This seminar outlines how to make a diagnoisis when evaluating a case of optic disc edema.

1. Differential Diagnosis
of Disc Edema
Moderated by: Dr. Parul Ichhpujani
Presented by: Dr. SahilThakur

2. Historical
Context
 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
with increased intracranial pressure (ICP) and optic neuritis.
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 nerve atrophy.

3. Pathogenesis
Weiss and Hiscoe’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 and somewhat
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 synaptic transmission.
Orthograde slow flow, which moves at 0.5 to 3 mm/day. Maintains the growth
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 axoplasmic flow.
The former situation occurs in anterior ischemic optic neuropathy, and the
latter in increased intracranial pressure.

4. Causes
of
Disc Edema
 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
 Retinal vein occlusion
 Diabetic papillopathy
 Causes of Bilateral optic disc swelling:
 Papilledema,
 Toxic optic neuropathy
 Malignant hypertension
 PIH
Jung JJ, Baek S-H, Kim US. Korean Journal of Ophthalmology : KJO. 2011;25(1):33-36.

5. Differential
Diagnosis of
Disc Edema

6. Diagnostic and
Prognostic
Clues from
Onset of vision
loss

7. Symptoms
(Ocular)  Transient visual obscuration (TVO)
 Central vision affected late
 Horizontal diplopia
 DecreasedVisual Acuity
 Blind spot enlargement

8.  Blind spot enlargement
and fundus findings in
bilateral disc edema

9. Features of raised ICT/mass lesions like:
 Headache more in the morning, intensifies with head
movement, coughing or straining.
 Projectile vomiting.
 Loss of consciousness/ generalized motor rigidity.
Symptoms
(Systemic)

10. Signs
of
Disc Edema
Mechanical signs
 Elevation of the optic disc
(3D=1mm)
 Blurring of the optic disc
margins
 Filling in of optic cup
 Edema of peripapillary nerve
fiber
 Retinal or choroidal folds
(Patton Lines)
 Diagnosis is done best by binocular stereoscopic viewing using a
high convex lens with magnification (90D)

11. Signs
of
Disc Edema
Vascular signs
 Hyperemia of disc
 Venous congestion
 Peripapillary
hemorrhages
 Exudates in disc or
peripapillary area
 Nerve fiber layer
infarcts
 Absence of SVP

12. Mechanism of
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 J Ophthalmol 105:261–265, 1988

13. I. Papilledema
Differential Diagnosis of Disc Edema

14. Frisen
Papilledema
GradingSystem
Grade 0
 Mild nasal NFL
elevation.
 Rare obscuration of a
portion of major vessel
(usually at superior
pole)
Frisen L. Swelling of the optic nerve head: A staging scheme. J Neurol Neurosurg Psychiatry 1982; 45:13-18

15. Grade 1
 Obscuration of
nasal border of disc
 Normal temporal
disc margin

16. Grade 2
Early
Papilledema
Obscuration of all the disc
borders
Elevation of nasal border
No major vessel obscuration

17. Grade 3
Moderate
Papilledema
 Obscuration of all
borders
 Increased diameter of
optic nerve head
 Obscuration of
segment of major
blood vessels as they
pass disc margin.

18. Grade 4
Marked
Papilledema
 Elevation of entire nerve
head
 Obscuration of all the
borders
 A segment of major
vessel obscured on the
disc

19. Grade 5
Severe
Papilledema
 Anterior extension of
optic nerve head
 Total obscuration of
vessel on disc surface
 Obliteration of optic
cup

21. Papilledema
or Papillitis?
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
Main difference:Visual acuity and optic nerve
function
Papilledema: Normal
Papillitis: Disturbed

22. Papilledema Papillitis
(Optic Neuritis)
Laterality Bilateral Unilateral
Symptoms Transient obscuration of vision Sudden diminution of vision
Extra ocular Movements No pain Pain on extra ocular movement
Pupillary Reaction Normal RAPD
Media Clear Posterior vitreous cells
Disc elevation 2-6 D Does not exceed 2-3D
Venous engorgement,
peripapillary hemorrhages
More frequent Less frequent

23. Papilledema
Check BP
Stage IV Hypertensive
Retinopathy
Bilateral disc
edema, other signs
of raised ICT

24. II.Grade IV
Hypertensive
Retinopathy
Malignant hypertension
Young individuals, smokers and uncontrolled blood
pressure
Severe attenuation of arterioles
Neuroretinopathy, presence of disc edema, multiple
cotton wool patches, hard exudates, macular star
Poor prognosis associated with renal insufficiency,
stroke, myocardial infarction and death
Henderson AD, et al. Western Journal of Emergency Medicine. 2012;13(6):529-534.

