This document provides an overview of optic disc swelling, including the anatomy of the optic nerve, blood supply, causes of optic disc swelling like papilledema and pseudopapilledema, differential diagnosis, and treatments. Key points include the definition of papilledema as optic disc swelling due to increased intracranial pressure, distinguishing features between papilledema and pseudopapilledema, grading scales for papilledema severity, causes of increased ICP like idiopathic intracranial hypertension, and distinguishing anterior ischemic optic neuropathy from optic neuritis.

1. WELCOME

2. OPTIC DISC SWELLING
Dr. Tareq Esteak
Resident( Neurology)
NINS

3. OUTLINE
Anatomy of Optic Nerve
Blood supply of Optic Nerve
Applied anatomy
Pathogenesis
Differentials

4. ANATOMY OF OPTIC NERVE
An outgrowth of brain
2nd cranial nerve
Comprised of axonal extension from ganglionic cell of retina
Optic disc to chiasma
Not covered with neurilemma : doesn’t regenerate when get cut
Sorrounded by meninges unlike other cranial nerves

5. PARTS OF OPTIC NERVE
Intraocular
Intraorbital
Intracanalicular
Intracerebral

6. INTRAOCULAR PART
Lamina
Cribrosa
Meninges
Meninges

7. ARTERIAL SUPPLY:
OPTIC NERVE
Internal carotid
artery
Ophthalmic artery
Central Retinal Artery
anterior part of the
retrobulbar nerve via
centrifugal arterioles
Short posterior ciliary
artery(6-12)
Supply the Optic nerve
head in Centripetal and
segmental fashion
Arteries & arterioles of
pial sheeth derived
from ophthalmic artery
PION
CRA
O
AION

8. BLOOD SUPPLY OF OPTIC NERVE

9. VENOUS DRAINAGE
Optic nerve head : Central ratinal vein
Orbital part: Central retinal vein,Pial plexus
Intracranial part: Pial plexus<anterior cerebral vein

10. APPLIED ANTOMY
Papilledema occur as prelaminar axons swells from statis of
axoplasmic flow at the level of lamina cribrosa.
Insufficient blood flow through posterior ciliary artery due to
hypotension, thrombosis, vascular occlusion, vasculitis(GCA) causes
Optic nerve head infraction in leading to AION( optic disc swelled).
Insufficient blood flow through pial vasculature due to hypotension,
thrombosis, vascular occlution, vasculitis(GCA) causes Optic nerve
infraction post-laminar leading to PION ( optic disc normal).

11. APPLIED ANTOMY
Intracanalicular part of optic nerve is vulnarable to
injury in skull fracture.
Intracranial part of optic nerve is usually compressed
due to internal carotid artery aneurysm
CRVO : Papilledeama with widespread hemorrhage.
CRAO : Pale Retina with cherry red spot.

12. OPTIC DISC SWELLING
Optic disc swelling or disc edema may be defined as swelling of optic
nerve anterior to lamina cribrosa .
When found must be differentiated from Pseudopapilledema
Pseudopapilledema mostly congenital and physiological , doesn’t
need further workup
True Disc swelling is associated with numerous condition

13. DIFFERENTIALS OF OPTIC DISC
SWELLING
True disc swelling
Elevated ICP(Papilledema)
Optic Neuritits
Neuroretinitis
Anterior Ischemic Optic
Neuropathy(AION)
Diabetic papillopathy
Hypertensive retinopathy
Compressive neuropathy:
Meningioma ,Lymphangioma,
lymphoma
Pseudopapilledema
Drusen
Myelinated nerve fiber
Hypermetropia

14. Papilledema Pseudopapilledema
Definition Disc swelling due to raised
ICP
Appearance of disc swelling not due to
any disease state
Cause ICSOL,IIH, malignant
hypertension
Drusen, Myelinated nerve fiber,
Hypermetropia
Symptoms Transient vison loss,
enlarged blind spot
Usually asymptomatic, sometimes Arcuate
scotoma
Fundoscopy
Disc Small cupless/ill-defined cup Loss of cup occurs late
Color of Disc Hyperemic Yellowish
Obscuration of
disc blood vessel
present absent
Spontaneous
Venous pulsation
absent present
Exudates May be present absent
Fluorescein dye Dye leaks in disc No leakage

