AED - Corneal Degenerations and Deposits - Week 10 Flashcards
Are corneal degenerations typically familial or non-familial? Are they early or late-onset?
Non-familial
Usually late onset
Are corneal degenerations symmetrical or asymmetrical? Are they uni- or bilateral? Do they occur centrally or peripherally?
Asymmetric, unilateral, and can be central or peripheral
Define corneal degeneration and note what it is characterised by (3).
Changes to the corneal tissue due to inflammation, age, or systemic disease
Characterised by a deposition of material, thinning of the tissue, or vascularisation
What is band keratopathy, what is it composed of, how does it appear, what colour and in what region (3)?
A band of grey/white deposits in the sub-epithelial region, bowmans layer, and/or the anterior stroma
List three general causes of band keratopathy and note hich one is rare.
Idiopathic
Age-related
Hereditary
List three ocular conditions that can result in band keratopathy. Are they common?
Chronic ocular inflammation
Silicone oil in the eye after ocular surgery
Longstanding glaucoma
Common
List three metabolic conditions that can cause band keratopathy. Are they common?
Hypercalcaemia
Gout
Chronic renal failure
Uncommon
List 3 symptoms of band keratopathy.
May develop surface irritation
Blurred vision
Glare
List a sign of band keratopathy.
Grey/white opacities in the anterior cornea
Do symptoms of band keratopathy arise immediately?
Is asymptomatic initially
Where on the cornea is band keratopathy opacities generally confined to? Are there any clear areas?
Interpalpebral area - clear zone at the limbus
What may opacities of band keratopathy contain and what may these possibly be? Explain these structures.
May contain holes, possibly nerve endings
They become elevated and nodular.
Is it easy or difficult to mistake band keratopathy?
Difficult
What is the treatment for mild band keratopathy (2)?
Artificial tears or bandage contact lenses for comfort it necessary and vision is unaffected
Identify the underlying cause and treat if able or refer
What is the treatment for band keratopathy with poor comfort and affected vision (4)?
Referral needed
-chelating agent after removing epithelium to manually remove calcium
-superficial (manual) or phototherapeutic keratectomy
What counsel may need to be given to band keratopathy patients?
Counselled that it is likely to recur if the underlying cause remains
Describe phototherapeutic keratectomy for treating band keratopathy. Explain its advantage over manual superficial keratectomy.
Laser thechnique to remove the corneal epithelium
Designed to minimise tissue removal and reduce surgical trauma
Is salzmanns nodular degeneration common or rare? Is it inflammatory? Does it progress quickly?
Rare, non-inflammatory, and slow progression
Is salzmanns nodular degeneration typically uni- or bilateral?
Usually bilateral, but can be unilateral
In what age and gender is salzmanns nodular degeneration most common?
Middle-aged women
Describe what salzmanns nodular degeneration looks like, including texture, colour (2), flatness, and composition. What is it sometimes described as?
Smooth, blue/white, elevated nodules sometimes described as avascular pannus
Made of fibrocellular material
Describe in detail the composition of salzmanns nodular degeneration, and between what two layers it occurs.
Irregularly arranged collagen fibrils with hyalinisation between the epithelium and bowmans layer
List three conditions associated with salzmanns nodular degeneration. What do these three have in common?
Associated with chronic keratopathy
-vernal keratoconjunctivitis
-trachoma
-interstitial keratitis
List 5 symptoms of salzmanns nodular degeneration.
Surface discomfort
FB sensation
Increased lacrimation
Photophobia
Blurred vision
Is salzmanns nodular degneration always symptomatic?
No, can be asymptomatic
What happens to the epithelium overlying a nodule in in salzmanns nodular degeneration?
Atrophies
What colour are the nodules in salzmanns nodular degeneration? Are they single or multiple lesions? Are they raised or flat? Where on the cornea can they be found?
Single or multiple grey-white elevated lesions found anywhere on the cornea
What can sometimes be found at the base of nodules in salzmanns nodular degeneration?
Possible iron deposits
List three differential diagnoses for salzmanns nodular degneration.
Phyctenule
Band keratopathy
Spheroidal degeneration
List two treatment options for salzmanns nodular degeneration.
