UNIVERSITY OF GONDAR

CMHS
DEPARTEMENT OF OPTOMETRY
Advanced clinical practice I
seminar presentation On corneal Degeneration
Prepared by:Haymanot.A.(1st year MSc optometry student)
Moderaters:Dr Wossen Mulugeta (MD, Assistant Prof of ophthalmology,
Cataract ,cornea and external eye disease specialist )
Mrs.Yezinash (BSc, MSc in clinical optometry)
Learning Objectives
At the end of this presentation we will able to
• Define what corneal degeneration mean
• Classify deferent type of corneal degeneration
• Explain the histophysiology of different type of corneal degeneration
• State Clinical Presentation of of corneal degeneration
• Explain some management option of corneal degeneration
 outline
Definition
classification
Pathogenesis
Clinical Presentation
Management
Reference
Corneal Degeneration
• corneal degenerations refers to the conditions in which the normal
cells undergo some degenerative changes under the influence of age or
some pathological condition
• Changes in tissues that cause deterioration and sometimes impair
function
• No genetic predisposition
• Affect the peripheral cornea mostly
• mostly unilateral, if bilateral often asymmetric
• Usually present middle to older age
 Classification
Depending upon etiology
Involutional degenerations non involutional degeneration
-arcus senilis -Fatty degeneration
-Vogt's white limbal girdle -Calcific degeneration
-Hassal-Henle bodies -Spheroidal degeneration
-Mosaic degeneration - Salzmann‘s nodular degeneration
- Cornea farinata -Terrin's marginal degeneration
- Furrow degeneration -Amyloidosis
-Coats’ white ring
-iron lines
 Cont.
Depending upon location
central or diffuse corneal degenerations Peripheral degenerations
-Fatty degeneration - Arcus senilis
- Hyaline degeneration - Vogt's white limbal girdle
- Amyloidosis - Hassal-Henle bodies
- Calcific degeneration -Terrien's marginal degeneration
-Salzmann's nodular degeneration - snail furrow degeneration
-iron lines
- Coats’ white ring
Involutional (Age related )degeneration
Arcus senilis
• The most common peripheral corneal opacity
• It refers to an annular lipid infiltration of corneal periphery
• occurring 60 percent of patients between 40 and 60 years of age and in
nearly all patients over the age of 80
• Sometimes, similar changes occur in young persons (arcus juveniles) which
may or may not be associated with hyperlipidemia
• Asymptomatic and usually occur bilaterally
• Unilateral corneal arcus is a rare condition associated with contralateral
carotid artery disease or ocular hypotony
 cont.
signs
• Hazy white , a sharp outer border, and an indistinct central border
• denser superiorly and inferiorly
• A lucid interval is usually present between the peripheral edge of the
arcus and the limbus
• Sometimes there may be
double ring of arcus

Treatment: not required

Vogt's white limbal girdle
• It is an age-related change seen frequently in elderly people
• appears as bilateral chalky white opacities in the interpalpebral area
both nasally and temporally
• There may or may not be a clear area between opacity and the limbus
• The opacity is at the level of Bowman's membrane
• Two forms of white limbal girdle of Vogt(Type I and type II)
 Cont.
Type I
 a narrow, concentric, whitish superficial band running along the
limbus in the palpebral fissure
generally thought to represent early calcific band keratopathy
 A lucid interval appears between the limbus and the girdle
 This girdle is a degenerative change of the anterior limiting
membrane, with chalklike opacities and small clear areas resembling
the holes in Swiss cheese
 Cont.
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
Hassal-Henle bodies
• A drop-like excrescences of hyaline material projecting into the
anterior chamber around the corneal periphery
• arise from Descemet's membrane
• form the commonest senile change and result from thickening of the
Descemet membrane,
• In pathological conditions they become larger and invade the central
area and the condition is called cornea guttae
Cont.
mosaic degeneration
• Anterior mosaic degeneration, is a bilateral, usually visually
insignificant condition with a characteristic mosaic pattern
• It consists of centrally located polygonal, gray opacities at the level of
the Bowman layer that are separated by clear zones
• Histologically, the Bowman layer is indented, forming ridges, and may
be calcified
• In posterior mosaic degeneration, there are similar changes in the deep
stroma, near the Descemet membrane
 cont.
•The opacities most frequently involve the anterior 2/3
of the stroma (anterior crocodile shagreen)

