摘要
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。
[Summary] Familial gigantiform cementoma （FGC） is a rare autosomal dominant, benign fibro-cementoosseous lesion that is generally limited to the facial bones, typically in the anterior portion of the mandible. The treatment remains extensive resection with reconstruction of the osseous defect to provide aesthetic and functional rehabilitation. However, patients with FGC often have concomitant long bone abnormalities and pubertal pathologic fractures. In addition, FGC always presents in the first or second decades. Therefore, the choice of bone reconstruction warrants extreme caution. Literatures regarding the treatment of such patients are limited. This paper reported 2 patients with FGC treated at our hospital. Postoperative appearance, oral function and radiographic images were evaluated. The treatment experience was summarized based on review of relevant literatures.
出处
《中国口腔颌面外科杂志》
CAS
2016年第1期89-92,共4页
China Journal of Oral and Maxillofacial Surgery
基金
上海市卫计委2014科研基金(201440403)
上海九院口腔颌面-头颈肿瘤科2014年“苗圃”基金
关键词
家族性巨大型牙骨质瘤
下颌骨重建
长骨骨折
血管化髂骨肌瓣
Fanfilial gigantiform cementoma
Mandible reconstruction
Long bone fracture
Vascularized iliac crest free flap
