Tuli A, Sachdev V, Singh A, Kumar A
Department of Pedodontics, Himachal Dental College, Mandi District, Himachal Pradesh, India.
J Indian Soc Pedod Prev Dent. 2011 Dec;29(6 Suppl 2):S83-6. doi: 10.4103/0970-4388.90750.
Oral-facial-digital (OFD) syndrome is the collective name of a group of rare inherited syndromes characterized by malformations of the face, oral cavity, hands and feet. OFD syndrome type I, also known as the Papillon-League-Psaume syndrome warrants our attention because early diagnosis from an odontologic viewpoint will minimize the sequalae of developing physical and dental abnormalities. The present article highlights the clinical as well as the radiographic findings and the treatment that was done of a 10-year-old girl child diagnosed with OFD I.
口面指综合征（OFD）是一组罕见的遗传性综合征的统称，其特征为面部、口腔、手和脚的畸形。I型口面指综合征，也称为帕皮永 - 勒格 - 普索姆综合征值得我们关注，因为从牙科学角度进行早期诊断将使身体和牙齿异常发展的后遗症最小化。本文重点介绍了一名被诊断为I型口面指综合征的10岁女童的临床、影像学表现以及所采取的治疗措施。
