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  • Updated 07.25.2022
  • Released 07.18.2005
  • Expires For CME 07.25.2025

Papilledema

Introduction

Overview

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 include relatively spared visual acuity in the setting of bilateral optic disc edema, though visual field deficits might include 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, meningeal processes, subarachnoid hemorrhage, traumatic brain injury, and idiopathic intracranial hypertension. The fundus exam and ancillary analyses, such as OCT, nonmydriatic fundus photography, and automated image analysis, have been used to diagnose papilledema. Treatment options for papilledema are determined by the underlying cause. The randomized, placebo-controlled IIH Treatment Trial, which was published in 2014, provided the first rigorous evidence for the benefit of acetazolamide in the management of papilledema secondary to idiopathic intracranial hypertension.

Key points

• Papilledema is optic disc swelling due to axoplasmic stasis caused by raised intracranial pressure.

• Papilledema is typically bilateral, but it can be asymmetric or, rarely, unilateral due to anatomic differences in the meningeal covering of the intracranial optic nerves leading to differences in transmitted pressure.

• Papilledema must be distinguished from other acquired causes of optic disc edema and from congenital optic disc elevation (“pseudopapilledema”) owing to small, crowded or tilted optic discs with or without optic disc drusen. This distinction is aided by the features of the clinical examination and by ancillary studies, including OCT with enhanced depth imaging of the optic nerve head, ocular ultrasound, fluorescein angiography, and CT or MRI.

• The differential diagnosis for raised intracranial pressure includes intracranial mass lesions or fistulas, venous sinus thrombosis, meningeal diseases, subarachnoid hemorrhage, traumatic brain injury, and idiopathic intracranial hypertension.

• 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 (61). Disagreement regarding terminology quickly followed. 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” (16).

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 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.

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