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 patient's dental care.
This article reports the case of a Brazilian child diagnosed with Kabuki make-up syndrome (KMS), addressing the clinical features observed, with emphasis on the disease-specific oral and craniofacial manifestations. The patient had the distinctive KMS craniofacial appearance, mild delayed mental development, fingers with prominent fingertip pads and visual deficit. The dental findings included fusion of the left mandibular incisors (central and lateral), gemination of the right mandibular central incisor and congenital agenesis of the right mandibular lateral incisor, in the primary dentition, as well as absence of both permanent mandibular lateral incisors. Fusion and gemination have not been previously referred to as typical dental features in KMS. The detection of unique dental findings, such as missing teeth and dental anomalies of form in the primary dentition by means of clinical and radiographic examinations, might consist of a helpful diagnosis parameter in identifying children who may have milder forms of Kabuki syndrome.
This article reports the cases of two young children aged 4 and 5 years, in whom biological restorations using tooth fragments were placed in primary molars with severely damaged crowns due to extensive carious lesions. After radiographic and clinical evaluation, tooth fragments obtained from extracted teeth in stock were autoclaved, adjusted to the prepared cavity and bonded to the remaining tooth structure with either adhesive system (Case 1) or dual-cure resin-based cement (Case 2) over a calcium hydroxide layer and a glass ionomer cement base. Occlusal adjustment was performed and topical sodium fluoride was applied to tooth surface. Periodical clinical and radiographic controls were carried out and the restored teeth were followed up for 4 and 3 years, respectively, until exfoliation. In these two reports, the technical aspects are described and the benefits and disadvantages of biological restorations as an alternative treatment for rehabilitation of severely destroyed primary molars are discussed.
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