title: Surgical Management of Polyostotic Craniofacial Fibrous Dysplasia: Long-Term Outcomes and Predictors for Postoperative Regrowth.
abstract: BACKGROUND
  The mainstay of treatment for craniofacial fibrous dysplasia is surgical; however, optimal indications and techniques are poorly understood, particularly in polyostotic disease and McCune-Albright syndrome. This study investigated surgical indications and risk factors for recurrence in a large cohort.
  
  
  METHODS
  One hundred thirty-three craniofacial fibrous dysplasia subjects in a natural history study were evaluated. Radiographic studies, operative reports, and clinical records were reviewed.
  
  
  RESULTS
  Thirty-six subjects underwent 103 craniofacial procedures (mean, 2.8 operations per subject), with 13.5 ± 10.5-year follow-up (range, 0 to 39 years). The most common indication was craniofacial deformity (n = 61 operations), including 36 initial operations (59 percent) and 26 reoperations (41 percent). Mean time to reoperation was 3.4 ± 3.2 years (range, 0.3 to 13.3 years). Regrowth occurred after 42 operations (68 percent), and was more frequent after operations in subjects with McCune-Albright syndrome growth hormone excess [22 of 25 operations (88 percent)] than without growth hormone excess [15 of 36 operations (58 percent); p = 0.02]. Of 11 subjects with growth hormone excess, nine (82 percent) were undiagnosed at the time of their initial operation. Regrowth was more frequent after debulking procedures [31 of 38 (82 percent)] than after more aggressive reconstructions [nine of 20 (45 percent); p = 0.007]. Eleven subjects underwent treatment for aneurysmal bone cysts, with recurrence in one subject. Eleven subjects underwent biopsies and none had complications or regrowth.
  
  
  CONCLUSIONS
  Craniofacial fibrous dysplasia regrowth and reoperation are common, particularly after debulking procedures. Outcomes are favorable for aneurysmal bone cysts and biopsies. McCune-Albright syndrome growth hormone excess is a risk factor for regrowth, and may be underdiagnosed in surgical patients. Surgeons should be aware of appropriate screening for endocrinopathies in fibrous dysplasia. These findings highlight the importance of a multidisciplinary approach to craniofacial fibrous dysplasia, and individualized care with long-term follow-up.
  
  
  METHODS
  Therapeutic, IV.
authors.affiliation: Bethesda and Chevy Chase, Md.; Washington, D.C.; and Knoxville, Tenn.|From the Skeletal Clinical Studies Unit, Craniofacial and Skeletal Diseases Branch, the Office of the Clinical Director, National Institute of Dental and Craniofacial Research, National Institutes of Health, the Department of Plastic Surgery, Georgetown University and Medical Center; the Division of Endocrinology and Diabetes, Bone Health Program, Division of Orthopaedics and Sports Medicine, Children's National Health System; and SouthEast Eye Specialists, PLLC.
authors.forename: Alison M
authors.initials: AM
authors.lastname: Boyce
authors.affiliation: Bethesda and Chevy Chase, Md.; Washington, D.C.; and Knoxville, Tenn.|From the Skeletal Clinical Studies Unit, Craniofacial and Skeletal Diseases Branch, the Office of the Clinical Director, National Institute of Dental and Craniofacial Research, National Institutes of Health, the Department of Plastic Surgery, Georgetown University and Medical Center; the Division of Endocrinology and Diabetes, Bone Health Program, Division of Orthopaedics and Sports Medicine, Children's National Health System; and SouthEast Eye Specialists, PLLC.
authors.forename: Andrea
authors.initials: A
authors.lastname: Burke
authors.affiliation: Bethesda and Chevy Chase, Md.; Washington, D.C.; and Knoxville, Tenn.|From the Skeletal Clinical Studies Unit, Craniofacial and Skeletal Diseases Branch, the Office of the Clinical Director, National Institute of Dental and Craniofacial Research, National Institutes of Health, the Department of Plastic Surgery, Georgetown University and Medical Center; the Division of Endocrinology and Diabetes, Bone Health Program, Division of Orthopaedics and Sports Medicine, Children's National Health System; and SouthEast Eye Specialists, PLLC.
authors.forename: Carolee
authors.initials: C
authors.lastname: Cutler Peck
authors.affiliation: Bethesda and Chevy Chase, Md.; Washington, D.C.; and Knoxville, Tenn.|From the Skeletal Clinical Studies Unit, Craniofacial and Skeletal Diseases Branch, the Office of the Clinical Director, National Institute of Dental and Craniofacial Research, National Institutes of Health, the Department of Plastic Surgery, Georgetown University and Medical Center; the Division of Endocrinology and Diabetes, Bone Health Program, Division of Orthopaedics and Sports Medicine, Children's National Health System; and SouthEast Eye Specialists, PLLC.
authors.forename: Craig R
authors.initials: CR
authors.lastname: DuFresne
authors.affiliation: Bethesda and Chevy Chase, Md.; Washington, D.C.; and Knoxville, Tenn.|From the Skeletal Clinical Studies Unit, Craniofacial and Skeletal Diseases Branch, the Office of the Clinical Director, National Institute of Dental and Craniofacial Research, National Institutes of Health, the Department of Plastic Surgery, Georgetown University and Medical Center; the Division of Endocrinology and Diabetes, Bone Health Program, Division of Orthopaedics and Sports Medicine, Children's National Health System; and SouthEast Eye Specialists, PLLC.
authors.forename: Janice S
authors.initials: JS
authors.lastname: Lee
authors.affiliation: Bethesda and Chevy Chase, Md.; Washington, D.C.; and Knoxville, Tenn.|From the Skeletal Clinical Studies Unit, Craniofacial and Skeletal Diseases Branch, the Office of the Clinical Director, National Institute of Dental and Craniofacial Research, National Institutes of Health, the Department of Plastic Surgery, Georgetown University and Medical Center; the Division of Endocrinology and Diabetes, Bone Health Program, Division of Orthopaedics and Sports Medicine, Children's National Health System; and SouthEast Eye Specialists, PLLC.
authors.forename: Michael T
authors.initials: MT
authors.lastname: Collins
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002675:Child, Preschool; D005147:Facial Bones / Q000601:surgery*; D005359:Fibrous Dysplasia, Polyostotic / Q000601:surgery*; D005500:Follow-Up Studies; D006801:Humans; D008875:Middle Aged; D011183:Postoperative Complications / Q000209:etiology / Q000601:surgery; D012008:Recurrence; D012086:Reoperation; D012886:Skull / Q000601:surgery*; D055815:Young Adult