Ocular Disease: Lecture 10: Cornea Part 2 Flashcards

1
Q

Again, What are the 7 Congenital Corneal Anomalies we discussed in class?

A
  1. Axenfeld-Rieger Syndrome
  2. Cornea Plana
  3. Microcornea
  4. Megalocornea
  5. Peters Anomaly
  6. Posterior Embryotoxon
  7. Sclerocornea
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2
Q

Congenital Corneal Anomalies

Posterior Embryotoxon

  1. Affects what % of the population?
  2. Uni or Bilateral?
  3. What kind of ridge is seen?
    a. Where is it?
A
  1. 10-15%
  2. Usually Bilateral
  3. Creamy White Arcuate Ridge
    a. At the Limbus and on the INNER SURFACE of the Cornea
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3
Q

Congenital Corneal Anomalies

Posterior Embryotoxon

  1. What happens to the SCHWALBE LINE?
  2. Associated with what 2 things?
  3. Treatment?
A
  1. Prominent, Anteriorly DISPLACED Schwalbe Line
  2. Axenfeld and Rieger’s Anomalies (100%)
  3. None
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4
Q

Congenital Corneal Anomalies

Axenfeld-Rieger Syndrome

  1. It’s a spectrum of anomalies that include what 4 things?
A
  1. Axenfeld Anomaly
  2. Axenfeld Syndrome
  3. Rieger Anomaly
  4. Rieger Syndrome
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5
Q

Congenital Corneal Anomalies

Axenfeld-Rieger Syndrome

  1. Uni or bilateral?
    a. Is it symmetrical?
  2. What other possible defects could there be?
  3. Family History: What genetic disorder?
  4. Who does it affect more, males or females?
A
  1. Bilateral Ocular Anomalies
    a. Not symmetrical
  2. Possible Systemic Defects and possible Glaucoma
  3. AD inheritance
  4. No gender predilection
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6
Q

Congenital Corneal Anomalies

Axenfeld Anomaly

  1. How common is it?
  2. Uni or bilateral?
    a. Is it symmetrical?
  3. How does it present? (2 things)
  4. % associated with GLAUCOMA?
A
  1. RARE, AD
  2. Bilateral
    a. May be asymmetric
  3. Posterior Embryotoxon, and Peripheral Iris Strands
  4. 50%
    • Axenfeld Syndrome
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7
Q

Congenital Corneal Anomalies

Axenfeld Anomaly

  1. Treatment? (2)
A
  1. Monitor

2. Manage Glaucoma

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8
Q

Congenital Corneal Anomalies

Rieger Anomaly

  1. How common is it?
  2. Uni or bilateral?
    a. Is it symmetric?
  3. How does it present? (3)
  4. 3 Other things that may be possible?
A
  1. Rare (AD)
  2. Bilateral
    a. May be asymmetric
  3. a. Iris Hypoplasia
    b. Iris Strands
    c. Posterior Embryotoxon
  4. a. Corectopia
    b. Ectropion Uvea
    c. Pseudopolycoria
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9
Q

Congenital Corneal Anomalies

Rieger Anomaly

  1. What 2 things might Gonioscopy show?
  2. % that develop Glaucoma?
  3. Treatment?
A
  1. a. Mild iris strands
    b. may show broad patches of Anterior Synechia
  2. 50% develop glaucoma
  3. Manage Glaucoma
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10
Q

Congenital Corneal Anomalies

Rieger Syndrome

  1. How common is it?
  2. Uni or bilateral?
    a. Is it symmetrical?
  3. In presentation, what 1 thing is always there?
  4. This happens plus one of two things?
A
  1. Rare (AD)
  2. Bilateral
    a. Usually Asymmetric
  3. Rieger Anomaly
  4. Dental Malformations (Hypodontia and Microdontia)

OR

Facial Malformations (Hypertelorism, Maxillary Hypoplasia, Telecanthus)

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11
Q

Congenital Corneal Anomalies

Rieger Syndrome

  1. 3 other possible Complications?
  2. % that develop Glaucoma?
  3. Treatment?
A
  1. Cardiac and Renal Anomalies, Hearing Loss, and Hydrocephalus
  2. 50%
  3. Manage glaucoma or other complications
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12
Q

Congenital Corneal Anomalies

Peter’s Anomaly

  1. How common is it?
  2. Inheritance pattern?
  3. Uni or Bilateral (% of the time)
    a. Symmetrical?
  4. What 4 things is it Associated with?
A
  1. Extremely RARE
  2. Sporadic Inheritance
  3. Bilateral (80% of the time)
    a. Asymmetric
  4. a. Axenfeld-Rieger Anomalies
    b. Glaucoma (50%): Difficult to manage and Prognosis is Worse than Congenital Glaucoma
    c. Lens Displacement or Cataract
    d. Numerous other Systemic Anaomalies
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13
Q

