Fibrous Dysplasia of the Zygomaticomaxillary Region

Abstract

Fibrous dysplasia is the most common craniofacial tumor, presenting in both monostotic and polyostotic forms with varying degrees of severity. No consensus exists regarding the surgical management of craniofacial fibrous dysplasia, particularly in the zygomaticomaxillary region. The present study compared long-term outcomes of limited reduction burring versus radical resection of zygomaticomaxillary fibrous dysplasia. Patients with craniofacial fibrous dysplasia at the University of California, Los Angeles, Craniofacial Center from 1982 to 2008 were studied based on demographics, treatment, and follow-up data, including examinations, computed tomographic scans, photographs, physician Whitaker scoring, and patient surveys (n=97). Outcomes were compared for zygomaticomaxillary disease treated with radical resection with cranial bone graft reconstruction or limited reduction burring (n=58). Thirty-four percent of patients had monostotic disease, 66 percent had polyostotic disease, 3 percent had McCune-Albright syndrome, and 2.1 percent had malignant degeneration into osteosarcoma. Most patients had surgical treatment (84.5 percent). Of the patients that required optic nerve decompression for vision changes (11.4 percent), most (75 percent) had vision stabilization postoperatively. Differences were recorded in zygomaticomaxillary disease treated with radical resection (63.8 percent) versus reduction burring (36.2 percent) according to age (19.6 versus 14.2 years), complications (13.5 percent versus 4.8 percent), recurrence (66.7 percent versus 24.3 percent), and number of subsequent procedures (2.8 versus 4.0). There were similarities in Whitaker outcome score (1.3±0.3 versus 1.5±0.6) and patient satisfaction (2.7±0.4 versus 2.8±0.3). Although different approaches have been advocated to treat fibrous dysplasia, the authors' data support a more aggressive management for zygomaticomaxillary disease with radical resection and cranial bone graft reconstruction, especially for more involved disease. CLINICAL QUESTION OF EVIDENCE: Therapeutic, III.