Oral Findings and Dental Treatment in a Child with Williams-Beuren Syndrome

Williams-Beuren syndrome (WBS), also known as Williams syndrome, is a rare congenital disorder involving cardiovascular problems, mental retardation, distinctive facial features and tooth anomalies. It is caused by the submicroscopic deletion of 1.5 to 1.8 Mb on chromosome 7q11.23. This paper reports the dental care to a 7-year-old child with WBS syndrome. The interview also revealed visual impairment, sensorineural hearing loss, hyperacusis, photophobia and hoarse voice. The intraoral clinical examination showed anterior open bite, tongue thrusting, excessive interdental spacing, enamel hypomineralization of the incisors, hypoplasia and caries lesions. The dental treatment included: modulating sessions to control aversion to noises, the photophobia, and the dental fear and anxiety because of his reduced visual acuity; oral hygiene instructions, dietary and daily use of a 0.05% sodium fluoride mouthwash; the permanent mandibular left first molar was treated endodontically, and maxillary and mandibular first molars were restored with amalgam. Due to the patient's heart defect, a prophylactic antibiotic regimen was prescribed prior to the dental procedures. This patient has been followed up for 4 years and this case report underscores the importance of early dental evaluation and counseling for parents of WBS patients.

A Síndrome de Williams-Beuren (SWB), também conhecida como síndrome de Williams, consiste em uma desordem congêntica rara a qual apresenta problemas cardiovasculares, retardo mental, alterações faciais e anomalias dentárias. É causada pela microdeleção de 1,5 a 1,8 Mb no cromossomo 7q11.23. Este trabalho relata o tratamento odontológico de uma criança de 7 anos com a síndrome. Durante a anamnese constatou-se deficiência visual, perda auditiva neurossensorial, hiperacusia, fotofobia e voz rouca. O exame clínico intra-oral revelou mordida aberta anterior, deglutição atípica, espaçamento interdental excessivo, hipomineralização dos incisivos, hipoplasia e lesões de cárie. O tratamento dentário incluiu: sessões de condicionamento comportamental afim de controlar a aversão a ruídos, a fotofobia e o medo e a ansiedade frente ao tratamento odontológico, provocadas principalmente por sua reduzida acuidade visual; instruções de higiene oral, dieta e uso diário de bochechos de fluoreto de sódio a 0,05%; endodontia do primeiro molar permanente inferior esquerdo e restaurações de amálgama nos primeiros molares superiores e inferiores. Devido ao defeito cardíaco do paciente, antibioticoterapia profilática foi realizada antes dos atendimentos odontológicos. Este paciente está em acompanhamento há 4 anos e este relato ressalta a importância da avaliação odontológica precoce e do aconselhamento aos pais dos pacientes com SWB.

Reabilitação oral de paciente portador de displasia ectodérmica anidrótica / Oral rehabilitation of a patient with anhidrotic ectodermal dysplasia

O presente trabalho teve como objetivo relatar um caso de reabilitação bucal de um paciente portador de displasia ectodérmica, através de próteses totais fixas aparafusadas sustentadas por implantes zigomáticos e convencionais. Os aspectos clínicos e radiográficos serão descritos juntamente com a sequência de tratamento realizada até a finalização do caso. Esse tipo de tratamento tem apresentado resultados satisfatórios.

This case reports on the use of total fixed implant-supported screwed prostheses with zygomatic and conventional implants in the treatment of a completely edentulous patient with anihidrotic ectodermal dysplasia. Clinical and radiographic aspects are demonstrated during treatment sequence. This kind of rehabilitation has presented satisfactory results.

Carillas de composite como alternativa a carillas cerámicas en el tratamiento de anomalías dentarias: reporte de un caso / Composite veneers as an alternative to ceramic veneers in the treatment of dental anomalies: case report

Las anomalías dentarias de forma y tamaño son alteraciones anatómicas frecuentes que comprometen la estética del sector anterior. Existen distintos abordajes para tratar esta alteración morfológica. El procedimiento con mayor demanda es la restauración con carillas estéticas, cuyos materiales de elección son la cerámica en el caso de restauraciones indirectas y resinas compuestas en el caso de las restauraciones directas. La cerámica conserva propiedades físicas y estéticas que la convierten en la primera opción; sin embargo, las resinas compuestas se convirtieron en una alternativa confiable. Se presenta a consulta paciente con inconformidad estética. Al examen intraoral se observan órganos dentarios 12 y 22 en forma de espiga, se observan diastemas en el maxilar superior, sector anterior. Se propuso como plan de tratamiento la restauración estética del sector anterosuperior con carillas en resina compuesta empleando preformas plásticas. El objetivo del presente caso es la descripción del tratamiento restaurador estético realizado con resinas compuestas, empleando cofias preformadas.

The tooth anomalies of size and shape are common anatomical alterations involving the aesthetics of the anterior sector. There are different approaches to address this morphological alteration. The procedure most in demand is restoration with veneers, with the materials of choice in this case being indirect ceramic restorations and direct composite resin restorations. The ceramic preserves physical and aesthetic properties that make it the first choice, but composite resins are becoming a reliable alternative. The case of a patient is presented who consulted with aesthetic anomalies. In the intraoral examination 2 pin-shaped dental organs 12 and 22 are observed, as well as diastemas in the maxilla, in the anterior sector. A proposed treatment plan was cosmetic restoration of anterior-superior sector with composite resin veneers using plastic preforms. The aim of the present case is to describe the aesthetic restorative treatment performed using composite resin copings.

An alternative treatment approach for a supernumerary tooth fused to a Mandibular second molar: a case report.

Since abnormal tooth morphology can predispose to caries and periodontal disease, careful management of fused teeth is essential. This paper reports a rare case of a fused molar and supernumerary tooth and describes its management. Caries was removed from the tooth under local anesthesia. The pulp chamber of the supernumerary tooth was exposed and the root canal of the supernumerary tooth was prepared using the step back technique and copious irrigation with H2O2. Obturation using the lateral condensation technique with gutta-percha and AH26 sealer was subsequently performed and final restoration was accomplished with composite resin.