Block 15: Lectures 4-6 Flashcards by Chelsea Vernaci

In what age group does keratoconus typically present?

Teens and 20’s

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Is keratoconus usually unilateral or bilateral?

Unilateral initially and becomes bilateral over time

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What is the cause for blurred vision in keratoconus?

Irregular astigmatism

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What is a rupture or tear in Descent’s Membrane that allows a sudden influx of aqueous into the cornea called?

Acute hydrops

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What type of reflex is seen upon retinoscopy on a keratoconus patient?

Scissor reflex

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What type of reflex is seen upon ophthalmoscopy on a keratoconus patient?

Oil droplet

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Define “Vogt Striae” that are commonly seen when examining an individual with keratoconus?

Vertical stress lines deep within stroma that disappear when pressure is applied to globe

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What is it called when the cornea takes a “cone-like” appearance that is observable from a side view or against the lower lid in downsize?
(Name the feature/sign, not the condition it may be associated with)

Munson sign

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What location/direction does the protrusion or “cone” ultimately progress to on the cornea in keratoconus?

Infero-temporal

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If present, where is Fletcher’s ring usually located on a keratoconus patient?

What filter on slit lamp allows best view of this?

Base of “cone”

Cobalt blue filter

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What are 2 tests commonly used to confirm keratoconus?

Keratometry

2. Corneal topography

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What is the difference between an individual with keratoconus who has no complaints of plain and another keratoconus individual who has pain and photophobia?

Pain and photophobia signifies a ruptured Descemet’s Membrane (“Acute hydrops”)

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Why might corneal cross-linking be used as a treatment strategy in keratoconus?

To stiffen collagen - stabilize and maybe even reverse the ectasia

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What day to day motion involving the eye should an individual with keratoconus avoid?

Eye rubbing

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What is a common keratometry reading on an eye with keratoconus?

50.00 D and above

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What type of intracorneal implants might be used as a treatment/management option for keratoconus?

Ring segments

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Which layer of the cornea becomes edematous with Acute Hydrops?

Stroma

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How long does it typically take Acute Hydrops to spontaneously heal?

2-4 months

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What can be used to draw fluid out of epithelium in Acute Hydrops?

Hypertonic saline drop

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What are 3 options that could be used to manage pain and discomfort in acute hydrops?

Cycloplegia
Bandage contact lens
NSAID

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What surgical procedure may accelerate recovery and reduce scarring in Acute Hydrops?

Injection of anterior chamber (intracameral) with gas/air

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Name the condition that involves peripheral corneal thinning from 4:00-8:00 without apical protrusion.

Pellucid marginal degeneration

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What pattern is classically seen on a topography map in an individual with Pellucid Marginal Degeneration?

Butterfly or Kissing Birds

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Although surgery is difficult in Pellucid Marginal Degeneration, what is currently the most favored technique?

Peripheral lamellar crescentic keratoplasty followed by central penetrating keratoplasty

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What is a key differentiating feature between Keratoconus and Keratoglobus?

The shape of the corneal ectasia - cone in keratoconus and globular in keratoglobus

Where is the corneal thinning and protrusion typically located in Keratoglobus?

Diffuse

Name two conditions that intrastromal ring segments and corneal cross-linking have a helpful benefit. Name one condition that they are not effective in treating/managing.

Beneficial: 1. Keratoconus 2. Pellucid Marginal Degeneration Not Effective: 1. Keratoglobus

Which condition is most difficult to manage/treat? | Keratoconus, Pellucid Marginal Degeneration, or Keratoglobus?

Keratoglobus - spectacles and contact lenses often cannot correct vision and surgery is very difficult and often ineffective

What is a genetic eye disorder in which abnormal material often accumulates in the cornea and is usually progressive? (General term)

Corneal dystrophy

How are most forms of corneal dystrophies inherited?

Autosomal dominant

Are most corneal dystrophies bilateral or unilateral?

Bilateral

What is the international classification of a corneal dystrophy based on?

The affected chromosomal loci and it's gene and mutation

What is the typical inheritance pattern in Epithelial Basement Membrane Dystrophy?

Sporadic (much more commonly than Autosomal dominant)

Where is the accumulation of material in Epithelial Basement Membrane Dystrophy?

Between basement membrane and Bowman's layer

"Maps, dots, and fingerprints" are often used to describe signs seen in what condition?

Epithelial Basement Membrane Dystrophy

Where are the intraepithelial cysts most prominent in Meesmann's Epithelial Dystrophy?

Interpalpebral zone

What is bowman's layer replaced with in Reis-Bucklers' Corneal Dystrophy?

