Papilledema

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Definition

  • Optic disc swelling due to increased intracranial pressure

Symptoms

  • Neurological symptoms
    • Headaches: new, usually severe; worse in the morning; exacerbated by coughing, physical exertion, lying down, or bending forward
    • +/- pulsatile tinnitus
    • +/- non-specific paresthesias or weaknesses
    • +/- other neurological deficits
  • Ophthalmological Symptoms
    • initially usually normal vision
    • Blurred vision / reduced visual acuity
    • Hypermetropisation
    • Transient visual obscurations
    • Photopsia
    • +/- diplopia
    • Secondary: reduced color and contrast sensitivity, visual field defects (initially typical in the mid-peripheral field)

Findings

  • Papilledema : graded according to Frisén Grading Scale (Grade 1-5)
    • Obscuration of vessels around the optic disc, absent optic cup, flame-shaped hemorrhages, spontaneous venous pulse not visible, dilated retinal veins, absent nerve fiber layer reflex
  • Usually bilateral, rarely unilateral
  • Initially normal afferent visual function (visual acuity, color/contrast vision, no RAPD)
  • Visual fields initially usually normal (apart from enlarged blind spots)
  • Secondary optic atrophy after regression of the swelling

Aetiology

  • Pseudotumor cerebri = idiopathic intracranial hypertension
  • Brain tumors, intracranial hemorrhage, brain abscess
  • Brain edema from trauma or metabolic origin
  • Small skull in craniosynostosis (very rare)
  • Hydrocephalus
    • Primary: congenital or early acquired (not in children or adults)
    • Secondary communicating or obstructive hydrocephalus (enlarged ventricles visible in imaging)
      • Subarachnoid hemorrhage (SAH)
      • Meningitis
      • Increased cerebrospinal fluid production by choroid plexus tumors (rare)
      • Sinus vein thrombosis (often of the superior sagittal sinus and/or transverse sinus)
      • Extracranial venous outflow obstruction e.g., obstruction of internal jugular vein or superior vena cava

Differential Diagnoses

  • “Pseudo-Papilledema”
    • No vascular obscuration or dilation, normal nerve fiber reflex, no haemorrhages, preserved optic cup, usually present spontaneous venous pulse
    • E.g., optic disc drusen, ‘crowded disc’/congenital optic disc anomaly, ’tilted disc’
  • Optic disc swelling of other etiology, including diabetic papillopathy, anterior ischemic optic neuropathy (AION), papillitis, etc.

Work-up

To distinguish true papilledema from pseudo-papilledema

  • Fundoscopy
  • Ultrasound: Hyperreflectivity of drusen
  • Autofluorescence: Hyperautofluorescence of drusen
  • Fluorescein angiography: No leakage in pseudo-papilledema, only staining
  • OCT (EDI images): Possibly visible drusen
  • CT: Intracranial space-occupying lesion? Increased intracranial pressure? Hyperreflectivity of drusen?
  • MRI: Intracranial space-occupying lesion? Enlarged optic nerve sheaths? Posterior flattening of the globe?
  • Lumbar puncture, if above investigations are inconclusive: Increased opening pressure? (normal opening pressure is <25cm CSF in adults or <28cm CSF in children), cerebrospinal fluid analysis

Sources

  • EyeWiki Papilledema
  • The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease; Kalla Gervasio MD, Travis Peck MD et al; Lippincott Williams&Wilkins; 8th Edition (2021)
  • Kanski’s Clinical Ophthalmology: A Systematic Approach; John E Salmon MD; Elsevier; 9th Edition (2019)