Differential Diagnosis

of DiscEdema
Moderated by: Dr. Parul Ichhpujani
Presented by: Dr. SahilThakur
 1860: Albrecht von Graefe first reported his observations in 4
patients with brain tumor and a swelling of the ONH; he
called it Stauungspapille.
1908:Parsons coined the English term papilledema
1911: Paton and Holmes differentiated between papilledema
Historical with increased intracranial pressure (ICP) and optic neuritis.

Context Basic differentiation was based on visual function.

Edema of the nerve head with decreased vision was considered optic
neuritis and edema of the ONH without decreased vision was
papilledema secondary to increased ICP.
As experience has taught us, decreased vision also occurs as a result
of chronic papilledema secondary to increased ICP and subsequent
optic nerveatrophy.
 Weiss andHiscoe’s concept:
Axoplasmic flow: Flow of subcellular and molecular particles along the axons
of the nerves
3 types of axoplasmic flow exist; these occur simultaneously andsomewhat
independently and are affected by different pathologic processes.
Orthograde rapid flow, which moves along the axon at a rate of 200 to 1000
mm/day and subserves synaptictransmission.
Orthograde slow flow, which moves at 0.5 to 3 mm/day. Maintains the growth
Pathogenesis and metabolic stability of the axon.
 Retrograde flow that moves at 50 to 75 mm/day. Allows the axon to sample its
environment and send information back to the cell body.
Different mechanisms of insult differentially affect the axoplasmic flow
components.
Ischemia predominantly blocks rapid axoplasmic flow,whereas compression of
an axon blocks slow axoplasmicflow.
The former situation occurs in anterior ischemic optic neuropathy, and the
latter in increased intracranial pressure.
 Most common causes of optic nerve swelling are:
Non-arteritic anterior ischaemic optic neuropathy (35%)
Optic neuritis (31%)
Intracranial pathology (14%)
Causes of Unilateral optic disc swelling:
Demyelinating optic neuritis
NA-AION
Cause Retinal vein occlusion
s of Diabetic papillopathy
DiscEdema Causes of Bilateral optic disc swelling:
Papilledema,
Toxic optic neuropathy
Malignant hypertension
PIH

Jung JJ, Baek S-H, Kim US. Korean Journal ofOphthalmology : KJO. 2011;25(1):33-36.
Differential
Diagnosisof
DiscEdema
Diagnostic and
Prognostic
Clues from
Onset ofvision
loss
Symptoms  Decreased VisualAcuity
(Ocular)  Transient visual obscuration (TVO)
 Central vision affectedlate
 Horizontal diplopia
 Blind spot enlargement
 Blind spot enlargement
and fundus findings in
bilateral discedema
 Features ofraised ICT/mass lesions like:

 Headache more in the morning, intensifies with head

movement, coughing orstraining.
 Projectile vomiting.
 Loss of consciousness/ generalized motor rigidity.

Symptoms
(Systemic)
 Mechanical signs
Elevation of the optic disc
(3D=1mm)
Blurring of the optic disc
margins
Filling in of optic cup
Sign Edema of peripapillary nerve
s of fiber
Retinal or choroidal folds
DiscEdema (Patton Lines)

Diagnosis is done best by binocular stereoscopic viewing using a

high convex lens with magnification (90D)
 Vascular signs
Hyperemia of disc
Venous congestion
Peripapillary
Sign hemorrhages
s of Exudates in disc
DiscEdema or peripapillary
area
Nerve fiber layer
infarcts
Absence of SVP
Mechanismof
Vision Loss
• Retinal displacement and retinal elevation of percipient elements and the Stiles-
Crawford effect are three of the more traditional explanations for vision loss.
• A report suggesting a relative scotoma on the basis of induced hyperopia has been
suggested by Corbett et al. The hypothesis is that edematous elevations of the
percipient elements away from the blood supply of the choriocapillaris results in a
decrease in sensitivity.

