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視神經膠質瘤-兩例報告

Optic Nerve Glioma (Report of Two Cases)

並列摘要


Optic nerve glioma is a slowly growing benign and very rare tumor, almost always unilateral and has no tendency to metastasize. It can occur at any places from optic disc to optic chiasma, but it frequently arises front intraorbital portion of the optic nerve. Optic nerve glioma usually occurs under the age of 11, female patient has a higher incidence than male. The three main classical symptoms are proptosis, limitation of ocular movement and visual deterioration. The fundus change is papilledema and optic nerve atrophy. In chiasma glioma, the visual impairment and optic nerve atrophy are bilateral with special visual field change. About 10% of optic nerve glioma accompanied with von Recklinghausen's disease. X-ray findings indicates enlargement of optic foramen, and orbitography demonstrates retrobulbar orbital filling defect in optic nerve glioma. In chiasma glioma, special anterior clinoid and sellar turcica change with enlargement of lateral and third ventricle can be demonstrated. Grossly, the tumor is grayish-whitish in color, surface smooth, spindle shaped or diffuse enlargement of the optic nerve. Histologically, it reveals moderate increase in cellularity with small stellate and piloid astrocyte, there is no mitosis or cytologic evidence of malignancy. The advancement of tumor in the nerve tissue is not by true tumor invasion, it is the result of transformation of adjacent neuroglia by the stimulation of tumor elements. The treatments include radiotherapy and surgery, that depending upon localization of tumor and the degree of invasion. Some authors suggest that radiotherapy is the choice of treatment but others emphasize surgical treatment. The surgical treatments are Krönlein operation, Killian type operation and transcranial unroofing method. Two cases of optic nerve glioma are presented here with pathological proof. Case 1 was a 14-month old Chinese female with initial symptoms of fever and proptosis (OS), The first impression was orbital cellulitis. Later, eye ball deviation, limitation of eye ball movement and papilledema appeared too, and X-rays showed enlarged left optic canal. Under the impression of orbital tumor (OS), Krönlein operation was performed and removed the eye ball, orbital part of optic nerve and tumor mass. Postoperative course was rather smooth and the patient received supplementary deep X-ray therapy. Follow up study two years later showed no evidence of recurrence. Case 2 was a 11-year old Chinese boy presenting himself with the symptoms of proptosis, impaired vision of right eye and papilledema. X-ray showed enlarged right optic canal and retrobulbar filling defect was detected by orbitography. Transfrontal craniotomy was performed at Surgical Department, a tumor mass extended around the chiasma and couldn't be separated from optic nerve but chiasma itself and left optic nerve seemed intact grossly. Right optic nerve and tumor mass were resected but right eye ball was preserved. Later, the patient received deep X-ray therapy as supplementary measure. No evidence of recurrence was noted during follow up.

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