Contact Lenses III MT 2 - Dyst&Degen

Dystrophies = primary or secondary disorders?*

Primary (not caused by another reason)

Degenerations = primary or secondary disorders?*

Secondary (from a specific insult e.g. trauma, infection, exposure)

Dystrophies = bilat or unilat?*

bilat

Degenerations = bilat or unilat?*

unilat

Dystrophies = symmetrical or asymm?*

symmetrical

Dystrophies = affect one or many layers of the cornea?*

one

Central location = Dystrophy or Degeneration?

Dystrophy

T/F - Dystrophies are inherited.

True

T/F - Degenerations involve inflammation.

True

What are other terms for EBMD?

Map-Dot-Fingerprint dystrophy
Cogan's Microcystic dystrophy

T/F - EBMD can be acquired.

True - after age 40 or from trauma

EBMD seen mostly in (males/females) and (hispanics/blacks/caucasians)?

Caucasian females

What is the most common change in EBMD?

geographic maps

What happens to the basement membrane in EBMD? Why does this happen?

Thickening of BM due to abnormal turnover, maturation, and synthesis of BM

The thickened BM in EBMD causes what effects on the epithelium?

Blocks normal migration of epithelial cells to surface; epith cells around abnormal BM do not form hemidesmosomes which results in weak adhesion of epith to BM

"Maps" in EBMD is due to?*

Thickening of BM

"Fingerprints" in EBMD is due to?*

Thickened and reduplicated BM

"Dots" in EBMD is due to?

Microcysts (trapped cellular debris)

T/F - EBMD is typically asymptomatic.

True

If EBMD Sx arises, what are the typical complaints?

FB sensation, photophobia, pain, tearing, transient bluring due to irreg epith surface

What percentage of EBMD develop recurrent corneal erosions?

10%

Tx EBMD?

Asympt = reassure & observe
No erosion = RGPs and SCLs to mask irregularities
Erosion = Bandage SCL for comfort and improved vision

Tx Recurrent Corneal Erosion?

- Cycloplegic for pain (2% homatropine)
- AB coverage (FQ)
- Epith scraping
- When healing complete: hyperosmotics, lubricants
- Stromal puncture
- PTK

T/F - Mild steroids are ok for Tx recurrent corneal erosions

True (e.g Lotemax, Alrex)

How long should you Tx recurrent corneal erosions?*

6-8 wks

T/F - Bland ointment is contraindicated for recurrent corneal erosions.

True - will seep into corneal layers and cause more swelling

T/F - Oral ABs are indicated for recurrent corneal erosions.

True - can use Tetra or Doxy PO 50-100mg BID 2 mos

What is the Tx regimen of oral Tetra or Doxy for recurrent corneal erosions? Why use these?

50-100 mg BID x 2mos
- Anti collagenase properties
- Inhibits metalloproteinases that cleave anchoring of Bowman's layer

What corneal disease occurs during the 1st 5 yrs of life and pts are asymptomatic until middle age?

Meesmans' Juvenile Epithelial Dystrophy

You are observing Christian Bale's cornea and notice a focal thickening of the BM and microcysts all over the cornea (mostly in the visual axis and mid-periphery). He doesn't complain of any Sx hence it didn't stop him from playing his role in "The Dark Knight". What is this?

Meesmans' Juvenile Epithelial Dystrophy

How is Meesmans' Juvenile Epithelial Dystrophy best viewed in the slit lamp?*

Direct focal and retroillumination

If Meesmans' Juvenile Epithelial Dystrophy is symptomatic, what kinds of Sx would you note and what causes these Sx?

- Blur - irreg astig due to vesicles
- Irritation, photophobia - when vesicles break
- Also can involve glare, photophobia, FB sensation

How do you Tx Meesmans' Juvenile Epithelial Dystrophy?

