OPTIC DISC SWELLING

Dr Abdul Munim KhanAssociate Professor & HOD

Ophthalmology

Dr Khawaja Abdul Hamid

Assistant Professor Ophthalmology

Mohtarma Benazir Bhutto ShaheedMedical College Mirpur AJK

CASE PRESENTATION

Patient xyz, 35 year old female, housewife, married with three children

Resident of Khaiaban-e-Sir Syed, Rawalpindi

PRESENTING COMPLAINT

Severe headache …………..for the last four months

The headache occurred mostly early in the morning

Severe and generalized in nature.

It was Aggravated by coughing.

Relived with OTC analgesics

It was associated with vomiting, which was episodic and projectile and used to give transient relief to the headache

There was Progressive worsening in severity of headache

Decrease in response to analgesics occurred with time.

The patient also complained of posture related vague, visual obscurations lasting for few seconds only.

There was no history of any head trauma drug intake, fits, unconsciousness, drowsiness and diplopia

Past Medical, Surgical Was Insignificant

Belonged Middle Class

General physical examination

The patient was healthy but anxious, was well oriented in time , place and person.

Her vitals were:

Pulse - 60/min

B.P – 145/90mm hg

Afebrile

R/R – 15/min

Systemic examination

(Non-conclusive)

EYE EXAMINATION

R L

VISUAL ACUITY 6/9 6/9

COLOUR VISION NORMAL NORMAL

PUPIL ROUND,REGULAR,

REACTIVE

ROUND,REGULAR,

REACTIVE

•EXAMINATIOM OF ADNEXA AND ANTERIOR

SEGMENT OF EYEBALL WAS NORMAL

•EXTRA OCULAR MOVEMENTS WERE FULLIN ALL DIRECTIONS OF

GAZE

Fundus : Bilateral Disc Edema

Fundus photographs of the patient

PROVISIONAL DIAGNOSIS

BILATERAL ESTABLISHED PAPILLEDEMA

Cause ????????

Next step …………investigations

INVESTIGATIONS

IN ADDITION TO ROUTINE

VISUAL FIELDS

CT SCAN HEAD

CT ScanNON CONTRAST CT SCAN OF HEAD SHOWS

A 6x4cm LYTIC LESION IN BONY SKULL AT LEFT PARIETO-OCCIPITAL REGIONCAUSING MODERATE COMPRESSIONON ADJUCENT CEREBELLUM , OCCIPITAL LOBE AND FORTH VENTRICLE.

There is mid line shift towards right along with mild to moderate obstructed hydrocephalus.

Conclusion: metastatic deposit in bone, DD

may include aggressive meningioma.

Final diagnosis

Bilateral established papilledema as a result of raised intracranial pressure, because of left sided parieto-occipital space occupying lesion.

MANAGEMENT:

NEUROSURGICAL CONSULTATION

SURGERY TO REMOVE THE TUMOUR

PATIENT RECOVERING FROM SURGERY

Further management after the histo-pathological report

Disc swelling

Optic disc edema

Disc edema

Definitions

Papilledema is swelling of optic nerve head secondary to raised intracranial pressure

All other causes of disc swelling in absence of raised ICP are called optic disc edema

Pseudo papilledema is not true edema but mimics optic disc edema.

All patients with papilledema should be suspected of having intracranial SOL, unless proven otherwise.

not all patients of intracranial SOL have papilledema

Any intra-cranial tumor may induce papilledema.

It is most evident with tumors in the posterior fossa which obstruct the aqueduct of Sylvius, and least likely to occur with pituitary tumors.

The site of the tumor is, more important than its nature, its size and rate of growth.

papilledema does not develop- if the optic nerve has already become atrophic

Unilateral papilledema with optic atrophy on the other side, suggests a …….

frontal lobe tumor or an olfactory meningioma of the opposite side –

the Foster-Kennedy Syndrome.

