Neurofibromatosis I, enlarged optic foramen
Neurofibromatosis I, enlarged optic foramen

Optic glioma

Definition:
An optic glioma is a tumor created by growth of abnormal cells in one or both optic nerves or the optic chiasm of the brain, or in conjunction with a hypothalamic glioma (a mass in the hypothalamus of the brain).

Alternative Names:

Glioma - optic; Optic nerve glioma



Causes, incidence, and risk factors:

Gliomas refer to a class of tumors that can be found in various parts of the brain. Optic gliomas are gliomas that affect part of the optic nerves (the nerves that carry visual information to the brain from the eyes) or the optic chiasm. The cause of optic glioma is unknown.

These tumors most often occur in children and almost always occur before age 20. Optic gliomas are rare. There is a strong association between optic glioma and neurofibromatosis Type 1 (NF1).

Symptoms:

Symptoms are due to progressive growth of a mass that invades and presses on the optic nerve and adjacent structures:

  • vision loss
  • squinting
  • involuntary movement of eyeball
  • eye may bulge outward (painless proptosis)

Signs and tests:

A neurologic examination reveals a loss of vision in one or both eyes. There may be changes in the optic nerve, including swelling or scarring of the nerve or paleness and atrophy of the optic disk.

There may be extension of the tumor into deeper locations of the brain and signs of increased pressure within the brain (intracranial pressure). There may be signs of NF1.

  • A head CT scan or MRI of the head confirms the diagnosis and the exact location of the tumor.
  • Cerebral angiography is not often necessary, but if used, it shows a space-occupying mass which is not vascular.
  • Visual field tests can help determine how invasive the tumor is
  • An examination of tissue removed from the tumor during surgery or CT scan-guided biopsy is used to confirm the exact type of tumor.
Treatment:

Treatment varies with the size of the tumor and the general health of the person. The goal of treatment may be to cure the disorder, relieve symptoms, or improve vision or comfort.

Surgical removal may cure some optic gliomas. Partial removal to reduce some of the bulk of the tumor is feasible for many cases, which will minimize pressure-induced damage from the tumor.

Radiation therapy may be advised in some cases where the tumor is extensive and surgery is not possible. In some cases, the use of radiation may be delayed because of the slow growth that this tumor typically displays. Corticosteroids may be prescribed to reduce swelling and inflammation during radiation therapy, or if symptoms recur.

Support Groups:
For organizations that provide support and additional information, see blindness resources.
Expectations (prognosis):

The outlook is highly variable. Early treatment improves the chance of a good outcome. Many tumors are curable with surgery, while others recur. Normally, the growth of the tumor is very slow, and the condition remains stable for long periods of time.

There are a small portion of these types of tumor that behave aggressively in about 20% of childhood cases where the optic chiasm is involved.

Complications:
Calling your health care provider:
Call your health care provider if vision loss occurs or you note a painless bulging of your eye forwards (proptosis).
Prevention:
Genetic counseling may be advised for people with neurofibromatosis-1. Regular eye exams may help early diagnosis of these tumors before they cause symptoms.

Review Date: 11/17/2002
Reviewed By: Joseph V. Campellone, M.D., Division of Neurology, Cooper Hospital/University Medical Center, Camden, NJ. Review provided by VeriMed Healthcare Network.
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