15. PAPILLEDEMA DRUSEN
Both disc are swollen with obscuration of
disc margins and vessel at disc
Retinal hemorrhage
Disc is elevated with visible yellowish
deposits within the optic nerve head

16. PAPILLEDEMA
DRUSEN
• Disc swelling, venous engorgement,
and vessel leakage shown by
fluorescein angiography, •Undilated capillary network with no
leakage of dye into the peripapillary region.
•Discrete foci of hyperfluorescence with
late staining of the drusen.

17. DRUSEN ( CT- ORBIT/HEAD)
• Calcification of the optic nerve insertion
bilaterally
• USG of eye demonstrates calcified (
drusen) optic nerve head

18. MYELINATED NERVE FIBER HYPERMETROPIA
• White, feathery ,Fanned out lesion
• Simulates disc edema
• Central retinal vessel crows in
small disc
• congested but no dialted vessels,
• Sponteneous venous pulsation
present

19. APPROACH
Optic disc selling
True Disc Swelling
Unilateral
1.Demyelinating : MS
2.Neuroretinitis: Viral,
Toxoplama, syphilis
3.Anterior Ischemic Optic
Neuropathy(AION)
4.Central Retinal Vein Occlusion
5.Compressive optic neuropathy
Bilateral
Papilledema
Malignant Hypertension
Diabetic papillpathy
Toxic Optic neuropathy
Demyelinating :NMO, Anti MOG
Hereditary optic neuropathy
Pseudopapilledema

20. PAPILLEDEMA
Once true disc swelling is confirmed , it should be determined
whether it is related to an optic neuropathy or raised ICP.
Papilledema is usually reserve for optic swelling secondary to raised
ICP
All other disc swelling is called optic disc swelling or disc edema.
Other of the major causes of disc edema other than papilledema is
Papillitis or optic neuritis,AION

21. PATHOGENEIS: PAPILLEDEMA

22. CAUSES OF PAPILLEDEMA
IIH( Idopathic Intracranial Hypertension)
Increased venous pressure : SSST ( superior Sagittal Sinus
Thrombosis)
ICSOL: Tumor ,Abscess, Large hematoma
Meningeal process: Infection, inflammation, neoplastic
Hydrocephalus

23. PAPILLEDEMA : GRADING ( THE FRISEN
SCALE)

24. NORMAL DISC : GRADE 0

25. GRADE 1

26. GRADE 2

27. GRADE 3

28. GRADE 4

29. GRADE 5

30. ANTERIOR OPTIC NEUROPATHIES
Etiology
Inflammatory .e.g.Optic
Neuritis(Papillitis)
Ischemic .e.g. AION
Compressive Optic neuropathy
Toxin induced.e.g. Methanol,
ethelene glycol
Heriditary.e.g. Leber’s optic
neuropathy
Infiltrative.e.g. sarcoisosis
Pathogenesis
Optic Disc Swelling
Inflammatory change
Local Optic nerve injury
e.g. Optic neuritis, Ischemia, Toxins

31. PAPILLITIS ( OPTIC NEURITIS)
Inflammation of optic papilla or optic disc is called papillitis or optic
neuritis
Usually associated with pain ,specially on eye movement
Dimness of vision
On examination: Imapired optic nerve function, Central scotoma
,RAPD present
Underlying disease :Demyelinating disease MS, NMO-SD,
If associated with retinal inflammation called neuroretinitis
Neuroretinitis usually never demyelinating. Causes include
Toxoplasmosis, Viral infection(CMV), Syphilis , Sarcoidosis.

