Papilledema refers to swelling of the optic disc caused by raised intracranial pressure. It is typically associated with symptoms of elevated intracranial pressure, such as headaches, pulsatile tinnitus, and transient visual obscurations. The visual hallmarks are relatively spared visual acuity in the setting of bilateral optic disc edema with enlarged blind spots, nasal visual field loss, or constriction of the visual fields. The causes of papilledema can be categorized into intracranial mass lesions or fistulas, venous sinus thrombosis, meningitic processes, subarachnoid hemorrhage, traumatic brain injury, and idiopathic intracranial hypertension. A multicenter, randomized clinical trial evaluated the efficacy of acetazolamide in addition to dietary changes for treatment of idiopathic intracranial hypertension. In addition, there have been advances in technologies, such as optical coherence tomography (OCT), nonmydriatic fundus photography, and automated image analysis in the detection and assessment of papilledema. The author addresses these issues in this updated review of papilledema.
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• Papilledema is optic disc swelling due to axoplasmic stasis caused by raised intracranial pressure.
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• Papilledema is typically bilateral, but it can be asymmetric, or even unilateral, due to anatomic differences in the meningeal covering of the intracranial optic nerves leading to differences in transmitted pressure.
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• Papilledema must be distinguished from other acquired causes of optic disc edema and congenitally dysplastic optic disc elevation. This distinction is aided by the features of the clinical examination and by ancillary studies, including ocular ultrasound, CT, optical coherence tomography, and fluorescein angiography.
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• The differential diagnosis for raised intracranial pressure includes intracranial mass lesions or fistulas, venous sinus thrombosis, meningitis, subarachnoid hemorrhage, traumatic brain injury, and idiopathic intracranial hypertension.
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• Formal visual field assessment is essential to the proper management of patients with papilledema.
Historical note and terminology
The term “papilledema” was first used in 1908 by J Herbert Parsons to describe disc edema due to elevated intracranial pressure (72). Disagreement regarding terminology quickly followed; for example, prominent physicians including Harvey Cushing preferred the term “choked disc,” coined by Albrecht von Graefe in 1861, stating “…though at first we were inclined to accept papilledema, this has seemed unwise on further consideration, for the term actually conveys nothing more of the process than does choked disc, and its adoption would only add confusion” (20).
Although the term papilledema has now been firmly adopted, Cushing was correct that confusion persists in application of the term. Clinicians should be careful to use the term papilledema to describe optic disc edema that is secondary to proven or suspected elevated intracranial pressure. It is best to avoid using the term papilledema to describe causes of optic disc edema in which the intracranial pressure is believed to be normal, as occurs with conditions such as anterior ischemic optic neuropathy or infiltrative diseases affecting the optic nerve head.