title: Sinonasal disease in polyostotic fibrous dysplasia and McCune-Albright Syndrome.
abstract: OBJECTIVE
  To characterize the spectrum, symptoms, progression, and effects of endocrine dysfunction on sinonasal disease in polyostotic fibrous dysplasia (PFD) and McCune-Albright Syndrome (MAS).
  
  
  METHODS
  Retrospective review.
  
  
  METHODS
  A prospectively followed cohort of subjects with PFD/MAS underwent a comprehensive evaluation that included otolaryngologic and endocrine evaluation, and imaging studies. Head and facial computed tomography scans were analyzed, and the degree of fibrous dysplasia (FD) was graded using a modified Lund-MacKay scale. Those followed for >4 years were analyzed for progression.
  
  
  RESULTS
  A total of 106 patients meeting inclusion criteria were identified with craniofacial FD. A majority (92%) demonstrated sinonasal involvement. There were significant positive correlations between the sinonasal FD scale score and chronic congestion, hyposmia, growth hormone excess, and hyperthyroidism (P < .05 for all). Significant correlations were not found for headache/facial pain or recurrent/chronic sinusitis. Thirty-one subjects met the criteria for longitudinal analysis (follow-up mean, 6.3 years; range, 4.4-9 years). Those who demonstrated disease progression were significantly younger than those who did not (mean age, 11 vs. 25 years). Progression after age of 13 years was uncommon (n = 3) and minimal. Concomitant endocrinopathy or bisphosphonate use did not have any significant effect on progression of disease.
  
  
  CONCLUSIONS
  Sinonasal involvement of fibrous dysplasia in PFD/MAS is common. Symptoms are usually few and mild, and disease progression occurs primarily in young subjects. Concomitant endocrinopathy is associated with disease severity, but not progression.
authors: Department of Otolaryngology-Head and Neck Surgery, Georgetown University Hospital, Washington, DC 20007, USA.
authors: Timothy R
authors: TR
authors: DeKlotz
authors: Hung Jeffrey
authors: HJ
authors: Kim
authors: Marilyn
authors: M
authors: Kelly
authors: Michael T
authors: MT
authors: Collins
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D018450:Disease Progression; D005260:Female; D005359:Fibrous Dysplasia, Polyostotic / Q000150:complications*; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D010254:Paranasal Sinus Diseases / Q000175:diagnosis* / Q000209:etiology; D055815:Young Adult