Definition, Etiology, Pathophysiology, Clinical features, Management.

1. PAPILLOEDEMA
Nisha. S.S
M. Optom
ASO

2. INTRODUCTION…
• Optic disc or Optic nerve head

3. • The ganglion cell axons form the optic nerve after they leave the eye.
• The optic disc represents the beginning of the optic nerve and is the point where
the axons of retinal ganglion cells come together.
• It is also the entry point for the major blood vessels that supply the retina.
• The optic disc in a normal human eye carries 1–1.2 million afferent nerve
fibers from the eye towards the brain.

4. • The optic disc is placed 3 to 4 mm to the nasal side of the fovea.
• It is a vertical oval, with average dimensions of 1.76mm horizontally by
1.92mm vertically.
• There is a central depression, of variable size, called the optic cup.
• A normal optic disc is orange to pink in color.

7. DEFINITION
• Papilledema is swelling of the optic disc secondary to raised intracranial
pressure(ICP).
• The terms papilledema and disc oedema look alike and mean swelling of the
optic disc.
• However, the term ‘papilloedema’ is the disc swelling associated with increased
intracranial pressure and is nearly always bilateral, although it may be
asymmetrical.

8. • All other causes of disc oedema in the absence of raised intracranial pressure are
referred to as ‘disc swelling’ and are usually associated with persistent visual
impairment.
• By definition, papilledema cannot exist in the absence of high ICP, but high ICP
can occur in the absence of papilledema.

9. CAUSES OF OPTIC DISC OEDEMAAND
PAPILLOEDEMA
I. Increased intracranial pressure (tumour, haemorrhage, infarction, abscess, oedema,
benign intracranial hypertension).
II. Inflammatory optic neuropathy (optic or retrobulbar neuritis).
III. Infiltrative optic neuropathy (sarcoidosis, leukaemia and other malignancies).
IV. Optic nerve tumours (angioma, meningioma, childhood optic nerve glioma,
malignant optic nerve glioma, metastatic carcinoma).

10. V. Compressive optic neuropathy (Grave’s disease, sphenoid wing meningioma).
VI. Vasculopathies (anterior ischaemic optic neuropathy, central retinal vein
occlusion, malignant hypertension).
VII. Intra-ocular disease (posterior uveitis, posterior scleritis).
VIII. Venous obstruction (due to space occupying lesions in the orbit, cortico-
cavernous fistula, intrathoracic venous obstruction as by neoplasms, aneurysm of
aorta).
IX. Conditions associated with a massive increase in the protein content of CSF
(e.g., some cases of Guillain Barre syndrome and spinal tumours).
X. Pseudopapilloedema (optic disc drusen, hyperopia and other anomalies).
PDF : Papilloedema (Choked Disc) AK Agarwal*,Pushpa Yadav**, RK
Sharma**, Ratnesh Singh Kanwar***

11. PRODUCTION OF CSF
• Cerebrospinal fluid (CSF) is formed by Choroid plexus in the ventricles of the brain.
Circulation
• It leaves the lateral ventricles to enter the 3rd ventricle through the foramina of
Munro.
• From the 3rd ventricle, it flows through the Sylvian aqueduct to the 4th ventricle.
• From the 4th ventricle, the CSF passes through the foramina of Luschka and
Magendie to enter the sub-arachnoid space, some flowing around the spinal cord
and the rest bathing the cerebral hemispheres.
• Absorption is into the cerebral venous drainage system through the arachnoid villi.

14. • Normal CSF contains 0-5 mononuclear cells.
• It is measured in millimetres of mercury (mmHg).
• The CSF pressure, measured at lumbar puncture(LP), is 100-180 mm of
H20 (8-15 mmHg) with the patient lying on the side and 200-300 mm with
the patient sitting up.

