Corneal Dysgenesis

Question 1

Absence of visible cornea, unilateral or bilateral. Superior and inferior lids are fused. Globe not visualized. Microphthalmia is present. No eyebrow is present.

Answer 1

Cryptophthalmos

Question 2

Flat cornea. Less than 30Ds. Inherited as dominant or recessive. Seen in sclerocornea or microcornea.

Answer 2

Cornea Plana

Question 3

Small cornea. Diameter is less than 10mm. Eye is normal or may have microphthalmos.

Answer 3

Microcornea

Question 4

What systemic issues are associated with microcornea?

Answer 4

Rubella Syndrome

Ehlers-Danlos Syndrome

Question 5

Bilateral enlargement of the cornea beyond 13 mm in diameter. Cornea is clear. High myopia and astigmatism. 90% of cases are sex-linked

Answer 5

Megalocornea

Question 6

What other disease do you want to test for in megalocornea patients?

Answer 6

Congenital Glaucoma

Question 7

May occur at birth or develop in early newborn period. Malformed chamber angle. Diffuse corneal edema w/ inc. IOP. Enlarged cupping, rupture of Descemet’s membrane

Answer 7

Congenital glaucoma

Question 8

What are the ocular symptoms of congenital glaucoma?

Answer 8

1. Photophobia
2. Tearing
3. Blepharospasm

Question 9

What are the differential diagnosis for congenital glaucoma?

Answer 9

1. Forceps injury
2. Corneal end. dys.
3. IK
4. Cong. Rubella Syn.

Question 10

Thickenened, curved, centrally displaced anterior border ring of schwalbe’s line. Found more temporally than nasally. Collagenous ridge central to limbus. Found in 15% of normal eyes

Answer 10

Posterior Embryotoxon

Question 11

Iris strands extend across the angle to insert into prominent schwalbe’s ring.

Answer 11

Axenfield rieger anomaly and syndrome

Question 12

About 50% of axenfield anomaly patients will develop ?

Answer 12

Juveile glaucoma

Question 13

Autosomal dominant disorder, bilateral, prominent schwalbe’s ring, iris strant attachments, hypoplasia of iris.

Answer 13

Rieger’s Anomaly

Question 14

Rieger’s Anomaly + skeletal anomalies

Answer 14

Rieger’s Syndrome

Question 15

Glaucoma is found in what percentage of rieger’s anomaly pts?

Answer 15

60%

Question 16

What are the associated systemic findings of rieger’s anomaly?

Answer 16

1. dental, skull, facial anomalies
2. chromosomal anomalies
3. down syndrome

Question 17

Localized, crater defect on the posterior corneal surface; concave toward AC. VA not affected

Answer 17

Circumscribed Posterior Keratoconus

Question 18

Anteriorly displaced lens resulting in Peripheral anterior synchiae, causing secondary glaucoma.

Answer 18

Peter’s anomaly

Question 19

What ocular defects are associated with peter’s anomaly?

Answer 19

Glaucoma

Microphthalmia

Question 20

What systemic diseases are associated w/ peter’s anomaly?

Answer 20

1. Cleft palate
2. Congenital heart defects
3. Skeletal anomalies
4. Craniofacial Dysplasia

Question 21

Congenital anomaly; bilateral opacities of peripheral, central or entire cornea. Tisue is supposed to become stroma, but becomes scleral instead.

Answer 21

Sclerocornea