中国口腔颌面外科杂志 ›› 2016, Vol. 14 ›› Issue (1): 89-92.

• 病例报告 • 上一篇    下一篇

家族性巨大型牙骨质瘤患者下颌骨重建术式的选择:2例报告及文献复习

王宏伟1, 马春跃1, 贺光2, 徐立群1, 张陈平1*, 秦兴军1*   

  1. 1.上海交通大学医学院附属第九人民医院·口腔医学院 口腔颌面-头颈肿瘤科,上海市口腔医学重点实验室,上海 200011;
    2.上海交通大学Bio-X研究院 遗传发育与精神神经疾病教育部重点实验室,上海 200030
  • 收稿日期:2015-04-01 出版日期:2016-01-20 发布日期:2016-02-01
  • 通讯作者: 秦兴军,E-mail: qinxj1989@sina.com;张陈平,E-mail:zhang.chenping@hotmail.com。 *共同通信作者
  • 作者简介:王宏伟(1988-),女,住院医师,E-mail: xnngo-go@163.com
  • 基金资助:
    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

Mandibular reconstruction in patients with familial gigantiform cementoma: report of 2 cases and review of the literature

WANG Hong-wei1, MA Chun-yue1, HE Guang2, XU Li-qun1, ZHANG Chen-ping1, QIN Xing-jun1   

  1. 1. Department of Oromaxillofacial Head and Neck Oncology, Shanghai Ninth People’s Hospital, College of Stomatology, Shanghai Jiao Tong University School of Medicine; Shanghai Key Laboratory of Stomatology. Shanghai 200011;
    2.Bio-X Institutes, Key Laboratory for the Genetics of Developmental and Neuropsychiatric Disorders, Ministry of Education, Shanghai Jiao Tong University. Shanghai 200030, China
  • Received:2015-04-01 Online:2016-01-20 Published:2016-02-01

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。

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

Abstract: 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.

Key words: Familial gigantiform cementoma, Mandible reconstruction, Long bone fracture, Vascularized iliac crest free flap

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