Optical Coherence Tomography in the Management of Skull Base Fibrous Dysplasia with Optic Nerve Involvement

Fibrous dysplasia (FD) of the skull base can manifest with optic nerve compression. As most patients initially do not experience vision loss, controversy exists whether to proceed with prophylactic surgical decompression or elect for conservative observation. Optical coherence tomography (OCT), a physiologic imaging modality widely used to assess the condition of the retinal nerve fiber layer (RNFL), has been useful in monitoring compressive tumors on the optic nerve. This study evaluated potential use of OCT in management of patients with fibrous dysplasia and optic nerve involvement. Six patients with suspected optic nerve compression who underwent OCT imaging as part of a neuro-ophthalmic examination were reviewed over a 2-year period. Patient records were evaluated for visual examination measures, most notably the presence of optic neuropathy, and radiographic measures on computed tomography. Measures were compared by age-adjusted RNFL thickness (above or below fifth percentile) on OCT imaging. Two patients were found to have mild optic neuropathy in 1 eye each. Three of 12 eyes fell below the age-adjusted fifth percentile of RNFL thickness. Presence of optic neuropathy was associated with abnormal age-adjusted RNFL thickness but not with optic nerve compression (P = 0.45). Abnormal RNFL thickness as measured by OCT better predicted the presence of optic neuropathy than computed tomography alone. OCT may be a valuable imaging modality to monitor patients with fibrous dysplasia for development of optic neuropathy during periods of conservative watchful waiting.