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Epidermolysis bullosa hereditaria (EBH) is a group of rare diseases characterized by a cutaneous-mucosal fragility with the formation of bullae, including the oral mucosa. Therapeutic choices, especially prosthetic rehabilitation, must anticipate the worsening of the limitation of oral opening while respecting the functional and aesthetic expectations of the patients. This review on the oral prosthetic rehabilitation of patients with epidermolysis bullosa hereditaria (EBH) to study the level of evidence and quality of the presented available articles and establish clinical recommendations for the prosthetic management of these patients. MATERIALS AND METHODS: An electronic search was done in July 2022 in five databases following PICOTS elements. The quality of the reports was established using the modified Pierson, Bradford Hills, and Ottawa Newcastle scale. RESULTS: Data extracted from 19 case reports for protocolized analysis corresponded to 64 patients and 80 dental prostheses with almost 9 out of 10 patients being completely edentulous. The distribution of EBH types was 84% dystrophic, 10.5% junctional, and 5.5% simplex. The difficulties encountered by the authors synthetized in this review characteristically reflected those most likely encountered in dental practice. Most rehabilitations were implant-supported prostheses (85%) followed by removable dentures (10%) and finally dental-supported rehabilitations (5%). Fixed full-arch implant-supported prostheses represented 76.4% of implant-supported prostheses and this last prosthetic solution described showed the highest scientific quality. CONCLUSIONS: In an individualized approach to treatment, we recommend that in cases of total edentulism, fixed full arch implant-supported prostheses are the most appropriate, as they allow the best computer-aided planning, design, manufacture, and fitting of the prosthesis in such a complex clinical context.
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Amelogenesis imperfecta, a rare disease, represents inherited, congenital defects that primarily affect enamel with esthetic and functional impairment affecting everyday life. We present oral rehabilitation of a mother and her son, respectively, suffering from an hypoplastic and an hypocalcified form of AI.
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INTRODUCTION: Orofacial pain diagnosis is a difficult process. This article reports the case of a 38 y.o. patient experiencing severe acute facial pain of dental origin initially diagnosed as non odontogenic. CASE REPORT: The patient consulted at the dental emergency department for severe and frequent neuropathic-like paroxysmal pain attacks located in the anterior right maxilla. The pain fulfilled the ICHD3 criteria for Trigeminal Neuralgia (TN) of the right maxillary branch (V2) and responses to sensitivity tests were ambiguous due to severe allodynia. After one week of carbamazepine treatment (600mg/day), the patient was pain free except for a slight mechanical allodynia on tooth #12. Sensitivity tests revealed pulp necrosis and tomodensitometry revealed a rare developmental abnormality, dens invaginatus. Root canal treatment was performed. RESULTS: No recurrence of the pain was noted after 18 months without any medication. CONCLUSIONS: Inflammatory pulpal pain may mimic TN, misleading experienced clinicians. Key words:Dens in dente, neuropathic pain, necrosis, diagnosis, atypical pain, endodontics.
