DISC EDEMA

Prof. Vasudev Anand Rao

CAUSES

UNILATERAL
Papillitis
Anterior Ischemic optic
neuropathy (AION)
Neuroretinitis
Papillophlebitis
retinal vein
Ischemic CRVO central
occlusion
Anterior compressive optic
neuropathies (orbital
tumors)
Infiltrative optic
neuropathies
Ocular hypotony IOP < 5mmHg
Foster-Kennedy syndrome

BILATERAL
Papilledema
Hypertension
Diabetic papillopathy
Advanced Graves disease
Cavernous sinus
thrombosis
Carotid cavernous fistula
Leber hereditary optic
neuropathy

PAPILLEDEMA: optic disc swelling

Conventionally the term refers to
hydrostatic non-inflammatory optic disc
swelling that results from raised intracranial
tension.
Normal IOP (10-20mmHg),
but increased CSF pressure (>20mmHg)

ETIOLOGY
1. Intracranial space occupying lesion
neoplasm (location of the tumor is more important than size)
abscess/inflammatory mass
hemorrhage/infarct
A-V malformation
2. Obstruction of ventricular system
3. Cerebral edema
4. Impaired CSF absorption by arachnoid villi:
Meningitis
Raised venous pressure
SAH/trauma subarachnoid hemorrhage
Communicating hydrocephalus
5. Severe systemic hypertension
6. Idiopathic (pseudo tumor cerebri):
7. Decreased size of cranial vault:
Craniosynostosis
Thickening of skull
8. Hypersecretion of choroids plexus tumor

CLINICAL FEATURES
SYMPTOMS
Ocular:
Visual acuity-normal in early ,decreased when
established and grossly affected when atrophic
Amaurosis fugax(spasm of arteries) transient monocular blindness
Central vision affected late(selective loss of peripheral
neurons)
Diplopia(assoc. 6th cranial nerve palsy in raised ICT)
intracranial tension

General:
Headache (bifrontal/occipital) more in the morning,
aggravated by coughing straining
Projectile vomiting
Loss of consciousness/ focal neurological deficits

CLINICAL FEATURES
SIGNS
1. PUPILLARY REACTION -normal until optic atrophy sets in
2. FUNDOSCOPY
EARLY PAPILLEDEMA

Hyperemia/elevation of disc
Blurred margins
Loss of SVP spontaneous venous pulsation
Superficial hemorrhage

+ Peripapillary halo

CLINICAL FEATURES
ESTABLISHED/FULLY DEVELOPED PAPILLEDEMA:

retinal nerve fiber

Engorged & tortuous veins
layer hemorrhage
Numerous flame shaped hemorrhages
Cotton wool spots, hard exudates
Peripapillary edema (patons lines)
Retinal folds/macular star

CLINICAL FEATURES
CHRONIC/VINTAGE PAPILLEDEMA:
Hyperemia decreases and optic atrophy sets in

Optic disc pale & elevated (champagne cork

appearance)
Disc obliterated
vessels connecting
Opticociliary shunts collateral
choroidal and retinal vasculature
* Peripapillary halo (high watermark)

CLINICAL FEATURES
ATROPHIC PAPILLEDEMA:

Pale grey disc with reactive gliosis

Narrow and sheathed vessels
Retina shows pigmentary changes and
choroidal folds

CLINICAL FEATURES
3. FIELD CHANGES
Early-normal
Established-enlargement of blind spot
Chronic-peripheral constriction
End stage-total loss
4. FLUORESCEIN ANGIOGRAPHY

To differentiate true and pseudopapilledema

Dilatation of surface capillaries and leakage of dye
in the late phase
5. NEUROIMAGING Intracranial tension
Features of raised ICT-silver beaten appearance with
erosion of posterior clinoid process and dorsum sellae
Cause of raised ICT may be identified.

UNILATERAL PAPILLEDEMA
Asymmetric
Foster Kennedy syndrome
Seen in patients with frontal lobe/olfactory lobe
tumors, meningiomas of olfactory
groove/sphenoidal wing, characterized by optic
atrophy on the side of the tumor caused by direct
pressure on the nerve and papilledema on the
opposite side because of raised ICT.
Prior optic atrophy, congenital abnormality in disc,
high myopia

PSEUDOTUMOR CEREBRI
Or Benign Intracranial hypertension
Defined by 4 criteria
1. Increased intracranial pressure
2. Normal or small ventricles
3. No evidence of intracranial mass lesion
4. Normal CSF composition
Usually idiopathic seen in young obese women

ETIOLOGY
Endocrine causes
Addisons disease
Hypoparathyroidism
Hyperthyroidism
Hypothyroidism
Menopause
Menarche
Pregnancy

Drugs
Vitamin A
Tetracycline
Steroids
OCP
Phenytoin anti-epileptic drug
Indomethacin NSAIDS
Growth hormone
lithium

TREATMENT

Weight loss
Acetazolamide carbonic anhydrase inhibitor - to reduce IOP
Lumbar puncture
Surgical decompression (ventriculo-peritomeal shunt)
VP-shunting
- shunt cerebrospinal fluid (CSF) from the
lateral ventricles of the brain into the peritoneum

DIFFERENTIAL DIAGNOSIS - Ocular

1. PAPILLITIS

inflammation of optic nerve head

Papillitis

Papilledema

1.Presentation

U/L

B/L

2.Vision

Sudden loss

Unimpaired initially

3.Pupil

RAPD present

RAPD absent

4.Media

Hazy near posterior vitreous

Media clear

5.Pain/tendeness of eyeball

Present

Absent

6.Hemorrhages/exudates

Less

More(in established)

7.Disc swelling

+2 to +3D

>+3D

8.Field defects

central/centrocaecal scotoma

Enlargement of blind spot, later

peripheral constriction.

