Sphenoid Dysplasia in Neurofibromatosis: Patterns of Presentation and Outcomes of Treatment

Introduction: Sphenoid wing dysplasia in patients with neurofibromatosis type 1 (NF-1) may result in challenging and significant changes, including ultimately vision loss. We describe the radiographic patterns of sphenoid dysmorphology with time and age, and the impact of surgical intervention on preservation of vision. Methods: A retrospective study was performed at a single pediatric hospital, identifying subjects with NF-1. Records were reviewed in their entirety for each subject, with attention to opthamalogic evaluation. Radiographic images were digitally analyzed and scored for sphenoid transformations on a grade of 0-3. Results: Fifty-two subjects were identified. On initial imaging, 42.31% had a normal sphenoid (grade 0), 32.69% had an abnormal contour (grade 1), 11.54% had thinning (grade 2), and 13.46% had a gross defect (grade 3). Among the 45 subjects with serial imaging, 55.56% demonstrated progression of the deformity of at least one grade. Deformity progression correlated with length of imaging interval. Enucleation was noted to occur more often in subjects with a gross sphenoid defect (p