25. • 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.
III. Diabetic
Pappillopathy
• 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
intracranial pressure.
• Hyperaemic disc swelling is
characteristic, and disc
telangiectasia occasionally
mistaken for neovascularization is
present in many affected eyes
• Resolution occurs over several
months, often leaving mild disc
pallor.
Management:
• Good glycemic control.
• AntiVEGF’s effective in resolution.
• No role of PRP

26. Neuroimaging
-CT scan
-MRI
Abnormal
1. Space occupying lesions like tumors,
abscesses, hemorrhages, infarcts, AV
malformations
2. Trauma
3. Inflammatory
4. Extra cranial lesions
1. Idiopathic intracranial
hypertension
2. Cerebral venous thrombosis
3. Endocrinal abnormalities
4. Drug overdose/ withdrawal
5. SLE
Normal
Normal BP

27. IV. IIH
• 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.

29. Modified
Dandy
criteria
for IIH

30. Papilledema
Check BP Hypertensive retinopathy
Neuroimaging
Abnormal Normal
Intracranial space
occupying lesions
Lumbar puncture
Opening pressure high
Idiopathic
intracranial
hypertension
Normal opening pressure
Abnormal spinal fluid
analysis
Meningitis

31. Characteristics
of optic
neuropathies
Optic neuropathies should be considered under
two circumstances
 Visual loss associated with anomalous, swollen
or pale disc
 Fundus is normal, but acuity, color vision, field
abnormalities are accompanied by RAPD

32. Other optic
neuropathies
causing
Disc Edema
 Anterior optic neuropathy
Inflammatory optic neuropathy
Ischemic optic neuropathy
Compressive optic neuropathy
Toxic and hereditary optic neuropathy
Infiltrative optic neuropathy
 Intraocular causes
 CRVO, posterior uveitis, posterior scleritis, hypotony
 Neuroretinitis

33. V.Optic neuritis
It is defined as inflammation
of the optic nerve head
associated with decrease in
visual acuity or visual field
loss.
Additional features
 Multiple sclerosis
 Pain and tenderness
 Central and centrocecal
scotoma
 Contrast sensitivity
 MRI:periventricular plaques

35. Typical optic neuritis
 Young adult
 Usually associated with
multiple sclerosis
 Vision starts to
improve by 2-3 weeks
 MRI is the only
required investigation
in typical optic neuritis
Atypical optic neuritis
 Marked disc swelling
 Vitritis
 Progression of visual
loss after 1 week
 Lack of partial
recovery within 4
weeks of onset
 Persistent pain, no
response to steroids
Requires:
 MRI
 CSF cytology
 Syphilis:
MHATP
 LymeTitre
 Sarcoidosis:
CXR, ACE
 Lupus:ANA
 Nutritional:B12

36. Pérez Bartolomé F, et al. (2015) Diagnosis Approach of Optic Neuritis. J Neurol Neurophysiol 6: 345.

38. VI. Ischemic
optic
neuropathy
 Sudden loss of vision
 Interference with blood supply of the posterior
ciliary artery to the anterior part of the optic
nerve
Can be arteritic or non arteritic:
 Arteritic: Associated with Giant cell arteritis.
Ophthalmic emergency
 Non arteritic: No overt symptoms, associated
with hypertension, DM, hypercholesterolemia
and shock.

39. Arteritic Non arteritic
Sex predilection Females>males Females=males
Age >60 years 40-60 years
Visual loss Severe Moderate, on awakening
Associated symptoms Pain, jaw claudication, headache,
bright light amarousis
No pain
Second eye involvement Within days or weeks(70%) In months (30-40%)
Disc Pallor> hyperemia, chalky white Hyperemic > pallor
Sectoral edema
ESR >90mm/hr <40mm/hr

40. Arteritic Non arteritic
Other signs of ocular ischemia May be present Not present
Anatomic predisposition None Small crowded disc
Late optic atrophy Can have cupping Simple pallor
Response to steroids Vision-sometimes
Systemically-definite
None
Fluorescein angiography Choroidal filling defects Normal, can have delayed optic nerve
head filling

41. 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

42. AION
NAIONDisc filling
defects
Sectoral edema
Chalky white disc
Disc edema

43. VII.Compressive
optic neuropathy
Disc appearance
 Disc swelling
 Opticociliary shunts
 Foster Kennedy syndrome
Additional features
Eg: Optic nerve gliomas
 Glioblastomas
 Meningiomas
 Aneurysms
 Slowly progressive visual loss
 Proptosis, gaze evoked
amarousis

44. 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.
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 of smell) ipsilaterally
Bhatnagar KR, Raulji C,
Kumar P, Solanki D.
Med J DY Patil Univ
2014;7:385-7
Natalia Pastora-Salvador, Jesus
Peralta-Calvo.CMAJ December 13,
2011 vol. 183 no. 18.

