Williams-Beuren Syndrome: What Orthodontists Need to Know

Abstract

Williams-Beuren syndrome is a rare disease with manifestations such as cardiovascular changes, distinct facial features, mental retardation, and learning disabilities. Oral manifestations are not commonly described and can often be misdiagnosed. This report describes the case of a male patient diagnosed with Williams-Beuren syndrome presenting classic clinical features that affect the face as a convex profile, with maxillary protrusion and mandibular retrusion, a discreetly acute nasolabial angle, passive labial sealing, and an open mandibular angle characteristic of Class II skeletal pattern. In addition, the patient has oral manifestations such as the absence of some dental elements, a Class II of Angle 1st division, dental cross bite, and atresic arches. The periodontal condition presents with generalized gingivitis. Knowledge about the possible manifestations of Williams-Beuren syndrome is important to improve the ability of orthodontists to better serve these patients.

Correlações entre o fenótipo na síndrome de Williams e os genes deletados / Correlations between the phenotype in Williams syndrome and the deleted genes

“…São observados, também, desvios maxilo-mandibulares, como inclinação anterior da maxila, deficiência anterior do terço médio da face, aumento do plano mandibular com padrão facial longo e a mordida aberta associada à macroglossia. Dentre as anomalias dentárias, incluem-se: hipoplasia do esmalte, simplificação anatômica dos incisivos, molares inferiores em forma de broto, hipodontia e microdontia (JONES, 1998;RIBEIRO et al, 2018).…”