中国口腔颌面外科杂志

中国口腔颌面外科杂志 ›› 2023, Vol. 21 ›› Issue (5): 472-478.doi: 10.19438/j.cjoms.2023.05.008

• 论著 • 上一篇    下一篇

20例面部先天性浸润性脂肪增殖症的临床及影像学分析

许贵松1, 杨功鑫2, 游元和1, 田卓炜1, 肖孟1, 杜仲1, 王延安1   

  1. 1.上海交通大学医学院附属第九人民医院 口腔颌面-头颈肿瘤科,上海交通大学口腔医学院,国家口腔医学中心,国家口腔疾病临床医学研究中心,上海市口腔医学重点实验室,上海市口腔医学研究所,上海市头颈肿瘤诊治和转化医学中心,上海 200011;
    2.上海交通大学医学院附属第九人民医院 放射科,上海 200011
  • 收稿日期:2023-04-12 修回日期:2023-05-12 出版日期:2023-09-20 发布日期:2023-10-11
  • 通讯作者: 王延安,E-mail：yan.an.wang@sjtu.edu.cn
  • 作者简介:许贵松（1996-）,男,硕士,E-mail：15821900378@163.com
  • 基金资助:
    国家自然科学基金（81371164、81870780、82201085）

Clinical and radiographic analysis of 20 patients of congenital infiltrating lipomatosis of the face

XU Gui-song1, YANG Gong-xin2, YOU Yuan-he1, TIAN Zhuo-wei1, XIAO Meng1, DU Zhong1, WANG Yan-an1   

  1. 1. Department of Oromaxillofacial Head and Neck Oncology, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine; College of Stomatology, Shanghai Jiao Tong University; National Center for Stomatology; National Clinical Research Center for Oral Diseases; Shanghai Key Laboratory of Stomatology, Shanghai Research Institute of Stomatology, Shanghai Center of Head and Neck Oncology Clinical and Translational Science. Shanghai 200011;
    2. Department of Radiology, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine. Shanghai 200011, China
  • Received:2023-04-12 Revised:2023-05-12 Online:2023-09-20 Published:2023-10-11

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摘要： 目的：总结分析面部先天性浸润性脂肪增殖症（congenital infiltrating lipomatosis of the face,CILF）的临床及影像学特征。方法：收集诊断为CILF患者的临床及影像学资料,回顾分析临床、影像学特征,并对其影像学特征进行统计学分析。采用GraphPad Prism 5.0软件包对数据进行统计学分析。结果：20例CILF患者纳入研究（男女比例1∶1）,患者均自出生时即观察到半侧面部肥大,影像学表现为半侧面部脂肪浸润性增厚（颊部、颞部、腭黏膜、咬肌肌间隙等）、上颌结节畸形、颌骨密度改变等特征。与健侧对比,软组织厚度及骨密度存在显著差异（P<0.05）。基于临床及影像学特征分析发现,CILF病变与三叉神经上颌支（80%~100%）、下颌支（25%~100%）分布区域有很强的潜在对应关系。结论：CILF具有鲜明的临床及影像学特征,对其早期诊断及后期随访具有重要意义。同时,CILF与三叉神经上颌支、下颌支的分布区密切相关,提示CILF可能与早期神经发育调控有关。

关键词: 面部先天性浸润性脂肪增殖症, 影像学特征, 三叉神经

Abstract: PURPOSE: To summarize and analyze the clinical and radiographic characteristics of congenital infiltrating lipomatosis of the face (CILF). METHODS: Clinical and radiographic data of patients diagnosed with CILF were collected and retrospectively analyzed. Statistical analysis of characteristic radiographic changes was performed using GraphPad Prism 5.0 software package. RESULTS: Twenty patients diagnosed with CILF were included in the study(male to female ratio was 1:1). All suffered from hemifacial hypertrophy since birth, and showed features of hemifacial fatty infiltration thickening(cheek, temporal region, palatal mucosa, masseter space, etc.), deformity of maxillary tuberosity, abnormal mandibular density, etc. Compared with the healthy side, there were significant differences in soft tissue thickening and bone density(P<0.05). According to the distribution pattern of the trigeminal nerve, the frontal branch-related region was rarely affected, while the superior (80%-100%) and mandibular(25%-100%) branches had strong potential correspondence with CILF. CONCLUSIONS: CILFs have unique clinical and imaging features which are of great significance for early diagnosis and follow-up. Furthermore, CILFs are closely related to the controlled area of the superior and mandibular branches of the trigeminal nerve, suggesting that CILF may be related to early neural development.

Key words: Congenital infiltrating lipomatosis of the face, Radiographic characteristic, Trigeminal nerve

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