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Sinonasal Disease in Polyostotic Fibrous Dysplasia and McCune–Albright Syndrome
Author(s) -
DeKlotz Timothy R.,
Kim Hung Jeffrey,
Kelly Marilyn,
Collins Michael T.
Publication year - 2013
Publication title -
the laryngoscope
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.181
H-Index - 148
eISSN - 1531-4995
pISSN - 0023-852X
DOI - 10.1002/lary.23758
Subject(s) - medicine , polyostotic fibrous dysplasia , fibrous dysplasia , mccune–albright syndrome , concomitant , craniofacial , gastroenterology , surgery , precocious puberty , hormone , psychiatry
Objectives/Hypothesis: To characterize the spectrum, symptoms, progression, and effects of endocrine dysfunction on sinonasal disease in polyostotic fibrous dysplasia (PFD) and McCune–Albright Syndrome (MAS).Study Design: 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.