Causes of Optic Disc swelling

1.Papilledema2.Disc edema

(Increased Intra Cranial Pressure)

SOL Tumor Glioma, Metastassis, Meningioma, Pituitary Adenoma, CP angle tumour

Hemorrhage

Trauma (hematoma, edema)

Increased CSF production

Choroid plexus tumor

Reduced CSFdrainage

Blockage of ventricular system

Tumor/cyst/infection (congenital/acquired)

Damage to arachnoidgranulations

Meningitis/Sub Arachnoidhemorrhage/cerebralvenous thrombosis

Idiopathic intracranial hypertension

other Malignant hypertension

Causes of papilledema

Inflammatory Optic neuritis

uveitis

Granulamatous TB

Sarcoidosis

Infiltrative leukemia

lymphoma

Vascular AION

CRVO

DM papillitis

Tumours Optic nerve (meningioma, glioma)

Hereditary LHON

Ocular hypotony

Causes of disc edema

Fundus Photographs of various conditions that cause disc edema

CAUSES OF PSEUDO-PAPILLEDEMA

OPTIC DISC DRUSEN

TILTED DISC

MEDULATED NERVE FIBERS

CONGENETAL DISC ANOMALIES

HYLOID REMANENTS OVER THE OPTIC DISC

GLIAL TISSUE OVER THE OPTIC DISC

CONGENETAL FULLNESS OF OD ASSOCIATED WITH HYPEROPIA

Fundus photographs of various conditions causing psuedo disc

edema

Patho-physiolgy

Sub arachniod space around the optic nerve is continuous with sub arachniod space of the brain

When ever the CSF pressure increases it is transmitted to optic nerve …….this causes

Interruption of axoplasmic flow in the optic nerve and

venous congestion.

histopathology The of acute optic disc edema shows

1. axoplasmic stasis,

2. edema, and

3. vascular congestion

Peri-papillary hemorrhages are also seen

Physiological cup is filled by edema

Small blood vessels are engorged and tortuous

Neural retina is displaced

On electron microscopy

Engorgement of axons

axons are filled with swollen mitochondria

The tissue in front of the lamina cribrosa has become more voluminous due to swelling of the nerve fibers and vascular congestion. The tissue bulges towards the vitreous cavity and pushes the retina sideways

COMMON PRESENTING SYMPTOMS

HEADACHE:

EARLY MORNING

PROGESSIVELY WORSINING (PATIENT USUALLY PRESENTS IN HOSPITAL WITHIN SIX WEEKS)

MAY BE LOCALISED / GENERALISED

TENDS TO GET AGGRAVATED WITH BENDING, HEAD MOVEMENT OR COUGHING.

VOMITING:

SUDDEN , PROJECTILE , PARTIALLY RELIEVING HEADACHE.

CAN OCCUR AS AN ISOLATED FEATURE

CAN PRECEDE THE ONSET OF HEADACHE BY MONTHS (SPECIALLY IN FOURTH VENTRICULAR TUMORS)

DETERIORATION OF CONSCIOUSNESS:

USUALLY SLIGHT, LEADING TO DROWSINESS AND SOMNOLENCE

DRAMATIC DETERIORATION OF CONCIOUSNESS IS INDICATIVE OF BRAINSTEM DISTORSION AND TENTORIAL / TONSILAR HERNIATION.

VISUAL SYMPTOMS:

TRANSIENT VISUAL OBSCURATIONS (FLASHES, BLACKOUTS , GREYOUTS)

HORIZONTAL DIPLOPIA

CAUSED BY STRETCHING OF SIXTH NERVE OVER THE PETROUS TIP

VISUAL FAILURE (LATE BECAUSE OF SECONDARY OPTIC ATROPHY).

STAGES OF PAPILLEDEMA

1. EARLY

2. ESTABLISHED

3. CHRONIC

4. ATROPHIC

VISION MECHANICAL CHANGES

VACULAR CHANGES

NO VISUAL SYMPTOMS

VA -NORMAL

BLURRING OF MARGINS OF OD

HYPEREMIA OF OD

LOSS OF SPONTANEOUS VENUS PULSATIONS

VISION MECHANICAL changes VACULAR CHANGES

TRANSIENT VISUAL DISTURBANCE

ENLARGING BLIND SPOT

ELEVATED DISC WITH INDISTINCT MARGIN CIRCUMFRENTIAL

RETINAL FOLDS (PATON’S LINES)

SEVERE HYPERMIA

VENOUS TORTUOSITY AND DILATION

FLAME SHAPED HEMORRHAGES.

