Original Article
Features of facioscapulohumeral muscular dystrophy in oral and maxillofacial region and MRI analysis of facial muscles
Yonghong Liu, Yuexiao Ma, Jing Hu, Guodong Gao, Yankai Wu, Zhiyong Zhang
Published 2016-12-09
Cite as Chin J Stomatol, 2016, 51(12): 739-745. DOI: 10.3760/cma.j.issn.1002-0098.2016.12.008
Abstract
ObjectiveTo investigate the manifestation of facioscapulohumeral muscular dystrophy (FSHD) in oral and maxillofacial region.
MethodsA 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.
ResultsThere 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.
ConclusionsThe 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.
Key words:
Muscular dystrophy, facioscapulohumeral; Magnetic resonance imaging; Masticatory muscle
Contributor Information
Yonghong Liu
Department of Oral Medicine, The Second Hospital of Hebei Medical University, Shijiazhuang 050000, China
Yuexiao Ma
Department of Oral Medicine, The Second Hospital of Hebei Medical University, Shijiazhuang 050000, China
Jing Hu
Department of Neuromuscular Disease, The Third Hospital of Hebei Medical University, Shijiazhuang 050051, China
Guodong Gao
Department of Radiology, The Second Hospital of Hebei Medical University, Shijiazhuang 050000, China
Yankai Wu
Department of Radiology, The Second Hospital of Hebei Medical University, Shijiazhuang 050000, China
Zhiyong Zhang
Department of Oral Medicine, The Second Hospital of Hebei Medical University, Shijiazhuang 050000, China
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。
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
力[（451.7±39.0）N]、女性最大
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。
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。
