Vierucci S, Baccetti T, Tollaro I
Department of Orthodontics, University of Florence, Italy.
J Clin Pediatr Dent. 1994 Summer;18(4):291-7.
A more detailed knowledge of dental and craniofacial features in hypohidrotic ectodermal dysplasia (HED) during childhood is needed in order to include these characteristics among diagnostic criteria. The present study comprised 5 HED children, 4 males and 1 female, during the primary dentition phase. Clinical and radiological dental findings consisted of multiple congenitally missing primary teeth, conoid primary incisors, moderate to severe taurodontic second primary molars. Supernumerary cusps and diastema were found as well. A pattern of symmetry was assessed for hypodontia in the primary dentition. The cephalometric study compared the HED sample to a matched non-syndrome sample and revealed abnormally short maxillary depth (p < 0.05), strongly reduced lower facial height (p < 0.01) and a reduction in facial soft tissue thickness (p < 0.05 - p < 0.01) in HED children. The importance of an early diagnosis and treatment of HED dento-facial malformations so as to improve esthetics and function is stressed.
为了将这些特征纳入诊断标准，需要更详细地了解儿童期少汗型外胚层发育不良（HED）的牙齿和颅面特征。本研究纳入了5名处于乳牙列期的HED儿童，其中4名男性，1名女性。临床和放射学牙齿检查结果包括多颗先天性缺失乳牙、锥形乳牙切牙、中度至重度牛牙样第二乳磨牙。还发现了额外牙尖和牙间隙。评估了乳牙列中牙缺失的对称性模式。头影测量研究将HED样本与匹配的非综合征样本进行比较，结果显示HED儿童上颌深度异常短（p < 0.05）、面下高度显著降低（p < 0.01）以及面部软组织厚度减小（p < 0.05 - p < 0.01）。强调了早期诊断和治疗HED牙颌面畸形以改善美观和功能的重要性。
