Ocular Disease: Lecture 10: Cornea Part 2 Flashcards
Again, What are the 7 Congenital Corneal Anomalies we discussed in class?
- Axenfeld-Rieger Syndrome
- Cornea Plana
- Microcornea
- Megalocornea
- Peters Anomaly
- Posterior Embryotoxon
- Sclerocornea
Congenital Corneal Anomalies
Posterior Embryotoxon
- Affects what % of the population?
- Uni or Bilateral?
- What kind of ridge is seen?
a. Where is it?
- 10-15%
- Usually Bilateral
- Creamy White Arcuate Ridge
a. At the Limbus and on the INNER SURFACE of the Cornea
Congenital Corneal Anomalies
Posterior Embryotoxon
- What happens to the SCHWALBE LINE?
- Associated with what 2 things?
- Treatment?
- Prominent, Anteriorly DISPLACED Schwalbe Line
- Axenfeld and Rieger’s Anomalies (100%)
- None
Congenital Corneal Anomalies
Axenfeld-Rieger Syndrome
- It’s a spectrum of anomalies that include what 4 things?
- Axenfeld Anomaly
- Axenfeld Syndrome
- Rieger Anomaly
- Rieger Syndrome
Congenital Corneal Anomalies
Axenfeld-Rieger Syndrome
- Uni or bilateral?
a. Is it symmetrical? - What other possible defects could there be?
- Family History: What genetic disorder?
- Who does it affect more, males or females?
- Bilateral Ocular Anomalies
a. Not symmetrical - Possible Systemic Defects and possible Glaucoma
- AD inheritance
- No gender predilection
Congenital Corneal Anomalies
Axenfeld Anomaly
- How common is it?
- Uni or bilateral?
a. Is it symmetrical? - How does it present? (2 things)
- % associated with GLAUCOMA?
- RARE, AD
- Bilateral
a. May be asymmetric - Posterior Embryotoxon, and Peripheral Iris Strands
- 50%
- Axenfeld Syndrome
Congenital Corneal Anomalies
Axenfeld Anomaly
- Treatment? (2)
- Monitor
2. Manage Glaucoma
Congenital Corneal Anomalies
Rieger Anomaly
- How common is it?
- Uni or bilateral?
a. Is it symmetric? - How does it present? (3)
- 3 Other things that may be possible?
- Rare (AD)
- Bilateral
a. May be asymmetric - a. Iris Hypoplasia
b. Iris Strands
c. Posterior Embryotoxon - a. Corectopia
b. Ectropion Uvea
c. Pseudopolycoria
Congenital Corneal Anomalies
Rieger Anomaly
- What 2 things might Gonioscopy show?
- % that develop Glaucoma?
- Treatment?
- a. Mild iris strands
b. may show broad patches of Anterior Synechia - 50% develop glaucoma
- Manage Glaucoma
Congenital Corneal Anomalies
Rieger Syndrome
- How common is it?
- Uni or bilateral?
a. Is it symmetrical? - In presentation, what 1 thing is always there?
- This happens plus one of two things?
- Rare (AD)
- Bilateral
a. Usually Asymmetric - Rieger Anomaly
- Dental Malformations (Hypodontia and Microdontia)
OR
Facial Malformations (Hypertelorism, Maxillary Hypoplasia, Telecanthus)
Congenital Corneal Anomalies
Rieger Syndrome
- 3 other possible Complications?
- % that develop Glaucoma?
- Treatment?
- Cardiac and Renal Anomalies, Hearing Loss, and Hydrocephalus
- 50%
- Manage glaucoma or other complications
Congenital Corneal Anomalies
Peter’s Anomaly
- How common is it?
- Inheritance pattern?
- Uni or Bilateral (% of the time)
a. Symmetrical? - What 4 things is it Associated with?
- Extremely RARE
- Sporadic Inheritance
- Bilateral (80% of the time)
a. Asymmetric - 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
Congenital Corneal Anomalies
Peter’s Anomaly
Presentation
- What happens to the Cornea?
- Anterior Chamber?
- 2 types of adhesions?
- What Defect?
- Central Corneal Opacity
- Shallow Anterior Chamber
- Iridocorneal and Lenticocorneal Adhesions
- Posterior Stromal/Descemets/Endothelium Defect
Congenital Corneal Anomalies
Peter’s Anomaly
Treatment?
- Manage Complications
* Glaucoma may be VERY DIFFICULT to Control
Corneal Ectasias
- What are the 3 we talked about?
- Keratoconus
- Pellucid Marginal Degeneration
- Keratoglobus
Keratoconus
- How common is it?
a. Inheritance Pattern? - Uni or Bilateral?
a. Symmetrical? - What is it?
- Incidence?
- COMMON
a. Minimal - Bilateral
a. May be Asymmetric - Progressive Thinning and Protrusion of the Cornea
- We aren’t sure. May be the same in males and females and possibly more in Asian Populations, but studies are contradictory and inconclusive
Keratoconus: Presentation
- What decreases gradually?
a. Uni or bilateral occurrence? - Possible History of what?
- Possible ACUTE what?
- 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) - hx of eye rubbing…may be involved in causing this
- Possible Acute decrease in Vision and Significant pain if it’s an advanced condition (HYDROPS)
Keratoconus: Signs
- What 2 things are progressive?
a. Increasingly what? - What do we see on Retinoscopy?
