This document discusses papilloedema, which refers to optic disc swelling caused by increased intracranial pressure. It defines various types of optic disc swelling including unilateral, bilateral, and papilloedema. It also discusses pseudo-papilloedema. The causes, signs, symptoms, diagnostic workup, and grading scale of papilloedema are explained in detail. Treatment options for increased intracranial pressure including idiopathic intracranial hypertension involve medical management with carbonic anhydrase inhibitors and weight loss as well as potential surgical interventions.

1. PAPILLOEDEMA
Dr/ Reyad Abo Yossif (MRCOphth)
Ophthalmology specialist
Shebin Elkom ophthalmology hospital

2. Definitions
- Optic disc oedema.
- Unilateral optic disc swelling.
- Bilateral optic disc swelling.
- Papilloedema.
- Pseudo- Papilloedema.

3. Optic disc oedema
Optic disc edema refers to the ophthalmoscopic swelling
of the optic disc with a concurrent increase in fluid within
or surrounding the axons.

4. Unilateral optic disc swelling
Unilateral optic disc swelling with the most likely
etiological categories are:
- Non Arteritic Ischemic Optic Neuropathy
- Arteritic Ischemic Optic Neuropathy
- Inflammatory optic neuritis
- Compressive optic nerve tumor
- Central retinal vein occlusion

5. Unilateral optic disc swelling
Optic nerve functions are affected:
- Visual Acuity diminution
- Pupillary affection (+ve RAPD)
- Color vision affection

6. Bilateral optic disc swelling
Bilateral optic disc swelling with the most likely
etiological categories are:
- Increased intracranial pressure (ICP) (Papilloedema)
- Infectious/inflammatory
- Demyelinating
- Toxic-metabolic/nutritional,
and Hypertensive emergency (ie, malignant HTN).

7. Bilateral optic disc swelling
Optic nerve functions are normal
- Normal Visual Acuity
- Normal Pupillary response
- No Color vision problem
(except late if optic atrophy occurred)

8. Papilloedema
Bilateral optic disc swelling due to increased ICP can be
caused by many disease processes including :
- intracranial space occupying lesions
, obstructions of the arachnoid villi,
, CSF producing tumors
and idiopathic intracranial hypertension (IIH).

10. Pseudo-Papilloedema
Pseudo-papilledema is defined as anomalous
elevation of one or both optic discs without edema
of the retinal nerve fiber layer.

11. It is important to distinguish pseudo-papilledema
from true papilledema, which can be the first sign
of disease process with the potential for vision loss,
neurological impairment, or death.
Patients thought to have papilledema are often
subjected to lumbar puncture, MRI, and extensive
laboratory studies to find the underlying cause.

12. Papilloedema

13. Aetiology
 Papilledema is due to increased intracranial pressure from
any cause.
 Normal ICP is typically less than 25 cm water when
measured with a manometer in the recumbinat position.
 Papilledema results from orthograde axoplasmic flow
stasis at the optic nerve head leading to edema of the nerve
from the increased intracranial pressure pressing on the
nerve behind the eye.

17. Increased intracranial pressure can be
caused by:
(1) a space occupying lesion such as a tumor or
hemorrhage,
(2) decreased CSF drainage due to:
blockage (obstructive hydrocephalus) or decreased
absorption (infection, hemmorhage, venous sinus
thrombosis or venous outflow obstruction),
(3) Idiopathic Intracranial Hypertenion (IIH, also called
pseudtumor cerebri) ,
(4) increased CSF production such as from a choroid plexus
tumor (rare),
(5) decreased skull volume (craniosynostosis).

18. Continued pressure can result in loss of axons and eventual optic atrophy
secondary to intraneuronal ischemia ultimately causing vision loss.
With optic atrophy there is little or no edema seen even with continuing
increased intracranial pressure since there are no fibers to swell.

19. History
 Headache is typical and is often worse on waking or
postural (worse on lying down and better on standing
up).
 It is useful to ask about medicines that may cause
increased intracranial pressure such as tetracyclines,
vitamin A and derivatives, lithium, steroids - especially
changes in the amount of steroids taken and withdrawal
of steroids.

20. Physical examination
 In addition to the usual ophthalmic exam including visual
fields , one should check blood pressure to rule out
hypertensive emergency (i.e., “malignant hypertension”)
which can mimic papilledema.
 Ocular motility exam may reveal a unilateral or bilateral
6th nerve palsy as a non-localizing signs of increased
intracranial pressure.

