Endoscopic endonasal optic nerve decompression: treatment of fibrous dysplasia in a pediatric population

Abstract

OBJECTIVE Patients with fibrous dysplasia (FD) of the anterior skull base can experience progressive visual loss and impairment. The authors reviewed their experience with endonasal decompression of the optic nerve (ON) in this patient population. Endoscopic ON decompression (EOND) is a feasible surgical approach for children with FD and visual deficit due to structural ON compression. METHODS Electronic medical records of children between 1 and 17 years of age with unilateral FD of the anterior skull base and concomitant ON compression, who required EOND between 2017 and 2022 (n = 4), were reviewed for demographic data, both pre- and postoperative imaging, and evaluations by an otolaryngologist, neurosurgeon, and ophthalmologist in a multidisciplinary fashion. RESULTS EOND was found to be a safe and effective surgery for children with FD. Visual acuity was stable in 80% of the eyes postoperatively. Visual fields improved in 40% of the eyes and remained stable in the rest. CONCLUSIONS EOND is beneficial for progressive optic neuropathy that is unresponsive to steroid therapy and can prevent permanent disability if performed prior to irreversible damage to the nerve. EOND can decompress the edematous ON with proper exposure of the optic canal and orbital apex, without any major complications.

Endoscopic endonasal optic nerve decompression in children younger than 2 years old with congenital optic canal stenosis: illustrative cases

“…Case reports and case series have described some favorable outcomes after EOND for nontraumatic etiologies of optic neuropathy such as fibrous dysplasia and hyperostosis in adolescent patients. 9 , 10 To our knowledge, EOND for younger patients who have not yet had sphenoid sinus pneumatization has never been reported. Here, we illustrate three cases of patients younger than 2 years of age without sphenoid sinus pneumatization who underwent EOND for congenital optic canal stenosis.…”