BACKGROUND: Fibrous dysplasia is a benign bone lesion characterized by replacement of normal bone with fibro-osseous connective tissue. The surgical treatment of fibrous dysplasia is based on one of two different approaches, conservative or radical. METHODS: From January of 1980 to December of 2002, 95 patients with fibrous dysplasia located in the craniomaxillofacial area presented to the authors' department. Twenty-one had the polyostotic type (22 percent), two had McCune-Albright syndrome (2 percent), and 72 had the monostotic type (76 percent). Of 95 patients, 68 underwent surgery; the remaining 27 refused. Among the 68 patients who underwent surgery, 61 had radical excision, six received conservative treatment, and a patient with mandibular involvement received radical excision and immediate reconstruction with a free fibula flap after a recurrence experienced 2 years after primary remodeling surgery RESULTS: No disease recurrence was observed in cases treated with complete excision, whereas a case of mandibular involvement treated with remodeling required further surgery. Infection, resolved with antibiotics, was seen in one case, and palatal fistula was seen in two cases. In one case with cranial base involvement liquorrhea was observed. CONCLUSIONS: In most cases of monostotic or monofocal fibrous dysplasia of the craniofacial region, modern surgical techniques allow an aggressive but definitive treatment with good functional and aesthetic results. The authors perform radical treatment even in cases involving the maxilla and mandible, and prefer a conservative approach only in polyostotic cases and McCune-Albright syndrome.
BACKGROUND:Fibrous dysplasia is a benign bone lesion characterized by replacement of normal bone with fibro-osseous connective tissue. The surgical treatment of fibrous dysplasia is based on one of two different approaches, conservative or radical. METHODS: From January of 1980 to December of 2002, 95 patients with fibrous dysplasia located in the craniomaxillofacial area presented to the authors' department. Twenty-one had the polyostotic type (22 percent), two had McCune-Albright syndrome (2 percent), and 72 had the monostotic type (76 percent). Of 95 patients, 68 underwent surgery; the remaining 27 refused. Among the 68 patients who underwent surgery, 61 had radical excision, six received conservative treatment, and a patient with mandibular involvement received radical excision and immediate reconstruction with a free fibula flap after a recurrence experienced 2 years after primary remodeling surgery RESULTS: No disease recurrence was observed in cases treated with complete excision, whereas a case of mandibular involvement treated with remodeling required further surgery. Infection, resolved with antibiotics, was seen in one case, and palatal fistula was seen in two cases. In one case with cranial base involvement liquorrhea was observed. CONCLUSIONS: In most cases of monostotic or monofocal fibrous dysplasia of the craniofacial region, modern surgical techniques allow an aggressive but definitive treatment with good functional and aesthetic results. The authors perform radical treatment even in cases involving the maxilla and mandible, and prefer a conservative approach only in polyostotic cases and McCune-Albright syndrome.
Authors: Timothy R DeKlotz; S Tonya Stefko; Juan C Fernandez-Miranda; Paul A Gardner; Carl H Snyderman; Eric W Wang Journal: J Neurol Surg B Skull Base Date: 2016-06-02
Authors: V Valentini; A Cassoni; V Terenzi; M Della Monaca; M T Fadda; O Rajabtork Zadeh; I Raponi; A Anelli; G Iannetti Journal: Acta Otorhinolaryngol Ital Date: 2017-10 Impact factor: 2.124