interval is seen between the limbus and the peripheral edge of the
keratopathy.
• Eventually, the deposits may coalesce to form a horizontal band of dense
calcific plaques across the interpalpebral zone of the cornea.
• A workup (eg, serum electrolytes and urinalysis) to rule out associated
metabolic/renal disease should be considered.
• Underlying conditions, such as keratoconjunctivitis sicca or renal failure,
should be treated or controlled.
• The calcium can usually be removed from Bowman layer by chelation with
a neutral solution of disodium ethylenediaminetetraacetic acid (EDTA).
• The problem can recur but may not do so for years, at which time the
treatment may be repeated.
• PTK using an excimer laser is not advised as a primary treatment.

17. moderate severe

18. Stromal Degenerations

19. Corneal Arcus
• It is most often an involutional change modified by genetic factors.
• The incidence is 60% in individuals between the ages of 50 and 60; it
approaches 100% in individuals older than 80 years, men more women.
• While in younger than 40 years, the arcus is sometimes indicative of a
hyperlipoproteinemia involving (LDL) with elevated serum cholesterol.
• It can be a prognostic factor for coronary artery disease in this age group.
• Arcus occurs occasionally as a congenital anomaly (arcus juvenilis), usually
involving only a sector of the peripheral cornea not associated with
abnormalities of serum lipid.
• Unilateral arcus is a rare condition associated with contralateral carotid
artery disease or ocular hypotony.
• Arcus is also seen in Schnyder corneal dystrophy.
• Corneal arcus has no visual significance, and thus no necessary treatment.

21. Limbal girdle (of Vogt)
• It is a symmetrical yellowish-white band located parallel to the
interpalpebral limbus.
• It is found more frequently on the nasal limbus than on the temporal.
• To be seen direct illumination, but is best seen with a combination of
retroillumination and scleral scatter.
• Two forms of the white limbal girdle of Vogt have been described.
• Type I may be a variant of band keratopathy, featuring a ‘Swiss cheese’
hole pattern and a clear area separating the lesion from the scleral margin.
• Type II consists of small, white, flecklike, and needlelike deposits that are
often seen at the nasal and temporal limbus in older patients. No clear
interval separates this girdle from the limbus.
• Histologically, there is epithelial elastotic degeneration of collagen,
sometimes with particles of calcium.
• This degeneration is an incidental finding and is asymptomatic, 60% aged
40 years. No treatment is required.

22. Slide Title
Vogt of limbal Type II Type I

23. Crocodile Shagreen
• It is bilateral, symmetric corneal opacity that has a mosaic pattern
resembling crocodile skin.
• Crocodile shagreen is most prominent in the central cornea, but may also
be observed in the peripheral cornea.
• Both anterior and posterior forms have been described and appear
clinically as polygonal gray opacities separated by thin lucent zones.
• Anterior shagreen is most often associated with trauma, hypotony, band
keratopathy, X-linked megalocornea, and rigid contact lens wear in
patients with keratoconus.
• Posterior shagreen is widely considered an age-related degenerative
condition.
• DDx; dystrophy of François.
• HPS; extracellular stromal vacuoles concentrated in the posterior stroma
and just anterior to the Descemet membrane. The vacuoles often contain
fibrillar-granular material.
• The opacity seen with anterior and posterior crocodile shagreen is visually
insignificant and requires no treatment.

25. Cornea Farinata
• It is an asymptomatic, senile degenerative condition
characterized by fine dustlike opacities located in the
posterior stroma adjacent to the Descemet membrane.
• These opacities are best seen on retroillumination and are
gray-brown to white, hence it has been described as having a
“flour dust” appearance.
• These deposits occur bilaterally
• More prominent centrally
• Do not interfere with vision.
• The condition may be inherited in some patients.
• HPS; abnormal posterior keratocytes were found to contain
membrane-bound intra-cytoplasmic vacuoles filled with lipid-
like inclusions that may represent lipofuscin.

26. Cornea farinata

27. Senile Furrow
• Senile furrow degeneration is an appearance of
peripheral thinning in the lucid interval of a corneal
arcus that is seen in older persons.
• Although slight thinning is occasionally present, it is
usually more apparent than real.
• The epithelium is intact.
• There is no inflammation, vascularization, or
tendency to perforate.
• Vision is rarely affected. No treatment is required.

28. Senile furrow

29. Salzmann nodular degeneration
• It is a noninflammatory corneal degeneration.
• It occurs as a late sequela to old, long-term keratitis, or it may be idiopathic.
• Causes include phlyctenulosis, trachoma, and interstitial keratitis.
• The lesions may not appear until years after the active keratitis has subsided.
• It can be bilateral and is more common in middle-aged and older women.
• The nodules are gray-white or blue-white and elevated, and they may be
associated with recurrent erosion.
• Histologic examination reveals localized replacement of Bowman layer with hyaline
and fibrillar material.
• Treatment
- for mild cases is lubrication
- manual superficial keratectomy may be indicated in more severe
• These nodules may cause decreased vision secondary to irregular astigmatism).
• This degeneration may recur after removal.

