Optic Nerve Anomalies and Diseases

Papillophlebitis

"big blind spot syndrome"

Retrobulbar neuritis

"pt sees nothing, doctor sees nothing"

Neuroretinitis Inflammatory optic neuritis Perioptic neuritis

3 types of atypical optic neuritis

scleral RING of optic disc neuroretinal RIM RETINAL nerve fiber layer REGION of parapapillary atrophy RETINAL hemorrhages

5 R's for optic nerve evaluation

emergency (tx will make a difference to outcome)

A-AION is an ophthalmic______________.

Occult GCA

A-AION with only ocular signs (no systemic signs or symptoms)

A-AION (aka giant cell arteritis)

Autoimmune process with vasculitis that can involve the internal and external carotid arterial tree; occlusion of the ophthalmiic and PCA that leads to sudden loss of vision

stage 5

Frisen scale stage of papilledema for dome-shaped protrusions and obliteration of the optic cup

stage 4

Frisen scale stage of papilledema for elevation of entire nerve head + total obscuration of disc on a segment of a major blood vessel; macular star possible

stage 3

Frisen scale stage of papilledema for obscuration of all borders and increased diameter of ONH; obscuration of one or more segments of major blood vessels leaving disc

stage 2

Frisen scale stage of papilledema for obscuration of all borders w/ elevation of nasal border; loss of cup

stage 1

Frisen scale stage of papilledema for obscuration of nasal border of disc w/ normal temporal disc margin

A-AION

Loss of vision is typically more severe in A-AION/NA-AION

MRI

MUST be done before a lumbar puncture to test for IIH

prodromal signs and sx (25%) -blurring, orbital pain, flashing/flickering vision Sudden decrease in vision upon awakening

NA-AION signs and sx

tilted optic disc syndrome

OHN exits at oblique angle; often bilateral, presents with inferior nasal crescent (fuch's coloboma)

multiple sclerosis

Patients with optic neuritis but with a normal brain MRI have a 16-22% chance of developing _________ w/i 5 yrs

HA scalp tenderness jaw ache Amaurosis fugax flashing/flickering diplopia color vision disturbances pale, swollen optic disc

S/Sx of A-AION

NA-AION

Small vessel disease of ON vascular supply indicating an imbalance in the pressure-perfusion ratio

false (begin steroids immediately to decrease arterial inflammation)

T/F: you should wait for the positive biopsy in GCA to begin treatment

MS

Uhthoff's sign, Lhermittes' symptom, and Romber's sign are associated with:

IIH

_____ can be secondary to medications such as tetracycline, oral contraceptives, accutane, etc.

high myopia

_________ may prevent or retard papilledema

pale (pallor>cupping=non-glaucomatous)

a ______ neuroretinal rim increases the likelihood for a non-glaucomatous optic neuropathy

AION (anterior ischemic optic neuropathy)

a disease of sudden, unilateral, painless loss of vision associated with optic disc edema; no identifiable inflammatory cause, demyelination, or mass

males age 15-30 females age 20-40

age of onset of Leber's hereditary optic atrophy

macular star

associated with really severe or chronic papilledema

neuroretinitis

associated with viral infections and cat scratch fever

Diabetic papillopathy

atypical form of mild NA-AION; characterized by diffuse diabetic microangiopathy of the optic disc

multiple sclerosis

autoimmune inflammatory disease of CNS, attacking myelin

2.2 mm

average vertical diameter for a large disc is greater than:

<1.5 mm

average vertical diameter for a small disc is less than:

absolute

beta zone parapapillary atrophy is associated with a relative/absolute scotoma:

bilateral, painless loss of vision over a few months dense sentral scotomas involvement of fellow eye concurrently or w/i months optic disc edema w/ circumpapillary telangiectasia that does not leak smoking increases risk males affected more than females

characteristics of Leber's hereditary optic atrophy

poor vision nystagmus at birth very pale optic discs

characteristics of congenital autosomal recessive optic atrophy

gradual loss of vision to 20/40-20/60 b/w ages 4-12 pallor variable no nystagmus

characteristics of juvenile autosomal dominant optic atrophy

papillitis + retinal edema macular star exudates serous detachment

characteristics of neuroretinitis

VF defects severely decreased VA at risk for retinal detachment

complications for morning glory syndrome

variable effects on VA and VF

complications of circumpapillary staphyloma

strabismus VF defects, decreased VA systemic abnormalities (CHARGE syndrome)

complications of coloboma of the ONH

decreased VA if macula involved (rare)

complications of myelinated nerve fiber

VA varies from 20/20 to NLP VF defects vary pupil abnormalities--APD possible if unilateral strabismus if unilateral nystagmus if bilateral

complications of optic nerve hypoplasia

Central serous retinopathy retinoschisis lamellar macular holes arcuate VF defects (correlate to pit location)

complications of optic nerve pits

myopic and astigmatic w/ oblique axis lacquer cracks and CNVM (rare) pseudo-bitemporal VF defect (does not respect vertical meridian)

complications of tilted optic disc syndrome

glial tissue

congenital gray tissue or membrane often seen on nasal aspect of disc; may block full view of optic disc or only a section

optic nerve hypoplasia

congenital or developmental, non-progressive; associated w/ multiple systemic and neurological abnormalities, especially if bilateral

myelinated nerve fiber

congenital retinal white opacification w/ soft feathered or frayed edges, tend to fan out

corkscrew vascular loop

congenital unilateral vessel loop arising from disc and returning to disc

central VF defect with +RAPD

critical sign of compressive optic neuropathy:

growth hormone

deficient ______________ is found in up to 93% of SOD/ON hypoplasia patients

Lhermittes' symptom

development of sudden transient electric-like shocks spreading down the body when pt flexes head forward

small discs

discs at risk for NA-AION

rim width

distance b/w border of disc and position of blood vessel bending

idiopathic intracranial hypertension (IIH, aka pseudotomor cerebri)

elevated ICP w/o clinical, laboratory, or radiological evidence of an intracranial space occupying lesion (can be caused by sudden increase in obesity)

optic atrophy

final endpoint of any disease process that causes axon degeneration in the retinogeniculate pathway

