Clinical Features And MRI Manifestations Of Oral And Maxillofacial Region In Facioscapulohumeral Muscular Dystrophy

Objective: To provide theoretical basis for changing FSHD patients ' face-shape and improving masticatory function,we study the clinical features of facioscapulohumeral muscular dystrophy and radiologic characteristics.Methods: Choose 12 patients who were diagnosed as facioscapulohumeral muscular dystrophy according to the diagnostic criteria and pathological biopsy,and 20 volunteers who were healthy.To gather relevant medical information,our study designed a unified questionnaire and collected data of all the patients and volunteers.And we made oral health examinations for them to record DMFT,CI-S,occlusal relationship and maximal opening of mouth.We also checked their maximum bite force.To measure the maximal hight of palate and the width of palate,patients and volunteers were made impressions.Then we took lateral cephalometric radiographs for them to measure MP-FH.At last,patients and volunteers were taken oral and maxillofacial region MRI to observe the affected situation of masseter muscle,medial pterygoid muscle and lateral pterygoid muscle.The data was analyzed by T-test or Wilcoxon signed ranks test between two independent samples in this study.Results: In study group,there was no significant gender difference of FSHD.The average age of treatment was 27.5±8.10,and the average age of onset was 15.67±7.54.9 patients(75%)liked to eat soft foods;4 patients(33.3%)were difficult to close eyes;8 patients(66.7%)were difficult to cheek-bulging;10 patients(83.3%)had pouty lips;9 patients(75%)had mesio-malocclusion.DMFT(4±2.30),CI-S(5.83±2.08),male maximal hight of palate(20.51±2.05mm),female maximal hight of palate(17.91±1.59mm),MP-FH(31.79±2.18°)of study group were greater than the control group.Male width of palate(34.79±1.39mm),female width of palate(33.71±1.45mm),male maximum bite force(451.69±39.01N),female maximum bite force(326.71±21.60N),maximal opening of mouth(3.50±0.35cm)of study group were less than the control group.And these had significant difference compared with control group(P<0.05).In MRI examination,there were 11 patients' masseter muscles,6 patients' medial pterygoid muscles and 5 patients ' lateral pterygoid muscles to atrophy asymmetrically.These three pieces of muscular fatty infiltration was slighter,focused on Grade 0,1 and 2.Conclusion:1 It is difficult for the patients of facioscapulohumeral muscular dystrophy to cheek-bulging.They have pouty lips and high palatal vault.Poor oral hygiene is another feature of them.Their masticatory function becomes lower,maximal opening of mouth becomes smaller,and mandibular angle becomes flat.The patients usually also have mesio-malocclusion.2 The shape of facioscapulohumeral muscular dystrophy patients' masseter muscle,medial pterygoid muscle and lateral pterygoid muscle are changed asymmetrically,and these muscles have fatty infiltration in different degrees.3 By early intervention,a timely correct orthodontic treatment,and aerobic exercise such as munch,FSHD patients may change face-shape and improve masticatory function.