Marfan Syndrome: oral implication and management.

Abstract

Marfan's Syndrome is a multisistemic pathology of connective tissues, a dominant autosomal transmission, first discovered by a French pediatrician, Antoine Bernard-Jean Marfan, who first found in some of his patients a disproportionate alteration of inferior infertility. This alteration was caused by the mutation of the FBN1 gene, located on the long arm of the chromosome 15, which encodes for an extracellular matrix protein, fibrin-1. Later it was discovered that the disease could occasionally be due also to the mutation of the TGFBR2 gene, which encodes for a TGF-beta receptor 1. The estimated incidence of the disease is 2-3 subjects affected every 10,000, in the absence of predilection ratial, ethnic, geographic and gender. It is believed that some 15,000 people in Italy suffer from Marfan Syndrome. The disease is characterized by a wide range of clinical manifestations that affect different organs. The study evaluates through a literature review the manifestations in the oral cavity of the marfan syndrome and the correct management of the patient during dental maneuvers.