Fibrous dysplasia of the orbital region : current clinical perspectives in ophthalmology and cranio-maxillofacial surgery

PURPOSE: To describe the multidisciplinary diagnosis and treatment of patients with orbital fibrous dysplasia, a slowly progressive disease that may lead to asymmetry, disfigurement, and functional ocular problems. METHODS: Ten patients with orbital fibrous dysplasia underwent bifrontal craniotomy through a coronal flap, with the removal of the supraorbital arch and dysplastic process involving the anterior and middle base of the skull. Four patients underwent superior orbital fissure and optic nerve canal decompression. Reconstruction was performed by using an autologous bone graft for both the adults and children, in whom a rib graft was preferred. The mean follow-up was 53.2 +/- 18.3 months (range, 14 to 94 months). The patients' preoperative status and postoperative status were compared. RESULTS: The immediate and long-term morphologic and aesthetic results were good in all cases. All of the patients complained of some degree of diplopia during the immediate postoperative period, but the problem spontaneously resolved within 1 to 6 months in all but one case. No postoperative reduction in visual function was observed in the patients who underwent optic nerve decompression. The only reported complication was the irregular reabsorption of regrafted dysplastic bone in one patient. CONCLUSIONS: A multidisciplinary approach to orbital fibrous dysplasia is fundamental for treatment planning and execution.