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Optic Nerve Glioma
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Is this a
life-threatening tumor?
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What are the
symptoms of this condition?
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Any
associated systemic conditions?
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What can be done to patients suffering from optic nerve glioma? How
is it diagnosed? How is progression monitored? Treatment options?
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Is this a life-threatening
tumor?
In
contrast to that found in adults, optic nerve gliomas in children are
benign. However, it is visually threatening because the tumors can
occur anywhere along the optic nerve, chiasm or optic tract. More
commonly, they involve the optic chiasm, the part of the visual system
where the neurons of both optic nerves cross. The tumor eventually
replaces the normal architecture of the nerves. Optic nerve glioma
represents 2/3 of all primary optic nerve tumors and is commonly seen in
children younger than 10 years of age.
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What are the symptoms
of this condition?
Optic nerve and chiasmal gliomas are slow-growing, and patients may
present with different symptoms. The most common presentation is visual
loss, which is usually bilateral, when the chiasm is involved. Other
findings include optic nerve atrophy, strabismus, nystagmus and
proptosis. Posteriorly located tumors can present with diabetes
insipidus, hyperactivity, hypotension, hypoglycemia, or simply failure
to thrive after a period of normal growth.
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Any associated systemic
conditions?
There is an important association between optic nerve glioma and
neurofibromatosis. Approximately 10-70% is associated to
neurofibromatosis type 1. Likewise, approximately 15% of
neurofibromatosis patients will develop optic nerve gliomas. Compared
to patients without neurofibromatosis type 1, the tumor progression is
slower, vision fluctuation is common, visual prognosis is good, but life
expectancy is shorter, in patients with neurofibromatosis type 1.
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What can be done to patients suffering from optic nerve glioma? How is
it diagnosed? How is progression monitored? Treatment options?
The classic fusiform enlargement of the optic nerve can be documented by
CT scan. However, MRI (particularly T2-weighted) is considered the best
modality to evaluate tumor anatomy in relation to other important
cranial structures.
Obtaining serial ocular examinations and neuroimaging are important to
monitor the tumor progression. Neuroimaging should be performed every 6
months and visual acuity with visual field testing at approximately 3
month intervals, at least for the first year after diagnosis. The NF1
Optic Pathway Glioma Task Force has recommended annual screening,
including neuroimaging, for children with asymptomatic NF1 until age 6
and examinations every 2 years thereafter.
The
treatment of optic nerve gliomas remains controversial because the
natural history is unknown. Some studies indicate that 50% show
progressive enlargement with visual loss. Progressive visual loss and
enlargement of the tumor are indications for therapy.
If
the tumor is localized in one optic nerve, many advocate sacrificing
vision, by surgical removal of the optic nerve. This is done to prevent
involvement of the chiasm and the more posterior structures.
There is no single accepted indication for treatment of chiasmal glioma.
If there is involvement of the hypothalamus or third ventricle or gross
enlargement of the optic tract, treatment by radiotherapy is 50%
effective in controlling the disease. In children younger than 4 years
of age, chemotherapy with vincristine and carboplatin is often preferred
over radiation therapy. Chemotherapy may delay radiation; however,
about 60% of these children eventually relapse.
Source: Listernick et al. Optic pathway gliomas in children with
neurofibromatosis 1: concensus statement from the NF1 Optic Pathway
Glioma Task Force. Ann Neurol 1997; 41: 143-149.
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EYE REPUBLIC
Ophthalmology Atlas
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EYE REPUBLIC Ophthalmology Clinic
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EYE REPUBLIC
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Asian Hospital
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EYE REPUBLIC
Ophthalmology Clinic
St. Luke's
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OPTIC NERVE GLIOMA
information compiled by
Dr. Barbara
L. Roque and initially uploaded on September 13, 2005.
Last updated on
September 13, 2007. |