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The Nerve-Racking Diagnosis: Swollen Optic Nerve Management

ANNA K. BEDWELL, OD, FAAO

CLINICAL ASSOCIATE PROFE SSOR

INDIANA UNIVE RSITY SCH OOL OF OPTOME TRY

No financial disclosures

Optic Nerve Evaluation• Stereoscopic view essential

• Note:• C/D

• Color (healthy pink vs pale)

• Elevation

• Margins (obscured vs distinct)• Presence of PPA, crescent, hemorrhage etc.

• Insertion• Malinsertion

• Tilt

Patton’s Lines Modified Frisen Scale (for papilledema)

Grade 0: no edema

Grade 2: low ◦ Circumferential halo

◦ Nasal elevation

◦ No BV obscuration

Image: www.aao.org

Grade 1: minimal◦ Grayish C-shaped halo

◦ Temporal disc normal

◦ Disruption of NFL striations

Grade 3: moderate◦ Halo

◦ Elevation of all borders

◦ Obscuration of at least one segment of major BV leaving disc

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Image: www.aao.org

Grade 4: marked◦ Complete halo with

borders obscured 360

◦ Elevation of whole nerve head

◦ Obscuration of at least one segment of major BV on disc

Grade 5: severe◦ Grade 4 findings

plus

◦ Obscuration of all BVs on/leaving the disc

Assessing ON FunctionTesting:◦ VA

◦ Pupils

◦ Color vision

◦ Red cap saturation

◦ Visual field

◦ Checking SVP (or eliciting venous pulsation)◦ (+) indicates an absence of papilledema

◦ ~20% of normals will not have SVP/EVP -> so a (-) SVP/EVP has no clinical value

Cranial Nerve ExamCranial Nerve: Test:

CN I Olfactory identify scents

CN II Optic nerve VA, VF, pupils, color vision

CN III, IV, VI EOMs, CT

(Oculomotor, Trochlear, Abducens)

CN V Trigeminal Motor (teeth clench and palpate temporal and masseter muscles; jaw movement)

Sensory - apply dull and sharp touch to V1, V2 and V3; check for +/- corneal reflex

Cranial Nerve ExamCranial Nerve: Test:

CN VII Facial Test lid closure, smile, show teeth, puff out cheeks, wrinkle brow

CN VIII Vestibulocochlear Hearing

CN IX, X say ahhh -> check for symmetric rise in palate

(Glossopharyngeal, Vagus)

CN XI Accessory shoulder shrug

CN XII Hypoglossal stick out tongue

OCT RNFLGreat tool for diagnosing/following ONH edema but…◦ Green doesn’t always mean normal and white

doesn’t always mean swollen

◦ Prone to segmentation errors with very swollen nerves

Pseudo-papilledemaONH drusen◦ Calcified deposits

◦ Prevalence 1-2% of population

◦ Often buried

◦ Can cause elevation and blurred disc margins ◦ Pseudo-swollen appearance

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Optic disc drusenBscan – hyperreflectivity◦ Gain on low

◦ May not show up with children (calcification occurs with age)

FAF – hyperfluorescence◦ cSLO FAF > camera based FAF for buried drusen

ODD - OCT◦ Enhanced depth imaging (EDI) preferred for better visualization

of ODD

◦ Consider avg RNFL thickness measurement◦ Drusen can compresses and lead to NFL thinning/VF defect

◦ Drusen can cause mild thickening of NFL

ODD - OCT◦ According to Optic Disc Drusen Studies Consortium

(J Neuro-Ophthalmol 2018):◦ ODD always have hyporeflective (signal-poor) core

◦ Often with a hyperreflective margin, most noticeable superior

◦ Always above the lamina cribrosa

◦ Sometimes small ODD conglomerate to create hyperreflectivity

Optic disc drusen

drusen

blood vessel

conglomerates

Not to be confused with…PHOMSSeen with papilledema, ODD, tilted discs and other causes of ONH edema.

Result of herniating nerve fibers from axoplasmic stasis.

