1Department of Oral and Maxillofacial Surgery, Surendera Dental College and research institute, Sriganganagar, Rajasthan, India
2Jagdamba Charitable eye hospital, Sriganganagar, Rajasthan, India
3Surendera Dental College and research institute, Sriganganagar, Rajasthan, India
*Address For Correspondence: Bansal Shallu. BDS, MDS. Senior Lecturer Department of Oral & Maxillofacial Surgery Surendera Dental College and research institute Sriganganagar, Rajasthan India PH: +919414343004 Fax: +911542440102 drshallu23@yahoo.com
The Marfan syndrome (MFS), initially described just over 100 years ago, was among the first conditions classified as a heritable disorder of connective tissue. MFS lies at one end of a phenotypic continuum, with people in the general population who have one or another of the features of MFS at the other end, and those with a variety of other conditions in between. Marfan syndrome is characterized by a triad of features of long thin extremities associated with other skeletal changes, ectopia lentis and aortic aneurysm. The cardiovascular abnormalities are main source of morbidity and mortality. There is no established treatment but several investigations recommended use of B- adrenergic blockers to delay or prevent aortic dilatation and have resulted in considerable improvement in life expectancy.
Here we are presenting a case of 12 years old female who presented with arachnodactyly, chest wall deformity, long and thin extremities and ocular changes includes upward subluxation of the lenses, myopia and retinal detachment. The diagnosis of Marfan syndrome was based on clinical manifestation, echocardiogram and roentgenological test.
Marfan Syndrome, Arachnodactyly, Aortic dilatation