•Occasion they may be found more posteriorly

(posterior crocodile shagreen)

•It resembles the central cloudy dystrophy of François

 Cornea farinata
• It is an involutional change, most likely dominantly transmitted
• the deep corneal stroma shows many subtle dotlike and comma ­
shaped opacities These opacities are often best seen in
retroillumination
• The opacities in pre-­Descemet corneal dystrophy are similar but larger
and more polymorphous
• . The deposits may consist of lipofuscin, a degenerative pigment that
appears in some aging cells
• The condition does not affect vision and has no clinical significance
Cont.
non involutional degeneration

Fatty degeneration (Lipoid keratopathy)

fatty degeneration of cornea is characterised by whitish or yellowish
deposits
The fat deposits mostly consist of cholesterol and fatty acids
 Initially fat deposits are intracellular but some become extracellular
with necrosis of stromal cells
It can be primary or secondary
 cont.

Primary lipid keratopathy

 Rare
 Not associated with vascularization

 Unilateral, focal white/yellow stromal deposits

with feathery edges
 Consisting of cholesterol, fats and phospholipids

 Lesions are avascular

 cont.
Secondary lipid keratopathy
• More common and associated with vascularization
• Occurs in vascularized corneas secondary to
• Corneal infections
• Interstitial keratitis
• Ocular trauma
• Glaucoma
• Chronic iridocyclitis
• Most common causes HS& HZ disciform keratitis
 management

• Medical control of the underlying inflammatory disease

• Argon laser photocoagulation
• Needle point cautery
• Penetrating keratoplasty
• subconjunctival and topical bevacizumab
 Band Shape keratopathy
• Band shape keratopathy consist of age related deposition of calcium
salts in Bowman's membrane, most superficial part of stroma and in
deeper layers of epithelium
• The degeneration 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
• coalesce to form a horizontal band of dense calcific plaques across the
interpalpebral zone of the cornea
 cont.
• Advanced lesions may become nodular &
elevated with considerable discomfort
due to epithelial breakdown.
 cause
The condition can be idiopathic, but the chief known causes are:
Chronic ocular disease
• Uveitis
• Interstitial keratitis
• Severe superficial keratitis
• Phthisis bulbi
 cont.
Hypercalcemia due to:
Hyperparathyroidism
Vitamin D toxicity
Milk-alkali syndrome
Sarcoidosis, and
Other systemic disorders
 cont.
Hereditary transmission primary hereditary band keratopathy, with or
without other anomalies

Elevated serum phosphorus with normal serum calcium, which

sometimes occurs in patients with renal failure
Chronic exposure to mercurial vapors or to mercurial preservatives
(phenylmercuric nitrate or acetate) in ophthalmic medications

Silicone oil instillation in an aphakic eye

 management
1. Chelation
• simple & effective for relatively mild cases and performed under
the microscope
• chemical removal of deposited calcium salts
• corneal epithelium is scraped under local anaesthesia
• 0.01 molar solution of EDTA (chelating agent) is applied to the
denuded cornea with the help of a cotton swab for about 10
minutes
• Pad and bandage is then applied for 2- 3 days to allow the
epithelium to regenerate
 cont.
2.PTK with excimer laser
3.Keratoplasty
 Spheroidal degeneration
• The condition has been reported under different names ( actinic keratopathy,
climatic droplet keratopathy, Bietti nodular dystrophy, and Labrador
keratopathy)
• typically occurs in men whose working lives are spent outdoor
• Ultraviolet exposure is likely to be an a etiological factor
• The condition is relatively innocuous but visual impairment may rarely occur
• In the primary the de‑ posits are bilateral and initially located in the nasal and
temporal cornea
• Secondary spheroidal degeneration is associated with ocular injury or
inflammation
 signs
• Histology. Irregular proteinaceous deposits in the anterior
stroma that replace the Bowman layer
• Amber-coloured granules in the superficial stroma of
the peripheral interpalpebral cornea
• Increasing opacification, coalescence and central spread