PHOMS assoc w/ papilledema disappear as ON edema subsides

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Causes of ONH edemaBilateral

-Papilledema from space-occupying mass

-Increased ICP◦ IIH, venous sinus thrombosis, intracerebral

hemorrhage, TBI

◦ Medication-induced

-Malignant HTN

-Nutritional/toxic

Unilateral

-Vascular

-Inflammatory

-Infectious

-Toxic

-Uveitis

-Compressive

-Hereditary

Case 1• CC: 41 year old Caucasian female presents for routine exam

• ROS: frontal HAs (1x/week)

• Med Hx: asthma, allergies, skin melanoma removed from cheek 20 yrs ago, scoliosis

• VA sc OD: 20/20OS: 20/20

• Pupils PERRL –APD

• EOMs Full

• VF screener Full OD, OS

• IOP 12/12 mmHg

• BP 125/78 mmHg

• Slit lamp anterior segment unremarkable

(-) EVP OD, OS

A/PAssessment◦ 41 year old Caucasian female with bilateral ONH edema (OS>OD)

◦ (+) HA, (-) tinnitus, (-) TVO, BMI 37.36

◦ +minocycline tx x 6 month, off x 3 months

◦ CN exam normal

Plan◦ Order MRI, MRA/MRV w/ and w/o contrast of the brain

◦ RTC 1 week for HVF

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MRI/MRV resultsNormal MRI/MRA/MRV

Signs suggestive for IIH

Neuro referralAvoid LP due to patient’s spinal hardware from scoliosis (would make procedure more challenging)

Monitor clinically w/o LP since visual field is clear

PLAN: Monitor q4month with OCT and VFPt instructed to not go back on minocyclineEncouraged salt reduction and diet/exercise

Baseline 2012

Initial Visit

Improves OS>OD

Papilledema• Term indicates bilateral ON swelling from ↑ICP

• Early VF loss is usually enlarged blind spot

• Can present with unilateral or bilateral CN 6 palsy

• Workup is time-sensitive

Papilledema Workup

MRI• Rule out

mass/space-occupying lesion

MRV • Rule out venous sinus thrombosis

LP• Opening

pressure

• CSF composition

ICP Dynamics• CSF is produced in the lateral ventricles• Flows through ventricles into the subarachnoid spaces

• ICP is measured via lumbar puncture into the subarachnoid space in the spine• Normal ICP 75–200 mm H2O

• Papilledema can be asymmetric• Asymmetry may be caused by difference in size of the

bony optic canal, limiting CSF flow

• Very asymmetric (>2 stages difference on Frisen scale) is rare• <4% of patients with IIH

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Idiopathic Intracranial HypertensionDiagnosis of exclusion:

◦ Elevated opening ICP on lumbar puncture (>250mm water)

◦ Normal cerebrospinal fluid biochemistry and microbiology

◦ Normal neuroimaging

Symptoms:◦ Headaches, nausea, pulsatile tinnitus, TVO

Associations:◦ Obesity

◦ Female>male

◦ Child bearing age

◦ Birth control use

◦ Medications: Tetracyclines, Isotretinoin, high dose Vit A, sudden withdrawal of oral steroids

Treatment:◦ Diuretics

◦ Weight loss

◦ CSF diversion shunts

◦ Optic nerve sheath fenestration

MRI features of IIH◦ empty or partially empty sella

◦ posterior globe flattening

◦ optic nerve tortuosity

◦ optic nerve sheath enlargement

◦ intraocular protrusion of the ONH

◦ transverse sinus narrowing

Case courtesy of Dr Dalia Ibrahim, Radiopaedia.org, rID: 29638

Idiopathic Intracranial Hypertension Treatment Trial

Acetazolamide in combo with low sodium diet resulted in decrease in papilledema, a decrease in CSF pressure and improved quality of life

Acetazolamide S/Es

**

Findings:• 9.4% (10/106) of patients with presumed IIH were

found to have CVST on MRV study• In only 1/10 was the CVST detected on MRI alone

What is CVST?A condition in which the balance between prothrombotic and prothrombolytic processes is disturbed leading to venous (blood clots) thrombosis

Symptoms:◦ Headaches (presenting sx in 70-90%)

◦ 18-38% of cases present with headaches, papilledema and visual disturbances resembling IIH

◦ Hemi-sensory disturbance

◦ Seizures

Associations:◦ Oral contraceptives

◦ Coagulation disorders, red blood cell disorders

◦ Head injury

◦ Post surgical

◦ Infection/inflammation

◦ Pregnancy

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CVST◦ Most often affects the larger sinuses (superior sagittal and lateral sinus)

