The Marfan Syndrome - is a disorder of connective tissue that is inherited as a simple dominant trait, is caused by a defect in the gene controlling the production of fibrillin. The majority of patients inherited the syndrome from a parent who is also affected. The incidence is 1 in 5,000-10,000 of the population. The condition affects both men and women of any race or ethnic group. The life threatening manifestation is aortic dissection that is related to the size of aortic dilatation.
Aortic diameter increases with age.
The severity differs from patient to patient. This condition was first described by a French pediatrician Antoine Marfan in 1896. Slit-lamp eye examination by an ophthalmologist. Skeletal examination by an orthopedist. Complete family history.