蝶骨气化在 1 型神经纤维瘤病患者侧位头颅侧位片中的表现。
Sphenoid Bone Pneumatisation on Lateral Cephalograms of Patients With Neurofibromatosis Type 1.
机构信息
Department of Oral and Craniomaxillofacial Surgery, Eppendorf University Hospital, University of Hamburg, Hamburg, Germany;
Neurofibromatosis Laboratory, Hamburg, Germany.
出版信息
In Vivo. 2021 Jan-Feb;35(1):349-361. doi: 10.21873/invivo.12266.
BACKGROUND/AIM: Neurofibromatosis type 1 (NF1) is an autosomal dominant hereditary disease that causes tumors and many developmental disorders, e.g., cranial dysplasia. The purpose of this retrospective study was to analyse the pneumatisation of the sphenoid bone in NF1.
PATIENTS AND METHODS
The anonymised lateral cephalograms of 166 NF1 patients and 166 age- and sex-matched controls were examined for anterior-posterior sphenoid pneumatisation. The patient group analysis considered whether the patients had been affected by a facial plexiform neurofibroma (FPNF).
RESULTS
Sphenoid pneumatisation was significantly lower in NF1 patients than in controls [odds ratio (OR)=0.184; 95%CI=0.11-0.32; p<0.001]. A FPNF statistically significantly reduced sinus formation in patients (OR=0.38; p=0.002).
CONCLUSION
The condition 'NF1' has an effect on sphenoid pneumatisation. The findings are relevant for planning surgical procedures in this region and confirm current concepts to evaluate NF1 as a histogenesis control gene. The examination technique and basis of calculation presented here are easy-to-use and low-irradiation exposure instruments for screening for differences in sphenoid bone pneumatisation in defined populations.
背景/目的：神经纤维瘤病 1 型（NF1）是一种常染色体显性遗传疾病，可导致肿瘤和许多发育障碍，例如颅面骨发育不良。本回顾性研究旨在分析 NF1 患者蝶骨的气化情况。
患者和方法
对 166 例 NF1 患者和 166 名年龄和性别匹配的对照者的侧位头颅侧位片进行了前后位蝶骨气化分析。在患者组分析中，考虑了患者是否患有面部丛状神经纤维瘤（FPNF）。
结果
NF1 患者的蝶骨气化明显低于对照组[比值比（OR）=0.184；95%可信区间（CI）=0.11-0.32；p<0.001]。FPNF 统计学上显著降低了患者的鼻窦形成（OR=0.38；p=0.002）。
结论
“NF1”状态对蝶骨气化有影响。这些发现对该区域的手术计划具有重要意义，并证实了当前将 NF1 评估为组织发生控制基因的概念。这里提出的检查技术和计算基础是一种易于使用且低辐射暴露的工具，可用于筛查特定人群中蝶骨气化的差异。
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