Wolf-Hirschhorn syndrome (WHS) - literature review on the features of the syndrome.

[wolf-hirschhorn syndrome]

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 MSX 1 -gene impairment .