32. MS NMO
T2WI- shows increased signal
intensity in small segmnt of
left optic nerve
T2WI-shows increased signal
intensity in large segment in
left optic nerve

33. MRI : MS; NMO TYPICAL SITE
Multiple sclerosis Neuromyelitis Optica

34. PAPILLEDEMA VS PAPILLITIS
The main difference between papilledema and papillitis is
Visual equity and optic nerve function
oPapilledema: Intact
oPapillitis : Impaired

35. points Papilledema Papillitis
Definition Disc swelling due to Raised ICP Disc swelling due to Inflammation
Laterality Bilateral Usually Unilateral
Vision Transient obscuration of vision Sudden, persistent dimness of vision
Pain none During ocular movement
Etiology Raised ICP due to ICSOL,IIH,SSST
etc.
MS, NMO, Syphilis, Sarcoidosis etc.
Visual
acuity
intact reduced
Visual field Enlarge blind spot /tunnel vision Central or paracentral scotoma to complete
blindness
Fundoscop
y
Disc swelling with hemorrhage
;exudate
Disc swelling without hemorrhage ;exudate
Color
vision
Intact Impaired
Light Normal(RAPD absent) Relative afferent pupillary defect(RAPD

36. ANTERIOR ISCHEMIC OPTIC
NEUROPATHY (AION)
Ischemic optic neuropathy, at the optic nerve head
AION is traditionally divided into Arteritic (AAION) and Non-arteritic (NAION)
Arteritic AION (AAION) usually associated with GCA; comprises only 10% to
15% of all AION
Early differentiation of AAION and NAION is critical for medical management.
AAION is a true medical emergency because GCA can cause imminent
ischemic damage to the fellow optic nerve, retina, brain, and heart.

37. AION : PATHOPHYSIOLOGY
Optic disc swelling
Optic nerve head infraction
Occlusion of posterior
short ciliary artery
oVasculitis(GCA) ,Hypotension,
Thrombosis, Vascular occlusion

38. AION: ALTITUDINAL VISION LOSS
Superior segmental Disc pallor
corresponding to Inferior,
Altitudinal field of vision defect
on visual field test

39. Points Arteritic AION (AAION ) Non-arterictic AION (NAION)
sex Female> Male Male=Female
Age(y) >60 40-60
onset Acute Sub-acute, insidious
Visual Loss Profound Less profound
Second Eye
involvement
Within days to weeks Weeks to months
Associated symptoms Headache,Jaw claudication, scalp
tendeness
absent
Underlying disease Giant cell arteritis DM, HTN, Dyslipidemia
Disc Pallor> Hyperemia Hyperemia > pallor
ESR >90 <40
CRP raised normal
Response to steroid Responsive Non-responsive
Prognosis Untreated visual loss upto50-95%, 95%
cases fellow eye involvement
Spontaneous improvement up
to 43% cases;<30% cases
involvement of fellow eye

40. ARTERITIC-AION VS
NONARTERITIC-AION
• Suoirior and inferior margin obscures
• Pale disc(arteritic-AION)
• Flame shape hemorrhage
• Hyperemic Disc(Nonarteritic-
AION)

41. TREATMENT APPROACH TO
AION(DUE TO GCA)
Koster, M.J., Warrington, K.J. and Kermani, T.A., 2016. Update on the epidemiology and treatment of giant cell
arteritis. Current Treatment Options in Rheumatology, 2(2), pp.138-152.

42. AION VS OPTIC NEURITIS
Why do we need to differentiate?
Similarities in presentation:
I. Unilateral dimness of vision
II. Painful optic neuropathy
III. Optic nerve dysfunction: field, acuity,color vision,RAPD,Optic disc
swelling
IV. May Respond to steroids

43. Points Optic Neuritis AION
Age of onset 20-30 >60
Pain 92% cases, exacerbate on eye
movement
Periorbital but no change with eye
movement
Associated Sensory disturbance, Limb
weakness,
Ataxia, Bowel bladder dysfunction,
Headache,Jaw claudication, scalp
tendeness
onset Insidious ,progresses of hours to
days
Sudden onset, often notice upon
awekenig
Disc swelling Present only one third of the
cases, in rest its retrobulbar
Present in most of the cases
Field defect Central scotoma Altitudinal
MRI Enhancement of optic nerve No enhancement of optic nerve
Recovery Within 2-4 weeks Over months
prognosis Good prognosis poor

