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Meningioma of the optic nerve sheath

 
, medical expert
Last reviewed: 07.07.2025
 
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Meningiomas develop from the meningoendothelial cells of the arachnoid. Primary orbital meningiomas, which originate from the optic nerve sheath, account for 2% of cases and are less common than optic nerve gliomas. They most often affect women.

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Symptoms of Optic Nerve Sheath Meningioma

The disease manifests itself in middle age with a gradual unilateral decrease in vision. Temporary visual impairment may be the first symptom.

The classic triad is: decreased vision, optic nerve atrophy, and opticociliary vascular shunts. However, the simultaneous appearance of all three signs is rare. The sequence of signs is as follows:

  • Dysfunction of the optic nerve and chronic stagnation of the disc, followed by atrophy.
  • Opticociliary vascular shunts, which are found in approximately 30% of cases, regress with the development of optic nerve atrophy.
  • Limited mobility, especially upward, since the tumor can “split”) the optic nerve.
  • Exophthalmos appears due to tumor growth within the muscular funnel and develops after a decrease in vision.

The sequence is the opposite of that seen in tumors growing outside the dura mater, where exophthalmos appears long before compression of the optic nerve.

Diagnostics

CT shows tubular thickening and calcifications of the optic nerve.

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Treatment of optic nerve sheath meningioma

  1. Monitoring of middle-aged patients with slow tumor growth, as the prognosis is good.
  2. Surgical removal in young patients with aggressive tumors, especially in a blind eye.
  3. Radiation in some cases.

Forecast

The prognosis in adults is good, although the tumor can be aggressive and sometimes fatal in children.

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