- Most are non-Sx therefore no Tx
- Bandage SCLs if vesicles break
- Erosion if occurs, treat like EBMD (epith scrape, stromal puncture, PTK)

Reis-Buckler's Dystrophy involves what layer of the cornea?*

Bowman's Layer

T/F - Reis-Buckler's typically occurs in the 1st decade of life, poor vision by age 30.

False - poor vision by age 20

You see a fishnet pattern of ring-like subepithelial opacities in Bowman's layer. What is this condition and what are these opacities composed of?

Reis-Buckler's. Made up of collagen fibrils and thickened epithelium

T/F - Reis-Buckler's can be associated with recurrent corneal erosions.

True

T/F - Reis-Buckler's can be in the peripheral cornea.

True, but starts from central cornea and progresses to periphery.

Your patient complains of spontaneous RCEs which has gotten worse over time, loss of corneal sensation, and decreased vision. In the slit lamp, you see opacities forming a honeycomb pattern. What do you suspect?

Reis-Buckler's

Tx Reis-Buckler's ?

- Bandage SCLs for RCEs
- Superficial keratectomy
- PTK
- PK (Penetrating Keratoplasty - high incidence of recurrence in donor graft)

What type of substance causes the opacities in Lattice Dystrophy?

Amyloid

Lattice Dystrophy occurs in what layer of the cornea?

Stroma

Lattice Dystrophy Type I involves (central/peripheral) opacities early on.

central

Lattice Dystrophy involves (thin/thick) lines that (thicken/thin) with age.

thin, thicken

RCEs in Lattice Dystrophy Type I are (rare/common)?*

common

How is Lattice Dystrophy Type I best seen in the slit lamp (illum and mag)?

Retroillumination, mod (16x) mag

T/F - Lattice Dystrophy Type I involves corneal areas between opacities that are clear.

True

T/F - Lattice Dystrophy Type I involves deeper stroma with age.

True

T/F - Lattice Dystrophy Type II involves many thick refractile lines in the central cornea.*

False - few refractile lines that extend to the limbus

T/F - Recurrent erosions in Lattice Dystrophy Type II are rare.

True

Lattice Dystrophy Type II is associated with what systemic conditions?

Systemic amyloidosis, Alzheimer's Disease

What is systemic amyloidosis?

Assoc w/ Lattice Dystrophy Type II. Deposition of amyloid in tissues of body, cranial & peripheral neuropathy, skin changes, OAG, pseudoexfoliation

Lattice Dystrophy Type II involves (vertical/horz/radial) lines in the (central/periph) cornea.*

radial, periph
(spares the central cornea therefore vision spared)

T/F - Both Lattice Dystrophy Types I and II are Autosomal Recessive.*

False - Autosomal Dominant

T/F - Both Lattice Dystrophy Types I and II involve erosions.

True - but common in Type I, rare in Type II

T/F - There is no systemic involvement in Lattice Dystrophy Type I.

True

Tx Lattice Dystrophy?

- Bandage Cls for RCE
- PTK
- PK

What is the most common stromal dystrophy?*

Granular Dystrophy

What are the deposits in granular dystropy made of?*

Eosinophilic Hyaline deposits

Where are the deposits in Granular Dystrophy typically seen early on?*

Anterior stroma

T/F - Stroma between opacities in early Granular Dystrophy is hazy.

False - clear, but later becomes hazy

T/F - Granular Dystrophy starts centrally and migrates to periphery

True

T/F - Late Granular Dystrophy involves coalesced opacities in the anterior stroma.

False - posterior stroma

RCEs in Granular Dystrophy is (common/rare)?*

rare

What are the Sx of Granular Dystrophy?

Glare, FB sensation, decr VAs after 5th decade of life (good VAs in early years)

Tx Granular Dystrophy?

- Superficial keratectomy/debridement (for early)
- PTK
- Bandage SCLs in RCE
- Lamellar Keratoplasty
- Penetrating Keratoplasty (high recurrence rate, occurs in periphery and migrates centrally)

Granular Dystrophy involves opacities that start from the (center/periphery) and move (centrally/peripherally).

center, peripherally

T/F - Granular Dystrophy has a high recurrence rate after PK.

