Title: Disc Edema
Author: Cole Swiston, 4th year medical student, University of Wisconsin School of Medicine and Public Health; Trey Winter, 1st Year Medical Student, University of Utah
Photographers: Danielle Princiotta, Becky Weeks
Figure 1. Bilateral optic disc edema (papilledema) in a patient with idiopathic intracranial hypertension (IIH). A) Grade 4 papilledema in the patient’s right eye, marked by circumferential disc elevation, and obscuration of vessels both on the disc and leaving the disc. Disc hemorrhages are also present. B) Grade 4 papilledema in the left eye with disc hemorrhages.Text:
Disc edema is an ophthalmoscopic finding defined by unilateral or bilateral swelling of the optic disc. There are several synonyms used to describe this finding including papillitis, papilledema, swollen or choked discs, and the most commonly used term – optic disc edema (ODE). Optic disc edema presents with a buldging of the optic disc seen on fundus exam and may lead to loss of vision (Figure 1).
Presentation: Patients with ODE are often asymptomatic but can present with a range of symptoms based on the underlying cause. For example, a patient who has disc edema due to increased intracranial pressure (ICP) may present with positional headache, visual obscurations, or pulsatile tinnitus. If the cause in infectious, systemic symptoms may be present. As for visual symptoms, patients can present with decreased visual acuity, field defects, and a relative afferent pupillary defect when the disc swelling is asymmetric or unilateral.
Differential Diagnosis: It is useful to separate differential diagnoses associated with ODE into unilateral disc edema or bilateral disc edema.
Table 1: Causes of unilateral optic disc edema
|Anterior Ischemic optic neuropathy (AION)||Arteritic AION: scalp tenderness, jaw claudication, fever, joint pains – associated with Giant cell arteritis.
Non-arteritic AION: history of vascular risk factors (Diabetes, Hypertension, Hyperlipidemia)
|Arteritis AION: Unilateral optic disc edema with a white, chalky appearance.
Non-arteritic AION: Unilateral optic disc edema, cup-less “disc at risk” in the fellow eye
|Neuroretinitis||Viral URI symptoms, fever, lymphadenopathy, rash||Optic disc edema with a macular star|
|Papillitis/Optic Neuritis||Orbital pain with visual changes (blurring, field defect, or decreased color vision) and an afferent pupillary defect||Usually only optic disc edema|
|Papillophlebitis||Young, healthy patient with blurred vision, with or without a field defect||Optic disc edema, venous dilation, and disc hemorrhages|
|Retinal Vein Occlusion||History of vascular risk factors (Diabetes, Hypertension, Hyperlipidemia), sudden painless vision loss, afferent pupillary defect||Optic disc edema with dilated and tortuous vessels, extensive retinal hemorrhages, cotton wool spots|
Table 2: Causes of bilateral optic disc edema.
|Increased intracranial pressure (Papilledema)||Headache, pulsatile tinnitus, visual obscurations, nausea and vomiting||Bilateral optic disc edema|
|Diabetic Papillopathy||History of diabetes, visual changes (blurring or field defects)||Bilateral optic disc edema, microaneurysms, cotton wool spots, hard exudates, retinal hemorrhages, neovascularization|
|Hypertensive Papillopathy||History of hypertension, visual changes (blurring or field defects), headache||Bilateral optic disc edema, cotton wool spots, hard exudates, retinal hemorrhages, arteriosclerosis|
|Pseudopapilledema (Optic Disc Drusen)||Generally asymptomatic||Round, white/yellow bodies on and buried in the optic nerve head|
Diagnosis: A fundoscopic exam is crucial for diagnosing disc edema. In general, a fundoscopic or slit lamp exam will reveal enlarged retinal venules, blurred disc margins, elevation of the optic disc, obscuration of blood vessels traveling through the optic disc, and in severe cases, hemorrhage overlying the optic disc. Other fundus findings are specific to the underlying etiology. Optic disc edema is graded based on severity using the modified Frisén scale, shown in Figure 2. These findings together with patient symptoms and history are key to arriving at the proper diagnosis. These differentiating factors are described in Tables 1 and 2, above.
Unexplained optic disc edema necessitates further imaging studies. An MRI and MRV of the brain will evaluate for the presence of increased intracranial pressure including structural lesions and venous outflow obstruction, which can both lead to papilledema. A lumbar puncture may also be needed to evaluate for infection and elevated ICP. The presence of elevated ICP in the absence of a cause found on imaging may lead to the diagnosis of idiopathic intracranial hypertension, also known as pseudotumor cerebri.
Management/Treatment: The management of disc edema is largely dependent on the causative agent. For example, antihypertensive medications can be administered for disc edema due to hypertension. Disc edema due to inflammatory causes can be treated with corticosteroids or immunosuppressive drugs. Infectious causes should be treated with an appropriate antibiotics. Treating the underlying condition will usually reduce disc edema and prevent progression to optic atrophy and permanent optic nerve damage.
Complications: The main complication with disc edema is permanent blindness secondary to optic atrophy. Disc edema typically only progresses to optic atrophy and irreversible blindness if left untreated for an extended period of time, leading to death of axons in the optic nerve. The speed of this progression is also dependent on the degree of disc swelling.
Faculty Approval: Griffin Jardine, MD
- Chan JW. Optic Nerve Disorders: Diagnosis and Management. 1. Aufl. ed. New York: Springer; 2007;2008
- Frisén L. Swelling of the optic nerve head: a staging scheme. Journal of Neurology Neurosurgery Psychiatry 1982; 45:13.
- Van Stavern GP. Optic Disc Edema. Seminars in Neurology. 2007;27:233-243.
- Khurana KA, Aruja K, Khurana B. Comprehensive ophthalmology 6th edition. New Delhi: Jaypee, The Health Sciences Publisher.
- Lee AG, Beaver HA. Acute Bilateral Optic Disk Edema with a Macular Star Figure in a 12-Year-Old Girl. Survey of Ophthalmology. 2002;47:42-49.
- Newton PN, Thai LH, Tip NQ, et al. A Randomized, Double-Blind, Placebo-Controlled Trial of Acetazolamide for the Treatment of Elevated Intracranial Pressure in Cryptococcal Meningitis. Clinical Infectious Diseases. 2002;35:769-772.
- Sadun AA, Currie JN, Lessell S. Transient visual obscurations with elevated optic discs. Ann Neurol 1984; 16:489.
- Tso MO, Hayreh SS. Optic disc edema in raised intracranial pressure. IV. Axoplasmic transport in experimental papilledema. Arch Ophthalmology 1977; 95:1458.
- Wall M. Idiopathic Intracranial Hypertension. Neurologic Clinics. 2010;28:593-617.