Fontinha Clara, Engvall Monica, Sjögreen Lotta, Kiliaridis Stavros
Department of Orthodontics, University of Geneva, Geneva, Switzerl.
Institute of Odontology, University of Gothenburg, Gothenburg, Sweden.
Eur J Orthod. 2018 Sep 28;40(5):544-548. doi: 10.1093/ejo/cjx104.
BACKGROUND/OBJECTIVES: This study investigated the craniofacial morphology of young individuals with congenital or childhood onset myotonic dystrophy type 1 (DM1) compared to healthy subjects. The study also followed growth changes in their facial morphology over a 5-year period.
MATERIALS/METHODS: Lateral cephalograms of the 26 subjects (young patients with DM1 from west and south Sweden) were taken at baseline and after a 5-year period. These radiographs were compared with normal standards based on healthy individuals from the Michigan Growth Study, according to their age and sex, using paired t-tests (P < 0.05).
On examination of initial radiographic measurements, patients with DM1 showed, in the sagittal plane, larger ANB and smaller SNPg angles. Analysis of the vertical plane showed the mandibular plane angle (ML-NSL) and the intermaxillary angle (ML-NL) to be larger. During the 5-year follow-up period, the intermaxillary angle (ML-NL) remained the same in the group with DM1 whereas this angle decreased in healthy individuals.
For ethical reasons, historical cephalometric norms were used to compare the growth and the craniofacial morphology of patients with DM1.
CONCLUSIONS/IMPLICATIONS: Young patients with DM1 had already from the beginning a more retrognathic profile and hyperdivergent skeletal aberration with a steep mandibular plane and large intermaxillary angle when compared with healthy individuals. The intermaxillary angle did not decrease during the observation period, contrary to what was observed in healthy individuals.
背景/目的：本研究调查了先天性或儿童期发病的1型强直性肌营养不良症（DM1）青年个体与健康受试者相比的颅面形态。该研究还追踪了他们面部形态在5年期间的生长变化。
材料/方法：对26名受试者（来自瑞典西部和南部的DM1青年患者）在基线时和5年后拍摄头颅侧位片。根据密歇根生长研究中健康个体的年龄和性别，将这些X线片与正常标准进行比较，采用配对t检验（P<0.05）。
在对初始X线测量结果的检查中，DM1患者在矢状面显示出更大的ANB角和更小的SNPg角。垂直面分析显示下颌平面角（ML-NSL）和颌间角（ML-NL）更大。在5年的随访期内，DM1组的颌间角（ML-NL）保持不变，而健康个体的这个角度减小。
出于伦理原因，使用历史头影测量标准来比较DM1患者的生长和颅面形态。
结论/启示：与健康个体相比，DM1青年患者从一开始就有更后缩的侧貌和骨骼高度离散异常，下颌平面陡峭且颌间角大。与健康个体观察到的情况相反，在观察期内颌间角没有减小。