• Advanced lesions commonly protrude above the corneal

surface and the surrounding stroma is often hazy;
the conjunctiva can be involved
 management
• protection against ultraviolet damage with sunglasses
• ocular lubrication
• Superficial keratectomy
• lamellar keratoplasty
 Salzmann nodular degeneration
• raised hyaline plaques are deposited between epithelium and Bowman's
membrane with associated destruction of Bowman's membrane and the
adjacent stroma
• Uncommon, noninflammatory degeneration which is always secondary to
chronic keratopathy
• occurs as a late sequela to long-­standing keratitis
• Cause include phlyctenulosis, trachoma, interstitial keratitis or idiopathic
• The degeneration 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
clinical features
• Clinically, one to ten bluish white elevations
(nodules), arranged in a circular fashion,
are seen within the cornea
• Patient may experience discomfort due to loss
of epithelium from the surface of nodules
• Visual loss occurs when nodules impinge
on the central zone
 management
 lubrication together with control of the cause
 Manual superficial keratectomy – the lesions can often be ‘peeled’
away and the surface flattened with a diamond burr
Polymorphic amyloid degeneration
• amyloid degeneration is a bilaterally symmetric, primarily axial, and
slowly progressive corneal degeneration that appears late in life and is
characterized by amyloid deposition
• The deposits, which can resemble some of those seen in early lattice
corneal dystrophy type 3, form corneal opacities, appearing as stellate
flecks in mid- to deep stroma and as irregular filaments
• The opacities are gray to white and somewhat refractile, but they
appear translucent in retroillumination
• The intervening stroma appears clear, and vision is usually normal
cont.
Senile furrow degeneration
 furrow degeneration is seen in older patients with corneal arcus and
refers to an appearance of peripheral thinning in the lucid interval of
the arcus
 True thinning can eventually occur in the affected area and should be
considered by the cataract surgeon, particularly with placement of the
clear corneal incision, because of the risk of wound leak
 The corneal epithelium is intact There is no inflammation,
vascularization, or potential for perforation
 Vision is rarely affected, and no treatment is required.
con.t
Terrien’s marginal degeneration
It is noninflammatory, slowly progressive thinning of the peripheral
cornea
 usually bilateral but can be asymmetric
Males and females are affected equally, with men affected slightly
more frequently
 The cause of this condition is unknown
 Initially presenting in the superonasal area, the thinning spreads
circumferentially; in rare cases, it involves the central cornea or
inferior limbus
 clinical presentation
Symptoms
• Affected patients are usually asymptomatic until the thinning results in
increased astigmatism and subsequent reduction in vision
• appears primarily in those older than 40  years
• Bowman’s layer and the corneal lamellae may split
 Cont.
Signs
• Peripheral thinning associated with superficial
vascularization (followed by stromal thinning) &
lipid deposits central to the thinned edge
• Pseudotrygia in long standing cases

Terrien marginal degeneration with a fine vascular

pannus (black arrow), superior thinning (red arrow), and lipid deposits (green arrow)
 Cont.
• Sever astigmatism
Flattening of peripheral thinned cornea, with steepening of the
corneal surface 90° away from the midpoint of the thinned area.
This pattern usually results in high ATR or oblique astigmatism
• Ruptures in the Descemet membrane
 can result in acute corneal hydrops or even a corneal cyst
• Spontaneous perforation
 is rare can easily occur with minor trauma
 management
Surgical correction
• is indicated when perforation is imminent or when marked
astigmatism significantly limits vision
• Crescent-­shaped lamellar or full-­thickness corneoscleral patch grafts
may be used
• Annular lamellar keratoplasty grafts may be required in severe cases
of 360° marginal degeneration
 Coats white ring
• refers to a small (1 mm or less in diameter) circular or oval area of
discrete gray-white dots seen in the superficial corneal stroma
• iron-containing fibrotic remnants of a metallic foreign body
• the condition causes no symptom and no require treatment
 Iron deposition
• Most iron lines are related to abnormalities of tear pooling due to
ocular surface irregularities
• Can see these lines only by using red-free or diffuse illumination with
a cobalt-­blue filter before instilling fluorescein
• The deposit includes
Ferry line
Fleischer ring
Hudson-Stahli line
Stocker line
LASIK line
 Reference

1. Kanski’s Clinical Ophthalmology eighth edition

2. Basic and Clinical Science Course in External Disease and
Cornea
3. Comprehensive clinical ophthalmology fourth edition
4. Corneal degeneration pdf
thank you!!!