Papilledema is swelling of the optic disc caused by increased intracranial pressure. It is characterized by blurred disc margins and retinal nerve fiber layer edema. Increased CSF pressure is transmitted through the subarachnoid space and causes axoplasmic stasis in the optic nerve head. Papilledema is graded based on degree of disc elevation and obscuration from Frisen scale 0-5. Treatment involves addressing the underlying cause of increased ICP through medications, repeated lumbar punctures, or neurosurgery.

1. z
Papilledema
Shreeji Shrestha

2. z
 Hydrostatic noninflammatory swelling of optic disc
secondary to increased ICP
 Optic Disc edema or
Choked disc

3. z

4. z
 Subarachnoid communication between brain and ON

5. z
Axoplasmic transport
 ON axoplasmic transport ( protein, chemicals, mitochondria)
retrograde
IOP (11-21mmhg)
(Force)
Tissue pressure at optic nerve (6-8mmHg)
Retinal ganglion cell
LGB(degraded and returned to Cell body)
axons Orthograde

6. z
 Blood flow depends on
Vascular resistance,
BP,
IOP
 ONH blood flow - PP/R; PP is MABP-IOP

7. z
Etiology
Primary - Idiopathic ICH/pseudo tumor cererbi
Secondary - space-occupying lesion(midbrain, parietooccipital and cerebellum);
cerebral edema;
decrease in total volume within the cranial vault by
thickening of the skull;
Blockage of the flow of CSF (hydrocephalus);
Reduced absorption of CSF or increased production

8. z
Idiopathic intracranial hypertension
 Risk factors –
idiopathic,
Obese female,
pregnancy,
endocrine disorders,
drugs - tetracycline, nalidixic acid, amiodarone, lithium,
corticosteroid in high dose,
hypervitaminosis

9. z
Pathogenesis
 Mechanical theory - Hayreh theory – Inc
CSF - ON tissue pressure – block
axoplasmic transport (lamina) – venous
congestion – swelling of RGC axons
 Ischemic theory - Vasomotor theory –
inc ICP – Inc SVP – venous stasis –
reduced perfusion of axons

10. z
Other hypotheses includes,
 direct infiltration of the optic nerve head by CSF,
 swelling of glia,
 absence of the restraining influence of Muller cells in the
peripapillary retina,
 disequilibrium of hydrostatic pressure in the tissue and
bloodstream, and
 compression of the central retinal vein as it traverses the
subarachnoid space or cavernous sinus with elevated central
venous pressure

11. z
3 components
1. increased and fluctuating pressure in the distal optic
nerve sheath;
2. elevated central retinal venous pressure; and
3. impaired perfusion of the nerve fibers traversing the
lamina cribrosa

12. z
Pathology• neuronal swelling, venous and
capillary dilatation, abnormal
protrusion of the optic nerve
head toward the vitreous, lateral
displacement of the adjacent
retina, and folds of the posterior
retinal layers
• intra-axonal swelling is
accompanied by an increase in
mitochondria, disorganization of
neurofilaments, intracellular
membrane-enclosed bodies.

13. z
Development and resolution
 Papilledema takes over a week to develop,
2-8 hours (SAH) and resolve within hours, days or
weeks depending on the way ICP is lowered
Usually 6- 8 weeks after successful craniotomy
Less than a week after ON sheath decompression
2-3 months in IIH

14. z
Unilateral papilledema
 Sheltering uninvolved eye from elevated
pressure due to absence of subarachoid
space around anterior portion of ON
 Congenital or acquired (scaring)
 Causes Foster kennedy syndrome
Pseudo Foster kennedy syndrome

15. z
Pseudopapilloedema
 Hypermetropic eyes with small
lamina cribosa and crowded nerve
fibers
Swelling isnot >2D, no venous
engorgement, edema, blind spot not
enlarged
FA – no leakage

16. z
Features
 Headache - stretching of pain sensitive dura
and pial vessels
worse in awakening, Valsalva
 Transient blurring of vision – few sec, 20-30
times, ppt by bending posture
 Color vision – normal
 False localizing sign – lat rectus palsy due to
inc ICP

17. z
Stages
 Early
 Established
 Chronic
 Atrophic

18. z

19. z
Early Established Chronic Atrophic
Visual
acuity
normal Normal or diminised Variable
VF decreased
Impaired
symptom Transient blurring of vis
OD Nasal Blurring
margin
Mild
hyperaemia
Cup - present
Severe disc hyperaemia
Moderate disc elevation
Indistinct margin with
CWS, cup-obliterated
Severe disc
elevation, no CWS
Cup- abs
Dirty grey
color(gliosis),
elevated,
indistinct
margin
backgrou
nd
peripapillary
nerve striations
Retinal folds- paton
lines, hemorrhage,
Macular fan –
imcomplete star
Optociliary shunts
and drusen like
deposit- corpora
amylacea
Attenuated
peripapillary
vessels
Venous
pulsation(
20)
absent absent absent absent

