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