15. ETIOPATHOGENESIS OF PAPILLOEDEMA
Causes of raised intracranial pressure :

16. Idiopathic intracranial hypertension (pseudotumour
cerebri).
Obstruction of the ventricular system by
congenital or acquired lesions.
• Space-occupying intracranial lesions,
including haemorrhage , Abscess ,
Arteriovenous malformation .
• Impairment of CSF absorption via arachnoid villi, which may be
damaged by meningitis, subarachnoid haemorrhage or cerebral
trauma
Cerebral venous sinus thrombosis
• Focal or Diffuse cerebral oedema from blunt head trauma or from
Toxic /Anoxia
• Severe systemic hypertension. • Hypersecretion of CSF by
a choroid plexus tumour, (very rare).

17. • Raised intracranial pressure associated with following conditions:
1. Congenital conditions include aqueductal stenosis and craniosynostosis.
2. Intracranial space-occupying lesions (ICSOLs) : These include brain tumours,
abscess, tuberculoma, subdural haematoma and aneurysms.
• The ICSOLs in any position excepting medulla oblongata may induce papilloedema.
• Papilloedema is most frequently associated with tumours arising in posterior fossa,
which obstruct aqueduct of Sylvius and least with pituitary tumours. Thus, the ICSOLs
of cerebellum, midbrain and parieto-occipital region produce papilloedema more rapidly
than the mass lesions of other areas.

18. 3. Intracranial infections such as meningitis and encephalitis may be associated
with papilloedema.
4. Intracranial hemorrhages : Cerebral as well as subarachnoid hemorrhage can
give rise to papilloedema which is frequent and considerable in extent.
5. Obstruction of CSF absorption via arachnoid villi which have been damaged
previously.
6. Tumours of spinal cord occasionally give rise to papilloedema.

19. 7. Idiopathic intracranial hypertension (IIH) also known as pseudotumour cerebri,
is an important cause of raised intracranial pressure. It is a poorly understood
condition, usually found in young obese women.
• It is characterised by chronic headache and bilateral papilloedema without any
ICSOLs or enlargement of the ventricles due to hydrocephalus.
8. Systemic conditions include malignant hypertension, pregnancy induced
hypertension (PIH), cardiopulmonary insufficiency, blood dyscrasias and nephritis.
9. Diffuse cerebral oedema from blunt head trauma may causes papilloedema.
10. Cerebral venous sinus thrombosis may also cause papilloedema.

20. Unilateral versus bilateral papilloedema
• Disc swelling due to ocular and orbital lesions is usually unilateral.
• In majority of the cases with raised intracranial pressure, papilloedema is
bilateral. However, unilateral cases as well as of unequal change do occur with
raised intracranial pressure.
• A few such conditions are as follows:

21. Foster-
Kennedy
syndrome
• It is associated with olfactory or sphenoidal
meningioma and frontal lobe tumours.
• In this condition, there occurs optic atrophy on the
side of lesion and papilloedema on the other side
(due to raised intracranial pressure).
Pseudo-Foster-
Kennedy
syndrome
• It is characterised by occurrence of unilateral
papilloedema associated with raised intracranial
pressure (due to any cause) and a pre-existing optic
atrophy (due to any cause) on the other side.

22. ❑ PATHOGENESIS
• Hayreh’s theory is the most accepted one.
• Increased intracranial pressure, malignant hypertension and orbital lesions produce
disturbance in the pressure gradient by increasing the tissue pressure within the
retrolaminar region.
• While, ocular hypotony alters it by lowering the tissue pressure within the
prelaminar area.
• Theory states that, `papilloedema develops as a result of stasis of axoplasm in the
prelaminar region of optic disc, due to an alteration in the pressure gradient across
the lamina cribrosa.’

23. • Thus, the axonal swelling due to axoplasmic stasis in prelaminar region is the
initial structural alteration, which in turn produces venous dilatation,
microaneurysm, hemorrhages in the disc and neighboring retina.
• The pressure-volume relationship between ICP, volume of cerebrospinal fluid
(CSF), blood, brain tissue, and cerebral perfusion pressure is known as the
Monro-Kellie doctrine.
• Because the total volume inside the cranium of blood, CSF, and brain is fixed,
any increase in volume of one of the cranial constituents must be compensated
for by a decrease in volume of the others or high ICP will result.

