ONH - CONGENITAL OPTIC NEUROPATHIES

Question 1

what is ON hypoplasia?

Answer 1

abnormally small ONH characterized by a reduced number of optic nerve fibers.

Question 2

who gets ON hypolasia?

Answer 2

• seen in children whose mother either had an endocrine disorder (DM) or abused drugs (quinine, alcohol, LSD) during pregnancy.

Question 3

symptoms of ON hypoplasia?

Answer 3

vision loss ranging from mild to sever (20/20 - NLP)

Question 4

signs of ON hypoplasia?

Answer 4

1. unilateral or bilateral
2. small disc (double ring sign)
3. severe – roving eye & min pupillary rxn

Question 5

tx for ON hypoplasia?

Answer 5

• no tx
• refer to low vision specialist

Question 6

what is morning glory disc syndrome?

Answer 6

congenital malformation of the disc

Question 7

who gets morning glory disc?

Answer 7

• females who have midline facial defects & forebrain anomalies

Question 8

symptoms of morning glory?

Answer 8

severe vision loss in the afflicted eye(s)

Question 9

signs of morning glory disc?

Answer 9

• enlarged funnel shaped optic nerve with overlying glial tissue (glial hyperplasia).
• BV leave the nerve in spoke-like pattern
• ON surrounded by elevated pigment
• secondary serous RD

Question 10

complication of morning glory disc?

Answer 10

• CNVM
• serous RD

Question 11

tx for morning glory disc?

Answer 11

• no tx
• refer to low vision

Question 12

what is optic nerve drusen?

Answer 12

congenital anomaly which result in drusen (hyaline bodies) buried in the nerve fiber layer surrounding the optic nerve.

Question 13

symptoms of ON drusen?

Answer 13

asymptomatic or can have enlarged blind spot

Question 14

signs of ON drusen?

Answer 14

• bilateral
• ONH will look swollen with possible blurred margins
• hyaline material may look like refractile clumps

Question 15

how to dx/ddx ON drusen from actual edema?

Answer 15

• B-scan
• FAF
• OCT

Question 16

what is optic nerve pit?

Answer 16

• gray, white, or yellow oval or orund depression in the optic nerve.

Question 17

symptoms of optic disc pit?

Answer 17

asymptomatic unless serous macular detachment forms.

Question 18

signs of optic disc pit?

Answer 18

• temporal gray-white depression of the optic nerve which can range from 0.1-0.7 DD in size.
• possible serous detachment

Question 19

tx for optic disc pit?

Answer 19

• asymptomatic – monitor yearly.
• serous RD – observe if not in the macula & laser photocoagulation if in the macula & monitor monthly.

Question 20

what is tilted disc syndrome?

Answer 20

it is characterized inferonasal tilting of the optic disc

Question 21

where is the neural tissue concentrated in tilted disc syndrome?

Answer 21

• concentrated at the superior and superior-temporal aspect
• deficient at the inferior & inferior-nasal aspect

Question 22

what is the most common VF defect in a tilted disc?

Answer 22

• superior bitemporal defects

Question 23

what is tilted disc syndrome associated with?

Answer 23

high myopia

Question 24

signs of Tilted disc syndrome?

Answer 24

• bilateral (common)
• inferonasal ON tilt
• situ inversus

Question 25

associations of tilted disc syndrome?

Answer 25

• CNVM
• staphyloma
• lacquer cracks
• bitemporal superior VF defect