Fibrous Dysplasia of the Orbital Region: A Multidisciplinary Approach

Abstract

Abstract: : Purpose: To assess the role of ophthalmologists, maxillo–facial surgeons and neurosurgeons in the diagnosis and management of patients with orbital fibrous dysplasia (OFD), an abnormal development of bony tissue with potential involvement of ocular structures . Methods: Fourteen patients affected by OFD underwent surgery. Twelve patients underwent the removal of the supraorbital arch and the dysplastic process involving the anterior and middle base of the skull. Four of these twelve patients underwent also the optic canal decompression and two patients underwent the removal of FD involving the maxillary bone with dacryocystorhinostomy. Reconstruction was performed using an autologus bone graft both in adults and in children. The mean follow–up was 48,2 months (range 14–92, SD +/–17,3). Postoperative ophthalmological status was compared to preoperative one. Results: The main outcomes were to obtain satisfactory functional and aesthetic results. During the immediate postoperative period, all patients complained of some degree of diplopia, which was also confirmed by the Hess–Lancaster test. Diplopia spontaneously resolved within 1–6 months without need for orthoptic or surgical treatment, except in the case of one patient who required the permanent use of prismatic lenses. No postoperative reduction in visual function occurred in the patients who underwent optic nerve decompression. No recurrence of epiphora was observed in patients who underwent to dacryocystorhinostomy. Conclusions: Orbital fibrous dysplasia may lead to asymmetry, disfigurement and functional ocular problems. Early diagnosis of the signs of optic nerve damage is the first aim of the preoperative ophthalmological follow–up even if the matter of prophylactic surgical decompression before any clinical signs of optic canal compression is still controversial. The multidisciplinary approach to this disease is essential both for treatment planning and surgical of the patients.

Keywords: orbit • eye movements • tumors