20. z

21. z
 Visual Field Defects
 Concentric enlargement of the blind spot(30%) –
due to compression and lateral displacement of peripapillary
retina
Stiles - Crawford effect – peripapillary folds – light falls obliquely
in retina
 central and arcuate scotomas

22. z

23. z
Papilledema Grading System (Frisen Scale)
Stage 0 Normal Optic Disc
no elevation of Radial nerve fiber layer
obscuration of a major blood vessel on the upper pole
Stage 1 Very Early Papilledema
Obscuration of the nasal border of the disc
No elevation of the disc borders
Disruption of the normal radial NFL arrangement
Concentric or radial retrochoroidal folds

24. z
Stage 2 Early Papilledema
Obscuration of all borders
Elevation of the nasal border
Complete peripapillary halo
Stage 3 Moderate Papilledema
Obscurations of all borders
Increased diameter of optic nerve
head
Obscuration of one or more
segments of major blood vessels
leaving disc
Peripapillary halo irregular outer
fringe with finger-like
Stage 4 Marked Papilledema
Elevation of the entire nerve head
Obscuration of all borders
Peripapillary halo
Total obscuration on the disc of a
segment of a major blood vessel
Stage 5 Severe Papilledema
Dome-shaped protrusions representing
anterior expansion of the optic
nerve head
Peripapillary halo
Total obscuration of a segment of a
major blood vessel
Obliteration of the optic cup

25. z

26. z

27. z
Mechanical signs Vascular signs
1. Blurring of the disc
margin
2. Filling in of the optic
disc cup
3. Anterior extension of
the nerve head (3D =
1mm of elevation)
4. Edema of the nerve
fibre layer
5. Retinal (paton’s line) or
choroidal folds or both
1. Venous congestion of
arcuate and
peripapillary vessels
2. Papillary and retinal
peripapillary
hemorrhages
3. Nerve fiber layer
infarcts
4. Hyperemia of the optic
nerve head
5. Hard exudates of the
optic disc

28. z
Chronic Papilledema

29. z
Postpapilledema Atrophy

30. z
 Optic atrophy that results from chronic papilledema has a specific pattern
of axonal loss.
 Loss of peripheral axons with sparing of central axons has been
demonstrated in postmortem studies.
 Good central visual acuity despite severe papilledema and optic atrophy
is found in most patients with chronic papilledema

31. z
In the Foster Kennedy
syndrome, patients with
frontal lobe or olfactory
groove tumors develop the
triad of
(a) optic atrophy in one eye;
(b) papilledema in the other
eye; and
(c) anosmia

32. z
Diagnosis
 careful ophthalmoscopic examination
 Direct ophthalmoscopy – 3D - 1mm elevation
difference of 2-6 D
 red-free ophthalmoscopy and slit lamp
biomicroscopy
 Fluorescein angiography is often used to confirm
early papilledema

33. z

34. z
 CT scan – rule out IC lesions
If MRI is contraindicated, pacemaker, metallic clip
 MRI of orbits - subarachnoid space becomes distended, the nerve
sheath widens and there is flattening of the posterior sclera.
prelaminar optic nerve may enhance and protrude anteriorly.
 Confocal scanning laser tomography - gradually increasing
retinal surface elevation from the center of the disc to the disc
margin, with a steeper contour nasally than temporally.

35. z
Differential Diagnosis
1. Anomalously elevated optic disc
2. Intraocular inflammation
3. Asymptomatic nonarteritic anterior ischemic optic neuropathy (e.g.,
diabetic papillopathy)
4. Hypertensive disc edema
5. Optic neuritis, optic perineuritis (perioptic neuritis)
6. Infiltrative optic neuropathy (e.g., from leukemia)
7. Compressive optic neuropathy (e.g., from optic nerve sheath
meningioma)

36. z

37. z
LP and papilledema
 Usually contraindicated due to herniation of
brain through foramen magnum – pressure in
medulla - sudden death
 Guarded LP usually done
 Can be done in Pseudotumor cerebri
CSF for investigations

38. z
Treatment
 Treat the underlying cause
 IIH medical– acetazolamide, Mannitol, corticosteroid
surgery – repeated LP,
decompression - if failure of medical treatment,
progressive headache, progressive optic neuropathy;
Direct fenestration of ONSD,
Suboccipital craniectomy,
subtemporal decompression,
shunting procedure

39. z
Visual Prognosis
 more severe papilledema - worse visual prognosis
 Disc pallor with papilledema - indication of poor visual
prognosis, even if ICP is lowered immediately, because
the pallor is caused by loss of axons
 severe venous engorgement, retinal hemorrhages,
and hard and soft exudates have no prognostic
significance.

40. z
References
 Myron yanoff – neuroophthalomolgy
 Jacobiek - vol 3 neuroophthalmology
 Kanski
 Neuro-ophthalmology – A K khuruna