True

T/F - Lattice Dystrophy has a low recurrence rate after PK.

True

A combined granular and lattice dystrophy is involved in...*

Avellino Dystrophy

What deposits are involved in Avellino Dystrophy?

hyaline and amyloid

T/F - In Avellino Dystrophy, granular changes occur first, then lattice changes occur later.

True ("Lattice Later")

What gene is involved with Avellino, Granular, and Lattice Degeneration?*

BIGH3

T/F - Macular Dystrophy is Autosomal Recessive.*

True

Macular Dystrophy is (more/less) severe than AD dystrophies.

more (both genes carry the mutation)

When is the onset for Macular Dystrophy?

1st decade of life

When is the onset for Granular Dystrophy?

1st decade of life

Your 30 y/o patient has a diffuse stromal haze with milky white opacities affecting all layers of the cornea with poor VAs. You suspect...

Macular Dystrophy

Macular Dystrophy involves what type of deposits?*

Glucosaminoglycans (MPS)

Macular Dystrophy affects what layer of the cornea?

All layers (milky white opacities of MPS)

RCEs in Macular Dystrophy is (rare/common)

common

Tx Macular Dystrophy?

- Bandage SCLs for RCE
- Tinted or cosmetic SCLs to reduce photophobia
- PTK
- PK (surgical modality of choice; need PK sooner b/c affects VAs earlier)

Recurrence after graft is (rare/common) in Macular Dystrophy.

Rare

What is the surgical modality of choice in Macular Dystrophy?*

PK

T/F - Onset of deposits in Granular, Macular, and Lattice Dystrophy starts in the 1st decade.

True

When are the onset of symptoms in the following dystrophies:
- Granular
- Macular
- Lattice

Decades:
- Granular = 3rd or no Sx
- Macular = 1st
- Lattice = 2nd

Endothelial dystrophy is characterized by what stages?

- Guttata
- Stromal or epithelial edema
- Corneal fibrosis or scarring

What is Guttata?

Excessive production of BM material which accumulates into these guttata, which projects into the anterior chamber; results in thinning or missing endoth cells over areas of guttata.

Fuch's Dystrophy = guttata (with/without) corneal edema.

with (Fuch's is a later stage of endoth dystrophy)

Endothelial Dystrophy = guttata (with/without) corneal edema.

without

Fuch's Dystrophy more common in (males/females).

Females

Fuch's Dystrophy more typical in (younger/older) people?

Older

T/F - Fuch's Dystrophy is associated with ocular HTN and closed angle glaucoma.

False - OPEN angle glaucoma

Fuch's Dystrophy is best seen with what kind of illumination?

Retro-illumination

Edema is greatest in what time of the day for Fuch's?

AM (reports "hazy" vision that improves later in the day)

T/F - Fuch's Dystrophy has an "orange peel" or "beaten metal" appearance.

True

Fuch's Dystrophy can involve (this corneal problem) in later stages.

Bullous keratopathy

Bullous Keratopathy (during Fuch's) occurs due to...

epithelial edema

Decreased VAs in Fuch's occurs due to...

cornea surface changes, subepithelial fibrotic scarring

T/F - Fuch's Dystrophy involves FB sensation but no pain.

False - does involve pain and discomfort

Tx Fuch's Dystrophy?

- Monitor early cases
- Hyperosmotics for edema
- Bandage SCLs (for RCE cause by bullae, and for discomfort)
- OK to use hyperosmotics over high water content disposable SCL
- PK (common indication, can combine w/ cataract extraction in older pts)
- Monitor pts w/ glaucoma or ocular HTN

Your patient comes in with blurry vision in AM that improves later in the day. You note multiple divots in retroillumination. What is the condition and how do you Tx?

Fuch's Dystrophy - monitor since it's just an early case

Posterior Polymorphous Dystrophy = AD or AR?

