Aplasia of the greater wing of the sphenoid is a characteristic orbital bony anomaly in von Recklinghausen's disease, responsible for unilateral proptosis. The radiological diagnosis is based on the so called "empty orbit" appearance at routine skull X rays. Computerised tomography has allowed a precise differential diagnosis in 6 patients with neurofibromatosis and congenital absence of the greater wing of the sphenoid. Not only the bony defect is well demonstrated in all its extent but also the associated soft tissue anomalies such as presence of abnormal neurofibromatous tissue, hyperplasia of the temporal lobe, arachnoid cyst etc.
Adolescent Adult