Mass E, Brin I, Belostoky L, Maayan C, Gadoth N
Department of Pediatric Dentistry, The Maurice and Gabriela Goldschleger School of Dental Medicine, Tel Aviv University, Israel.
Cleft Palate Craniofac J. 1998 Mar;35(2):120-6. doi: 10.1597/1545-1569_1998_035_0120_aceocm_2.3.co_2.
The purpose of this study was to delineate the craniofacial and dentoalveolar morphology of patients with familial dysautonomia (FD) in order to contribute to the understanding of the association between progressive sensory and autonomic neuropathy and the characteristic appearance of the dysautonomic face.
The study group comprised 32 patients with FD (15 females and 17 males; mean age 10.8 years, SD 3.5 years, range 5.8-19.8 years).
Lateral cephalograms from each patient were traced twice. The means of the two measurements were compared with homologous cephalometric normal values of ethnic-specific and classical norms from the literature.
In some parameters, the craniofacial morphology of the FD group was significantly different from the classical norms. There was a pronounced retrognathism in the mandible and a steep mandibular plane angle. The skeletal features of FD patients more closely resembled those of their ethnic group, although they were more retrognathic, and the mandibular growth axis was more horizontal. The incisors of these patients were more retropositioned and retroclined than were those of their healthy counterparts.
The results suggest an insufficiency of the expected dentoalveolar compensatory mechanism that usually helps to bridge skeletal discrepancies. It is postulated that the neuropathy is probably the important factor in the lack of this compensatory mechanism.
本研究旨在描绘家族性自主神经功能异常（FD）患者的颅面和牙槽形态，以促进对进行性感觉和自主神经病变与自主神经功能异常面容特征性表现之间关联的理解。
研究组包括32例FD患者（15名女性和17名男性；平均年龄10.8岁，标准差3.5岁，范围5.8 - 19.8岁）。
对每位患者的头颅侧位片进行两次描记。将两次测量的均值与文献中特定种族和经典标准的同源头影测量正常值进行比较。
在某些参数方面，FD组的颅面形态与经典标准有显著差异。下颌存在明显的后缩，下颌平面角陡峭。FD患者的骨骼特征与其种族群体更为相似，尽管他们的后缩更明显，且下颌生长轴更水平。这些患者的切牙比健康对照者的切牙更向后移位和倾斜。
结果提示通常有助于弥补骨骼差异的预期牙槽代偿机制不足。据推测，神经病变可能是缺乏这种代偿机制的重要因素。