28. ❖Evolution and recovery
• Papilloedema usually develops quickly, appearing within 1–5 days of raised
intracranial pressure.
• In cases with acute subarachnoid haemorrhage it may develop even more
rapidly (within 2–8 hours).
• However, recovery from fully developed papilloedema is rather slow.
• It takes about 6–8 weeks to subside after the intracranial pressure is normalised.

29. ❑ EPIDEMIOLOGY
• Papilledema from various causes of IH may develop at any
age, in either sex, and in any racial or ethnic group.
Although high ICP can occur in infants and very young
children also.
• Idiopathic intracranial hypertension (IIH) predominantly
affects obese women of childbearing age.

30. INCIDENCE OF IDIOPATHIC INTRACRANIAL
HYPERTENSION
Country Incidence in
general
population
Incidence in
women
(childbearing
age)
Incidence in
obese women
(childbearing
age)
USA 0.9/100,000 3.5/100,000 13/100,000> 10% ideal
body weight
19.3/100,000 >20% ideal
body weight
UK 1.56/100,000 2.86/100,000 11.9/100,000

31. • In the USA, the annual incidence per 100,000 persons has been estimated to be 0.9 in
the general population and 3.5 in females 15–44 years of age.
• Durcan et al estimated that the incidence of IIH is up to 13 per 100,000 in obese
women aged 20–44 years of age who were 10% overweight and up to 19.3 per
100,000 where 20% over ideal weight.
• The incidence of IIH varies from country to country, probably related to the
prevalence of obesity.
• The incidence was 1.56/100,000 persons/year, 2.86/100,000 in women, and
11.9/100,000 in obese women in the Sheffield UK study.
• The reported annual incidence of IIH in Middle Eastern countries has been estimated
at 2.02–2.2/100,000 in the general population.

32. CLINICAL FEATURES

33. A. General features
• Patients usually present to general physicians with general features of raised
intracranial pressure.
• These include headache, nausea, projectile vomiting and diplopia.
B. Ocular features
• Patients may give history of recurrent attacks of transient blackout of vision
(amaurosis fugax).
• Visual acuity and pupillary reactions usually remain fairly normal until the late
stages of diseases when optic atrophy sets in, so, loss of vision is not a common
feature of papilloedema.

34. • Clinical features of papilloedema can be described under four stages:
✓Early
✓Fully developed
✓Chronic
✓Atrophic

35. 1. EARLY (INCIPIENT) PAPILLOEDEMA
Symptom
s
• Usually absent and visual acuity is normal.
• Pupillary reactions are normal.
Ophthalmos
copic
features
• Obscuration of the disc margins (nasal margins are involved first
followed by the superior, inferior and temporal).
• Blurring of peripapillary nerve fibre layer.
• Absence of spontaneous venous pulsation at the disc (appreciated in
80% of the normal individuals).
• Mild hyperaemia of the disc.
• Splinter haemorrhages in the peripapillary region may be present.
• Visual fields are fairly normal.

37. 2. ESTABLISHED (FULLY DEVELOPED)
PAPILLOEDEMA
Symptoms
• Patient may give history of transient visual obscurations in one or
both eyes, lasting a few seconds.
• Visual acuity is usually normal.
Pupillary
reaction
• remain fairly normal.

38. Ophthalmosc
opic features
• Apparent optic disc oedema is seen as its forward elevation above the plane of
retina; usually up to 1–2 mm (1 mm elevation is equivalent to +3 dioptres).
• Physiological cup of the optic disc is obliterated.
• Disc becomes markedly hyperaemic and blurring of the margin is present all
around.
• Multiple cotton wool spots and superficial haemorrhages may be seen near the
disc.
• Veins become tortuous and engorged.
• In advanced cases, the disc appears to be enlarged and elevated (mushroom or
dome shaped) so that vessels bend sharply over its margins.
• Paton’s lines i.e., circumferential greyish white folds may develop due to
separation of nerve fibres by the oedema.
• Rarely, hard exudates may radiate from the fovea in the form of an incomplete
star (macular star or macular fan).
• Visual fields show enlargement of blind spot.

