Tsang Annetta K L, Taverne Aart, Holcombe Trevor
The University of Queensland, Brisbane, Queensland, Australia.
Spec Care Dentist. 2013 Sep-Oct;33(5):248-54. doi: 10.1111/scd.12018. Epub 2013 Feb 28.
Marfan syndrome (MFS) is a connective tissue disorder of variable inheritance that affects multiple organ systems. Cardiovascular, ocular, and skeletal abnormalities are cardinal features of the syndrome. Orofacially, MFS patients typically exhibit skeletal class II malocclusion, dolichofacial growth pattern, mandibular retrognathia, malar hypoplasia, high arched palate, dental crowding, and root anomalies. The purpose of this paper is to provide a review of the literature, as well as describe an 11-year-old female with MFS diagnosed at the age of 10.5 years. This report emphasizes the orofacial findings in MFS and highlights particularities of dental treatment when social deficits and intellectual disabilities are also implicated.
马凡综合征(MFS)是一种遗传性可变的结缔组织疾病,会影响多个器官系统。心血管、眼部和骨骼异常是该综合征的主要特征。在口面部,马凡综合征患者通常表现出安氏II类错牙合、长面型生长模式、下颌后缩、颧骨发育不全、高拱腭、牙列拥挤和牙根异常。本文旨在对相关文献进行综述,并描述一名11岁女性,她在10.5岁时被诊断为马凡综合征。本报告强调了马凡综合征的口面部表现,并突出了在存在社交缺陷和智力障碍情况下牙科治疗的特殊性。