Corbett JJ, Jacobson DM, Mauer RC,Thompson HS.Am JOphthalmol 105:261–265, 1988
Differential Diagnosis of Disc Edema

I. Papilledema
  Mild nasalNFL
elevation.
 Rare obscuration of a
portion of major vessel
Frisen (usually at superior
pole)
Papilledema
GradingSystem

Grade0

Frisen L. Swelling of the optic nerve head: A staging scheme. JNeurol Neurosurg Psychiatry 1982; 45:13-18
  Obscuration of
nasal border ofdisc
 Normal temporal
disc margin

Grade1
  Obscuration of all the disc
borders
 Elevation of nasalborder
 No major vesselobscuration

Grade 2
Early
Papilledema
  Obscuration of all
borders
 Increased diameter of
optic nervehead
 Obscuration of
Grade 3 segment of major
blood vessels asthey
Moderate pass disc margin.
Papilledema
  Elevation ofentire nerve
head
 Obscuration ofall the
borders
 A segment of major
vessel obscured onthe
Grade 4 disc
Marked
Papilledema
  Anterior extensionof
optic nervehead
 Total obscurationof
vessel on discsurface
 Obliteration of optic
Grade 5 cup
Severe
Papilledema
 Once true disc edema is established, papilledema
(due to raised ICT) has to be distinguished from
other optic neuropathies which can be of varied
etiology
Papilledema
or Papillitis? Main difference: Visual acuity and optic nerve
function
Papilledema: Normal
Papillitis: Disturbed
 Papilledema Papillitis
(Optic Neuritis)
Laterality Bilateral Unilateral

Symptoms Transient obscuration of vision Sudden diminution of vision

Extra ocular Movements No pain Pain on extra ocularmovement

Pupillary Reaction Normal RAPD

Media Clear Posterior vitreous cells

Disc elevation 2-6 D Does not exceed2-3D

Venous engorgement, More frequent Less frequent

peripapillaryhemorrhages
 Bilateral disc
edema, other signs
of raisedICT

Papilledema

Check BP

Stage IVHypertensive
Retinopathy
  Malignant hypertension
 Young individuals, smokers and uncontrolled blood
pressure
 Severe attenuation of arterioles
II. Grade IV
 Neuroretinopathy, presence of disc edema, multiple
Hypertensive cotton wool patches, hard exudates, macular star
Retinopathy  Poor prognosis associated with renal insufficiency,
stroke, myocardial infarction and death

HendersonAD, et al. Western Journal of Emergency Medicine. 2012;13(6):529-534.

 • Benign unilateral or bilateral optic neuropathy with transient optic disk
edema and minimal reduction in visual function.
• Disc edema typically resolves in a few months with no resulting optic
atrophy and minimal or no decrease in acuity.

• Occurs mainly in younger diabetics

• Mild painless visual impairment
that is unilateral in more than half
of cases; bilateral disc swelling
mandates the exclusion of raised
III. Diabetic intracranial pressure.
• Hyperaemic disc swelling is
Pappillopathy characteristic, and disc
telangiectasia occasionally
mistaken for neovascularizationis
present in many affected eyes
• Resolution occurs over several
months, often leaving mild disc
pallor.
Management:
• Good glycemic control.
• Anti VEGF’seffective in resolution.
• No role ofPRP
 Normal BP

Neuroimaging
-CT scan
-MRI

Abnormal Normal

1. Space occupying lesions like tumors, 1. Idiopathic intracranial

abscesses, hemorrhages, infarcts, AV hypertension
malformations 2. Cerebral venous thrombosis
2. Trauma 3. Endocrinal abnormalities
3. Inflammatory 4. Drug overdose/ withdrawal
4. Extra cranial lesions 5. SLE
 • Idiopathic intracranial hypertension is characterized by signs and
symptoms of raised intracranial pressure (ICP) with no established
pathogenesis.
• The disorder is strongly associated with obesity, and patients are
mostly female and typically of reproductive age.

