Síndrome de Marfan. Manifestaciones bucofaciales en el paciente pediátrico

Abstract

Introduction: Marfan Syndrome is an autosomic dominant genetic disorder of the elastic fibers of connective tissue. This autosomic dominant condition has an incidence of 2-3 per 10,000 children. Due to the natural evolution of the disease there is a progressive involvement of different organs or systems such as skeletal, cardiovascular, dura, ocular, skin-integument and lungs. However, the suspicion must arise on skeletal clinical aspects which are first evident signs. The cardiovascular involvement appears later but is the major life threatening complication. Purpose: Review the prevalence of known pediatric orofacial alterations of Marfan Syndrome. Methods: A systematic search in June 2012 in PubMed database with keywords: Marfan syndrome, pediatric dentistry, orofacial alterations and oral health. Results: These patients present high and narrow palatal vaults, crowding of teeth, extreme maxillary overjet and open bite were. Temporomandibular joint dysfunction appears to be an important aspect in Marfan Syndrome. Marfan Syndrome patients have medical conditions that can hinder the dental treatment. These conditions creates a predisposition to dental caries, periodontal disease and malocclusion. The orofacial alterations should be treated early to prevent further complications. Conclusions: Early diagnosis of dental and craniofacial anomalies is better for these patients and improve their quality of life. Good communication between the pediatrist and the dentist is essential for these patients.