Craniofacial fibrous dysplasia surgery: a functional approach

Abstract

Background: Craniofacial fibrous dysplasia has not only esthetic but functional impact. Surgery is controversial, ranging from conservative to radical. It involves elevated hemorrhage risk, and should be progressive, based on an individual risk/benefit analysis with the aim of improving quality of life.

Case reports: Three patients (one male, two female; mean age, 35 years) with evolutive orbital-temporal maxillary dysplasia were treated between 2008 and 2009 in our department. All showed exophthalmia and nasal obstruction. In one patient, symptomatology was aggravated by a frontal sinus cyst within the dysplasia. Another had associated auditory canal obstruction inducing recurrent external otitis. Optic nerve decompression was achieved on a combined coronal and endonasal approach, assisted by neuronavigation. Complementary remodelling resection, dacryocystorhinostomy and internal optic nerve decompression were performed. Functional results showed 70 % improvement on a subjective scale for eye tension and nasal obstruction. Surgery was feasible in all patients, with no complications.

Conclusion: Current surgical management allies esthetic and functional concerns. Remodeling resection is the reference technique. The coronal approach is a good primary option for optic nerve decompression. Endonasal surgery with neuronavigation improves nasal ventilation and lacrimal canal permeability.

Keywords: Craniofacial fibrous dysplasia; Endonasal surgery; Optic nerve decompression; Remodeling resection.