TMOD (Optic Nerve)

Papilledema

BILATERAL swollen disc due to increased intracranial pressure (from intracranial mass like hemorrhage or edema, pseudotumor cerebri, inhibition of arachnoid villi, malignant HTN, venous sinus thrombosis) posterior to optic chiasm

• foster-kennedy syndrome has unilateral papilledema with contralateral optic atrophy

May so present with elevated IOP, abnormal color vision, red cap desaturation, loss of spontaneous venous pulsation, VF defect (enlarged blind spot), paton’s line (retinal folds)

May be asymptomatic or complaints of HA or diplopia (from CN 6 - elevated intracranial pressure that compress CN6 along boney petrous ridge; or CN 7 paresis)

Normal VAs (if poor VA then suspect bilateral disc edema)

Papilledema Treatment/Managaement

MRI and CT to rule out mass

CBC, FBS, ESR, CRP, ANA, ACE

Measure BP

Can tx with acetazolamide

Refer to neurology

Papilledema prognosis

Typically good if early intervention

Pseudotumor Cerebri (Idiopathic Intracranial Hypertension)

Usually in women of child bearing age (fat, fertile females)

Can be associated with CATS (contraceptives, accutane, tetracycline (mino or doxy), synthroid (Nalidixic acid)), vit A, or idiopathic

Blur, HA, nausea, color vision abnormalityes, transient vision disturbances

3 Criteria required to dx Pseudotumor Cerebri

1.Papilledema

2.Normal brain MRI/CT

3.High cerebral spinal fluid pressure on lumbar puncture (>200 in nonobese, >250 in obese pt)

Pseudotumor Cerebri Treatment/Management

D/C associated medication

Lose weight

Oral acetazolamide to reduce production of cerebral spinal fluid at the choroidal plexus

• if sulfa allergy, give furosemide

If tx is unsucessful, consider neurosurgical shunt or optic nerve sheath decompression

FU 3 weeks (if chronic)/month (if acute and no VF defect)

Nonarteritic Ischemic Optic Neuropathy (NAION)

Disc edema due to ischemia from blockage of PCA in pts >55yo

Caused by cardiovascular disorder (HTN, DM, hypercholesterolemia, smoking), amiodarone (for arrhythmia), imitrex (for migraine), vardenafil or viagra

Long term finding is optic nerve pallor

Sudden painless, unilateral vision loss and abnormal color perception

Presents with unilateral swollen optic nerve with APD, VF defect, disc at risk

Nonarteritic Ischemic Optic Neuropathy (NAION) Treatment/Management

Immediate IV steroids

STAT test for CBC and CRP and ESR (abnormal if greater than age/2 in men and age+10/2 for women)

Temporal biopsy (possible false negatives)

If testings rule out AION, discontinue steroids and take daily aspirin as prophylaxis and refer to PCP

Arteritic Ischemic Optic Neuropathy (AION)

Disc edema due to ischemia from blockage of PCA in pts >55yo

Caused by GCA (inflammation of medium AND large arteries)

Emergent condition, without tx pt can lose vision in fellow eye within 24 hours

Sudden painless, unilateral vision loss and abnormal color perception, amaurosis fugax, HA, malaise, fever, scalp tenderness, jaw claudication, weight loss

Presents with unilateral swollen optic nerve with APD, VF defect (altitudinal), disc at risk

Arteritic Ischemic Optic Neuropathy (AION) Treatment/Management

Immediate IV steroids

STAT test for CBC and CRP and ESR (abnormal if greater than age/2 in men and age+10/2 for women)

Temporal biopsy (possible false negatives)

Optic Neuritis

Frequently the first sign of MS (if not yet dx, 50% of pts will be dx with MS within 10yrs of optic neuritis)

• symptoms associated with MS include uhthoff’s phenomenon (decrease vision with increaded body temp), lhermitte’s phenomenon (electric shock down the back), internuclear ophthalmoplegia (lack of adduction on affected side contralateral nystagmus on abduction

PAIN on eye movement! (compression on EOM)

Vision loss (hours to weeks due to swollen nerve)

Unilateral swollen disc, APD, color vision and VF defect

If disc are not swollen, inflammation may be behind the globe (retrobulbar optic neuritis)

• can be confirmed with MRI

Optic Neuritis Treatment/Management

Refer to neurology for MRI (if one or more lesion, risk of MS is 72% in 15 yrs)

IV steroids for inflammation then transition to oral

FU every 1-6 months (depending on severity and response to tx)

Optic Neuritis pronosis

Visual prognosis is typically good

Toxic Optic Neuropathy

Most commonly caused by ethambutol (TB drug)

• other causes are chloramphenical (50s), digitalis, isoniazid (TB), streptomycin, lead, malnutrition, alcohol, deficiency of B1 or B12 or folate

May cause centrocecal VF defect

Acute onset of permanent reduced vision (as poor as 20/400) and dyschromatopsia

Bilateral optic nerve pallor with swelling, NFL defect, reduced contrast sensitivity, may have APD (if asymmetric damage)

Toxic Optic Neuropathy Treatment/Management

D/C offending agent

Spontaneously improve within 1 yr (but permanent visual changes may occur)