COTTON WOOL SPOTS

HARD EXUDATES, FOVEAL STAR

STAGE 2 ESTABLISHED

VISION MECHANICAL CHANGES

VASCULAR CHANGES

VA – IMPAIRED

VISUAL FIELD DEFECTS

GLIOSIS OF PERI-PAPILLRAY NERVE FIBRE LAYER

DECREASE IN HYPEREMIA ,COTTON WOOL SPOTS AND HEMORRHAGES

OPTICOCILLIARY SHUNTS

SHEATING OF BLOOD VESSELS

STAGE 3 CHRONIC PAPILLEDEMA

CHAMPAGNE CORK APPEARANCE

VISION MECHANICAL CHANGES VASCULAR CHANGES

SEVERELY IMPAIRED VA

SWELLING DECREASES

SECONDARY OPTIC ATROPHY

DIRTY GREYISH WHITE

INDISTINCT MARGINS

NUMBER AND CALIBER of blood vessels on the disc is reduced

STAGE 4 ATROPHIC SECONDARY OPTIC ATROPHY

Another grading of papilledema especially for

benign intracranial hypertension

Grade I papilledema is characterized by a C-shaped halo with a temporal gap in the peri-papillary nerve fiber layer

With Grade II papilledema, the halo becomes circumferential

Grade III papilledema is characterized by loss of major vessels AS THEY LEAVE the disc

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

Grade V papilledema has total obscuration of all vessels of the disc.

it is extremely important to find out whether

Disc swelling present or not …..

And if there is disc swelling……is itpapilledema or optic neuritis .

•A careful history like hypertension, diabetes etc., should be taken. It should also include drug history particularly over dosage of Vitamin A, oral contraceptives, anti psychotics.

•A complete and thorough eye examination comprising of visual acuity, visual fields, refraction (with appropriate cycloplegic especially in children, and slit lamp examination of the fundus, vitreous, and macula).

Papilledema should be graded.

Difference between early papilledema and normal disc

1. Rule out pseudo-disc-edema by typical fundus appearance and other clinical signs

2. Spontaneous venous pulsations are present 80 % of the normal discs

3. For rest of 20 % do a FFA …..dye does not leak in normal discs

Difference between papilledema and other causes of disc edema

especially optic neuritis

history Headache, vomiting, Sudden loss of vision

VA normal Severely reduced

pain absent On movement of eye especially superiorly

laterality bilateral unilateral

pupil normal RAPD

Disc swelling +3 dioptres Less than 3 dioptres

Hemorrhages/ exudates More in established less

Visual fields Enlargement of blind spot Central or centraocecalscotoma

CT MRI SOL demyelination

Difference between papilledema and optic neuritis

INVESTIGATIONS

PERIMETRY:SHOW ENLARGED BLIND SPOT IN

ESTABLISHED STAGE AND ARCUATE FIELD DEFECTS IN LONG STANDING PAPILLEDEMA

B-SCAN:SHOWS RAISED OPTIC DISC

NEUROIMAGING (CT/MRI):TO LOCALIZE THE SPACE OCCUPYING

LEISION

FFAARTIRIAL PHASE:

CONGESTED CAPILLARIES ALONG

THE

NERVE FIBER LAYER.

AV PHASE HYPERFLOCESCENE OF DILLATED

CAPILLARIES, EXTENDING TO

ADJACENT RETINA.

LATE PHASE

MARKED HYPERFLORESCENCE

DUE TO LEAKAGE

A multidisciplinary approach is mandatory.

Ophthalmologist should guide the neuro-physicians/surgeons about the urgency of treatment by serially monitoring the

visual acuity

visual fields and

color vision

all these vital functions of eye change irreversibly when the papilledema progresses from established stage into chronic stage.

Neurophysician/surgeon should step up the anti-edema measures or intervene surgically at the earliest at this juncture

Treatment:

Treatment of the cause

And reduction of the increased CSF pressure by

Drugs

Shunt

CONCLUSION:

•Papilledema could be VISION AND LIFE THREATENING

•all doctors should be well aware about the importance of an eye examination in a case of headache when associated with visual disturbances like diplopia and vomiting.

Take home message

PERSISTANT HEADACHES SHOULD NOT BE TAKEN LIGHTLY

FUNDUS EXAMINATION IS MANDATORY IN CLINICAL EVALUATION OF SUCH PATIENTS.

TIMELY REFFERAL OF SUCH PATIENTS TO OPHTHALMOLOGY DEPARTMENT CAN BE LIFE AND VISION SAVING.

Thank you