Bone Changes in Orbital Neurofibromatosis

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Abstract
Neurofibromatous involvement of the orbit and visual apparatus produces a distinctive (but not specific) radiological pattern which may include a congenital defect of the sphenoid bone, overgrowth of the bony orbit, a bulging temporal fossa, an enlarged optic canal and an abnormal pituitary fossa. This pattern is fairly constant and not uncommon in neurofibromatosis[long dash]it was present in 15 of 59 proven cases whose radiographs were examined. About 10 per cent of all optic gliomata occur in patients with neurofibromatosis. The clinical and radiological features of 12 of the 15 patients are described and the radiological differential diagnosis discussed. The importance of correct radiological interpretation lies in the prevention of needless craniotomy since a space occupying lesion may mistakenly be thought responsible for the radiological features associated with the condition.

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