Zidane Fatima Ezzahra, El Alloussi Mustapha
College of Health Sciences, International Faculty of Dental Medicine, BioMed Unit, International University of Rabat, Technopolis Parc, Rocade of Rabat-Salé, Sala-Al Jadida 11100, Morocco.
Case Rep Dent. 2022 Dec 26;2022:1045327. doi: 10.1155/2022/1045327. eCollection 2022.
Oligodontia is a dental abnormality in which the patient is missing teeth. It is a hereditary disorder characterized by agenesis of more than six primary or permanent teeth, excluding the wisdom teeth. Oligodontia is often related with an abnormal size of teeth, conical shape, taurodontism, frequent enamel abnormalities, and delayed eruption. Oligodontia may be clinically isolated or associated with ectodermal dysplasia, a large group of rare diseases, and other syndromes. Dental characteristics of a six-and-a-half-year-old Moroccan boy with oligodontia and in apparent good health were described. Three syndromes associated with oligodontia have been discussed. Above all, based on the facial phenotype, Dubowitz syndrome has been retained as the most likely diagnostic hypothesis. This case could be the first reported case described in Morocco, but a thorough examination with genetic analysis must be carried out.
Oligodontia could clinically be isolated or associated with ectodermal dysplasia, a large group of rare diseases, and other syndromes.
少牙症是一种牙齿异常疾病，患者存在牙齿缺失的情况。它是一种遗传性疾病，其特征为除智齿外，超过六颗乳牙或恒牙先天性缺失。少牙症常伴有牙齿大小异常、锥形牙、牛牙症、频繁的釉质异常以及萌出延迟。少牙症在临床上可能是孤立存在的，也可能与外胚层发育不良（一大类罕见疾病）及其他综合征相关。本文描述了一名六岁半、健康状况良好的摩洛哥少牙症男孩的牙齿特征。讨论了与少牙症相关的三种综合征。最重要的是，基于面部表型，杜波维茨综合征被认为是最有可能的诊断假设。该病例可能是摩洛哥首例报道病例，但必须进行全面的基因分析检查。
少牙症在临床上可能是孤立存在的，也可能与外胚层发育不良（一大类罕见疾病）及其他综合征相关。
