Papilledema HANDOUT



Papilledema HANDOUT
Evaluation and Management
• Swelling of the optic nerve head- "disc" –
due to increased intracranial pressure (ICP)
• Cerebrospinal fluid exerts transluminal
Papilledema (intracranial hypertension)
almost always causes bilateral disc edema
but not all bilateral disc edema is papilledema
pressure at lamina cribosa
Aki Kawasaki
Hôpital Ophtalmique Jules Gonin
Lausanne, Switzerland
Bilateral Optic Disc Edema
• Bad central vision in
one or both eyes
Optic neuritis
●  Ischemic optic
●  Infiltrative neuropathy
●  Inflammatory neuropathy
●  Toxic optic neuropathy
•  Good central vision
• Papilledema
(increased ICP)
●  Diabetic papillopathy
●  Malignant hypertension
Optic perineuritis
Patient with bilateral disc edema
Visual function is relatively preserved
Evaluate for suspected papilledema
1. Measure blood pressure
2. Rule out uveitis
3. Labs: glucose, Hgb A1C , hematogram,
BUN/creatinine, calcium
4. Rule out drusen – if not visible, ancillary tests needed
5. Neuroimaging
6. Lumbar puncture
Don’t Skip the Lumbar Puncture…
• Lateral decubitus position
• Opening CSF pressure = ICP
• measure with legs un-flexed, no Valsalva , no talking
• CSF pressure varies—be cautious in interpreting any
single measure!
• A!dults: > 25 cm is too high
• Children: >28cm is too high
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Revised Criteria for Pseudotumor Cerebri
Friedman et al
Neurology 2013
• Bilateral disc edema
• Normal BP, Normal neurologic exam
except sixth nerve palsy
• Normal neuroimaging (except MRI signs
associated with increased ICP)
• Normal CSF composition
• Elevated CSF pressure
PTC : A-E fulfilled
Probable: A-D but CSF pressure is lower than E
Secondary PTC
• cerebral venous thrombosis
• sleep apnea
• medications
tetracycline, vitamin A
analogues, steroid
withdrawal, growthhormone
• endocrine disorders
Addison disease,
• systemic illness
! anemia,
lupus, uremia
Idiopathic PTC
(also called
Diagnosis of PTC Confirmed:
Identify causes and associated conditions
Pseudotumor Cerebri without Papilledema
• If no disc edema, substitute sixth nerve palsy
and fulfill all other criteria (B-E)
• Apnea
• Anemia
• Renal failure
• Polycystic ovarian
• If no disc edema and no sixth nerve palsy, can only
suspect dx of PTC if patient has all other criteria
PLUS MRI signs of increased ICP
Wall et al.
JAMA Neurol, April 2014
• 161 women (98%),
4 men
• Aged 18-52years—mean 29yr
• 65% white, 25% black
• All overweight--88% obese
spontaneous thrombosis
jugular compression/ligation
mastoid/middle ear infection
hypercoagulable states
• Dural AV malformation
• Medications
tetracycline, doxycycline, vitamin A and retinoids,
IIH: Clinical Profile at Baseline
• Addison disease
• Hypoparathyroidism
• Cerebral venous abnomalities
human growth hormone, anabolic steroids,
lithium, chemotherapeutic agents
Sociologic Emergency of Obesity and
Idiopathic Intracranial Hypertension
• Obesity: BMI greater than 30 (based on height/weight)
normal BMI is less than 25
• Incidence of obesity in United States has doubled
between 1980 and 2000
• Obesity is associated with greater risk for IIH
burden of IIH is 450 millionUSD/year
• Economic
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Management of Visual Loss due to
Papilledema and Increased ICP
Despite good acuity and central vision,
many patients early, subtle visual field loss
Only way to stop progressive visual loss is
to decompress the disc edema
Idiopathic Intracranial Hypertension
Treatment Trial (IIHTT)
• First randomized prospective trial for treatment of
•  Not a management emergency
visual loss due to IIH
• Multicenter, double-blind,placebo-controlled
• 38 centers in USA and Canada
• 165 patients with mild visual loss (mean deviation
between -2 to -7db on SITA)
• A! ll patients required to follow low sodium diet
Patient with Papilledema:
Mild-Moderate Visual Field Loss
•  Medical treatment is recommended
Acetazolamide (start 500mg twice daily- 4gmax)
•  Low salt diet and weight loss, if obese
•  Monitor fundus and visual fields monthly:
most normalize in 3 to 6 months
with weight loss counselor
When Should Surgical Treatment of
Papilledema Be Considered ?