Congenital Corneal Anomalies

Peter’s Anomaly

Presentation

  1. What happens to the Cornea?
  2. Anterior Chamber?
  3. 2 types of adhesions?
  4. What Defect?
A
  1. Central Corneal Opacity
  2. Shallow Anterior Chamber
  3. Iridocorneal and Lenticocorneal Adhesions
  4. Posterior Stromal/Descemets/Endothelium Defect
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14
Q

Congenital Corneal Anomalies

Peter’s Anomaly

Treatment?

A
  1. Manage Complications

* Glaucoma may be VERY DIFFICULT to Control

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15
Q

Corneal Ectasias

  1. What are the 3 we talked about?
A
  1. Keratoconus
  2. Pellucid Marginal Degeneration
  3. Keratoglobus
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16
Q

Keratoconus

  1. How common is it?
    a. Inheritance Pattern?
  2. Uni or Bilateral?
    a. Symmetrical?
  3. What is it?
  4. Incidence?
A
  1. COMMON
    a. Minimal
  2. Bilateral
    a. May be Asymmetric
  3. Progressive Thinning and Protrusion of the Cornea
  4. We aren’t sure. May be the same in males and females and possibly more in Asian Populations, but studies are contradictory and inconclusive
17
Q

Keratoconus: Presentation

  1. What decreases gradually?
    a. Uni or bilateral occurrence?
  2. Possible History of what?
  3. Possible ACUTE what?
A
  1. Gradual Decreasing Vision
    a. Unilateral usually (the other eye tends to do decrease in vision as well w/in 16 years for 50% of cases)
  2. hx of eye rubbing…may be involved in causing this
  3. Possible Acute decrease in Vision and Significant pain if it’s an advanced condition (HYDROPS)
18
Q

Keratoconus: Signs

  1. What 2 things are progressive?
    a. Increasingly what?
  2. What do we see on Retinoscopy?
  3. What do we see on Keratometry?
  4. Where does the cornea steepen at?
  5. Thinning of what and where?
A
  1. Progressive MYOPIA and ASTIGMATISM
    a. Increasingly Irregular
  2. Scissor Reflex
  3. Irregular Mires
  4. Inferior Corneal Steepening
  5. Central or Paracentral Stromal Thinning
19
Q

Keratoconus

  1. What are the big 5 signs we should know and how to explain how to see them for CLINIC?
    * See Slides 35-39 on Lecture 10!!
A
  1. Fleischer’s Ring
  2. Munsons Sign
  3. Oil Drop Ophthalmoscopy (Red Reflex)
  4. Vogt Striae (they disappear w/pressure on the Globe)
  5. Rizutti’s Sign
20
Q

Keratoconus

Classification: Morphology

  1. If it’s s Greater than 5 mm in diameter?
  2. If Cone Makes up 75% of the CORNEA?
A
  1. Nipple Cone
  2. Oval Cone
  3. Globus Cone
21
Q

Keratoconus: Classification: Disease Evolution

  1. Stage 1
    a. What forms?
    b. How do we diagnose it? (with what exam)
    c. VA?
  2. Stage 2: What happens?
A
  1. a. Forme Fruste (Subclinical)
    b. with Topography
    c. 20/20 w/Spec Rx
  2. Mild Corneal Thinning
22
Q

Keratoconus: Classification: Disease Evolution

  1. Stage 3
    a. What’s significant here?
    b. Type of Striae?
    c. Ring?
    d. VA possible w/Spec Rx?
    e. Va possible w/Contact Lens Rx?
    f. Irregular Astigmatism: About how much?
A
  1. a. Significant Corneal Thinning
    b. Voigt Striae
    c. Fleischer Ring
    d. Less than 20/20
    e. 20/20
    f. about 2-8 diopters
23
Q

Keratoconus: Classification: Disease Evolution

  1. Stage 4
    a. Severe what?
    b. Corneal Steepening: Greater than what?
    c. What else happens to the cornea?
    d. VA w/contact lens Rx?
    e. What sign?
A
  1. a. Severe Corneal Thinning
    b. Greater than 55 diopters
    c. Corneal Scarring
    d. Less than 20/25 with Contact Lens Rx.
    e. Munson Sign
24
Q

Keratoconus: Classification: Graded

  1. Mild
  2. Moderate
  3. Severe
A
  1. Less than 48 Diopter K’s
  2. 48-54 Diopter K’s
  3. Grater than 54 Diopter K’s
25
Q

