Oral manifestations: Patients typically present with prominent lips and a wide intercommissural distance, with an open mouth. Intraorally, the buccal mucosa is often thickened and prominent labial frenula are also occasionally seen. The maxillary arch is often broad and microdontia, hypodontia and enamel hypoplasia are commonly seen. The teeth also have small, thin roots, The maxillary deciduous 2nd molars and mandibular permanent 1st molars often erupt as bud-shaped teeth.

Radiographic features: Small, hypoplastic teeth with slender roots.

Occasional osteosclerotic changes of the lamina dura, mainly in the premolar-molar regions.

Dens invaginatus may be seen.

Dental treatment considerations: Due to the enamel hypoplasia, these patients may have an increased risk for caries. Therefore regular dental hygiene visits and good home care are required. Behaviorally, these patients are frequently pleasant and easy to manage.

Dentists wishing more information about patients with Williams syndrome should check www.wsf.org, the prize-winning Comprehensive Williams Syndrome Page.

This dental information was adapted from The International Association for Disability and Oral Health.