IV.IIH
 Papilledema

Check BP Hypertensive retinopathy

Neuroimaging
Intracranial space
Abnormal Normal
occupying lesions

Lumbar puncture

Opening pressurehigh Normal openingpressure

Abnormal spinalfluid
analysis
Idiopathic
intracranial
hypertension
Meningitis
 Opticneuropathies should be considered under
two circumstances
Characteristics
Visual loss associated with anomalous, swollen
of optic or paledisc
neuropathies Fundus is normal, but acuity, color vision, field
abnormalities are accompanied byRAPD
 Anterior optic neuropathy
 Inflammatory optic neuropathy
 Ischemic optic neuropathy
Other optic  Compressive optic neuropathy
 Toxic and hereditary opticneuropathy
neuropathies  Infiltrative optic neuropathy
causing
Intraocular causes
DiscEdema CRVO, posterior uveitis, posterior scleritis,
hypotony
Neuroretinitis
 It is definedas inflammation
of the optic nerve head
associated with decrease in
visual acuity or visual field
loss.
Additional features
V. Opticneuritis Multiple sclerosis
Pain and tenderness
Central and
centrocecal scotoma
Contrast sensitivity
MRI:periventricular
plaques
 Atypical optic neuritis
Typical optic neuritis
Marked disc swelling Requires:
Young adult
Usually associated with
Vitritis  MRI
multiple sclerosis Progression of visual  CSFcytology
loss after 1week  Syphilis:
Vision starts to
improve by 2-3weeks Lack of partial MHATP
recovery within 4  LymeTitre
MRI is the only  Sarcoidosis:
weeks of onset
required investigation
Persistent pain, no CXR,ACE
in typical optic neuritis
response to steroids  Lupus:ANA
 Nutritional:B12
Pérez Bartolomé F, et al. (2015) Diagnosis Approach of Optic Neuritis. J Neurol Neurophysiol 6: 345.
 Sudden loss of vision
Interference with blood supply of the posterior
ciliary artery to the anterior part of the optic
nerve
VI. Ischemic Can be arteritic or non arteritic:
optic Arteritic: Associated with Giant cell arteritis.
neuropathy Ophthalmic emergency
Non arteritic: No overt symptoms,
associated with hypertension, DM,
hypercholesterolemia and shock.
 Arteritic Non arteritic
Sex predilection Females>males Females=males

Age >60 years 40-60 years

Visual loss Severe Moderate, onawakening

Associated symptoms Pain, jaw claudication, headache, No pain

bright light amarousis

Second eyeinvolvement Within days orweeks(70%) In months (30-40%)

Disc Pallor> hyperemia, chalkywhite Hyperemic >pallor

Sectoral edema

ESR >90mm/hr <40mm/hr

 Arteritic Non arteritic

Other signs of ocularischemia May bepresent Not present

Anatomic predisposition None Small crowded disc

Late opticatrophy Can have cupping Simple pallor

Response tosteroids Vision-sometimes None

Systemically-definite

Fluorescein angiography Choroidal filling defects Normal, can have delayed opticnerve
head filling
Arteritic anterior ischaemic optic neuropathy
(AAION) is caused by giant cell arteritis (GCA).About
50% of patients with GCA have polymyalgia
rheumatica (PMR) at diagnosis, while around 20% of
PMR patients will develop GCA. PMR is characterized
by pain and stiffness in proximal muscle groups,
typically the shoulders and biceps, that is worse on
waking
 AION

Chalky white disc

Disc edema

Disc filling
defects
NAION

Sectoral edema
 Disc appearance Additional features
Disc swelling Eg: Opticnerve gliomas
Glioblastomas
Opticociliary shunts
Meningiomas
Foster Kennedysyndrome Aneurysms

Slowly progressive visualloss

VII. Compressive Proptosis, gaze evoked
optic neuropathy amarousis
Foster–Kennedy syndrome refers to a constellation of
findings associated with tumors of the frontal lobe.
• Optic atrophy in the ipsilateral eye
• Disc edema in the contralateral eye
• Central scotoma (loss of vision in the middle of the visual
fields) in the ipsilateral eye
• Anosmia (loss ofsmell) ipsilaterally
Pseudo-Foster Kennedy Syndrome: Unilateral optic disc
swelling with contra lateral optic atrophy in the absence of an
intracranial mass causing compression of the optic nerve.
Occurs typically due to bilateral sequential optic neuritis or
ischemic optic neuropathy.