◦ Can be seen with MRI, but potentially missed if MRV not performed

◦ Can be acute, subacute or chronic

◦ Can result in stroke or death

◦ Treatment: blood thinners

MRV in Papilledema WorkupMRV still debated as part of the routine papilledema workup◦ Low diagnostic yield with high cost of procedure vs missing a life-threatening

condition

◦ Particularly in those that are asymptomatic and fit the typical IIH profile

In a case of incidentally detected papilledema (aka no symptoms) and patient fits profile for IIH (overweight/obese female) without risk factors for thrombus -> MRI only (no MRV)

When in doubt…. order MRV

VITT and CVSTVaccine-induced immune thrombotic thrombocytopenia (VITT)

◦ Linked to AstraZenaca and J&J COVID vaccines

◦ likely response to adenoviral vector (AZ: chimpanzee AV 5; J&J: human AV 26)

Attributed as likely response to adenoviral vector ◦ AZ: chimpanzee AV 5; J&J: human AV 26

Of the cases reported, most common thrombotic event was CVST

VITT◦ Who is at risk?

◦ Young females (<60 years old)

◦ Incidence estimated at 4-5 per million doses

◦ Occurs ~7-10 days post-vaccination

◦ Proper diagnosis is key. These patients can’t be given heparin!◦ Tx: immunoglobulins and non-heparin anticoagulants

Causes of ONH edemaBilateral

-Papilledema from space-occupying mass

-Increased ICP◦ IIH, venous sinus thrombosis, intracerebral hemorrhage, TBI

◦ Medication induced

-Malignant HTN

-Nutritional/toxic

Malignant HTNHypertensive Emergency: systolic >180 mmHg and/or diastolic >120 mmHg

Keith-Wagner-Barker classification

Group 1: Slight constriction of retinal arterioles

Group 2: Group 1 plus focal narrowing of retinal arterioles + AV nicking

Group 3: Group 2 plus flame-shaped hemes + CWS + HYEs

Group 4: Group 3 plus papilledema

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Toxic/NutritionalCan cause low-grade edema (or normal) -> ON pallor/atrophy◦ More often bilateral than unilateral

Predilection for the papillomacular bundle ◦ Causing centrocecal scotoma, reduced VA and color vision deficiency

Toxic◦ Medications

◦ TB drugs (ethambutol, rifampin, isoniazid, streptomycin)

◦ Linezolid, chloramphenicol

◦ Isotretinoin (acne)

◦ Amiodarone

◦ Methanol

◦ Heavy metal exposure

Toxic/NutritionalNutritional often deficiency in vitamin B12 and folic acid◦ Eating disorders, gastric bypass surgery, homelessness

◦ Cuban Epidemic Optic Neuropathy (1991-1993) ◦ Caused visual loss, peripheral neuralgias, and other neurologic sxs in over 50,000 people

◦ Combination of nutritional deficiency (B1, B6, B12 and folic acid), alcohol and tobacco use

Where history comes in to play…◦ Medical, surgical, medications/dosage, lifestyle, recent vaccinations

Unilateral ONH edema-Vascular◦ NA-ION

◦ Giant cell arteritis

◦ Papillophelbitis, CRVO

-Inflammatory ◦ Demyelinating

◦ Autoimmune

◦ Post-vaccination

-Infectious◦ Bacterial – cat scratch, syphilis, TB, Lyme

◦ Parasitic - Toxoplasmosis, toxocariasis, DUSN

◦ Viral – HSV, VZV, CMV, HIV

-Toxic

-Uveitis

-Compressive◦ Neoplastic

◦ Thyroid

-Hereditary◦ Leber’s optic neuropathy

Case 2• CC: 54 year old Caucasian male presents for urgent care, black spot in vision OD noticed upon

awakening this morning

• Ocular Hx: “kinked optic nerve” OS 2 years ago

• Med Hx: GI upset (takes Prilosec prn)

