Abstract

IntroductionPatients with fronto-orbital fibrous dysplasia (FD) occasionally present fronto-orbital protrusion, exophthalmos, and visual acuity disturbance. Simultaneous management of these conditions has not been previously described.Case descriptionA-10-year-old female with fronto-orbital FD complained of left visual acuity disturbance. Head computed tomography showed compressed optic canal secondary to thickened bone. Decompression of the optic canal via the left frontotemporal extradural approach, opening of the lateral orbital wall, and dissection of the prominent zygoma were done simultaneously. The patient’s visual acuity disturbance and exophthalmos subsequently improved postoperatively.Discussion and evaluationWhen optic canal decompression is performed by the fronto-temporal approach, opening of the lateral orbital wall can be done simultaneously to decrease the intraorbital pressure and to prevent exophthalmos. In addition, although aesthetic plastic surgery is not generally recommended during the growing phase (due to the possibility of recurrence), this approach can prevent skin loosening and adverse cosmetic outcomes.ConclusionsAesthetic plastic surgery for fronto-orbital FD is recommended to prevent skin loosening. Opening of the lateral orbital wall should be performed when optic canal decompression is planned.

Highlights

  • Patients with fronto-orbital fibrous dysplasia (FD) occasionally present fronto-orbital protrusion, exophthalmos, and visual acuity disturbance

  • When optic canal decompression is performed by the fronto-temporal approach, opening of the lateral orbital wall can be done simultaneously to decrease the intraorbital pressure and to prevent exophthalmos

  • Fronto-orbital fibrous dysplasia (FD) is a relatively rare disease characterized by fronto-orbital protrusion, exophthalmos, and downward mobilization of the eyeball that leads to orbital dystopia

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Summary

Discussion and evaluation

Fronto-orbital FD sometimes causes disturbance of ocular movement as well as deterioration and disturbance of visual acuity (Papay et al 1995; Kaneshige et al 2014; Rahman et al 2009). Therapeutic decompression is an accepted approach for this condition, especially for the cases with progressive gradual visual disturbance and within 1 week of a sudden visual loss secondary to FD (Ying et al 2007; Yu et al 1997). Decompression of the optic canal, especially for FD, carries a risk of iatrogenic visual impairment, even when treated by experienced neurosurgeons, as reported by Kaneshige et al (2014). Management of visual acuity disturbance caused by FD with involvement of the optic canal remains. Visual acuity disturbance of this patient is probably caused by increased orbital pressure and optic canal stenosis. Lateral orbital wall decompression has a beneficial cosmetic effect in patients with exophthalmos.

Conclusions
Background
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