45. VIII. Hereditary
optic
neuropathy
Disc appearance
 Disc hyperemia
 Obscuration of disc margins
 Dilated capillaries on disc
surface that may extend into
surrounding retina
(telangiectatic
microaneurysms),
Additional features
 Swelling of NFL layer and
dilatation
 Tortuosity of posterior pole
vasculature
 Maternally inherited mt DNA
mutations
 Males, 15-35 years
 Subacute painless severe loss
of vision in one eye, followed
by the other

46. Leber Hereditary
Optic
Neuropathy A. Acute Stage of LHON
B. MarkedTelangiectatic
Microangiopathy
C. Late Atrophic
appearance

47. IX.Toxic optic
neuropathy
 Optic neuropathy due to methanol poisoning is different from
others as it causes sudden visual loss and disc edema.
 Disc edema is indistinguishable from papilledema
 Other symptoms are headache, dyspnoea, vomiting,
abdominal pain and bilateral visual blurring.
Sharma P, Sharma R.Toxic optic neuropathy. Indian J Ophthalmol 2011;59:137-41

49. X. Neuroretinitis
 Leber’s stellate
neuroretinitis
 No risk of MS
 Cat scratch disease,
syphilis, Lyme disease,
HIV
 Look for systemic cause
 Presents like Optic
Neuritis
 Good prognosis
responds to steroids

50. XI.CRVO
Disc appearance
 Hyperemic edematous disc
 Neovascularization
Additional features
 Retinal hemorrhages in all
four quadrants
 Dilated, tortuous veins in all
four quadrants
 Macular edema
 Decreased Acuity
 RAPD

51. Disc edema in
children
Pediatric papilledema
 Infrequent in infants
 In children, most common cause is neoplasms
 Craniosynostosis
 Child abuse, shaken baby syndrome, battered baby
syndrome-look for retinal hemorrhages.
 Papilledema indicates subdural hematoma

52. Pediatric
optic neuritis
 Usually bilateral, disc swelling more common
 More aggressive treatment
 Immune mediated
 Usually bilateral, post infectious
 Acute demyelinating encephalopathy
 Good prognosis
 Idiopathic
 Demyelination
 10-50% eventually develop MS

53. Is there
true disc
edema…?
Causes of pseudo disc edema
 Optic nerve head drusen : disc elevation
 Medullated nerve fibres : blurred margins
 Morning glory syndrome: elevated disc
 Tilted disc: blurred margins
 Small hyperopic disc: hyperemic disc
 Optic disc dysplasia
 Bergmeister’s papilla

54. True disc edema Pseudo disc edema
Disc color Hyperemic Yellow
Nerve fibre layer Opacified Transparent
Large vessels Normal Anomalous- trifurcation, spoke like
Spontaneous venous pulsation Absent Present in 80%
Hyaline bodies Absent May be present
Optic cup Normal initially, filled Small or absent
Nerve fibre layer hemorrhages Frequent Absent
Fluorescein angiography Dye leakage at disc No leakage/ late staining

56. Role of
investigations
in disc edema
1.Visual fields
 Papilledema, perineuritis: enlarged blind
spot, nasal arcuate scotomas
 AION: altitudinal defects
 Optic neuritis, toxic optic neuropathies:
central scotoma, centrocecal scotoma
 Tilted disc syndrome: bitemporal hemianopia
which does not respect the vertical midline

57. 2. Fundus
fluorescein
angiography
 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 at macula
 Hypertensive retinopathy: leakage from small
vessels at macula
NAIONStage IV HTNR

58. 3. Neuroimaging

59. 6. Role of USG in
Differential Diagnosis of
Disc Edema
Optic Disc
Drusen

61. 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 3 or 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. 1995 Oct;102(10):1504-8.

62. Management
1.AAION
2.NAION
NAIONTreatment:
• There is no definitive
treatment.
• Optic nerve fenestration
has not been shown to be of
benefit.
• Some authorities advocate
short-term systemic steroid
treatment.
• Any underlying systemic
predispositions should be
treated.
• Although aspirin is
effective in reducing
systemic vascular
events and is frequently
prescribed in patients with
NAION,it does not appear to
reduce the risk of
involvement of the
fellow eye.
AAIONTreatment:
• Absence of visual symptoms: oral prednisolone
1 mg/kg/day
• CRP may play an important role in monitoring
disease activity
• Treatment is aimed at preventing blindness of
the fellow eye
• The second eye may still become involved in
25% despite early steroid administration.
• Intravenous methylprednisolone, 500 mg to 1
g/day for 3 days followed by oral prednisolone
1–2 mg/kg/day. After 3 more days the oral dose
is reduced to 50–60 mg (not less than 0.75
mg/kg) for 4 weeks or until symptom resolution
and ESR/CRP normalization
• Antiplatelet therapy, e.g. aspirin 600 mg stat
then 100 mg/day should be commenced as this
has been .shown to reduce the risk of visual loss
and stroke.
• Immunosuppressives such as methotrexate
may be used as adjuncts or as steroid-sparing
agents.

63. Medical treatment options for IIH:
• Lifestyle and diet alterations
• Corticosteroid therapy

64. Papilledema in PIH
 General : Bed rest.
 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.

65. Surgical
Decompression
Indications:
 Failure of Medical treatment
- Marked disc swelling(>5D)
- Engorged veins
- Extensive haemorrhages
- Early exudate spots
- Progressive headache
 Progressive optic neuropathy
(early field constriction)
Surgical options:
• Optic nerve sheath decompression
• Bariatric surgery
• Frequent lumbar punctures
• Sub temporal decompression
• Placement of Lumbo-peritoneal shunts.