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Virtual Pediatric Hospital: Paediapaedia: Optic Nerve Glioma Paediapaedia: Neurological Diseases

Optic Nerve Glioma

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed


Clinical Presentation:
Symptoms are proptosis, decreased visual acuity, exophthalmus, optic atrophy, papilledema, nystagmus, increased intracranial pressure, and visual field defects. Disturbances in hypothalamic function such as precocious puberty, increased growth, panhypopituitarism, and diabetes insipidus can occur with large chiasmatic lesions. Fifty percent of patients are less than 10 years old, and 90% present by 20 years old it is associated with neurofibromatosis in 10-20% of cases.

Etiology/Pathophysiology:
Arise from optic nerves, optic chiasm, and hypothalamus. Is slow growing and low grade.

Pathology:
Not applicable

Imaging Findings:
Fusiform expansion of the optic nerve is seen. Lesions in the hypothalamus and optic chiasm grow into the third ventricle.

DDX:

References:
See References Chapter.

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