- What do we see on Keratometry?
- Where does the cornea steepen at?
- Thinning of what and where?
- Progressive MYOPIA and ASTIGMATISM
a. Increasingly Irregular - Scissor Reflex
- Irregular Mires
- Inferior Corneal Steepening
- Central or Paracentral Stromal Thinning
Keratoconus
- 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!!
- Fleischer’s Ring
- Munsons Sign
- Oil Drop Ophthalmoscopy (Red Reflex)
- Vogt Striae (they disappear w/pressure on the Globe)
- Rizutti’s Sign
Keratoconus
Classification: Morphology
- If it’s s Greater than 5 mm in diameter?
- If Cone Makes up 75% of the CORNEA?
- Nipple Cone
- Oval Cone
- Globus Cone
Keratoconus: Classification: Disease Evolution
- Stage 1
a. What forms?
b. How do we diagnose it? (with what exam)
c. VA? - Stage 2: What happens?
- a. Forme Fruste (Subclinical)
b. with Topography
c. 20/20 w/Spec Rx - Mild Corneal Thinning
Keratoconus: Classification: Disease Evolution
- 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. Significant Corneal Thinning
b. Voigt Striae
c. Fleischer Ring
d. Less than 20/20
e. 20/20
f. about 2-8 diopters
Keratoconus: Classification: Disease Evolution
- 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. Severe Corneal Thinning
b. Greater than 55 diopters
c. Corneal Scarring
d. Less than 20/25 with Contact Lens Rx.
e. Munson Sign
Keratoconus: Classification: Graded
- Mild
- Moderate
- Severe
- Less than 48 Diopter K’s
- 48-54 Diopter K’s
- Grater than 54 Diopter K’s
Keratoconus: Etiology
- Is it clear?
- Disease related?
- Unclear (genetics? Biochemical? Disease Related?)
- 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
Keratoconus: Topography
- Role that it plays?
- Why?
- What 2 things might it show?
- Important role
- Most Sensitive method for detecting early keratoconus
- a. Irregular Astigmatism
b. Central or Inferior Steepening
Keratoconus: Complications
- Acute Hydrops
a. What is it?
b. Influx of what? What can this do? - Treatment? (4)
- a. Rupture in Descemet
b. Influx of Aqueous into the Cornea; Can lead to RAPID PAINFUL EDEMA and can Lead to STROMAL SCARRING - a. Cycloplegia
b. Consider Bandage Contact Lens
c. Consider Topical Antibiotic
d. Saline ung 5% (muro)
Keratoconus: Treatment
- Main thing for VA?
- What other Cl’s is used and why?
- What is a relatively NEW treatment? (he talked a lot about this)
- KERAtoplasty: He talked about 2 surgeries
- What else?
- Spectacles or Soft Cl’s
- Rigid Gas Permeable: Provides Regular Refracting Surface
- Corneal Crosslinking (Relatively New)
- a. PKP: Best visual Outcome
b. DALK: Lower risk of Graft Rejection - Intra Corneal Rings (Intacs)
Pellucid Marginal Degeneration
- How common is it?
- Bi or Uni?
a. Symmetrical? - What is it?
- May Co-exist with what 2 things?
- Rare
- Bilateral
a. Asymmetric - A Progressive PERIPHERAL Thinning and Corneal Protrusion
- with Keratoconus and/or Keratoglobus
Pellucid Marginal Degeneration
Presentation
- Similar to what?
- What happens to VA’s and when does it happen?
- What else? (it’s rare)
- to Keratoconus
- Decreased VA’s in Early Adulthood
- Acute decrease and Pain (Hydrops)
Pellucid Marginal Degeneration: Signs
- What kind of Astigmatism is seen?
- 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? - What 2 things DO NOT OCCUR?
- What do we see on the Topography?
- HIGH, IRREGULAR, Astigmatism
- 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 - Fleisher Ring and Vogt Striae DO NOT OCCUR
- A Distinct Pattern
Pellucid Marginal Degeneration
Treatment
- Similar to what?
- 3 main things
- What about surgery?
a. What has been promising though?
- to Keratoconus
- Glasses, Soft Contact Lenses, and RGP’s
- Not optimistic; Keratoplasty Requires a LARGE ECCENTRIC Button
a. Corneal Crosslinking has been promising
Keratoglobus
- How common is it?
- Uni or Bi?
- Type of defect?
- What is it?
- Extremely Rare
- Bilateral
- Congenital
- Uniform Peripheral Corneal Thinning
Keratoglobus: Signs
- Globular Protrusion that involves what?
- Generalized Thinning of the Cornea
a. Where is Maximal Thinning at?
b. What is seen on the Topography? - Very Rarely develops into what?
- Very Prone to what?
- the Entire Cornea
- a. in the Midperiphery
b. Generalized Steepening - Hydrops
- to Rupture w/minimal Trauma
Keratoglobus: Treatment
- Spec Rx
a. When given? (what kind of case: mild or severe)
b. What does it help protect against? - What is given in a SEVERE CASE?
- Surgical treatment a good way to go?
- a. Mild cases
b. Against Trauma - Scleral Contact Lens
- No. It’s Problematic