Usually tear supplements for comfort
Ocassional manual removal, or PTK, or at worst a lamellar or penetrating graft
Can salzmanns nodular degeneration recur with removal of the nodules?
May recur
Is terriens marginal degneration common or rare?
Rare
Is terriens marginal degneration uni- or bilateral? Does it cause pain? Does it progress fast or slow?
Bilateral (can be unilateral), painless, and slowly progressive
What is terriens marginal degneration? What is its aetiology? What age and gender does it most commonly affect?
Progressive thinning of the peripheral corneal stroma
Unknown aetiology
Most commonly affects older males
List three symptoms of terriens marginal degneration. Explain what happens initially (3).
No pain
No redness
No photophobia
Initially asymptomatic with periodic irritation, then decreasing vision
What occurs as a result of corneal thinning in terriens marginal degneration?
Irregular astigmatism
What can generally be seen with terriens marginal degneration (colour, composition, and transparency) and where does it usually begin? What eventually happens?
Fine white punctate stromal lipid opacities, usually beginning superiorly
Coalesces and progresses to mild superficial vascularisation (pseudopterygium)
How does the thinning pattern of terriens marginal degneration progress?
In a circumferential pattern
Is the overlying epithelium intact in terriens marginal degneration?
Yesd
Is there a clear zone in terriens marginal degneration (aside from the central cornea)? What can often be found on the leading edge?
Separated from the limbus by a clear zone
Leading edge often has yellow lipid deposits
What does terriens marginal degneration ultimately result in?
A peripheral corneal gutter, with the cliff end facing the central cornea
What is there a higher risk of with terriens marginal degneration?
Perofration potential
What magnitude of astigmatic changes can terriens marginal degneration cause?
> 10D
List three differential diagnoses for terriens marginal degneration.
Pellucid marginal degeneration
Pannus/pterygium
Other peripheral ulcerations
List 4 treatment options for terriens marginal degneration.
Corneal topography
Pachymetry
Astigmatic correction
Keratoplasty for poor vision
Is spheroidal degeneration common or rare?
Rare
Is spheroidal degeneration uni- or bilateral?
Can be either
What kind of people does spheroidal degeneration generally affect?
People who work outdoors (high UV, extreme temperature, dry environment etc)
Which gender does spheroidal degeneration affect more?
M>F
What is the primary form of spheroidal degeneration? Are there any other forms?
Primary form affects the cornea (corneal form)
Can also affect the conjunctiva
What associated structure may be present with spheroidal degeneration?
Pterygium
List three symptoms of spheroidal degeneration.
Surface irritation
Burning
FB sensation
How does spheroidal degeneration appear, including location, size, shape, colour, composition, and layer found in.
Interpalpebral, small, globular, yellow-brown, proteinaceous droplets in bowmans layer and superficial stroma
What is the surrounding corneal stroma of spheroidal degeneration lesions like?
Often opaque
Does spheroidal degeneration begin centrally then peripherally or vice versa, or everywhere at once?
Begins peripherally and progresses centrally
What can advanced lesions of spheroidal degeneration look like (2)?
Become nodular and elevated
List 4 treatment options for spheroidal degeneration.
Advice on UV protection
Initially, ocular lubricants for comfort
Referral for epithelial debridement - superficial keratectomy
Occasionally penetrating keratoplasty
Is polymorphic amyloid degeneration common or rare?
Relatively uncommon
Is polymorphic amyloid degeneration uni- or bilateral?
Bilateral
Is polymorphic amyloid degeneration harmful?
Innocuous
When does polymorphic amyloid degeneration typically occur?
After the age of 50
How does polymorphic amyloid degeneration appear, including appearance, shape, composition, and layer found in.
Varying sizes of refractile, punctate, comma-shaped and filamentous amyloid deposits throughout the stroma
What slit lamp technique is best to see polymorphic amyloid degeneration?
Retroillumination
Does polymorphic amyloid degeneration have any systemic associations?
No
What is the treatment for polymorphic amyloid degeneration?
None required
What layer of the cornea are polymorphic amyloid degeneration lesions typically found
Mid to deep stroma
What are corneal deposits due to? What are two key features of them?
