ON 2

Question 1

T/F: Optic Nerve Hypoplasia is a unilateral condition

Answer 1

Eh, actually it can be both lol

Unilateral: likely strab & APD

Question 2

T/F: ON Hypoplasia is congenital

Answer 2

TRUE (not progressive)

Question 3

Potential causes for OH Hypoplasia

Answer 3

1. Toxicity
2. Infection
3. Ischemia
4. Idiopathic

Question 4

Expected VA for ON Hypoplasia

Answer 4

20/20 to NLP (just varies 🤷🏻‍♀️)

Question 5

Describe the nerve fiber layer in ONE Hypoplasia

Answer 5

LESS fibers (thinned retina)

Question 6

Double pigment ring sign

Answer 6

Yellow-white peripapillary halo, absence of RPE (represents size of normal ONH)

Question 7

How do retinal vessels appear in ON Hypoplasia?

Answer 7

Appears larger and enters disc centrally, may be tortuous

Question 8

Septo Optic Dysplasia

Answer 8

Rare congenital defect during embryological development

Question 9

Triad for SOD

Answer 9

1. ON Hypoplasia
2. Absence (partial or complete) of septum pellucidum/corpus colluscum
3. Hypopituitarism

Question 10

T/F: Optic Atrophy is hereditary

Answer 10

TRUE

Question 11

Which OA exhibits the best VA?

Answer 11

Juvenile AD OA

Question 12

Expected vision in Congenital AR OA?

Answer 12

Worse than 20/200

Question 13

TX for Congenital AR OA?

Answer 13

Genetic counseling and low vision/rehab

Question 14

Juvenile AD OA exhibits pallor in which quadrant?

Answer 14

Temporal

Question 15

What VF defect in Juvenile AD OA?

Answer 15

Cecocentral scotoma

Question 16

Describe the vision loss in Juvenile AD OA

Answer 16

Gradual 4-12 yo

Question 17

Juvenile AD OA involves a defect on Chromosome ___

Answer 17

3

Question 18

TX for Juvenile AD OA

Answer 18

Low vision devices, cochlear implants, genetic counseling, and routine f/u

note: neuro-imaging require to rule out other causes

Question 19

Genetic transmission of Leber’s

Answer 19

X-linked recessive
(Mitochondrial mutation in DNA)

Question 20

What defect precedes atrophy in Juvenile AD OA?

Answer 20

Blue yellow color defect

Question 21

Systemic deficits also exhibited in Juvenile AD OA

Answer 21

Hearing loss and ataxia

Question 22

Main ocular symptom of Leber’s

Answer 22

Severe vision loss (worse than 20/200)

Question 23

VF defect in Leber’s

Answer 23

Central and cecocentral scotoma

Question 24

Triad of Leber’s

Answer 24

1. Circumpapillary telangiectasia of BV
2. Pseudoedema
3. Absense of leakage on FA

Question 25

ON Glioma arise from _____ of the ON and visual pathway

Answer 25

Astrocytes

Question 26

Typical ON Glioma pt

Answer 26

Female < 20 yo