Oral features in five adult patients with Wolf-Hirschhorn syndrome.

[wolf-hirschhorn syndrome]

The Wolf-Hirschhorn syndrome (WHS ) is a rare genetic condition presenting with severe mental disability , growth retardation , muscular hypotonia , seizures , craniofacial abnormalities and defects in the cardiovascular , genitourinary and digestive apparata . To date , few data about oral status of afflicted patients were reported , and this syndrome is still unfamiliar among dental and maxillofacial professionals . Aim of the present case series was to provide oral data from five patients with WHS (3 women and 2 men ) aged 19 -41 years . All patients entered in a long -stay Institution with an inner dental service in 1998 and underwent regular dental examination and hygiene treatment . Neither tooth agenesis or dental size and shape anomalies were found in the analyzed subjects except for one man showing multiple cone-shaped teeth . At the beginning , bad dental and periodontal conditions with gingival signs and recurrent mucosal inflammation were found in all patients . After motivation of their tutors , dental and periodontal parameters were recorded during periodic assessments and a large decrease in the gingival index was found over time . These data could aware dentists about the therapeutic modalities to improve oral health of WHS patients .