Marfan's Syndrome: General Informations and Odontologic Manifestations

Marfan syndrome (MS) is a multisystemic, inherited disorder of the connective tissue. It is caused by mutations in the gene FBN1, encoding fibrillin, a major component of microfibrils. Thus, this disturbance causes effects in many systems composed of connective tissue such as skeletal and cardiovacular mainly. The skeletal phenotype of patients with Marfan syndrome is characterized by tall stature, joint hypermobility, ligamentous laxity, protrusion acetabular arms and long legs, disproportionate fingers (arachnodactyly) dolichocephaly, high palate, scoliosis, protrusion (pectus carinatum) or depression ( pectus excavatum) of the sternum; decreasing the relationship between the upper and lower third of the skeleton and espondilolistesis. The main cardiovascular manifestations are mitral valve prolapse and aortic dilation, and these increases the risk of dissection and rupture of the aorta and aortic regurgitation. Orofacial characteristics are frequently described and used in the diagnosis of the syndrome. The orofacial defects more prevalent are constriction of the maxilla and high palate, with concomitant dental crowding, posterior cross bite and open bite. The cranium and the face is present Benthic, usually the dolicocephalic and type II malocclusion is often found. The maxillary constriction can influence the increase in nasal resistance, which can cause severe obstructive sleep apnea, which has a high prevalence in These Patients who are respirators mouth often. The looseness of the capsular ligaments and muscles can hyperextensibility contribute to dysfunction and habitual movements or subluxation of the temporomandibular joint. Dental treatment of these patients is mainly focused on the resolution of orthopedic disorders, which include features like dolichocephaly, upper deep palate and occurrence of obstructive apnea. It is important to know the systemic features that accompany the syndrome to enable safe and proper treatment.