31. Polymorphic amyloid degeneration
• It is a bilaterally symmetric, slowly progressive corneal degeneration that
appears late in life.
• These deposits are usually axial, polymorphic, and filamentous.
• The opacities are gray to white and somewhat refractile but appear
translucent in retroillumination.
• There is also an acquired (secondary localized) corneal amyloidosis, which
may be associated with corneal inflammation (eg, trachoma, leprosy
[Hansen disease], phlyctenulosis) or intraocular disease (eg, uveitis,
retinopathy of prematurity) or may be secondary to trauma.
• Clinically, amyloid deposits in acquired corneal amyloidosis can appear as
raised, yellow-pink nodular masses in the cornea or be nonspecific.

32. Polymorphic amyloid degeneration

33. Terrien marginal degeneration
• The cause is unknown.
• This condition is a quiet, essentially noninflammatory, unilateral or
asymmetrically bilateral, slowly progressive thinning of the peripheral
cornea.
• Prevalence is roughly equal between men and women.
• Cases usually occur in the second or third decade of life.
• The corneal thinning can be localized or involve extensive portions of the
peripheral cornea.
• Degeneration begins superiorly, spreads circumferentially, and in rare
cases involves the central cornea or inferior limbus.
• The epithelium remains intact, and a fine vascular pannus traverses the
area of stromal thinning.
• A line of lipid deposits appears at the leading edge of the pannus

34. • Spontaneous perforation is rare, although it can easily
occur with minor trauma.
• Corneal topography reveals flattening of the peripheral
thinned cornea, with steepening of the corneal surface
approximately 90deg away from the midpoint of the
thinned area.
• This pattern usually results in high against-the-rule or
oblique astigmatism.
• Surgical correction is indicated when perforation is
imminent due to progressive thinning or when marked
astigmatism significantly limits vision.
• Crescent-shaped lamellar or full-thickness corneoscleral
patch grafts may be used.

35. Terrien marginal degeneration

36. Lipid keratopathy
• In lipid keratopathy, yellow or cream-colored lipids containing cholesterol,
neutral fats, and glycoproteins are deposited in the superficial or deeper
cornea.
• Prolonged corneal inflammation with scarring and corneal vascularization
(eg, herpes simplex or herpes zoster keratitis, trachoma).
• Treatment is indicated in cases of compromised cosmetic appearance or
decreased vision.
1. Argon laser treatment with and without fluorescein
2. Photodynamic therapy with verteporfin
3. Subconjunctival and topical bevacizumab have been reported to reduce
corneal neovascularization and lipid deposition.
• DDX; Schnyder corneal dystrophy.

37. Lipid Keratopathy

38. Endothelial Degenerations

39. Iridocorneal endothelial syndrome
• (ICE) syndrome is a spectrum of disorders characterized by varying
degrees of iris changes, abnormal corneal endothelium, and peripheral
anterior synechiae.
• The pathogenesis of ICE syndrome is unknown.
• Varying degrees of progressive endothelialization take place in the
anterior chamber angle and on the iris surface.
• Three clinical variations of the syndrome have been described.
1. Chandler variant of ICE syndrome, confined to the inner corneal surface,
corneal edema may result from subnormal endothelial pump function.
2. Essential iris atrophy variant of ICE syndrome
3. The Cogan-Reese variant shows multiple pigmented iris nodules.
• ICE syndrome becomes apparent most commonly in middle-aged females
and is almost always unilateral.
• Penetrating keratoplasty and endothelial keratoplasty are treatment
options for the corneal component of this syndrome.

40. ICE syndrome

41. Peripheral cornea guttae
• (Hassall-Henle bodies) are small, wartlike excrescences that
appear in the peripheral portion of Descemet membrane.
• A normal aging change, they result from the thickening of
Descemet membrane that happens throughout life; they
occur on the posterior part of the membrane and protrude
toward the anterior chamber.
• Rarely seen before age 20, they then increase steadily in
number with age.
• When they appear in the central cornea, they are pathologic
and are called cornea guttae.

42. Cornea verticillata
• Cornea verticillata refers to linear opacities located
within the corneal epithelium that assume a
characteristic whorl-like pattern.
• The opacities are located primarily in the inferior
paracentral cornea and are not elevated.
• Their color may vary from white to brown.
• Cornea verticillata is seen in Fabry's disease and
• as a side effect from multiple systemic medications
including amiodarone, chloroquine, chlorpromazine,
indometacin, naproxen.

44. References
• External disease and Cornea, AAO, BCSC 22016-2017
• Cornea, fundamental, diagnosis and management, Mark J. Mannis,
Edward J. Holland, 4th Edition, 2017
• Kanski Clinical Ophthalmology 8th Edition, 2016

45. THANK YOU