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Surgical Management of Syndromic v. non-Syndromic Craniofacial Fibrous Dysplasia: A Systematic Review and Meta-Analysis

      Abstract

      The main purpose of this study was to identify an algorithm for the surgical management of Fibrous Dysplasia in syndromic (McCune-Albright Syndrome) and non-syndromic patients (monostotic and polyostotic sub-types). The secondary objectives were to assess the prevalence of affected craniofacial bones and its main clinical presentation.
      The authors performed a systematic review and meta-analysis by conducting a comprehensive electronic search from 1st January 2000 to 31st December 2019. A total of 1260 patients were included. The maxilla was the most affected facial bone (41%) (p<0.001 [CI 38.3-43.8]), whereas facial asymmetry was the chief complaint (p<0.001 [CI (31.7-37.1]).
      Conservative surgery registered higher recurrence rates than radical resection in both syndromic (84%) (p<0.001 [CI 70.9-92.8]) and non-syndromic patients (26%) (p<0.001 [CI 21.8-30.6]). Therapeutic optic nerve decompression showed better post-operative outcomes in both syndromic (p=0.9 [CI 18.6-55.9]) and non-syndromic patients (p=0.09 [CI 9.3-28.4]) compared to prophylactic decompression. Watchful waiting showed excellent results in both sub-groups when asymptomatic (p<0.001).
      Syndromic and non-syndromic patients share the same treatment strategies. Radical resection is the preferred surgical technique to eradicate the disease; however, it is often difficult to perform due to the extent and location of the disease. Furthermore, the authors advise on early therapeutic over prophylactic optic nerve decompression to prevent optic nerve atrophy. Asymptomatic patients should be managed expectantly. Finally, medical management helps decrease symptoms of bone pain (p=0.02 in non-syndromic and p<0.001 in syndromic patients).

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