Papilledema & Pseudopapilledema

Jennifer Hue Integrative seminar Fall 2011

Case Presentation
CC: 47 y/o WF for VFSS24-2 f/u OS Active Hx: myopia, pseudo-papilledema OS BCVA: 20/20 OD, OS, OU Externals WNL Current Rx: OD -4.75 sph OS -4.25 sph

Additional Testing
VFSS24-2 3 pts flagged centrally & inferonasally only 1 pt repeatable, but overall consistent with previous VF in 2010 9/14 fixation losses Red cap desaturation OD/OS: both central & peripheral caps equal Fundus photo, on comparison to 2010 photos: no change in appearance, ONH borders & vessels appear stable OD/OS

Assessment & Plan

A: Pseudo-papilledema OS ONH appears stable, performed VFSS24-2 Fundus photos taken P: RTC 2 mos for GDx OD/OS & VF Sita-Fast screening OD/OS (pt unable to maintain steady fixation with SS24-2) Pt edu on physiological variation of ONH as of current info from testing.

2010

2011

Pseudopapilledema
Causes most commonly produced by optic disc drusen; may be buried within disc and therefore not visible drusen may be from axonal degeneration from altered axomplasic flow drusen tends to be on surface in adults > children high hyperopia with heaping up of nerve fibers Characteristics incidence = 20 in 1000 present in whites >> blacks VF defects: ~70% in those with visible drusen; ~35% in those without VA normal, (-) visual sx's

Differential Dx
Feature
disc color

Early papilledema
hyperemic

Pseudo-papilledma
pink, yellowish-pink

disc margins

indistinct early

irregularly blurred, may be lumpy

disc elevation minimal

minimal to marked, center of disc is most elevated emanate from center, frequent anomalous pattern, (+/-) SVP

vessels

normal distribution, (-) SVP

NFL

dull due to edema, which may be NO EDEMA; may glisten obscuring blood vessels more flame-shaped more dot-blot

hemorrhages

Pathogenesis of Papilledema
Elevated intracranial pressure ==> elevated CSF pressure in the subarachnoid space of the intraorbital portion of optic nerve Nerve fibers within the optic nerve are compressed Obstruction of axoplasmic flow, which appears to be the primary cause of optic disc swelling This results in axonal & retinal ganglion cell destruction Axoplasmic stasis leads to venous obstruction & dilation, hypoxia of the NFL & vascular telangiectasias of the optic discs

Ophthalmoscopic Features
Bilateral disc edema: may be asymmetric, but rarely unilateral Opacification of peripapillary NFL Disc hyperemia Loss of SVP; (+) SVP is a good sign --> indicates that the ICP <200 mmH20 Veinous distention Hemorrhages Exudates Cotton-wool spots Circumferential retinal folds in peripapillary region Obliterated central cup (late finding)

Classification of Papilledema

Early: disc changes before dev't of obvious disc swelling opacification of peripapillary NFL (obscuring sup & inf disc margins) disc hyperemia from dilation of capillaries on disc surface (use red-free filter)

http://content.lib.utah.edu/cdm4/item_viewer.php? CISOROOT=/EHSL-WFH&CISOPTR=251

Classification of Papilledema
Fully developed: the surface of disc lies above plane of retina small & large vessels buried usu. accompanied by flameshaped hemorrhages & CWS Paton's folds: circumferential retinal folds (may be due to the lateral displacement of retina) possible presence of macular star

http://webeye.ophth.uiowa.edu/eyeforum/cases/papilledema-grading.htm

Classification of Papilledema

Chronic: with persistence of elevated IOP over months, hemorrhagic & exudative components resolve disc develops a "champagne cork" appearance obliteration of central cup ONH appears milky gray

http://www.osnsupersite.com/view.aspx?rid=29815

Classification of Papilledema

Post-papilledema atrophy: disc is atrophic & grayishwhite narrowed, sheathed vessels significant visual field, color vision & VA loss from chronic obstruction of central retinal veinous drainage

http://eyewiki.aao.org/Papilledema

Associated Clinical Features

Visual symptoms in both early & fully developed papilledema, pts are usually asymptomatic this is useful in differentiating from other types of disc swelling, such as inflammation or ischemia. possible transient dimming of vision VA loss - in late stage; occurs gradually & only after severe peripheral field loss VF defect - enlargement of blind spot is most common and often the only VF defect; however, this is not helpful in early dx bc disc swelling is visible on o-scope viewing prior to this field change Pupillary function - normal in early papilledema afferent pupillary defect may be present in eye with larger visual field loss Diplopia - increased intracranial pressure may result in abducens nerve palsies

Management
Long-term lowering of intracranial pressure neurosurgical intervention if imaging reveals mass lesion if lesion cannot be removed, then alternate procedure for diverting CSF is indicated (i.e. shunt) Surgery for decompression of optic nerve sheath does not lower ICP, but 2/3 of pts report improvement in HA's long-term improvements in visual fcn & optic disc edema have been noted

Visual Prognosis
difficult to determine! disc pallor & vascular sheathing signify irreversible changes in nerve tissue extensive field loss, color vision abnormalities, & afferent pupillary defect indicate at least some permanent visual damage severe venous engorgement, retinal hemorrhages, and exudates are of no significance

The efficacy of optic nerve ultrasonography for differentiating papilloedema from pseudopapilloedma in eyes with swollen optic discs Neudorfer, et al.
Methods: prospective study that evaluated 44 pts with bilateral optic disc swelling who underwent a thorough neuro-ophthalmic exam, which included optic nerve ultrasound (A- & B-mode); findings were compared with clinical assessment. Results: U/S detected papilloedema with: high sensitivity: 85% when normal optic nerve width was set at <= 3.3mm; 95% when ONW set at <=3.0mm high negative predictive value: 83% when normal optic nerve width was set at <= 3.3mm; 93% when ONW set at <=3.0mm Conclusions: Ultrasonography findings correlated well with the final dx of papilloedema or pseudopapilloedema, especially when the upper limit of normal ONW was set at 3.0mm. Ultrasonography could be a a useful tool to augment differential diagnoses of swollen discs.

References

Kline, L. B. & Foroozan, R. (2007). Optic Nerve Disorders, 2nd York: Oxford University Press. Neudorfer, M., & Siegman, M., et al. Acta Ophthalmologica. The efficacy of optic nerve ultrasonography for differentiating papilloedema from pseudopapilloedema in eyes with swollen optic discs, September 2011, 1-5.

Ed. New