44. LEBER’S HERIDITARY OPTIC NEUROPATHY(LHON)
• Mitocondrial disease
• Bilateral involvement of optic nerve
• May occur at any age
• Acute phase present with optic disc
swelling and hyperemia
• Ultimately patient develops optic
atrophy
• Associated with cardiac conduction
defects, Tremor, limb
weakness(LHON plus)

45. LEBER’S
HERIDITARY OPTIC
NEUROPATHY(LHON)
• Blilateral hyperemic disc
swelling
• Central scotomas;
Right>left

46. HYPERTENSIVE RETINOPATHY
excessive cerebral
blood flow
breakdown of the
blood–brain
barrier
secondary
vasogenic edema
Papilledema
Precapillaries in the
posterior fundus
get occluded
ischemic process
leading to
fibrinoid necrosis
cytotoxic edema

47. TREATMENT
It’s medical emergency
Must be managed promptly and
adequately to prevent further
target organ damage.
[AHA guideline,2017]

48. DIABETIC PAPILLOPATHY
Benign Optic disc swelling , typically bilateral
Young juvenile diabetics
No or minimal effects on visual acuity and visual fields are typically restricted.
Usually occur in the absence of diabetic retinopathy
36% of cases of diabetic papillopathy progressing to NAION.
Usually a diagnosis of exclution
Spontaneous resolution within 2-10 months without any optic atrophy
The exact pathogenesis is unclear, but is believed to occur due to disruption of the
peripapillary vasculature
Wallace, I.R., Mulholland, D.A. and Lindsay, J.R., 2012. Diabetic papillopathy: an uncommon cause of bilateral optic disc swelling. QJM: An

49. DIABETIC
PAPILLOPATHY
(A and B) Bilateral marked swelling
and superficial flame haemorrhages in
the left eye.
Note, absence of significant diabetic
retinopathy.
(C and D) Swelling gradually resolving
without atrophy 3 months later.
Treatment:
• Good glycemic control
• regular intervals with close
monitoring.

50. CRVO
Papilledema ;Flame shape
hemorrgahe in all qudrants
Macular hemorrhage
Setting sun Appearance

51. CRVO
Pathogenesis
Papilledema and Hemorrhage
Hypoxia
Stagnation
Venous occlution
Due to HTN, DM, Dyslipidemia,
Hyperviscosity
Examination finding
Visual acuity : impaired
RAPD : present
Treatment:
I. Intra-vitreal injection :1st line: VEGF
inhibitor, reduce macular edema
II. Intra-vitreal injection: 2nd
line,triamsinolone,dexamethasone
III. Neovascularization: PRP

52. IIH
Idiopathic intracranial hypertension is characterised by signs and
symptoms of raised intracranial pressure (ICP) with no established
pathogenesis.
predominantly affects young, obese women
Pathogenesis has not been fully elucidated
Three main proposed mechanism
1. increased venous sinus pressure,
2. decreased CSF drainage across the arachnoid granulations or
lymphatics
3. enhanced CSF production at the choroid plexus

53. PATHOGENESIS

54. NEUROIMAGING
:IIH
(A)T1 - MRI; sagittal; empty Sella
turcica (arrow) presents in 70% of
patients with idiopathic intracranial
hypertension.
(B) T2 weighted MRI; axial; distension
of the optic nerve sheath (arrows)
has been reported in 45% of patients
with the disorder.28
(C) MRI venography; posterior view;
hypoplastic right transverse sinus
(arrow).
(D) T2 weighted MRI; axial; flattening of
posterior globes (arrows) can be seen in
80% of patients.

55. FUNDAL PHOTOGRAPHS WITH
CORRESPONDING SPECTRAL
DOMAIN OPTICAL
COHERENCE
TOMOGRAPHY(OCT)
Fundal photographs and optical
coherence tomography can be
used for the diagnosis of
papilledema.
(A) Fundal photograph and (B)
corresponding optical coherence
tomography (healthy control).
(C) Fundal photograph of
papilloedema with an elevated
disc and peripapillary halo,
(D) corresponding OCT showing
an increase in retinal nerve fibre
layer thickness in IIH

