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A multidisciplinary approach to sphenoid wing dysplasia presenting with pulsatile proptosis in neurofibromatosis Type
S Prathibha1, Vandana Parasar1, S Yasmin2
1Department of Ophthalmology, Rajarajeswari Medical College and Hospital, Bengaluru, Karnataka, India.
Abstract:
Neurofibromatosis (NF) with sphenoid wing dysplasia is a rare clinical entity. Herewith, we present a case of NF with sphenoid wing dysplasia which presented with pulsatile progressive proptosis. Other ocular symptoms or visual disturbances were absent. Diagnosis of the condition was not easy and the management was a challenging task which needed multidisciplinary approach as there were ocular, neurological, orthopedic, and dermatological manifestations. With neurosurgical intervention, reconstruction of the sphenoid wing was possible. Proptosis was corrected without any disturbance of vision.
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