Papaefthymiou Petros, Kekou Kyriaki, Özdemir Fulya
Department of Orthodontics, Marmara University, Faculty of Dentistry, İstanbul, Turkey.
Department of Medical Genetics, National and Kapodistrian University of Athens, School of Medicine, Athens, Greece.
Turk J Orthod. 2022 Mar;35(1):67-73. doi: 10.5152/TurkJOrthod.2021.21021.
The aim of this review is to evaluate the developmental, functional, and morphological aspects of the craniofacial complex in patients with myotonic dystrophy type 1 (DM1), Facioscapulohumeral muscular dystrophy (FSHD), and Duchenne muscular dystrophy (DMD). The degree of disease onset and severity varied from patient to patient, and most parameters indicated a greater degree of deterioration in older patients. It was found that all the muscular dystrophies studied showed altered craniofacial morphology, with malocclusion as the most consistent clinical characteristic. Particularly DM1 patients, who are the most studied, showed significant vertical aberration and post-normal occlusion. DMD patients are reported mainly with altered dental arch dimensions which influence functional capacities. Data for FSHD patients are very limited, but facial asymmetry and muscular weakness appear to be the most prominent findings. Patients with muscular dystrophies present deviations in growth and development as well as in orofacial morphology. Increased prevalence of malocclusions, of both skeletal and dental origins, characterize patients with muscular dystrophies. Different dentofacial characteristics are reported among patients with different types of muscular dystrophies. Further research is needed to clarify the orofacial phenotypic expression of muscular dystrophies.
本综述的目的是评估1型强直性肌营养不良(DM1)、面肩肱型肌营养不良(FSHD)和杜氏肌营养不良(DMD)患者颅面复合体的发育、功能和形态学方面。疾病的发病程度和严重程度因患者而异,大多数参数表明老年患者的恶化程度更大。研究发现,所有研究的肌营养不良症均显示颅面形态改变,错牙合是最一致的临床特征。特别是研究最多的DM1患者,表现出明显的垂直偏差和后正常咬合。DMD患者主要报告牙弓尺寸改变,这会影响功能能力。FSHD患者的数据非常有限,但面部不对称和肌肉无力似乎是最突出的发现。肌营养不良症患者在生长发育以及口面部形态方面存在偏差。骨骼和牙齿来源的错牙合患病率增加是肌营养不良症患者的特征。不同类型肌营养不良症患者报告了不同的牙颌面特征。需要进一步研究以阐明肌营养不良症的口面部表型表达。