56. PROGRESSION OF VISUAL FIELD
DEFECT IN IIH

57. DIAGNOSTIC CRITERIA FOR ADULT
IDIOPATHIC INTRACRANIAL
HYPERTENSION

59. CEREBRAL VENOUS SINUS
THROMBOSIS
CSF drains into Intracranial venous sinuses
CSF passively resorbrd into arachanoid granulations ,mostly withi
supiriror sagittal sinus
Incase of venous thrombosis CSF resorption decreased andvthe CSF
pressure increases
This raised ICP may ultimately give rise to papilledema

61. NCCT -head : Cord sign
Contrast CT –head : Empty
Delta sign

62. Figure : Sagittal sinus thrombosis.
(A) MR venogram showing absent venous
flow signal in the middle third of the
superior sagittal sinus (white arrows)
(B) Axial gadolinium-enhanced T1-weighted
MR scan showing an irregular filling defect of
the superior sagittal sinus (white arrow)
(C) Sagittal gadolinium-enhanced T1-
weighted MR scan also showing a focal filling
defect indicating thrombus (white arrow)
(D) Axial susceptibility-weighted imaging
showing low signal indicating blood products
(thrombus) in the superior sagittal sinus
(white arrow)

63. TREATMENT
Should be started as soon as the diagnosis of CVT is clearly confirmed
- Rapid anticoagulant therapy
- Treatment of underlying cause (eg, dehydration, sepsis, stopping
any prothrombotic medications)
- Control of seizures
- Management of intracranial hypertension if required.

64. COMPRESIVE OPTIC NEUROPATHY
• Lesion may compress or infiltrate the intraorbital, intracranial or prechiasmal optic nerve
• Large and intraorbital lesions often produce optic disc swelling
Presentation
o Progressive unilateral optic neuropathy
o Usually no pain with eye movement
o Headache occurs if raised ICP or involvement of Trigeminal nerve
o Proptosis is common in orbital lesion
o Cranial nerve palsys occur if lesion extending to orbital apex, superior orbital fissure or
cavernous sinus.
o Early disc change: Swelling ; Late change : Optic Atrophy

65. Compressive
Optic
neuropathy
Neoplastic
Intraorbital
Tumor(hemangioma,lympha
ngioma,Mets)
Optic nerve sheeth
meningioma
Chraniopharyngioma
Non-neoplastic
•Grave’s Ophthalmopathy
•Tolosa-Hunt syndrome
•Ophthalmic artery anyurism

66. OPTIC SHEATH MENINGIOMA GRAVES
OPTHALMOPATHY
,T1-w MRI reveals an contrast enhancing
mass surrounding the optic nerve.(Red Arrow)
T1WI:Bilateral Fusiform enlargement of
medial rectus ;Right compressing the
optic nerve

67. TOXIN INDUCED NEUROPATHY/ TOXIC
AMBLYOPIA
• Patient presents with sudden vison loss
• Abrupt onset central/cecocentral scotoma
• Associated nausea, vomiting, abdominal pain
• Metabolic acidosis is one of the hallmark
• Initially optic disc is swollen but eventually
patient develops optic atrophy
• Treatment:
I. Lavage
II. IV NaHCO3
III. Antidote: Ethanol, fomepizole
IV. Others: Folinic acid
V. Hemodialysis
III
IV

68. TOXIN INDUCE NEUROPATHY( METHANOL)
• Bilateral optic atrophy
• Visual field defects

69. TAKE HOME MESSAGE
I. Papilledema must be differentiated from Pseudopapilledema cause true
papilledema needs extensive workup and specific treatment but
pseudopapilledema doesn’t.
II. Not all bilateral optic disc swellings are papilledema e.g,NMO,anti-
MOG,Toxic amblyopia, Diabetic papillopathy etc.
III. Optic neuritis and AION both may present with painful visual loss but
should be differentiated early specific management and better prognosis.
IV. Early differentiation of AAION and NAION is critical for medical management
V. Though IIH is called idiopathic there are multiple proposed mechanism for
its pathogenesis
VI. Diabetic and hypertensive patient both can present with Bilateral optic disc
swelling. In hypertension it’s a medical emergency but in diabetic papillopathy it
self limiting.

70. THANK YOU ……..