43. 3. CHRONIC OR LONG-STANDING (VINTAGE)
PAPILLOEDEMA
Symptom
s
• Visual acuity is variably reduced depending upon the duration of the papilloedema.
• Pupillary reactions are normal.
Ophthalmo
scopic
features
• In this stage: • Acute hemorrhages and cotton wool spots resolve, and peripapillary
oedema is resorbed.
• The optic disc gives appearance of the dome of a champagne cork.
• The central cup remains obliterated.
• Small drusen like crystalline deposits (corpora amylacea) may appear on the disc
surface.
• Visual fields : Blind spot is enlarged and the visual fields begin to constrict.

45. 4. ATROPHIC PAPILLOEDEMA
Symptoms
• Atrophic papilloedema develops after 6–9 months of chronic papilloedema and is
characterized by severely impaired visual acuity.
• Pupillary reaction , light reflex is impaired.
Ophthalm
oscopic
features
• There occur greyish white discoloration and pallor of the disc due to atrophy of
the neurons and associated gliosis.
• Retinal arterioles are narrowed and veins become less congested.
• Whitish sheathing develops around the vessels.
• Visual felds.: Concentric contraction of peripheral fields becomes apparent as
atrophy sets in.

47. • Fundus photograph showing papilloedema: A, Early B, Established;
C, Chronic; D, Atrophic.

48. • Early (incipient) papilloedema
• Established (fully developed) papilloedema
• Chronic or long-standing (vintage) papilloedema
• Atrophic papilloedema
VISION….???
PUPILLARY REACTION…..???
VISUAL FIELD…….????

49. DIFFERENTIAL DIAGNOSIS

50. • Papilloedema should be differentiated from pseudopapilloedema and papillitis.
• Pseudopapilloedema is a non-specific term used to describe elevation of the disc
similar to papilloedema, in conditions such as optic disc drusen, and persistent
hyaloid tissue.
• The differentiating points between papilloedema, papillitis and
pseudopapilloedema (pseudopapillitis) :

53. ❑ DIAGNOSIS
1. Headaches may be of onset at any time of day but characteristically occur early in the
morning and may wake the patient from sleep. They tend to get progressively worse
and patients usually present to hospital within 6 weeks.
• The headaches may be generalized or localized, and may intensify with head
movement, bending or coughing. Patients with lifelong headaches often report a change
in character of the headache. Very rarely, headache may be absent.
2. Sudden nausea and vomiting, often projectile, may partially relieve the headache.
Vomiting may occur as an isolated feature or may precede the onset of headaches by
months, particularly in patients with fourth ventricular tumours.
3. Deterioration of consciousness may be slight, with drowsiness. Dramatic deterioration
of consciousness is indicative of brainstem distortion with tentorial or tonsillar herniation
and requires prompt attention.

54. 4. Visual symptoms
a) Transient obscurations lasting a few seconds are frequent in patients with
papilloedema.
b) Horizontal diplopia due to 6th nerve palsy caused by stretching of one or both
6th nerves over the petrous tip .
c) Visual failure occurs late with secondary optic atrophy due to long-standing
papilloedema.

55. ❑ INVESTIGATIONS
B-scan ultrasonography
CT scan of brain
MRI of Brain
Venography
Lumbar puncture
( Show an enlarged optic nerve diameter in most cases.)

56. ❑ TREATMENT
• High ICP due to a space-occupying mass, or hydrocephalus is typically treated
surgically.
• In the absence of a surgical lesion, patients should be screened for secondary causes
of IH and treated accordingly.
• Medications causing IH should be discontinued if possible.
• In cases where there is no identifiable cause for IH, the available options include
both medical and surgical modalities.
• Patients with minimal papilledema, no visual loss, and no other symptoms can be
treated conservatively and monitored frequently for development of symptoms or
vision loss.