Connective tissue bands

What feature of Reis-Bucklers' Corneal Dystrophy typically spontaneously presents during early childhood?

Recurrent corneal erosions

What is considered a less severe version of Reis-Bucklers' Corneal Dystrophy?

Thiel-Behnke corneal dystrophy

What treatment option should usually be tried before keratoplasty in Reis-Bucklers' Corneal Dystrophy?

Laser keratectomy

What makes keratoplasty less ideal and saved for last option in Reis-Bucklers' Corneal Dystrophy?

Condition can recur on the graft

How do the connective tissue bands appear in Reis-Bucklers' Corneal Dystrophy?

Coarse grey/white subepithelial deposits

What layer of the cornea are deposits found in Lattice Corneal Dystrophy?

Stroma

What abnormal material accumulates in Lattice Corneal Dystrophy?

Amyloid

What could be the cause of progressively impaired vision in Lattice Corneal Dystrophy?

Filamentous appearance of amyloid deposits across cornea

How is opacification usually treated in Lattice Corneal Dystrophy?

Penetrating or lamellar keratoplasty

What condition is characterized by white deposits resembling sugar granules that gradually coalesce?

Granular Corneal Dystrophy

What substance is accumulated in Granular Corneal Dystrophy? In what layer of the cornea?

Hyaline Stroma

What are the two types of Granular Corneal Dystrophy referred to as?

Type 1: Classic | Type 2: Avellino

What feature does Type 2 (Avellino) Granular Corneal Dystrophy have that Type 1 does not?

Hyaline + Amyloid deposits | Type 1 has Hyaline only

What is an example of an autosomal recessive corneal dystrophy that is relatively common in Iceland?

Macular Corneal Dystrophy

What material is accumulated in Macular Corneal Dystrophy?

Glycosaminoglycans

How is Macular Corneal Dystrophy usually treated?

Penetrating keratoplasty

A disorder of _______ metabolism is responsible for the deposits found in Schnyder Corneal Dystrophy?

Lipid

What material accumulates in Schnyder Corneal Dystrophy?

Phospholipids and cholesterol

What treatment is used for Schnyder Corneal Dystrophy?

Excimer/laser keratectomy or keratoplasty

Accelerated endothelial loss is called ______?

Fuchs' Endothelial Dystrophy

What is the typical inheritance pattern for Fuchs' Endothelial Dystrophy?

Sporadic

When is the blurred vision typically worse in Fuchs' Endothelial Dystrophy?

Morning

Specular reflection typically shows the appearance of what sign in earlier phases of Fuchs' Endothelial Dystrophy?

Guttata

In Fuchs' Endothelial Dystrophy, "guttata" often progress to what clinical sign?

"Beaten metal" appearance of endothelium

What effect is seen first in Fuchs' Endothelial Dystrophy, stromal or epithelial edema?

Stromal edema, causing blur

What clinical sign is apparent when Fuchs' Endothelial Dystrophy has progressed from stromal blur to epithelial?

Microcysts/bullae

What would be the cause of acute pain in later stages of Fuchs' Endothelial Dystrophy?

Rupturing microcysts/bullae causing exposed nerve endings

Why might a hairdryer be used to manage Fuchs' Endothelial Dystrophy?

To dehydrate cornea

What two surgical procedures have a high success rate in Fuchs' Endothelial Dystrophy?

1. Posterior lamellar keratoplasty | 2. Posterior penetrating keratoplasty

How would cataract surgery affect Fuchs' Endothelial Dystrophy?

Cataract surgery would make it worse due to loss of more endothelial cells

What is included in a "triple procedure" for a patient with Fuchs' Endothelial Dystrophy?

- Cataract surgery - Lens implantation - Keratoplasty

What are 3 ways to dehydrate cornea in Fuchs' Endothelial Dystrophy?

1. 5% NaCl drops/ointment 2. Decrease IOP 3. Hairdryer

What cells are abnormally developed in Posterior Polymorphous Corneal Dystrophy?

Endothelial cells

Abnormal endothelial cells have the characteristics of what type of cell in Posterior Polymorphous Corneal Dystrophy?

Epithelial

What two conditions should be watched for in Posterior Polymorphous Corneal Dystrophy?

1. Peripheral anterior synechiae | 2. Glaucoma

What are 3 appearances the endothelial lesions may have in Posterior Polymorphous Corneal Dystrophy?

1. Vesicular 2. Band-like 3. Diffuse

If opacification occurs in Posterior Polymorphous Corneal Dystrophy, what treatment is preferred?

Deep lamellar keratoplasty

What is an involutional (age-related) change to the eye that tends to affect the peripheral portion of the cornea more than the central? (General term)

Corneal Degeneration

Are corneal degenerations more often unilateral or bilateral?

