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BMC Oral Health Feb 2024Moebius syndrome (MS) is a rare, non-progressive, neuromuscular, congenic disease involving the oral maxillofacial region. The present study aimed to describe the oral...
BACKGROUND
Moebius syndrome (MS) is a rare, non-progressive, neuromuscular, congenic disease involving the oral maxillofacial region. The present study aimed to describe the oral and extraoral findings in MS patients and their comprehensive dental management.
METHODS
A digital search was carried out in PubMed/MEDLINE, Scopus, Web of Science, and Google Scholar, restricted to articles in English from Jan 01, 2000, to Apr 02, 2023, following PRISMA guidelines. The methodological quality of the studies was evaluated following the JBI guidelines. Qualitative analysis was carried out on the overall result, extraoral and intraoral manifestations, considering dental management as appropriate.
RESULTS
Twenty-three studies were included, and a total of 124 cases of patients with MS were analyzed. The 82% of patients with MS were younger than 15 years of age. The most frequent extraoral manifestations were blinking and visual problems (78,22%), malformations of the upper and lower limbs (58,22%), bilateral facial paralysis (12,90%), lack of facial expression (12.09%), and unilateral facial paralysis (6,45%). On the other hand, the most frequent oral manifestations were tongue deformities (78,22%), micrognathia (37,90%), labial incompetence (36,29%), cleft palate (22,87%), gothic palate (16,12%), microstomia (15,32%), anterior open bite (15,32%), dental caries (8,87%), and periodontal disease (8,06%). The majority of MS patients were treated by pediatric dentistry (60,86%), using a surgical approach (56,52%), and orthodontic and orthopedic maxillary (43,47%) followed by restorative (39,13%), and periodontal treatments (21,73%).
CONCLUSIONS
This systematic review demonstrates that patients with MS present a wide variety of oral and extraoral manifestations, for which dental treatments are planned and tailored to each patient in accordance with oral manifestations. These treatments encompass problem resolution and oral health maintenance, incorporating recent techniques in managing and treating patients with MS.
Topics: Child; Humans; Mobius Syndrome; Facial Paralysis; Dental Caries; Cleft Palate; Dental Care
PubMed: 38321523
DOI: 10.1186/s12903-024-03968-6 -
The International Journal of... Dec 2023Epidermolysis bullosa hereditaria (EBH) is a group of rare diseases characterized by a cutaneous-mucosal fragility with the formation of bullae, including the oral...
UNLABELLED
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.
PubMed: 38096448
DOI: 10.11607/ijp.8791