Papilledema

An overview

1. PAPILLOEDEMA

2. Definition ▪  Passive hydrostatic non inflammatory swelling of optic nerve head secondary to raised intracranial pressure. ▪  Usually bilateral ; may be unilateral. ▪  Optic disc swelling in the absence of raised intracranial pressure is referred to as optic disc edema.

3. Pathophysiology  Disturbance in axoplasmic flow causing stasis swelling of axons and leakage.  Increased intracranial pressure(ICT) is transmitted along subarachnoid space with optic nerve sheath acting as a tourniquet.  Increased ICT leads to increased optic nerve tissue pressure which alters pressure gradient resulting in stasis

4. Theories of Genesis  Mechanical Theory  Ischemic Theory  In most cases combined mechanism operates.

5. Causes(Bilateral)  Space occupying lesions.  Blockage of CSF flow.  Reduction in CSF resorption.  Increased CSF production.  Idiopathic Intracranial Hypertension.  Focal or diffuse cerebral edema.  Reduction in size of Cranial Vault.  Vitamin A toxicity.

6. Causes(Unilateral)  Foster kennedy syndrome  Previous unilateral optic atrophy.  Posterior fossa tumor.  Brain abcess .  Subarachnoid haemorrhage .  Optochiasmatic choroiditis .

7. Symptoms(Ocular)  Visual Acuity  Transient obscuration of vision.  Central vision affected late.  Horizontal Diplopia

8. Symptoms(General)  Headache more in the morning, intensifies with head movement, coughing or straining.  Projectile vomiting.  Loss of consciousness/ generalized motor rigidity.

9. Signs(Mechanical)  Elevation of the optic disc.  Blurring of the optic disc margin.  Filling in of the physiological cup.  Edema of the peripapillary nerve fiber layer.  Retinal or choroidal folds(Paton’s lines)  Macular fan .

10. Signs(Vascular)  Hyperemia of the optic disc.  Vascular congestion.  Peripapillary haemorrhage .  Exudates in the disc or peripapillary area.  Nerve fiber layer infarcts .

11. Grading of Papilledema (according to severity and its chronicity)

12. Early Papilledema  Disc elevation.  Venous distention and tortuosity.  Obscuration of the normal disc margin and overlying retinal vessels.  Absence of spontaneous venous pulsations

13. Established Papilledema  Marked elevation of nerve head with blurring of margins.  Engorged tortous venules.  Peripapillary splinter hemorrhages.  Cotton wool spots.  Hard exudates over the disc and macular area.

14. Chronic Papilledema(Classical “Champagne cork appears of disc)  disc hyperemia decreases and disc progressively appears pale in color.  Optociliary shunts and drusen like deposits may be present on the disc.  High water mark.

15. Atropic Papilledema Onset of optic disc pallor (secondary optic atrophy) .  Decrease in disc haemorrhage .  Narrowing of blood vessels and their ensheating .  Optic disc appears dirty white and blurred due to glial reaction.

16. Papilledema Grading System ( Frisen Scale)

17. Grade 0  Mild nasal NFL elevation.  Rare obscuration of a portion of major vessel (usually at superior pole)

18. Grade 1 (Very early Papilledema)  Obscuration of nasal border of disc  Normal temporal disc margin

19. Grade 2 (Early papilledema)  Obscuration of all the disc borders  Elevation of nasal border  No major vessel obscuration

20. Grade 3 (Moderate papilledema)  Obscuration of all borders  Increased diameter of optic nerve head  Obscuration of segment of major blood vessels as they pass disc margin.

21. Grade 4 (Marked papilledema)  Elevation of entire nerve head  Obscuration of all the borders  A segment of major vessel obscured on the disc

22. Grade 5 (Severe papilledema)  Anterior extension of optic nerve head  Total obscuration of vessel on disc surface  Obliteration of optic cup

23. Histopathological Findings Acute disc edema  Accumulation of extracellular fluid in and anterior to retinal lamina cribrosa, with enlargement of subarachnoid space with stretching.  Engorgement of axons occurs in prelaminar portion.

24.  Sensory retinal changes - Displacement of retina away from optic disc. - Buckling of the outer layers of retina. - Displacement of rods and cones away from their anchor near Bruch’s membrane. - Serious RD in peripapillary area

25.  Electron microscopy of axons - Axonal swelling and accumulation of mitochondria. - Mitochondrial swelling and disruption. - Disruption of fascicles of the microtubules.