Both AD and AR

Posterior Polymorphous Dystrophy onset?

Early in life

Posterior Polymorphous Dystrophy progression?

non or slowly progressive

You note the following on your patient's cornea - what do you suspect?
- Abnormal endoth cells
- Areas of thickened Descemet's with gray-white circular rings/vesicles
- Diffuse stromal and epith edema
- Peripheral anterior synechiae

Posterior Polymorphous Dystrophy

Posterior Polymorphous Dystrophy is best seen in what illum?

focal and retro-illum

Posterior Polymorphous Dystrophy Sx?

Slowly progressive, VAs good, usualy no Sx.

If Sx = blurry vision, edema (ranging from mild to bullous keratopathy)

Posterior Polymorphous Dystrophy Tx?

- Most pts no Sx & remain stable.
- Hyperosmotics for edema
- Bandage SCLs for pain mgt of bullous keratopathy
- PK
- Treat elevated IOPs

Corneal degenerations assoc w/ aging typically are (central/peripheral) and usually visually (significant/insignificant).

peripheral, insignificant

Acute corneal degenerations assoc w/ ocular insult are typically due to...

bacterial keratitis

Chronic corneal degenerations assoc w/ ocular insult are typically due to...

recurrent uveitis

T/F - Corneal degenerations assoc w/ ocular insult are typically (peripheral/central)?

central (visually impacting)

Name the four corneal degenerations related to aging changes.

Arcus Senilis
Limbal Girdle of Vogt
Crocodile Shagreen
Corneal Farinata

What is the most common peripheral corneal opacity?

Corneal arcus

What is the theory behind corneal arcus?

Lipids leak from limbal blood vessels

Corneal arcus is seen in __% of those over 70 and __% of those over 80.

75%, 100%

Systemic hyperlipidemia is associated with what corneal degeneration?*

Corneal arcus (men under 50)

Your patient presents with arcus on the left eye, but not on the right. What does this mean? What course of action is next?*

Possible carotid occlusive disease; side with arcus is normal. Order an MRI/ultrasound.

If your corneal arcus pt is under 40 years old, what is the course of management?

Refer for serum cholesterol measurement

Over __% of Dr. T's patients questioned to admitting having elevated cholesterol levels.

80%

Where is the Limbal Girdle of Vogt localized? What shape, color?

Interpalpebral zone @ 3:00 & 9:00, symmetrical. White arcuate opacity.

What degeneration is an early form of calcific band keratopathy?

Limbal Girdle of Vogt Type I

Limbal Girdle of Vogt Type I is (separated from/connected to) the limbus, and Type II (is/is not) connected.

separated from, is

T/F - both types of Limbal Girdle of Vogt require treatment.

False - no Tx necessary for either

T/F - Limbal Girdle of Vogt Type II is the degeneration of elastic collagen in the epithelium.

False - in Bowman's Layer.

You see polygonal white patches separated by clear intervals at the level of Bowman's; looks like a mosaic/cracked ice. The patient is asymptomatic. What is this?

Crocodile shagreen

Corneal Farianata = fine, powder-like, punctate gray-white opacities in what layer of the cornea?

Deep stroma, pre-Descemet's

Where is Corneal Farianata found (location and depth)?

Posterior stroma, exclusively in the interpalpebral region

T/F - Corneal Farianata is a progressive condition.

False

Corneal arcus = dystrophy or degeneration?

Degeneration

Salzmann's Nodular Degeneration is caused by...

Non-specific corneal response to chronic insult (phlyctenulosis, trachoma, keratitis, cornea inflammation)

Salzmann's Nodular Degeneration is typically (central/peripheral).

Peripheral (or mid-peripheral)

Salzmann's Nodular Degeneration is more in (men/women).

Women

What is the pathologic process involved in Salzmann's Nodular Degeneration?

Hyaline plaques form between epith and Bowman's, then epith thins and Bowman's is destroyed.

