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