Surgical Treatment of Papilledema
Optic Nerve Sheath Fenestration
Digre and Corbett,
Neurologist 2001
1. Progressive visual loss while on medical therapy
• Optic nerve sheath fenestration
•  Fenestrations or window in nerve sheath
lets CSF drain into orbit
2. Moderate-to-severe visual loss at presentation
• Cerebrospinal fluid (CSF) diversion
lumboperitoneal shunt
ventriculoperitoneal shunt
•  Advantage:
local anesthesia
any etiology of increased ICP
e.g. pregnant women with eclampsia
3. Very severe swelling of optic disc
4. Unstable blood pressure , e.g. dialysis patients
?5. Progressive loss of ganglion cell layer on OCT?
• Bariatric surgery – not used as
emergency procedure
•  Intraoperative risk of visual loss – 1%
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Lumboperitoneal Shunt
Efficacy of Surgical Treatment
Ventricular Shunts
Fonseca et al.
BJO, May 12, 2014
• Retrospective study : ONSF (14 patients) or CSF
•  Advantage:
treats both optic nerves and headache
•  Drawback:
high complication rate later…
nearly 50% develop shunt
obstruction in 2 years
shunt infection
•  Stereotactic approach
•  Advantages over LP shunt:
greater control of ICP regulation
usable in patients with tonsillar herniation
lower risk of later complications
McGirt et al, J Neurosurg 2004
Surgical Intervention for Papilledema:
Visual Outcome
Surgical Intervenstion for Papilledema:
Post-operative Followup
shunt (19 patients) for papilloedema and severe
vision loss.
• Preoperative papilloedema was qualitatively
worse in the ONSF group.
• Acuity and field improved after both procedure
• Postoperative visual acuity did not differ.
• ! Improvement of MD in both groups was same
magnitude (6 dB)
Acute, Rapidly Progressive Visual Loss:
"Malignent Pseudotumor Cerebri" is an
Ophthalmic Emergency
• Following either ONSF or CSF shunt,
• Monitor visual field and disc appearance
•  No randomized controlled studies comparing
visual outcome of ONSF and CSF shunt
•  Immediate MRI
•  Check the blood pressure
•  Correct any metabolic derangements
e.g. anemia
every day for few days, then every week
•  Choice is individually-based
until vision stable , papilledema decreases
•  In the short term, both ONSF and CSF shunt are
highly efficacious in reducing papilledema:
>90% of patients have stable or improved
vision (acuity and/or fields)
• If vision does not stabilize with one
procedure, then do the other procedure
•  Intravenous steroids
•  Intravenous acetazolamide
•  Lumbar puncture - large volume, repeated
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Acute, Rapidly Progressive Visual Loss:
Acute, Rapidly Progressive Visual Loss:
• Fulminant IIH – acute HA, rapid severe visual loss
• 16 patients, aged 14-39 years
• Median time to surgical intervention –3 days
• Either ONSF or CSF shunt
• Monitor visual field closely: daily for 1 week
ONSF 5 patients ,LPS 9 patients, VPS 2 patients
• If vision does not stabilize with one procedure
within 1-2 days, then do the other procedure!
• Vision improved in 14 patients
8 (50%) still legally blind
What is Drusen?
• Congenital anomaly that causes elevation of the
optic nerve head
• Progressive degenerative process
Abnormal axoplasmic metabolism
and rupture of axons
Accumulation of extruded material
disintegrating nerve fibers
Thambisetty et al,
Neurology 2007
• In children, drusen are often
thus not visible.
• Fundus exam can still provide some clues to
distinguish buried drusen (vs papilledema)
Paton’s lines and choroidal folds
type of hemorrhage
peripapillary nerve fiber layer
disc vasculature
spontaneous venous pulsations
Spontaneous Venous Pulsations
buried and
• In healthy young patient without neurologic
deficits, presence of spontaneous venous
pulsations means ICP is not high and thus
suggests congenital disc anomaly, e.g. drusen
• Absence of venous pulsations is not helpful
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