Bhatnagar KR, RauljiC,

Kumar P, Solanki D.
Natalia Pastora-Salvador, Jesus Med JDY Patil Univ
Peralta-Calvo.CMAJ December 13, 2014;7:385-7
2011 vol. 183 no. 18.
 Disc appearance
Disc hyperemia
Obscuration ofdisc margins
Dilated capillaries on disc
VIII. Hereditary surface that may extend into
surrounding retina
optic (telangiectatic
neuropathy microaneurysms),
Additional features
Swelling of NFL layer and
dilatation
Tortuosity ofposterior pole
vasculature
Maternally inherited mt DNA
mutations
Males, 15-35years
Subacute painless severe loss
of vision in one eye, followed
by the other
LeberHereditary
Optic
Neuropathy A. Acute Stage of LHON
B. MarkedTelangiectatic
Microangiopathy
C. LateAtrophic
appearance
 Optic neuropathy due to methanol poisoning is different from
others as it causes sudden visual loss and disc edema.
Disc edema is indistinguishable frompapilledema
Other symptoms are headache, dyspnoea, vomiting,
abdominal pain and bilateral visual blurring.
IX.Toxic optic
neuropathy

Sharma P,Sharma R.Toxic optic neuropathy. Indian JOphthalmol 2011;59:137-41

 Leber’s
stellate
neuroretinitis
No risk ofMS
Cat scratch disease,
syphilis, Lyme disease,
X.Neuroretinitis HIV
Look forsystemic cause
Presents like Optic
Neuritis
Good prognosis
responds to steroids
 Disc appearance
Hyperemic edematous disc
Neovascularization

Additional features
XI.CRVO Retinal hemorrhages inall
four quadrants
Dilated, tortuous veins in all
four quadrants
Macular edema
DecreasedAcuity
RAPD
 Pediatric papilledema
Infrequent in infants
In children, most common cause is neoplasms
Craniosynostosis
Child abuse, shaken baby syndrome, battered baby
syndrome-look forretinal hemorrhages.
Disc edemain Papilledema indicates subduralhematoma
children
 Usually bilateral, disc swelling more common
More aggressive treatment
Immune mediated
Usually bilateral, postinfectious
Pediatric Acute demyelinating encephalopathy
Good prognosis
optic neuritis
Idiopathic
Demyelination
10-50% eventually developMS
 Causes of pseudo discedema
Optic nerve head drusen : disc elevation
Is there Medullated nerve fibres : blurred margins
Morning glory syndrome: elevated disc
true disc Tilted disc: blurredmargins
edema…? Small hyperopic disc: hyperemicdisc
Optic disc dysplasia
Bergmeister’s papilla
 True disc edema Pseudo discedema
Disc color Hyperemic Yellow

Nerve fibrelayer Opacified Transparent

Large vessels Normal Anomalous- trifurcation, spokelike

Spontaneous venouspulsation Absent Present in 80%

Hyaline bodies Absent May be present

Optic cup Normal initially, filled Small or absent

Nerve fibre layerhemorrhages Frequent Absent

Fluorescein angiography Dye leakage at disc No leakage/ latestaining

 Papilledema, perineuritis: enlargedblind
spot, nasal arcuatescotomas
AION: altitudinaldefects
Role of Opticneuritis, toxic optic neuropathies:
central scotoma, centrocecalscotoma
investigations Tilted disc syndrome: bitemporal hemianopia
in discedema which does not respect the vertical midline