• VA sc OD: 20/25 NIPH OS: 20/70 NIPH

• Pupils PERRL –APD

• EOMs Full

• VF screener Full OD, inferior altitudinal defect OS

• IOP 12/12 mmHg

• BP 135/80 mmHg

• Slit lamp anterior segment unremarkable

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A/PAssessmentUnilateral ONH edema – presumably acute NA-ION OD

◦ Pt denied jaw claudication, scalp tenderness, neck pain or weight loss

◦ Denied use of ED meds, amiodarone

◦ No vascular co-morbidities

◦ Screener VF full, BCVA 20/25

Unilateral ONH pallor from past NA-ION OS

◦ Inferior altitudinal defect

Plan◦ Order labs: ESR, CRP and CBC (all came back in normal range)

◦ Order sleep study

◦ RTC 3 weeks for f/u and HVF

DDX: Foster-Kennedy SyndromeFoster-Kennedy Syndrome: ◦ Compressive optic atrophy in one eye

◦ Other eye ON edema caused by ↑ICP

Pseudo Foster-Kennedy Syndrome:◦ Acute NA-ION with ON edema

◦ Other eye atrophy from prior NA-ION

1 week later…• CC: presents with worsening vision OD

• VA sc OD: 20/100 NIPH OS: 20/70 NIPH

• Pupils PERRL –APD

HVF 24-2

New superior altitudinal defect

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Case 2 Final Outcome (6 months post onset of sxs)

Final VA:20/50 OD, OS

Sleep study showed mild sleep apnea

NA-ION

Hayreh

Acute ischemia of the ONH caused by hypoperfusion◦ Non embolic event

◦ Blood flow to the anterior ONH is via the short posterior ciliary arteries

Incidence of 2.3 per 100,000 persons age 50 and older

Sudden painless monocular loss of vision◦ Sxs typically onset in morning

Features of NA-AIONInitial visual acuity

◦ Half better than 20/64

◦ 1/3 progress, 1/3 stable, 1/3 worsen over first month◦ Can improve for up to 6 months

+APD

Visual Field defect◦ Most commonly inferior altitudinal

Risk factors: HTN, DM, hyperlipidemia, sleep apnea◦ PDE 5 inhibitors (tx erectile dysfunction)

~20% risk of occurrence in contralateral eye

Tx controversial -> ? High dose oral steroids (SS Hayreh only advocate), ASA◦ No proven tx

Recurrence in same eye rare, but possible… Old NA-ION ODAcute NA-ION OS

6 months later….

New NA-ION OD

Multifactorial diseaseVascular risk factors

Nocturnal Hypotension◦ BP drops during sleep

Disk at Risk◦ Crowding of optic nerve fibers

◦ Ischemia -> axoplasmic flow stasis -> swollen axons -> compression of surrounding capillaries

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Incipient NA-ION• Subset of NA-ION• Swollen, hyperemic optic nerve

• Asymptomatic, VA and VF normal

• Often caught on routine exam

• (+)Risk factors for NA-ION

• 75% spontaneously resolve, 25% progress to full-blown NA-ION• study of 54 cases, median time for conversion 5.8 weeks (SS Hayreh, 2007, Ophthlamology)

• Treatment: address/workup vascular risk factors, ? brimonidine, ? oral steroids

Arteritic ION• Presenting s & sxs similar to NA-ION • Swollen and pale ON, “chalky” (vs hyperemic swelling)• A-ION more likely to report pain• Initial visual acuity is poor, >20/400• Possible preceding sxs of amaurosis fugax

• Workup = Lab testing, temporal artery biopsy• ESR, CRP, CBC• 3-5% false negatives with biopsy, “skip lesions”

• Central bright spot sign on MRI (Reymond P, Am J Neuroradiol, 2017)

• ON enhancement

Giant Cell ArteritisVasculitis affecting small to medium-sized arteries• F>M• Age >50, avg 72• Historically thought to be exclusive to Caucasians• Found equal incidence of biopsy proven-GCA between AAs and Caucasians (Guerner, 2019, JAMA)

Symptoms: jaw claudication, neck pain, scalp tenderness, fatigue, malaise, weight loss, HA

Subclavian bruits, diminished pulses, aortic regurgitation, or Raynaud’s phenomenon are found in patients with large vessel disease

Treatment IV steroids followed by oral steroids (chronic, x years)• New tx FDA approved Actmera used in conjunction with steroids (allows for lower dose)

