SYMPTOMS Ocular symptoms: Transient vision loss, Diplopia
Systemic symptoms: Headaches, Vomiting, Pulsatile tinnitus
If acute, vision is typically be normal unless there is macular involvement
If chronic, central and peripheral vision will be reduced
SIGNS Findings of papilledema are bilateral but in the early stages of papilledema, the findings tend to be asymmetric
Optic nerve edema, Optic nerve hyperemia, Loss of the cup, Blurring of the optic disc margins, Obscurations of the small vasculature at or around the optic nerve, Thickened and edematous retinal nerve fiber layer, Opacification of the retinal nerve fiber layer, Loss of sharp light reflexes around the optic disc, Splinter retinal hemorrhages, Cotton wool spots, Star-shaped macular exudates, Dilated tortuous retinal veins, Absence of the spontaneous venous pulse, Paton lines
In chronic papilledema, all signs associated with acute papilledema can be seen including Optic atrophy, Glistening white deposits on the disc, and Optocilliary shunt vessels
WORK-UP Cranial nerve testing, Pupils (typically normal but can present with an APD if papilledema is asymmetric), EOMs, Color vision (typically normal), Visual field (enlarged blind spot may be present), Blood pressure evaluation (in order to rule out malignant hypertension), Slit lamp examination, Dilated retinal exam, Fundus photos, OCT, Fundus autofluorescence (typically normal or slight decrease in AF that extends beyond the optic disc margins), Fluorescein angiography (leakage from dilated capillaries starting in the venous stage and extending into the late stage), B-scan ultrasound (will show an enlarged optic nerve sheath diameter)
OCT (Optic nerve analysis with EDI and RNFL analysis): *Elevation of the neuroretinal rim thickness *Smooth optic nerve contour *Elevation of the optic disc with involvement of the retinal nerve fiber layer with a smooth, hill-like appearance *Anterior displacement of the Bruch's/RPE complex especially near the Bruch's membrane opening *Presence of peripapillary hyper-reflective ovoid-mass like structures (PHOMS) which represent bulging optic nerve axons *Presence of subretinal fluid (presence of “lazy V sign” especially at the Bruch’s membrane opening) *Thickening of the of the RNFL with typically the nasal side being > 86 microns, temporal side being > 97 microns, superior side being > 149 microns, and inferior side being > 165 microns (Nasal RNFL thickening has the greatest specificity and sensitivity) *Subretinal hyporeflective space between the optic disc and Bruch's membrane opening (SHYPS) is >464 microns
B-scan ultrasound (measurement needs to be done 3mm behind the globe): *If < 1 years old, optic nerve sheath diameter should be < 4mm *If 1-15 years old, optic nerve sheath diameter should be < 4.5mm *If >15 years old, optic nerve sheath diameter should be < 5mm (>5.7mm is highly indicative of optic nerve edema and elevated intracranial pressure)
TREATMENT Refer to a neuro-ophthalmologist/ER STAT for additional testing and treatment
Treatment is directed at the underlying cause of the papilledema
FOLLOW-UP After resolution of papilledema, patient should be followed-up every 4-6 months
ADDITIONAL LAB | TESTS Testing will typically be ordered through the neuro-ophthalmologist/ER: MRI of the brain and orbits with and without contrast, MRV of the brain with or without contrast, Lumbar puncture especially if MRI and MRV are normal
ETIOLOGY Optic nerve edema secondary to an increase in intracranial pressure can occur secondary to space occupying lesions in the brain, obstruction of the cerebrospinal fluid, increased production of the cerebrospinal fluid, decreased absorption of the cerebrospinal fluid, medications such as tetracycline, and idiopathic intracranial hypertension
DIFFERENTIAL DX Pseudopapilledema (such as optic disc drusen), Diabetic papillopathy, Hypertensive optic neuropathy, Optic neuritis, Central retinal vein occlusion, Ischemic optic neuropathy, Infiltrative optic neuropathy
NOTES 20% of the population normally does not have a spontaneous venous pulse (SVP) so the absence of one is not pathognomonic. If a SVP is documented and later absent, that is more cause for concern
Increase in intracranial pressure can still be present in optic nerves that have pallor (dead fibers/axons don’t have the ability to swell)
If an increase in intracranial pressure is gradual and mild, it may take weeks for signs to appear. If an increase in intracranial pressure is rapid and severe, it may take hours to days for signs to appear
CN 6 palsies are commonly associated with papilledema
OCT will go from showing swelling of the RNFL to thinning of the RNFL as optic atrophy develops
The Frisen scale can be used to grade the severity of the papilledema
For idiopathic intracranial hypertension, patients will typically be a young female with a history of recent weight gain and history of thyroid disease, polycystic ovarian syndrome, and/or sleep apnea. These patients will typically have a normal MRI/MRV of the brain. Treatment is typically directed at losing weight and controlling underlying systemic diseases. Taking oral Acetazolamide has also been shown to help.