Papilledema: A review of etiology, pathophysiology, diagnosis, and management

Papilledema is optic nerve head edema secondary to raised intracranial pressure (ICP). It is distinct from other causes of optic disk edema in that visual function is usually normal in the acute phase. Papilledema is caused by transmission of elevated ICP to the subarachnoid space surrounding the optic nerve that hinders axoplasmic transport within ganglion cell axons. There is ongoing controversy as to whether axoplasmic flow stasis is produced by physical compression of axons or microvascular ischemia. The most common cause of papilledema, especially in patients under the age of 50, is idiopathic intracranial hypertension (IIH); however, conditions that decrease cerebrospinal fluid (CSF) outflow by either causing CSF derangements or mechanically blocking CSF outflow channels, and rarely conditions that increase CSF production, can be the culprit. When papilledema is suspected clinically, blood pressure should be measured, and pseudopapilledema should be ruled out. Magnetic resonance imaging of the brain and orbits with venography sequences is the preferred neuroimaging modality that should be performed next to look for indirect imaging signs of increased ICP and to rule out nonidiopathic causes. Lumbar puncture with measurement of opening pressure and evaluation of CSF composition should then be performed. In patients not in a typical demographic group for IIH, further investigations should be conducted to assess for underlying causes of increased ICP. Magnetic resonance imaging of the neck and spine, magnetic resonance angiography of the brain, computed tomography of the chest, complete blood count, and creatinine testing should be able to identify most secondary causes of intracranial hypertension. Treatment for patients with papilledema should be targeted toward the underlying etiology. Most patients with IIH respond to weight loss and oral acetazolamide. For patients with decreased central acuity and constricted visual fields at presentation, as well as patients who do not respond to treatment with acetazolamide, surgical treatments should be considered, with ventriculoperitoneal shunting being the typical procedure of choice.