57. • The visual field defects may not be recognized by patients, so they should be
monitored for visual field defects by formal perimetry.
• The main goals of treatment are alleviation of symptoms and preservation of vision.
• Conservative management options for IIH include weight loss and diet, carbonic
anhydrase inhibitors (typically acetazolamide), loop diuretics, and topiramate.
• Surgery is generally reserved for those patients who fail, are non-compliant with, or
are intolerant of maximal medical therapy.
• However, patients with acute fulminant papilledema and visual loss usually require
urgent surgical intervention to prevent further irreversible visual loss.

59. • The combination of pharmacotherapy, weight loss, and diet is the mainstay of
medical treatment of IIH.
• Weight loss, when it can be achieved, is probably the most effective long-term
therapy.
• It is important to emphasize that only a modest amount of weight loss (5%–10%
of total body weight) is usually required for improvement in symptoms and signs.

60. ➢ DIURETICS
• Acetazolamide, a carbonic anhydrase inhibitor, combined with a low sodium
weight reduction diet is a popular first line combination in IIH patients with mild to
moderate vision loss (500 mg of acetazolamide twice daily).
• Furosemide is a weak carbonic anhydrase inhibitor and chloride reuptake blocker
and has been used to treat IIH and it appears to be effective in some patients,
including those patients who fail acetazolamide.
• Weaker diuretics, including thiazides, digoxin, and glycerol, do not appear to be as
effective.

61. ➢ TOPIRAMATE
• Topiramate has also been used for IIH, and treats primary headache disorders.
• In one small randomized treatment trial, topiramate appeared to have similar
efficacy to acetazolamide for treatment of mild to moderate IIH.
• Topiramate often causes some weight loss as a side effect, which might have an
added advantage over other agents used for prevention of headache in IIH.
• Neurocognitive side effects may limit the use of topiramate in IIH, and is not our
first-line agent.

62. ➢ CORTICOSTEROIDS
• Steroids were once used commonly to treat IH, but their long-term use to lower ICP
is not recommended due to the risk of rebound IH on withdrawal.
• Further, the unfavorable long-term side effects of steroids such as weight gain in
these already obese patients could work against the overall goal of weight reduction
in IIH.
• High-dose intravenous steroids can, however, be utilized as a temporizing measure
to treat fulminant IIH patients with acute and severe visual loss until more definitive
surgical intervention is completed.

63. SURGICAL TREATMENT

64. • In general, patients with acute fulminant IIH, severe vision loss at presentation or
progressive vision loss despite maximum medical management, require surgical
intervention.
• The surgical options include
✓ Optic nerve sheath fenestration (ONSF)
✓Shunt technique
Ventriculoperitoneal shunt [VPS]
Lumboperitoneal shunt [LPS]
Ventriculoarterial shunt (VAS)
✓Venous Sinus Stenting.

65. • ONSF is an effective method for stabilizing visual loss and protecting the optic
nerve from further damage with IIH.
• Keltner proposed that the mechanism of action of ONSF is local
decompression of the subarachnoid space.
• VPS and LPS are CSF pressure-lowering procedures that can be effective in
selected patients with IIH who fail maximum medical therapy.

66. SUMMARY OF CLINICAL MANAGEMENT
CONSIDERATIONS
Etiology of papilledema Treatment considerations
Space-occupying mass, Chiari malformation, or
hydrocephalus
Consider specific surgical therapy.
Direct treatment to the underlying cause.
Secondary causes Treat the underlying cause .
Cerebral venous sinus thrombosis Consider adding anticoagulation.
Potential contributing factors
IIH with minimal signs or symptoms, and no visual
loss
Diet, weight loss
Monitor visual loss by formal perimetry
IIH with symptoms and visual loss Diet, weight loss, diuretics
Monitor closely,Medical therapy

67. IIH with failed maximal medical therapy Consider surgical options
IIH with acute fulminant papilledema and visual loss Consider urgent surgical intervention
Use temporizing measures such as serial lumbar
punctures or lumbar drain to halt visual loss while
definitive surgical treatment is awaited.

68. THANKYOU….