Rare genetic syndromes, conditions with a global average prevalence of 40 cases/100,000 people, are associated with anatomical, physiological, and neurological anomalies that may affect different body districts, including the oral district. So far, no classification of oral abnormalities in rare genetic syndromes is present in the literature. The aim of this narrative review is to analyze literature on rare genetic syndromes affecting dento-oro-maxillofacial structures (teeth, maxillary bones, oral soft tissues, or mixed) and to propose a classification according to the detected oral abnormalities. In addition, five significant cases of rare genetic syndromes are presented. The Scale for the Assessment of Narrative Review Articles (SANRA) was followed for this review. From 674 papers obtained through PubMed search, 351 were selected. Sixty-two rare genetic syndromes involving oral manifestations were found and classified. The proposed classification aims to help the clinician to easily understand which dento-oro-maxillofacial findings might be expected in the presence of each rare genetic syndrome. This immediate framework may both help in the diagnosis of dento-oro-maxillofacial anomalies related to the underlying pathology as well as facilitate the drafting of treatment plans with the involvement of a multidisciplinary team.

Background: Poor oral health is often more prevalent in rural and resource-limited areas. Evaluating oral health status in these communities is the first step in ensuring adequate future health care for the population. The aim of this study was to assess the oral health status of children aged 6–12 years living in the indigenous Ngäbe-Buglé communities. Methods: A cross-sectional study was conducted in two rural indigenous communities of Ngäbe-Buglé on San Cristobal Island in Bocas del Toro, Panama. All children between 6 and 12 years of age and attending local schools were invited to participate, and those whose parents provided oral consent were enrolled. Dental examinations were performed by one trained dentist. To describe oral health, plaque index, DMFT/dmft (decayed, missing, and filled for permanent and primary teeth) index, and developmental defects of enamel index were recorded. Orthodontic characteristics were also evaluated, assessing the prevalence of different molar classes and the prevalence of open bite, lateral crossbite, and scissor bite. Results: A total of 106 children, representing 37.3% of the child population in the age range attending local schools, were included in this study. The mean plaque index of the entire population was 2.8 (SD 0.8). Caries lesions were more common in children living in San Cristobal (80.0%) compared to those living in Valle Escondido (78.3%), p = 0.827. The mean DMFT/dmft for the entire population was 3.3 (SD 2.9). Developmental defects of enamel were recorded in 49 children (46.2%). The majority of the population had a class I molar relationship (80.0%). Anterior open bite, lateral crossbite, and anterior crossbite were found in 10.4%, 4.7%, and 2.8% of the participants, respectively. Conclusions: The oral health of children living in Ngäbe-Buglé communities is generally poor. Oral health education programs for children and adults might play a crucial role in improving the oral health status of the Ngäbe-Buglé population. In addition, the implementation of preventative measures, such as water fluoridation as well as regular toothbrushing with fluoridated toothpaste and more accessible dental care, will be essential in improving future generations’ oral health.