Craniofacial fibrous dysplasia of the fronto-orbital region: a case series and literature review

Abstract

Purpose: Craniofacial fibrous dysplais (CFD), involving the fronto-orbital region often is challenging to treat because of the proximity of neurovascular and ocular structures. This article presents the surgical experience with 6 patients.

Patients and methods: Six patients ranging in age from 7 to 23 years, who had undergone surgery for CFD of the orbital region, were retrospectively reviewed. All patients had progressive complaints of deformity, pain, paresthesia or visual disturbances. Surgery generally involved extensive tumor excision and immediate orbital reconstruction with autogenous bone grafts. Two patients also were reconstructed with resected and treated autogenous bone, which then was immediately reimplanted, using rigid fixation. Three patients also had intracranial microsurgical optic canal decompression. All patients received perioperative corticosteroids.

Results: Five patients experienced partial relief of their sensory and visual disturbances. These patients also felt that there had been improvement in facial appearance. One patient with very extensive circumferential optic canal involvement, and who underwent 2-wall decompression, suffered visual loss.

Conclusion: Early surgery to address progressive sensory disturbances is recommended so as to avoid the hazards of late-stage decompression. The risks of surgery must be weighed against the benefits of achieving a more functional and aesthetic result. Long-term neuro-ophthalmologic monitoring is essential.