21. Symptoms and signs of increased ICP
 Headache is common with increased intracranial
pressure and often worse in the morning.
 Nausea and vomiting are noted with increased
intracranial pressure, especially with an acute rise in
pressure. This is made worse by straining, coughing or
bending.
 Pulse synchronous tinnitus may be present from
increased flow in stenotic cerebral venous sinuses.

22. Symptoms and signs of increased ICP
 Visual field loss (usually an increased size of the blind spot
initially) can occur acutely (i.e., fulminant) or be slowly
progressive.
 The patient may note short periods lasting seconds of losing
vision which are called transient visual obscurations. These
are often observed when raising to stand or changing
position.
 A hyperopic shift may occur in the refraction due to axial
length shortening.

23. Symptoms and signs of increased ICP
 Long standing papilledema may result in severe peripheral
visual field loss and may progress to involve central
vision if untreated.
 Increased intracranial pressure can also lead to cranial
nerve palsies, usually an abducens palsy as a non-
localizing finding. It is thought that the long course of the
6th cranial nerve is compressed by the increased CSF
pressure.

25. Clinical diagnosis
Papilledema Grading System (Frisen Scale)

26. Papilledema Grading System
(Frisen Scale) in detail

27. Papilledema Grading System (Frisen Scale)
Stage 0 - Normal Optic Disc
Blurring of nasal, superior and inferior poles in inverse
proportion to disc diameter.
Radial nerve fiber layer (NFL) without NFL tortuosity.
Rare obscuration of a major blood vessel, usually on the
upper pole.

28. Papilledema Grading System (Frisen Scale)
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 with grayish opacity
accentuating nerve fiber layer bundles.
Normal temporal disc margin. Subtle
grayish halo with temporal gap (best
seen with indirect ophthalmoscopy).
Concentric or radial retrochoroidal
folds.

29. Papilledema Grading System (Frisen Scale)
Stage 2 - Early Papilledema
Obscuration of all borders.
Elevation of the nasal border.
Complete peripapillary halo.

30. Papilledema Grading System (Frisen Scale)
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 the disc.
-Peripapillary halo-irregular
outer fringe with finger-like
extensions.

31. Papilledema Grading System (Frisen Scale)
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.

32. Papilledema Grading System (Frisen Scale)
Stage 5 - Severe Papilledema
Dome-shaped protrusions representing anterior expansion of
the optic nerve head.
Peripapillary halo is narrow and smoothly demarcated.
Total obscuration of a segment of a major blood vessel may or
may not be present.
Obliteration of the optic cup.

34. Papilledema Grading System
(Frisen Scale) in breif

35. Grade I papilledema is characterized by a C-shaped halo
with a temporal gap

36. Grade II papilledema, the halo becomes circumferential

37. Grade III papilledema is characterized by loss of major
vessels AS THEY LEAVE the disc (arrow)

38. Grade IV papilledema is characterized by loss of major
vessels ON THE DISC.

39. Grade V papilledema has the criteria of grade IV plus partial
or total obscuration of all vessels of the disc.

40. Diagnostic procedures
-CNS imaging study (CT or MRI with contrast) to identify a
central nervous system mass lesion.
-MR venography(MRV) or CTV should also be considered to
look for venous sinus thrombosis.

41. Diagnostic procedures
Lumbar puncture (to be done after imaging demonstrates no risk
for herniation) to document increased ICP and to look for
neoplastic, infectious or inflammatory causes. At a minimum,
opening pressure along with glucose, protein, cell count and
differential, culture are obtained..

42. Diagnostic procedures
Fluorescein angiography can detect disc leakage in true
optic disc edema as opposed to disc staining without
leakage in pseudopapilledema.

43. disc leakage in true optic disc
edema

44. disc staining without leakage in
pseudopapilledema

45. Diagnostic procedures
Autofluorescence may show pseudopapilledema (e.g.,
optic disc drusen).

46. Diagnostic procedures
B scan ultrasound may show pseudopapilledema (e.g.,
optic disc drusen).

47. Dilated Optic Nerve Sheath with Papilledema with
disc elevation , no calcification

49. Diagnostic procedures
Successive fundus images may be helpful to follow the disc
status over time.

50. Diagnostic procedures
Automated perimetry commonly shows enlarged blind spots and
nerve fiber layer type visual field defects. In severe chronic
papilledema worsening peripheral field loss may be seen and can
eventually involve central vision loss.

