Kranya Victoria Díaz-Serrano

Dental treatment in a patient with epidermolysis bullosa

Epidermolysis bullosa (EB) is a rare inherited group of genodermatoses characterized by mucocutaneous fragility and blister formation, either spontaneously or as a result of minimal mechanical trauma. The repetition of these episodes in the oral cavity leads to atrophy of the mucosa, causing microstomia, ankyloglossia, tongue denudation, and vestibule obliteration, characteristics that make dental treatment difficult. Patients with EB are at high risk for caries due to the presence of dental anomalies; they also tend to have a soft diet and difficulties with mechanical removal of the dental biofilm. This case report presents a patient diagnosed with EB and describes the difficulties faced by the clinician during dental treatment as well as the measures adopted to safely manage the patients dental care.

Development of an intraoral device for social inclusion of a physically disabled patient

The aim of this study is to describe the use of an intraoral assistive technology for a patient with idiopathic generalized muscular dystonia, presenting temporomandibular disorder and severe anterior tooth mobility and diastema. A multidisciplinary team developed an intraoral device to provide typing and painting functions, and promote relaxation of masticatory muscles without compromising the teeth and supporting tissue structures. The occlusal splint associated with the device promoted muscle relaxation and relief of the signs and symptoms of temporomandibular dysfunction, in this case with generalized muscle dystonia, allowing typing and painting with her mouth without causing tooth mobility or occlusal alteration. This intraoral device has low cost, easy adaptation and was efficient in TMD symptoms. Furthermore, the patient returned to her rehabilitation allowing performance of her duties without compromising dental structures, facilitating the social and the digital inclusion.

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 patients 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.