9.X-ray skull

Normal

Silver beaten appearance, erosion

of dorsum sellae,post clinoid

10.CT/MRI

Demyleinating

ICSOL etc.
intracranial space occupying lesion

DIFFERENTIAL DIAGNOSIS - Ocular

2. PSUEDOPAPILLEDEMA
Hypermetropia:
Crowded nerve fibers at disc. More in
children, no enlargement of blind spot
Astigmatism
Optic nerve head drusen:
Calcium containing refractile bodies
within substance of optic nerve head.
Seen in USG. Autofluorescence
ultrasound

Hazy media

DIFFERENTIAL DIAGNOSIS - Ocular

anterior ischemic optic neuropathy

3. AION/LHON/TOXIC AMBLYOPIAS
Leber hereditary optic neuropathies

4. OCULAR HYPOTONY

Effusion from choroidal vessels

5. RAISED INTRAOCULAR PRESSURE:
Obliteration of peripapillary vessels by raised IOP

6. CRVO

OPTIC NEURITIS : Inflammation of the

optic nerve

ETIOPATHOGENESIS
1.
2.
3.

IDIOPATHIC
DEMYELINATING (Always Retrobulbar)
Isolated
a/w multiple sclerosis
neuromyelitis optica
schilders disease

ETIOPATHOGENESIS

INFECTIOUS AND PARAINFECTIOUS

LOCAL:
Orbital cellulites
Sinusitis
Teeth, tonsil
Meninges, brain or base of skull.
SYSTEMIC:
VIRAL-measles, mumps, rubella, chickenpox, herpes, CMV
and EBV.
BACTERIAL-T.B,syphilis,cat scratch disease,lymes
FUNGAL-cryptococcosis,histoplasmosis
PARASITImalaria,pneumocystis,toxoplasma,toxocara,cystice
rcosis
VACCINES:BCG,DPT,TT,HepB,variola and influenza

ETIOPATHOGENESIS
4.IMMUNE RELATED
LOCAL
Uveitis, sympathetic ophthalmitis.
SYSTEMIC
sarcoidosis, Wegeners polyarteritis nodosa, SLE etc.
5.METABOLIC
Anemia
Diabetes
Starvation
6.DRUGS AND TOXINS
INH, ethambutol, etanercept, INFa, tobacco, alcohol,
quinine.

CINICAL FEATURES
Commonly unilateral, more in females and mean age is 30-35 yrs.
SYMPTOMS
Triad of
Loss of central vision
Eye pain
Decreased colour vision

Other
Altered perception of moving objects
Worsening of symptoms with elevation of body
temperature(uhthoff sign)

CINICAL FEATURES
SIGNS

Decreased visual acuity

Tenderness
Marcus gunn pupil (RAPD)
Decreased colour vision and contrast sensitivity
Visual field defects: classically central/centrocaecal scotoma
but other defects can also occur
Fundus changes
1. Papillitis: edema, hyperemia, blurred margins, dilated
tortuous vs, few exudates and vitreous haze
2. Retrobulbar neuritis: normal
3. Neuroretinitis: macular star with exudates

VEP-Delayed latency and decreased amplitude

FAG to differentiate from other causes-dilated and telangiectatic
vs with leak from capillaries

CINICAL FEATURES

Field defects in optic neuritis

Papillitis

Neuroretinitis

INVESTIGATIONS
To determine cause for optic neuritis

1.Complete Hemogram
2. CRP, ESR, Mantoux
3. VDRL
4. Serology-ANA, Toxoplasma, Lymes
5. PNS X-ray, chest x ray(sarcoidosis)
6. X ray skull, CT
7. MRI(demyleinating plaques-2 or more predictive of
deveplopment of MS)
8. Lumbar puncture-CSF pleocytosis and oligoclonal
bands

MRI scan showing demyelinating

optic neuritis

TREATMENT
1. ONTT Regimen - Intravenous methylprednisolone 250mg q
6 h for 3 days followed by
Oral prednisolone 1 mg/kg/day for 11 days,
tapered with 20mg on 15th day and 10mg on 16th and 18th
day
2. Posterior sub-tenon injection of triamcinolone

3. Vitamin B12
4. Treatment of identifiable cause

Ischemic optic neuropathy

Infarction of prelaminar or laminar portions of optic
nerve caused by occlusion of posterior ciliary artery.
Seen in >50 yrs.
H/s/o giant cell arteritis or predisposing factors like
DM/HT
Pale swollen disc with splinter hemorrhages
Altitudinal scotoma
Classified as
Arteritic & Non-Arteritic

Clinical Features
Features

Arteritic AION

Non Arteritic AION

Age

>60yrs

40-60yrs

Sex Ratio

F>M

F=M

Vision loss

Severe

Moderate (>6/60)

Laterality

Fellow eye affected in

95% within days to wks

Fellow eye affected in <30% in

months or yrs

Optic disc

Pale edema, may be

sectoral

Hyperemic or pale edema

Assoc. Signs

Scalp tenderness, palpable

tender, non-pulsatile
temporal artery

Assoc. HT 40%, DM 24%

Shock, nocturnal hypotension

ESR

>40 mm in 1st hr

20-40mm in 1st hr

FAG

Disc and choroidal filling

delay

Disc filling delay

Treatment

IV methylprednisolone

? Levadopa-carbidopa

Prognosis

Poor

Improvement in upto 43%

THANK YOU