Causes of disc edema - Unilateral Vascular Infectious Diabetic Papillopathy
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6/14/2021
Differential Diagnosis
of ONH Edema
Beth A. Steele, OD, FAAO
bsteele@uab.edu
2
Causes of disc edema …
Unilateral
• Vascular
• Infectious
• Diabetic Papillopathy Bilateral
• Hypertensive
• Toxic
• Inflammatory
• Infectious
• Compressive
• Hydrocephalus
• IICHtn
6/14/2021
Is it…? Myopic / tilted discs?
Be careful not to hide behind a comfortable label…
And if so then why is it?
Pseudopapilledema
• ONH drusen
• Anatomically crowded discs
• Hyperopic disc
• Myopic / tilted / obliquely inserted disc
31 AAF
Terminology “nasal margins mildly indistinct;
RTC x 1 week for further
evaluation”
• Disc Edema
• Papilledema
• Bilateral papilledema
• Pseudopapilledema
• Pseudotumor
• Benign Idiopathic Intracranial
Hypertension
6/14/2021
Early on – can appear as a nasal “C‐shaped”
edema 6 years later….
• Temporal area
spared
• VA often
normal
Not a perfect system…..
3 years earlier was consistent with anatomical
elevation; then lost to f/u x 3 years
Bilateral Buried Disc Drusen –
…3 years earlier the
patient was asked to A common diagnostic dilemma
RTC x 1 week
6/14/2021
43 AA Female
• 20/15
• 140/90
• DFE: drance heme Normal RNFL
OD and disc elevated
nasally
Recommendations
from the ODD
Consortium
• Radial ONH scans with EDI
• RNFL scans (no EDI)
• FAF
• Located above lamina
• Signal poor core, with
superficial
hyperreflectivity
• Often conglomerates
• Can mimic vessels
Malmqvist et al. Optic Disc Consortiom
Recommendations for Diagnosis of ODD using
OCT. Neuro‐Opthalmology 2017.
6/14/2021
OCT: Globe Convexity
• Increased ICP will push the globe
anteriorly
• Easiest to appreciate with a 9mm scan
• With EDI, can see an anteriorly
displaced Bruch’s membrane
• What about with high myope? Look at
most posterior aspect of sclera…
24 WM
6/14/2021
Fundus
Autofluorescence
• Drusen ‐ Hyper AF
• Buried – harder to
visualize
Optos.com
6/14/2021
Normal ONH Sheath Diameter can ease your
mind as well…
• Axial images of the
optic nerve (V and H)
• 3 mm behind the
posterior eye wall
Bruch’s Membrane
Opening?
• Predictive: 1785 microns
• 78.8% sensitivity
• 81% specificity What if Drusen Ruled Out?
Other possibilities?
6/14/2021
Globe
34 WF convexity is
normal
• 20/20, pupils normal, CF
normal
• ‐7.00 OU
• BMI 31
• Denies H/A
• Elevated ONH
Vs…
• .1/.1 CD
• +SVP
• Normal RNFL thickness
32 AA FM
This patient… Severe headaches, BMI 42
• Exam
• Elevated disc
• +SVP
• B‐scan
• (‐) hyperreflectivity consistent with
drusen
• (‐) crescent sign
• Normal ONH sheath diameter
• OCT
• Normal RNFL thickness
• Normal globe convexity
• Stability….
• Presumed crowded discs but
careful follow up
6/14/2021
Papilledema Suspected ? Now what…
• Brain Imaging
• MRI – rule out space occupying mass
• MRV – rule out cerebral venous thrombosis
• Lumbar Puncture
• With opening pressure
• Higher than 25/30 cm H2O is abnormal
• Referral?
• Determine underlying cause/association if any
• Weight
• Associated medications
Large meningioma
at base of skull
MRI features
• Order with/without contrast, T1/T2‐weighted, with fat suppression
• Empty sella
• Enlarged ON sheath
Lumbar Puncture
• Increased tortuosity of ON
opening pressure =
28cm H20
• Flattened sclera • 41 year old male
• Anterior protrusion of ONH • Severe headaches
• Attenuation of cerebrovenous sinuses • BMI 38
6/14/2021
26 WF, “blurry vision”
Hx hydracephalous, multiple previous surgeries Our Patient:
VA 20/40 OD, OS MRI Clear
H/A developing with more intensity Opening Pressures of 28
BMI 42
MRI “all clear”
…. ICP was borderline
Idiopathic Intracranial HTN IIHT – Who Gets It?