Arteritic vs Non-arteriticA-ION NA-ION

Patient demographics Elderly Age >40

Visual acuity Severely reduced Mild-Mod reduced

Complaint of pain (+) (-)

ONH appearance Pallored swelling Hyperemic swelling

Other eye C/D Variable Small, crowded

Symptoms of GCA +/- (-)

Clinical criteria most strongly suggestive of GCA:1) Jaw claudication2) CRP >2.45 mg/dl3) Neck pain4) ESR > 47 mm/hour

Posterior (P-ION)• Retrobulbar ischemia

• Sudden painless visual loss (VA and/or VF) in one or both eyes with a normal optic disc and fundus appearance

• Diagnosis of exclusion

• Arteritic (GCA), Non-arteritic or Perioperative/Surgical

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Case 336 year old AA female

CC: painless, progressive vision loss OS over 2 weeks, smudge in central vision

Med Hx: type II DM x 5 years, HTN, hyperlipidemia

Meds: Lantus, Glipizide, Metformin, Atorvastatin

BCVA: 20/20 OD, CF OS

Pupils: grade 2 APD OS

EOMs: full, denies pain

VF FDT: full OD, general depression OS

HRR: 10/10 OD, unable to see plates OS

IOP: 19/17 goldman

Ant seg: unremarkable

A/PAssessment36 year old AA female with ONH edema and acute vision loss OS, suspect optic neuritis

+ APD

Generalized VF defects

Elevated, swollen L optic nerve with hemorrhage

Vascular risk factors for NA-ION, but age atypical

(-) pain

PlanOrder MRI of brain w/ and w/o contrast to rule out demyelinating disease

3 weeks later

BCVA OS improved to 20/30

ONH swelling resolved

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5 months later

BCVA OS improved to 20/20

Mild ONH pallor appreciable OS

Optic Neuritis Acute optic nerve inflammation presenting with vision loss +/- pain on eye movement◦ Most often linked to demyelinating disease

F>M

Younger age compared to NA-ION pts; typically 20s-40s

+APD

2/3 have normal ONH appearance (aka retrobulbar)

Visual field loss ◦ most common centrocecal scotoma

Optic NeuritisGood prognosis for vision recovery◦ Most fully recover by 6 months

Sxs typically worsen over the first 1-2 weeks, then gradually recover over weeks

Rarely bilateral presentation◦ Except in Asian population, 30% of cases bilateral

Diagnosing demyelinating dzFinding of white matter lesions on MRI is the strongest predictor for development of MS◦ Abnormal MRI (1 or more lesions) -> 15 year risk of MS is 70%

◦ Compared to normal MRI (no lesions) -> 15 year risk of MS is 25%

Early MS Sxs (variable):◦ Numbness in extremities or face

◦ Fatigue

◦ Balance problems

◦ Bladder problems

◦ Uhthoff’s phenomenon: worsening of neurologic symptoms with a rise in body temperature

Optic neuritis treatment trial• Compared oral steroid, IV followed by oral and placebo.

• Steroids do not improve end VA in optic neuritis

• IV (3 days) followed by oral steroids (11 days) help to delay MS sxs up to 5 years

ON vs NA-ION

OPTIC NEURITIS

• Age 20s-40s

• 2/3 retrobulbar, if swelling mild w/o hemorrhage

• Most common VF defect: centroceccalscotoma

• (+) pain, dull, worsened with eye movement

NA-ION

• Age >40

• hyperemic, swollen disc +/- hemorrhage

• Presence of vascular risk factors

• Most common VF defect: altitudinal

• Small C/D ratio in contralateral eye

• (-) pain

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Atypical Optic NeuritisMost common cause of optic neuritis is demyelinating disease◦ But can also be autoimmune or infectious (bacterial, viral etc)

Optic neuritis workup should always include MRI of brain, may also necessitate a serology workup particularly if atypical findings…

Red flags for atypical ON: ◦ Unusual pt demographics◦ Bilateral signs or sxs◦ Vitreous and/or chorioretinal involvement