1.Visual fields
 Papilledema: disc capillary dilatation, dye leakage and
microaneurysm formation
AAION: delayed filling in choroidal phase
NAION: delayed disc filling, segmental disc
fluorescence (surface telangiectasias)
Neuroretinitis: no leakage atmacula
2. Fundus Hypertensive retinopathy: leakage from small
fluorescein vessels at macula
angiography

Stage IVHTNR
NAION
3. Neuroimaging
 6. Role of USG in
Differential Diagnosis of
Disc Edema

Optic Disc
Drusen
Management

ONTT Based on the findings of the ONTT, the study group made several recommendations:

• Chest x-ray, blood tests and lumbar puncture are not indicated for typical cases of
optic neuritis
• Treatment with oral prednisolone in conventional doses alone, is contraindicated
• Consider treatment with intravenous steroids when 3or more signal abnormalities
are present on MRI to reduce 2-year risk of developing MS, or in patients requiring
expedited recovery of vision (i.e., monocular patients, employment demands,
bilateral involvement and patients desiring intervention)

Beck RW,Trobe JD,What we have learned from the Optic NeuritisTreatmentTrial,Ophthalmology. 1995Oct;102(10):1504-8.
 AAION Treatment: NAION Treatment:
• Absence of visual symptoms: oral prednisolone •There is nodefinitive
1mg/kg/day treatment.
• CRPmay play an important role in monitoring •Optic nerve fenestration
disease activity has not been shown to be of
• Treatment is aimed at preventing blindness of benefit.
the fellow eye •Some authorities advocate
• The second eye may still become involved in short-term systemic steroid
25% despite early steroidadministration. treatment.
• Intravenous methylprednisolone, 500 mg to 1 •Any underlying systemic
Management g/day for 3 days followed by oral prednisolone predispositions should be
1–2 mg/kg/day.After 3more days the oral dose treated.
1.AAION is reduced to 50–60 mg (not less than 0.75 •Although aspirin is
mg/kg) for 4 weeks or until symptom resolution effective in reducing
2.NAION and ESR/CRPnormalization systemic vascular
• Antiplatelet therapy, e.g. aspirin 600 mg stat events and isfrequently
then 100 mg/day should be commenced as this prescribed in patients with
has been .shown to reduce the risk of visual loss NAION,it does not appear to
and stroke. reduce the risk of
• Immunosuppressives such as methotrexate involvement of the
may be used as adjuncts oras steroid-sparing fellow eye.
agents.
Medical treatment options for IIH:
• Lifestyle and dietalterations
• Corticosteroid therapy
 Papilledema inPIH
 General : Bedrest.

 Control of BP.

 Control of Edema : Diuretic, Hypertonic glucose.

 Non responders :Termination of pregnancy.

Schiffman JS,Scherokman B,Tang RA, Dorotheo EU, Prieto P,Varon J, Evaluation and treatment of papilledema in
pregnancy.,Compr Ophthalmol Update. 2006 Jul-Aug;7(4):187-202.
 Indications:
 Failure of Medicaltreatment
- Marked disc swelling(>5D)
- Engorged veins
- Extensive haemorrhages
- Early exudate spots
- Progressive headache
Surgical  Progressive optic neuropathy
Decompression (early field constriction)
Surgical options:
• Optic nerve sheathdecompression
• Bariatric surgery
• Frequent lumbar punctures
• Sub temporal decompression
• Placement of Lumbo-peritonealshunts.
 Circulation of cerebrospinal fluid
a (a) Subarachnoid space

b (b) Lateral ventricle

c
d (c) Third ventricle

e
(d) Aqueduct

(e) Fourth ventricle

 Hydrocephalus
Dilated cerebral ventricles

Communicating - obstruction to CSF flow in basilar

cisterns or cerebral subarachnoid space

Non-communicating - obstruction to CSF flow in ventricular

system or at exit of foramina of fourth ventricle