Unilateral (asymmetric when bilateral)

Lipid deposits in a circumferential stromal band seen in a young patient with dyslipidemia is known as ______? (specific!)

Corneal Arcus - Arcus Juvenilis

Lipid deposits in peripheral stroma in an elderly patient without any predisposing systemic condition is known as ______? (specific!)

Corneal Arcus - Arcus Senilis/Gerontoxon

What is a key difference between corneal lipid deposits in Schnyder Corneal Dystrophy and Corneal Arcus (Sinilis)?

Location of deposits - Central in Schnyder corneal dystrophy - Peripheral in Arcus Sinilis

If an elderly patient has unilateral Corneal Arcus with no other known conditions, what should be checked for?

Carotid disease on the uninvolved side

What is the condition that presents in the majority of those over 40 years old as peripheral interpalpebral whitish crescents?

Vogt Limbal Girdle

Vogt Limbal Girdle has histological changes similar to _____?

1. Pinguecula | 2. Pterygium

Where are the deposits found in Corneal farinata?

Deep stroma, most prominent centrally

What illumination technique is best for observing Corneal Farinata?

Retroillumination

What layer of the cornea is most frequently involved in Crocodile shagreen?

Anterior 2/3 stroma

What condition is characterized by grayish-white polygonal stromal opacities separated by relatively clear spaces?

Crocodile shagreen

What condition is defined by deposits of calcium within and adjacent to Bowman's layer?

Band Keratopathy

What material is deposited in the cornea in Band Keratopathy? Which layer of the cornea?

Calcium Bowman's Layer

Where does the plaque of calcium typically begin forming in Band Keratopathy? (not referring to layer of cornea)

Nasal and temporal, extending centrally

What are two ways Band Keratopathy could be ocular-caused?

1. Chronic inflammation/edema | 2. Silicone oil in Anterior chamber

When is treatment indicated in Band Keratopathy?

When decreased vision and pain are present, due to epithelial breakdown

If treatment is needed for Band Keratopathy and the treating doctor uses EDTA during the procedure, what procedure was most likely performed?

Chelation

Other than calcium, what else could be elevated in serum levels if a metabolic defect is the cause of Band Keratopathy?

Phosphate

What forms nodules in Salzmann's Nodular Degeneration?

Hyaline

A rare autosomal recessive disorder that results in the accumulation of cystine crystals in many organs throughout the body is known as _____?

Cystinosis

What are the three forms of Cystinosis?

1. Infantile 2. Late-onset juvenile 3. Adult

Which form of Cystinosis typically does not involve renal failure?

Adult

What part of the cornea does Cystinosis start and then progress to?

Starts in anterior periphery and extends posteriorly and centrally as it progresses

How might earlier onset forms of Cystinosis affect ocular tissues in ways that late-onset might now?

Pigmentary retinopathy

What treatment is given as soon as possible after diagnosis of infantile or juvenile cystinosis?

Oral cysteamine

A rare autosomal recessive condition involving abnormal deposition of copper in the liver, brain and cornea is known as ____?

Wilson Disease

What is the most common ocular manifestation of Wilson Disease?

Kayser-Fleischer ring

What are two systemic manifestations commonly seen in Wilson Disease?

1. Liver dysfunction | 2. Parkinsonism

What layer does copper accumulate in with Wilson Disease?

Descent's membrane - around corneal limbus

Are Kayser-Fleischer rings more commonly present when there is neurologic involvement or liver involvement?

Neurologic (Greater than 90%)

What clinical finding in the lens is also associated with Wilson disease?

Sunflower cataract

What are three systemic treatments for Wilson Disease?

1. Penicillamine 2. Zinc 3. Ammonium tetrathiomolybdate

What is an X-linked recessive disorder in which an enzyme deficiency leads to tissue accumulation of a glycolipid?

Fabry disease

What is an early ocular sign that most patients develop in Fabry Disease?

Vortex keratopathy (Cornea Verticillata or Whorl Keratopathy)

Why is the diagnosis Fabry Disease often overlooked until later in life?

Early signs/symptoms often dismissed as "growing pains" | Episodic pain in hands/feet during childhood

If a diagnosis of Fabry Disease has been missed (when the individual does in fact have it), what may lead to a diagnosis when the patient reaches 30-40?

Kidney or heart problems

What medications can be used to treat Fabry disease?

Pain meds and ERT

A wedge or spoke-shaped posterior cataract is known as ______?

Fabry cataract

Block 15: Lectures 4-6 Flashcards

(113 cards)