5 - Optic Neuropathies II

Question 1

Benign disorders of fetal vasculature
-prepapillary loops
—most arise from
—complications (2)

Answer 1

Arterial supply

Predispose CRAO, vit hem
Twisting/kinking

Question 2

Benign disorders of fetal vasculature
-congenital retinal macrovessels
—main thing to know

Answer 2

VA is normal, vessels remain competent thru-out life***

Question 3

Benign disorders of fetal vasculature
-familial retinal arteriolar tortuosity
—charaterized by

Answer 3

Marked tortuosity of 2nd/3rd order retinal arteries (normal 1st order arteries, venous system)***

Question 4

Detrimental disorders of fetal vasculature
-persistent hyperplastic primary vitreous (PHPV)
—pathophys

Answer 4

Pathologic entity resulting from abnorm persistence of fetal fibrovascular primitive stroma (hyaloid system)***

Question 5

Detrimental disorders of fetal vasculature
-persistent hyperplastic primary vitreous (PHPV)
—complications

Answer 5

Can give rise to leukocoria, RD, subret hemorrhage***

Question 6

Detrimental disorders of fetal vasculature
-persistent hyperplastic primary vitreous (PHPV)
—spectrum of anterior (5)***

Answer 6

• Cataracts
• Mittendorf dot
• Retrolental fibrous membr/stalk
• Ciliary body disruption
• Microcornea

Question 7

Detrimental disorders of fetal vasculature
-persistent hyperplastic primary vitreous (PHPV)
—spectrum of posterior (7)***

Answer 7

• Bergmeister’s pap
• Vitreoretinal veils
• Persistent fibrous stalks (post lens -> ONH)
• Vitreoretinal traction
• Retinoschisis
• RD
• Macular ectopia

Question 8

Detrimental disorders of fetal vasculature
-ateriovenous malformation (AVM)
—profile and pathology (3)***

Answer 8

Failure to develop capillary networks b/w A + V circulations

App as “bag of intestines”

Wyburn-Mason syndrome is AVM with cutaneous and intracranial abnormalities/proliferations

Question 9

Detrimental disorders of fetal vasculature
-capillary hemangioma
—profile and pathophys (3)***

Answer 9

Vasoproliferative tumor

Egg-shaped lesion

VHL disease includes ocular and intracranial findings

Question 10

Detrimental disorders of fetal vasculature
-capillary hemangioma
—useful dx clue

Answer 10

Exudates around disc***

Question 11

Inherited neuropathies
-dominant optic atophy
—ex pt complaints***

Answer 11

Things have not been clear at distance or near

Colors not vivid

Slowly been getting worse over last 4 years

Question 12

Inherited neuropathies
-lebers hereditary optic neuropathy (LHON)
—what is LHON plus

Answer 12

Involves MS-like white matter lesions in brain***

Question 13

Inherited neuropathies
-lebers hereditary optic neuropathy (LHON)
—presentation (2 main, 4 other)

Answer 13

Pseudodisc edema, hyperemia, telangiectatic vessels present at onset***
-pseudodisc edema - no stain/leakage of fluoro around disc

VF loss typically permanent***
-central/cecocentral

BCVA ranges 20/20-20/200
Painless VA loss
Sparing of pupillary function
CV may be affected first

Question 14

Inherited neuropathies
-lebers hereditary optic neuropathy (LHON)
—LHON vs Foster-Kennedy syndrome (FKS) (3)

Answer 14

LHON mimics FKS

FKS: one eye shows optic atrophy, fellow eye shows disc edema***

LHON may easily be differentiated when central VF defects are present OU***