A-AION

in _____________, the fellow eye is very likely to be affected within 1-2 wks without treatment

7-10 days (vision remains stable for a pd of time, then recovery begins)

in optic neuritis, the maximum loss of vision occurs by _______ then stabilizes

Paton lines

indicated chronic papilledema (had some time in the past)

perioptic neuritis

inflammation of the dura sheath as it covers the ON entering the optic canal (VERY RARE)

Optic neuritis

inflammation of the optic nerve that will decrease nerve conductivity and will therefore produce ocular sx

optic nerve pits

larger-than-normal ONH w/ crater-like defect often found in temporal margin; focal, round, oval depression associated w/ peripapillary atrophy

urgent MRI (intracranial mass or hemorrhage until proven otherwise) check BP to r/o malignant HTN

management plan for papilledema

50 yrs

most AION occur in pts over the age of:

retrobulbar neuritis

most common form of optic neuritis associated with demyelinating disease (MS)

high myopia and amblyopia

myelinated nerve fibers are associated with:

tinnitus HA vomiting loss of consciousness

neurological symptoms of papilledema

50-200 mmH20

normal range for CSF pressure

transient visual obscurations (5-30 secs of blurred vision) enlarged blind spots diplopia secondary to CN6 palsy

ocular symptoms of papilledema

small disc, 1/2 size of normal, pale

opthalmoscopic appearance of optic nerve hypoplasia

Foster-Kennedy syndrome

optic atrophy with contralateral papilledema (optic atrophy on side of tumor, papilledema in other eye)

papilledema

optic disc swelling due to proven increased ICP (URGENT)

unilateral

optic nerve pits are usually unilateral/bilateral:

Atypical optic neuritis

optic neuritis in those not 20-50 yrs old

Variable VA decreased contrast sensitivity color desaturation visual field defects (all types) vitreous cells optic disc edema deep retinal exudates or macular star

optic neuritis signs and sx

minimal loss of vision (20/30) no RAPD

optical sx of papillophlebitis

majority (90%) of pts recover useful visual function w/i 6-8 wks

prognosis of optic neuritis

tilted optic disc syndrome

pseudo-bitemporal VF defect is associated with:

Romberg's sign

pt falls when they close their eyes

septo-optic dysplasia (SOD)

rare congenital defect during embryological development that causes optic nerve hypoplasia (growth hormone deficiency = short stature)

coloboma of the optic nerve head

results from incomplete closure of the embryonic choroidal fissure; larger-appearing ONH w/ unusual vascular pattern

No benefit of ONDS (optic nerve decompression surgery) compared w/ careful follow-up in pts w/ NA-AION

results of ION decompression treatment trial

Fastest recovery w/ IV steroids, but no difference at 1 yr

results of ONTT

no clear benefit found for either steroid therapy or optic canal decompression surgery (decision to treat should be made on an individual basis)

results of international optic nerve trauma study

opticociliary shunt vessels

retinochoroidal venous collaterals shunt blood from high pressure in choroidal circulation to exit the eye by the vortex vein; a sign of chronic swelling of the disc

persistent hyaloid artery

short stub of vessel projecting into vitreous congenital, most are bloodless

bilateral papilledema visual field loss HA nausea/vomiting tinnitus 5% have no sx

signs and sx of IIH

compressive optic neuropathy

slowly progressive visual loss (rarely acute); can be caused by optic nerve glioma, meningioma, or any intraorbital mass

MRI (dependent on # of lesions found)

strongest predictor of MS according to ONTT

HA vertigo nervousness

systemic Sx of Leber's hereditary OA

polymyalgia rheumatica

systemic association of A-AION

Situs Inversus

temporal branches of CRA and CRV merging more nasally from disc before returning to normal temporal location

Rim width

the ISNT rule applies to:

vision loss dyschromatopsia eye pain

triad associated with optic neuritis

weight loss Diamox (CAI) Diuretics shunts

tx for IIH

oral prednisone

tx that was discontinued in ONTT b/c only showed increase rate of new attacks of optic neuritis

scotomas (no metamorphopsia)

typical amsler grid defect in optic nerve disease

papillitis w/ exudates hx of URI, post-infectious, or other systemic disease Not consistent with MS More often bilateral

typical characteristics of atypical optic neuritis

gross differences (desaturation of all colors)

typical color comparison defect in optic nerve disease

red-green (usuallly very evident)

typical color vision defect in optic nerve disease

papillitis

typical optic neuritis where optic disc is swollen and hyperemic

retrobulbar neuritis

typical optic neuritis with acute inflammation of the optic nerve posterior to the lamina cribosa (optic disc appears normal)

normal recovery

typical outcome of photo stress test in optic nerve disease

NA-AION

typically more unilateral condition b/w A-AION and NA-AION:

circumpapillary staphyloma

unilateral anomaly with normal ON lying at the base of a staphyloma

Papillophlebitis (optic disc vasculitis)

unilateral optic disc edema with dilated tortuous veins and possibly retinal hemorrhage

morning glory syndrome

very rare, usually unilateral, more common in females; larger than normal ONH with unusual vascular pattern (radiate from disc, abnormally straight)

gradual, painless, bilateral loss of vision central scotomas

visual characteristics of toxic/nutritional optic neuropathy

rim width (larger beta zone=thinner rim)

width of beta zone inversely correlates with:

Uhthoff's sign

worsening of neurological symptoms associated with overheating of the body