Primary tumor of the optic nerve in general is rare. That arising at the intracranial portion of the optic nerve and the optic chiasm is particularly so. This usually occurs in childhood and sometimes is associated with generalized neurofibromatosis, or Recklinghausen's disease. The most significant and often the only signs are ocular. Primary atrophy of the optic nerve associated with divergent strabismus in one eye is a common finding. Occasionally there is a superimposed papilledema. Studies of the visual fields show either total blindness in one eye and hemianopia in the other, or bitemporal or homonymous hemianopia. The roentgen rays are helpful in diagnosis. The sella turcica is often deformed and the optic foramen enlarged. The growth of the tumor is slow; invasion of the orbit producing exophthalmos occurs late. Surgical intervention is not indicated. The diagnosis is often difficult, and surgical intervention is undertaken as an exploratory measure. The
LEVITT JM. TUMOR OF THE OPTIC CHIASM AND OPTIC NERVES: REPORT OF A CASE. Arch Ophthalmol. 1937;18(1):91–94. doi:10.1001/archopht.1937.00850070103013
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