title: Fibrous dysplasia of the zygomaticomaxillary region: outcomes of surgical intervention.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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).
  
  
  RESULTS
  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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Los Angeles, Calif. From the Division of Plastic and Reconstructive Surgery, David Geffen School of Medicine, University of California, Los Angeles.
authors.forename: Joubin S
authors.initials: JS
authors.lastname: Gabbay
authors.forename: Joyce T
authors.initials: JT
authors.lastname: Yuan
authors.forename: Brian T
authors.initials: BT
authors.lastname: Andrews
authors.forename: Henry K
authors.initials: HK
authors.lastname: Kawamoto
authors.forename: James P
authors.initials: JP
authors.lastname: Bradley
mesh_terms: D000293:Adolescent; D000328:Adult; D016025:Bone Transplantation / Q000379:methods; D002648:Child; D003299:Cooperative Behavior; D019299:Decompression, Surgical; D005260:Female; D005358:Fibrous Dysplasia, Monostotic / Q000175:diagnosis / Q000601:surgery*; D005359:Fibrous Dysplasia, Polyostotic / Q000175:diagnosis / Q000601:surgery*; D006801:Humans; D033183:Interdisciplinary Communication; D008297:Male; D008439:Maxillary Diseases / Q000175:diagnosis / Q000601:surgery*; D009408:Nerve Compression Syndromes / Q000601:surgery; D009901:Optic Nerve Diseases / Q000601:surgery; D012516:Osteosarcoma / Q000175:diagnosis / Q000601:surgery; D010781:Photography; D011183:Postoperative Complications / Q000175:diagnosis / Q000209:etiology; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014792:Visual Acuity; D055815:Young Adult; D015050:Zygoma / Q000473:pathology / Q000601:surgery*