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

Naran, Sanjay M.D.; Swanson, Jordan W. M.D., M.Sc.; Ligh, Cassandra A. M.D.; Shubinets, Val M.D.; Taylor, Jesse A. M.D.; Bartlett, Scott P. M.D.

Plastic and Reconstructive Surgery: October 2018 - Volume 142 - Issue 4 - p 518e-526e
doi: 10.1097/PRS.0000000000004779
Pediatric/Craniofacial: Original Articles
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Background: Sphenoid wing dysplasia in patients with neurofibromatosis type 1 may result in challenging and significant changes, including ultimately vision loss. The authors 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 neurofibromatosis type 1. Records were reviewed in their entirety for each subject, with attention to ophthalmologic evaluation. Radiographic images were digitally analyzed and scored for sphenoid transformations on a grade of 0 to 3.

Results: Fifty-two subjects were identified. On initial imaging, 42.31 percent had a normal sphenoid (grade 0), 32.69 percent had an abnormal contour (grade 1), 11.54 percent had thinning (grade 2), and 13.46 percent had a gross defect (grade 3). Among the 45 subjects with serial imaging, 55.56 percent 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 < 0.0001). Of subjects identified as having a gross sphenoid defect, 26.7 percent were managed using orbitosphenoid reconstruction with titanium mesh and cranial bone graft, and 33.3 percent were managed with soft-tissue debulking and canthopexy only. Vision was more likely to be preserved with early orbitosphenoid reconstruction (p < 0.05).

Conclusions: Sphenoid dysplasia is a progressive disease. Loss of vision is associated with a gross defect, and appears to be better preserved with early orbitosphenoid reconstruction with titanium mesh and cranial bone graft.

Park Ridge, Ill.; and Philadelphia, Pa.

From the Division of Pediatric Plastic and Reconstructive Surgery, Advocate Children’s Hospital; and the Division of Plastic and Reconstructive Surgery and the Section of Neuro-Oncology, Division of Oncology, Children’s Hospital of Philadelphia.

Presented at the 16th Biennial Meeting of the International Society of Craniofacial Surgeons, in Tokyo Bay, Japan, September 14 through 18, 2015.

Received for publication October 30, 2017; accepted March 29, 2018.

Disclosure:The authors have no financial interest to declare in relation to the content of this article.

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Scott P. Bartlett, M.D., Children’s Hospital of Philadelphia, Colket Translational Research Building, 3501 Civic Center Boulevard, 9th Floor, Philadelphia, Pa. 19104, bartletts@email.chop.edu

Copyright © 2018 by the American Society of Plastic Surgeons