◦ Neuroretinitis

◦ Does not follow typical clinical course

Unilateral ONH edema-Vascular◦ NA-ION

◦ Giant cell arteritis

◦ Papillophlebitis, CRVO

-Inflammatory ◦ Demyelinating

◦ Autoimmune

◦ Post-vaccination

-Infectious◦ Bacterial – cat scratch, syphilis, TB, Lyme

◦ Parasitic - Toxoplasmosis, toxocariasis, DUSN

◦ Viral – HSV, VZV, CMV, HIV

-Toxic

-Uveitis

-Compressive◦ Neoplastic

◦ Thyroid

-Hereditary◦ Leber’s optic neuropathy

Infectious Optic NeuritisBacterial Causes:◦ Cat scratch disease

◦ Bartonella henselae, gram -

◦ Transmitted by scratch, lick or bite from infected cat (usually kitten)

◦ Flu-like syndrome 2-3 weeks prior to ocular sxs

◦ Posterior segment involvement characterized by neuroretinitis (optic nerve edema + macular star)

◦ Syphilis◦ STD

◦ Treponema pallidum, spirochete

◦ ON involvement would be highly suggestive for neurosyphilis

◦ Oral or genital sores (primary), rash on palms or soles of feet (secondary)

◦ Lab work: FTA-ABS, VDRL, RPR

Infectious Optic NeuritisBacterial Causes:◦ Tuberculosis

◦ Mycobacterium tuberculosis

◦ Characterized by caseating granulomas

◦ Optic nerve can be involved in ocular TB

◦ Lyme disease◦ Borrelia burgdorferi, spirochete

◦ Transmitted by tick bites

◦ Optic neuritis, neuroretinitis can occur in the early and disseminated stages

◦ Regarded as CNS involvement; IV antbiotic therapy

Infectious Optic NeuritisViral Causes:◦ Herpes family: HSV, VZV, CMV

◦ +/- ARN, PORN

◦ HIV

Parasitic ◦ Toxoplasmosis, toxocariasis, DUSN

Post vaccine optic neuritis (non-infectious)◦ Can occur hours to weeks after the shot

Case 4• 46 year old Caucasian male in for routine exam with mild near blur OS

• Ocular Hx: trauma to OS, car accident at age 10, went through windshield, “left eye went out of socket” had reconstructive surgery on orbit, hx of poor vision OS since accident

• Med Hx: healthy, no meds

• BCVA: 20/20 OD, OS

• Pupils: tr-1 APD OS

• Color: HRR 9/10 OD, OS

• VF FDT: Cl OD, diffuse depression OS

•IOP: 15/16 mmHg

•Slit lamp: ant seg unremarkable

•Dilation: normal, ONHs 0.25/.3

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Case 4A: Hx of injury OS with decreased screening visual field

P: return in one month for HVF 30-2

Patient lost to follow up until….

2 years later…• CC: In for routine exam, no D/N’ blur, wants updated glasses, no change in health or meds

since last visit

• BCVA 20/20 OD, OS

• Pupils 3+ APD OS

• EOMs Full OU

• IOP 14/14 mmHg

• Visual Field FDT screener Cl OD, defects OS

• Color HRR 10/10 OD, 6/10 OS

Con’t• External: proptosis OS

• Ant seg: unremarkable

• DFE ONH: see photos

macula: clear and flat OD

mild ERM OS

periphery: normal OU

• Exophthalmometry: Base 100: 15 OD, 18.5 OS

Visual Fields HVF 30-2

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A/PAssessment

49 year old Caucasian male with unilateral disc edema… suspect compressive lesion

hx of L eye trauma

+ L Proptosis

+ L APD

Significant L VF defects

Elevated, swollen L optic nerve

PlanOrder MRI of brain and orbits w/ and w/o contrast

MRICompressive optic neuropathy

MRI Report

*Compared to head CT dated 9/7/2006

Compressive optic neuropathyInjury to the optic nerve by an extrinsic lesion

Compression can cause ON swelling, cupping or pallor

Any lesion or injury that produces mass effect can cause compression:◦ Tumors

◦ Orbital inflammation

◦ Grave’s/thyroid

◦ Orbital pseudotumor

◦ Traumatic

◦ Infectious

Thyroid Eye DiseaseHyper > hypo or euthyroid

High association with smoking

Severity scale:

0 No signs or sxs

1 Only signs (lid retraction, stare, lid lag)

2 Soft tissue involvement

3 Proptosis

4 EOM involvement

5 Corneal involvement

6 Sight threatening complications (ON involvement)

Thyroid Optic NeuropathyOptic nerve compression◦ Signs: APD, color vision defect, loss of VA, VF defect, ON

edema or pallor

◦ Neuroimaging shows crowding at the apex from muscle enlargement

◦ Treated with oral or IV steroids or orbital decompression surgery

◦ Tepezza (FDA approved Jan 2020) new treatment for TED◦ Reduces muscle and fatty tissue swelling to reduce proptosis

CT scan Radiopedia.org

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Case 531 year old AA female

CC: constant spot in L eye vision x 1 day

Med hx: type I DM (hospitalized one month ago with diabetic ketoacidosis), HTN, anemia

Meds: humalog, amlodipine, metoprolol

VA: sc 20/20 OD, OS

Pupils: sluggish, tr APD OS

EOMs: full OD, OS

VF screener: nonspecific defects, no pattern OD, OS

IOP icare: 22/20

BP: 125/80 mmHg

A/PSummary:31 year old AA female with uncontrolled type 1 DM and bilateral ONH edema and moderate NPDR OU• Hx suggestive for diabetic origin, recent hospitalization for

ketoacidosis, BS was in 600s and quickly stabilized

Plan: Obtain hospital records, must consider neuroimaging to rule out causes of papilledema

Received records. During hospitalization patient had brain MRI, head CT and lumbar puncture (all WNL). Since workup was so recent no further testing ordered. F/u in one month.

Case 5, 4 months laterCC: presents for urgent care, L red eye x 1 week

VA: sc 20/20 OD, OS

Pupils: sluggish, no APD

EOMs: full OD, OS

VF screener: clear OD, OS

IOP GAT: 18/19 mmHg

BP: 118/78 mmHg

Ant seg: WNL OD, temporal wedge of injection OS (episcleritis)

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Resolved ONH edema OD and OS

Diabetic Papillitis• Unilateral or bilateral ON edema

• Type 1 DM more often the type 2

• Often linked to rapid ↓ in BS

• +/- retinopathy

• ON swelling can be present for weeks-months

• Causes minimal impairment (if any) to VA/VF• self-limiting, resolving without need for treatment

• Diagnosis of exclusion• Bilateral ON edema must be treated as papilledema until proven otherwise

• Exact mechanism unclear…but may be similar to NAION?• disc edema persists longer in DP than in NAION

Case 631 year old AA female complaining of progressive vision loss OS, started 3 months ago, vision has declined substantially in last week

• Medical hx: healthy, no meds

• BCVA: 20/25 OD, HM OS

• Pupils: 2+ APD OS

• Color HRR: 7/10 plates OD, unable OS

• FDT VF: cecocentral defect OD, unable OS

• GAT: 16/16 mmHg

• Ant seg: unremarkable OU

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A/PSummary:31 year old AA female with probable LHON• +Fhx of vision impairment of multiple family members

• Brother has genetically confirmed LHON

Plan: No approved tx for LHONHelped pt to investigate active clinical trialsSet up LV consult

One week later…BCVA OD dropped to 20/70

Hereditary ONMitochondrial

◦ Lebers Hereditary Optic Neuropathy

Autosomal dominant◦ Dominant Optic Atrophy (Kjer’s)

Autosomal recessive ◦ Rare, manifests at young age

LHON◦ Presents with acute unilateral, painless, visual loss

◦ Followed by other eye involvement weeks to months later

◦ Sxs develop in late childhood/early adulthood (teens-20s)

◦ Mitochondrial inheritance◦ Mitochondrial DNA carried in eggs (female carriers)

◦ Affects M>>F

◦ Variable expression

LHON◦ Papillo-macular bundle is effected first and damaged the most

◦ resulting in initially severe GCC thinning and central or cecocentral VF loss

◦ Optic nerve: initially may appear normal or mildly swollen (pseudo-edema), hyperemic → weeks later pallor sets in

◦ Visual outcome varies

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Wrap UpSwollen optic nerve(s) may seem daunting…

Diagnosis can be narrowed down with a good history, exam, visual fields and careful ONH evaluation

Recognize the red flags for vision-threatening or life-threatening conditions