面肩肱型肌营养不良口腔颌面部特征及颜面肌MRI分析

Features of facioscapulohumeral muscular dystrophy in oral and maxillofacial region and MRI analysis of facial muscles

目的 研究面肩肱型肌营养不良（facioscapulohumeral muscular dystrophy,FSHD）患者口腔颌面部临床及其影像学特征,为口腔临床医师明确FSHD诊断、改善FSHD面型及提高咀嚼功能提供理论依据.方法 收集2014年6月至2015年12月就诊于河北医科大学第三医院神经肌病科12例FSHD确诊病例（FSHD组）和20名身体健康的志愿者（健康对照组）的相关信息并进行口腔检查,记录恒牙龋失补牙数（decayed missing filled teeth,DMFT）、简化牙石指数（calculus index-simplified,CI-S）、咬合关系和最大开口度;运用咬合压力测试仪检查最大（佮）力;制取口腔全牙列模型,测量腭弓宽度和最大腭高度;拍摄头颅侧位X线片测量下颌平面角（mandibular plane-frankfurt horizontal plane angle,MP-FH）;颌面部MRI显示咬肌、翼内肌、翼外肌受累程度与范围,不同层面观察肌肉的外形、肌肉内脂肪浸润分级.对获得的数据进行两独立样本t检验或两独立样本Wilcoxon符号秩检验.结果 FSHD组无明显性别差异,平均就诊年龄（27.5±8.1）岁,发病年龄（15.7±7.5）岁.FSHD组喜进软食9例;闭眼困难4例;鼓腮困难8例;撅嘴面容10例;错（佮）畸形9例.FSHD组DMFT（4.0±2.3）、CI-S（5.8±2.1）、男性最大腭弓高度[（20.5±2.1） mm]、女性最大腭弓高度[（17.9±1.6）mm]及下颌平面角（31.8°±2.2°）均显著大于健康对照组（P＜0.05）;FSHD组男性腭弓宽度[（34.8±1.4） mm]、女性腭弓宽度[（33.7±1.5） mm]、男性最大（佮）力[（451.7±39.0）N]、女性最大（佮）力[（326.7±21.6）N]及最大开口度[（3.5±0.4）cm]均显著小于健康对照组（P＜0.05）.颌面部MRI结果显示,咬肌11例、翼内肌6例、翼外肌5例外形不对称;上述3块肌肉轻度脂肪浸润,主要集中在0级、1级和2级.结论 FSHD患者存在口腔卫生差、咀嚼功能低、开口度小、腭盖高拱、多数有不同程度的错（佮）畸形;颌面MRI显示咬肌、翼内肌、翼外肌呈不同程度、不对称性肌萎缩和脂肪浸润;临床医师对待颜面肌受累的口腔疾病应重视全身疾病的诊断与评判.

Objective To investigate the manifestation of facioscapulohumeral muscular dystrophy （FSHD） in oral and maxillofacial region.Methods A total of 12 patients diagnosed as FSHD and 20 healthy volunteers were included in the study.Their medical history was collected from these patients.The decayed missing filled teeth （DMFT）,calculus index-simplified （CI-S）,occlusal relationship,maximal opening of mouth and maximum bite force were recorded.The impressions were taken to measure the maximal hight of palate and the width of palate.The lateral cephalometric radiographs were also taken to measure the mandibular plane-frankfurt horizontal plane angle （MP-FH）.They finally received oral and maxillofacial region MRI examination to observe the masseter muscle,medial pterygoid muscle and lateral pterygoid muscle.The data were analyzed by t-test or Wilcoxon signed ranks test.Results There was no significant gender difference in FSHD group.The average age of treatment was （27.5±8.1） years and the average age of onset was （15.7±7.5） years.Nine patients liked to eat soft foods,4 patients had difficulties of closing eyes,8 patients had difficulties of cheek-bulging,10 patients showed pouty lips and 9 patients had mesio-malocclusion.DMFT （4.0±2.3）,CI-S （5.8±2.1）,male maximal hight of palate （20.5±2.1） mm,female maximal hight of palate （17.9±1.6） mm,MP-FH （31.8°±2.2°） of FSHD group were greater than those of the control group.Male width of palate （34.8±1.4） mm,female width of palate （33.7±1.5） mm,male maximum bite force （451.7±39.0） N,female maximum bite force （326.7± 21.6） N,maximal opening of mouth （3.5±0.4） cm of FSHD group were less than those of the control group （P 〈 0.05）.Maxillofacial MRI showed muscle asymmetr in 11 cases of masseter and 6 cases of medial pterygoid muscle,5 cases of lateral pterygoid,and these muscle showed mild fatty infiltration mainly concentrating in the grade 0,grade 1 and grade 2.Conclusions The FSHD patients have poor oral hygiene,low masticatory function,limited mouth opening,high palate and narrow arch and different degree of malocclusion.The patients＇ masseter muscle,medial pterygoid muscles and lateral pterygoid muscles exhibit asymmetrical atrophy and fatty infiltration.