Optic gliomas in neurofibromatosis type 1 (NF-1). Presentation of 31 cases

We present a series of 31 cases of optic glioma associated with neurofibromatosis type 1 in patients under 11 years of age. They represent 64% of all the optic gliomas seen in our service between September 1965 and September 1993. The optic nerves were affected in 25 cases (83%); 9 children (30%) had a isolated, unilateral tumor; 16 (53%) had involvement of the optic chiasm as well as of one or both optic nerves. In 6 cases (19%) only the chiasm was affected, with or without involvement of other intracerebral structures. A coincidental orbital plexiform neurofibroma was associated with a poor prognosis. Other complications included 8 cases (26%) presenting with slowly developing aqueductal stenosis requiring treatment with a shunt. High-resolution computed-tomographic scans provided well-defined images for the diagnosis of optic nerve glioma, but magnetic resonance imaging was the preferred procedure for the diagnosis of gliomas involving the chiasm and the optic tracts and radiations, particularly if there was no mass effect. In our patients, tumor growth was hardly noticeable during follow-up even up to 20 years, with no difference between patients who were treated with radiation and those who were not treated. However, endocrine disturbances developed in all cases subjected to radiotherapy, and were less frequent in untreated patients. Aqueductal stenosis was observed with similar frequency among both treated or untreated patients. Three patients died, two due to complications of hydrocephalus secondary to aqueductal stenosis and one because of respiratory problems due to compression of the hypothalamus and brainstem by the chiasm tumor. Two of the 3 patients who died had orbital plexiform neurofibroma.