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

Mandana BehbahaniDivisions of Neurological Surgery and

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Shanik FernandoOtolaryngology, Ann and Robert H. Lurie Children’s Hospital;

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Sophia PengDepartments of Neurological Surgery and

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Luis G. Fernandez IIIDepartments of Neurological Surgery and

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Natalia HajnasOtolaryngology, University of Illinois at Chicago;

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Shelly SharmaDivisions of Neurological Surgery and

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Jeffrey C. RastatterOtolaryngology, Ann and Robert H. Lurie Children’s Hospital;
Otolaryngology-Head and Neck Surgery, Northwestern University Feinberg School of Medicine, Chicago, Illinois

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Tord D. AldenDivisions of Neurological Surgery and
Departments of Neurological Surgery and

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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.

ABBREVIATIONS

EOND = endoscopic OND; FD = fibrous dysplasia; OCT = optical coherence tomography; ON = optic nerve; OND = ON decompression; RNFL = retinal nerve fiber length; VF = visual field.
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