Dominant Optic Atrophy (Kjer or Juvenile Optic Atrophy)

Hereditary optic neuropathy

Most common hereditary optic neuropathy

AD around the age of 5

Slow progressive loss of vision (as poor as 20/800)

Wedge of temporal pallor

Dominant Optic Atrophy (Kjer or Juvenile Optic Atrophy) Treatment/Management

Refer for genetic counseling and low vision specialist

Leber’s Hereditary Optic Neuropathy

(dont confuse with Leber’s Congential Amaurosis)

Hereditary optic neuropathy

Pseudo optic disc edema

From mother to son via mitochondria

Onset age 10-30

Severe, acute, painless, loss of central vision in one eye and progress to fellow eye within days to months

Presents with abnormal blood vessels, optic nerve hyperemia (cause blurring of disc margin), dilated capillaries with telangiectasia, optic atrophy

Leber’s Hereditary Optic Neuropathy Treatment/Management

Spontaneous vision recovery

Refer for genetic counseling and low vision specialist

Optic Nerve Hypoplasia (Midline Dysgenesis - De Morsier Syndrome)

Congenital optic neuropathy

Reduced number of optic nerve fibers

Unilateral or bilateral (more common)

Seen in infants whose mothers with endocrine disorder (DM) or abused drugs (quinine, alcohol, LDS) while pregnant

Vision loss (mild to NLP), small disc (“double ring sign”), APD, roving eye and min pupillary rxn to light (sever cases)

Optic Nerve Hypoplasia (Midline Dysgenesis - De Morsier Syndrome) Treatment/Management

No effect tx

Refer to low vision consult

If young, MRI and endo workup

If older, no work up needed

Morning Glory Syndrome

Congenital optic neuropathy

Rare, unilateral, usually females with midline facial defects and forebrain anomalies

Severe vision loss

Enlarged funnel shaped optic nerve with overlying glial tissue, blood vessels leave the nerve in spoke-like pattern, nerve. is surrounded by pigment

May develop secondary serous retinal detachment

Morning Glory Syndrome Treatment/Management

No effective tx

Refer to low vision

Optic Nerve Drusen

Congenital optic neuropathy

Hyaline-like material (may look like refractile clumps) buried in the NFL around optic nerve

asymptomatic or enlarged blind spot

Hyperr-reflective on Bscan

May cause CNVM if there is a break in Bruch’s within an area of peripapillary atrophy

Optic Nerve Drusen Treatment/Management

MRI to differenctiate drusen vs opitic neuritis

No tx necessary if optic neuritis is rule out

Optic Nerve Pit

Congenital optic neuropathy

Asymptomatic unless serous macular detachment forms

Temporal grey-white depression of the optic nerve (0.1-0.7DD)

Optic Nerve Pit Treatment/Management

If asymptomatic, monitor annually

If serous retinal detachment, monitor

If serous macular detachment, tx with laser photocoagulation and monitor monthly

Optic Nerve Glioma

Progressive unilateral vision loss with painless proptosis

Presents with proptosis, APD, disc edema, enlargement of the optic nerve on CT scan

2 Types of Optic Nerve Glioma

1.Glioblastoma Multiforme

• rare malignant form found in adults

• leads to blindness within months and death within a year

2.Juvenile Pilocytic Astrocytoma

• most common intrinsic neoplasm of optic nerve

• dx at age 6 (benign if found in childhood, malignant if found in adults)

• associated with neurofibromatosis type 1

Optic Nerve Glioma Treatment/Management

If stable with no vision changes and no cosmetic disturbance, monitor

Otherwise, surgical excision or radiotherapy combined with chemo

Optic Disc Melanocytoma

Rare tumor seen in darkly pigmented 50yo that rarely metastasize

Usually asymptomatic

Jet-black tumor with fuzzy edges adjacent to optic nerve, can be flat or slightly elevated

Optic Disc Melanocytom Treatment/Management

If metastasis, refer to oncologist

Otherwise monitor yearly

Meningioma

Usually seen in middle aged women

Usually benign but can be malignant

Complaints of progressive unilateral vision loss with painless proptosis and color vision defects

Presents with APD, optic nerve edema then atrophy, optociliary shunt vessels, and angiomas

Tumor may compress the globe and cause EOM restriction, increased IOP, metamorphopsia

Meningioma Treatment/Management

If increased IOP, tx with hypotensives

If angiomas, tx with laser photocoagulation

Malignant tumors tx with radiation, chemo, or surgical excision

Pituitary Gland Tumor

Located in the sella turcica inferior to optic chiasm

No complaints or have reduced vision, HA, color vision/VF defect

Possible optic atrophy and APD

May have reduced libido, malaise, infertility

Bitemporal hemianopia or junctional scotoma

Bow-tie atrophy (horizontal band of pallor across ONH)

Pituitary Gland Tumor Treatment/Management

Refer to neurosurgeon for MRI, surgery, bromocriptine, radiation, or hormone replacement therapy

Pituitary apoplexy (loss of blood flow) tx with systemic steroids and surgical decompression

Pituitary Gland Tumor prognosis

Good