• BMI >30
• 90‐98% complain of headache • >40 – worse visual outcome
• Rapid weight gain – more severe
• Nausea/vomiting/dizziness – 40%
• Mostly females
• Pulsatile tinnitus – 16‐60% • Males 10% of the time
• Visual disturbances – 30% • Not as likely to have H/A – may not come in
• No other neurologic findings (some with VIth palsy) • Race – more aggressive in AA
• 3 x more likely to have vision loss
• 5 x increase in blindness
• Co‐morbidities
• HTN
• Sleep apnea
Mollan SP, et al.
Pract Neurol 2018
Biousse V et al. Am J Oph 2007, 2012.6/14/2021
Toxic Oral contraceptives IIH Treatment Trial – JAMA, April 2014
Etiologies Tetracyclines
Steroids
Vitamin A • acetazolamide + a low‐sodium weight‐reduction
• Idiopathic diet vs. diet alone
But what causes the papilledema ? Retinoids
Tamoxifen → modest improvement in visual field func on
Nalidixic Acide
Axoplasmic flow stasis → swollen
nerve fibers → compression of venules Other Obesity • OCT Substudy of the IITT, Ophthalmology, Sept
in the area and so venous 2015
stasis/leakage → accumula on of • RNFL and Total Retinal Thickness (TRT) useful in
extracellular fluid following and monitoring response to treatment
• Better RNFL thickness, TRT, and ONH volume swelling
measurements
Acetazolamide + Low Sodium/Wt Loss Diet Treatment Goals …
• Contraindications:
• Sulfa allergy?
• chemical structure different than antibiotics – little
evidence of cross‐sensitivity
• consider avoiding if hx of severe reaction
• Long‐term: liver, kidney disease, severe COPD
• Caution with sickle cell
• Caution with low potassium
• Visual Fields – for life
Common Side Effects
The Idiopathic Intracranial Hypertension Treatment Trial.
paresthesia metabolic acidosis • OCT
metallic taste electrolyte imbalance
• Labs ?
JAMA 2014
125,250mg tablets, and fatigue (including hypokalemia)
500mg SR capsules malaise
GI disturbances
renal calculi
blood dyscrasias
• Co‐management ?
(Diamox Sequels, Duramed
Pharmaceuticals) decreased libido Mollan SP, et al. Pract Neurol 2018
Cello KE J Neuroophth 2016
Than T, Smith H. http://www.reviewofoptometry.com/ce/10‐must‐have‐oral‐meds.6/14/2021
Other treatment options….
• Surgical – controversial and provider‐dependent
• Ventricular‐peritoneal shunt
• Optic nerve sheath fenestration
• Repeated lumbar puncture ? Following Tx Diamox
• Not well reported 250mg BID… x 2 years
• Procedure ‐ causes anxiety, local discomfort, complications, headache
• LP‐induced reduction of ICP is only short‐lived
• On the horizon
• Topamax – off label
• Bariatric surgery – in trials
34 year old Caucasian FM
• (+) H/A
• BMI 34
• Meds – Mirena IUD6/14/2021
• MRI – empty sella,
flattened sclera, distended
ONH sheaths
• hgh
Those tools can miss subtle elevation…..
21 AAF
3 years earlier was consistent with anatomical
• High BMI
• MRI clear of mass
elevation; then lost to f/u x 3 years
• Initial opening pressure of 52
…3 years earlier the
patient was asked to
RTC x 1 week6/14/2021
After 1 month of
250mg Diamox BID
still (+) H/A
Watching VF carefully…
• H/A’s improved
• RNFL thickness
reduced
2 mos, then 5 mos
(now TID Diamox) • Total volume
Denies H/A reduced6/14/2021 And now… • Extracellular edema leads to venous stasis…
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