51. Management of bilateral optic disc swelling
1- Complete routine history taking and examination
2- Answer these questions:
- Is it a unilateral or bilateral disc swelling ?
- What about optic nerve functions (VA , RAPD ,color) ?
- Differential diagnosis of bilateral disc oedema ?
- Blood pressure measurement to rule out malignant HTN (search
for hypertensive changes during fundus examination)
- MRI and MRV with contrast to rule out space occupying lesions
or venous sinus thrombosis
- Lumbar puncture to rule out IIH (Idiopathic Intracranial
Hypertension)

52. Management of bilateral optic disc swelling
- Fundus photo for documentation and follow up
- Automated perimetry for follow up
- Think about other causes of bilateral disc oedema other than
increased intracranial pressure as sarcoidosis , toxoplasmosis ,
VKH ,posterior scleritis , posterior uveitis causes
- Don’t forget to exclude pseudo-papilloema (optic disc drusen ,
crowded disc in hypermetropia, tilted disc in high myopia , optic
disc hypoplasia)

55. Notes on workup
When evaluating a patient for IIH, a complete ocular
examination including a dilated fundus examination,
visual field examination, and optic nerve photographs is
required.
Subsequently, neuroimaging is needed to exclude
secondary causes of intracranial hypertension. Magnetic
resonance imaging (MRI) and MR venography (MRV) of
the brain are usually the imaging modalities of choice.

56. Notes on workup
Furthermore, a lumbar puncture is recommended for all patients
who are suspected of having IIH.
The diagnosis is based upon an elevated opening pressure greater
than 25 cm H2O taken with the patient lying in the lateral
decubitus position.
Values (20 - 25 )cm H2O are considered equivocal.
In obese or sedated children, pressures up to 28 cm H2O may be
considered normal.

57. Notes on workup
The CSF must also be studied to rule-out
inflammation, tumor cells, and infection.
Patients with IIH usually have normal or low protein
level, normal glucose levels and a normal cell count

58. Notes on workup
The primary cause of the increased intracranial pressure
must be addressed.
If a mass is present, primary therapy should be directed
towards that lesion (e.g., surgery).
If medications (tetracyclines, vitamin A analogues, etc.)
are felt to be causative they should be discontinued.

59. Notes on workup
Patients with mild papilledema should be
monitored for visual field defects by formal
perimetry as the patients may not be able to
recognize them.

60. Prognosis of papilloedema
Chronic papilledema leads to axon loss with constriction
of the visual field, loss of disc substance and in the end,
loss of central acuity.
Patients (especially those with IIH) need to be followed
to prevent vision loss.

61. Pseudotumor cerebri
(Idiopathic Intracranial Hypertension)
(IIH)

62. Pseudotumor cerebri
(idiopathic intracranial hypertension) (IIH)
It is a disorder characterized by increased intracranial
pressure (ICP) of unknown cause that predominantly
affects obese women of child-bearing age.
Papilledema is the primary ocular finding and may
progressively lead to optic atrophy and blindness if no
treatment is provided.

63. Pseudotumor cerebri
(idiopathic intracranial hypertension)
IIH may be seen in any gender or age group but has a high
predilection for females of childbearing age, especially
when coupled with obesity.
While males are less frequently affected, constituting less
than 10% of adult IIH patients, the affected population
also tend to be obese and are more likely to sustain worse
visual prognosis compared to their female counterparts.

64. Pseudotumor cerebri
(idiopathic intracranial hypertension)
Interestingly, in the prepubertal age group, IIH has no
particular predilection for obesity or female gender.
Associated conditions include migraines and Down
syndrome.

65. Pseudotumor cerebri
(idiopathic intracranial hypertension)
Certain systemic illnesses have been associated with IIH, including
obstructive sleep apnea, hypothyroidism, anemia, Addison disease,
systemic lupus erythematosus, Behçet's syndrome, polycystic
ovary syndrome, coagulation disorders, and uremia.
Certain medications have also been associated with IIH, including
tetracyclines, vitamin A, lithium, anabolic steroids, oral
contraceptive pills, nalidixic acid, and cyclosporine.

