Chiasmal Glioma Research Articles

The angiographic delineation of sellar and parasellar masses.

The anatomic and normal angiographic appearance of small vessels which vascularize the neural and glandular structures in and about the sella turcica is discussed. Findings in a group of abnormal cases illustrate alterations in the local vessels which occur in the presence of sellar and parasellar masses. Some of the changes seen appear to be helpful in differentiating the various types of tumor. Tumors in which characteristic dislocations were seen include pituitary adenoma, “empty” sella, nonmeningiomatous parasellar masses, parasellar meningioma, craniopharyngioma, optic chiasm glioma, and colloid cyst of the third ventricle.

Visual morbidity with chiasmal glioma. Long-term studies of visual fields in untreated and irradiated cases.

Analysis and comparison of the natural history of visual field changes in ten untreated and ten irradiated chiasmal gliomas revealed (1) no consistent relationship between the pattern and severity of field defects and the location, size, or extent of the tumor, and (2) a monotonously stable course of field defects and visual acuity in most cases. Over prolonged periods of follow up, the untreated patients fared as well as the irradiated group. There was no clinical evidence to support the alleged efficacy of radiation therapy for these indolent congenital tumors.

Surgical treatment of unilateral optic nerve gliomas.

ARIOUS manifestations of the unpredictable natural history of optic gliomas have resulted in controversy as to the best form of treatment. Some reports of successful partial removal of these tumors 1,12 may be questioned because of the limited follow-up period at the time of reporting. Taveras, et al., 1~ described remissions and arrest with improvement in exophthalmos and vision in a group of patients treated by radiotherapy alone. On the basis of this experience they concluded that tumors limited to the orbital cavity may be treated successfully by intraorbital surgical removal but that all other cases should be treated by irradiation. Later, Chutorian and his coworkers 2 at the same institution found four cases of recurrent optic nerve glioma following radiotherapy. One of these had been reported by Taveras, et al., ~ as free of recurrence after 21 years. Some ophthalmological surgeons have advocated transorbital excision of optic nerve gliomas as adequate therapy2 TM In one of the 13 cases in this report, the tumor recurred with chiasmal spread 6 years after orbital resection. These experiences lend credence to the impression that, although radiotherapy may be effective in nonresectable optic glioma, transcranial chiasmal inspection and prechiasmal resection is the treatment of choice when tumor is confined to a single optic nerve. Furthermore, excellent cosmetic results have been possible when the structures of the orbital apex have been meticulously approached by the frontal transcranial route. The operative technique to be described is based on our surgical experience with nine cases of optic glioma. Proptosis and visual impairment were the most common presenting symptoms. The diagnosis of optic nerve glioma may be made on the basis of radiographic evidence of enlargement of one optic foramen2 Pneumoencephalography has been useful in outlining the intracranial portion of the optic nerve. Preservation of the chiasmarie notch, between the optic and infundibular recesses of the anterior third ventricle, usually indicates that the chiasm is not involved. Radioactive mercury scan has been of great value as supporting evidence for the presence of optic nerve or chiasmal glioma. Technetium scanning has proved to be less reliable.

Neurofibromatosis as a cause of conductive hearing loss. A case report.

NEUROFIBROMATOSIS EUROFIBROMATOSIS of von Recklinghausen has many manifestations. It has been reported as a cause of optic atrophy and optic chiasmal glioma by Pugh and Schlesinger.¹Gillespie²reported a case of unilateral buphthalmus in a patient with neurofibromatosis. Patients with neurofibromatosis also manifest bony abnormalities. These may include pseudarthrosis, bone defects, and asymmetrical overgrowths as mentioned by Kragh et al.³A case of pseudarthrosis of the tibia in a 41-year-old man was reported by Berk and Mankin.⁴Dunn⁵(1965) reported a case of anterolateral bowing of the tibia in a 14-year-old boy. The association of cutaneous lesions in the form of subcutaneous neurofibromata and café au lait spots with neurofibromatosis is well known. Preston et al,⁶found café au lait spots in 62% of their patients who, in addition, had other manifestations of the disease. They also reported one patient with glioma of the

OPTIC ATROPHY IN CHILDHOOD

An attempt has been made to determine the significance and outcome of optic atrophy as an apparently isolated entity in the pediatric age group. A follow-up study was made of 48 children who presented in early childhood with optic atrophy with no known cause and no obvious systemic or neurological disease. It was found that the prognosis for useful vision is in general favorable in this group of cases. However, the significant incidence of mental retardation and other neurological impairment was noted for those children who had manifested severely impaired vision under one year of age. There was no correlation between specific symptoms and the outcome of vision. The patients with idiopathic optic atrophy were compared with other patients who presented with optic atrophy without signs of systemic or neurological disease on initial examination but who were later found to have underlying disease. X-ray findings of enlargement of the optic foramina, deformity of the sella turcica, and suprasellar calcification or hyperostitic changes in the region of the optic foramen were found to be important differential diagnostic features, as was the presence of proptosis or visual field defects, reflecting involvement of the optic chiasm or retrochiasmal visual pathways. By the use of these features and certain laboratory determinations it was possible to separate the idiopathic group from patients whose optic atrophy was secondary to optic nerve and chiasm gliomas, other intracranial neoplasms, fibrous dysplasia, congenital syphilis, and orbital neurofibroma. Problems in the diagnosis and differential diagnosis of optic atrophy have been discussed.