Abnormal accumulation of material in the cornea
Key features are depth and pigmentation
Is corneal arcus common or rare?
Very common
Is corneal arcus uni- or bilateral?
Usually bilateral
What is corneal arcus (composition), where does it come from, and in what two layers is it most prominent?
Lipid deposits in the cornea from the vasculature
Most prominent near bowmans layer and descemets membrane
Define corneal senilis. What is it rarely associated with?
Universal in those over 80 years old
Rarely associated with abnormal blood lipids
Define corneal juvenilis. What may it be due to? If unilateral, what should be checked for and on which side?
May be due to hyperlipidaemia in those under 40 years of age
If unilateral, need to check for carotid disease on the uninvolved side
What can corneal juvenilis occur in?
Schnyders crystalline dystrophy
What is a symptom of corneal arcus?
Asymptomatic
Where do corneal arcus lesions begin (2) and which direction do they later extend?
Lipid deposits begin inferiorly and superiorly and later extend laterally
Is there a clear zone with corneal arcus?
Yes, at the limbus, thinner in old people
What is the treatment for corneal arcus (2)?
No treatment required unless a referral is needed to check blood lipids
Is lipid keratopathy common or rare?
Relatively uncommon
Is lipid keratopathy uni- or bilateral? What region of the eye can it affect (3)?
Unilateral, may be central, peripheral, or diffuse
What form of lipid keratopathy is less common, and what features does it have? Is it uni- or bilateral?
Primary form
Has features of corneal dystrophy
Describe the secondary form of lipid keratopathy, including colour, composition, edge appearance, blood supply, clear zone, and layer found in.
White/yellow corneal stromal lipid deposits
Have feathery edges and are separated by a clear narrow zone from corneal stromal neovascularisation
What two conditions may the secondary form of lipid keratopathy be associated with?
Previous HSV or HSK
In what case can vision be affected in lipid keratopathy?
If located centrally
What is the treatment for lipid keratopathy and why?
Topical steroids to reduce neovascularisation
Is vogts limbal girdle common or rare? Which age group?
Very common in patients >45 years of age
Is vogts limbal girdle uni- or bilateral?
Bilateral
What does vogts limbal girdle look like? What colour, region of the eye (2), and shape?
Chalky white deposits along the nasal and temporal limbus, in the inter-palpebral fissure, crescent-shaped
What does histology suggest of the deposits found in vogts limbal girdle (2) and from this what may it be related to?
Either elastoid or calcified deposits
-possible relation to band keratopathy
What is a symptom of vogts limbal girdle?
Asymptomatic
Which layer are the deposits of vogts limbal girdle found in?
They are sub-epithelial
Are deposits found in vogts limbal girdle connected to the limbus (2)?
They may or may not be separated from the limbus
What is the treatment for vogts limbal girdle?
No treatment necessary and totally benign
Is vortex keratopathy uni- or bilateral? Is it symmetrical?
Bilateral and symmetrical
What colour do deposits in vortex keratopathy have? How are they arranged and in what layer? How does it extend?
Golden-brown deposits in the corneal epithelium arranged in a curvi-linear fashion and extends outward in a swirl (whorl-shaped)
Is the limbus spared in vortex keratopathy?
Yesd
Where do vortex keratopathy deposits begin and as what?
Below the pupil as punctate deposits
What a natural cause of vortex keratopathy? What is the mode of inheritance and what kind of disease is it? What else can cause it? Give the most common as an example.
Fabrys disease, an x-linked lipid metabolism disorder
Can be drug-induced
-amiodarone
Can female carriers of vortex keratopathy still show signs?
Yesd
List 6 ocular signs of fabrys disease.
Optic atrophy
Conjunctival aneurysms
Lens opacities
Papilloedema
Macular oedema
Retinal oedema
List 6 systemic signs of fabrys disease.
Hand and foot pain
Skin blemishes
Heart attack
Stroke
Kidney disease
What is a symptom of vortex keratopathy?
Largely asymptomatic
What is required when assessing vortex keratopathy? What is the treatment (3)?