66. Diagnostic Criteria
IIH is a diagnosis of exclusion. Nonetheless, diagnostic criteria were established
by Dandy in 1937. However, over the years some modifications were provided
and the new criteria are now known as the modified Dandy criteria:
1- Signs and symptoms of increased ICP (headaches, nausea, vomiting, transient
visual obscurations, papilledema)
2- No localizing neurologic signs, except for unilateral or bilateral sixth cranial
nerve palsy
3- CSF opening pressure >25 cm H2O with normal CSF composition
4- No evidence of hydrocephalus, mass, structural, or vascular lesion (including
venous sinus thrombosis) on imaging
5- No other cause of increased ICP identified

67. Medical therapy
Carbonic anhydrase inhibitors (acetazolamide) and weight loss (of
about 5-10% ) are first line therapy for IIH but can be used in
patients with elevated ICP from other etiologies.
Recent clinical trials (e.g., Idiopathic Intracranial Hypertension
Trial (IIHT)) have provided strong support for safe and effective
use of acetazolamide combined with weight reduction in
effectively treating IIH and improving papilledema in these
patients with mild vision loss

68. Medical therapy
Furosemide (Lasix) and topiramate are considered second line
agents in IIH but may be useful in patients who are intolerant of
acetazolamide.
Topiramate, sold under the brand name Topamax , is
a Carbonic anhydrase inhibitor medication used to
treat epilepsy and prevent migraine)

69. In general however as steroids (especially with change in dosage
or withdrawal) have also been causative of increased ICP we
generally do not recommend steroids for IIH.
Serial lumbar punctures are not typically recommended as a
treatment for IIH but can be used to treat intracranial hypertension
if the patient is not suitable for medical therapy or is pregnant or
has cryptococcal meningitis related increased ICP.
Medical therapy

70. Surgery
Potential indications for surgery in patients with IIH:
Development of a new visual-field defect
Worsening of a previous visual field defect
Severe visual loss at the time of presentation
Anticipated hypotension induced by treatment of high blood
pressure of renal dialysis
Psychosocial reasons: non-compliance to medication, inability to
perform visual field studies
Refractory headache

71. Surgery
The two most used procedures are CSF diversion via shunt and
optic nerve sheath fenestration. The choice of the procedure is
based on patients’ signs and symptoms.

72. Surgery
Optic nerve sheath fenestration is the preferred surgical procedure
for papilledema with associated severe vision loss but no or minimal
ICP symptoms (such as headache). It has been shown to preserve or
restore vision in 80-90% of patients. The procedure involves
incisions in the abnormally bulbous anterior dural covering of the
optic nerve sheath which creates an outlet for continuous CSF
drainage. Consequently, the CSF no longer distends the sheath and
axoplasmic flow in the optic nerve is restored. It is also considered
to be the safest approach among patients with renal failure requiring
hemodialysis and for vision loss occurring during pregnancy.

73. Surgery
CSF shunting produces rapid reduction in ICP and is therefore most
beneficial among patients with vision loss and symptoms of raised ICP.
Two types are available: lumbo-peritoneal (LP) and ventriculo-peritoneal
(VP). Although VP shunting is more difficult, as IIH patients do not have
enlarged ventricles, it is the preferred method due to its lower complication
rate. Complications of CSF shunting including shunt obstruction, shunt
migration, intracranial hypotension, and tonsillar herniation. Sinclair et al.
found that shunt revisions were needed in 51% of patients, with 30%
requiring multiple revisions.

74. Surgery
Venous sinus stenting (VSS) is an emerging procedure based on the findings of
venous sinus stenosis in IIH patients. After identification of the stenotic area on
MRV, further testing (e.g. digital subtraction venography and manometry) is
needed to establish a pressure gradient (typically ≥ 10mmHg) before proceeding
with stent placement. VSS has been shown to decrease cerebral venous pressure,
leading to increased CSF absorption and consequent decrease ICP. Some studies
have demonstrated improvement in symptoms following this procedure. However,
despite its possible beneficial outcomes, the procedure may be associated with
serious complications such as stent migration, venous sinus perforation, in-stent
thrombosis, subdural hemorrhage and recurrent stenosis formation proximal to the
stent.

75. Prognosis
Factors independently associated with a worse visual outcome:
-Gender (male)
-Race (Black)
-Morbid obesity
-Anemia
-Obstructive sleep apnea
-Acute onset of symptoms and signs of raised ICP (fulminant IIH)
Recurrence may occur in 8 to 38% of patients weeks to years following recovery
from initial presentation or a prolonged period of stability.
Weight gain has been associated with disease recurrence.