Surgical management of syndromic versus 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 subtypes). The secondary objectives were to assess the prevalence of affected craniofacial bones and the main clinical presentation. The authors performed a systematic review and meta-analysis by conducting a comprehensive electronic search from 1 January 2000 to 31 December 2019. A total of 1260 patients were included. The maxilla was the most affected facial bone (41%) (p<0.001, CI 38.3 to 43.8) and facial asymmetry was the chief complaint (p<0.001, CI 31.7 to 37.1). Conservative surgery registered higher recurrence rates than radical resection in both syndromic (84%) (p<0.001, CI 70.9 to 92.8) and non-syndromic patients (26%) (p<0.001, CI 21.8 to 30.6). Compared with prophylactic decompression, therapeutic optic nerve decompression (OND) showed better postoperative outcomes in both syndromic (p=0.9, CI 18.6 to 55.9) and non-syndromic patients (p=0.09, CI 9.3 to 28.4). Watchful waiting showed excellent results in both subgroups 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, but it is often difficult to perform due to the extent and location of the disease. Furthermore, the authors advise early therapeutic over prophylactic OND to prevent optic nerve atrophy. Asymptomatic patients should be managed expectantly. Finally, medical management helps reduce the symptoms of bone pain (p=0.02 in non-syndromic and p<0.001 in syndromic patients).