Determine the cause
Referral for opinion regarding dose if dose-induced
If fabrys is suspected, referral for diagnosis
Describe a rust ring (corneal iron deposit). What two layers will it typically be found in? What may it require?
Epithelial or stromal rust from corneal foreign body
May require further debridement with appropriate trauma management
What is the cause of hudson-stahli line? Describe it, the layer its found in, and location. What does it occur with? What treatment is required?
Idiopathic
Epithelial iron deposit just below the mid-point of the interpalpebral fissure
Occurs with advancing age
No management required
Where can fleischers ring be found and in what condition? What formation does it have? What can it assist with? What treatment is required?
Found at the base of the cone in keratoconus with a circulat formation
May assist in diagnosing keratoconus, but doesnt alter management if present
Where can stockers line be found and in what condition? What layer? What may it indicate?
Epithelial iron deposit at the leading edge of a pterygium
May indicate slowed growth of the pterygium
Where can ferrys line be found and in what condition? What layer? Are they common or rare? Are they significant?
Epithelial iron deposit at the front edge of a filtering bleb
Rare with no significance
Where can siderosis be found (what layer) and following what? What does location affect? What may be necessary and why?
Stromal iron deposits from an intraocular foreign body
Location affects management of the foreign body
Graft may be necessary to restore visiond
Where can haemosiderosis be found (what layer) and following what?
Stroml iron deposits as a result of intraocular blood (hyphaema)
Are kayser-fleischer rings common or rare? Are they uni- or bilateral?
Rare and bilateral
What is the composition of kayser-fleischer rings? What layer are they found in? What colour?
Copper deposits found in descemets membrane
Green-brown deposits
List 4 possible causes of kayser-fleischer rings.
Wilsons disease
Liver cirrhosis
Motor disorders
Non-wilsonian liver diease
What is wilsons disease? What mode of inheritance?
Autosomal recessive disease caused by ceruloplasmin enzyme deficiency
In what meridian do kayser-fleischer rings begin? Where does it extend to eventually? What technique may be necessary to see the rings in early cases?
Begins in the vertical meridian
Extends to involve corneal circumference
Early cases may need gonioscopy to see deposits
What may be associated with kayser-fleischer rings? What can this result in?
May have associated sub-capsular lenticular deposits, resulting in a sunflower cataract
What is the treatment for kayser-fleischer rings (2)?
Referral for diagnosis/management of systemic disease
-copper celating agents
Is crocodile shagreen common or rare? Is it uni- or bilateral? Is it symptomatic? In what patients is it often evident?
Uncommon
Asymptomatic
Bilateral
Often edivent in older patients
How does crocodile shagreen appear (including shape)? What layer? What is it separated by? Is it a clear corneal dystrophy?
Greyish-whitte polygonal stromal opacity separated by relatively clear areas
Is not a clear corneal dystrophy
What part of the eye is crocodile shagreen most common? Explain.
Most common anterior but can be posterior
What is the treatment for crocodile shagreen?
No management required
Is corneal farinata common or rare? Is it uni- or bilateral? Is it symptomatic?
Relatively common
Asymptomatic
Bilateral
How does corneal farinata appear? What layer? What region of the eye is it most prominent?
Minute flour dust-like white deposits that are in the deep stroma
Most prominent centrally
What is the mechanism behind corneal farinata (accumulation of what compound in what cells)? What causes this?
Cause unknown but thought to be lipofuscin accumulation in stromal keratocytes
What slit lamp technique best allows you to see corneal farinata?
Retroillumination of the iris
What is the treatment for corneal farinata?
None required
Accumulation of what compound at what layer of the eye causes corneal guttata? This is caused by what cell abnormality? What appearance does this give the endothelium?
Due to focal accumulations of abnormal collagen on the posterior surface of descemets membrane
Caused by endothelial cell abnormalities
Gives the endothelium a beaten metal appearance
What disease are corneal guttata most commonly associated with?
Fuchs endothelial dystrophy
Are peripheral guttatae related to Fuchs endothelial dystrophy? Explain.
Are visible in normal adult corneas, and unrelated to fuchs endothelial dystrophy
