โ Scribed by Timothy R. DeKlotz; Marilyn Kelly; Michael Collins; H. Jeffrey Kim
No coin nor oath required. For personal study only.
Objectives: 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 cohort. Methods: A cohort of PFD/MAS subjects underwent a comprehensive evaluation that included otolaryngologic and endocrine evaluation, and imaging studies. Head CT scans were analyzed, and the degree of fibrous dysplasia (FD) was graded using a modified Lund-MacKay scale. Those followed for more than 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 < 0.05 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 demonstrating disease progression were significantly younger than those who did not (mean age = 11 vs. 25 years). Progression was more common in younger patients; rarely occurring after the age of 13. 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. Conservative management is advised with surgical intervention reserved for intolerable symptoms.
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