Manifestations of Marfan Syndrome in Maxillofacial Pathology in Children

Abstract

Marfan syndrome (SM) is a multisystem disease transmitted in an autosomal dominant manner with incomplete penetrance. Classic manifestations of SM include a triangular-shaped face with a sharp chin (bird-face syndrome), dolichostenomelia, aortic dilatation, prolapsed heart valves, lens subluxation, scoliosis, and in many cases joint hypermobility. At the same time, the dental system is a concentrate of connective tissue. But there is not enough information about the condition of orofacial structures. Purpose. To assess the oral phenotype in children and adolescents with SM. Material and methods. 28 children and adolescents with SM were examined. The median age is 12.6 years, the range of fluctuations is 10–17 years. The diagnosis of SM was made according to the Ghent criteria. Along with the general clinical and instrumental picture, dolichocephaly, retrognathia, malocclusion, “Gothic palate”, temporomandibular joint disorder, and dental caries were determined. A comparison group without signs of SM was formed according to the “case-control” principle. Results. Patients with SM, compared with children and adolescents without this syndrome, were characterized by pathology of the temporomandibular joint (p 0.001), multiple enamel defects (p=0.019) and caries (p<0.001), retrognathia (p<0.001), malocclusion (p<0.001), defects in the shape and location of teeth (p<0.001). In a number of cases, familial cases of dental pathology were noted. Conclusion. Dental pathology is often recorded in patients with SM, which can be considered as one of the manifestations of the systemic nature of the syndrome. The presence of SM in a patient requires consultation with a dentist.