Craniofacial fibrous dysplasia: Long-term postoperative outcomes in a retrospective case series with up to 40 years of follow-up.
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BACKGROUND: Craniofacial fibrous dysplasia (CFD) is a non-malignant disease characterized by fibro-osseous lesions in the affected bones. Management is multidisciplinary, with surgery often serving as the mainstay of treatment and stratified according to four anatomical zones. This study aimed to evaluate the long-term outcomes and assess potential modifications to the algorithm. METHODS: This retrospective study included patients diagnosed with CFD or McCune-Albright syndrome (MAS) who received surgical intervention between 1972 and 2000, with a follow-up period exceeding 20 years. Demographic characteristics, surgical procedures, and follow-up computed tomographic scans and photographs were included in the analysis. RESULTS: Of 93 surgically treated patients with CFD/MAS, 13 were available for ≥20-year follow-up, with complete data and CT imaging (mean 30 ± 6 years, range 20-40 years). Ten patients underwent radical resection and immediate bone graft reconstruction, and two of them experienced recurrence. Three patients underwent therapeutic optic nerve decompression, and two patients experienced post-surgical vision improvement. One patient underwent surgery for hearing loss in the external auditory canal and experienced improved hearing post-surgery. One patient diagnosed with MAS experienced significant relief from craniofacial pain after receiving adjuvant hormone therapy. CONCLUSIONS: Surgical strategies tailored to the four anatomical zones of CFD yield satisfactory outcomes. For Zone 1 lesions, based on our experience, radical resection followed by immediate bone graft reconstruction is a viable option. In Zone 3, therapeutic rather than prophylactic optic nerve decompression is suggested. Orthognathic surgery is also a viable option for such patients, improving occlusion and facial appearance.