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Optic Nerve Glioma

 

 

Signs & Symptoms

 

An optic nerve glioma was first described in 1833, by JH Wishart, FRSE (Surgeon in Ordinary to the King in Scotland).  He published the case of Euretta Douglas, a 13-year-old girl who presented with proptosis.

 

Common Presentations for Optic Pathway Gliomas:

Sign/Symptom

Significance/Additional Notes

Impaired visual acuity

  • Most common presenting complaint.

 

  • Usually becomes apparent after substantial disease progression - often during a routine school eye exam.

 

Painless Proptosis

  • Proptosis is often painless and irreducible. 

 

  • Tumor pushes the globe forward.

 

  • Often the first sign of an intra-orbital mass.

 

Visual field defects

  • Suggests the disease has an infiltrative nature.

 

  • Defects are variable, may be central, or eccentric field cuts.

 

Nystagmus

  • Rapid and short oscillations when scanning or searching.

 

  • Suggests chiasmatic involvement.

 

Strabismus

  • Deviation in alignment of one eye relative to the other.

 

  • Results in the inability to focus on objects.

 

Hypothalamic dysfunction

 

  • Precocious puberty relatively common.

 

Raised intracranial pressure
  • Often due invasion of the third ventricle. 

 

  • May result in hydrocephalus and papilledema. 

 

  • Only becomes apparent after substantial tumor growth.

 

Diencephalic Syndrome
  • Emaciation, failure to thrive, amnesia, abnormal eye positions.

 

  • Observed in infants and young children.

 

Papilitis
  • Blurring of the optic disc and loss of central vision. 

 

  • Suggests an intraorbital tumor.

 

 

Location of Optic Pathway Glioma and associated symptoms:

Tumor location

Associated symptoms

Intraorbital

  • Painless proptosis
  • Papillitis

Optic nerve

  • Unilateral visual loss

Optic chiasm

  • Nystagmus
  • Bilateral field cuts
  • Papilledema

Optic tract

  • Bilateral field cuts

Hypothalamus

  • Precocious puberty
  • Diencephalic Syndrome

Ventricular system/ Foramen of Munro

  • Hydrocephalus and papilledema

 

 

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