Paradowska-Stolarz Anna M
Department of Dentofacial Anomalies, Department of Orthodontics, Wroclaw Medical University, Poland.
Adv Clin Exp Med. 2014 May-Jun;23(3):485-9. doi: 10.17219/acem/24111.
Wolf-Hirschhorn syndrome (WHS) is a congenital disorder associated with 4 chromosome microdeletion. The patients suffer from various deformities. Among them, mental and growth retardation, even in the fetus, are observed. Most of the characteristics concern facial features. The "Greek warrior helmet appearance" is the most characteristic feature and refers to the facial view with prominent glabella, high arched eyebrow, broad nasal bridge and hypertelorism. Another characteristic feature is microcephalia with micrognathia. The features are more pronounced in infants. Clefts of lip and/or palate are observed in almost half of the cases. The characteristic thing is that the more genetic material is missing, the more pronounced are the dimorphic features of the syndrome. Mostly, the dental status does not differ much from that of the healthy individuals. It had been proven though that WHS-patients are more prone to anomalies in dental structures. Cone-shaped and taurodontic teeth were observed. Multiple tooth agenesis (mainly at premolars and molars) with over-retained deciduous dentition might be associated with MSX1-gene impairment.
沃尔夫-赫希霍恩综合征（WHS）是一种与4号染色体微缺失相关的先天性疾病。患者患有各种畸形。其中，即使在胎儿期也可观察到智力和生长发育迟缓。大多数特征涉及面部特征。“希腊战士头盔样外观”是最具特征性的表现，指的是面部外观具有突出的眉间、高拱形眉毛、宽鼻梁和眼距过宽。另一个特征是小头畸形伴小颌畸形。这些特征在婴儿中更为明显。几乎一半的病例中可观察到唇裂和/或腭裂。其特点是缺失的遗传物质越多，该综合征的双形特征就越明显。大多数情况下，牙齿状况与健康个体差异不大。不过，已证实WHS患者更容易出现牙齿结构异常。观察到锥形牙和牛牙症。多生牙缺失（主要在前磨牙和磨牙）伴乳牙滞留可能与MSX1基因受损有关。