T/F - Salzmann's Nodular Degeneration typically affects VAs.

False - only unless in visual axis

Tx Salzmann's Nodular Degeneration?

- Superficial nodules: Superficial keratectomy & PTK
- Lesions extending to mid-stroma: PK (recurrence possible)

Lipid Degeneration is in what layer of the cornea?

Stroma

T/F - Lipid Degeneration Type I is associated with vascularization.

False - Type II is assoc w/ vascularization

Lipid Degeneration is typically central or peripheral?

either

Lipids leaking from corneal vessels as a result of corneal insult is characteristic of...

Lipid Degeneration

Lipid Degeneration Tx?*

Stop the deposition of lipids, esp towards pupil - treat the primary disease. Also control inflammation w/ topical corticosteroids.

T/F - Lipid Degeneration is associated with hyperlipidemia.

False - lipid metabolism in these pts is normal

What is the cause of Mooren's Ulcer?*

Unknown etiology, possibly Type III immune hypersens rxn

T/F - Mooren's Ulcer involves pain but no inflammation.

False - there IS inflammation

T/F - Mooren's Ulcer starts peripherally.

True, then progresses centrally, circumferentially, and posteriorly

T/F - Mooren's Ulcer affects both the epithelium and stroma.

True (epith breakdown and stromal melt)

T/F - Mooren's Ulcer involves vascularization.

True

T/F - Mooren's Ulcer does not involve corneal perforation.

False - perf is possible since cornea is thinned

Which type of Mooren's Ulcer is more devestating?

Type II - not responsive to therapy of any kind

Which type of Mooren's Ulcer is seen in the elderly?

Type I

Mooren's Ulcer Type II = unilat or bilat?

bilat

Mooren's Ulcer Type II = painful or no pain?

Painful

Tx Mooren's Ulcer?

- DON'T manage on your own
- Topical ABs
- Cycloplegic
- Topical steroids
- Systemic steroids
- Immunosuppressive agents
- Serious cases: conj or corneal resection, tissue adhesive
- Bilat cases: systemic dz workup

You see bilateral gradual thinning of superior and inferior cornea, forming a "gutter." You also see lipid deposition and vascularization. What is this?

Terrien's Marginal Degeneration

Terrien's Marginal Degeneration is more common in men or women?

Men (3x)

Terrien's Marginal Degeneration involves what Sx?

Decr VA due to induction of ATR astig; thinning causes flattening of vertical meridian.

Terrien's Marginal Degeneration Type I = young or old?

Young (under 20)

T/F - Terrien's Marginal Degeneration Type I involves episcleritis or scleritis.

True

Tx Terrien's Marginal Degeneration?

- Spectacle or GP Rx
- Custom shape lamellar keratoplasty ("banana" shaped lamellar donor to fit "gutter")
- Conj flap resection

What corneal degeneration involves a painless corneal thinning just peripheral to an arcus, seen typically in elderly?

Furrow degeneration

Furrow degeneration involves an intact or degenerated epith?

Intact

Furrow degeneration = inflam or no inflam?

No inflam

Furrow degeneration = perf or no perf risk?

no perf risk

Tx Furrow degeneration?

No Tx necessary

Tx Dellen?

- Tx or remove adjacent elevation if possible
- Keep dellen lubricated esp at night

What corneal dystrophy is assoc w/ peripheral anterior synechiae?

Posterior Polymorphous Dystrophy (abnormal endoth can grow across trab mesh resulting in glaucoma)

T/F - Reis Buckler's tends to have a high recurrence rate after PK.

True

Which corneal dystrophy involves treating elevated IOPs?

Posterior Polymorphous Dystrophy

Macular Dystrophy involves opacities that start from the (center/periphery) and move (centrally/peripherally).

periphery, centrally

Lattice Dystrophy Type I involves good vision until age __, Type II at age __.

40's, 60's

What typically asymptomatic degeneration involves a breakdown of Bowman's layer?

Crocodile shagreen