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家族性巨大型牙骨质瘤(familial gigantiform cementoma,FGC)是一类具有常染色体显性遗传特征的良性纤维-牙骨质-骨病变。病灶可累及上、下颌骨,以下颌骨前份的膨胀性生长最为典型,常导致颜面畸形和口腔功能障碍。由于该病罕见,其治疗的相关报道十分有限。该病好发于青少年,患者多伴发长骨骨折,因此治疗方案的选择尤为慎重。本文报告1个FGC家系中2例患病者下颌骨重建的不同方式,在随访过程中对患者术后面部外观、口腔功能和影像学资料进行评价,结合相关文献复习,对FGC患者下颌骨重建方式选择的经验进行总结。
Familial gigantiform cementoma (familial gigantiform cementoma, FGC) is a type of autosomal dominant genetic characteristics of fibro-osteodomatic lesions. Lesions can affect the upper and lower jaws, most of the expansion of the former part of the mandible most typical growth, often leads to facial deformities and oral dysfunction. Because of the rare disease, the treatment of its related reports is very limited. The disease occurs in adolescents, many patients with long bone fractures, so the treatment options are particularly cautious. This article reports on two different ways of mandibular reconstruction in 2 patients with FGC pedigree. During the follow-up, the facial appearance, oral function and imaging data of the patients were evaluated. Combined with related literatures, the mandibular reconstruction of FGC patients The way to choose the experience to be summarized.