Medical considerations in the dental treatment of patients with Williams-Beuren syndrome - report of four clinical cases

Abstract

Williams-Beuren syndrome (WBS) is a rare genetic disorder characterized by peculiar facies, eyes, dental, cardiovascular, renal, and skeletal abnormalities, mental retardation, friendly and loquacious personality, and occasionally hypercalcemia in infancy. The orofacial aspects and especially the dental management of these patients have received little attention in the literature. The aim of this manuscript is to describe four cases of patients with WBS attended at Special Care Dentistry Center, School of Dentistry, University of São Paulo, with emphasis on the oral findings and systemic conditions of dental interest. The clinical management of patients with WBS is discussed. We conclude that congenital heart defects and behavioral changes are the aspects that most interfere on dental treatment

Oral characteristics and medical considerations in the dental treatment of individuals with Williams syndrome

“…Tooth abnormalities, such as generalized diastemas, hypodontia, microdontia, short dental roots, fused teeth, enamel hypoplasia and altered morphology, including malocclusion (Angle class II and III) were already described in case reports and small case series. Relatively common systemic comorbidities include, hypertension, risk of sudden death, joint limitation, and premature gray hair …”

“…Relatively common systemic comorbidities include, hypertension, risk of sudden death, joint limitation, and premature gray hair. [9][10][11] The dentist should be aware of the medical condition, as well as recognize and diagnose the dental disorders for an efficient dental management. The aim of this study was to evaluate oral characteristics and medical considerations related to dental treatment of subjects with Williams syndrome (WS).…”

Restorative treatment in patient with Williams Syndrome: case report

“…The Williams syndrome, also known as Williams-Beuren Syndrome (SWB), was first described in 1961 by a cardiologist named John Williams and colleagues, who evaluated four children with similar faces, supravalvular aortic stenosis, cognitive impairment, reading and writing difficulties, and affinity for music. In the following year, Beuren and his collaborators added strabismus, pulmonary artery stenosis and dental abnormalities as characteristics of the syndrome [1][2][3][4][5][6].…”

“…Individuals with SWB have a peculiar face, described as "elf face", due to the accumulation of periorbital fat, small nose with low nasal bridge, anteverted nostrils and long filter, thick lips, and little development of the jaw. Furthermore, dental anomalies, such as generalized diastemas, hypodontia, microdontia, short roots, enamel hypoplasia, pressure from the tongue on the teeth, and altered morphology including malocclusions (Angle Class II and III) are common [1,2,4,5]. In addition to dental cleaning, a four-month recall interval for dental follow-up has been recommended to prevent the risk of caries, periodontal and endodontic infections in these patients [2,4,6].…”

“…Compared with the general population, the risk of sudden cardiac death is 25 to 100 times higher in patients with SWB [1,3]. Therefore, the dental surgeon must know the precautions and protocols to be followed when treating patients with SWB [3,7,8].…”