The Elevated Optic Disc

    • Protrusion of the optic disc above the surface of the retina
    • Common causes: congenitally elevated optic disc anomalies, papilledema, optic neuritis, ischemic optic neuropathy
    • Uncommon causes: Leber hereditary optic neuropathy, infiltrative optic neuropathy from meningeal cancer, pilocytic astrocytoma (“optic glioma”), hypertensive optic disc edema, central retinal vein occlusion, toxic optic neuropathy
    • Optic disc surface is not flush with the surface of the adjacent retina
    • Elevation may be congenital or acquired
    • Congenital optic disc elevation has these features
      • Physiologic cup is usually absent
      • Peripapillary retinal nerve fiber layer appears distinct
      • Optic disc vasculature is anomalous
      • Extruded mitochondria visible as white flakes (“drusen”) within the optic disc may be present
        • Sometimes easily visible on the optic disc surface
        • Often partially or completely buried within disc substance and better detected with autofluorescence fundus photography CT, ultrasound, or optical coherence tomography
        • Tip: most congenitally elevated optic discs do not contain drusen
    • Acquired optic disc elevation has these features
      • Physiologic cup is usually preserved with an indistinct peripapillary retinal nerve fiber layer
      • Optic disc surface hemorrhages or cotton wool spots are often present in severe and acute cases
    • Tilted optic disc of myopia, which displays elevation of the nasal portion of the optic disc
    • Myelinated nerve fibers
    • Distinguish congenital from acquired optic disc elevation
    • Trap: ophthalmoscopy may not be reliable in this task
    • Tip: optical coherence tomography, ultrasound, autofluorescence photography, and orbit CT are useful in disclosing buried drusen
    • Tip: ancillary imaging is not yet reliable in distinguishing papilledema from other causes of acquired optic disc elevation
    • If you have excluded congenital optic disc elevation, use these clues to determine the cause of acquired optic disc elevation
      • Symmetrical elevation in the two eyes and absence of major visual dysfunction suggests papilledema
      • Segmental elevation of the optic disc suggests non-arteritic infarction
      • Pallid elevation suggests arteritic infarction
    • Tip: in the absence of these signs, use non-ophthalmoscopic information to diagnose the cause of acquired optic disc elevation
    • Failure to distinguish congenital from acquired optic disc elevation leads to improper management
    • Failure to distinguish one cause of acquired optic disc elevation from another leads to improper management
    • Tip: papilledema and optic disc infarction from giant cell arteritis are the most urgent causes of acquired optic disc elevation; atypical optic neuritis (including infections, neuromyelitis optica spectrum disorder and myelin oligodendrocyte associated disorder) must also be diagnosed urgently because prompt treatment improves outcome

    Abnormal Optic Discs

    The Elevated Optic Disc The Excavated Optic Disc The Pale Optic Disc The Small Optic Disc