Papilledema (Optic Disc Swelling)

Differential Diagnosis

• Pseudotumor cerebri
  –Other symptoms: Headache, nausea, and vomiting all worse in morning, transient visual obscurations, diplopia
  –Diagnosis includes increased ICP, normal imaging, normal CSF
  –More common in obese females

• Optic neuritis
  –May be associated with postviral syndromes or meningoencephalitis
  –Loss of vision, pain on eye movement
  –Vision usually improves within a few weeks, but not full recovery

• Optic neuropathy
  –Compressive: Associated with NF1 and optic nerve glioma, presents with progressive visual loss, strabismus, nystagmus, proptosis
  –Infiltrative: From cancers (leukemias, lymphomas), infection, or inflammation (sarcoidosis, TB, toxocariasis, toxoplasmosis, CMV); optic disc swelling, vision loss, and hemorrhages
  –Toxic/nutritional optic neuropathy: Symmetric neuropathy from nutritional deficiency (thiamine, B12), drugs (tobacco/alcohol, chloramphenicol, rifampin), toxins (lead, methanol); visual field and vision loss; may recover with treatment
  –Leber optic neuropathy: Mitochondrial DNA transmission, presents late teens to middle 20s; visual field and vision loss, may spontaneously improve

• Increased ICP: Idiopathic intracranial hypertension, intracranial hemorrhage, space-occupying lesion
• Growth hormone supplementation
• Retinal hemorrhage and loss of vision
• Retinal vein occlusion
• Malignant hypertension: Associated with retinal hemorrhage, exudates, and cotton wool spots
• Optic neuropathy, nonarteritic or arteritic
• Demyelinating disease
• Infectious conditions: Toxoplasmosis, Lyme disease, Bartonella; hard exudates may be visible funduscopically

Workup and Diagnosis

• History
  –History of HA, nausea or vomiting, recent viral illness
  –Family history of visual loss, neurologic disorder
  –PMH or signs and symptoms consistent with known systemic diseases; e.g., hypertension, diabetes, thyroid disease, growth hormone therapy
  –Nutritional deficiencies; exposure to toxins such as tobacco or alcohol; recent drug use; exposure to ticks and animals

• Physical exam
  –Visual acuity, confrontational visual fields, pupillary response, extraocular muscle movements, proptosis
  –Dilated fundus evaluation
  –Neurologic exam for signs and symptoms of demyelinating disease, localizing deficit

• Labs
  –Titers for CMV, Lyme, toxocariasis, toxoplasmosis
• Radiology
  –CT or MRI of the brain and orbits for suspicion of intracranial mass, mass effect or hemorrhage
• Studies
  –Lumbar puncture may be indicated to establish presence or absence of, or to relieve, increased intracranial pressure
• Ophthalmologic consultation to rule out congenital variation to avoid unnecessary and expensive differential testing

Treatment

• Condition-dependent: Treatment of underlying systemic disease is often the only treatment
• Pseudotumor cerebri and other causes of intracranial hypertension: Weight loss, Diamox or Lasix, planned recumbency, LP shunt or optic nerve sheath fenestration if loss of visual function
• Space-occupying lesion or hemorrhage: Neurosurgical intervention
• Meningoencephalitis: IV antibiotics
• Infectious optic neuropathy: Treat underlying cause and consider systemic steroids (controversial)
• Optic neuritis: IV (not oral) steroids
• Optic nerve glioma treatment controversial: Observation if slowly progressive, resection if only one nerve involved, radiation if chiasm involved, shunts if increased ICP
• Toxic or nutritional: Stop offending toxin or supply nutritional supplementation