Keratoconus: Etiology

  1. Is it clear?
  2. Disease related?
A
  1. Unclear (genetics? Biochemical? Disease Related?)
  2. Down Syndrome: 10-300x’s higher
    a. Eye Rubbing (46% w/chronic Blepharitis)

b. Turner syndrome
c. Ehler Danlos
d. Marfan
e. Atopy
f. Osteogenesis Imperfecta
g. Mitral Valve Prolapse

26
Q

Keratoconus: Topography

  1. Role that it plays?
  2. Why?
  3. What 2 things might it show?
A
  1. Important role
  2. Most Sensitive method for detecting early keratoconus
  3. a. Irregular Astigmatism
    b. Central or Inferior Steepening
27
Q

Keratoconus: Complications

  1. Acute Hydrops
    a. What is it?
    b. Influx of what? What can this do?
  2. Treatment? (4)
A
  1. a. Rupture in Descemet
    b. Influx of Aqueous into the Cornea; Can lead to RAPID PAINFUL EDEMA and can Lead to STROMAL SCARRING
  2. a. Cycloplegia
    b. Consider Bandage Contact Lens
    c. Consider Topical Antibiotic
    d. Saline ung 5% (muro)
28
Q

Keratoconus: Treatment

  1. Main thing for VA?
  2. What other Cl’s is used and why?
  3. What is a relatively NEW treatment? (he talked a lot about this)
  4. KERAtoplasty: He talked about 2 surgeries
  5. What else?
A
  1. Spectacles or Soft Cl’s
  2. Rigid Gas Permeable: Provides Regular Refracting Surface
  3. Corneal Crosslinking (Relatively New)
  4. a. PKP: Best visual Outcome
    b. DALK: Lower risk of Graft Rejection
  5. Intra Corneal Rings (Intacs)
29
Q

Pellucid Marginal Degeneration

  1. How common is it?
  2. Bi or Uni?
    a. Symmetrical?
  3. What is it?
  4. May Co-exist with what 2 things?
A
  1. Rare
  2. Bilateral
    a. Asymmetric
  3. A Progressive PERIPHERAL Thinning and Corneal Protrusion
  4. with Keratoconus and/or Keratoglobus
30
Q

Pellucid Marginal Degeneration

Presentation

  1. Similar to what?
  2. What happens to VA’s and when does it happen?
  3. What else? (it’s rare)
A
  1. to Keratoconus
  2. Decreased VA’s in Early Adulthood
  3. Acute decrease and Pain (Hydrops)
31
Q

Pellucid Marginal Degeneration: Signs

  1. What kind of Astigmatism is seen?
  2. What do we see happen to the cornea?
    a. How long is it?
    b. What range do we see it in?
    c. What is above this?
  3. What 2 things DO NOT OCCUR?
  4. What do we see on the Topography?
A
  1. HIGH, IRREGULAR, Astigmatism
  2. Crescent Shaped Band of Inferior Corneal Thinning
    a. 1-2 mm long
    b. 4 o’clock to 8 o’clock
    c. Corneal Protrusion above the Band
  3. Fleisher Ring and Vogt Striae DO NOT OCCUR
  4. A Distinct Pattern
32
Q

Pellucid Marginal Degeneration

Treatment

  1. Similar to what?
  2. 3 main things
  3. What about surgery?
    a. What has been promising though?
A
  1. to Keratoconus
  2. Glasses, Soft Contact Lenses, and RGP’s
  3. Not optimistic; Keratoplasty Requires a LARGE ECCENTRIC Button
    a. Corneal Crosslinking has been promising
33
Q

Keratoglobus

  1. How common is it?
  2. Uni or Bi?
  3. Type of defect?
  4. What is it?
A
  1. Extremely Rare
  2. Bilateral
  3. Congenital
  4. Uniform Peripheral Corneal Thinning
34
Q

Keratoglobus: Signs

  1. Globular Protrusion that involves what?
  2. Generalized Thinning of the Cornea
    a. Where is Maximal Thinning at?
    b. What is seen on the Topography?
  3. Very Rarely develops into what?
  4. Very Prone to what?
A
  1. the Entire Cornea
  2. a. in the Midperiphery
    b. Generalized Steepening
  3. Hydrops
  4. to Rupture w/minimal Trauma
35
Q

Keratoglobus: Treatment

  1. Spec Rx
    a. When given? (what kind of case: mild or severe)
    b. What does it help protect against?
  2. What is given in a SEVERE CASE?
  3. Surgical treatment a good way to go?
A
  1. a. Mild cases
    b. Against Trauma
  2. Scleral Contact Lens
  3. No. It’s Problematic