26. Chronic disc edema  Degenerative and fibrotic changes in both anterograde and retrograde manner. (hence atrophy may occur anywhere from retinal nerve fiber layer to optic nerve)

27. Visual field changes  Enlargement of blind spot.  Earliest loss of visual field commonly involves inferior nasal quadrant.  Peripheral concentric constriction.

28.  Relative scotoma (first to green and red).  Complete blindness.  In all cases visual field changes should be monitored carefully and decompression to be done before peripheral constriction sets in.

29. Differential diagnosis of Papilledema  Papillitis.  Pseudopapilledema . - Drusen of optic disc. - High Hypermetropia (crowded nerve fibers at disc). - AION.  Optic neuritits .  Tilted optic disc.  Hypoplastic disc.  Myelinated nerve fibers.

30.  Papillitis

31.  Pseudopapilledema - Drusen of Optic disc

32.  Pseudopapilledema - Hyperopic disc

33.  Tilted optic disc

34.  Hypoplastic disc

35.  Myelinated nerve fibers

36. Investigations  History and physical examination including blood pressure measurement.  Ophthalmic examination - In addition to fundus examination, assessment of visual acuity, pupillary examination, ocular motility & alignment, and visual fields.  MRI with or without contrast is the best investigation of choice.

37. CT Scan  To rule out - Intracranial lesions. - Obstructive hydrocephalus. Can detect - Subarachnoid, epidural & subdural hemorrhages. - Acute infarctions. - Cerebral edema. Contraindication for MRI

38. Lumbar puncture Therapeutic procedure - Pseudotumor cerebri CSF for microbial and infectious studies.  Diagnostic - Recording opening pressure.

39.  Fundus Fluoroscence Angiography(FFA) Early Phase disc capillary dilation Dye leakage spots Microaneurysm over the disc Late Phase Leakage of dye beyond disc margin Pooling of dye around the disc

40. TREATMENT:  Treatment directed at underlying cause.  Timely intervention has a remarkable effect on prognosis. (unless nerve is irreversibly damaged)  Vision recovery is faster then subsidence of fundus features.

41.  Brain Tumor - Craniotomy to remove tumor.                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                      Resolution of papilledema within 6-8weeks.

42. Pseudotumor Cerebri - Medical Acetazolamide Oral Glycerol Corticosteroids Weight reduction - Surgical Repeated Lumbar puncture Decompression Shunting procedure  Resolution of papilledema within 2-3weeks of shunt procedure.

43.  Papilledema in PIH  General – bed rest.  Control of BP.  Control of edema – Diuretic, Hypertonic glucose.  Non responders – Termination of pregnancy.

44. Surgical Decompression Indications  Failure of Medical treatment - Marked disc swelling(>5D) - Engorged veins - Extensive hemorrhages - Early exudate spots - Progressive headache Progressive optic neuropathy (early field constriction)  Direct Fenestration of optic nerve sheath.

45. THERAPEUTIC SUCCESS :  Relief of headache.  Transient visual obscuration decreased.  Stability/ improvement of field defects.

46. Case report on papilloedema due to IIH A 20 year old female patient came with chief complaint of headache and blurring of vision since 3 days.She has been using Tab.Brutaflam 4mg.BD Tab.MAHAGABA M75OD since 1 week for generalized myalgia.On examination BCVA RE-6/24,LE-6/18.Pupils-Grade 1 RAPD BE present.Colour vision is normal.Anterior segment is within normal limits.Fundus examination with slit lamp indirect ophthalmoscopy with 90D revealed bilateral grade 4 optic disc edema.General physical examination is normal.Routine investigations were advised and patient was referred to neurophysician.Hb is 10.2mg/ dL.CT Brain is normal with bilateral basal ganglia calcifications.Patient denied for CSF analysis.She was diagnosed as IIH and treated with Tab.Diamox 250mg.BD,Tab.Lasilactone 50/20mg.OD and Oral Glycerol 20ml.TID.At one month follow up BCVA RE-6/9,LE- 6/6.Pupils are round reacting to light.Fundus showed resolving papilloedema,hard exudates in macula in a star pattern Conclusion: NSAIDs and GABA Agonists may cause IIH.

47. THANQ!!!